11-01-2007, 08:39 AM
elbamaritza - 11/01/07 08:51
œPediatrics
--)Vesicoureteric reflex is the retrograde flow of urine from the bladder to the ureter n renal pelvis. Normally the ureter is attached to the bladder in oblique fashion, leading to a flap-valve mech. that prevent reflex. Reflex occurs when submucosal portion of ureter b/w the mucosa n detrusor muscle is short or absent, it is congenital n seen in1% of children”Reflex is a risk factor of recurrent UTI(results in renal scarring n major cause of ESRD n HTN in children) as it facilitate the transport of bacteria from bladder to the upper UT. Reflex is present in 35-40% of children with UTI(MCC) Dx is made by VCUG (voiding Cystouretherogram)----Ureteral duplication can lead to VUR n Ac UTI, but most of the VUR r congenital, run in families”due to anomalous intravesical portion of ureter
--)IVH(Intraventricular Hemorrhage), results from bleeding in the germinal matrix, occurs MC in Premature n low birth wt(LBWI) infants. Other predisposing factors r Hypoxia, ischemia or hypotensive injury, reperfusion of damaged vessles, inc venous pressure n inc or dec cerebral flow.”It is subdivided into 4 grades acc to the severity of the bleeding. Sx include pallor, cyanosis, Hypotension, seizures, focal neurogical signs, bulging or tense fontanel, apnea n bradycardia. However many cases r asymptomatic, thus mandating transfrontal u/s for all NB with predisposing factors.
--)Arthrocentesis n empiric Tx with IV nafcillin r the most appropriate measures for the Mx of suspected Septic Arthriis. SA can occure following skin or URTI., c/f r painful joint(knee), fever, chills, o/e swollen n erythematous joint with dec range of Nïƒ motion. Inc WBC, Inc ESR, x-ray
commonïƒ --)Hereditary Spherocytosis cause of hemolytic anemia in NB, present with anemia n inc bilirubin suff severe to require phototherapy or exchange transfusion, prevalent in people of Northern European descent, Inc retic count. Confirm by +ve Osmotic fragility test------Autoimmune Hemolytic Anemia is due to warm antibodies directed against RBCs, can present as Ac n transient type lasting 3-6m in children aged 2-12yrs, preceded by an inf, usually RTI.Considerable spherocytosis n polychromasia +ve . Coomb™s test +ve confirm the Dx
--)Ac Post streptococcal GN, MC form of GN in children, occurs after pharyngitis or pyoderma. Pt r hypertensive n edematous. 1- Complement Levels (C3 n CH50) r low at the onset of ds should resolve in 8-12wks, 2-Hematuria is microscopic n persist for 6m (persistent hematuria is s/o IgA nephropathy), 3”Proteinuria resolve slowly, upto 15% of pt persist for 3yrs, 4”Renel Fxn returns to normal in 1-2 wks, 5”ASO Titre, tends to rise after 7days,peak after 1m and return to normal in 3-4m
--)HUS, sudden onset of abd pain, bldy diarrhea, and a triad of anemia,thrombocytopenia and renel failure. Caused by a toxin released by E-Coli
--)Subarachnoid hemorrhage, char. by Ac onset of (migraine like)headache, vomiting, altered mental status, seizures n nuchal rigidity. MCC in children is AVM(atriovenous malformation) with rupture into subarachnoid space. Dx by CT scan.
--)In pt with Sicle cell Ds(SCD), acute severe anemia with absent reticulocytes in peripheral bld is due to aplastic crisis, ass with Parvovirus B19 inf and folat def, usually req bld trasnfusion. --Severe anemia with reticulocytosis is due to splenic sequestration, char. by vaso-occlusion withpooling of RBCs in spleen, occurs in those pt whove not yet developed auto-splenectomy. Pt may develop severe hypotensive shock ass with 10-15% mortality n has 50% chance of recurrence, splenectomy is usually recommended after the 1st episode.”Ac Chest Syndrome, MC complication of SCD, charc by chest pain, fever, and a new infilterate on chest x-ray
--)Autism, any pt <3yrs old present with impairement in social interaction n communication, delayed language development and stereotypical behaviors. Tx include special education n behavioral modification techniques
--)Hand-foot syndrome, or dactylitis, occurs in children ages 6m-2yrs. It is the earlist manifestation of vaso-occlusion in SCD. Dactylitis present with acute onset of pain and swelling, symmetrical involving the hands n feet due to vascular necrosis of metacarpal n metatarsal seen on x-ray as osteolytic lesion; however, initially, only soft tissue swelling is seen. Ac painful episodes in SCD is also due to vaso-occlusion triggered by infection, dehydration or hypoxia in some. Pt c/o severe unbearable pain involving the area of chest, abd, lower back , thigh n knees. Mx is hydration n opiod analgesics. Hydroxyurea is indicated in pt with frequent, acute, painful episodes (>6/yr), a cytotoxic agent that inc Hb F by stimulating erythropoiesis. HbF retard sickling, thus reducing vaso-occlusion
--)Galactosemia(due to galactose-1-phophate uridyle trnsferse def) should be considered in a NB or young infant who has failure to thrive, bilateral cataract, jaundice and hypoglycemia. Early Dx n Tx by elimination of galactose from the diet r mandatory. Pt are at inc risk of E coli N/N sepsis
--)Always suspect pyloric stenosis in a neonate who present with non-billous, projectile n persistent vomiting, (a mass due to hypertrophied pylorus may not b palpable), order U/S abd to establish dx
--)Increased gastric residues in a preterm neonate is highly suspicious of Necrotizing Enterocolitis, present at 3-10days after birth, due to bowl wall injury resulting from perinatal asphyxia. Tx depends on the degree of bowl involvement n severity.”The preterm baby is also at high risk of other comp. i-e Hayline Memb Ds, present with resp. distress n a ground glass appearance on chest x-ray.
--)Osteogenic Imperfecta is caused by mutation in type 1 collegen, charc by blue sclera n recurrent fractures.
--)Turner™s syndrome, usually cahrc by 45XO karyotype, however, variation in karyotype i-e 46XY also seen n ass with a higher incidence of Gonadoblastoma, ----hence prophylactic bilateral gonadectomy is indicated.
--)Meconium Aspiration Syndrome, MC in low birth wt n post-mature infants, charc by resp distress at birth, rales n rhonchi on auscultation, hyperinflation, coarse streaking n patchy opacities on chest x-ray.(The greenish color of the amniotic fluid indicates the presence of meconium)
--)Early N/N care in an uncomplicated preg. Include initial physical assessment, removal of airway secretions, drying the infant n keeping warm, n early preventive measures (gonoccal ophthalmia prevention, n vit K supplementation).
--)Strep Pneumonia(30%) is the MCC of Ac Bacterial Sinusitis in childhood, followed by H. Inf (20%) n Morexalla Catarrhalis(20%)
--)GERD in infants charc by regurgitation after feed, failure to thrive n Sandifer™s Syndrome(child assume a position with his head tilted to left side n his back arched) is the mech by which the child protect the airway n reacts to the pain of the acid reflux. The 24 hrs esophageal PH monitoring is the Gold standard for the Dx of GERD.
--)Duodenal atresia(bilious vomiting) is the MC GI anomaly in Down™s syndrome (double bubble sign) pt. ass with annular pancrease. Hirschsprung™s ds (no passage of stool after birth) also occurs with inc freq in pt with Down™s Syndrome.
--)Migraine Headache, no investigation needed unless neurological exam is abnormal and/or fever is +ve. Migraine is a clinical Dx n Tx is done initially with acetaminophen or NSAID.
--)Todd™s Paralysis represent a postictal condition that usually rapidly improves with restoration of motor function within 24hrs.
--)N/N sepsis suspected when a baby develops jaundice after the 3rd day n within the 1st wk, esp if he is feeding poorly n lethargic, work-up with blood c/s n LP is the next step.
--) DOC for nocturnal enuresis is DDAVP. Bed wetting is considered normal until the age of 4-5yrs. But if it persist either DDAVP or imipramine is used.
--)Medulloblastoma is the 2nd MC infratentorial (post fossa) tumor in children n arises from the Cerebellar Vermis.(1st one is Cerebellar Astrocytoma).it is highly radiosensitive n can metastasize thru CSF. Post Vermis Syndrome (Truncal Dystaxia) charc by unbalanced gait, horizontal nystagmus, papilledema.
--)Aseptic(Viral) Meningitis charc. By lymphocytic/monocytic predominance (generally total WBC <250), pr <150mg/d, and normal glucose.
--)Fetal Alcohol Syndrome, charc by irritability, mild-mod mental retardation, hypoplstic maxilla, long philtrum, thin upper lip border and microcephaly
--)Hyper-IgM Syndrome (HIM), charc. By inc level of IgM with deficiency of IgG, IgA n poor specific antibody response to immunization. HIM present with sinopumonary inf. and PCP. Unique susceptibility to opportunistic inf , neutropenia , with high IgM, distinguish HIM from XLA.
--Bruton™s agammaglobulinemia (XLA X-linked agammaglobulinemia) is charc by 1-Onset of recurrent bacterial inf in the 1st 5yrs of life, 2-Serum IgG, IgM and IgA values are at least 2SD below the normal for age, 3-Absent isohemagglutinins or poor respose to vaccines, 4-Less tha 2%CD19+Bcell in the peripheral blood
--)DiGeorge Syndrome (microdeletion of 22q11) is ass with cyanotic heart ds, craniofacial anomalis with low set ears n microganthia, thymic dysplasia(prone to recurrent inf, parathyroid glands r also missing), cognitive impirement and hypoparathyroidism. It is essential to monitor the Ca levels in these infants (Hypocalcemia leads to laryngeal spasm, convulsions, cramping n prolonged Q-T interval).
--)HIV testing should be offered to all pregnant women in the 1st trimester in order to prevent further transmission. Persistent oral thrush, lymphadenopathy n hepatosplenomegaly may be the presenting features of AIDS in infants
--)Children with a parentral h/o elevated total cholesterol level (>240mg/d), or risk factors for coronary artery disease should get a screening test for total cholesterol level.
--)Midgut Volvulus present in a child <1m with bilious vomiting, abdominal distension and n blood-stained stool.
--)Congenital Rubella Syndrome occurs most commonly following the transmission in the first 4wks of pregnancy. Classic triad of CRS”sensorineural deafness, cardiac malformation (PDA(MC), ASD), and cataract.
--)Iron Poisoning present with NVD, abd pain, GI bleeding n metabolic acidosis. As iron is radio-opaque, the tablets can be seen in the stomach on abd x-ray. Dx is confirmed by measuring serum iron level. Deferoxamine , an iron chelator, is used IV in mod-severe intoxication.
--)Gaucher™s diseases is dx based on radiological app of Erlenmeyer flask deformity of the distal femur, n charc Gaucher cells with their wrinkled paper appearance in BM examination.
--)MC mutation that leads to Cystic Fibrosis is a deletion of a three base pair encoding for phenylalanine (DA508) in the CFTR gene located on Ch 7, it is believed that this mutation prevent CFTR protein from trafficking to the correct cellular location----Meconium ileus, char by bilious vomiting and failure to pass meconium at birth, n groung glass apperanace on abd x-ray”diagnostic for Cystic Fibrosis”(AR)
--)Children with TOF will get ˜tet™ spells due to inc pulm vascular resistance leading to hyoxia. Crying, infection, and exercise can lead to ˜tet™ spell. There may be paroxysm of rapid n deep breathing. If the tet spells are severe, the infant may have seizures and loss of conciousness. Tx is O2 n placing a child in aknee-chest position, fluids, morphine n propranolol. Classical presentation of TOF child is squatting, which inc the systemic peripheral resistance n thus dec the Rt-Lt shunt n hence improves Cyanosis
--)Severe dehydration in a neonate in the presence of Hyponatremia, Hyperkalemia, Hypoglycemia and metabolic acidosis s/o Dx of CAH(Congenital adrenal hyperplasia). Classic salt wasting 21-hydroxylase def is inherited as an AR trait. Of particular note is the fact that male infants affected by this ds will not present with ambiguous genitalia at birth unlike female infants.
--)An epiglottis rolling in from side to side is the diagnostic finding of laryngomalacia, n all such babies should be fed in the upright postion n hold upright for half an hr after feeding, self limiting, subsides by 18m. The noisy breathing(stridor) charcterstically worsens when the child cries n while lying in supine position.
--) Mild cases of œMetatarsus adductus do not need any Tx n correct spontaneous.----Interanl tibial torsion is also a physiological finding in NB n spont resolution occurs in more than 95% of the children. Next step is reassurance .
--)Prevention of SIDS, infant should be placed on their backs while sleeping. Home monitor not dec the risk of SIDS.
--)ITP(Ideopathic Thrombocytopenia), children develop thrombocytopenis after viral infection, self limiting n spontaneous recovery occurs in the majority. Corticosteroids r the DOC in all age gps, when platelet count is <30,000 and/or symptoms r severe. Pt with a platelet count of >30,000 usually ve few symptoms n do not require Tx.
--)McCune-Albright syndrome is charc by (3P™s) Precocious puberty, Pigmentation(café-au-lai spot) and Polyostotic fibrous dysplasia(multiple bone defect). There is autonous stimulation of œaromatase enzyme production of estrogen by the ovaries It is responsible for 5% of the cases of female precocious puberty n may be ass with other endocrine abnormality, i-e hyperthyroidism, prolactin n GH-secreting pituitary adenomas n adrenal hypercortisolism. Mx is administration of aromatase enzyme inhibitor. Unlike VonRecklinghausen syndrome, the café-eu-lait spots r large , have irregular borders n r not ass with axillary and genital freckles. It is due to defect in the G-protein cAMP-kinase function in the affected tissue”gives autonomous activity
--)Kartegener™s syndrome(AR), is ass. with recurrent sinusitis, bronchiectasis, n dextrocardia. This is the result of primary ciliary dyskinesia.
--)Young chills who present with recurrent RTI and bilateral nasal polyps, Cystic Fibrosis must be r/o.
--)Rubella is char. by low-grade fever, lymphadenopathy (sub-occipital n post auricular) and rash (maculopapular).
--)Aplastic anemia(aquired) suspected in any pt with pancytopenia following drug intake, exposure to toxins or viral infections.
--)Guillain-Barre syndrome consists of demyelinization of the peripheral nerves. It may occur about 10days after a nonspecific GI and/ or RTI (campylobacter). O/E symmetric flaccid paralysis of both legs(ascending paralysis), absent deep tendon reflex and dec superficial touch n vibratory sense, no nuchal rigidity.Nerve conduction velocities r slowed indicating demyelinization. CSF shows a significant inc of protein levels n mildly inc cells(albumino-cytologic dissociation). Tx Plasmapheresis or human immunoglobulin.
--)Peroxisomal disorder such as Zellweger™s syndrome should be considered in N/N seizures, esp if the N/N present with typical facial dysmorphism (high forhead, up slanting palpebral fissures, hypoplastic supra-orbital ridges, epicanthal folds n redundant skin fold of the neck), severe weakness n hypotonia, N/N seizures, wide-open sutures, cloudy cornea, cataract or glaucoma, rarely live more than few months.
--)Three MC organism causes Pneumonia in Cystic Fibrosis pt are; 1-Hemophilus, 2-Pseudomonas, 3-Staphyloco.
--)The MCC of Amblyopia(dec in visual acuity) is strabismus (deviation of eye mostly inward), other causes include error of refraction n opacity of media along the visual axis. Dx by cover test. The standered of Tx is occlusion of normal eye-(HY)
--)Unilateral flank mass in a child <3yrs is highly suspicious of Neurobalstoma, a malignancy of the neural crest cells (displaces the kidney inferolaterally making the kidneys non-palpbale), whereas in a child >3yrs old, a unilateral flank mass is highly suspicious of Wilm™s tumor, originate from the metanephros (kidneys are palpable).
--)Asthma when it cannot be controlled by medication, should be an indication for mechanical ventilation---Racemic epinephrine may help a little in mod asthma, but when the condition is severe, its administration just delay the initiation of MV, Racemic epinephrine has a better role in Croup.
--)Leukocyte adhesion defect char. by recurrent bacterial infection, delayed separation of the umbilical cord >3wks, n necrotic periodontal infection. ( leukocyte # is inc, but the inter-leukocyte communication is defective. N lymphocyte n gamma globulin help to differentiate)
--)Cerebral anoxia is the MCC of Cerebral palsy charc. by impaired motor fxn,(hypotonia, inc deep tendon reflexes, n learning disability)
--)Infection by resp syncytial virus (Bronchiolitis) charc. by first episode of wheezing ass with URTI in wintor weather n affects 50% of children in 1st 2yrs of life---WBC is normal n chest x-ray shows air trapping n atelectasis Tx is supportive care with Humidified O2 n bronchodilators. There is an Increased risk of Asthma in later life
--)Kawasaki™s Disease is char. by fever, conjunctival vascular injection, cracked lips, polymorphous erythenatous rash, diffuse erythma of palm and soles and unilateral lymphadenitis, next step is echocardiogram to r/o coronary aneurysm. Tx is aspirin(due to anti-platelet n anti-inflammatory action) n IV Immunoglobulin. This is one of the condition in which aspirin is recommended, Influenza vaccine is recommended in pt with KD taking life-long aspirin.
--)Upper GI endoscopy is the diagnostic study of choice when a pt present with Acute Alkali ingestion(like oven cleaner, char by drooling with blood tinged secretion, lips n chin r swollen n erythematous, cloths r contaminated with material)
--)Reye™s syndrome is a rare illness seen in children <15yrs old treated with salicylates for a viral infection(C/I in children for viral inf), char by vomiting, agitation, lethargy, stupor n convulsion. Lab findings r Inc ammonia, N or slightly inc bilirubin and AlkPO4, inc prothrombin time, dec glucose, inc AST, ALT n lactate dehydrogenas. Tx is supportive.
--)Red eye in a NB(<24hrs) is due to Chemical irritation after silver nitrate prophylaxis(given to all NB at birth to prevent Gonococcal conjunctivitis), resolve generally in 24hrs---Gonococcal Conjunctivitis is the most destructive, present as hyperacute n highly purulent that appears 2-5days after birth, may results in corneal perforation n permanent loss of vision if left untreated. It is Tx with Tropical Erythromycin.----Chlamydia trachomatis causes the most infectious conjunctivitis in NB n appears even after prophylactic drops, it is milder than gonococcal n begins few days to several wks sfter birth, presents with congestion n a discharge, may be scant n mucoid or frankly purulent, Tx with Systemic Erythromycin b/c of risk of Pneumonia.
--)A Leukocoria (+ve white reflex), is a Retinoblastoma until proven otherwise, prompt ophthalmologist referral is required.---Retinoblastoma is the MC intraocular tumor of childhood, highly malignant (due to inactivation of Rb suppressor gene), if untreated leads invariably death due to liver n brain metastases. Other features include strabismus, dec vision, ocular inflammation, eye pain, glaucoma n orbital cellulites. Dx by clinical, n presence of calcification on US or CT scan.--HY
--)Growing pain, occurs at night only n no obvious physical or laboratory finding, Reassurance is the TOC.
--)Craniopharngiomas, derived from epithelial ramnant of Rathke™s pouch, not a true pituitary tumor, char by nests of squamous cells in a loose stroma, resembling the app of embryonic tooth bud enamel. Location is supraseller n inferior to optic chiasma. They cause Bi-temporal Hemianopia by pressing optic chiasma, also give s/s of Diabetes Insipidous. Dx”head X-ray shows calcified lesion above the sella. Next step will be MRI n may need Craniotomy.
--)N/N Tetanus, seen in infants born to unimmunized mothers, frequently followed umbilical stump infection, char by poor sucking n fatigue, followed by rigidity, spasm n opisthotonus in 1st 2wks of life.
--)In children<9yrs old, amoxicillin, for 21days is the recommended TOC for Erythma Migrans or Stage I Lyme Ds.
--)Cephalohematoma is a subperiosteal hemorrhage, present few hrs after birth as scalp swelling limited to one cranial bone, no discoloration, usually resorbed spontaneously within 2wks-3m depending upon the size. Rarely, Phototherapy required to improve the Hyperbilirubinemia.----Caput succedaneum is a diffuse, sometimes ecchymotic, swelling of the scalp, usually involves the portion of the head presenting during vertex delivery, may extend across the mid-line and across the suture-line.
--)Vit A has been shown to dec morbidity n mortality through immune enhancement. It also helps GI resp epithelium to regenerate
--)Iron supplementation in the form of iron fortified formula or medicinal iron, should be started at 6wks in all Premature and LBW babies.
--)Vaginal discharge(bleeding/secretion), due to effects of maternal estrogen, reassurance of the mother is required.
--)Fragile X syndrome, char by low to N IQ with learning disability, gen language disability, short attention span, autism, large head, prominent jaw, large low set ears n macro-orchidism(enlarge testes). 2nd MC of MR next to DS
--)Cri-du-chat (cry of the cat)syndrome (Deletion of 5p(MC), char by hypotonia, short stature, cat like cry, microcephaly with protruding metopic suture, moonlike face, hypertelorism, bilateral epicanthal fold, high arched palate, wide n falt nasal bridge n MR.
--)A 12 m old child walks alone, speaks 2words, throw objects n comes when called----Language development, socal smile “2m, Babbles--6m, 2words, obeys 1 step command---1yr, 2-3 word phrase, obeys 2 step commands---2yrs-----Gross Motor Development; Holds head---3m, Roll back to front n front to back---4m, Sits well unsupported”6m, walks alone”12m, Walks up n down stairs without help---24m------Fine Motor Development; Raking grasp”6m, Throw object---12m, Builds tower of 2 blocks”15m, Builds tower of 6 blocks/turn book pages”24m, ------Social Development; Recognize parents---2m, Recognize strangers(stranger anxiety)----6m, Imitates action/comes when called---12m, Plays with other children”18m, Parallel play---24m.
--)Supracondylar Fractures, MC # of peds, due to extension injuries, brachial artery may be compromised n radial artery pulse must be assessed when the # is reduced.-----The axillary artery is injured during Proximal humeral #.
--)Edward™s syndrome(Trisomy 18),char by microcephaly, prominent occiput, microganthia, closed fists with index finger overlapping the 3rd digit n the 5th digit overlapping the 4th, rocker bottom feet n MR. 95% die in the 1styr of life.
--)Epiglottitis seen in 3-7yrs old children, appears very toxic, ve stridor, high fever, drooling, tachypnea, n tachycardia. Lateral CXR shows,1---swollen epiglottis(thumb sign),2--thickened aryepiglottic folds, 3--obliteration of the vallecula( not wait for x-ray result). When child has stridor n dec O2 saturation, sudden airway obstruction may occur n intubation may be difficult, so preparation made for intubation n tracheostomy.
--)Mastitis is not a C/I to breast feeding, as it may actually be alleviate by continued n frequent nursing on the affected breast, which keeps it from becoming engorged. Additional measures, such as local heat application n antibiotic, can also be used.---Maternal HIV infection is an absolute C/I to breast feeding
--)Replacement of diarreal loss of fluid with lots of low solute fluid like water can lead to water intoxication, hyponatremia n even seizures.
--)Infant botulism, transmitted by ingestion of food contaminated with C-botulism(honey). The germ grows in GI n produce toxin (protease that block the acetylecholine). c/f ptosis, dilated pupil with sluggish light reflex,intact deep tendon reflexes, dec muscle tone, constipation, difficulty with breast feeding etc. Tx supportive
--)Pt with Sx of short attention span, impulsivity n hyperactivity, poor concentration for >6m in more than one setting(like school n home), persistently present since age 7, ADHD should be considered as the MC Dx.
--)Scarlet fever, char by fever, toxicity, pharyngitis, n sandpaper-like rash. Circumoral pallor n strawberry tongue also present, Dx +ve streptococcal throat test n normal appearing lips. TOC is 10days course of Penicillin V; however in Penicillin resistant pt , erythromycin/clindamycin can be used. Also Tx with penicillin does not prevent from Post-streptococcal GN.
--)In Mx of Enuresis, urinalysis should always be the 1st step, then behavioral modification (pass urine right b4 going to bed, no drinks b4 going to bed, wake up the pt in the night to pass urine), n finally meds (Imipramine n Desmopressin) r the DOC in refractory cases.
--)Diamond-Blackfan Anemia(DBA), Macrocytic pure red aplasia ass. with several congenital anomalies including short stature, webbed neck, cleft lip, shielded chest n triphalangial thumbs. On electrophoresis , fetal Hb is elevated n chromosomal studies r N. Tx is mainly corticosteroid n for unresponsive pt, Transfusion therapy is indicated.
--) Fanconi™s Anemia, (AR) char by progressive Pancytopenia n Macrocytosis, age of Dx is 8yrs, ass. Deformities include café-au-lait spots, microcephaly, microphthalmia, short stature, horseshoe kidney n absent thumb(not Triphalangial thumb)
--) Tx of Recurrent Aortic Coarctation is Balloon Angioplasty.
--)Triad of Henoch-Schonlein Purpura(HSP) or Anaphylactoid purpura, 1)-Petechial rash, 2)-Arthritis, 3)-Abd/renel complications, ass. with Bacterial(strept, mycoplasmal), Viral(adenovirus, varicella), Drugs(penicillin, erythromycin), n Vaccination ( Measles, typhoid, yellow fever). HSP causes intestinal intussusception, usually ileo-ileal. Barium enema may be “ve, Plain abd x-ray shows findings of intestinal obstruction, U/S shows the classical œDoughnut or target sign with hypoechogenic, edematous bowl, surrounding the central more echogenic, invaginated bowl (representing the intussusception). Tx is surgical reduction as they r located more proximally compared to the ileo-colonic intussusceptions n r not reducible by barium or air .
--)TCA(tricyclic antidepressant) Intoxication char by seizure, hypotension, dilated pupils, n QRS complex prolongation, After initial resuscitation, NaHCO3 is the DOC in pt experiencing a seizure or QRS complex prologation (correct acidosis n narrows the QRS complex)
--)Minimal change disease is the MCC of Nephrotic syndrome in pt younger than 16yrs old. Light microscopy n immunofluorescence shows normal findings. Electron microscopy shows diffuse effacement of foot processes of podocytes. Steroid r the TOC for 4-6wks, over 90% children demonstrate complete remission with disappearance of proteinuria.
--)Rotavirus is the 1st MCC of acute diarrhea in children, esp b/w 6m-2yrs. Norwalk virus is the 2nd MCC of non bacterial, acute diarrhea in children.
--)The growth of axillary hair results from androgen secretion by adrenal gland(adrenarche). Premature adrenarche or isolated appearance of axillary hair n thelarche b4 the age of 6yrs, is generally benign n has no clinical significance; however premature pubarche, or growth of pubic hair b4 th age of 8yrs, is more alarming, as it is ass. In 50% of cases with CNS disorder.
--)Perinaud™s syndrome consist of paralysis of vertical gaze, which may be ass with papillary disturbances n eyelid retraction(collier™s sign). It results from lesion of the rostral midbrain at the level of the superior colliculus n CNIII. It is most commonly caused by Germinomas n Pinealomas at this region. The endocrine syndrome(dark facial hair, deepening of voice n increasing ht at early age(10yrs) results from interruption of hypothalamic inhibiting pathways, n sometimes beta HCG secretion n consequent Leydig™s cell stimulation.
--)Vit A deficiency, MC in 2-3yrs old child present with dry eyes n skin, photophobia, n impaired dark adaptation, results in night blindness. Later, drying of conjunctiva(xerosis conjunctivae), n of cornea(xerosis cornea) followed by wrinkling n cloudiness of cornea (keratomalacia)are seen. Bitot spot(silver-gray plaque may appear on the bulbar conjunctiva
--)Marfan™s syndrome(MFS) (AD), resulting from the mutation of the fibrillin-1(FBN1) gene, present with tall stature. Long n amaciated extremities, aracnodactyly, hypermobility of the joints, upward lens dislocation and aortic root diltation.
--)Racemic epinephrine decrease the need for intubation in pt with croup(Laryngeotracheobrnochitis) n should always be tried b4 any invasive procedure.
--)Dermatitis Herpetiformis and chronic non-bloody diarrhea in a child of 12-15m, s/o Celiac disease(gluten sensitive enteropathy), when gluten containing food such as wheat, rye or barley are introduced into the child™s diet
--)Hyaline Memb Ds(HMD), seen in preterm infants(60-80% <28wks) with resp distress soon after birth and hypoxia not responding to Oxygen therapy,CXR shows fine reticular granularity of parenchyma. Tx early with mechanical ventilatation and surfactant
--) In a pt with severe bouts of coughing n subcutaneous emphysema, a pneumothorax must be r/o by CXR
--)Vit D def Rickets is more common in dark skinned infants who r less exposed to sunlight n r 1-2 yrs of age. It presents with craniotabes, rachitic rosary, n thickening of the lower end of long bones, due to defective demineralization
--)Wilm™s tumor diagnosed b/w of 2 and 5yrs, MC primary renal neoplasm in children, in 10% of cases is bilateral. In 80% of cases, an asymptomatic abd mass is present. Other may present with HTN, abd pain and vomiting, in few it may present with lung mets. It is ass with Beckwith-wiedeman and Denys-drash syndromes.Tx is nephrectomy.”If child age is <1yr think of neuroblastoma.
--)Guillain-barre syndrome consist of demyelinization of the peripheral nerves. It may occur abt 10days after a nonspecific GI and/or respiratory infection.TX is Plasmapheresis or human immunoglobulin
--)Stranger Anxiety start anywhere b/w 6-8m ,and peaks at 12-15m. It is the normal anxiety experienced by infants on exposure to individuals unfamiliar to them.
--)Nursemaid™s elbow (subluxed radial head) is one of the MC elbow injuries I young children b/w 1-5yrs. It occurs when infan or child are lifted or pulled by the hand or arm results in radial head slipping through parts of annular ligament resulting in injury. No significant swelling or angular deformity should be visible. The hand typically held in pronated position with refusal and pain with attempted forearm supination. Rotating the hand and forearm to a supinated position with pressure over the distal head usually reduces the annular ligament with a click and restores full, normal use of extremity---Diagnostic
--)Cystic fibrosis, presents with failure to thrive, occurs due to fat and protein metabolism from a pancreatic insufficiency.
--)Croup(laryngotrachietis/laryngotraceobronchitis) is caused by Parainfluenza virus commonly present in children < 3ys with hoarseness, a barking cough, and varying degree of resp distress, Dx clinically, Lateral neck x-ray shows sub-glottic narrowing.
--)Spondylolisthesis is a development disorder char by a forward slip of vertebrae (usually L5-S1)that usually manifest in predolescent children. C/F back pain, neurological dysfunction (e.g urinary incontinence), and a palpable œstep-off at the lumbosacral area present if the disease is severe.
--)Duchenne muscular dystrophy char by proximal muscle weakness and exhibits a Gower™s sign with depressed reflexes and pseudohypertrophy, obesity, MR and cardiomyopathy. Dx by muscle biopsy n family pedigree, nowadays, the most appropriate way to confirm the dx of DMD is genetic (DNA)studies. Tx supportive and most pt are wheel chair bound by 12yrs of age.
--)ALL is the MC leukemia in children. 1st Sx is non-specific(lethargy, anorexia n irritability). o/e pallor, hepatosplenomegaly n/or lymphadenopathy.Dx by anemia, thrombocytopenia and blast cell on bld smear, confirmed by bone marrow biopsy(>25% blast cell)
--)Myotonic muscular dystrophy(MMD) is an AD disorder and is 2nd MC muscular dystrophy in North America, involves striated as well as smooth n cardiac muscle, ass with multiple endocrinopathies, immunologic deficiencies, cataract, neurological abnormalities, intellectual impairement and dysmorphic facies. Pt r usually normal at birth, Sx begins in childhood and include weakness and progressive muscle wasting esp in the distal muscle of the hands, post forearm muscle and anterior compartment of the lower leg. Prox muscles are subsequently affected leading to difficulty in walking n climbing stairs, a +ve Gower™s sign and winging of the scapulae. Myotonia char by delayed relaxation of the affected muscles after contraction(inability of the pt to release the handshake). Ass endocrine abnormalities includes testicular atrophy, DM, frontal baldness n hypothyroidism. CNS ---Normal
--)Rheumatic fever char by chorea, fever, pericarditis(friction rub), arthritis, erythma marginatum , s/c nodules, Inc ESR, prolonged PR interval due to Gp A strep. Dx by +ve ASO titre, c/s and antigen test. Tx Penicillin
--)Friedreich ataxia (AR), MC type of spinocerebeller ataxias, Sx begins <22yrs n include gait atxia, frequent falling n dysarthria due to degeneration of spinal tract(spinocerebeller tract, post column, pyramidal tract). Also ass with Conc hypertrophic cardiomyopathy(MC 90%), DM n sk deformaities(scoliosis, hammer toes). Median survival rate <20yrs after Dx. MCC of death in these pt are cardiomyopathy n resp comp.(HY).
--)Phenyleketonuria(AR) due to def in phenylalanine hydroxyalse (enzyme that breakdown phenylalanine into tyrosine) causing accumulation of phenylalanine and its metabolic product in the blood n bldy tissue(brain). If untreated can cause MR n seizures ( mousy odor in urine). MC screening test is blood phenylalanine level. Guthrie test, qualitative (coloration) test, detect the presence of metabolic product of phenylalanine(PA) in the urine. Tx low PA diet(little amounts r necessary for growth n development). Cereals, starches, fruits, vegetables n PA free milk formula should be recommended n high protein foods avoided .
--)Lesch-Nyhan syndrome(seen in male) is sec to def in HRPT(Hypoxanthine-guanine phophoribosyl transferase), an enzyme involved in purine metabolism, results in inc level of uric acid which accumulate in peripheral tissue. It presents with self-mutation n neurological features including MR, dystonia, choreoathetosis n spasticity. Uric acid causes gouty arthritis, tophus formation n obstructive nephropathy. Tx Allopurinol n inc fluid intake .
--)Mongolian spot MC in dark skinned population, present from birth n usually fades n then disappears in several years caused by entrapment of melanocytes in the dermis during their migration from neural crest into epidermis in fetal development. No Tx.
--)Severe combined immune deficiency(SCID), a life threatening syndrome presents with recurrent bacterial, viral and fungal infection. Dx by absence of L.nodes n tonsils, lymphopenia n absence of thymic shadow on CXR.
--)Group B streptococcus is the MCC of n/n sepsis. Early sepsis present within the 1st wk of life n usually involves several organs. Late-onset GBS infection is usually a focal infection, most frequently meningitis
--)Benign/Innocent Mumurs; most cardiac murmurs in childhood r benign n require only observation. Features of benign murmurs are;---1-Asymptomatic pt, 2-Murmur intensity grade 2or< , 3-Normal S2, 4-No audible clicks, 5-Normal puses, 6-No other abnormality.
--)Neurofibromatosis: an AD ds ass with multisystem manifestations. 6or> coffee color macules measures ~0.7mm on upper trunk with s/c nodules on extremeties, also there are nodular swelling protruding from irises most likely Hamatomas. Pt are at inc risk of developing Gliomas, presents with pale optic discs n dec visual acuity. Tx not specific, regular bld monitoring and annual CNS and opthalmological exam for early signs of optic gliomas n other CNS tumors.----Tuberous sclerosis---char by the development of renal angiomyolipomas
--)Clavicle Fracture(shoulder dystocia) are common comp in mothers with GDM and large babies with prolonged labour, dx by asymmetric Moro reflex n crepitus over the clavicle., does not need Tx-----Erb-duchenne™s paralysis is another complication in large infants n difficult labor, presents with the arm in adduction n pronation, n wrist n fingers in flexion c/a wiater™s tip position due to paralysis of C5 n C6 nerve roots of the brachial plexus, sec to traction on the infant™s head during delivery.
--)Infratentorial tumors r more common than supratentorial tumors, n benign astromas r the MC histological type in both gp (CNS tumors r the MC solid tumors in children, n the 2nd MC malignancies in the pediatric population, after leukemias-----Medulloblastoma is the 2nd MC tumor of post fossa in children. ~90% of cases arises from vermis.-----Glioblastoma multiforme is the highly malignant astrocytic tumor more common in adults----Pinealoma develops in the dorsal aspect of the midbrain. Sx consist of endocrine syndrome, intracranial HTN, Perinaud™s sign(paralysis of vertical gaze)and Collier sign(retraction of the eyelid)-----Craniopharyngiomas arise in the sella turcica, n Sx usually include visual field defects n endocrine syndrome, char by a cystic structure with presence of calcification.
--)Foreign body aspiration, presents as sudden onset of resp distress in an otherwise normal child. CXR shows finding of hyperinflation of the Rt lung with the mediastinal shift to the Lt n a severely hypoinflated Lt lung. Such pt requires immediate direct laryngoscopy n rigid bronchoscopy;both diagnostic n therapeutic purposes
--)Intussusception, the MCC of intestinal obstruction in the 1st two yrs of life, presents with intestinal obstruction n red current jelly stools containing blood n mucus, mostly idiopathic, but in some cases, it may be sec to intestinal mass i-e polyp, lymphoma, hamartoma or Meckel™s diverticulum. MC type is iliocolic.
--)Physiological jaundice usually presents after 24hrs of birth. Any jaundice in the 1st 24hrs of birth, serum bilirubin rising at a rate > 5mg/d/24hrs, serum bilirubin >12mg/d in full-term(in the absence of risk factor) or 10-14mg/d in preterm infants, jaundice persist after 10-14 days of life(DOL) or direct hyperbilirubinemia,>2mg/d needs detailed evaluation for the cause of jaundice------Bacteral sepsis/UTI----Jaundice appears 3rd-7th DOL.--------Breast milk jaundice---appears after 1st wk of life.Cessation of breast feeding for 1-2 days n substitution for formula results in a rapid decline in serum bilirubin, after which nursing can be resumed.----Biliary Atresia, presents any time since birth with conjugated hyperbilirubinemia ass with acholic stool n dark urine------Erythroblastosis fetalis, resents at birth or within the 1st 24hrs of life(direct/indirect). Hemolysis s/o by rapid rise of serum bilirubin(>0.5mg/d/hr), anemia, pallor, reticulocytosis, n hepatosplenomegaly
--)Conduct disorder, char by disruptive behavioral patter that violate basic social norm presents for >1yr, in pt <18yrs old.-------Antisocial personality disorder”pt are > 18yrs old.
--)Congenital cataract is the MC cause of Leukocoria (not retinoblastoma). Causes of cataract include familial, congenital infections, metabolic conditions(e.g DM, Galactosemia), genetic disorders (e.g Down™s syndrome, Turner™s syndrome), n long term/high dose steroid use
--)Transient synovitis of the hip char by an inflammation n swelling of the tissue around the hip joint, cause is unknown may be due to virus or sec to an allergic reaction to an infection somewhere else in the body. Bed rest with affected joint in the position of comfort is the TOC, Pt usually gets better in 3-4 days.
--)Trachoma, caused by Chlamydia trachomatis is the MCC of blindness in the world. Dx presence of lymphoid follicles on the conjunctiva, scarring n limbal follicles. Tx erythromycin or tetracycline.
--)Esophageal atresia presents with chocking, regurgitation n coughing ass with the 1st feeding of the NB infant. The inability to pass a feeding tube into the stomach s/o n U/S confirm the diagnosis.
--)Meckel™s diverticulum presents with painless melena in 2-3 yrs old children, present in 2-3% of population n MC anomaly of the GIT. Heterotropic gastric tissue may be present in the diverticulum, results in ulceration n bleeding.
--)Osgood-Schlatter disease (age 10-17yrs), presents with knee pain at the tibial tuberosity (at the site of insertion of the quadriceps tendon)
--)N/N tetnus, seen in infants born to immunized mothers, frequently following umbilical stump infection, n is char by poor suckling and fatigue, followed by rigidity, spasm n opisthotonus in 1st 2wks of life
--)Chronic Granulomatous dz”caused by a defect of phagocytes cells with dysfunction of the NADPH oxidase enzyme complex, leading to recurrent and uncontrolled infections with catalase-positive organism. MC clinical findings are lymphadenopathy, hypergammaglobulinemia, hepatomegaly, splenomegaly, anemia of ch dz, under wt, ch diarrhea, short stature, gingivitis n dermatitis. Dx by Nitroblue tetrazolium (NBT) slide test, flow cytometry or cytochrome C reduction. Tx “-Trimethoprim-sulfamethoxazole/day and gamma-interferon 3times/wk, BM transplantation is experimental but curative.
--)WAS(Wiskott-aldrich syndrome)caused by defective gene encoding for WAS protein, presents with eczema, microcytic thrombocytopenia n recurrent infection with encapsulated germs in young boy, present at birth n consist of petechiae, bruises, bleeding from circumcision or bldy stools. Dx by low IgM, and high IgA n IgE n moderately dec Tcell n platelets.
--)Chediak-Higashi syndrome char by dec degranulation, chemotaxis n granulopoisis, multisystem dz char by mild coagulopathy, peripheral n cranial neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, freq bacterial infections(S aureus), n progressive lymphoproliferative syndrome. Dx neutropenia n giant lysosomes in neutrophil. Tx trimethoprim-sulphamethaxazole n Ascorbic acid/ daily
--)Hyper-IgE(job™s) syndrome char by ch pruiritic dermatitis, recurrent staph inf(skin, resp tract n joints), markedly elevated IgE, eosinophilia n coarse facial features, dental abnormality n bone # r diagnostic.
--)Whooping cough caused by Bordetella pertussis, highly contagious infection, presents with severe bouts of coughing rectal prolapse, epistaxis n pneumothoraces, may last from 2-10wks.ïƒ spells can Tx is erythromycin for 14days, regardless of the age or the immunization status, n regardless of the stage of the dz, Prevention”all close contacts should be given erythromycin for 14 days, regardless of age, immunization, or Sx.
--)The introsseous technique is an excellent site for IV access in children
--)Systemic form of Juvenile RA(Still™s dz), char by fever, fleeting maculaopapular rashes, hepatosplenomegaly, lymphadenopathy, pleuropericarditis n myocarditis. RF rarely +ve. Tx NSAID therapy with monitoring of Liver enzyme. In refractory cases steroids r used.
--)Absence seizure is a generalized seizure of childhood n is char by sudden n brief lapses of conciousness without loss of postural control n absent postictal confusion, usually present with multiple œdaydreaming episodes n decline in school performance.
--)Postexposure prophylaxis for chicken pox is provided with VZIG (varicella Zoster mmune Globulin) or Acyclovir. VZIC is preferred n indicated in susceptible high risk pt exposed to varicella within 96hrs (preferably 72hrs) of exposure.
--)Pubertal gynecomastia, seen in ~1/2 of adolescent boys, at an average age of 14yrs, often asymmetric or transiently unilateral, frequently tender, regresses within 6-18m n just need observation”HY”
--)Tuberous sclerosis, presents with infantile spasm, often during 1st yr of life with cluster of brief symmetrical contraction of the neck, trunk n extremeties with unique pattern on EEG c/a Hypsarrhthmia (chaotic, high voltage, bilateral, asynchronous, slow wave activity), hypopigmented lesions(ASH leaf spots) n cortical tubers on CT head r charcterstics. Tx for infantile spasm is ACTH i/m.
--)Klumpke™s paralysis is a brachial palsy that occurs in NB following excessive traction on the arm, consists of hand paralysis n ipsilateral Horner™s syndrome (ptosis n miosis), is sec to injury to 7th n 8th cervical nerves n 1st thoracic nerve
--)Hyaline membrane dz(HMD), occurs in premature infants 60-80% <28wks of GA.-, caused by dec perfused but non-ventilatedïƒ atelectasisïƒ production n secretion of Surfactant hypoxia n resp distress not responding to O2 therapy, Tx early withïƒ alveoli Mechanical ventilation n Surfactant
--)Fulminant meningococcemia occurs after meningococcus inf, ~10-20% infants will presents with vasomotor collapse, large petechiae n purpuric lesion due to Adrenal Heamorrhage”caries 100% mortality(Waterhouse-Friederchsen syndrome).
--)Friedreich Ataxia an AR disorder(genetic counceling is recommended for prenatal dx for parents with one affected child) char by an excessive number of trinucleotide repeat sequences, resulting in an abnormality of a tocopherol transfer protein, ass with necrosis n degeneration of cardiac fibers leading to myocarditis, myocardial fibrosis n cardiomyopathy. Cardiac arrhythmia n CHF r the MCC of death. Most pt r wheelchair bound by the age of 25, with death occurring by 30-35yyrs of age MRI shows marked atrophy of the cervical spinal cord n minimal cerebellar atrophy. Dec vibratory n position sense in LL n ankle jerk r absent bilaterally
--)Congenital Hypothyroidism; In USA MCC is thyroid dysgenesis
--) Ch peylonephritis char by focal parenchymal scarring n blunting of calcies on IVP
--)Sicle cell Trait”most commonly presents as painless gross hematuria.----Pt with splenectomy or functional asplenia (SCD pt have at the age of 2-3yrs repeated microinfarction due to clumping of SCs) r at risk of infection with capsulated org like Pneumococcus n H. influenza. MCC of sepsis in SCD pt is Pneumococcus.
--)Homocystinuria (AR) = Marfan™s features + MR + Thromboembolitic events + downward dislocation of the lens, caused by cystathionine synthase def. DxInc both homocystine n methionine(metabolite) in body fluids. Tx high doses of Vit B6. Restriction of methionine along with supplementation of cysteine is used for refractive pt
--) Mast cell stabilizers(Na cromolyn) r the DOC for asthmatic pt who have evidence of other allergic disorders
--) Cong Toxoplasmosis”triad of chorioretinitis, hydrocephalus n intracranial calcification, acquired by exposure to feces from infected cats n ingestion of raw meat n unpasteurized goat™s milk. Risk of transmission during inc as the preg porogresses , but severity of the N/N dz dec
--)Suspected Diaphragmatic hernia, 1st step in the Mx is the immediate placement of orogastic tube (b4CXR) n connecting it to continuous suction, in order to prevent bowl distension n further lung compression. Endotracheal intubation n mechanical ventilation r also priorities for all infants with severe CDH who present in 1st hr of life
--)Measles(Rubeola) char by fever, non-productive cough, coryza, non-purulent conjunctivitis n koplik spots, pathognomonic bluish white lesions on buccal mucous memb, opposite to 1st n 2nd upper molars, n sometimes on inner conjunctivae n vaginal mucosa. Lab findings r Leucopenia n lymphopenia, proteinuria may be present, Dx is clinical
--)Tricuspid atresia”cyanotic CHD char by cyanosis early in the life n left axis deviation, single S2, most caese90% r ass with VSD ( char by holosystolic murmur) n 30% r ass with TGA. Tx infant should receive PGE1 to keep ductus open, n have balloon arterial septostomy if ASD is not large enough.
--)Choanal atresia, Cyanosis , aggravated by feeding n relieved by crying, MC nasal malformation. In children who r unable to breathe through the mouth, it is more severe n presents with cycles of cyanosis n relieved after the distressed infant start crying. Unilateral remains undiagnosed until the infant develops his 1st URTI. Dx by the failure to pass a catheter through the nose 3-4cm into the orophaynx. 1st step in Mx is placing an oral airway n lavage feeding. Dx is confirmed by CT scan shows narrowing at the level of pterygoid plate.ïƒ with I/nasal contrast Definitive Tx is repairing the obstruction with surgery or endoscopy
--)Displaced anterior fat pad is a radiographic sign of supracondylar fracture of Humerous, which may be complicated by Volkmann™s ischemic contracture. Tx immediate compartment release fasciotomy to prevent damage.
--) Meningitis may be complicated by regression of developmental milestones (inability to identify colors n to copy triangles n squares.
--) Meningococcus is the MCC of bacterial meningitis in pt b/w 2-18yrs. Pneumococcus is the 2nd MCC i
Tanner
Pubic hair (both male and female)
Tanner I -- no pubic hair at all [10 and under] (prepubertal state)
Tanner II -- small amount of long, downy hair with slight pigmentation at the base of the penis and scrotum (males) or on the labia majora [10-11](female)
Tanner III -- hair becomes more coarse and curly, and begins to extend laterally [12-14]
Tanner IV -- adult-like hair quality, extending across pubis but sparing medial thighs [13-15]
Tanner V -- hair extends to medial surface of the thighs [16+]
Genitals (male)
Tanner I -- prepubertal (testicular volume less than 1.5 ml; small penis of 3 cm or less)
Tanner II -- testicular volume between 1.6 and 6 ml; skin on scrotum thins, reddens and enlarges; penis length unchanged
Tanner III -- testicular volume between 6 and 12 ml; scrotum enlarges further; penis begins to lengthen to about 6 cm
Tanner IV -- testicular volume between 12 and 20 ml; scrotum enlarges further and darkens; penis increases in length to 10cm and circumference
Tanner V -- testicular volume greater than 20 ml; adult scrotum and penis of 15 cm in length
Breasts (female)
Tanner I -- no glandular tissue; areola follows the skin contours of the chest (prepubertal)
Tanner II -- breast bud forms, with small area of surrounding glandular tissue; areola begins to widen
Tanner III -- breast begins to become more elevated, and extends beyond the borders of the areola, which continues to widen but remains in contour with surrounding breast
Tanner IV -- increased breast size and elevation; areola and papilla form a secondary mound projecting from the contour of the surrounding breast
Tanner V -- breast reaches final adult size; areola returns to contour of the surrounding breast, with a projecting central papilla.
œPediatrics
--)Vesicoureteric reflex is the retrograde flow of urine from the bladder to the ureter n renal pelvis. Normally the ureter is attached to the bladder in oblique fashion, leading to a flap-valve mech. that prevent reflex. Reflex occurs when submucosal portion of ureter b/w the mucosa n detrusor muscle is short or absent, it is congenital n seen in1% of children”Reflex is a risk factor of recurrent UTI(results in renal scarring n major cause of ESRD n HTN in children) as it facilitate the transport of bacteria from bladder to the upper UT. Reflex is present in 35-40% of children with UTI(MCC) Dx is made by VCUG (voiding Cystouretherogram)----Ureteral duplication can lead to VUR n Ac UTI, but most of the VUR r congenital, run in families”due to anomalous intravesical portion of ureter
--)IVH(Intraventricular Hemorrhage), results from bleeding in the germinal matrix, occurs MC in Premature n low birth wt(LBWI) infants. Other predisposing factors r Hypoxia, ischemia or hypotensive injury, reperfusion of damaged vessles, inc venous pressure n inc or dec cerebral flow.”It is subdivided into 4 grades acc to the severity of the bleeding. Sx include pallor, cyanosis, Hypotension, seizures, focal neurogical signs, bulging or tense fontanel, apnea n bradycardia. However many cases r asymptomatic, thus mandating transfrontal u/s for all NB with predisposing factors.
--)Arthrocentesis n empiric Tx with IV nafcillin r the most appropriate measures for the Mx of suspected Septic Arthriis. SA can occure following skin or URTI., c/f r painful joint(knee), fever, chills, o/e swollen n erythematous joint with dec range of Nïƒ motion. Inc WBC, Inc ESR, x-ray
commonïƒ --)Hereditary Spherocytosis cause of hemolytic anemia in NB, present with anemia n inc bilirubin suff severe to require phototherapy or exchange transfusion, prevalent in people of Northern European descent, Inc retic count. Confirm by +ve Osmotic fragility test------Autoimmune Hemolytic Anemia is due to warm antibodies directed against RBCs, can present as Ac n transient type lasting 3-6m in children aged 2-12yrs, preceded by an inf, usually RTI.Considerable spherocytosis n polychromasia +ve . Coomb™s test +ve confirm the Dx
--)Ac Post streptococcal GN, MC form of GN in children, occurs after pharyngitis or pyoderma. Pt r hypertensive n edematous. 1- Complement Levels (C3 n CH50) r low at the onset of ds should resolve in 8-12wks, 2-Hematuria is microscopic n persist for 6m (persistent hematuria is s/o IgA nephropathy), 3”Proteinuria resolve slowly, upto 15% of pt persist for 3yrs, 4”Renel Fxn returns to normal in 1-2 wks, 5”ASO Titre, tends to rise after 7days,peak after 1m and return to normal in 3-4m
--)HUS, sudden onset of abd pain, bldy diarrhea, and a triad of anemia,thrombocytopenia and renel failure. Caused by a toxin released by E-Coli
--)Subarachnoid hemorrhage, char. by Ac onset of (migraine like)headache, vomiting, altered mental status, seizures n nuchal rigidity. MCC in children is AVM(atriovenous malformation) with rupture into subarachnoid space. Dx by CT scan.
--)In pt with Sicle cell Ds(SCD), acute severe anemia with absent reticulocytes in peripheral bld is due to aplastic crisis, ass with Parvovirus B19 inf and folat def, usually req bld trasnfusion. --Severe anemia with reticulocytosis is due to splenic sequestration, char. by vaso-occlusion withpooling of RBCs in spleen, occurs in those pt whove not yet developed auto-splenectomy. Pt may develop severe hypotensive shock ass with 10-15% mortality n has 50% chance of recurrence, splenectomy is usually recommended after the 1st episode.”Ac Chest Syndrome, MC complication of SCD, charc by chest pain, fever, and a new infilterate on chest x-ray
--)Autism, any pt <3yrs old present with impairement in social interaction n communication, delayed language development and stereotypical behaviors. Tx include special education n behavioral modification techniques
--)Hand-foot syndrome, or dactylitis, occurs in children ages 6m-2yrs. It is the earlist manifestation of vaso-occlusion in SCD. Dactylitis present with acute onset of pain and swelling, symmetrical involving the hands n feet due to vascular necrosis of metacarpal n metatarsal seen on x-ray as osteolytic lesion; however, initially, only soft tissue swelling is seen. Ac painful episodes in SCD is also due to vaso-occlusion triggered by infection, dehydration or hypoxia in some. Pt c/o severe unbearable pain involving the area of chest, abd, lower back , thigh n knees. Mx is hydration n opiod analgesics. Hydroxyurea is indicated in pt with frequent, acute, painful episodes (>6/yr), a cytotoxic agent that inc Hb F by stimulating erythropoiesis. HbF retard sickling, thus reducing vaso-occlusion
--)Galactosemia(due to galactose-1-phophate uridyle trnsferse def) should be considered in a NB or young infant who has failure to thrive, bilateral cataract, jaundice and hypoglycemia. Early Dx n Tx by elimination of galactose from the diet r mandatory. Pt are at inc risk of E coli N/N sepsis
--)Always suspect pyloric stenosis in a neonate who present with non-billous, projectile n persistent vomiting, (a mass due to hypertrophied pylorus may not b palpable), order U/S abd to establish dx
--)Increased gastric residues in a preterm neonate is highly suspicious of Necrotizing Enterocolitis, present at 3-10days after birth, due to bowl wall injury resulting from perinatal asphyxia. Tx depends on the degree of bowl involvement n severity.”The preterm baby is also at high risk of other comp. i-e Hayline Memb Ds, present with resp. distress n a ground glass appearance on chest x-ray.
--)Osteogenic Imperfecta is caused by mutation in type 1 collegen, charc by blue sclera n recurrent fractures.
--)Turner™s syndrome, usually cahrc by 45XO karyotype, however, variation in karyotype i-e 46XY also seen n ass with a higher incidence of Gonadoblastoma, ----hence prophylactic bilateral gonadectomy is indicated.
--)Meconium Aspiration Syndrome, MC in low birth wt n post-mature infants, charc by resp distress at birth, rales n rhonchi on auscultation, hyperinflation, coarse streaking n patchy opacities on chest x-ray.(The greenish color of the amniotic fluid indicates the presence of meconium)
--)Early N/N care in an uncomplicated preg. Include initial physical assessment, removal of airway secretions, drying the infant n keeping warm, n early preventive measures (gonoccal ophthalmia prevention, n vit K supplementation).
--)Strep Pneumonia(30%) is the MCC of Ac Bacterial Sinusitis in childhood, followed by H. Inf (20%) n Morexalla Catarrhalis(20%)
--)GERD in infants charc by regurgitation after feed, failure to thrive n Sandifer™s Syndrome(child assume a position with his head tilted to left side n his back arched) is the mech by which the child protect the airway n reacts to the pain of the acid reflux. The 24 hrs esophageal PH monitoring is the Gold standard for the Dx of GERD.
--)Duodenal atresia(bilious vomiting) is the MC GI anomaly in Down™s syndrome (double bubble sign) pt. ass with annular pancrease. Hirschsprung™s ds (no passage of stool after birth) also occurs with inc freq in pt with Down™s Syndrome.
--)Migraine Headache, no investigation needed unless neurological exam is abnormal and/or fever is +ve. Migraine is a clinical Dx n Tx is done initially with acetaminophen or NSAID.
--)Todd™s Paralysis represent a postictal condition that usually rapidly improves with restoration of motor function within 24hrs.
--)N/N sepsis suspected when a baby develops jaundice after the 3rd day n within the 1st wk, esp if he is feeding poorly n lethargic, work-up with blood c/s n LP is the next step.
--) DOC for nocturnal enuresis is DDAVP. Bed wetting is considered normal until the age of 4-5yrs. But if it persist either DDAVP or imipramine is used.
--)Medulloblastoma is the 2nd MC infratentorial (post fossa) tumor in children n arises from the Cerebellar Vermis.(1st one is Cerebellar Astrocytoma).it is highly radiosensitive n can metastasize thru CSF. Post Vermis Syndrome (Truncal Dystaxia) charc by unbalanced gait, horizontal nystagmus, papilledema.
--)Aseptic(Viral) Meningitis charc. By lymphocytic/monocytic predominance (generally total WBC <250), pr <150mg/d, and normal glucose.
--)Fetal Alcohol Syndrome, charc by irritability, mild-mod mental retardation, hypoplstic maxilla, long philtrum, thin upper lip border and microcephaly
--)Hyper-IgM Syndrome (HIM), charc. By inc level of IgM with deficiency of IgG, IgA n poor specific antibody response to immunization. HIM present with sinopumonary inf. and PCP. Unique susceptibility to opportunistic inf , neutropenia , with high IgM, distinguish HIM from XLA.
--Bruton™s agammaglobulinemia (XLA X-linked agammaglobulinemia) is charc by 1-Onset of recurrent bacterial inf in the 1st 5yrs of life, 2-Serum IgG, IgM and IgA values are at least 2SD below the normal for age, 3-Absent isohemagglutinins or poor respose to vaccines, 4-Less tha 2%CD19+Bcell in the peripheral blood
--)DiGeorge Syndrome (microdeletion of 22q11) is ass with cyanotic heart ds, craniofacial anomalis with low set ears n microganthia, thymic dysplasia(prone to recurrent inf, parathyroid glands r also missing), cognitive impirement and hypoparathyroidism. It is essential to monitor the Ca levels in these infants (Hypocalcemia leads to laryngeal spasm, convulsions, cramping n prolonged Q-T interval).
--)HIV testing should be offered to all pregnant women in the 1st trimester in order to prevent further transmission. Persistent oral thrush, lymphadenopathy n hepatosplenomegaly may be the presenting features of AIDS in infants
--)Children with a parentral h/o elevated total cholesterol level (>240mg/d), or risk factors for coronary artery disease should get a screening test for total cholesterol level.
--)Midgut Volvulus present in a child <1m with bilious vomiting, abdominal distension and n blood-stained stool.
--)Congenital Rubella Syndrome occurs most commonly following the transmission in the first 4wks of pregnancy. Classic triad of CRS”sensorineural deafness, cardiac malformation (PDA(MC), ASD), and cataract.
--)Iron Poisoning present with NVD, abd pain, GI bleeding n metabolic acidosis. As iron is radio-opaque, the tablets can be seen in the stomach on abd x-ray. Dx is confirmed by measuring serum iron level. Deferoxamine , an iron chelator, is used IV in mod-severe intoxication.
--)Gaucher™s diseases is dx based on radiological app of Erlenmeyer flask deformity of the distal femur, n charc Gaucher cells with their wrinkled paper appearance in BM examination.
--)MC mutation that leads to Cystic Fibrosis is a deletion of a three base pair encoding for phenylalanine (DA508) in the CFTR gene located on Ch 7, it is believed that this mutation prevent CFTR protein from trafficking to the correct cellular location----Meconium ileus, char by bilious vomiting and failure to pass meconium at birth, n groung glass apperanace on abd x-ray”diagnostic for Cystic Fibrosis”(AR)
--)Children with TOF will get ˜tet™ spells due to inc pulm vascular resistance leading to hyoxia. Crying, infection, and exercise can lead to ˜tet™ spell. There may be paroxysm of rapid n deep breathing. If the tet spells are severe, the infant may have seizures and loss of conciousness. Tx is O2 n placing a child in aknee-chest position, fluids, morphine n propranolol. Classical presentation of TOF child is squatting, which inc the systemic peripheral resistance n thus dec the Rt-Lt shunt n hence improves Cyanosis
--)Severe dehydration in a neonate in the presence of Hyponatremia, Hyperkalemia, Hypoglycemia and metabolic acidosis s/o Dx of CAH(Congenital adrenal hyperplasia). Classic salt wasting 21-hydroxylase def is inherited as an AR trait. Of particular note is the fact that male infants affected by this ds will not present with ambiguous genitalia at birth unlike female infants.
--)An epiglottis rolling in from side to side is the diagnostic finding of laryngomalacia, n all such babies should be fed in the upright postion n hold upright for half an hr after feeding, self limiting, subsides by 18m. The noisy breathing(stridor) charcterstically worsens when the child cries n while lying in supine position.
--) Mild cases of œMetatarsus adductus do not need any Tx n correct spontaneous.----Interanl tibial torsion is also a physiological finding in NB n spont resolution occurs in more than 95% of the children. Next step is reassurance .
--)Prevention of SIDS, infant should be placed on their backs while sleeping. Home monitor not dec the risk of SIDS.
--)ITP(Ideopathic Thrombocytopenia), children develop thrombocytopenis after viral infection, self limiting n spontaneous recovery occurs in the majority. Corticosteroids r the DOC in all age gps, when platelet count is <30,000 and/or symptoms r severe. Pt with a platelet count of >30,000 usually ve few symptoms n do not require Tx.
--)McCune-Albright syndrome is charc by (3P™s) Precocious puberty, Pigmentation(café-au-lai spot) and Polyostotic fibrous dysplasia(multiple bone defect). There is autonous stimulation of œaromatase enzyme production of estrogen by the ovaries It is responsible for 5% of the cases of female precocious puberty n may be ass with other endocrine abnormality, i-e hyperthyroidism, prolactin n GH-secreting pituitary adenomas n adrenal hypercortisolism. Mx is administration of aromatase enzyme inhibitor. Unlike VonRecklinghausen syndrome, the café-eu-lait spots r large , have irregular borders n r not ass with axillary and genital freckles. It is due to defect in the G-protein cAMP-kinase function in the affected tissue”gives autonomous activity
--)Kartegener™s syndrome(AR), is ass. with recurrent sinusitis, bronchiectasis, n dextrocardia. This is the result of primary ciliary dyskinesia.
--)Young chills who present with recurrent RTI and bilateral nasal polyps, Cystic Fibrosis must be r/o.
--)Rubella is char. by low-grade fever, lymphadenopathy (sub-occipital n post auricular) and rash (maculopapular).
--)Aplastic anemia(aquired) suspected in any pt with pancytopenia following drug intake, exposure to toxins or viral infections.
--)Guillain-Barre syndrome consists of demyelinization of the peripheral nerves. It may occur about 10days after a nonspecific GI and/ or RTI (campylobacter). O/E symmetric flaccid paralysis of both legs(ascending paralysis), absent deep tendon reflex and dec superficial touch n vibratory sense, no nuchal rigidity.Nerve conduction velocities r slowed indicating demyelinization. CSF shows a significant inc of protein levels n mildly inc cells(albumino-cytologic dissociation). Tx Plasmapheresis or human immunoglobulin.
--)Peroxisomal disorder such as Zellweger™s syndrome should be considered in N/N seizures, esp if the N/N present with typical facial dysmorphism (high forhead, up slanting palpebral fissures, hypoplastic supra-orbital ridges, epicanthal folds n redundant skin fold of the neck), severe weakness n hypotonia, N/N seizures, wide-open sutures, cloudy cornea, cataract or glaucoma, rarely live more than few months.
--)Three MC organism causes Pneumonia in Cystic Fibrosis pt are; 1-Hemophilus, 2-Pseudomonas, 3-Staphyloco.
--)The MCC of Amblyopia(dec in visual acuity) is strabismus (deviation of eye mostly inward), other causes include error of refraction n opacity of media along the visual axis. Dx by cover test. The standered of Tx is occlusion of normal eye-(HY)
--)Unilateral flank mass in a child <3yrs is highly suspicious of Neurobalstoma, a malignancy of the neural crest cells (displaces the kidney inferolaterally making the kidneys non-palpbale), whereas in a child >3yrs old, a unilateral flank mass is highly suspicious of Wilm™s tumor, originate from the metanephros (kidneys are palpable).
--)Asthma when it cannot be controlled by medication, should be an indication for mechanical ventilation---Racemic epinephrine may help a little in mod asthma, but when the condition is severe, its administration just delay the initiation of MV, Racemic epinephrine has a better role in Croup.
--)Leukocyte adhesion defect char. by recurrent bacterial infection, delayed separation of the umbilical cord >3wks, n necrotic periodontal infection. ( leukocyte # is inc, but the inter-leukocyte communication is defective. N lymphocyte n gamma globulin help to differentiate)
--)Cerebral anoxia is the MCC of Cerebral palsy charc. by impaired motor fxn,(hypotonia, inc deep tendon reflexes, n learning disability)
--)Infection by resp syncytial virus (Bronchiolitis) charc. by first episode of wheezing ass with URTI in wintor weather n affects 50% of children in 1st 2yrs of life---WBC is normal n chest x-ray shows air trapping n atelectasis Tx is supportive care with Humidified O2 n bronchodilators. There is an Increased risk of Asthma in later life
--)Kawasaki™s Disease is char. by fever, conjunctival vascular injection, cracked lips, polymorphous erythenatous rash, diffuse erythma of palm and soles and unilateral lymphadenitis, next step is echocardiogram to r/o coronary aneurysm. Tx is aspirin(due to anti-platelet n anti-inflammatory action) n IV Immunoglobulin. This is one of the condition in which aspirin is recommended, Influenza vaccine is recommended in pt with KD taking life-long aspirin.
--)Upper GI endoscopy is the diagnostic study of choice when a pt present with Acute Alkali ingestion(like oven cleaner, char by drooling with blood tinged secretion, lips n chin r swollen n erythematous, cloths r contaminated with material)
--)Reye™s syndrome is a rare illness seen in children <15yrs old treated with salicylates for a viral infection(C/I in children for viral inf), char by vomiting, agitation, lethargy, stupor n convulsion. Lab findings r Inc ammonia, N or slightly inc bilirubin and AlkPO4, inc prothrombin time, dec glucose, inc AST, ALT n lactate dehydrogenas. Tx is supportive.
--)Red eye in a NB(<24hrs) is due to Chemical irritation after silver nitrate prophylaxis(given to all NB at birth to prevent Gonococcal conjunctivitis), resolve generally in 24hrs---Gonococcal Conjunctivitis is the most destructive, present as hyperacute n highly purulent that appears 2-5days after birth, may results in corneal perforation n permanent loss of vision if left untreated. It is Tx with Tropical Erythromycin.----Chlamydia trachomatis causes the most infectious conjunctivitis in NB n appears even after prophylactic drops, it is milder than gonococcal n begins few days to several wks sfter birth, presents with congestion n a discharge, may be scant n mucoid or frankly purulent, Tx with Systemic Erythromycin b/c of risk of Pneumonia.
--)A Leukocoria (+ve white reflex), is a Retinoblastoma until proven otherwise, prompt ophthalmologist referral is required.---Retinoblastoma is the MC intraocular tumor of childhood, highly malignant (due to inactivation of Rb suppressor gene), if untreated leads invariably death due to liver n brain metastases. Other features include strabismus, dec vision, ocular inflammation, eye pain, glaucoma n orbital cellulites. Dx by clinical, n presence of calcification on US or CT scan.--HY
--)Growing pain, occurs at night only n no obvious physical or laboratory finding, Reassurance is the TOC.
--)Craniopharngiomas, derived from epithelial ramnant of Rathke™s pouch, not a true pituitary tumor, char by nests of squamous cells in a loose stroma, resembling the app of embryonic tooth bud enamel. Location is supraseller n inferior to optic chiasma. They cause Bi-temporal Hemianopia by pressing optic chiasma, also give s/s of Diabetes Insipidous. Dx”head X-ray shows calcified lesion above the sella. Next step will be MRI n may need Craniotomy.
--)N/N Tetanus, seen in infants born to unimmunized mothers, frequently followed umbilical stump infection, char by poor sucking n fatigue, followed by rigidity, spasm n opisthotonus in 1st 2wks of life.
--)In children<9yrs old, amoxicillin, for 21days is the recommended TOC for Erythma Migrans or Stage I Lyme Ds.
--)Cephalohematoma is a subperiosteal hemorrhage, present few hrs after birth as scalp swelling limited to one cranial bone, no discoloration, usually resorbed spontaneously within 2wks-3m depending upon the size. Rarely, Phototherapy required to improve the Hyperbilirubinemia.----Caput succedaneum is a diffuse, sometimes ecchymotic, swelling of the scalp, usually involves the portion of the head presenting during vertex delivery, may extend across the mid-line and across the suture-line.
--)Vit A has been shown to dec morbidity n mortality through immune enhancement. It also helps GI resp epithelium to regenerate
--)Iron supplementation in the form of iron fortified formula or medicinal iron, should be started at 6wks in all Premature and LBW babies.
--)Vaginal discharge(bleeding/secretion), due to effects of maternal estrogen, reassurance of the mother is required.
--)Fragile X syndrome, char by low to N IQ with learning disability, gen language disability, short attention span, autism, large head, prominent jaw, large low set ears n macro-orchidism(enlarge testes). 2nd MC of MR next to DS
--)Cri-du-chat (cry of the cat)syndrome (Deletion of 5p(MC), char by hypotonia, short stature, cat like cry, microcephaly with protruding metopic suture, moonlike face, hypertelorism, bilateral epicanthal fold, high arched palate, wide n falt nasal bridge n MR.
--)A 12 m old child walks alone, speaks 2words, throw objects n comes when called----Language development, socal smile “2m, Babbles--6m, 2words, obeys 1 step command---1yr, 2-3 word phrase, obeys 2 step commands---2yrs-----Gross Motor Development; Holds head---3m, Roll back to front n front to back---4m, Sits well unsupported”6m, walks alone”12m, Walks up n down stairs without help---24m------Fine Motor Development; Raking grasp”6m, Throw object---12m, Builds tower of 2 blocks”15m, Builds tower of 6 blocks/turn book pages”24m, ------Social Development; Recognize parents---2m, Recognize strangers(stranger anxiety)----6m, Imitates action/comes when called---12m, Plays with other children”18m, Parallel play---24m.
--)Supracondylar Fractures, MC # of peds, due to extension injuries, brachial artery may be compromised n radial artery pulse must be assessed when the # is reduced.-----The axillary artery is injured during Proximal humeral #.
--)Edward™s syndrome(Trisomy 18),char by microcephaly, prominent occiput, microganthia, closed fists with index finger overlapping the 3rd digit n the 5th digit overlapping the 4th, rocker bottom feet n MR. 95% die in the 1styr of life.
--)Epiglottitis seen in 3-7yrs old children, appears very toxic, ve stridor, high fever, drooling, tachypnea, n tachycardia. Lateral CXR shows,1---swollen epiglottis(thumb sign),2--thickened aryepiglottic folds, 3--obliteration of the vallecula( not wait for x-ray result). When child has stridor n dec O2 saturation, sudden airway obstruction may occur n intubation may be difficult, so preparation made for intubation n tracheostomy.
--)Mastitis is not a C/I to breast feeding, as it may actually be alleviate by continued n frequent nursing on the affected breast, which keeps it from becoming engorged. Additional measures, such as local heat application n antibiotic, can also be used.---Maternal HIV infection is an absolute C/I to breast feeding
--)Replacement of diarreal loss of fluid with lots of low solute fluid like water can lead to water intoxication, hyponatremia n even seizures.
--)Infant botulism, transmitted by ingestion of food contaminated with C-botulism(honey). The germ grows in GI n produce toxin (protease that block the acetylecholine). c/f ptosis, dilated pupil with sluggish light reflex,intact deep tendon reflexes, dec muscle tone, constipation, difficulty with breast feeding etc. Tx supportive
--)Pt with Sx of short attention span, impulsivity n hyperactivity, poor concentration for >6m in more than one setting(like school n home), persistently present since age 7, ADHD should be considered as the MC Dx.
--)Scarlet fever, char by fever, toxicity, pharyngitis, n sandpaper-like rash. Circumoral pallor n strawberry tongue also present, Dx +ve streptococcal throat test n normal appearing lips. TOC is 10days course of Penicillin V; however in Penicillin resistant pt , erythromycin/clindamycin can be used. Also Tx with penicillin does not prevent from Post-streptococcal GN.
--)In Mx of Enuresis, urinalysis should always be the 1st step, then behavioral modification (pass urine right b4 going to bed, no drinks b4 going to bed, wake up the pt in the night to pass urine), n finally meds (Imipramine n Desmopressin) r the DOC in refractory cases.
--)Diamond-Blackfan Anemia(DBA), Macrocytic pure red aplasia ass. with several congenital anomalies including short stature, webbed neck, cleft lip, shielded chest n triphalangial thumbs. On electrophoresis , fetal Hb is elevated n chromosomal studies r N. Tx is mainly corticosteroid n for unresponsive pt, Transfusion therapy is indicated.
--) Fanconi™s Anemia, (AR) char by progressive Pancytopenia n Macrocytosis, age of Dx is 8yrs, ass. Deformities include café-au-lait spots, microcephaly, microphthalmia, short stature, horseshoe kidney n absent thumb(not Triphalangial thumb)
--) Tx of Recurrent Aortic Coarctation is Balloon Angioplasty.
--)Triad of Henoch-Schonlein Purpura(HSP) or Anaphylactoid purpura, 1)-Petechial rash, 2)-Arthritis, 3)-Abd/renel complications, ass. with Bacterial(strept, mycoplasmal), Viral(adenovirus, varicella), Drugs(penicillin, erythromycin), n Vaccination ( Measles, typhoid, yellow fever). HSP causes intestinal intussusception, usually ileo-ileal. Barium enema may be “ve, Plain abd x-ray shows findings of intestinal obstruction, U/S shows the classical œDoughnut or target sign with hypoechogenic, edematous bowl, surrounding the central more echogenic, invaginated bowl (representing the intussusception). Tx is surgical reduction as they r located more proximally compared to the ileo-colonic intussusceptions n r not reducible by barium or air .
--)TCA(tricyclic antidepressant) Intoxication char by seizure, hypotension, dilated pupils, n QRS complex prolongation, After initial resuscitation, NaHCO3 is the DOC in pt experiencing a seizure or QRS complex prologation (correct acidosis n narrows the QRS complex)
--)Minimal change disease is the MCC of Nephrotic syndrome in pt younger than 16yrs old. Light microscopy n immunofluorescence shows normal findings. Electron microscopy shows diffuse effacement of foot processes of podocytes. Steroid r the TOC for 4-6wks, over 90% children demonstrate complete remission with disappearance of proteinuria.
--)Rotavirus is the 1st MCC of acute diarrhea in children, esp b/w 6m-2yrs. Norwalk virus is the 2nd MCC of non bacterial, acute diarrhea in children.
--)The growth of axillary hair results from androgen secretion by adrenal gland(adrenarche). Premature adrenarche or isolated appearance of axillary hair n thelarche b4 the age of 6yrs, is generally benign n has no clinical significance; however premature pubarche, or growth of pubic hair b4 th age of 8yrs, is more alarming, as it is ass. In 50% of cases with CNS disorder.
--)Perinaud™s syndrome consist of paralysis of vertical gaze, which may be ass with papillary disturbances n eyelid retraction(collier™s sign). It results from lesion of the rostral midbrain at the level of the superior colliculus n CNIII. It is most commonly caused by Germinomas n Pinealomas at this region. The endocrine syndrome(dark facial hair, deepening of voice n increasing ht at early age(10yrs) results from interruption of hypothalamic inhibiting pathways, n sometimes beta HCG secretion n consequent Leydig™s cell stimulation.
--)Vit A deficiency, MC in 2-3yrs old child present with dry eyes n skin, photophobia, n impaired dark adaptation, results in night blindness. Later, drying of conjunctiva(xerosis conjunctivae), n of cornea(xerosis cornea) followed by wrinkling n cloudiness of cornea (keratomalacia)are seen. Bitot spot(silver-gray plaque may appear on the bulbar conjunctiva
--)Marfan™s syndrome(MFS) (AD), resulting from the mutation of the fibrillin-1(FBN1) gene, present with tall stature. Long n amaciated extremities, aracnodactyly, hypermobility of the joints, upward lens dislocation and aortic root diltation.
--)Racemic epinephrine decrease the need for intubation in pt with croup(Laryngeotracheobrnochitis) n should always be tried b4 any invasive procedure.
--)Dermatitis Herpetiformis and chronic non-bloody diarrhea in a child of 12-15m, s/o Celiac disease(gluten sensitive enteropathy), when gluten containing food such as wheat, rye or barley are introduced into the child™s diet
--)Hyaline Memb Ds(HMD), seen in preterm infants(60-80% <28wks) with resp distress soon after birth and hypoxia not responding to Oxygen therapy,CXR shows fine reticular granularity of parenchyma. Tx early with mechanical ventilatation and surfactant
--) In a pt with severe bouts of coughing n subcutaneous emphysema, a pneumothorax must be r/o by CXR
--)Vit D def Rickets is more common in dark skinned infants who r less exposed to sunlight n r 1-2 yrs of age. It presents with craniotabes, rachitic rosary, n thickening of the lower end of long bones, due to defective demineralization
--)Wilm™s tumor diagnosed b/w of 2 and 5yrs, MC primary renal neoplasm in children, in 10% of cases is bilateral. In 80% of cases, an asymptomatic abd mass is present. Other may present with HTN, abd pain and vomiting, in few it may present with lung mets. It is ass with Beckwith-wiedeman and Denys-drash syndromes.Tx is nephrectomy.”If child age is <1yr think of neuroblastoma.
--)Guillain-barre syndrome consist of demyelinization of the peripheral nerves. It may occur abt 10days after a nonspecific GI and/or respiratory infection.TX is Plasmapheresis or human immunoglobulin
--)Stranger Anxiety start anywhere b/w 6-8m ,and peaks at 12-15m. It is the normal anxiety experienced by infants on exposure to individuals unfamiliar to them.
--)Nursemaid™s elbow (subluxed radial head) is one of the MC elbow injuries I young children b/w 1-5yrs. It occurs when infan or child are lifted or pulled by the hand or arm results in radial head slipping through parts of annular ligament resulting in injury. No significant swelling or angular deformity should be visible. The hand typically held in pronated position with refusal and pain with attempted forearm supination. Rotating the hand and forearm to a supinated position with pressure over the distal head usually reduces the annular ligament with a click and restores full, normal use of extremity---Diagnostic
--)Cystic fibrosis, presents with failure to thrive, occurs due to fat and protein metabolism from a pancreatic insufficiency.
--)Croup(laryngotrachietis/laryngotraceobronchitis) is caused by Parainfluenza virus commonly present in children < 3ys with hoarseness, a barking cough, and varying degree of resp distress, Dx clinically, Lateral neck x-ray shows sub-glottic narrowing.
--)Spondylolisthesis is a development disorder char by a forward slip of vertebrae (usually L5-S1)that usually manifest in predolescent children. C/F back pain, neurological dysfunction (e.g urinary incontinence), and a palpable œstep-off at the lumbosacral area present if the disease is severe.
--)Duchenne muscular dystrophy char by proximal muscle weakness and exhibits a Gower™s sign with depressed reflexes and pseudohypertrophy, obesity, MR and cardiomyopathy. Dx by muscle biopsy n family pedigree, nowadays, the most appropriate way to confirm the dx of DMD is genetic (DNA)studies. Tx supportive and most pt are wheel chair bound by 12yrs of age.
--)ALL is the MC leukemia in children. 1st Sx is non-specific(lethargy, anorexia n irritability). o/e pallor, hepatosplenomegaly n/or lymphadenopathy.Dx by anemia, thrombocytopenia and blast cell on bld smear, confirmed by bone marrow biopsy(>25% blast cell)
--)Myotonic muscular dystrophy(MMD) is an AD disorder and is 2nd MC muscular dystrophy in North America, involves striated as well as smooth n cardiac muscle, ass with multiple endocrinopathies, immunologic deficiencies, cataract, neurological abnormalities, intellectual impairement and dysmorphic facies. Pt r usually normal at birth, Sx begins in childhood and include weakness and progressive muscle wasting esp in the distal muscle of the hands, post forearm muscle and anterior compartment of the lower leg. Prox muscles are subsequently affected leading to difficulty in walking n climbing stairs, a +ve Gower™s sign and winging of the scapulae. Myotonia char by delayed relaxation of the affected muscles after contraction(inability of the pt to release the handshake). Ass endocrine abnormalities includes testicular atrophy, DM, frontal baldness n hypothyroidism. CNS ---Normal
--)Rheumatic fever char by chorea, fever, pericarditis(friction rub), arthritis, erythma marginatum , s/c nodules, Inc ESR, prolonged PR interval due to Gp A strep. Dx by +ve ASO titre, c/s and antigen test. Tx Penicillin
--)Friedreich ataxia (AR), MC type of spinocerebeller ataxias, Sx begins <22yrs n include gait atxia, frequent falling n dysarthria due to degeneration of spinal tract(spinocerebeller tract, post column, pyramidal tract). Also ass with Conc hypertrophic cardiomyopathy(MC 90%), DM n sk deformaities(scoliosis, hammer toes). Median survival rate <20yrs after Dx. MCC of death in these pt are cardiomyopathy n resp comp.(HY).
--)Phenyleketonuria(AR) due to def in phenylalanine hydroxyalse (enzyme that breakdown phenylalanine into tyrosine) causing accumulation of phenylalanine and its metabolic product in the blood n bldy tissue(brain). If untreated can cause MR n seizures ( mousy odor in urine). MC screening test is blood phenylalanine level. Guthrie test, qualitative (coloration) test, detect the presence of metabolic product of phenylalanine(PA) in the urine. Tx low PA diet(little amounts r necessary for growth n development). Cereals, starches, fruits, vegetables n PA free milk formula should be recommended n high protein foods avoided .
--)Lesch-Nyhan syndrome(seen in male) is sec to def in HRPT(Hypoxanthine-guanine phophoribosyl transferase), an enzyme involved in purine metabolism, results in inc level of uric acid which accumulate in peripheral tissue. It presents with self-mutation n neurological features including MR, dystonia, choreoathetosis n spasticity. Uric acid causes gouty arthritis, tophus formation n obstructive nephropathy. Tx Allopurinol n inc fluid intake .
--)Mongolian spot MC in dark skinned population, present from birth n usually fades n then disappears in several years caused by entrapment of melanocytes in the dermis during their migration from neural crest into epidermis in fetal development. No Tx.
--)Severe combined immune deficiency(SCID), a life threatening syndrome presents with recurrent bacterial, viral and fungal infection. Dx by absence of L.nodes n tonsils, lymphopenia n absence of thymic shadow on CXR.
--)Group B streptococcus is the MCC of n/n sepsis. Early sepsis present within the 1st wk of life n usually involves several organs. Late-onset GBS infection is usually a focal infection, most frequently meningitis
--)Benign/Innocent Mumurs; most cardiac murmurs in childhood r benign n require only observation. Features of benign murmurs are;---1-Asymptomatic pt, 2-Murmur intensity grade 2or< , 3-Normal S2, 4-No audible clicks, 5-Normal puses, 6-No other abnormality.
--)Neurofibromatosis: an AD ds ass with multisystem manifestations. 6or> coffee color macules measures ~0.7mm on upper trunk with s/c nodules on extremeties, also there are nodular swelling protruding from irises most likely Hamatomas. Pt are at inc risk of developing Gliomas, presents with pale optic discs n dec visual acuity. Tx not specific, regular bld monitoring and annual CNS and opthalmological exam for early signs of optic gliomas n other CNS tumors.----Tuberous sclerosis---char by the development of renal angiomyolipomas
--)Clavicle Fracture(shoulder dystocia) are common comp in mothers with GDM and large babies with prolonged labour, dx by asymmetric Moro reflex n crepitus over the clavicle., does not need Tx-----Erb-duchenne™s paralysis is another complication in large infants n difficult labor, presents with the arm in adduction n pronation, n wrist n fingers in flexion c/a wiater™s tip position due to paralysis of C5 n C6 nerve roots of the brachial plexus, sec to traction on the infant™s head during delivery.
--)Infratentorial tumors r more common than supratentorial tumors, n benign astromas r the MC histological type in both gp (CNS tumors r the MC solid tumors in children, n the 2nd MC malignancies in the pediatric population, after leukemias-----Medulloblastoma is the 2nd MC tumor of post fossa in children. ~90% of cases arises from vermis.-----Glioblastoma multiforme is the highly malignant astrocytic tumor more common in adults----Pinealoma develops in the dorsal aspect of the midbrain. Sx consist of endocrine syndrome, intracranial HTN, Perinaud™s sign(paralysis of vertical gaze)and Collier sign(retraction of the eyelid)-----Craniopharyngiomas arise in the sella turcica, n Sx usually include visual field defects n endocrine syndrome, char by a cystic structure with presence of calcification.
--)Foreign body aspiration, presents as sudden onset of resp distress in an otherwise normal child. CXR shows finding of hyperinflation of the Rt lung with the mediastinal shift to the Lt n a severely hypoinflated Lt lung. Such pt requires immediate direct laryngoscopy n rigid bronchoscopy;both diagnostic n therapeutic purposes
--)Intussusception, the MCC of intestinal obstruction in the 1st two yrs of life, presents with intestinal obstruction n red current jelly stools containing blood n mucus, mostly idiopathic, but in some cases, it may be sec to intestinal mass i-e polyp, lymphoma, hamartoma or Meckel™s diverticulum. MC type is iliocolic.
--)Physiological jaundice usually presents after 24hrs of birth. Any jaundice in the 1st 24hrs of birth, serum bilirubin rising at a rate > 5mg/d/24hrs, serum bilirubin >12mg/d in full-term(in the absence of risk factor) or 10-14mg/d in preterm infants, jaundice persist after 10-14 days of life(DOL) or direct hyperbilirubinemia,>2mg/d needs detailed evaluation for the cause of jaundice------Bacteral sepsis/UTI----Jaundice appears 3rd-7th DOL.--------Breast milk jaundice---appears after 1st wk of life.Cessation of breast feeding for 1-2 days n substitution for formula results in a rapid decline in serum bilirubin, after which nursing can be resumed.----Biliary Atresia, presents any time since birth with conjugated hyperbilirubinemia ass with acholic stool n dark urine------Erythroblastosis fetalis, resents at birth or within the 1st 24hrs of life(direct/indirect). Hemolysis s/o by rapid rise of serum bilirubin(>0.5mg/d/hr), anemia, pallor, reticulocytosis, n hepatosplenomegaly
--)Conduct disorder, char by disruptive behavioral patter that violate basic social norm presents for >1yr, in pt <18yrs old.-------Antisocial personality disorder”pt are > 18yrs old.
--)Congenital cataract is the MC cause of Leukocoria (not retinoblastoma). Causes of cataract include familial, congenital infections, metabolic conditions(e.g DM, Galactosemia), genetic disorders (e.g Down™s syndrome, Turner™s syndrome), n long term/high dose steroid use
--)Transient synovitis of the hip char by an inflammation n swelling of the tissue around the hip joint, cause is unknown may be due to virus or sec to an allergic reaction to an infection somewhere else in the body. Bed rest with affected joint in the position of comfort is the TOC, Pt usually gets better in 3-4 days.
--)Trachoma, caused by Chlamydia trachomatis is the MCC of blindness in the world. Dx presence of lymphoid follicles on the conjunctiva, scarring n limbal follicles. Tx erythromycin or tetracycline.
--)Esophageal atresia presents with chocking, regurgitation n coughing ass with the 1st feeding of the NB infant. The inability to pass a feeding tube into the stomach s/o n U/S confirm the diagnosis.
--)Meckel™s diverticulum presents with painless melena in 2-3 yrs old children, present in 2-3% of population n MC anomaly of the GIT. Heterotropic gastric tissue may be present in the diverticulum, results in ulceration n bleeding.
--)Osgood-Schlatter disease (age 10-17yrs), presents with knee pain at the tibial tuberosity (at the site of insertion of the quadriceps tendon)
--)N/N tetnus, seen in infants born to immunized mothers, frequently following umbilical stump infection, n is char by poor suckling and fatigue, followed by rigidity, spasm n opisthotonus in 1st 2wks of life
--)Chronic Granulomatous dz”caused by a defect of phagocytes cells with dysfunction of the NADPH oxidase enzyme complex, leading to recurrent and uncontrolled infections with catalase-positive organism. MC clinical findings are lymphadenopathy, hypergammaglobulinemia, hepatomegaly, splenomegaly, anemia of ch dz, under wt, ch diarrhea, short stature, gingivitis n dermatitis. Dx by Nitroblue tetrazolium (NBT) slide test, flow cytometry or cytochrome C reduction. Tx “-Trimethoprim-sulfamethoxazole/day and gamma-interferon 3times/wk, BM transplantation is experimental but curative.
--)WAS(Wiskott-aldrich syndrome)caused by defective gene encoding for WAS protein, presents with eczema, microcytic thrombocytopenia n recurrent infection with encapsulated germs in young boy, present at birth n consist of petechiae, bruises, bleeding from circumcision or bldy stools. Dx by low IgM, and high IgA n IgE n moderately dec Tcell n platelets.
--)Chediak-Higashi syndrome char by dec degranulation, chemotaxis n granulopoisis, multisystem dz char by mild coagulopathy, peripheral n cranial neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, freq bacterial infections(S aureus), n progressive lymphoproliferative syndrome. Dx neutropenia n giant lysosomes in neutrophil. Tx trimethoprim-sulphamethaxazole n Ascorbic acid/ daily
--)Hyper-IgE(job™s) syndrome char by ch pruiritic dermatitis, recurrent staph inf(skin, resp tract n joints), markedly elevated IgE, eosinophilia n coarse facial features, dental abnormality n bone # r diagnostic.
--)Whooping cough caused by Bordetella pertussis, highly contagious infection, presents with severe bouts of coughing rectal prolapse, epistaxis n pneumothoraces, may last from 2-10wks.ïƒ spells can Tx is erythromycin for 14days, regardless of the age or the immunization status, n regardless of the stage of the dz, Prevention”all close contacts should be given erythromycin for 14 days, regardless of age, immunization, or Sx.
--)The introsseous technique is an excellent site for IV access in children
--)Systemic form of Juvenile RA(Still™s dz), char by fever, fleeting maculaopapular rashes, hepatosplenomegaly, lymphadenopathy, pleuropericarditis n myocarditis. RF rarely +ve. Tx NSAID therapy with monitoring of Liver enzyme. In refractory cases steroids r used.
--)Absence seizure is a generalized seizure of childhood n is char by sudden n brief lapses of conciousness without loss of postural control n absent postictal confusion, usually present with multiple œdaydreaming episodes n decline in school performance.
--)Postexposure prophylaxis for chicken pox is provided with VZIG (varicella Zoster mmune Globulin) or Acyclovir. VZIC is preferred n indicated in susceptible high risk pt exposed to varicella within 96hrs (preferably 72hrs) of exposure.
--)Pubertal gynecomastia, seen in ~1/2 of adolescent boys, at an average age of 14yrs, often asymmetric or transiently unilateral, frequently tender, regresses within 6-18m n just need observation”HY”
--)Tuberous sclerosis, presents with infantile spasm, often during 1st yr of life with cluster of brief symmetrical contraction of the neck, trunk n extremeties with unique pattern on EEG c/a Hypsarrhthmia (chaotic, high voltage, bilateral, asynchronous, slow wave activity), hypopigmented lesions(ASH leaf spots) n cortical tubers on CT head r charcterstics. Tx for infantile spasm is ACTH i/m.
--)Klumpke™s paralysis is a brachial palsy that occurs in NB following excessive traction on the arm, consists of hand paralysis n ipsilateral Horner™s syndrome (ptosis n miosis), is sec to injury to 7th n 8th cervical nerves n 1st thoracic nerve
--)Hyaline membrane dz(HMD), occurs in premature infants 60-80% <28wks of GA.-, caused by dec perfused but non-ventilatedïƒ atelectasisïƒ production n secretion of Surfactant hypoxia n resp distress not responding to O2 therapy, Tx early withïƒ alveoli Mechanical ventilation n Surfactant
--)Fulminant meningococcemia occurs after meningococcus inf, ~10-20% infants will presents with vasomotor collapse, large petechiae n purpuric lesion due to Adrenal Heamorrhage”caries 100% mortality(Waterhouse-Friederchsen syndrome).
--)Friedreich Ataxia an AR disorder(genetic counceling is recommended for prenatal dx for parents with one affected child) char by an excessive number of trinucleotide repeat sequences, resulting in an abnormality of a tocopherol transfer protein, ass with necrosis n degeneration of cardiac fibers leading to myocarditis, myocardial fibrosis n cardiomyopathy. Cardiac arrhythmia n CHF r the MCC of death. Most pt r wheelchair bound by the age of 25, with death occurring by 30-35yyrs of age MRI shows marked atrophy of the cervical spinal cord n minimal cerebellar atrophy. Dec vibratory n position sense in LL n ankle jerk r absent bilaterally
--)Congenital Hypothyroidism; In USA MCC is thyroid dysgenesis
--) Ch peylonephritis char by focal parenchymal scarring n blunting of calcies on IVP
--)Sicle cell Trait”most commonly presents as painless gross hematuria.----Pt with splenectomy or functional asplenia (SCD pt have at the age of 2-3yrs repeated microinfarction due to clumping of SCs) r at risk of infection with capsulated org like Pneumococcus n H. influenza. MCC of sepsis in SCD pt is Pneumococcus.
--)Homocystinuria (AR) = Marfan™s features + MR + Thromboembolitic events + downward dislocation of the lens, caused by cystathionine synthase def. DxInc both homocystine n methionine(metabolite) in body fluids. Tx high doses of Vit B6. Restriction of methionine along with supplementation of cysteine is used for refractive pt
--) Mast cell stabilizers(Na cromolyn) r the DOC for asthmatic pt who have evidence of other allergic disorders
--) Cong Toxoplasmosis”triad of chorioretinitis, hydrocephalus n intracranial calcification, acquired by exposure to feces from infected cats n ingestion of raw meat n unpasteurized goat™s milk. Risk of transmission during inc as the preg porogresses , but severity of the N/N dz dec
--)Suspected Diaphragmatic hernia, 1st step in the Mx is the immediate placement of orogastic tube (b4CXR) n connecting it to continuous suction, in order to prevent bowl distension n further lung compression. Endotracheal intubation n mechanical ventilation r also priorities for all infants with severe CDH who present in 1st hr of life
--)Measles(Rubeola) char by fever, non-productive cough, coryza, non-purulent conjunctivitis n koplik spots, pathognomonic bluish white lesions on buccal mucous memb, opposite to 1st n 2nd upper molars, n sometimes on inner conjunctivae n vaginal mucosa. Lab findings r Leucopenia n lymphopenia, proteinuria may be present, Dx is clinical
--)Tricuspid atresia”cyanotic CHD char by cyanosis early in the life n left axis deviation, single S2, most caese90% r ass with VSD ( char by holosystolic murmur) n 30% r ass with TGA. Tx infant should receive PGE1 to keep ductus open, n have balloon arterial septostomy if ASD is not large enough.
--)Choanal atresia, Cyanosis , aggravated by feeding n relieved by crying, MC nasal malformation. In children who r unable to breathe through the mouth, it is more severe n presents with cycles of cyanosis n relieved after the distressed infant start crying. Unilateral remains undiagnosed until the infant develops his 1st URTI. Dx by the failure to pass a catheter through the nose 3-4cm into the orophaynx. 1st step in Mx is placing an oral airway n lavage feeding. Dx is confirmed by CT scan shows narrowing at the level of pterygoid plate.ïƒ with I/nasal contrast Definitive Tx is repairing the obstruction with surgery or endoscopy
--)Displaced anterior fat pad is a radiographic sign of supracondylar fracture of Humerous, which may be complicated by Volkmann™s ischemic contracture. Tx immediate compartment release fasciotomy to prevent damage.
--) Meningitis may be complicated by regression of developmental milestones (inability to identify colors n to copy triangles n squares.
--) Meningococcus is the MCC of bacterial meningitis in pt b/w 2-18yrs. Pneumococcus is the 2nd MCC i
Tanner
Pubic hair (both male and female)
Tanner I -- no pubic hair at all [10 and under] (prepubertal state)
Tanner II -- small amount of long, downy hair with slight pigmentation at the base of the penis and scrotum (males) or on the labia majora [10-11](female)
Tanner III -- hair becomes more coarse and curly, and begins to extend laterally [12-14]
Tanner IV -- adult-like hair quality, extending across pubis but sparing medial thighs [13-15]
Tanner V -- hair extends to medial surface of the thighs [16+]
Genitals (male)
Tanner I -- prepubertal (testicular volume less than 1.5 ml; small penis of 3 cm or less)
Tanner II -- testicular volume between 1.6 and 6 ml; skin on scrotum thins, reddens and enlarges; penis length unchanged
Tanner III -- testicular volume between 6 and 12 ml; scrotum enlarges further; penis begins to lengthen to about 6 cm
Tanner IV -- testicular volume between 12 and 20 ml; scrotum enlarges further and darkens; penis increases in length to 10cm and circumference
Tanner V -- testicular volume greater than 20 ml; adult scrotum and penis of 15 cm in length
Breasts (female)
Tanner I -- no glandular tissue; areola follows the skin contours of the chest (prepubertal)
Tanner II -- breast bud forms, with small area of surrounding glandular tissue; areola begins to widen
Tanner III -- breast begins to become more elevated, and extends beyond the borders of the areola, which continues to widen but remains in contour with surrounding breast
Tanner IV -- increased breast size and elevation; areola and papilla form a secondary mound projecting from the contour of the surrounding breast
Tanner V -- breast reaches final adult size; areola returns to contour of the surrounding breast, with a projecting central papilla.
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