opngicy TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUN( eves: merge sow en Mg RY NECN CIN LDN, «DIAGNOSIS OF RECURRENT ‘RHEUMATIC FEVER (March 2 vena itn in the face of documented GAS infection: ‘cues Level of Evidence C) ‘¢ When minor manifestations alone are presen the exclusion inthe more likely causes ofthe clinical presentation Is Pacommended before a diagnosis of an ARF recurrence ‘an be made (Class I; Level of Bvidence C). « DIAGNOSIS OF POSSIBLE RHEUMATIC FEVER (March 2015) in Where there is genuine uncertainty iis reasonable to consider offering 12 months of secondary prophylaxis followed by re-evaluation to include a careful history and physical examination in adltion toa repeat Echocardiogram (Clas lla; Level of Evidence C) co Ina patient with recurrent symptoms (particularly involving the joints) who has been adherent to prophylaxis recommendations but lacks serological Epldence of group A streptococcal infection and lacks schocardiographic evidence of valvults itis reasonable to conclude that the recurrent symptoms are not likely related to ARF, and discontinuation of antibiotic prophylaxis may be appropriate (Clas la; Level of Evidence 0} pve diagnosis (Cass Ib; «+ Sedreumstances wherein diagnosis of ARF can be made without ‘stictadherence to Jones criteria 4. When chorea isthe only major manifestation 2, When indolent carditis i the only manifestation upon frst checkup, months after the apparent onset of ARF 3. Apparent ARF ina limited numberof patients with recurrences of ARF in particularly high-risk populations ‘of mild carditis disappear rapidly in weeks but those * Diaonastcs 0 CBC ESR/CRP 6 Throatculture 0 ASO titer 0 Chest X-ray 0 ECG © 2-Decho ‘Management Antibiotic = Onces a oe sof acute RF has been made and regardless throat culture results the patient should recelve 10 oral Penelli or Erythromycin o single IM 3m of benzathinePenillin to eradicate GAS from the upper resplratory tract + After this intial eourse of antibiotics, patient shouldbe started ‘on long-term antibote prophylaxis. ° to ‘eradicate Streptococcus to prevent 1* episode of RF * Penicillin VK- 200 - 500 mg QID x 10 days ‘= Benzathine PCN- 0.6 - 1.2 MUIM ® Erythromycin- 250mg TID x 10 days ° prevent recurrences of RF * Penicilin VK 250 mg BID * Benzathine PCN 0.6-1.2 M units q21 days IM in * ASA 100 mg/kg/day 4-6 doses (mild carditis) + Predaisone -2 mg/kg/day (severe carditis) Duration of prophylaxis for patients who have had acute RF (2009 AHA recommendation) ° ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOI ‘nial Course “4. Only carditis can cause permanent cardiac damage, Sey a ‘years or unt 21 years of age, sicher longer Tier aa [1 yn oan eT sahos ae yeart disease wir longer a sat ver hari and | 10 er rl yas oa arinibertdanes | wicorrnbngerometne Hitine (© Treatment of CHF includes some oral ofthe 1. Complete bed rest and oxygen 2, Morphine 0.2 mg/kg at 4-hr interval for severe CHF with respiratory distress 3. Restriction of sodium and fluid intake 4. Digoxin 5. Furosemide 1 mg/kg/dose every 6-12 hrs VALVULAR HEART DISEASE . ee inorigin + Mitral valve %; aortic valve % of cases «Tet valve lavivment irae ) : ona ene ano a ‘MITRAL STENOSIS ‘+ Most common valvular invol LO «+ Thickening ofeafets and ecommissures “calcification with immdlity ofthe valve results + LA and rightsidedhegg chairs become dilated ané hypertrophied + fit pulmonaryyetge hypertension > pulmonary congestion, oss of alveolar wall, hypertrophy of pulmonat es ross of ung compliance acu. oa fi] ghee = “~ p = 1 + Clinical Manifestations 0 Asymptomaticif mild 6 Dyspnea with or without exertion 6 Orthopnea, nocturnal dyspnea, palpitation in more severe cases 6 Increased RV impulse along LSB Weak; pulses with narrow pulse pressure 6 Loud $1 atthe apex & a narrowly splits2 with loud 2 © Opening snap is fellowed by a low-frequency mitra diastolic rumble atthe apex Diagnostics © CAR: A & RV enlarged; prominent MPA; Lung fields show pulmonary venous congestion & interstitial edema (Werley's B lines) © 2D-echo Management ‘0 Good dental hygiene & antibiotic prophylaxis against SBE © Closed mitral commissurotomy for those without calcification 6 Valve replacement ifvalves ae calcified (o Regular checkups for possible dysfunction of the replaced/repaired valve + Most common valvular involvement in children with RHD + Shortened leaflets due to flbrosis + Dilated LA & LV with dilated MV ring ‘0 Asymptomatic during childhood ‘0 Hyperdynamic apical impulse is palpable n severe MR 6 S1 isnormal or diminished; 2 may widely split; oud $3 © Hallmark: systolic regurgitant murmur, Grade I-1V/VI, atthe apex with transmission to the left axilla Shor, low-frequency diastolic rumble at the apex: UT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 50 of 127 For inquiries visit woww.topnotchboardprep.com.ph or https//www.facebook.com/topnotchmedicalboardprep/{EP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Hypocalcemia is common from 12-72 hrs of life especially in: ‘L premature infants 2. iminfants with asphyxia at birth 3 ininfants of diabetic mothers ‘© Muscular pain and cramps ~ early signs hy 1 Numbness, stiffness tingling ofthe hands & fet © (+)Chvostek or Trosseau sign or laryngea! &¢ahpopedal spasm © Conlon with os of consciousness? 6 Chron ate eth eruption regular i rman, soft teeth % = ree Pay - ° =a meal, Sa calcu 45% of the tata islow ¢ Serutipkefbras>712 may @ Lowserun PTH © Xray ofthe bones increased density limited to the metaphyses ‘Serum ealetum: ‘About 0% of calelum is fonized 40-45% is bound to albumin ‘Emergency trestmentor neonatal tetany 5-10 ml of 10% solton of caleum gluconate IV at arate of 5-1 mL/min while HRs rmonitored (© 1.25-dydronycholecalferol calcitriol) shouldbe given ~ inal dose 0.25 wg/day & MD 0.01-0.1ug/eg/éay ven in 2 equal divided doses + 5.1096 bound to other anion (sulfate, PO, lactate, crate) * Only ionized fraction is physiologically active & can be rapidly measured ICS HANDOUT BY RUBY PUNONGBAYAY ~ For Inquiries vist ww. topnotchboardprep.comphor ye Ie Aaebodh cotton oe is dor HYPERPARATHYROIDISM * Childhood occurrence stare me occurrence is rare * Usually due toa single benign adeno a ile benign adenoma manifested after 10 yrs * Some occur as part of multiple endocrine fr neoplasia (MEN) ‘drome characterized by hyperplasia or neoplasia ofthe Fone Me PaPeteas, the anterior plutary & parathyroid * Wows calcu regulated ithe bay? © PTH stimulates osteoclasts to resorb calcium from bone > Inet Ca reabirpton inte Dt 9 nce ction oft Dby the kidney decreas FP el of phosphate ras aes! © Vitamin D promotes calcium reabs ‘Gana ete sorption from bone and © Galeton nhibis xed acy 9 derese reabsorption of Ca from bone a ° ‘Muscular weakness, anorexia, hausea, vomit constipation, polydipsia, polyuria, loss. fever ‘© Progressively diminished renal functiofin clironic cases © Ossenu changes may pads ne za frei pittance Mnemonic for the symptoms ofhypg tec "Panlbones BON peat * Renal stones um + Abdominal groans Payee moans (cates in mental satus, concentration and mood) + Fatigue overtones: © Primary - most commonly ‘due to a PTH-producing u that snot responsive to normal “6 feedback regulation va an increased Ca concentration fx Secondary high PH production ue to decreased aleve! inc secretion of corticotropin > adrenocortical hyperplasia & overproduction of intermediary metabolites + Deficiency of i-hudraxvlase accounts for 90% of affected les on Seas eee fee ot ae ee we Gemeinde Pathogenesis ‘Lack of 21-hydroxylase “> excess substrates which are shunted towards synthesis of sex hormones -> decreased cortisol > loss of feedback inhlbition > increased ACTH > further stimulation of the conversion of cholesterol into sex ‘hormone precursors w/in 1st 6 mos of ie ‘+ Usually normal mental development ‘+ Sal testes & enlarged penis «Females: enlarged clitoris or labial fusion ‘= Symptoms begin shorty after birth «+ progressive weight os, prominent voting, dehydration «+ Females: mote pronounced vilization of external genktals Salelosing 1» Males: genitals appear normal © Low serum Na & Cl; in. K/low sérum cortisol 6 Increased plasma renin and serum aldosterone © 21-hydroxylase defidiency: increased serum 17-OHP o Pelvic ultrasound toajsualize presence of uterus in female pseudohermaphroditism * Management: © Glucocorticoids inhibit excessive production of androgens 4 aces progressive vriltzation © Hydrogbrtisone 10-20 mg/m2/day orally in 2-3 divided doses * Continued indefinitely n all patients with classe forms of CAH © Monitor growth & hormonal levels 0 Flurohydrocortisone (0.05-0.3 mg daily) & NaCl 1-3 gms ‘given to normalize plasma renin activity ‘ACTH INSUFFICIENCY ADDISON'S DISEASE ADDISON'S DISEASE___ * Deficient production of cortisalor aldasterone due to ‘congenital or acquired lesions ofthe hypothalamus, pituitary gland or adrenal cortex. + Etiology: congenital hypoplasia or aplasia of the pituitary {abnormalities of skull & brain, craniopharyngioma), adrenal hypoplasia congenita, inborn defects of steroidogenesis, autoimmune destruction of the glands, CNS demyelination, etc For inquiries visit www.topnotchboardprep.com.ph or https://www. facebook. com/topnotchmedicalboardprep/ Clinical Manifestations: ‘0 Gradual, muscular weakness, lassitude, anorexia, weight Toss, general wasting, hypotension, intense craving for salt © Increased skin pigmentation on face & hands, most intense ‘around the genitals, umbilicus, axilla, nipples, joints o Fale of santan to dsappes maybe he rst clue © Low serum Na & Cl increased K f° Inc, urinary excretion of Na & Cl ‘0 hypoglycemia © Most definitive test: measurement of plasina or serum level of cortisol before & after administration of ACTH Management: ‘0 DS 0.9 NSS IV to correct hypoglycemia & the Na loss ‘o Hydrocortisone succinate IV (25 mg for infants & 75 mg for children) every 6 hrs forthe 1824 hrs © After 48 hr; may discontinue uids& shift oral cortisol in 5-20 mg 8 hrs, CUSHING SYNDROME ‘Characteristic pattern of dbesity with assoclated hypertensi ‘which ithe Fesult of abnormally high blood levels of cortisol ‘esultig frdm hyperfunction ofthe adrenal cortex + Btilogys functioning adrenocortical tumor (nants); pituitary idenomas; Kyperplasia of adrenals Rounded face, prominent cheeks, moon facies, buffalo hump, generalized obesity, abnormal masculinization, impaired ‘hypertension, inc. susceptibility to Infection ‘0 Older children: purplish striae on hips, abdomen & thighs, , emotional lability, weakness, headache + Diaanostics © Serum cortisol levels are normally elevated at @ am & decrease to <50% by 8pm > diurnal rhythm i lost ‘© Urinary excretion of free cortisol & 17- hydronycorticosterods are increased © CTand MAL 0 Single-dose dexamethasone suppression test = Dexamethasone 25-30 mcg/kg (max: 2 mg) given at ‘ip then determine plasma cortisol levels tam. + Normal: <5 meg/dl. Cushing syndrome if>5 megdL « 2-step dexamethasone suppression tet to determine whether it is caused by a/an: + Pituitary adenoma + Ectopic ACTH-secreting tumor + Comtisl-secreting adrenal tumor + Management ‘© Unilateral adrenalectomy for benign cortical adenomas 6 Bilateral tumors: subtotal adrenalectomy © Transsphenoidal pituitary microsurgery for children 0 Adequate preoperative & postoperative replacement therapy with corticostroi 6 Substantial catch-up growth; abnormal bone density TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDER‘ For inquiries visit www.topnotchboardprep.com.ph or tbs/ ww facebook comfpnotchmedlcalbosrdoren! eeeforos TOPNOTCH MEDICAL BOARD PREP PED| IATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD For i oF Inquiries visit wwrw.topnotchboardorep.com.ph or https://www book.com/topnotchmedicalboardorep/ | Adrenal Medulla | + Catecholamines: dopamine, norepinephrine, epinephrine KLINEFELTER SYNDROME * Sybesgecarnthebrinspatecneneenung ain |” EE RTOmonome- stconmon se cranial + Metabolite are excreted in the urine: MA, metanephrine, | * 2u®tOmelticnondisjunction ofan X chromosome during normetanephrine | _ parental gametogenesis; the extra X chromosome is maternal in + Batheprnorepnephrneraechememnararalan, |, SUBnASAN Apatenalin de ofp increase PVR-->inc.systolic & diastolic BP * Cinta Manifestations ‘© Dx rarely made before puberty due to paucity of s/sx in childhood PHEOCHROMOCYTOMA Catecholamine-secreting tumor arising from the chromaffin cel Most common site of origin is the adrenal medulla ‘Tumors may develop anywhere along the abdominal ‘sympathetic chain, likely to be located near the aorta at the level of the IMA or at its bifurcation, * Other locations: Periadrenal area, urinary bladder, ureteral walls, thoracic cavity, cervical region Occur in children 6-14 yrs old ‘+ Tumors found more often on the right side, about 1-10 cm in diameter, but bilateral in >20% affected children Inherited as AD trait + May be associated with other syndromes such as neurofibromatosis, part of MEN syndromes, tuberous sclerosis, Sturge-Weber syndrome, ataxia-telangiectasia Biosynthesis: Pantin = —Tymine tenance —— Dir henane gain Les uibendopumioe Nonbetaneivine Matanesane | \ / mortise Satonengony mace * Clinical Manifestations: ‘0 S/Sy result from excessive secretion “mn norepinephrine o May be symptom-free in between attacks stp © Headache, palpitations, abdominal pain, didz{ntss,;° pallor, vomiting, sweating, convulsions.» 6 Severe: precordial pain radiate into the arms, phmonary edema,cardio- & hepatomegaly.” * Diagnostics By, © Dre demonstration of elevated blo o unary levels of catecholamines & their metat co Predominant catecholamige thghi}ireh is norepinephrine rived from te adrent apes adrenergicnerve endings t. 6 Urinary excretion ofyapillylmandeltc actd (major metabolite of epimiprepi- & metanephrine) is increased 6 Ultrasound, Chséan, MRI: tumors ‘0 Surgicakremoval of tumors ° 3- & B-adrenergic blockers © ThorougMtransabdominal exploration of al the usual sites © Accurate indicators of malignancy ~ presence of metastatic disease or local invasiveness that precluded complete resection or both NOONAN SYNDROME > Boys & girls have normal karyotypes * Occurs in %,000 live births * Autosomal dominant + Short stature, webbing of the neck, pectus carinatum/excavatum, cubitus valgus, right-sided CHD, hypertelorism, downward slanted palpebral fissures, ‘micrognathia, moderate MR in 25%, SNHL; delayed puberty, cxyptorchidism «+ Human growth hormone has been used, © Considered in all boys with MR & in children with psychosocial, learning, or school adjustment problems 6 Tall slim, underweight, long legs, small testes & penis, ‘gynecomastia, azoospermia, associated with leukemia & Iymphoma (15-30 yrs old) )* oes ester 25-50 mg IM every 3-4 wks with S0-mg sctenfents every 6-9 mos until a maintenance dose for “Bales is achieved (200-250 mg every 3-4 wks) ‘TURNER SYNDROME, ~7" 45X chromosomal complement * Unknown mechanism of chromosome loss + Risk does not increase with maternal age + Recognizable at birth: edema of the dorsa of the hands & feet, loose skin folds at the nape, LBW, decreased length, webbed neck. low posterior hairline, small mandible, prominent ‘ears, eplcanthal folds, high arched palate, widely spaced nipples, hyperconvex fingernails, delayed sexual ‘maturation © Short stature - cardinal finding in all girls © Nonstenotic bicuspid aortic valves, horseshoe kidney, {idiopathic hypertension, [BD 6 Ultrasound of the heart, kidneys & ovaries is indicated after, the dx is established ‘© Most common skeletal abnormalities: shortening of the 4 ‘metatarsal & metacarpal bones, epiphyseal dysgenesis in the joints of the knees & elbows ‘© Recombinant GH increases height velocity & ultimate stature in most but not al children (starting dose 0.375 mg/kg/wk) 6 Replacement therapy with estrogens is indicated (litte ‘consensus re; initiation) © Premarin (conjugated estrogen) 0.3-0.625 mg given daily for 3-6 mos ~ effective in inducing puberty TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN, MD & COCOY CALDERON,MD Page 87 of 127 6 er leguer tmeentistes, produce cytokines--> induce destruction of islet cells «+ Type! absolute insulin deficiency «Type it insulin resistance and increased insulin levels ‘Both types can result in retinopathy, nephropathy, and ‘neuropathy. + ets oftnsuln define 'o With progressive deficiency > excessive glucose production ‘impairment ofits utilization > hyperglycemia w/ sBlucosuria > resultant osmotic diuresis produces polyuria, urinary losses of electrolytes, dehydration, polydipsia > Iypersecretion of epinephrine, glucagon, cortisol & GH ‘which amplifies & perpetuates metabolic derangements & ‘accelerates metabolic decompensation ie ‘© Combination of inulin deficiency & inc. counterregulatory. hormones is responsible for accelerated ipalysi impaired ipd synthesis > inc plasma total lipids, cholesterol, 1G, FFA> ketone body formatign Wc : exceeds the capacity for peripheral utizat Mei excretion > metabolic acidosis & rapid 4é@Rprthing te’, ipsa plyphaglwitas (fen © Classic: polyuria, poly inaless than a month) © Clue to polyuria: onset of enur trained child ‘9 Pyogenicskin infections &'mohiial females 5 resdina revo ale aS. | vaginitis in adolescent + Pigonostc Cater: © Symptoms of diabetes lus a ‘L random plasm flucose >200 mg/dl, of; 2 fasting pve glucose >126 mg/d © Polyuria, poyilipsia, & unexplained weight loss with luca & ketonuria Reliable index of long-term glycemic control ~ measure glycosylated Hgb ‘0 Glycohemoglobin (HbA1c) represents the fraction of Hgb ‘to which glucose has been nonenzymatically attached in the bloodstream = Reflects the average blood glucose concentration of the receding 2-3 months + Glycated High predicts risk of progression of diabetic complications * The more consistently lower the level the better the metabolic contro, the more likly itis that ‘microvascular complications will be less severe, delayed in appearance, or avolded. « Inknown diabetics = 7% indicates good diabetic control + 10% indicates fair diabetic control = 13-20% indicates poor diabetic control (MY & LULU LALUERYTYM wf (DOUT BY KUBY PUNUNUDATAN, For inquiries visit www.topnotchboardprep.com.ph or htt ps: mw facebook.com/topnotchmedicalboardprep/ DIABETIC KETOACIDOSIS. * Glucose >300 mg/dl and glucosuria + Ketonemfa and ketonuria + Acidosis (pH «7.3 & HCO3 <15 mEq/L), « Precipitating factors: trauma, infections, disturbances vomiting psychologic Classification of DKA: eens | Mad | Meat | Soe af ee | eo | os [ae | asvas | aszas [estas [as * ‘ic | Necarge | Ovened | “Kinmnal | Hea thet | rong | depress fated | cred | pions ty | dered omile | sean . et 2The latter has: © Blood glucose >800 mg/dl. 6 absence of or ony slight ketosis ‘nonketotic acidosis severe dehydration ay 0 depressed sensorium or coma; yagous,néurologic signs ‘Smeal © Pre-existing neurologic dag me 23 Ne © Ketoacdosis:expansog of jtravascular volume, correction of deficit influ, elettlyte & acid-base staus; initiation of insulin the «Initial hydrating Mud is isotonic saline (hypotonic relative to the patent’ séhum osmolality) Administtation Of glucose (5% solution in 0.2 N saline) is _, nifated When blood glucose approaches 300 mg/dl to athe decline of serum osmolality & reduce cerebral ema, ivepotassium added after the intial 20 ml/kg UO is equate. *y bicarbonate only i pH <7.2 given slowly ~o Anticipate cerebral edema limitrate of fuldto4 L/m2/day or less co Insulin 0.1 U/kg of regular insulin followed by constant Infusion of 0.1 U/kg/br ‘Muteitional Management: ‘© CHO 55%, fat 30%, CHON 159% © 70% of CHO content shouldbe derived from complex CHO & intake of surose'& highly refined sugars should be limited © Polyunsaturated:saturated fat ratio 1.2:1 Total dally caloric intake divided to provide 20% at breakfast, 20% atlunch, 30% at dinner with 10% for each ofthe midmorning, midafternoon & evening snacks SOMOGYI EFFECT VS. DAWN PHENOMENON. + Somogy! Effect ‘© Pattern of undetected hypoglycemia followed by hyperglycemia ‘© When does it occur? * ‘Typically in the middle of the night o Whatis the cause? ‘A result of insulin or diabetes meds working too strongly atthe wrong time © During periods of hypoglycemia, the body releases hormones > chain reaction to release stored glucose D glucose level swings too high > hyperglycemia © How can you test forts presence? * Wake up between 2-3 am and test your blood glucose > iflow > signifies Somogyi effects in action ‘© How do you correct it? * Have a snack with protein before bedtime lke toast with peanut buter or yogurt and a small piece of cheese + Dawn phenomenon: © Body's response to hormones released Inthe early ‘morning hours > when we seep, hormones ae released tohelp maintain and restore cells w/in our bodies > ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN, Toren ot win tanctbardre.comah or Mis wen, fcockcotapnctemeseaterioaay feJone TTOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD For inquiries visit wwew.topnotchboardprep,com,ph or https:/www facebook, com/topnotchmedicalboardorep/ counterregulatory hormones (growth hormone, cortisol, catecholamines) cause the glucose level to rise © For diabetics who do not have enough circulating insulin to ‘keep the increased glucose under control > hyperglycemia © How to Treat Dawn Phenomenon: + Bxercise later inthe day > has a glucose-lowering effect nthe night * Consult your doctor for possible medication adjustments, * Limit bedtime carbohydrates and try more ofa proteln snack, ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN MD & COCOY CALDERON,MD Page 89 of 127 Fear ennnctele wasn tannatrhhasednran ram nh ar ine: /hwunu farahank com/tannatchmedicalboardor1: ‘4 Most children “outgrow” milk and egg allergies with about 50% doing so within 3-5 years. « About 80-90% of children with peanut or seafood allergy retain ‘thelr allergy fr life. + Buiology: egg, milk, peanuts, fish soy, wheat account for 90% ‘of food allergies during childhood + Class 1 food allergens: penetrates the Gl barrier «© Class 2 food allergens: penetrates the respiratory tract + all milkallerges develop by 12 months of age and all egg allergies by 18 months of age. + Glinical Monifescacions: GIT, skin, respiratory tact ‘© Management Elimination dlet for 14 days followed by food challenge. ADVERSE REACTIONS TO DRUGS ‘ORGANS OF THE IMMUNE SYSTEM The Reticuloendothelial System 1. The Lymphoid Organs: 1. Bone marrow 2. Thymus 3. Lymph Nodes 4. Spleen IIL, Other Peripheral Lymphoid Tissues ‘THE RETICULOENDOTHBLIAL SYSTEM. © Mesenchymal celisand Ussues distributed throughout 80d}, « Either fixed or wandering and able to engulf particulate «bin ookne seating fnctn x 1 ee ep and + cele 2 Digerentiation of CellLineages: gu Lymphocytes = f ilooytes oe, er - : < Extiooytes Megakiryocites -=PMATURECELLS ~ LYMPHOID ORGANS * Bone Marrow © Growth-promoting cytokines push precursor cells to “iferentite (also called colony-stimulatng factors lke 3) + Thymus ° saturation of precursor T-lymphocytes ‘& induction of immunocompetence co Treels first detected in the thymus on the 74-84 week of “gestation & undergoes physiologic involution at 7 maturation & selection of T lymphocytes puberey ‘continue into adult life + Lymph Nodes ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANI icy: TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN MD & COCOY CALDERON,MD “= For inquiries visit www.topnotchboardorep.com.ph or https://www.facebook,com/topnotchmedicalboardprep/ (9 Small encapsulated nodular aggregates of ymphold ‘dssues with anatomical groupings in the neck, axila, femoral and popliteal areas 6 Antigens are delivered via the Iymphaticstolymph nodes ‘where specificimmune responses are generated © Both T-cells and B-cells are found here 6 Anatomy & sie vary with te state of lymphocyte activation * Spleen fo Vital for immune responses to blood-borne antigens © Acts as a waste disposal system © Lymphocytes occupy the periarterioler sheath where 2/3 are CD4+ T-cells and 1/3 are CD8+ T-cells ‘© Becells occupy the follicular and germinal centers © Asplenic patients susceptible to infection by encapsulated bacteria OTHER PERIPHERAL LYMPHOID TISSUES ages & sal Mucosal immune system ‘A function of the aggregates of lymphocyte other accessory cells located beneath epithelium © Mucosa associated lymphoid + Peyer's patches in Lymphoid fllic upper ai .& effector cells performing as killer & gulatory cells & Ig-producing cells id secrete cytokines which mediate cell growth lifferentiation jor classes: T-cells (70%) & B-cells (30%) ~ ofd subsets: 2Toells Stem cells ature in thymus-> develop cell membrane proteins called cluster diferentiation (CD) proteins ~> used toidentify diferent subsets of Tells + CD cells are helper/Inducer cells + CD8+ are suppressor/cytotaxic cells (kill microorganisms by direct action on thelr surfaces) « Subpopalation of lymphocytes identified as large granular ‘mphocytes that comprise 5% of peripheral blood Iymphocytes «- Nonphagocytic and perform antibody dependent cell cytotoxicity «+ Bind and trigger release ofcytolysin (a protein that creates pores on cell membranes of target cells resulting in lysis) + Principally involved in ysis of tumors & virus infected target cells «First line of defense against neonatal herpes virus infection 4.Phagocytes: ‘+ Two important cell types: polymorphonuclears & ‘mononuclear phagocytes that arise from the BM «Tetales 14 days fora PMN to mature from a myeloblast;t1/2 in bloodstream 6 hrs t1/2n tssues is 1-2 days + MNPs (monocytes and tissue macrophages) circulate 1-4 days (DOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 111 of 127 For inquiries visit www.topnotchboardprep.com.ph of https://www, facebook. com/topnotchmedicalboardprep/1 * Clinical disorders of neutrophil function: © Degranulation abnormality * Chediak-Higashi syndrome - disordered coalescence of lysosomal granules (failure of phagolysosomal fusion) -> decreased neutrophil ‘chemotaxis; neutropenia; recurrent pyogenic infections; hepatosplenomegaly. Treatment: anitibiocics ‘Adhesion abnormality * Leukocyte adhesion deficiency - neutrophilia; ‘mutations in integrins or defective adhesion proteins (LFA-1) proteins on the phagocyte surface > recurrent bacterial infection associated with alack of pus formation; delayed separation of ‘umbilical cord. Treatment: Antibiotics, BMT Denressed motile response * Hyper-igE syndrome ~ autosomal dominant and the responsible gene is ‘Stat3 + lmpaired chemotaxis and regulation of cytokine production + recurrent skin and sinopulmonary infections, ‘eczema, mucocutaneous candidiasis, eosinophilia, retained primary teeth, scolosis, characteristic facies * Chronic Granulomatous Disease + Failure of oxidative burst = Normal B- and T-cell activity + Recurrent infections with catalase-positive bacteria and fungi; widespread granulomas of unknown etiology ‘Treatment: antibiotic chemoprophylaxis ‘S.Eosinophils: * Contains granules with major basic proteins that are toxic for helminths * IL¢S activates and influences its production ‘+ Major cationic proteins mediate late-phase allergic reaction ‘S.Mast cells & Basophils: * Effectors of immediate hypersensitivity since they carry Pe receptors for IgE 2 + Inallergic individuals, ther is an Increased numberof allergen specific igé molecules bound to these receptors. HUMORAL ELEMENTS Ig or antibodies are produced by cells (secretory Igs) + Found in germinal centers ofr rd splenic follicles, Peyer's patches, tonsils, a md colostrum, saliva, land urinary tracts, * Comprise 20% ofthe totakplasm proteins fice igs) & plasma 1. immunoglobulins * 1gM: Largest; 10% of normal serum igs; earliest Ab in responge to an Ag + IgG: Most abundant; only ig that can traverse the placental barrier * gh: Synthesized inthe submucosa of the respiratory & GIT & ‘excretory glands; does not fix complement or cross the placenta rredominantly found on the human B lymphocytes like IgM; differentiation of B-cells ‘+ IgE: Less than 10% in the serum; role in parasitic infection & allergic reaction 2. Complement ‘+ Main function: mediates inflammatory processes, facilitates ingestion by opsonizing pathogens for phagocytosis, mediates cell ysis & stimulates B- & T-cell responses + 2 pathways of activation: classical & alternative * Classical components: Cig, Ctr, Cis, C2, C3, C4 + Alternative components: properdin, C3, factor B, factor D Pns2ish FOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON MD Inquiries visit www. topnotchboardprep.com.ph or https://www. facebook, com/topnotchmedicalboardprep/ ‘+ Common to both pathways: C5, C6, C7, C8, C9 (precursors of the Membrane Attack Complex) 3. Qrtokines + Growth regulating factors for hematopoletic cells; Immunomodulators * S distinct families: interleukins, colony stimulating factors, Interferons, chemokines, growth factors © Interferon - antiviral © Chemokines - chemoattractant & growth regulating © Growth factors control tissue function 4. Accessory molecules * Also called phenotypic markers as they identify and differentiate between cell populations © Potential use: monitors graft reection, SLE, and RA, © Cell surface proteins that identity a particular cell lineage that have a defined structure * Human leukocyte antigens (HLA) or malotbistocompanibility complex (MHC) © HLA-A,B, C, DR, DQ DP © Basis of tissue compatibility ig reciplent-donor screening . ‘0 HLA matching predicts short? and long-term graft survival © Associated with suscepetbility to certain diseases IMMUNOPATHOLOGIC REACTIONS Type - immediate hypersensitivity ‘+ Typell - cytotoxic reaction ‘= Type Itt - immune complex-mediated reaction ‘+ Type IV ~ delayed type hypersensitivity t Pu tothe lease of metre hsanie eae. ‘oni far scdnton by Merges ot epi ‘as usp wich are pose ens by + secon re rgns raccoon, Inened motel sereton + Examples: asthma, urticaria, allergic rhinitis, anaphylaxis Type: ‘Ab bind to Ag and the immune complex formed activates ‘complement ->membrane attack complex (MAC) is formed > celllysis + Bxamp 1. Hemolytic disease 2. Erythroblastosis fetalis 3 Graft rejection 4. Goodpasture's syndrome 5. Blood transfusion reaction 6.ITP Type Ml: ‘Ab reaction with Ag ~> immune complex ts formed > complement activation > tissue Inflammation release lysosomal enzymes > tissue destruction + Examples: Post-infectious glomerulonephritis 2, Serum sickness 3. Rheumatoid arthritis 4. Systemic lupus erythematosus 5. Stevens-Johnson syndrome 6. Polyartertis nodosa Type WV: + Cell infiltration with accumulation of CD4+ & CD8+ cells & ‘macrophage activation -> release of cytokines -> stimulate fibroblast proliferation & collagen production > fibrosis + Examples: 1, PPD test 2, Contact allergic dermatitis 3. Syphilis 4. Leprosy ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 112 of 127 For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardpren/Nice TOPNOTCH MEDICAL BOARD PREP PEDIATRICS Hi NEONATAL SEPSIS ‘Early onset - birth to 78 day of life © Late onset - 8 to 28% day of life 1 Very late onset ->28 days ofife ‘9 Maternal infection during pregnancy (© Prolonged rupture of membranes (18 hours) © Prematurity -most important neonatal factor predisposing to infection © Bacteria: GBS (most common), & coll (most common in the local setting), and Listeria monocytogenes co Viruses: HSV, enteroviruses «Clinical Manifestations: Nonspecific (temperature instability, ‘hypotension, poor perfusion with pallor and motled skinmetabolic acidosis, tachyearda or bradycardia, resplratory distress Iitabiity, lethargy, slaures, feeding Intolerance, abdominal distention, jaundice, petechae, purpura, and bleeding) + Diagnastcs: © CBC ‘= Neutropenia may be a better marker for neonatal ‘sepsis; has better specificity than leukocytosis + /T (immature to total neutrophil) ratio has the best sensitivity of any ofthe neutrophil indices. ‘9 Chest X-ray (if with respiratory manifestations) © Blood culture ~required for all neonates with suspected sepsis 6 Urine cultures - not part of routine workup as UT in ‘neonates are attributed to seeding of kidneys during bacteremia Lumbar tap for CSF studies (for late-onset sepsis) for ‘neonates with (+) blood culture, suspected sepsis, or with nonresponse to antibiotics + Treatment ‘0 Empiric antibiotics * Early-onset: Ampicillin + aminoglycoside or 3 gen cephalosporin * Late-onset: Vancomycin + aminoglycoside or 3r¢gen cephalosporin i © Supportive care ‘CTRANSPLACENTAL INFECTIONS. 7 ‘TORCHINFECTIONS 7 “Toxoplasmosis, Others (Varicella Parvovirus B19, Syphilis, others), Rubella, CMY, Herpes simplex arom f feed raw mea handing a eee © Triad of congenital toxoplabmosis: chorioretinitis, Intracranial (cerebralfcaleifications, hydrocephalus o Hepatitis, IUGR," «© Treatment: pyrimethamine & sulfonamide Rubia alt ffeanental retardation, blueberry mul rash 6 Cataracts - most serious eye finding, seen in athird of infants © Congenital heart disease - seen in half of infants Infected during the first 8 weeks of gestation. Most common defects patent ductus arteriosus 0 Deafness (sensorineural) - single most common finding ‘among infants with congenital rubella syndrome 0 Neurologic abnormalities are common. ‘* No specific treatment available. ‘© Prevent with maternal rubella vaccination prior to pregnancy ‘© Most common congenital infection © Triad: chorioretinitis, microcephaly, periventricular calcifications © Hepatosplenomegaly, petechlal rashes, jaundice |ANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD * For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ * Diganostics: ‘© Direct hyperbilirubinemia, elevation of hepatic transaminases © Thrombocytopenia and anemia © Cranial UTZ + Teatment: Ganciclovir Herpes Simplex Virus rom passage through an infected birth canal + Local vs disseminated disease (worse prognosis). There are 3 patterns of disease among infants: 1. Disease localized to the skin, eyes, or mouth itis with or without skin, eyes, and mouth 3. Disseminated infection involving multiple organs + Clinical Manifestations: © Skin vesicles or scarring © Chorloretinitis or keratoconjunctivitis © Microcephaly or hydranencephaly ' 6 ltritability, lethargy, poor feeding, poortene,and seizures (ifencephaltis pattern) co Temperature instability ritabilty, pookfeeding, ‘vomiting, respiratory distress jatpdlce, purpuric rash (ifdisseminated pattern) » + Treatment: Acyclovir, deliver by C-section if with active lesions Syphilis “Early stage (<2 yrs of life}~ analogous to secondary stage of acquired syphilis co Maculopapuiar ras! by desquamation ‘0 Snuffles (persistent rhinitis), mucous patchesand ‘condylomatous lesions are highly characteristic features of mucus membrane Involvement, © Osteochondritis and pertostitis of long bones, with irritability and refusal to move the involved extremity aralysis of Parrot), and Wimberger lines h, or vesiculobullous lesions followed a (pseudop: ss.” (metaphyseal demineralization ofthe medial aspect of oh the proximal tibia) © Hepatosplenomegaly, jaundice, and elevated liver enzymes o Failure to thrive, chorioretinitis, nephritis, and nephretic syndrome Late stage (> 2 yrs): ‘© Chronic granulomatous inflam: CNS co Hutchinson teeth; saddle nose co Hypersensitivity phenomena: interstitial keratitis, Clutton joints, choroiditis, retinitis, optic atrophy = Treatment: Penicillin imation of bone, teeth, and 1UBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 12 of 127 TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY Rt For inauiries visit www. topnotchboardprep.com,ph or https://www.facebook,com/topnotchmedicalboardprep/+ Glink © Paralysis usually follows a nonspecific vital infection (GIT or RT) by 10 days (Campylobacter JeJun{ and herpesvicus) ° (Landry ascending paralysis) ‘© Cranial nerve deficits leading to dysphagia, dysarthria, {factal weakness, papliledema, autonomic dysfunction, respiratory muscle paralysis © Miller-Fisher syndrome - acute ophthalmoplegia, ataxia, areflexia (© Benign clinical course with spontaneous recovery within 2- 3 wks © Tendon reflexes usually the last function to recover & lower extremity weakness last to resolve * Prognosis: 3 clinical features are predictive of poor outcome with sequela: cranial nerve involvement, need for intubation & ‘maximum disability at the time of presentation + Diagnostics © CSF analysis: increased protein, normal glucose, no pleocytosis 6 Dissociation hetween high CSF proteln and alack of sellular response is diagnostic (albuminocwtologic dissociation) ‘o Reduced motor NCVs + Management Acute stage: admitfor observation bec. ascending paralysis may ocur within 24 hrs «Rapidly progressive ascending paralysis: 1V1G (04 s/t ay for 5 consecutive days) « Supportive care; prevention of ulcers 6 High-dose pulse methyiprednisolone V fr relapses ENCEPHALOPATHY 7 Generalized disorder of cerebral function that may be acute or chronle, progressive or static —EEEEEEeeeeEe—Ee——eEE a) + Ebiloge: 2 toxic 3. metabolic 4 genetic ng 5. ischemic fy ‘CEREBRAL PALSY 5 7 Group of pemanent disorders of movementond posture ausing activity limitation that are i Sonprogressvedctarbances nthe ev@oping fetal or infant brain a «Motor disorders ae often accompanied bydisturbancs of ‘sensation, perception, cogniti®y, cofmmunication, and behavior as wel as by eplepsy and secondary musculoskeletal problems ™ . + Boidemioloavof Ce: =. 0 Incidence of 36,000 with M:F 14:1 0 In 80% of mnatal factors identified ‘o Less than 10% had intrapartum asphyxia ° Those 'WOG had intracerebral hemorthage and peritiricularlevkomalaca . i st fjcongenital anomalies external tothe CNS > atrisk for developing asphyxia during the perinatal period 2 Intrauterine exposure to maternal infections 3. Low birth welghtesp.<1000 gatbirth + Gasification: © Physiologic - identifies the major motor abnormality 4. Spastic 2. Athetoid (15%) 3. Rigid ‘A Atazie 5.Tremor 6 Atonie 7. Mixed 8. Uncassined ving nae le tra eka See eg eee ee ee ee ee Joo TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD ‘ot inquiries visit www topnotchboardprep.com.ph or https//www.facebook,com/topnotchmedicalboardprep/ ‘0 Topographic indicates the involved extremities 1 Monoplegia 2. Paraplegia 3.Hemiplegia 4. Triplegia 5. Quadriplegia 6.Diplegia 7, Double hemiplegia * Glnical Manifestations: o Spastic hemiplegia (2594) = Arms > legs + Decreased spontaneous movement on the affected side * Shows hand preference ata very early age + Delayed walking or walks on tptoes + Spasticity apparent esp. in the ankles * Seizures & cognitive impairment * Bilateral spasticity of he legs * Damage tothe immature white matte, * Commando craw! increased DTRs, +) Babinski sign, ad ankle clonus Normal intellect, ‘* ° uadriple Spastic auadriplegla (209%) * Most severe form of CP dug tOmgared motor impairment ofall crag hh Povodatlon with MR & seizures + Swallowing dificutis =” + Hypotanle poo" head control and marked head lag 1 UE LE afeged wit dystonia (gid muscles ‘hrogou thelr range of motion and ivountary contractions can occur) + Feeding difficulties, tongue thrust, drooling + Absent or slurred speech -Jnellectis preserved; setzures ae uncommon an iso be caused by kernicterus -Didanostics ‘o Baseline EG & cranial CT scan <9 Hearing & visual function tests se uliistpinary approach inthe assessment & treatment 6 Fortight hel cord: tenotomy of Achiles tendon (Drugs for spasticity + Diazepam - oral; 0.5-0.75 mg/dose BID or QID. + Baclofen ~0.2-2 mg/kg/day BID or TID + Dantrolene ~05-10/kg/day BID + Levodopa ~ for dystonia; 05-2 mg/kg/day + Reserpine or Tetrabenzine - for hyperkinetic movements + Botulinum toxin BRAIN TUMORS 7 del most prevalent malignancy in childhood; most common so! tumors «2/3 ofall intracranial tumors occur bet. 212 yrs old - infratentorial (posterior fsss) «+ Bvoluton of brain tumors involve sequential mutation or iéeletion of genes + lncal Manifestations: 102 distinct patterns of presentation: symptoms & signs of increased ICP and fal neurologic signs «Alteration in personality (lethargic irtabe, deteriorating school performance) oo Head sting & nuchal igidty may indicate herniation of ‘cerebellar tonsils « Nystagmus & ataxia prominent in posterior fossa tumors tid «+ Infratentorial or supratentorial tumors (hs year-old boy sbrougtto is docor by his mother for follow-up | appointment Two montis ago, the patient was seen fora chef complaint of morning beadache, vomiting and decreased activity. At this vis, the mater reports that her son continues to have worsened symptoms as well | SSnew onetfaligand estunbllng galt. | What is your most lkely diagnosis? ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 92 of 127 For inquiries visit www.topnotchboardorep.com.oh or https: //www. facebook. com/t notchmedicalboardprep/1 “Amother brings he I rings her £2 monih-ld bay to the pedarican because Fe a had watery area almost dl forthe pat month Previa god eat, | {he aby iow fing fed and irate mot a he ine We olng the baby, she noted a mass nbisbelly that has ot resolved in | Ince ly that has not resolved | Whats your impress NOICH irieoa ae BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD ‘wwew.topnotchboardprep.com.ph or https:/www facebook, com/topnatchmedicalboardprep/ MUSCULOSKELETAL TUMORS ‘RHABDOMYOSARCOMA «A tumor of striated muscle, most common sof tissue sarcoma in children + Head & neck 40%, extremities 18-20%, GUT 20%, trunk 10% + Histologic types: © Embryonal - most common, Intermediate prognosis © Undifferentiated © Alveolar - worst prognosis © Botryotd- vaginal grape-ke mass (sarcoma botryoides) © Pleomorphic~ adult 1 | —! * Embryonal tumor of neural crest cell origin + 3rd most common pediatric cancer + 8% of childhood malignancies * Neuroblasts (pluripotent stem cells) nvaginate and migrate along the neuraxis where neural crest cells are present - sympathetic ganglia, adrenal glands * Differential diagnosis of small, round, blue-cell tumors: © Wilms tumor © Acute leukemia © Rhabdomyosarcoma ‘© Mesothelioma / Medulloblastoma © Ewing's sarcoma © Retinoblastoma © Primitive neuroectodermal tumor (PNET) © Neuroblastoma ‘** W-A-R-M-E-R-P-N (warmer in the Phillpplnes) ‘© Median age at diagnosis is 2 yrs old & 90% of cases are diagnosed by 5 yrs old ‘+ Associated with: (0 N-myc oncogene - tuberous sclerosis © Neurofibromatosis - pheochromocytoma © Hirschprung's disease * Clinical Manifestavions: Reflect the tumor site & extent of disease; non-tender abdominal mass which may cross the midline, Horner syndrome, hypertension, cord compression (from a paraspinal tumor) ‘0 Metastases: periorbital bruising ("raccoon eyes"), (© Subcutaneous tumor nodules, opsoclonus /myoclonus (dancing eyes, dancing feet’) © Most common sites of metastasis: ong bones & skull, BM, be liver, lymph nodes, skin %) lymph nodes, sk abe a” * Glinical Manifestations depend on Toeatonf cs: ‘© Nasopharyma~ congestion, epistaxis, © Face or cheek paralysis swelifiapain $ Trunk or extromitles-may della hematoma 8 Genitourinary tract ~hematinadbstructin, sarcoma botryoides 4 '* Diagnostics: Cranial & ne¢k'CT’Seain, neck & chest x-rays, ‘abdominal UTZ & CT Séagstystourethrogram + Treaument: surgicaengisign, chemoTx, radiation yr patients, disseminated disease + Differentials: Abdominal CT scan (caeifcation & hemoretidegf 24-hr urine VMA & HVA (elevated in 95% of eases)’ «Highest risk period for development is during adolescent x ‘growth spurt ‘BUN/Crea, CXR, bone scan, LFTs, CBC + International Neuroblastoma Staging System (NSS, > (0 Prognostic factors: age at dlagnosis, open due, ‘Shimada histology ap ei + Unknown cause «= RiskJactor: hereditary retinoblastoma + Glinical Manifestations: ‘TOPNOTCH MEDICAL B For inauiries visit www.toonotchboardprep.com.ph or 6 St 1: tumors confined tothe organ orstractuteof origin o Se tumors extend beyond the rate ‘origin but not across the midline with (28) opythou (2A) Ipsilateral lymph node involvement =, ‘0 St3: extend beyond the migifire ipmph node velvengate o sed diareminatd to stn tes (bone, BM, iver, distant rhgrw/oblatera lymph nodes) . ‘9 StS: <1 yrold y/ijgemination to liver, skin, or BM w/o bone involveifens & with a primary tumor Prognosis ‘0 With located flsease: regardless of age, 80-90% S-yr ‘supgtvgl rate younger age at diagnosis carries a more favOFable prognosis, co Syrsurvival rate: 0 83% for infants (0 55-60% for 1-5 yrs old (0 40% for children >5 yrs old ° ‘Treatment (0 Lowrisk St 1 & 2: surgery © Observation for St 4S (nearly 100% survival) 0 St2: cure rateis>90% © Local recurrence: chemotc and radiation © If with spinal cord compression at Dx: chemotx surgery, or radiation 6 Intermediate risk: chemotx (cisplatin, cyclophosphamide, etoposide & doxorubicin), surgery, or radiation 0 5¢3.& 4 surgery + chemotherapy; 90% survival fo High-risk: chemotx, stem cell transplantation, surgery, radiation © Paln, limp, swelling: most common ‘© Any bone or joint pain not responding to conservative therapy must be investigated thoroughly Limitation of motion, joint effusion, tenderness and warmth * Diagnostics ° highly malignant, pleomorphic, spindle cell neoplasm associated with the formation of malignant osteoid and bone Alkaline phosphatase and LDH may be high © Suspected in a patient who presents with deep bone pan soften causing nighttime awakening 2 palpable mass, ‘and an x ray demonstrating the lesion «9 Lesion may be blastic or lytic In appearance but new bone formation is visible co Classic x-ray finding: sunburst pattern (due to mineralization & formation of periosteal spicules in the surrounding tissue) JOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 108 of 127 https:/Awww facebook, com/topnotchmedicalboardprep/it © MRI ofthe primary lesion & entire bone to evaluate the tumor fr its proximity to nerves, blood vessels, soft tissue & joint extension © Blopsy for molecular and biologi stules | 0 Complete surgical resection fr cure ‘© Chemotherapy & surgery has 65-75% 5-yr disease-free survival rate for patients with nonmetastatic extremity © Preop chemotx: doxorubicin, cisplatin, methotrexate, and ifosfamide + Prognosis: With metastasis at diagnosis: <20% survival : 12 = old gi eam ‘of & weeks of pala &swelling over her Fight | mlésthigh ep atnight She denies ay trauma or recent ness PE ly tached sof tase asin henge wihtenderes&warath but» | | enema Krayot the leg shows re pory demareted‘modeten” | | IyScleson inte rightfemoral dps wit enenson theo cue, | What is your impression? EWING SARCOMA * Small, round cell undiferentiated tumor with small uniform nuclei and scant cytoplasm thought to be of neural crest origin «Primary tumors arising from the chest wall are called Askin tumors ‘+ Symptoms similar to osteosarcoma | © Glinical Manifestations: With systemic manifestations like fever and weightloss © Whatis the most likely chromosomal aberration? © 85% of cases show a t(11;22) translocation. + What percentage of patients have metastatic disease a the time of diagnosis? 0 Only 25% of patients have overt metastases atthe time ' of diagnosis. ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MO & COCOY CALDERON A For inquiries visit www.topnotchboardprep.com.ph or https://www. facebook. com/topnotchemedicalooe: Gore SS ‘© Diagnostic: Suspected in a patient who presents with pain and oy ' ‘welling with or w/o systemic manifestations, and with an x ray finding ofa primarily lytic bone lesion with periosteal » reaction called onion-skinning "math-eaten”appearange) due to splitting & thickening of the cortex by tumor cell", + Treatment: Intensive chemotherapy on ) S prognosis Without metastases, 69% cured; with metasiges 20-30% survival Fol ee Se ff ated small satay | Ulett cae = ere esis —| teagan Spd — cept orig i Bepiome | otenwnnnnoryat | whee iu Kei ‘Sunburst pattern “Onion-skinning, moth: sun | [tangs bones Langs, bon eee —| emt aat | enter aon Simesepet | afore N ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 109 of (2 For inquiries vistt www, topnotchboardprep.com.ph oF https://www,Aromoren ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY Pl. UNONGBAYAN.MD & COCOY CALDERON.MD wwarupiep! Jacevoun comm iupnuiLiuTeut ede on ands = asdf ema Panes shar 1ST Rat Ineedors_| 39 nent lee | sen ‘Preston eae) Sparing te pnd oe soeranmaton panies Ur anquneves Vian prerm AUpHULLHuaLUplEy CUI pIY OF HNLYD:/ + Other clinical conditions due to HSV: © Genital herpes © Herpetic whitlow © Conjunctivitis keratoconjunctivitis ( Aseptic meningitis (© HSV encephalitis "+ Whatare the parts ofthe brain affected by HSV? Frontal lobe, temporal lobe, and limble system «© What are the signs/symptoms of HSV encephalitis? “Anosmia, memory loss, peculiar behavior, ‘expressive aphasto, hallucinations, focal seizures + Why do you need to treat? Untreated infections yresses to coma & death in 75% of cases. # What is the usual CSF analysis? Moderate ‘mononuclear cells & PMNs, mildly high proteln, normal or slightly decreased glucose, moderate number of RBCS Diagnostics ‘ * Biology: HSV aa hat isthe gold standard in diagnosing sy tfections? © Dstrain: type 1 ~ face & skin above the waist: ype 2 Virus culture ‘me infects the genital & the neonate ‘© What is the test of choice in examining. tig CSF7PCR to. © 3types of infection: detect HSV DNA Perimary _ HSV seronegative; subclinical + Management af 21+ Infection, nonprimary ~ infection in a person with © Disseminated infection: ia alin 3 es immunity to one type of HSV & infection by a 2"! as one hour I infusion fora hays type less severe 0 Genital herpes: topical (but not beneficial to recurrent cases); va famciclovir se 5x/day for 7 days started 3. Recurrent - reactivation ofa latent infection in an ‘Immune host with circulating Abs; follows stimuli like cold, stress, fever + Mode of Transmission: Type 1 due to direct contact with infected secretions; Type 2 from sexual activity * Incubation Period: 2-12 days « Affected neonates have generalized systemic infections or & localized CNS infection ivostomatits: Type 1 & © Clinical St mI ‘most common manifestation of primary HSV infection © Hallmark: Skin vesicles and shallow ulcers that resolves in 76days a © Who are most common © Herpes labialis or fever blisters or § cold sores -most corimon manifestation of recurrent H 1 infection o Thin-walled vesicles on a red base usually atthe ‘mucocutaneous junction & gum line ‘© Burning tingling, itching, pain 6 Heal without scars within 6-10 da sly affected? 6 months -§ yrs old Fin 8 Rees tres bua 8 ay este pe, a i cau main havo pena 9 6 Pajn in the mouth © Drooling ‘0 Refusal to drink or eat fo Fever up to 40°C ‘0 Shallow indurated uicers may be covered with a yellow-gray membrane co Untreated, the illness resolves in 7-14 days TOPNOTCH MEDICAL B( “A 14 yrold male was brought tothe ER ve toLUQpaln, History: 1 wk PTC- fever, sore throat, malaise; no consultation done PE: (x) anterior & submandibular lymph nodes, hyperemic & enlarged tonsils with exudates, palpable spleen 2-3 ms below the LCM. What ls your impression? 0 Oral acyclovir 15 mg within 72 hhoupf onset of lesions has benefits in children wi eh ES poLiomveLinis” = Uh rar Sviras from Picornaviridae family (coxsacke, ‘echbyitis, polio), with 3 antigenically distinct serotyp re + Mégedttransmission:fecal-oral fin shorly before & after the onset of ical liness > virus persists in the throat until a week after “onset & excreted in the feces for 3-4 wks Incubation Period: 7-21 days ‘inical Eorms 0 Inapparent: 90-95% asymptomatic 0 Abortive: non-specific febrile illness © Nonparalytic or aseptic meningitis © Paralytic: weakness of one or more muscle groups either skeletal or cranial © Complications: GI hemorrhage, hypertension, renal failure, ‘myocarditis Diagnosis 1° Isolation of virus in blood (end of 1 wk), throat (end of 2x8 wh), feces (end of 3% wk), CSF ‘9 CSF analysis: pleocytosis with early followed by a shift to mononuclear cells ‘and increased CHON predominanceof PMNs ‘normal glucose ‘© Supportive for the other forms ¢ Paralytic: sutable body alignment (es knees slightly flexed, hips & spine straight); 10 mg po for bladder paralysis; tracheostomy for respiratory distress Prognosis depends on the type § iv for HIV(+), household contacts of an immunodeficient patient, unimmunized adults at future risk of exposure © OPV adults at imminent (within 4 whs) risk of exposure to pollo who are unimmunized et ata right angle, Bethanecol 5- }OARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 76 of 127 of ee eeINFECTIOUS MONONUCLEOSIS. + Best known clinical syndrome due to Epstein-Barr virus (EBV) * Etiology: EBV (member of the herpesviruses) with 2 types (type ‘Land type 2 or type A and type B) © First human virus tobe associated with malignancy 6 EBV-associated malignant proliferations: 1. Nasopharyngeal carcinoma 2. Burkitt lymphoma 3. Hodgkin disease 4 Lymphoproliferative diseases 5. Leiomyosarcoma in immunodeficient states 6, PE: generalized lymphadenopathy (90%), splenomegaly (5096), hepatomegaly (10%) 7. Severe pharyngitis with marked tonsilar enlargement th ptechie atthe cin of the hard & soft palate + Man is the sole source. + Incubation Period: 30-50 days + Modeof Transmission: via sexual intercourse & in oral secretions (exchange of saliva between children) ‘* How does the clinical presentation of IM differ in children. and “adolescents? In children, primary infection is usually Inapparent. «primary infection in adolescents & adults manifests In >50% of ‘cases. Classic triad: fatigue, pharyngitis, generalized lymphadenopathy + Diagnostics © Presumptive diagnosis: typical elinfcal symptoms with atypical Iymphocytasis o Diagnosis is confirmed by serologs testing ether by heterophile antibody (Paul-Bunnell antibodies) or specifi EBV antibodles. +? ifheterophile-negatve, the IM may be caused by cytomegalovirus. 0 Culture requires 4-6 weeks, Culture method: ‘transformation assay «+ Most feared complication: splenfcrupture due to Shysterduring the 2e¢ week of illness ‘+ Management 'o Nospecific treatment % © High doses of Acyclovir with or withour steroids may ryngeal shedding iecrease vir repleston 82 a yns during the Complete recovery if without conics ate liess (ass 2-4 wh) + Glanott-Crost syndrome: Syndrome associa SO Symmetrical rash onthe cheeks Maple erythematous papules which may coalesce into plaques o Persists for )5:S0-days © Looks likeatdple dermatitis © May involve extremities & buttocks ced with EBV CYTOMEGALOVIRUS (CMI > Member of the Herpesviridae family > Most infections are inapparent ction which causes the syndrome > Most common congenital infec of ertamegallc Inclusion disease (hepatosplenomegaly. Jaundice, petechize, purpura, mirocephaly) «+ Forms of infection primary Infection: seronegative, susceptible host © Recurrent infection: reactivation of latent infection or reinfection ina seropositive immune host ‘9 Transmission sources salva, breastmilk, cervical & ‘raginal secretions, urine, semen, stool and blood «Direct person-to-person contact «isk for fetal infection greatest with maternal primary infection (40%) « perinatal transmission 10-60% by 6 months of age (genial tract secretions & breast milk) -TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT or inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/t ‘TOPNOTCH MEDICAL BOARD PREP PEDIATRICS HANDOUT BY RUBY PUNONGBAYAN, HD & COCOY CALDERON MD For inquiries visit www. topnotchboardprep.com.ph or ht www. facebook, com/topnotchmedicalboardprep/ + Pathognomonic of CMV infection: strikingly enlarged epithelial ‘or mesenchymal cells with large intranuclear Inclusions I © Signs & symptoms vary with age, rout of transmission & “immunocompetence ofthe patient ‘0 Subclinical in most patients © Congenital form: hearing loss or learning disability, [UGR, {aundice, purpura, hepatosplenomegal. intracerebral calcifications, chorloredialtis Congenital CMY infection «Diagnosis virus isolation from urine;sallva, breast mill, cervca! secretions, BAL washing > CMV/Ag by PCR & DNA hybridization ‘Teatment. : © Ganciclovir 7.5 mg/kg/day 1V every Bhrsfor 34 days © IVIG 500 mg/kgevery other day for 10 doses INFLUENZA Worldwidgacare, due to this viru ANA virus from the family Orthomyxoviridas Seer rr, cases | sporadic cases of tpldenle an $ jas animal host_ ‘No animal host | *. ‘Causes | mnyeeardisand | myosts 4 «Virus causes a bjt infection ofthe respiratory epithelium thst permit secondary bacterial invasion « onsetisabrupt: coryza, conjunctivitis, pharyngitis dry cough with igh temperature, myalgia, malaise & headache + Diagnass: confirmed serologically by hemagglutination inhibition + Treatment eaamantadine & Rimantadine as prophylaxis & treatment of type A outbreaks © Oseltamivir taken within 48 hours may decrease the severity & duration + Complications: Otitis media & pneumonia. + Excellent prognosis ras brought to the ER due to 5 days: iptermittent moderate- ‘AT yrold boy ws | grade ever with headache. PE: coherent T=38-5*,BP=100/60, | GcetioyminRR=24/min no TP no murmurs ear brea sounds soft & | jon-tender abdomen, with full pulses | Wat is your impression? DENGUE FEVER + Etiology: 4 serotypes from the family Flaviridae + Vector: Aedes aegypti * Incubation Period: 4-6 days: (1-7 days in Nelson) ‘Virus replication ~ viremia > Ag-Ab complex “> complement cascade & activation of Hageman factor > Increased vascular permeability ~ shock + WHO 2012 Classification ‘Live n/travel to endemic areas «Fever and 2 ofthe fcrteria: 1. Nausea, vomiting 2. Rash 3. Aches & pains BY RUBY PUNONGBAYAN,MD & COCOY CALDERON,MD Page 77 of 127 notchmedicalboardprep/