March 2017 Flashcards

Question

Adolescent with vitiligo, orthostatic hypotension. Labs: hypoNa, hyperK, hypogly and metabolic acidosis. Diagnosis? A. Primary hypoparathyroidism B. Addison's disease C. McCune-Albright syndrome D. Autoimmune thyroiditis

Answer 1

C Sudden death: Competitive high school sports (basketball, football) are high-risk environmental factors. Common identifiable causes of death in competitive athletes includes hypertrophic cardiomyopathy, with or without obstruction to left ventricular outflow, other cardiomyopathies, and anomalous coronary arteries; most are sudden unexplained deaths (Nelson 21st p2448)

Answer 2

A Neonates have the weakest immune reponse, thus would need further workup

Answer 3

A The recommended treatment regimens for C. trachomatis conjunctivitis or pneumonia in infants are erythromycin (base or ethylsuccinate, 50 mg/kg/day divided 4 times a day PO for 14 days) and azithromycin suspension (20 mg/kg/day once daily PO for 3 days). The rationale for using oral therapy for conjunctivitis is that 50% or more of these infants have concomitant nasopharyngeal infection or disease at other sites, and studies demonstrate that topical therapy with sulfonamide drops and erythromycin ointment is not effective. (Nelson 21st p1617)

Answer 4

A Duodenal atresia - double bubble sign (gas-filled stomach and proximal duodenum), bilious vomiting Pyloric stenosis - string sign, shoulder sign, double tract sign, nonbilious vomiting, 2x2cm olive-shaped firm movable mass Intussussception - painful paroxysms, sausage shaped mass, currant jelly stool, target sign Volvulus - vomiting, abdominal distension, target sign, coffee bean sign

Answer 5

INCOMPLETE QUESTION, answer is A

Answer 6

A Diagnosis is laryngotracheobronchitis. Croup is a clinical diagnosis and does not require radiographs. Direct laryngoscopy is more useful in epiglottitis

Answer 7

A ANA is positive in 95-99% of SLE patients, however with poor specificity for SLE. Autoantibodies associated with SLE 1. Anti DSDNA - specific for diagnosis of SLE, correlates with disease activity, especially nephritis 2. Anti Smith - specific for diagnosis of SLE 3. Anti-RNP - increased risk for Raynaud phenomenon, interstitial lung disease, and pulmonary hypertension 4. Anti Ro, Anti La - Associated with sicca syndrome, may suggest diagnosis of Sjogren syndrome. Associated with cutaneous and pulmonary manifestations of SLE. Increased risk of congenital lupus in offspring 5. Anti phospholipid - Increased risk for venous and arterial thrombotic events 6. Anti histone - Associated with drug-induced lupus (Nelson 21st p1277)

Answer 8

A The 2017 AAP guideline recommends that children 3 yr or older should have their BP measured during annual preventive visits, unless the childhas risk factors such as obesity, chronic kidney disease (CKD), or diabetes, in whom it should be checked at every healthcare encounter (Nelson 21st p2490)

Answer 9

B Lyme disease - erythema migrans, arthralgia, myalgia, other constitutional symptoms (Nelson 21st p1606) Spondyloarthritis - arthritis of the axial skeleton and hips, enthesitis, symptomatic eye inflammation, GI inflammation, rash is not as prominent (Nelson 21st p1269) JIA - Peripheral arthritis, evanescent rash, lymph node enlargement, hepatomegaly/splenomegaly, serositis (Nelson 21st p1259) SLE may present with arthritis and rash

Answer 10

A Adverse effect of anti-TB drugs 1. Isoniazid - hepatitis, peripheral neuropathy, allergic skin reactions, possible hemolysis in G6PD patients. Inhibits drug-metabolizing enzymes (DME) leading to increased risk of phenytoin, ethosuximide, and carbamazepine toxicity 2. Rifampicin - hepatitis, hypersensitivity reactions (including a systemic flu-like syndrome +/- thrombocytopenia in patients given intermittent high dose therapy), orange discoloration of body fluids. Induces drug-metabolizing enzymes 3. Pyrazinamide - Nausea, vomiting, most common cause of hepatotoxicity in regimens also containing H and R, hypersensitivity reactions, polyarthralgia 4. Ethambutol - peripheral neuropathy and retrobulbar optic neuritis (impairment of visual acuity and red-green color vision) (TBIC 2016 p141-142)

Answer 11

D Primary peritonitis is a well-described infectious complication of NS. It usually occurs within the first 2 years of diagnosis, especially during relapse periods; its incidence is about 1.4–3.7% of children with NS, with a mortality rate of 9%. Streptococcus pneumoniae is the most common organism causing bacterial peritonitis and sepsis in these patients Conti, G., Carucci, N.S., Comito, V. et al. Acute abdominal pain and nephrotic syndrome: pediatric case reports and review of the literature. Ann Pediatr Surg 17, 61 (2021). https://doi.org/10.1186/s43159-021-00130-7

Answer 12

A Properly timed cord clamping at 30-60 seconds of life or when pulsations cease, about 2cm above the base

Answer 13

C Indicators of iron deficiency anemia 1. Low Hemoglobin - when used alone, has low specificity and sensitivity 2. Low MCV - reliable but late indicator of iron deficiency; low values can also be a result of thalassemias and other causes of microcytosis. False negative results in liver disease 3. Low serum ferritin (<12 less than 5 yrs, <15 in greater than 5yrs) - most useful laboratory measure of iron stores. Low ferritin is diagnostic of iron deficiency anemia in a patient with anemia. Ferritin is an acute phase reactant that increases in many acute or chronic inflammatory conditions independent of iron status. In all age groups with infection, ferritin <30-100 4. Low Reticulocyte hemoglobin content - sensitive indicator that alls within days of onset of iron deficient erythropoiesis and is unaffected by inflammation. Not widely available 5. High Serum transferrin receptor - upregulated in iron deficiency 6. Low transferrin - limited by diurnal variation in serum iron and inflammatory conditions, aging, and nutrition 7. High Erythrocyte zinc protoporphyrin - useful screening test 8. Low Hepcidin - extremely elevated in anemia of inflammation and suppressed in IDA but limited availability (Nelson 21st p2523)

Answer 14

B 1. Caput succedaneum - caused by scalp pressure from the uterus, cervix, or pelvis; circular boggy area of edema with indistinct borders, crossing suture lines, with overlying ecchymosis 2. Cephalhematoma - well-circumscribed fluid filled mass that does not cross suture lines. Not present at delivery, develops over first few hours of life 3. Subgaleal hemorrhage - not restricted by boundaries of sutures, larger, more diffuse (Nelson 21st p869)

Answer 15

B Neonatal Skin Lesions 1. Sebaceous hyperplasia - minute profuse yellow-white papules in forehead, nose, upper lip, cheeks 2. Milia - superficial epidermal inclusion cysts with laminated keratinized materia, firm pearly opalescent white cyst 1-2mm diamter on face and gingivae and in the middle of palate (Epstein pearls) 3. Cutis marmorata - evanescent lacy reticulated red and blue cutaneous vascular pattern over most of the body surface when infant is cold 4. Harlequin color change - autonomic vascular regulation instability, longitudinal half pale, half red 5. Nevus simplex (salmon patch) - small pale pink ill defined vascular macule on glabella eyelids, upper lip, nuchal area 6. Dermal melanocytosis (mongolian spots) - blue or slate gray macular lesions with variably defined margins most common on sacral area 7. Erythema toxicum - benign self limited evanescent eruption; firm yellow-white 1-2mm papules or pustules with surrounding erythematous flare; eosinophilic infiltrates 8. Neonatal pustular melanosis - transient benign self-limited dermatosis; evanescent superficial pustules, ruptured pustules with colarette of fine scale, hyperpigmented macules; polymorphonuclear infiltrates (Nelson 21st 3453-3455)

Answer 16

A Abusive head trauma / shaken baby syndrome - poor neck muscle tone and relatively large heads of infants make them vulnerable to acceleration-deceleration forces from shaking. Features of AHT (sensitive especially when occurring together) 1. Subdural hematoma 2. Retinal hemorrhages - multiple, involve >1 layer of retina and extend to periphery; traumatic retinoschisis 3. Diffuse actional injury (Nelson 21st p104)

Answer 17

D Neonatal Skin Lesions 1. Sebaceous hyperplasia - minute profuse yellow-white papules in forehead, nose, upper lip, cheeks 2. Milia - superficial epidermal inclusion cysts with laminated keratinized materia, firm pearly opalescent white cyst 1-2mm diamter on face and gingivae and in the middle of palate (Epstein pearls) 3. Cutis marmorata - evanescent lacy reticulated red and blue cutaneous vascular pattern over most of the body surface when infant is cold 4. Harlequin color change - autonomic vascular regulation instability, longitudinal half pale, half red 5. Nevus simplex (salmon patch) - small pale pink ill defined vascular macule on glabella eyelids, upper lip, nuchal area 6. Dermal melanocytosis (mongolian spots) - blue or slate gray macular lesions with variably defined margins most common on sacral area 7. Erythema toxicum - benign self limited evanescent eruption; firm yellow-white 1-2mm papules or pustules with surrounding erythematous flare; eosinophilic infiltrates 8. Neonatal pustular melanosis - transient benign self-limited dermatosis; evanescent superficial pustules, ruptured pustules with colarette of fine scale, hyperpigmented macules; polymorphonuclear infiltrates (Nelson 21st 3453-3455)

Answer 18

D Vascular disorders of the skin 1. Port-wine stain - mature dilated dermal capillaries; macular, circumscibed, pink to purple, varied in size, common on the head and neck, unilateral 2. Sturge-Weber syndrome - associated with lateral port wine stain localized to the forehead and upper eyelid; components: Glaucoma, leptomeningeal venous angioma, seizures, hemiparesis contralateral to the facial lesion, intracranial calcification 3. Infantile hemangioma - proliferative vascular disorder; bright red protuberant, compressible, sharply demarcated lesions 4. Kasabach-Meritt syndrome - rapidly enlarging kaposiform hemangioendothelioma, thrombocytopenia, microangiopathic hemolytic anemia, acute/chronic consumption coagulopathy --> from sequestration of platelets in the enlarging lesion PHACES syndrome 1. Posterior fossa brain defects (dandy-walker malformation, or cerebellar hypoplasia) 2. Hemangioma, large segmental facial 3. Arterial cerebrovascular abnormalities 4. Coarctation of the aorta 5. Eye abnormalities 6. Sternal raphe defects (Nelson 21st Chap 669)

Answer 19

A Scleroderma Range of conditions unified by presence of skin fibrosis. Juvenile localized scleroderma (JLS/morphea) is more common. Juvenile systemic sclerosis has multiorgan involvement Mechanism of disease is a combination of vasculopathy, autoimmunity, immune activation and fibrosis. Triggers injure vascular cells, which promotes fibrosis. Vascular changes include Raynaud phenomenon and pulmonary hypertension. Raynaud phenomenon is the most frequent initial symptom - classic triphasic sequence of blanching, cyanosis, and erythema of the digits induced by cold exposure/emotional stress No diagnostic test is conclusive for scleroderma. Most patients are ANA positive and antihistone positive. Treatment for topical lesions involves topical corticosteroids or UV radiation. For deep lesions, methotrexate and systemic corticosteroids are used. (Nelson 21st Chap 185)

Answer 20

C Congenital syphilis Proven or highly probable congenital syphilis = maternal penicillin treatment during pregnancy more than 4 weeks before delivery, no maternal symptoms + infant RPR/VDLR fourfold or greater than maternal RPR/VDRL + abnormal PE Possible congenital syphilis = none or incomplete maternal treatment + normal PE + infant RPR/VDRL same or less than fourfold of maternal RPR/VDRL Congenital syphilis less likely = maternal penicillin treatment during pregnancy more than 4 weeks before delivery, no maternal symptoms + infant RPR/VDLR fourfold or greater than maternal RPR/VDRL + normal PE Congenital syphilis unlikely = adequate maternal treatment with negative titer + normal PE TREATMENT FOR SYPHILIS 1. Congenital syphilis - Pen G IV x 10 days 2. Primary, secondary, and early latent syphilis - Pen G IM x SD 3. Late latent syphilis - Pen G IM x 3 doses 1 week apart 4. Neurosyphilis - Pen G IV x 10-14 days (Nelson 21st p1597-1598)

Answer 21

A Isoniazid preventive therapy (10mkd OD x 6 months) for exposed: 1. all-HIV positive individuals 2. Children less than 5 years old who are houshold contacts of a bacteriologically confirmed TB case, regardless of TST status 3. Children less than 5 years old who are household contacts of a clinically diagnosed TB case, if the TST results are positive (TBIC 2016 p152)

Answer 22

B Birth weight is doubled at 4 months

Answer 23

B Kawasaki disease diagnostics No diagnostic test for Kawasaki disease, but there are characteristic laboratory findings 1. Elevated leukocyte count with predominance of neutrophils 2. Normocytic normochromic anemia 3. Platelet is normal in first week, butincreases in 2nd and 3rd week 4. ESR/CRP elevated in acute phase of illness 5. Other findings: sterile pyuria, mild elevations of transaminase, hyperbilirubinemia, CSF pleocytosis 6. 2D echo is most useful for prognostication and monitoring for coronary artery aneurysm. Echo should be performed at diagnosis and again at 1-2 week of illness, and again at 6-8 week after onset of illness. Recommended repeat echo at 1 year after illness (Nelson 21st p1313-1314)

Answer 24

A Vitamin Deficiencies 1. Vitamin A: Nyctalopia/night blindness, xerophthalmia, bitot spots, follicular hyperkeratosis 2. Vitamin D: Rickets, reduced bone mineralization 3. Vitamin E: Hemolysis in preterm infants, areflexia, ataxia, ophthalmoplegia 4. Vitamin K: VKDB 5. Vitamin B1 (thiamine): beriberi - polyneuropathy, calf tenderness, heart failure, ophthalmoplegia 6. Vitamin B2 (riboflavin): anorexia, mucositis, cheilosis, nasolabial seborrhea 7. Vitamin B3 (niacin): pellagra - photosensitivity, dermatitis, diarrhea, dementia, death 8. Vitamin B6 (pyridoxine): seizures, hyperacuisis, microcytic anemia, nasolabial seborrhea, neuropathy 9. Vitamin B7 (biotin): alopecia, dermatitis, hypotonia 10. Vitamin B9 (folate): megaloblastic anemia 11: Vitamin B12 (cobalamin): megaloblastic anemia, peripheral neuropathy, posterolateral spinal cord disease, vitiligo 12. Vitamin C: scurvy - irritability, purpura, bleeding gums, periosteal hemorrhage, bone pain

Answer 25

D Indices of nutritional status 1. Height for age is a measure of linear growth, and a deficit represents the cumulative impact of adverse events, usually in the first 1000 days from conception, that results in STUNTING or chonic malnutrition. 2. Low Weight for height indicates acute malnutrition (WASTING) High weight for height indicates OVERWEIGHT 3. Weight for age is the most commonly used index of nutritional status, although a low value has limited clinical significance because it does not differentiate between wasting and stunting 4. MUAC - used for screening wasted children in humanitarian emergencies and some community/outpatient settings 5. BMI for age - screening tool for thinness, overweight, and obesity (Nelson 21st p333-334)

Answer 26

A The refeeding syndrome may occur if high-energy feeding is started too soon or too vigorously, and it may lead to sudden death with signs of heart failure. (Nelson 21st p342) Treatment of malnutrition 1. Supplemental oral feeds in outpatient setting with close followup 2. Rehabilitation for suck-and swallow evaluation if the history suggests difficulty in oral feeds 3. The type of caloric supplementation is based on the severity of the malnutrition and underlying medical condition 4. The same anthropometric measures used to diagnose malnutrition should be used to measure progress and recovery 5. Multivitamin supplementation should be given to all children with malnutrition 6. Therapy for psychosocial factors, parent education (Nelson 21st p344)

Answer 27

A Treatment of malnutrition 1. Supplemental oral feeds in outpatient setting with close followup 2. Rehabilitation for suck-and swallow evaluation if the history suggests difficulty in oral feeds 3. The type of caloric supplementation is based on the severity of the malnutrition and underlying medical condition 4. The same anthropometric measures used to diagnose malnutrition should be used to measure progress and recovery 5. Multivitamin supplementation should be given to all children with malnutrition 6. Therapy for psychosocial factors, parent education (Nelson 21st p344)

Answer 28

D A. Rubeola/measles. Measles is a serious infection characterized by high fever, an enanthem (Koplik spots), cough, coryza, conjunctivitis with photophobia, and a prominent exanthem (erythematous maculopapular rash) (Nelson 21st p1671) B. Kawasaki disease. Features involve fever + bilateral nonexudative conjunctivitis, polymorphous nonvesicular rash, unilateral cervical adenopathy, strawberry tongue, erythema of hands and feet (CRASH + Burn mnemonic) C. Dengue. Mucosal involvement is estimated to occur in 15% to 30% of patients with dengue viral infections and more commonly in patients with DHF than with DF.[12] The mucosal manifestations noted in dengue viral infections are conjunctival and scleral injection (MUCOCUTANEOUS MANIFESTATIONS OF DENGUE FEVER, Indian J Dermatol. 2010 Jan-Mar; 55(1): 79–85.) D. SLE. Skin manifestations of lupus: malar rash, discoid rash, photosensitive rash, cutaneous vasculitis (petechiae, palpable purpura, gangrene, urticaria). Ocular manifestations of lupus: Retinal vasculitis, scleritis, episcleritis, papilledema, dry eyes, optic neuritis Lupus may manifest with conjunctivitis and rashes, however FEVER is not included in SLICC criteria (Nelson 21st p1275)

Answer 29

A Conjunctivitis 1. Acute purulent conjunctivitis - generalized, often bilateral conjunctival hyperemia, edema, mucopurulent exudate, glued eyes, ocular pain/discomfort. No pruritus or periauricular lymph node enlargement. Most common cause is H. influenza. Treatment is topical antibiotics 2. Viral conjunctivitis - watery discharge, often unilateral, associated with periauricular lymph node swelling. Most common cause is adenovirus. Treatment is supportive 3. Epidemic keratoconjunctivitis - caused by adenovirus 8, 19, 37. Sensation of foreign body beneath the lids with itching, burning, chemosis, periauricular adenopathy, conjunctival pseudomembrane, BOV from subepithelial corneal infiltrates. Treatment is supportive 4. Membranous and pseudomembranous conjunctivitis - classically in diphtheria. Fibrin rich exudate/membrane on conjunctival surface permeating the epithelium. Bleeding when membrane is removed in membranous, no bleeding in pseudomembranous 5. Allergic conjunctivitis - intense itching, clear watery discharge, conjunctival edema, seasonal. Treatment include cold compress, topical antihistamine, mast cell stabilizers, prostaglandin inhibitors 6. Vernal conjunctivitis - associated with atopy, with cobblestone appearance. Treatment is topical corticosteroids (Nelson 21st P3366-3367)

Answer 30

C The metabolic syndrome consists of central obesity, hypertension, glucose intolerance, and hyperlipidemia (Nelsons 21st p351)

Answer 31

C | Nelsons 21st p357

Answer 32

A Rectal suction biopsy is the “gold standard” for diagnosing Hirschsprung disease. The biopsy material should contain an adequate amount of submucosa to evaluate for the presence of ganglion cells. To avoid obtaining biopsies in the normal area of hypoganglionosis, which ranges from 3 to 17 mm in length, the suction rectal biopsy should be obtained no closer than 2 cm above the dentate line Anorectal manometry evaluates the internal anal sphincter while a balloon is distended in the rectum. In patients with Hirschsprung disease, the internal anal sphincter fails to relax in response to rectal distention, and there is absence of the rectoanal inhibitory reflex An unprepared contrast enema is most likely to aid in the diagnosis in children older than 1 mo of age because the proximal ganglionic segment might not be significantly dilated in the first few wk of life. Classic findings are based on the presence of an abrupt narrow transition zone between the normal dilated proximal colon and a smaller-caliber obstructed distal aganglionic segment. The sensitivity (~70%) and specificity (50–80%) of barium enema studies diagnosing Hirschsprung disease is lower than other methodologies (Nelson 21st p1963-1964)

Answer 33

``` D Rhinitis medicamentosa (RM), also known as ‘rebound congestion’ is inflammation of the nasal mucosa caused by the overuse of topical nasal decongestants. It may be also caused by oral beta-adrenoceptor antagonists, antipsychotics, oral contraceptives, and antihypertensives. It classifies as a subset of drug-induced rhinitis. ``` Topical nasal decongestants can classify as either beta-phenylethylamine derivatives (ephedrine, phenylephrine) or imidazoline (naphazoline, oxymetazoline, xylometazoline) derivatives (Rhinitis medicamentosa NCBI 2021)

Answer 34

C Posterior fossa tumors of childhood 1. Medulloblastima (35-40%), 2-3 month hx of headaches, vomiting, truncal ataxia; CT: heterogenously or homogenously enhancing 4th ventricular mass; survival 65-85% depending on stage 2. Cerebellar astrocytoma (35-40%), 3-6 month hx of limb ataxia, secondary headaches, vomiting; CT: cerebellar hemisphere mass usually with cystic and solid components; survival 90-100% esp. in pilocytic type 3. Brainstem glioma (10-15%), 1-4 month of diplopia, ataxia, cranial nerve dysfunction; CT: diffusely expanded, minimally or partially enhancing mass in 80%, 20% more focal tectal or cervicomeedullary region; survival <10% in diffuse tumors 4. Ependymoma (10-15%), 2-5 month hx of unsteadiness, headaches, diplopia, facial asymmetry; CT: usually enhancing 4th ventricular mass with cerebellopontine predilection; survival >75% in totally resected 5. Atypical teratoid/rhabdoid (>5%, 10-15% of infantile malignant tumors); similar to medulloblastoma but primarily in infants with more lateral extension; <20% survival in infants (Nelsons 21st p2668)

Answer 35

D Nelson 21st p3169 Athetoid CP, also called choreoathetoid, extrapyramidal, or dyskinetic CP, is less common than spastic CP and makes up approximately 15–20% of patients with CP. Affected infants are characteristically hypotonic with poor head control and marked head lag and develop variably increased tone with rigidity and dystonia over several years. Generally, upper motor neuron signs are not present, seizures are uncommon, and intellect is preserved in many patients. This form of CP is also referred to in Europe as dyskinetic CP and is the type most likely to be associated with birth asphyxia.

Answer 36

D They are usually AR. AD disorders are usually structural defects

Answer 37

D Neonatal peripheral nerve injuries 1. Brachial palsy - occurs in delivery of shoulders during vertex presentation, raising of warms during breech presentation, shoulder dystocia a. Erb-Duchenne paralysis - 5th and 6th cervical nerve injury, no abduction, external rotation, supination. Biceps reflex is absent, moro reflex is absent b. Klumpke paralysis - 7th and 8th cervical nerves + 1st thoracic nerve injury. Paralyzed hand and miosis (Horner Syndrome), may involve shoulder drop (deltoid atrophy from lack of innervation) 2. Phrenic nerve paralysis - injury to 3rd, 4th, 5th cervical nerves. Causes diaphragmatic paralysis with thoracic breathing, diaphragmatic asymmetry during respiration. May require O2 support and MV 3. Facial nerve palsy a. Peripheral facial paralysis - flaccid, invoolves entire side of the face including forehead. No movement in affected side of the face b. Central facial paralysis - facial sparing (forehead wrinkles) (Nelson 21st p924-925)

Answer 38

A Among the choices, only x-linked agammaglobulinemia presents with lack of lymphoid tissue (no lymph nodes, tonsils). Males are more affected than females, reflecting X linked recessive inheritance Immunodeficiencies 1. Disorders of humoral immunity (XLA, selective IgA deficiency, hyper IgM syndrome, X linked lymphoproliferative disease) - Impaired opsonization, inability to agglutinate, inability to neutralize toxins - Pyogenic infections with extracellular and ecapsulated organisms - Lack of lymphoid tissue in X linked agammaglobulinemia 2. Disorders of cell-mediated immunity (Congenital thymic aplasia, chronic mucocutaneous candidiasis) - Inability of T cells to function to present antigens to B cells - Opportunistic, low grade infections with viruses and fungi; autoimmune defects, malignancies 3. Phagocyte disorders (leukocyte adhesion deficiency, chronic granulomatous disease, Chediak-Higashi syndrome) a. Quantitive - insufficient number of neutrophils - skin/mucosal infections, sinopulmonary infections b. Functional - inability to kill pathogens due to decreased O2 metabolites - infections with catalase positive bacteria, fungi; chronic lymphadenitis, granulomas, otitis media, colitis c. Leukocyte adhesion - inability of phagocytes to migrate - infections with catalase positive bateria and fungi; gingivitis, intestinal fistulas, poor wound healing 4. Complement disorders (C2 deficiency, C3/C4 deficiency, membrane attack complex deficiency) - Impaired opsonization - Infections with encapsulated extracellular organisms, meningococcal/gonococcal disease; autoimmune disease esp. SLE 5. Combined immunodeficiencies (severe combined immunodeficiency, Wiskott -Aldrich syndrome) - Disorders in more than one component of the immune system

Answer 39

B Breastfeeding jaundice - 3rd-4th DOL - Inadequate supply of breastmilk leading to increased enterohepatic circulation - Tx: Increase breastfeeding to 8-10x/day Breast milk jaundice - 1st-2nd week of life - Glucoronidase in breast milk increases enterohepatic circulation - Tx: Increase breastfeeding frequency, phototherapy

Answer 40

A Neonatal tetanus, the infantile form of generalized tetanus, typically manifests within 3-12 days of birth. It presents as progressive difficulty in feeding (sucking and swallowing), associated hunger, and crying. Paralysis or diminished movement, stiffness and rigidity to the touch, and spasms, with or without opisthotonos, are characteristic. The umbilical stump, which is typically the portal of entry for the microorganism, may retain remnants of dirt, dung, clotted blood, or serum, or it may appear relatively benign. (Nelson 21st p1550)

Answer 41

A Neurocutaneous syndromes - heterogenous group of disorders characterized by abnormalities in both the integument and CNS arising from a defect in the differentiation of the primitive ectoderm 1. Neurofibromatosis 1, neurofibromatosis 2, schannomatosis - Autosomal dominant (NF1 and NF2) - Tumors grow on nerves and result in systemic abnormalities - Features of NF1 1. Cafe au lait macules - hallmark of neurofibromatosis 2. Axillary or inguinal freckling 3. Iris Lisch nodules - hamartomas within the iris 4. Neurofibromas or plexiform neurofibroma 5. Osseous lesioons - sphenoid dysplasia, cotical thinning of long bones 6. Optic glioma 7. First degree relative with NF1 - Features of NF2 1. bilateral vestibular schawannoma 2. First degree relative with NF2 3. Unilateral vestibular schwannoma and any two of: meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities 4. Multiple meningiomas and unilateral vestibular schwannoma - Feature of schwannomatosis - multiple schwannomas in the absence of bilateral vestibular schwannomas 2. Tuberous sclerosis - Autosomal dominant - Disinhibition of TSC1, TSC2 (tumor suppressor genes) causing loss of tuberin or hamartin resulting in the formation of numerous benign tumors - Features 1. Hamartomas (elevated mulberry lesions or plaque like lesions) 2. Skin lesions (ash leaf patches, facial angiofibromas, shagreen patch) 3. Cortical tuber - Most common manifestations: epilepsy, cognitive impairment, autism - May present with cardiac rhabdomyoma, renal angiomyolipoma 3. Sturge-Weber syndrome - segmental vascular neurocutaneous disorder characterized by capillary malformation on face (port-wine stain) and brain (leptomeninges), abnormal eye blood vessels leading to glaucoma 4. Von Hippel-Lindau disease - autosomal dominant mutation affecting VHL (tumor suppressor), presents with cerebellar hemangioblastomas, retinal angiomas 5. PHACE - posterior fossa malformations (Dandy Walker), hemangiomas, arterial anomalies, coarctation of the aorta, eye abnormalities PHACES syndrome - PHACE + ventral developmental defects including sternal clefting and supraumbilical raphe 6. Kippel-Trenaunay syndrome - mixed capillary, venous, or lymphatic malformations involving bone and muscle in one limb (Nelson 21st Chap 614)

Answer 42

A EINC (1) immediate and thorough drying of the baby, (2) early-skin-to-skin contact between the mother and the newborn, (3) properly-timed cord clamping, and (4) non-separation of the mother and baby for early breastfeeding initiation

Answer 43

A daw ang sagot pero ang labo ng recall nila Vitamin A supplementation for measles: 2 doses 24 hours apart 1. Infants less than 6 months: 50,000 IU/day 2. 6 months - 11 months: 100,000 IU/day 3. >1 year old: 200,000 IU/day The therapeutic doses of Vitamin A for measles should be given as soon as diagnosis is made regardless of when the last dose of Vitamin A was given (PIDSP Interim Management Guidelines for Measles 2013)

Answer 44

Guillan-Barre

Answer 45

Orbital cellulitis

Answer 46

A Aspirin is a COX inhibitor that directly inhibits platelet aggregation; in patients with low platelet from dengue, it may cause life threatening bleeding (Katzung 10th ed)

Answer 47

D Leading causes of pediatric death (US, 2016) 1. Motor vehicle crashes 2. Firearm-related injuries 3. Malignant neoplasms 4. Suffocation Leading causes of pediatric death, Philippines (DOH, 2010) Infant 1. Bacterial sepsis 2. Pneumonia 3. Respiratory distress of newborn Age 1-4 1. Pneumonia 2. Gastroenteritis 3. Congenital anomalies Age 5-9 1. Pneumonia 2. Dengue 3. Drowning Age 10-14 1. Pneumonia 2. Drowning 3. Diseases of nervous system

Answer 48

A Opportunistic infections like oral candidiasis are a hallmark of disorders of cell-mediated immunity (T cells). Choices B-D are disorders of humoral immunity. Immunodeficiencies 1. Disorders of humoral immunity (XLA, selective IgA deficiency, hyper IgM syndrome, X linked lymphoproliferative disease) - Impaired opsonization, inability to agglutinate, inability to neutralize toxins - Pyogenic infections with extracellular and ecapsulated organisms - Lack of lymphoid tissue in X linked agammaglobulinemia 2. Disorders of cell-mediated immunity (Congenital thymic aplasia, chronic mucocutaneous candidiasis) - Inability of T cells to function to present antigens to B cells - Opportunistic, low grade infections with viruses and fungi; autoimmune defects, malignancies 3. Phagocyte disorders (leukocyte adhesion deficiency, chronic granulomatous disease, Chediak-Higashi syndrome) a. Quantitive - insufficient number of neutrophils - skin/mucosal infections, sinopulmonary infections b. Functional - inability to kill pathogens due to decreased O2 metabolites - infections with catalase positive bacteria, fungi; chronic lymphadenitis, granulomas, otitis media, colitis c. Leukocyte adhesion - inability of phagocytes to migrate - infections with catalase positive bateria and fungi; gingivitis, intestinal fistulas, poor wound healing 4. Complement disorders (C2 deficiency, C3/C4 deficiency, membrane attack complex deficiency) - Impaired opsonization - Infections with encapsulated extracellular organisms, meningococcal/gonococcal disease; autoimmune disease esp. SLE 5. Combined immunodeficiencies (severe combined immunodeficiency, Wiskott -Aldrich syndrome) - Disorders in more than one component of the immune system

Answer 49

Toxicity of kerosene is due to its local irritating effect and systemic effects. There is a high aspiration potential due to its low viscosity. CNS depressant effects may occur, and seizures may occur due to hypoxia Ingestion of >30ml with vomiting presents a higher risk for development of pulmonary toxicity Major complications 1. Aspiration pneumonia 2. Gastritis 3. Hypoprothrombinemia (low PT) 4. Seizures (Algorithms of Common Poisonings 4th ed)

Answer 50

A The goal of imaging studies in children with UTI is to identify anatomic abnormalities that predispose to infection, determine whether there is active renal involvement, and assess whether renal function is normal or at risk. The AAP practice parameter recommends initial ultrasound of kidneys, ureters, and bladder for children 2-24 months old with a first episode of UTI (Nelson 21st p2793-2794)

Answer 51

B VUR is usually discovered during evaluation for UTI. Among these children, 80% are female, and the average age of diagnosis is 2-3 years. (Nelson 21st p2797)

Answer 52

B Risk factors for adolescent suicide 1. Preexisting medical disorder (90%) - most often major depression - Females: chronic anxiety, especially panic disorder - Males: conduct disorder and substance abuse 2. Cognitive distortions - negative self-attributions leading to hopelessnes 3. Biologic factors - serotonergic system, adrenergic system, hypothalamic-pituitary axis; family history of mental disorders 4. Social, environmental, and cultural factors - Most adolescent suicide attempts are precipitated by stressful life events - Suicide attempts may be precipitated by exposure to news or media - Physical and sexual abuse increase suicide risk - Poor access to mental health programs increases suicide risk (Nelson 21st p225-226)

Answer 53

C FISTS Mnemonic to Assess and Adolescent's Risk of Violence F: Fighting (How many fights were you in last year? What was the last?) I: Injuries (Have you ever been injured? Have you ever injured someone else?) S: Sex (Has your partner hit you? Have you hit your partner? Have you ever been forced to have sex?) T: Threats (Has someone with a weapon threatened you? What happened? Has anything changed to make you feel safer?) S: Self-Defense (What do you do if someone tries to pick a fight? Have you ever carried a weapon in self-defense?) (Nelson 21st p1038)

Answer 54

C Bradypnea is a sign of impending respiratory failure

Answer 55

C Croup/Laryngotracheobronchitis often caused by parainfluenza virus Laryngitis is frequently viral Bronchitis is frequently viral Epiglottitis is bacterial (H. influenza type B in unvaccinated; S. pyogenes, S. pneumonia, nontypeable H. influenza, S. aureus in vaccinated) (Nelson 21st Chap 412)

Answer 56

A Early childhood risk factors for persistent asthma MAJOR 1. Parental asthma 2. Atopic dermatitis 3. Inhalant allergen sensitization ``` OTHER Allergic rhinitis Food allergy Food allergen sensitization Pneumonia Bronchiolitis requiring hospitalization Wheezing apart from colds Male gender Low birthweight Environmental tobacco smoke exposure Reduced lung function at birth Formula feeding ``` (Nelson 21st p1187)

Answer 57

B First Line TB Drugs 1. Isoniazid (10 (10-15) mkday, max 300 mg/day) - bactericidal against actively growing MTB, inhibits mycolic acid synthesis, inhibits catalase-peroxidase enzyme 2. Rifampicin (15 (10-20) mkday, max 600 mg/day) - Inhibits DNA dependent RNA polymerase 3. Pyrazinamide (30 (20-40) mkday, max 2 g/day) - Disruption of membrane of energy metabolism 4. Ethambutol (20 (15-25) mkday, max 1.2 g.day) - Inhibits transferase enzymes involved in cell wall synthesis) (TBIC p141-142)

Answer 58

A Management of apnea of prematurity 1. Gentle tactile stimulation or provision of flow and/or supplemental oxygen by nasal cannula is often adequate therapy for mild and intermittent episodes 2. Nasal CPAP and heated humidified high flow nasal cannula are appropriate therapies. nCPAP may be preferred in extremely preterm infants 3. Recurrent or persistent apnea is treated with methylxanthines (increase respiratory drive by lowering the threshold of response to hypercapnia) 4. Caffeine and theophylline are similarly effective, but caffeine is preferred due to longer half life and less side effects (tachycardia, feeding intolerance) 5. Caffeine PO or IV with 20mkdose loading then maintained at 5-10mkday (Nelson 21st p931)

Answer 59

A Interpretation of growth indicators Height for age Below -2: Stunted Below -3: Severely stunted Weight for age Below -2: Underweigh Below -3: Severely underweight ``` Weight for height / BMI for age Above 3: Obese Above 2: Overweight Above 1: Risk for overweight Below -2: Wasted Below -3: Severely wasted ``` (Prev Ped 2018 p35)

Answer 60

B ASA Physical status classification system ASA 1: Normal healthy patient ASA 2: Patient with mild systemic disease ASA 3: Severe systemic disease ASA 4: Severe systemic disease that is a constant threat of life ASA 5: Moribund patient who is not expected to survive without the operation ASA 6: Declared brain dead patient whose organs are being removed for donor purposes (American society for Anesthesiologists, 2013)

Answer 61

C Duration of followup for patients with primary TB depends on clinical status after therapy. Radiologists can recommend followup study for as early as three to six months or sooner if the patient is not responding to treatment. If the patient has good clinical response, followup xray will be at the clinician's discretion (TBIC 2016 p100)

Answer 62

B Nelson 21st p3590 Growth and Development of Bones and Joints Consideration of growth and development helps formulate treatment strategies designed to preserve or restore normal growth potential. Growth is subject to many variables, including genetics, nutrition, general health, endocrine status, mechanical forces, and physiologic age. Growth also varies between two anatomic regions and even between two bones of the same region.

Answer 63

A Measles patients are infectious from 3 days before to 4-6 days after the onset of the rash. Portal of entry is through droplet or aerosol exposure. (Nelson 21st p1670) Mumps is spread from person to person by respiratory droplets. Virus appears in the saliva from 7 days before to as long as 7 days after onset of parotid swelling. The period of maximum infectiousness is 1-2 days before to 5 days after onset of parotid swelling (Nelson 21st p1680) Rubella viremia is most intense from 10-17 days after infection. Viral shedding in the nasopharynx begins 10 days after infection and may be detected up to 2 weeks following onset of the rash. The period of highest communicability is from 5 days before to 6 days after the appearance of the rash. (Nelson 21st p1677-1678)

Answer 64

A Persons with varicella may be contagious 24-48 hours before rash is evident and until vesicles are crusted, usually 3-7 days after onset of rash. Varicella is spread by aerosolization of virus in cutaneous lesions and by oropharyngeal secretions (Nelson 21st p1709) Mortality is particularly high in neonates born to susceptible mothers who contract varicella around the time of delivery. Infants whose mothers demonstrate varicella in the period from 5 days prior to delivery to 2 days afterwards are at high risk for severe varicella. (Nelson 21st p1710)

Answer 65

A Hepatitis A is highly contagious, transmitted through the fecal-oral route, and is highly contagious 2 weeks before to 7 days after jaundice appears (Nelson 21st p2110)

Answer 66

A Rabies post exposure prophylaxis Cat I: Intact skin contact with animal, casual contact with infected person --> NO RV, RIG; wash only Cat II: Nibbling of intact skin with bruising, abrasions induced to bleed: --> RV only, NO RIG Cat III: Transdermal puncture with bleeding, licks on broken skin or mucus membrane, handling of carcassess, exposure to body fluids, all Cat II on head and neck --> RV + RIG

Answer 67

C Secure circulation-airway-breathing in that order

Answer 68

A High quality CPR: 1. Chest compression fraction >80% 2. Compression rate of 100-120bpm (30:2 for single rescuer, 15:2 for double resucer) 3. Compression depth of at least 50 mm (2 inches) in adults and at least 1/3 the AP dimension of the chest in infants and children. 4. No excessive ventilation (https://cpr.heart.org/en/resuscitation-science/high-quality-cpr)

Answer 69

A Gray baby syndrome is an adverse reaction to chloramphenicol that is characterized by abdominal distention, hemodynamic collapse, and ashen-gray skin discoloration in neonates. (NCBI) Bronze baby syndrome is causes by phototherapy, characterized by an intense grey-brown discoloration of the skin, serum, and urine, and anemia (NCBI)

Answer 70

A Bilirubin absorbs light maximally in the blue range (420-470nm). 1. Conversion of 4Z,15Z bilirubin into 4Z,15E bilirubin which can then be excreted in bile through reversible photoisomerization 2. Conversion of bilirubin into lumirubin, an irreversible structural isomer that can be excreted in kidneys (Nelson 21st p959)

Answer 71

A SSSS - S. aureus; tender erythematous blistering exfoliating rash from exotoxins (epidermolysin A, B) Erythema toxicum - papules on an erythematous base, benign in neonates Erythema multiforme - immune-mediated reaction, erythematous target-like rash on the skin or mucous membranes. Erysipelas - S. pyogenes; tender raised erythematous rash

Answer 72

A Consolidation - crackles, ronchi, dull percussion, increased fremitus Atelectasis - decreased BS, dull percusion, decreased fremitus Pleural effusion - decreased BS, dull percussion, decreased fremitus Pneumothorax - decreased BS, resonant percussion, decreased fremitus (USMLE First Aid)

Answer 73

B Effusion is transudative. Do repeat CXR Lights criteria 1. Transudate ``` Serous WBC <10,000 pH >7.2 (alkaline) Protein < 3.0 (low) PF:serum protein ratio <0.5 LDH <200 (low) PF: serum LDH ratio <0.6 Glucose >60 (high) ``` 2. Exudate ``` Cloudy WBC >50,000 pH <7.2 (acidic) Protein > 3.0 (high) PF:serum protein ratio > 0.5 LDH > 200 (high) PF: serum LDH ratio <0.6 Glucose < 60 (low) ``` TRANSUDATE: Alkaline, low protein, low LDH, high glucose EXUDATE: Acidic, high protein, high LDH, low glucose (Nelson 21st p2274)

Answer 74

B Nelson 21st p2112 HBV infection can also result in chronic hepatitis, which leads to cirrhosis, end-stage liver disease complications, and HCC Hepatitis B and D may be transmitted perinatally, but Hep D can only infect individuals with Hep B infection.

Answer 75

B Otitis externa - Acute ear pain, edema of ear canal, erythema, thick, clumpy otorrhea; cerumen is white and soft in consistency Acute otitis media 1. Moderate to severe bulging of the TM or new onset otorrhea not caused by otitis externa 2. Mild bulging of the TM and recent (<48hr) onset of ear pain or intense TM erythema Otitis media with effusion - bulging of the TM is absent or slight, erythema absent or slight Signs of middle ear effusion 1. White, yellow, amber, blue TM discoloration 2. Opacification other than that caused by scarring 3. Decreased or absent mobility (Nelson 21st p3421)

Answer 76

A Diagnosis is laryngotracheobronchitis Barking cough - croup Brassy cough - bacterial tracheitis Whooping cough - pertussis

March 2017 Flashcards

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