ATI Pediatrics Test Bank 2022_ Solution guide.

ATI Pediatrics Test Bank 2022_ Solution guide. ______ is a staphyloccocal, streptococcal, or combined infxn characterized by discrete, thin-walled vesicles that rapdily become pustular and then rupture, leaving a honey/straw-colored crust. - Impetigo contagiosa The most common infectious cause of Stevens-Johnson syndrome is... - mycoplasma pneumoniae The diagnosis of stevens-johnson syndrome is made when lesions occur in 2+ mucosal surfaces covering less than __% of the body surface area - 10% Toxic epidermal necrosis is the dx when there is > __% of the body affected - 30% _____ are necessary to establish a diagnosis of asthma if the patient has a classic clinical presentation and history compatible with asthma - No additional studies Asthma is a _____ diagnosis - clinical Dermacentor andersoni aka... - the wood tick Dermacentor andersoni is the principal vector of... - Rocky Mountain Spotted Fever Pediculus humanous capitis aka... - head louse Which bone disease is associated with uveitis? (a) Slipped capital femoral epiphysis (b) Juvenile idiopathic arthritis (c) Henoch-schonlein purpura (d) Legg-calve-perthes disease - (b) Juvenile idiopathic arthritis Henoch-schonlein purpura aka... – anaphylactoid purpura ____ is defined as the passage of feces in inappropriate locations after bowel control would be expected - Encopresis What is the tx for retentive encopresis? – - Clearing of fecal mass - Mineral oil or stool softners - Behavioral modification When does chlamydial pneumonia present? - At several weeks of life In a patient that is 8 hours old, with low WBC count and left shift, and infiltrates bilaterally on CXR, what is the most likely culprit? - GBS pneumonia What is a true contraindication to administration of the 4th DTaP vaccine? (a) Positive Fam hx of adverse rxns to DTaP vax (b) Past hx of infantile spasms (c) Child is currently febrile to 39 degrees (102.2F) (d) Prolonged seizures 6 days after the last DTaP vax - (d) Prolonged seizures 6 days after the last DTaP vax How long does TB meningitis linger on for? - 3 weeks What is the age range for seeing TB meningitis - 6 mos to 4 yrs What kind of mass can grow in the tympanic membrane as a result of recurrent otitis media? - Cholesteatoma What do you do next when you discover a cholesteatoma in the tympanic membrane? – - ENT referral - CT scan of temporal bones ____ is a chronic dermatitis that occurs in a population with a strong personal or family hx of atopy - Eczema What is the first line tx for eczema? (a) Coal-tar soaps and shampoo (b) Topical antifungal cream (c) U/V light therapy (d) Moisturizers and topical steroids (e) Topical abx - (d) Moisturizers and topical steroids What should you use to replenish fluids for severe dehydration? - Normal Saline Ringer's Lactate _____ is a common organism in infected cat and dog bites - Pasteurella multocida How do you tx dysfunctional uterine bleeding? - Progestin only contraceptive or combined oral contraceptives Sign and sx of ______ include disturbance in mood or sleep, decline in school performance, truancy, alterations in family relationships and peer groups, and diminished appetite. - Substance abuse How do you remember what things you need to measure for the APGAR score? - ~ Her Royal Majesty Requests Crumpets - Heart rate - Respirations - Muscle tone - Reflex irritability - Color Antibiotic ppx during animal bites are recommended for what groups of people? – - Infants - Diabetics - Immunocompromised What's the most common way for an infant to get a seizure? - Diluting formula and getting hyponatremia Found in about 50% of term newborns. Lesions are 2-3 cm erythematous macules; some have a central yellow-white pustule. Examination of fluid from these lesions demonstrates eosinophils. - Erythema toxicum More common in blacks. 1-2 mm pustules that result in hyperpigmented lesion encircled by a collarette of scale upon rupture of the pustule. Last for wks to months. - Pustular melanosis ______ are small, sharply edged lesions that occur most commonly on the head and neck of infants. These lesions are yellow-orange in color, slightly elevated, and hairless. - Sebaceous nevi ___ are fine, yellowish white, 1-2 mm firm raised lesions scattered over the face of the neonate. They are cysts that contain keratinized material. Usually resolves on its own. - Milia Night terrors are ____ phenomena (a) REM (b) Non-REM - (b) Non-REM Somniloquy aka... - sleeptalking Somnambulism aka... - sleepwalking ____ are small, ulcerated lesions on exposed areas such as ears and fingers from cold. Lesions can last 1-2 wks. - Chillblains _____ is destruction of fat cells caused by exposure to cold weather or a cold object, which is usually a benign condition that self-resolves - Cold panniculitis ____ is manifest by small, firm, white, cold patches of skin in exposed areas; treatment is rewarming the areas before they become numb. - Frostnip Which deficiency has cheilosis, glossitis, keratitis, conjunctivitis, corneal vascularization, and seborrheic dermatitis? - Riboflavin deficiency Which deficiency has seizures, peripheral neuritis, dermatitis, and microcytic anemia? - Vitamin B6 (pyridoxine) deficiency What does VACTERL syndrome stand for? – - Vertebral defect - Anal atresia - Cardiac defects - Tracheoesophageal fistula - Renal/radial defect - Limb abnormalities Which limb abnormality is most common among obese boys with delayed skeletal maturation or in thin, tall adolescents having recently enjoyed growth spurt. - Slipped capital femoral epiphysis _____ is avascular necrosis or idiopathic osteonecrosis of the femoral head. Happens to boys btwn ages 2 and 12 years. Sxs include limp and pain in the anterior thigh, groin, knee, "painless limp" - Legg-Calve-Perthes disease Painful limp usually from 2 to 6 years of age. WBC and ESR may be normal of slightly elevated. - Transient synovitis How do you treat salicylate poisoning? – - Acetazolamide - IV sodium bicarb Morphine and narcotic toxicity on newborns during birth can be treated with... - Naloxone ____ can cause an idiosyncratic rxn causing extrapyramidal sxs such as oculogyric crisis, temors, torticollis, opisthonus, dysphagia - Phenothiazine How do you tx phenothiazine toxicity? - Diphenhydramine What toxicity is this? A 2 year-old boy found a bottle of his mother's prenatal vitamins and consumed the majority of them. He now has hematemesis and abdominal pain. He is febrile, and lab tests reveal a leukocytosis and hyperglycemia. - Iron toxicity For trichomonas, you can use metronidazole or... - tinidazole What is the first line treatment for giardia? - Tinidazole What is the hallmark sxs of cyclospora cayetanesis? - Prolonged nonbloody diarrhea after incubation of 7 days Bilateral microtia or anotia, congenital heart disease, CNS abnormalities, what chemical exposure? - Retinoic acid What is the first line tx of cyclospora cayetanesis? - TMP-SMZ What is the first line tx of dipyllobothrium latum? - Praziquantel ACEi in pregnancy causes... - renal dysgenesis, oligohydramnios Congenital rubella causes... - cataracts What kind of heart condition can occur when a patient with SLE gives birth to a kid? - Congenital heart block ____ is administered shortly after birth prevents hemorrhagic dz of the newborn - Vitamin K A child that has reoccurring liver abscesses may have... - chronic granulomatous disease (CGD) ____ defects are likely to result in low immunoglobulin A, G, and M levels and result in multiple infxns like otitis media and sinusitis. - B-cell What test do you use to test for T-cell deficiencies? - Intradermal skin test using Candida albicans 3 key characteristics of fetal alcohol syndrome - - Small palpebral fissures - Smooth philtrum - Thin upper lip Which condition is associated with a upslanting palpebral fissures? (a) Fetal alcohol syndrome (b) Trisomy 21 - (b) Trisomy 21 Which of the following factors is associated with an increased risk of neurologic damage in a jaundiced newborn? (a) Metabolic alkalosis (b) Increased attachment of bilirubin to binding sits caused by drugs such as sulfisoxazole (c) Hyperalbumniemia (d) Neonatal sepsis (e) Maternal ingestion of phenobarbital during pregnancy - (d) Neonatal sepsis Varicella-zoster immunoglobulin should be administered to the infant immediately after delivery if the mother had the onset of varicella within __ days prior to delivery, and immediately upon dx if her chicken pox started within __ days after delivery - 5; 2 If a patient was exposed to chicken pox 2 or more days postnatally, does she need to give varicella IG to the infant? - No What happens if a previously infected CMV mother has a reactivation during pregnancy? - IgG to CMV is passed to infant If a newborn exhibits hematemesis and melena, what test do you need to use to differentiate between maternal vs. fetal blood? - Apt-Downey test Which of the following are contraindicated in taking during breast feeding? (a) Ibuprofen (b) Labetalol (c) Amphetamines (d) Carbamazepine (e) Acyclovir - (c) Amphetamines What kind of electrolyte abnormality do newborns of diabetic mothers suffer from? (a) Hypernatremia (b) Hypocalcemia (c) Hypokalemia - (b) Hypocalcemia Small for dates infants have a higher risk of... - major congenital anomalies Apnea in a well premature infant is thought to be secondary to... - incompetely developed respiratory center The ____ is the most commonly fractured bone in the delivery process - clavicle ______ is bleeding in the potential space between the skull periosteum and the scalp galea aponeurosis. - Subgaleal hemorrhage What can cause neonatal seizures? (a) Hypocalcemia (b) Hypermagnesemia (c) Polycythemia - (c) Polycythemia What causes neonatal polycythemia? - Pregnancy-induced HTN In NRDS, what happens to lung compliance, lung volume, and which direction does the shunt occur? - - Decreased lung compliance - Decreased lung volume - R to L shunt What do you do after an HIV mother gives birth to a newborn? - Give newborn zidovudine The only vaccine routinely given in the newborn nursery is the ____ vaccine - Hepatitis B When is the first dose of TDaP vaccine given? - 2 months What two shots do you give newborns immediately after they are born? - - Hep B vax - Vitamin K What birth defects can happen with maternal use of propranolol? - - Growth retardation - Asphyxiated infant cant inc HR or CO - Hypoglycemia - Apnea Regular reticulocyte count - 1% Goat's milk, by itself, is not an ideal source of infant nutrition as it contains inadequate... - folate and iron In cleft palate, when should it be surgically closed? - Btwn 6 mo - 5 yrs What is the biggest complications for bilateral cleft lip and palate? - - Recurrent otitis media - Hearing loss - Speech deficits When do you see transient tachypnea of the newborn? - After scheduled cesarean delivery Why do you see transient tachypnea of the newborn? - Retained fetal lung fluid What do most infants with congenital infxns present with? - Maculopapular peeling rash on face, palm, soles What do you do for post-axial polydactyly without bone involvement? - Remove it surgially Idiopathic apnea is common in what kind of infants? (a) Full term (b) Premature - (b) Premature If apnea happens to full term babies, what should you search for? - - Sepsis - GERD - Congenital heart dz - Seizures - RSV - Hypoglycemia - Central hypoventilation - Airway obstruction What do you about a natal tooth found in a newborn? - Removal of tooth by pediatric dentist if loose A newborn infant becomes markedly jaundiced on the 2nd day of life, and a faint petechial eruption, first noted at birth, is now a generalized purpuric rash. Hematologic studies for hemolytic dzs are negative. Acute mgmt should include which of the following (a) Liver u/s (b) Isolation of infant from pregnant hospital personnel (c) Urine drug screen on infant (d) D/c w/ early f/u visit in 2 days to recheck bilirubin (e) Thyroid hormone assay - (b) Isolation of the infant from pregnant hospital personnel ~ patient has some kind of neonatal infxn ___ is the most common sort of congenital infxn - CMV A newborn has microcephaly, intracranial calcifications, hepatosplenomegaly, and marked hyperbilirubinemia and thrombocytopenia. (a) Congenital toxo (b) Congenital syphilis (c) Congenital CMV (d) Congenital rubella - (c) Congenital CMV How do you tx maple syrup urine dz? - Careful regulation of intake of leucine, isoleucine, and valine What disease? Lateral displacement of medial canthi, broad nasal bridge, medial hyperplasia of the eyebrows, partial albinism commonly expressed by a white forelock or heterochromia (or both), and deafness in 20% of cases. - Waardenburg syndrome A flat capillary vascular malformation in the distribution of the trigeminal nerve is the primary cutaneous lesion in what congenital disease? - Sturge-Weber syndrome Infants who have ____ are often born with hypopigmented oval or irregularly shaped skin macules (ash leaf spots). - tuberous sclerosis What is the most common finding in rheumatic fever? - Polyarthritis Children with a first or second degree relative with early onset of coronary heart disease should be evaluated early in life but after __ years of age - 2 Hereditary angioedema is transmitted ____ - autosomal dominant (T/F) Abdominal pain, vomiting, and diarrhea is not symptoms associated with hereditary angioedema. - False The risk of congenital heart dz in a family with one child born with heart dz ranges from ____ - 2-6% Prolonged fever, polymorphous rash, swelling of the legs, non-purulent conjunctivitis, LAN, fissured lips, and oropharyngeal mucosal erythema. What disease? (a) Rheumatic fever (b) Kawasaki dz (c) Juvenile rheumatoid arthritis - (b) Kawasaki dz What is the most likely cause of congestive heart failure in children? - Myocarditis due to adenovirus or coxsackie virus B Fetal SVT can lead to... - hydrops fetalis A quadruple rhythm associated with murmur of tricuspid regurgitation and a middiastolic murmur at the lower left sternum suggests the diagnosis of... - Ebstein anomaly Which arrhythmia frequently accompanies Ebstein malformation? - WPW syndrome What is Ebstein anomaly? - Downward displacement of the tricuspid valve Are there spiking high fevers and diffuse rash in rheumatic fever? - No Spindle-shaped swelling of finger joints/sternoclavicular joints with rheumatoid rash, and high spiking fevers. What is it? (a) Juvenile idiopathic arthritis (b) Rheumatic fever (c) Toxic synovitis (d) Septic arthritis (e) Osteoarthritis - (a) Juvenile idiopathic arthritis Patients with tricuspid atresia typically have a ____ right ventricle (a) hyperplastic (b) hypoplastic - (b) hypoplastic What does the EKG show on someone with tricuspid atresia? (a) Right axis deviation (b) Left axis deviation - (b) Left axis deviation What is truncus arteriosus? - A single blood vessel comes out of the right and left ventricles, instead of the normal two vessels (pulmonary artery and aorta). Transposition of the great vessels shows what on ekg? (a) Left axis deviation (b) Right axis deviation (c) Left ventricular hypertrophy (d) Right ventricular hypertrophy - (b) Right axis deviation (d) Right ventricular hypertrophy A newborn that has profound thrombocytopenia, bilateral absence of radius, and abnormally shaped thumbs concurrent with TOF and ASD. What does this patient have? - Thrombocytopenia Absent Radius syndrome What congenital heart anomaly do males with Turner syndrome often have? - Pulmonic stenosis Short stature, downslanting palpebral fissures, ptosis, low set and malformed ears, webbed neck, shieldlike chest, pulmonic stenosis, and cryptorchidism. What does the patient have? - Noonan syndrome or male Turner syndrome Infants born to mothers with gestational diabetes are at risk for being large for their gestational age and thus at increased risk for peripheral nerve injuries such as Erb-Duchenne and ______ - phrenic nerve paralysis If an infant has something that looks like asthma, also think of... - bronchiolitis What is the first line tx for bronchiolitis? - - Monitor oxygenation - Hydration What is the initial mgmt of gasoline poisoning? - - Pulse oximetry - Arterial blood gas What is the initial workup on an infant with phrenic nerve paralysis? - Chest ultrasound ____ stridor is typical in croup (a) Inspiratory (b) Expiratory (c) Biphasic - Biphasic Biphasic stridor. Several days of viral upper respiratory symptoms, followed by acute elevation of temp and increase in respiratory distress. Consider... - Bacterial tracheitis What is the mgmt of bacterial tracheitis? - - Intubation - IV abx What is the initial mgmt of epiglottis? - - Intubation What are all the likely bugs that cause epiglottis when someone who has been vaccinated w/ HiB? - - GAS - Moraxella catarrhalis - Strep Pneumo When eats dirt and get sick consider... - ELISA for Toxocara A cold lasting longer than 10 to 14 days with fever and facial pain is suggestive of ____ - rhinosinusitis In the ER, what can you use to treat croup? - - Epinephrine - Corticosteroids What sign in anaphylaxis suggests impending airway compromise? - Change in voice If there is impending airway compromise in anaphylaxis, what is your initial management plan? - Intubation Where do foreign bodies lodge during esophageal obstruction in children? - Below cricopharyngeal muscle at level of the aortic arch (T/F) Kids do not typically want to eat if they have esophageal obstruction - True Being on too many diuretics can cause metabolic (a) Acidosis (b) Alkalosis - (b) Alkalosis A common cause of chronic hypoventilation in children is... - hypertrophied tonsils and adenoids If PCO2 goes up, PO2 has to go... - down If PO2 goes up, PCO2 has to go... - down Tension pneumothorax causes respiratory (a) Acidosis (b) Alkalosis - (b) Acidosis Strictures is more common in (a) Crohns (b) UC - (a) Crohns Finding a dilated, stool-filled anal canal with poor tone on physical exam of a well grown child supports the diagnosis of _____ - functional constipation Nocturnal abdominal pain and GI bleeding in a pt w/ positive fam hx supports a diagnosis of... - Peptic Ulcer Disease (PUD) Mgmt of rumination - Behavioral therapy _____ and _____ should be considered in the diagnostic eval of any child with obstructive jaundice - CF and alpha1-antitrypsin Choledochal cysts usually produce excess ____ bilirubin (a) Direct (b) Indirect - (a) Direct What exactly are choledochal cysts? - Cystic dilatation of bile ducts An awake, alert infant with a 2-day history of diarrhea presents with a depressed fontanelle, tachycardia, sunken eyes, and the loss of skin elasticity. Which of the following is the correct percentage of dehydration? (a) < 1% (b) 1%-5% (c) 5%-9% (d) 10%-15% (e) > 20% - (c) 5%-9% A moribound state is characteristic of a loss of > ____% of body weight from dehydration - 10% Does activated charcoal absorb lithium? - No Typical dose of activated charcoal - 1 g/kg How do you tx a phenobarbital overdose? - Activated charcoal What is the initial diagnostic test of choice for necrotizing enterocolitis? - Plain film radiographs Can infants get mallory-weiss syndrome? - Yes Initial diagnostic procedures for mallory-weiss syndrome - Nothing Can vitamin C deficiency cause a low grade fever? - Yes What is the ddx of an infant who gags and chokes while feeding? - - GERD - H-type tracheoesophageal fistula What is the initial diagnostic procedure of an infant who gags and chokes while feeding? - Modified barium swallow with fluoroscopy What is Sandifer syndrome? - A type of GERD where infants arch and become tonic to protect airway from refluxing gastric contents What is the initial diagnostic procedure for Sandifer syndrome? - Esophageal pH monitoring Why do labial adhesions happen in infants? - Low estrogen Mgmt of labial adhesions in infants - Nightly application of estrogen cream for a week A 2 week baby with grayish and sometimes bloody vaginal d/c since birth. No sexual abuse is suspected. What is it? - Physiologic discharge related to withdrawal of estrogen. Pain that radiates from the abdomen to the testis is most likely... - a kidney stone Pain that radiates from the testis to the abdomen is most likely... - testicular torsion Orchiopexy for cryptorchidism eliminates what risk for these infants? - Testicular torsion Does orchiopexy eliminate the risk of testicular malignancy for cryptorchidism? - No An infant that has an enlarged kidney w/ hx of oliguria and hematuria and polycythemia. What does he have? - Renal vein thrombosis What is the initial diagnostic procedure for renal vein thrombosis? - Renal vein doppler studies If you suspect testicular torsion, what should you do immediately? (a) Consult surgery (b) Prepare for U/S - (a) Consult surgery In infant, vaginal discharge that is brown/green and has fetid odor with no hx of sexual abuse is usually... - vulvovaginitis What are two very common ways of getting vulvovaginitis? - - Wearing tight clothing - Soap in vagina from bubble baths IgA-mediated vasculitis aka... - henoch schonlein purpura Rash on buttocks, arthralgia, edema, colicky abdominal pain with GI bleeding, scrotal pain, microscopic hematuria. Think... - henoch scholein purpura CNS sx of henoch scholein purpurua - Headache Initial mgmt of a varicocele - Reassurance and education If an infant gets a hydrocele, what is the initial mgmt? - Observation The most common type of hereditary nephritis is... - Alport Syndrome While alport syndrome affects the kidneys and causes ESRD, what other part of the body should you check in these patients? - Eyes & Ears What is the most common symptom of infantile botulism? - Constipation What is the diagnostic test for infantile botulism? - Stool toxin test How much formula milk should an infant be getting a day? - 2-3x the weight in ozs How is craniosynostosis managed? - Surgery Craniosynostosis is associated with... - genetic defects Seizure-like activity with no epileptiform activity on EEG is consistent w/... - pseudoseizure How is pseudoseizures managed? - psychiatry Should you take people w/ pseudoseizures off of seizure medication? - No A hx of ___ usually precedes the signs of an acute subdural hematoma - trauma The abrupt onset of a hemisyndrome, especially with the eyes looking away from the paralyzed side, strongly indicates a diagnosis of... - acute infantile hemiplegia What eye problems can pts with myotonic dystrophy get? - Cataracts What is the main sx of myotonic dystrophy? - Psychomotor retardation Which CNS infxn causes the lowest glucose levels in CSF? (a) Viral meningitis (b) Tuberculous meningitis (c) Meningeal leukemia (d) Medulloblastoma - (b) Tuberculous meningitis In CT, exudates in basal cisterns that shows enhancement by contrast material is typical. What is the dz? - Tuberculous meningitis Subacute disorder of CNS that produces cranial nerve abnormalities (CN7 & lower bulbar nerves), long-tract signs, unsteady gait secondary to spasticity, and some behavioral changes. What is it? - Pontine glioma (T/F) Craniopharyngioma limits growth in kids - True Supratentorial tumors are most common in what age range (a) 0-1 yo (b) 1-10 yo (c) 10+ yo - (a) 0-1 yo (c) 10+ yo Subtentorial tumors are most common in what age range (a) 0-1 yo (b) 1-10 yo (c) 10+ yo - (b) 1-10 yo Cherry red spots are often caused by the lack of which enzyme? (a) Reduced serum hexosaminidase A activity (b) Deficient activity of acid B-galactosidase (c) Defective gene on the X chromosome (d) Complete lack of acid B-galactosidase activity (e) Deficient activity of galactosyl-3-sulfate-ceramide sulfatase (cerebroside sulfatase) - (d) Complete lack of acid B-galactosidase activity Cherry-red spots in the eye, what metabolic disorder? - Niemann-Pick Dz Age range for febrile seizures - 6 mo to 6 yrs Normal CSF white cell count - 0-5 cells/uL Periventricular calcifications are a characteristic finding in infants who have... - congenital CMV infxn Pattern of calcifications of CMV vs. Toxoplasmosis congenital infxns - CMV- Ventricular ToXoplasmosis- all over the corteX What is not associated with migraines in childhood? - Scotomas The condition is caused by the degeneration of anterior horn cells in the spinal cord which eventually kills the infant - Spinal motor atrophy How do you tx vasovagal syncope? - Counseling on diet and fluid intake Throat clearing in kids is more likely (a) Transient tic disorder (b) Tourette syndrome - (a) Transient tic disorder Emotionally labile, hypotonic, and milkmaid grip (tightening and relaxing of hands), what is it? - Sydenham chorea A child with an unilateral facial lesion and blindness suggests what two disorders? - - Sturge-Webber - PHACE syndrome PHACE syndrome, what does it stand for? - - Posterior fossa malformations - Hemangiomas - Arterial anomalies - Coarctation of aorta - Eye abnormalities A 6-month child with blindness on the same side as a large lesion (a) NF1 (b) PHACE syndrome (c) Tuberous sclerosis - (b) PHACE syndrome What disease shares many characteristics with scarlet fever? - Kawasaki dz The most serious complication of kawasaki dz and scarlet fever is ____ - cardiac involvement What is kerion? - A severe form of tinea capitis that causes boggy scalp and enlarged occipital LNs ____ is a deficiency of all 3 major classes of immunoglobulins - X-linked agammaglobulinemia ____ has diminished serum IgM, marked elevation of IgA and IgE, eczema, recurrent middle-ear infxns, lymphopenia, and thrombocytopenia. - Wiskott-Aldrich syndrome ____ have deficient T and B cells that can have agammaglobulinemia, chronic diarrhea, rashes, recurrent bacterial, fungal, and viral ifnxns - Severe Combined Immunodeficiency Disease A rash after taking ampicillin, with headache, abdominal pain, myalgia, and splenic enlargement. What is this? - EBV When someone you suspect has endocarditis, what should you do? - Echocardiogram How do you treat someone who is exposed to pertussis? - Erythromycin Acute osteomyelitis can follow an episode of... - deep cellulitis or septic joint How long does it take osteomyelitis to show up on the bone scan? - 24 to 48 hrs Early onset GBS is seen in the first ___ days of life - 7 In an infant who appears otherwise normal, the sudden onset of high fever, together with a marked elevation and shift to the left of the WBC ct, suggests... - pneumococcal bacteremia If someone has a negative titer, that means... - they are not immune to whatever the pathogen of interest is Sxs of ____, usually a mild dz, include a diffuse maculopapular rash that lasts for 3 days, marked enlargement of the posterior cervical and occipital LNs, low-grade fever, mild sore throat, and, occasionally, conunctivitis, arthralgia, or arthritis. (a) Rubeola (b) Rubella (c) Roseola - (b) Rubella Can meningitis happen in a mumps infection? - Yes Why do patients with wiskott-aldrich syndrome have bloody diarrhea? - Thrombocytopenia (T/F) ITP patients have a susceptibility to infxn. - False _____ has been recognized as an agent responsible for epidemics of diarrhea in day-care centers. (a) Salmonella sonnei (b) Enterobius vermicularis (c) Sporothrix schenkii (d) Toxoplasma gondii (e) Cryptosporidium - (e) Cryptosporidium Cryptosporidium diarrhea can be described as... - watery, pale, frothy ____ is a common acute illness of young children, characterized by several days of high fever followed by a rapid defervescence and the appearance of an evanescent, erythematous, maculopapular rash. - Roseola (HHV-6) What culture do you use to diagnose gonorrhea? - Modified Thayer-Martin How do you diagnose malaria? - Peripheral smear A pneumonia that has gradual onset of headache, malaise, fever, and lower respiratory sxs. Dry cough for first 2 weeks and slowly resolves wks 3-4. - Mycoplasma pneumoniae What disease has a vascular tumor that leads to thrombocytopenia, coagulopathy, and MAHA? - Kasabach-Merritt phenomenon An 11-year-old child arrives to your hospital w/ a hx of recurrent episodes of oral ulcers, fever, pharyngitis, and LAN. These fever episodes, which also have been noted in other family members, occur about every 21 days and some episodes have been associated with pnx and sepsis. What is this? - Cyclical neutropenia How do you tx cyclical neutropenia? - Recombinant human granulocyte colony-stimulating factor (rhG-CSF) Passage of fetal blood into the maternal circulation through a defect in the placental membrane. - Fetomaternal transfusion If a patient has sickle cell and is stroking out, what is the first step in mgmt? - Blood transfusion Clinical and laboratory evidence of marrow failure with anemia and thrombocytopenia. What might be this? - Leukemia Does EBV have anemia and thrombocytopenia? - No How do you treat Von Willebrand's disease? - Desmopressin (DDAVP) Which hereditary blood disorder present in newborn period with anemia, hyperbilirubinemia, and reticulocytosis? (a) vW disease (b) Hemophilia A (c) Hereditary spherocytosis (d) Sickle cell disease - (c) Hereditary spherocytosis What kind of RBCs do you see in hemoglobin C disease? - Target cells Which hereditary metabolism d/o? XR shows erlenmeyer-flask appearance of long bones. - Gaucher's dz The diagnosis of Gaucher's disease is confirmed by absence of _____ activity in leukocytes, in cultured skin fibroblasts, and in liver cells. (a) Sphingomyelinase (b) Hexoaminidase A (c) Glucocerebrosidase - (c) Glucocerebrosidase Deficiency of ____ causes type A Niemann-Pick disease (a) Sphingomyelinase (b) Hexoaminidase A (c) Glucocerebrosidase - (a) Sphingomyelinase In true precocious puberty, how do you manage? - Reassurance Puberty is said to be delayed in males if physical changes are not apparent by __ years of age. - 14 Evidence of pubertal development: penis length > ___ cm, and testicular vol > ___cm^3 - 2.5; 3.0 ____ is the most common of several syndromes that are characterized by both deafness and pigmentary changes - Waardenburg syndrome Waardenburg syndrome features - - Deafness - White furlock - Heterochromia irides (blue eyes) Most common cause of juvenile hypothyroidism - Lymphocytic thyroidits Although the administration of exogenous adrenocorticotropic hormone or of glucocorticoids is the most common cause of Cushing syndrome, it can also be caused by... - bilateral adrenal hyperplasia Male Turner syndrome aka... - Noonan syndrome Child is short for his age, downslanting palpebral fissures, ptosis, low-set and malformed ears, a broad and webbed neck, shield chest, and cryptorchidism. What does he have? - Noonan syndrome (T/F) Infants with galactosemia have an increased risk for E. coli sepsis - True How do you treat galactosemia? - Lactose free milk A newborn has constipation, prolonged jaundice, sluggishness, poor feeding, apnea, choking, macroglossia, and excessive sleepiness. What does he have? - Congenital hypothyroidism Patients born normal who then have progressive developmental delay and hepatomegaly with coarse facial features are likely to have... - Hurler's dz When bone age and height age are equally retarded several years behind chronologic age, a child is described as having _____ short stature - constitutional The first step in the mgmt of a pediatric pt w/ a solitary thyroid nodule is... - measurement of thyorid fxn Patients with pseudohypoparathyroidism have ___ calcium and __ phosphorus (What levels?) - Low calcium and High phosphorus Patients with pseudohypoparathyroidism have ___ PTH - high (T/F) Patients with pseudohypoparathyroidsm have perivascular calcifications of the basal ganglia - True HbA1c reflects glucose control for what range of time? - 2-3 mos (T/F) 21-hydroxylase deficiency is a salt-losing congenital adrenal hyperplasia. - True In classic 21-hydroxylase deficiency, serum levels of ______ are markedly elevated beyond 3 days of life - 17-hydroxyprogesterone Hypoglycemia with hyperinsulinemia but w/o evidence of C-peptide suggests that the insulin is... - exogenous Macroglossia, macrosomia, midline abdominal wall defects, ear creases, neonatal hypoglycemia, hepatoblastoma. Associated with hepatoblastoma and wilm's tumor. - Beckwith-wiedemann syndrome Vitamin D deficiency initially causes ___calciuria (a) Hyper (b) Hypo - (b) Hypo Most common cause of hypoglycemia in neborns - Inadequate nutrition What is vitamin D-resistant rickets? - Genetic abnormality in the renal tubular reabsorption of phosphate with hyperphosphaturia and hypophosphatemia and also in conversion of 25 vit D to 1,25 vit D What is the serum calcium in vitamin D-resistant rickets? - Normal What is the serum calcium and phosphate concentrations in osteogenesis imperfecta? - Normal What is the serum calcium and phosphate concentrations in medullary carcinoma? - Normal What is the serum phosphate in vitamin D-resistant rickets? - Hypophosphatemia ____ syndrome is a disorder consisting of hypotnia, hypogonadism, hyperphagia, after the newborn period, hypomentia, and obesity. - Prader-willi Obesity, mental retardation, hypogonadism, polydactyly, and retinitis pigmentosa with night blindness are the principal findings in affected children. - Laurence-Moon-Biedl syndrome (T/F) Persons with compensated Addison disease can hae relatively normal physical and laboratory findings. - True Patients with a deficiency of ______ in von gierke disease are hyperlipidemic. Increased triglyceride concentration in serum decreases vol of aqueous compartment. - glucose-6-phosphatase People with Von Gierke disease can have ___ sodium and ___ potassium but this will be factitious - low; low ______ syndrome is characterized by abnormalities in the upper extremities, hypoplastic radii, thumb abnormalities, and cardiac anomalies. - Holt-Oram (T/F) Trisomy 18 babies can have horseshoe kidneys - True A small newborn w/ a large VSD, clenched hands, cleft palate, rounded heels, and a horseshoe kidney (a) Trisomy 21 (b) Trisomy 18 (c) Holt-Oram syndrome (d) Fetal alcohol syndrome - (b) Trisomy 18 A 6-year-old girl with cognitive delay, a blowing systolic heart murmur, short stature, round face, bilateral transpalmar crease, upslanting palpebral fissures, small ears, and epicanthal folds (a) Trisomy 21 (b) Trisomy 18 (c) Holt-Oram syndrome (d) Fetal alcohol syndrome - (a) Trisomy 21 A 2-year-old boy, less than the 5% for weight and height, is in early childhood intervention for developmental delay. He has a short nose, thin upper lip with thin vermillion border, a ventricular septal defect, and short palpebral fissures. (a) Trisomy 21 (b) Trisomy 18 (c) Holt-Oram syndrome (d) Fetal alcohol syndrome - (d) Fetal alcohol syndrome Young mothers have a higher risk of several pregnancy-related complications, including which of the following? (a) Twin gestation (b) Low-birth weight infants (c) Hypotension (d) Excessive weight gain (e) Infants with genetic defects - (b) Low-birth weight infants The first sign of puberty in boys is... - scrotal and testicular growth Sparse, thin, and long pubic hair with slight penile enlargement (a) Tanner 1 (b) Tanner 2 *******************************CONTINUED***********************