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1025 Cards in this Set

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Q001. vaccinations given at birth
A001. hepatitis B
Q002. vaccinations given at 1 month
A002. hep B, only if not given at birth
Q003. vaccinations given at 2 months
A003. Pediarix (HBV, DTaP, IPV); Prevnar (pneumococc); Rotavirus; Hib
Q004. what is in pediarix
A004. HBV; DTaP; IPV
Q005. vaccinations given at 4 months
A005. Pediarix; Prevnar; Hib; Rotavirus
Q006. vaccinations given at 6 months
A006. Pediarix; Prevnar; Hib; Rotavirus
Q007. vaccinations given at 12 months
A007. DTaP; Hib; MMR; VZV; Prevnar; Influenza; Hep A
Q008. vaccinations given at 24 months
A008. none
Q009. vaccinations given at 4-6 yrs
A009. DTaP; IPV; MMR; Influenza
Q010. vaccinations given at 11 yo
A010. Tdap; Meningococcus vaccine
Q011. what is the schedule for well visits starting from birth
A011. 1 mo; 2 mo; 4 mo; 6 mo; 9 mo; 12 mo; 15 mo; 18 mo; 24 mo; then annually
Q012. why shouldn't babies drink water
A012. because their kidneys aren't mature enough to handle the extra fluid so the babies will become hyponatremic since they can't excrete the water, and then they can have seizures
Q013. plagylocephaly
A013. mishapen head
Q014. sunsetting sign
A014. increased ICP, eyes are half closed secondary to the increased ICP on the cranial nerves
Q015. craniosynostosis
A015. premature closure of the fontanelles
Q016. rash pattern in rocky mountain spotted fever
A016. palms and soles, then spreads to trunk; petechial
Q017. rash pattern in rubella
A017. rash on face that spreads to the rest of the body
Q018. rash pattern in measles
A018. rash starts at the head and spreads downwards and disappears in the same manner
Q019. rash pattern for erythema infectiosum
A019. slapped cheek rash;; lacy, reticular
Q020. VZV rash pattern
A020. begins on trunk,; followed by head, face, and extremities
Q021. gross motor skills at 1 mo old
A021. raises head
Q022. gross motor skills at 3 mo
A022. holds head up
Q023. gross motor skills at 4-5 mo
A023. rolls front to back and back/front; sits supported
Q024. gross motor skills at 6 mo
A024. sits unsupported
Q025. gross motor skills at 9 mo
A025. crawls; cruises; pulls to stand
Q026. gross motor skills at 12 mo
A026. walks alone
Q027. gross motor skills at 15 mo
A027. walks backwards
Q028. gross motor skills at 18 mo
A028. runs
Q029. gross motor skills at 24 mo
A029. walks well up and down stairs
Q030. gross motor skills at 3 yrs
A030. rides tricycle; throws ball overhand
Q031. gross motor skills at 4 yo
A031. alternates feet going down stairs; skips
Q032. fine motor skills at 1 mo
A032. follows eyes to midline
Q033. fine motor skills at 3 mo
A033. hands open at rest
Q034. fine motor skills at 4-5 mo
A034. grasps with both hands together
Q035. fine motor skills at 6 mo
A035. transfers hand to hand, reaches with either hand
Q036. fine motor skills at 9 mo
A036. pincher grasp; finger feeds
Q037. fine motor skills at 12 mo
A037. throws, releases objects
Q038. fine motor skills at 15 mo
A038. builds 2 block tower
Q039. fine motor skills at 18 mo
A039. feeds self with utensils
Q040. fine motor skills at 24 mo
A040. removes clothing; builds 5 block tower
Q041. fine motor skills at 3 yrs
A041. draws circle
Q042. fine motor skills at 4 yrs
A042. catches ball; dresses alone
Q043. fine motor skills at 5 yrs
A043. ties shoes
Q044. Simple febrile seizure
A044. between 6mo - 6yr; tonic clonic; associated with fever >100.4; seizure lasts <15 mins; only 1 seizure in 24 hrs; minimal post-ictal state
Q045. complex febrile seizure
A045. 6 mo - 6 yrs; focal seizure; >15 mins; >1 seizure/24 hrs
Q046. management of simple febrile seizure
A046. determine the source of the fever, otherwise, no other w/u is needed
Q047. management of complex febrile seizure
A047. full w/u should be done, but no anti-epileptics, no eeg needed; if patient is <18 mo, LP
Q048. eeg abnormality associated with infantile spasm
A048. hypsarrythmia
Q049. definition of recurrent abdominal pain
A049. >3x in 3 mo
Q050. #1 cause of abdominal pain
A050. gastroenteritis
Q051. mesenteric lymphadenitits
A051. persistent pain following an infection
Q052. clinical features of HENOCH-SCHONLEIN PURPURA
A052. condition preceded by uri; non-thrombocytopenic palpable purpura; hematuria (good prognosis); proteinuria (poor prognosis); spasmodic abdominal pain; ileus, n/v; ugi/lgi bleed
Q053. tx of Henoch-Schonlein Purpura
A053. steroids
Q054. complication of Henoch-Schonlein Purpura
A054. intussusception; kidney probs
Q055. clinical features of kawasaki
A055. CRASH and BURN; Conjunctivitis; Rash (on trunk mostly); Aneurysm (coronary); Skin peels off, Strawberry tongue; Hands/Feet edema; BURN = FEVER (x 5d)
Q056. phases of kawasaki disease
A056. acute; subacute (aneurysm formation); convaslescent; takes 2-3 months to resolve
Q057. tx of kawasaki
A057. ASA; IVIG
Q058. when is colicky pain associated with
A058. constipation
Q059. what type of stool is seen with bacterial enterocolitis
A059. bloody, mucinous stool
Q060. when will an appendix perforate in appendicitis
A060. w/i 36 hrs
Q061. which infection can clinically mimic appendicitis
A061. yersinia; campylobacter
Q062. what imaging study for appendicitis
A062. ct
Q063. meds used to tx of perforated appendix
A063. amp, gent, flagyl
Q064. clinical presentation of intussusception
A064. currant jelly stools; lethargy; palpable tubular mass; paucity of gas on xr or evidence of obstruction
Q065. imaging most specific for intussusception
A065. barium (or air) enema; is also therapeutic
Q066. most common location for intussusception
A066. ileocolic; can also develop at meckel's divertic
Q067. major complication of intussusception
A067. there is impaired venous return so bowel edema develops -- > ischemia, necrosis --> perforation
Q068. etiology of intussusception
A068. ileum invaginates into colon at ileocecal valve; a previous viral infection --> hypertrophy of the peyer's patches... this can develop into a lead point; Henoch-Schonlein Purpura can be association with an ileal- ileal intussusception
Q069. tx of intussusception
A069. must do fluid resusc first, if needed; hydrostatic reduction with air/barium
Q070. recurrence rate of intussusception
A070. 0.15
Q071. at what age would a patient present with pyloric stenosis
A071. 1-3 mo
Q072. which medicaation can be associated with pyloric stenosis
A072. erythromycin
Q073. best imaging for pyloric stenosis
A073. u/s; will also see a string sign ugi study
Q074. tx of pyloric stenoSIS
A074. MUST correct fluids and lytes first!; then pyloromyotomy
Q075. describe malrotation
A075. small intestines rotate abnormally in utero, so there is an abnormal fixation posteriorly to the mesentary; it can twist on its vascular supply --> volvulus
Q076. clinical presentation of malrotation
A076. bilious emesis; possibly abdominal distention/shock; + guiac test = bowel ischemia, poor prognostic sign
Q077. tx of malrotation
A077. surgery ASAP
Q078. tx for scd induced priapism
A078. sedation
Q079. disease that scd can mimic if htere is abdominal pain
A079. appendecitis
Q080. when is frontal bossing seen?
A080. beta-thal or some other hemolytic process that requires rapid hematopoesis
Q081. tx for beta-thal
A081. serial transfusion + chelation therapy (desferoxamine) b/c of fe overload
Q082. consequence of fe overload
A082. hemochromatosis
Q083. complications of g6pd deficiency
A083. rbcs are destroyed but there is increased amounts of hb liberated in the process --> hb-uria
Q084. findings in classic hemophilia
A084. bleeding problems + hemarthrosis
Q085. battle's sign
A085. basilar skull fracture that leads to bleeding/bruise behind the ear
Q086. mechanism behind bell's palsy in neonates
A086. forceps deliver... usually resovles
Q087. social milestones at 5 yo
A087. competetive games; understands rules and abides by them
Q088. social milestones at 4 yo
A088. imaginative play
Q089. social milestones at 3 yo
A089. group play; shares
Q090. social milestones at 2 yo
A090. parallel play
Q091. social milestones at 18 mo
A091. plays around other children
Q092. social milestones at 12 mo
A092. comes when called; cooperates with dressing
Q093. social milestones at 9 mo
A093. pat-a-cake
Q094. social milestones at 6 mo
A094. recognizes strangers
Q095. social milestones at 4-5 mo
A095. enjoys observing environment
Q096. social milestones at 3 mo
A096. reaches for familiar objects/ppl
Q097. social milestones at 2 mo
A097. recognizes parent
Q098. social milestones at 1 mo
A098. fixes on face
Q099. developmental dysplasia of the hip
A099. abnormal relationship between head of femur and acetabulum --> instabilility and dislocation of hip joint; develops secondary to lack of contact of acetabulum and femur during intrauterine development
Q100. dx of developmental dysplasia of hip
A100. u/s: see "false acetabulum" in lateral ileum
Q101. tx of developmental dysplasia of hip
A101. pavick harness (keeps hip abducted and flexed), or body casting on older pts
Q102. complications of developmental dysplasia of hip
A102. Avascular necrosis; degenerative arthritis of hip
Q103. metatarsus adductus
A103. dorsiflexion and plantarflexion are UNRESTRICTED (diff from clubfoot);; heels go out and toes go in
Q104. tx of metatarsus adductus
A104. stretching or a brace; surgery not usualy needed
Q105. idiopathic talipes equinovarus (aka); appearance
A105. congenital clubfoot; medial rotation of tibia, fixed plantar flexion, inversion of foot, forefoot adduction; CANNOT DORSIFLEX (unlike metatarsus adductus)
Q106. tx of clubfoot
A106. bracing; serial casting
Q107. legg-calve-perthes disease
A107. avascular necrosis of femoral head; ischemic bone is eventually resolved and re-ossification occurs; --> limp, pain referred to thigh/knee
Q108. what movements are restricted in legg-calve-perthes disease
A108. abduction; flexion; internal rotation
Q109. tx of legg-calve-perthes disease
A109. bracing; surgery; observation
Q110. complications of legg-calve-perthes disease
A110. collapse of femoral head
Q111. SLIPPED CAPITAL FEMORAL EPIPHYSIS
A111. gradual or acute separation of proximal femoral growth plate; fem head slipps off of femoral neck and rotates inf-post postition
Q112. etiology of slipped capital femoral epiphysis
A112. common during puberty, could be hormonal
Q113. presentation of slipped capital femoral epiphysis
A113. limp, pain in hip and groin, pain referred to knee
Q114. dx of slipped capital femoral epiphysis
A114. plain film frog-leg, lateral position
Q115. tx of slipped capital femoral epiphysis
A115. goal is to prevent further misalignment; pin fixation is done acutely; chronic cases require osteotomy
Q116. Osgood Schlatter disease
A116. inflammation, swelling and tenderness over tibial tuberosity secondary to tendonitis of distal insertion of infrapatellar tendon
Q117. when does Osgood Schlatter disease occur
A117. During growth spurt, in teens
Q118. tx of Osgood schlatter
A118. conservative, supportive management
Q119. complication related to achondrodysplasia
A119. small foramen magnum seen in homozygotes --> brainstem compression
Q120. what is achondrodysplasia
A120. disorder of cartilage calcifications and remodeling
Q121. why do children have an increased risk of fracture
A121. tendons and ligaments are stronger than bones so in kids injuries often lead to fracture when they would only cause sprain in adult
Q122. types of fracture
A122. spiral (twisting forces on tibia during fall); epiphyseal fracture (use salter classification); stress fracture (hairline crack from repeated activity); torus fracture (at metaphysis)
Q123. nursemaids elbow
A123. subluxation of radial head
Q124. define upper airway
A124. nose --> carina
Q125. sx of upper airway disease
A125. inspiratory stridor; tachypnea; respiratory distress
Q126. choanal atresia
A126. most proximal abnormality of airway; bony or membranous septum between 1 or both nasal passages and pharynx, preventing airflow through nose; life threatening if b/l (most young infants are obligate nose breathers); can't pass NG tube
Q127. complications of long-term intubation
A127. subglottal stenosis
Q128. laryngeal or tracheomalacia
A128. floppiness that closes off airway
Q129. how to confirm dx of laryngeal or tracheomalacia
A129. bronchoscopy
Q130. ddx for wheezing and respiratory distress
A130. asthma; bronchiolitis; foreign body aspiration; gerd; TE fistula; vascular sling
Q131. pathophysiology of sx in CF
A131. CFTR is abnormal --> altered cl channel; cl stays in cells and Na/water enter the cell to maintain osmotic balance --> viscous secretions
Q132. GI effects in CF
A132. pancreatic insufficiency; bowel obstruction; rectal prolapse; DM; cirrhosis; large bulky smelly stools; later in life stools --> distal obstruction
Q133. Pathognomonic finding in CF
A133. meconium ileus
Q134. Tx of CF
A134. chest physiotherapy; exercise; frequent cough; recombinant human dnase given through nebs to break down thick mucous complexes
Q135. other than respiratory infection, what are some other complications of CF
A135. hemoptysis (>500 cc/d = emergency) tx with embolization; spontaneous ptx (1/2 will recur unless sclerosis is performed, but if that is done then transplant is very difficult)
Q136. mesenteric lymphadenitis leads to
A136. persistent pain after infection
Q137. GI complication from Henoch-Schonlein Purpura
A137. ileal-ileal intussusception
Q138. Sx of Henoch-Schonlein Purpura
A138. non thrombocytopenic palpable purpura in dependent areas; an IgA mediated vasculitis involving GI, skin, joints, and kidneys
Q139. what is Henoch-Schonlein Purpura usually preceded by
A139. URI
Q140. GI sx of Henoch-Schonlein Purpura
A140. spasmodic pain, ileus, vomiting, ugi/lgi bleed
Q141. Tx of Kawasaki disease
A141. iv Ig; ASA
Q142. 3 phases of kawasaki
A142. acute; subacute (aneurysms form); convalescent (resolution over 2-3 mo)
Q143. which bacterial infection can mimic appendicitis
A143. campylobacter; Yesinia
Q144. sx of intussusception
A144. BOWEL OBSTRUCTION; currant jelly stools; lethargy; palpable tubular mass; paucity of gas on XR or evidence of obstruction
Q145. dx of intussusception
A145. barium (or air) enema
Q146. progression of complications of intussusception
A146. impaired venous return; bowel edema; ischemia; necrosis; perforation
Q147. pathophys of intussusception
A147. most are ileocecal, and occur when the ileum invaginates into colon at ileocecal valve
Q148. lead points in intussusception
A148. hypertrophy in Peyer's patches (often p viral infection); Meckel's divertic; intestinal polyp; lymphoma; foreign body; Henoch-Schonlein Purpura
Q149. at what age would you expect pyloric stenosis
A149. 1-3 months
Q150. what medication if given is associated with pyloric stenosis
A150. erythromycin
Q151. dx of pyloric stenosis
A151. u/s, see hypertrophic pyloris; UGI study shows string sign
Q152. embryology behind omphalocele
A152. when midgut loop fails to return to abdominal cavity; see light gray shiny sac protruding from base of umbilical cord
Q153. embryology behind malrotation of midgut
A153. midgut undergoes partial rotation and --> abnormal position of abdominal viscera, can be assoc with volvulus --> compromised blood flow and gangrene
Q154. gastroschisis
A154. weakness in abdominal wall --> herniation of bowel through the rectus muscle, usually to the right of the umbilicus
Q155. clinical features of malrotation
A155. bilious emesis; possible abdominal distension; XR shows gas in stomach, but no gas in intestines
Q156. risk factors for developing GERD in babies
A156. prematurity; esophageal disease; obstructive lung disease; overdistension of stomach from overeating; meds (theophylline)
Q157. dx of GERD
A157. pH probe placement in esophagus or ugi endoscopy; barium swallow to confirm normal anatomy
Q158. tx of GER/GERD
A158. small frequent feedings; keep head up for 20mins p eating; thicken feeds with cereal; metoclopramide (increases gastric motility); h2 blocker or PPI; last resort: nissen fundlopication (fundus of stomach is wrapped around distal esophagus to increase les pressure)
Q159. tx of diarrhea in children
A159. if no serious complications, feed through the diarrhea --> decreased denudement and faster return to normal stooling patterns; don't give antidiarrheals b/c --> toxic megacolon, unless salmonella, Shigella, c diff, or parasites
Q160. when to do w/u for diarrhea in a child
A160. in infant <3mo, do blood culture; 0-12 mo, do stool culture; do blood and stool culture if >5d of enterocolitis or salmonella exposure; any infant with + stool dx looking toxic or + blood culture should be evaluated for pyelo, meningiits, PNEUMONIA, osteo
Q161. when should a patient with diarrhea be admitted to hospital
A161. >5% dehydration and can't rehydrate effectively at home
Q162. constipations and complications
A162. no bowel movement, if after neonatal pd, #1 cause is voluntary withholding; can be caused by pain, on defecation --> fear of defecation - -> further retention; voluntary holding increases distension of rectum, decreases rectal sensation, requiring increased amounts needed to receive urge
Q163. sx of Hirchsprung’s
A163. h/o diarrhea, fecal spotting alternating with constipation
Q164. how to tx functional constipation
A164. increase fluid,; decrease junk food,; increase fiber,; increase ingestion of undiluted juice
Q165. pathophys of Hirchsprung’s
A165. failure of ganglion cells of myenteric plexus to migrate down colon in utero; therefore distal colon is tonically contracted and there is obstruction; usually limited to rectosigmoid colon
Q166. when to suspect Hirchsprung’s
A166. in any infant who doesn't pass meconium, then --> bilious vomiting, abdominal distension, and poor feeding
Q167. tx of Hirchsprung’s
A167. diverting colostomy with bowel that contains ganglion cells; aganglionic segment is removed by pulling ganglionic segment through rectum
Q168. sx of Meckel's diverticulum
A168. remnant of vitilline duct w/i 2 inches of ileocecal valve; bleeding from diverticulum; melena, obstruction (from intussusception), diverticulitis
Q169. tx of Meckel’s diverticulum
A169. surgical resection
Q170. dx of Meckel’s diverticulum
A170. technetium-99 scan p h2 antagonist to locate hemorrhagic cells
Q171. encephalocele
A171. projection of cranial content through bony skull defect in occiput --> severe mental retardation, seizure, movement disorder
Q172. myelomeningocele
A172. protrusion of neural and meningeal tissue
Q173. meningocele
A173. meninges protrude
Q174. spina bifida II is associated with
A174. Chiari II malformation
Q175. complications of spinal bifida
A175. caudal end of cord is tethered to distal spine and can't ascend to adult position --> scoliosis, sphincter dysfxn, LE deformities
Q176. non-communicating hydrocephalus
A176. block in exiting ventricles;; above blockage, ventricles are big
Q177. causes of non-communicating hydrocephalus
A177. secondary to narrowing at 4th ventricle/aqueduct or malformation at posterior fossa; Chiari II malformation; spina bifida occulta
Q178. communicating hydrocephalus
A178. subarachnoid villi are dysfunctional/obliterated
Q179. sx of motor cerebral palsy
A179. fixed lesion in immature brain -> nonprogressive disorder of movement and posture; spasticity is #1 type from injury to motor tracts in brain; pts are hypotonic in early months then later become spastic; CONDITION IS NOT PROGRESSIVE
Q180. sx of extrapyramidal cerebral palsy; etiology
A180. from basal ganglia damage --> choreoathetoid movement, postural ataxia, spasticity; kernicterus, there is usually some sort of brain insult
Q181. what gcs is required for a head ct to be obtained
A181. <12
Q182. #1 cause of ICH in kids
A182. Arteriovenous malformation
Q183. what meds are associated with pseudotumor cerebri
A183. tetracyclines; corticosteroids
Q184. define encephalopathy
A184. generalized cerebral dysfxn, ms change, disorientation
Q185. risk factors for developing retinopathy of prematurity
A185. Body weight <1250 g; age <32 weeks; mechanical ventilation; need for supplemental o2
Q186. causes of leukocoria
A186. retinoblastoma (--> death and visceral metastasis in all cases); cataracts (most common cause); retinopathy of prematurity
Q187. amblyopia
A187. visual impairment not corrected by glasses and not due to an ocular lesion; often from strabismus
Q188. tx of amblyopia
A188. occlusion of better seeing eye forces development of affected eye and visual cortex of affected eye
Q189. at what age will tx not be successful in ambylopia
A189. after 8yo
Q190. tx of retinoblastoma
A190. enucleation (removal of the affected eye); radiation tx; chemo
Q191. complications of neonatal cataracts
A191. if not remoed by 3-4 months, there is irreversible amblyopia
Q192. course of retinopathy of prematurity
A192. most regress spontaneously; if not, cryotherapy can be performed to reduce progression; are still at risk for amblyopia even if treated
Q193. dacryostenosis
A193. congenital nasolacrimal duct obstruction; causes overflow tearing - 6% of neonates; secondary to failure of distal membranous end of nasolacrimal duct to open
Q194. tx of dacryostenosis
A194. probing of nasolacrimal dut at 12-15 mo; although most resolve spontaneously by 1 yo (in 96% of infants)
Q195. ophthalmia neonatorum
A195. conjunctivitis occurring in first month of life; p/w eyelid edema, conjunctival hyperemia and ocular d/c
Q196. when is it normal for there not to be tears
A196. in the first few weeks of life
Q197. age of onset of gonococcal ophthalmia neonatorum
A197. 2-4 days
Q198. age of onset of chlamydia ophthalmia neonatorum
A198. 4-10 days
Q199. clinical features of gonococcal ophthalmia neonatorum
A199. eyelid edema; chemosis; purulent d/c
Q200. complications of gonococcal ophthalmia neonatorum
A200. sepsis; meningitis; arthritis; corneal ulceration; blindness
Q201. complications of chlamydia ophthalmia neonatorum
A201. corneal scarring; PNEUMONIA
Q202. tx of chlamydia ophthalmia neonatorum
A202. oral and topical erythromycin; tx parents; tx with erythromycin despite the risk of developing pyloric stenosis
Q203. tx of gonococcal ophthalmia neonatorum
A203. topical erythromycin; IV cefotaxime; tx parents
Q204. when should steroid containing eye drops not be given in conjunctivitis
A204. if hsv-1 is suspected etiology; can make disease worse
Q205. hordeolum
A205. acute infection of sebacous tarsal glands; staph aureus is usually cause
Q206. tx of hordeolum
A206. warm compresses
Q207. chalazion
A207. area of sterile lipogranulomatous reaction within meibomian glands that can enlarge; can be chronic and recurrent
Q208. is periorbital or orbital cellulitis an emergency
A208. orbital cellulitis
Q209. sx of periorbital cellulitis
A209. skin around eye is indurated, warm, and tender; no eye pain; may have si/sx of sinus infection
Q210. sx of orbital cellulitis
A210. severe pain with eye movement; proptosis; vision changes; decreased ocular mobility
Q211. dx of orbital cellulitis
A211. ct scan
Q212. organisms to cover with orbital cellulitis
A212. strep; H. flu; M. cat
Q213. tx of periorbital cellulitis
A213. iv antibiotics; can --> meningitis, tx aggressively with vanco, PCN, 1st generation cephalosporin
Q214. orgs to tx in periorbital cellulitis
A214. strep; h flu; m cat
Q215. features of fragile x syndrome
A215. hyperactive; mental retardation; large body; long face; prominent jaw and ears; thickened nasal bridge; large testes; +/- autism
Q216. facial features of 47 XYY
A216. long asymmetric ears; increased length: breadth in hands, feet, and cranium
Q217. nutritional deficiencies in goat's milk
A217. decreased vit d, iron, folate, b12
Q218. infectious disease associated with drinking raw cow's milk
A218. brucellosis
Q219. what supplements should moms receive if they are vegan
A219. b12 to prevent buildup of mma
Q220. effects of excess vit D
A220. hypercalcemia; azotemia; poor growth; n/v/d
Q221. vit E deficiency in premies -->
A221. hemolytic anemia
Q222. b1 deficiency -->
A222. beriberi; (neuritis, edema, CHF); hoarseness; anorexia
Q223. b2 deficiency -->
A223. photophobia; cheilosis; glossitis; corneal vascularity
Q224. b3 deficiency -->
A224. pellagra (dermatitis, dementia, diarrhea)
Q225. pathophys of primary hypophosphatemia
A225. defective po4 resorption; no conversion of 25-vit D --> 1,25-vit D in proximal tubules
Q226. lab studies in primary hypophosphatemia; ca; po4; alkaline phosphatase
A226. low/normal; low; high
Q227. clinical findings in primary hypophosphatemia
A227. smooth LE bowing (not angular, as is seen in Ca deficiency); intraglobulin dentin deformities (ca deficiency --> enamel defects); coarse trabecular bone and fraying
Q228. somogyi phenomenon
A228. nocturnal hypoglycemia manifested as night terrors, early am sweating, then later has hyperglycemia, ketonuria, glucosuria (sugars increase because of glucagon release)
Q229. what is an absolute contraindication for DTaP
A229. if first dose --> encephalopathy or encephalitis
Q230. clinical presentation of pb poisoning
A230. emotional lability; abdominal pain; achy bones; intermittent vomiting and constipation
Q231. at what BLL should tx be initiated
A231. >45
Q232. clinical presentation of acute hg poisoning
A232. GI pain; fever; chills; HA; visual changes; cough; cerebral palsy
Q233. clinical presentation of chronic hg poisoning
A233. gingivostomatitis; tremor; neuropsych features
Q234. clinical presentation of acute arsenic poisoning
A234. n/v/d, abdominal pain; 3rd spacing; hemorrhage; hypovolemic shock; Ventricular tachycardia; QT prolongation
Q235. narcosis
A235. deep stupor, unconsciousness
Q236. clinical presentation of cerebral palsy
A236. postural hypotonia; failure ot reach for toys; gross and fine motor delay; cognitive and sensory deficits
Q237. complications of cerebral palsy
A237. seizure; mental retardation
Q238. differences in etiology for quadriplegia and paraplegia
A238. quadriplegia results from UMN damage; paraplegia results from LMN or spinal cord damage
Q239. most likely organisms Otitis --> pneumnia in CF
A239. staph or pseudomonas
Q240. clinical presentation of meconium ileus
A240. vomiting; abdominal distension; distended bowel loops; "soap bubble" on XR
Q241. pathophys of meconium ileus
A241. obstruction begins in utero --> underdeveloped distal lumen
Q242. tx of meconium ileus
A242. surgical emergency; gastrograffin enema
Q243. vitamin a deficiency -->
A243. pseudotumor cerebri (among other things); will see bulging fontanelles, headache, n/v; may be seen as first presenting sign in a patient with CF
Q244. what effect does phenobarb have on jaundice
A244. it improves it by increasing gluocoronyl transferase
Q245. cancer that is associated with (germline) retinoblastoma
A245. osteosarcoma is most common; melanoma; squamous cell ca
Q246. conditions associated with aniridia
A246. congenital glaucoma; surge-weber; marfan; neurofibromatosis
Q247. presentation of malrotation
A247. obstruction; typically normal for first few days of life than malrotation worsens --> abdominal fullness, especially in ruq --> bilious vomiting --> ischemia and necrosis
Q248. #1 malrotation
A248. volvulus
Q249. pathophys of volvulus
A249. cecum fails to move to right lower quadrant and never adheres to abdominal wall; mesentary and SMA are tethered to narrow stalk and twist about itself; band of adhesive tissue can extend from cecum to ruq -> duodenal obstruction
Q250. presentation of children with secondary HTN
A250. ha; epistaxis; visual sx; easy fatiguability
Q251. describe how biliary atresia can occur post-natally
A251. from scarrin gan dinflammation of intrahepatic or extrahepatic biliary ducts; etiology is unclear
Q252. describe findings of prenatal biliary atresia
A252. Gallbladder is absent
Q253. sx of congenital toxoplasmosis
A253. chorioretinitis; hydrocephalus; intracranial calcifications
Q254. complement levels in post-strep GN
A254. decreased c3
Q255. what are "currant jelly stools"
A255. bloody stools; indicative of intussusception
Q256. germinal matrix
A256. embryonic tissue present near caudate nucleus, often gets damaged by hypoxia/ischemia; #1 place for intraparenchymal bleed
Q257. progression of intraparenchymal bleed in a newborn
A257. blood can flow into ventricles w/i 3 days of life
Q258. what blood vessels are damaged in shaken baby syndrome
A258. bridging veins
Q259. what blood vessels are involved in SAH
A259. circle of willis
Q260. signs of SVT
A260. Heart rate 220-270; no P waves
Q261. pathophys of strawberry hemangioma
A261. vascular tissue fails to communicate with adjoining tissue; enlarges --> raised tumor
Q262. most common causes for pneumonia in a child > 6 yo
A262. mycoplasm; strep pneumo
Q263. cardiac complications in Marfan syndrome
A263. aortic root dilatation,; aortic dissection; MVP
Q264. most common orgs in peritonsillar abscess
A264. anaerobes; GAS
Q265. physical findings of Patau syndrome
A265. forebrain fails to develop (holoprosenceph); midface developmental abnormalities; abnormal genitalia; severe mental retardation
Q266. which chromosomes are associated with; 1. Edwards; 2. Patau
A266. Edwards = Election age (18); Patau = Puberty age (13)
Q267. porencephaly
A267. cyst/cavity in brain that communicates with ventricles
Q268. causes of euvolemic hyponatremia
A268. SIADH; glucocorticoid deficiency; hypothyroid; water intoxication
Q269. features of neonatal listerosis
A269. respiratory distress at 5 days; meningitis
Q270. clinical presentation of congenital hypothyroid
A270. constipation; jaundice; FAILURE TO THRIVE; enlarged fontanelle; umbilical hernia
Q271. presentation of neuroblastoma
A271. asymptomatic abdominal mass; Horner’s; dancing eyes; dancing feet; blueberry muffin lesions; HTN
Q272. Patau's or Edwards syndrome:; microcephaly
A272. Patau
Q273. Patau’s or Edwards syndrome; prominent occiput
A273. Edwards
Q274. Patau’s or Edwards syndrome; narrow forehead
A274. Edwards
Q275. Patau’s or Edwards syndrome; microphthalmia
A275. Patau
Q276. Patau’s or Edwards syndrome; cutis aplasia
A276. Patau
Q277. Patau’s or Edwards syndrome; micrognathia
A277. Edwards
Q278. Patau’s or Edwards syndrome; low-set malformed ears
A278. Edwards
Q279. Patau’s or Edwards syndrome; cleft lip
A279. Patau
Q280. Patau’s or Edwards syndrome; congenital heart disease
A280. both
Q281. Patau’s or Edwards syndrome; omphalocele
A281. Patau
Q282. Patau’s or Edwards syndrome; clenched hands with overlapping fingers
A282. both
Q283. Patau’s or Edwards syndrome; rocker bottom feet
A283. Edwards
Q284. Patau’s or Edwards syndrome; polydactyly
A284. Patau
Q285. Patau’s or Edwards syndrome; polycystic kidney disease
A285. Patau
Q286. Patau’s or Edwards syndrome; horseshoe kidney
A286. Edwards
Q287. Patau’s or Edwards syndrome; crytorchidism
A287. Patau
Q288. Patau’s or Edwards syndrome; agenesis of corpus callosum
A288. Patau
Q289. genetic changes in Prader Willi
A289. paternal deletion, 2 defective maternal chromosomes
Q290. physical appearance of pts with Prader Willi
A290. obese; almond shaped eyes; downturned mouth; small hands and feet
Q291. genetic changes in Angelman's
A291. maternal deletion, 2 defective paternal chromosomes
Q292. physical appearance of Angelman’s
A292. large mouth; short stature; tiptoe walk; seizures
Q293. galactosemia
A293. galactose 1-p builds up and accumulates in liver and brain
Q294. complications of galactosemia
A294. increased risk of e coli sepsis; LD; premature ovarian failure
Q295. tx of galactosemia
A295. eliminate galactose containing foods from diet
Q296. complications of PKU during pregnancy
A296. if diet not followed, baby can develop microcephaly, mental retardation, and congenital heart disease
Q297. physical features of homocystinemia
A297. Marfan like appearance; dislocated lens
Q298. what effect does sepsis have on bilirubin
A298. disrupts the BBB so it can cause diffusion of bili into the brain
Q299. what effect does temp have on bili
A299. cold temperature can --> bili dissociation from albumin
Q300. protocol for neonates whose mom developed varicella infection just after delivery
A300. if mom develops sx within 2 days of delivery or during end of pregnancy, treat baby with iv Ig and acyclovir. if greater amt of time is lapsed, no tx needed
Q301. consequences of neonatal asphyxia
A301. cerebral edema; irritability; seizure; cardiomegaly; renal and heart failure; DIC; RDS
Q302. what is the apt test
A302. used to differentiae fetal from maternal blood
Q303. which drugs are contraindicated for breastfeeding
A303. lithium; cyclosporin; antineoplastic drugs; ergots; bromocriptine; tetracyclines
Q304. classic finding on XR for necrotizing enterocolitis
A304. pneumanitis intestinalis
Q305. consequences of cold temperature in a premie
A305. increased metabolic rate in order to raise body temp, but their ventilation rate increased as well; but, because of respiratory problems in premies they can't oxygenate enough so lactic acid accumulates --> metabolic acidosis
Q306. which is worse:; ABO incompatibility or Rh incompat
A306. Rh incompatibility
Q307. lab findings in ABO incompat
A307. increased reticulocyte count; weakly + coomb's
Q308. lab findings in Rh incompat
A308. strongly + coombs
Q309. pathophys of transient apnea of the newborn
A309. immature respiratory centers, especially in premies
Q310. how to id the underlying cause of congenital hypothyroid
A310. iodine uptake scan
Q311. late development of clavicle fracture during delivery... when does this occur?
A311. callous formation in anterior shoulder; 1 week
Q312. physical findings of subgaleal hemorrhage
A312. feels like cephalohematoma that crosses midline; can lose 1/3 of
Q313. onset of gonococcal conjunctivitis in a newborn
A313. DOL 2-5
Q314. onset of chlamydia conjunctivitis
A314. DOL 5-14
Q315. what effects does surfactant deficiency have on lung compliance and lung volume; cardiac effect
A315. decreases both; R-->L shunt
Q316. what should be given to newborns whose moms are infected with Hep B
A316. Hep B Ig and HBV
Q317. effect of propanolol in utero
A317. IUGR; decreased ability of asphyxiated newborn to increase heart rate and CO; associated with hypoglycemia and apnea
Q318. when is surgery for cleft lip done
A318. 3 mo
Q319. when does transient tachypnea of newborn resolve
A319. DOL #3
Q320. significance of 5th finger polydactyly
A320. in black infants, no consequence; in white infants, can be associated with cardiac abnormalities, must do an echo
Q321. twin twin transfusion
A321. donor twin --> oligohydramnios, anemia, hypovolemia; recipient twin --> polyhydramnios, larger size (20% difference in body weight), hyperviscocity, respiratory distress, hyperbili, hypocalcemia, renal vein thrombosis
Q322. when does serum bilirubin peak
A322. DOL 3-5
Q323. Top Childhood Cancers - What are they
A323. 1. Leukemia (ALL); 2. CNS tumors; 3. Lymphoma; 4. Neuroblastoma
Q324. Neuroblastoma - What is it; Associated with?
A324. Embryonal tumor of; neural crest cell origin; MC cancer in infants; more than 1/2 kids < 2 y/o; associations - neurofibromatosis; Hirchsprung’s; n-myc oncogene
Q325. Neuroblastoma - History/PE
A325. Can occur anywhere; Sx vary with location; nontender abdominal mass; (may cross midline); Horner's syndrome; HTN; cord compression; anemia; FAILURE TO THRIVE; fever; site-specific metastasis can cause proptosis; periorbital bruising; subq tumor nodules; bone pain with pancytopenia; opsoclonus/myoclonus; "dancing eyes, dancing feet"
Q326. Neuroblastoma - Dx
A326. Abdominal CT; 24-hr urinary catecholamines; assess extent of disease - CXR; bone scan; CBC; LFTs; BUN/Cr; coag panel
Q327. Neuroblastoma - Tx
A327. Excision - localized tumors; chemo includes – cyclophosphamide, doxorubicin; adjunctive radiation - if tumor spread beyond origin; prognosis improved for kids with low-risk dis., < 1 y/o, no N- myc amplification, localized
Q328. Wilms' Tumor - What is it; Associated with?
A328. Embryonal cancer of kidney; MC renal tumor in kids; 2-4 y/o; associated with - family History, Beckwith-Wiedemann syndrome, WAGR, neurofibromatosis
Q329. Wilms' Tumor - History/PE
A329. Painless, palpable abdominal mass; does not cross midline; n/v; fever; weight loss; hematuria; HTN
Q330. Wilms' Tumor - Dx
A330. Abdominal CT or US - intrarenal mass; check for metas – CXR, chest CT; CBC; LFTs; BUN/Cr
Q331. Wilms' Tumor - Tx
A331. Transabdominal nephrectomy; postsurgical chemo – vincristine, dactinomycin; flank irradiation (for some); prognosis good - depends on staging & histo
Q332. RDS - What is it
A332. MCC of respiratory failure in premies; surfactant deficiency => increased surface tension (poor lung compliance); and alveolar collapse (atelectasis); surfactant made by T2 pneumocytes, mainly ~35th week; dipalmitoyl phosphatidylcholine; risk factors - maternal diabetes, males, 2nd born of twins
Q333. RDS - History/PE
A333. Presents in 48-72 hrs of life; RR > 60/min; intercostal retractions; expiratory grunting; nasal flaring; cyanosis; progressive hypoxemia
Q334. RDS - Dx
A334. ABGs; CBC (to rule out infection); BC (to rule out infection); CXR - bilateral diffuse atelectasis, causing ground-glass appearance with visible air bronchograms; lecithin:sphingomyelin ratio < 2
Q335. RDS - Tx
A335. CPAP or intubation and mechanical ventilation; artificial surfactant to prevent - mom gets corticosteroids; monitor fetal lung maturity (L:S ratio)
Q336. RDS - Complications
A336. Persistent PDA; bronchopulmonary dysplasia; retinopathy of prematurity; intraventricular hemorrhage; NEC
Q337. Intussusception - What is it
A337. MCC of bowel obstruction in 1st 2 yrs. of life; males > females; usually proximal to ileocecal valve; cause – idiopathic, in older kids - mass or intestinal abnormality triggers the telescoping:; adenovirus or rotavirus; parasites; CF; celiac disease; polyps; intestinal lymphoma; Meckel's diverticulum; Henoch-Schonlein purpura
Q338. Intussusception - History/PE
A338. History - colicky abdominal pain in apparently healthy kids; n/v; too young to talk - cry; draw knees up to chest; dyspnea with pain; advanced signs - red "current jelly" stool; lethargy; fever; PE - abdominal tenderness, positive stool guaiac, palpable "sausage-shaped", RUQ abdominal mass
Q339. Intussusception - Dx
A339. Abdominal XR; abdominal US; air contrast barium enema; CBC
Q340. Intussusception - Tx
A340. Correct volume & electrolytes; check CBC; air-contrast barium enema - diagnostic and therapeutic; surgical reduction or resection (if gangrenous)
Q341. Pyloric Stenosis - What is it
A341. Hypertrophy of pyloric sphincter; 1st-born males more affected
Q342. Pyloric Stenosis - History/PE
A342. 1st 2 wks - 4 mos. of life; nonbilious emesis => projectile emesis after each feeding; so, babies feed well initially => malnutrition & dehydration; palpable olive-shaped, mobile, NT epigastric mass; visible gastric peristalsis
Q343. Pyloric Stenosis - Dx
A343. Abdominal US - diagnostic; barium studies - string sign, pyloric beak; hypochloremic, hypokalemic; metabolic alkalosis
Q344. Pyloric Stenosis - Tx
A344. First - hydration; correct acid-base & electrolyte abnormalities; NG tube - possible; longitudinal pyloromyotomy
Q345. Child Abuse - What is it
A345. Neglect; physical abuse; sexual abuse; emotional abuse; suspect - if History doesn't match physical findings, if there was a delay in getting medical care
Q346. Child Abuse - History/PE
A346. Infants may have apnea, seizures, FAILURE TO THRIVE Exam findings include; cutaneous - ecchymoses of varying ages, patterned injuries; skeletal - spiral fractures of femur and humerus in kids < 3 = abuse unless prove else; epiphyseal/metaphyseal injuries - can happen in infants from pulling/twisting limbs; rib injuries < 2 y/o; sexual abuse - STDs or genital trauma
Q347. Child Abuse - Dx
A347. Rule out conditions that mimic skeletal survey & bone scan; can show fractures in various stages of healing; if sexual abuse suspected - test for gonorrhea, chlamydia and HIV; to rule out shaken baby syndrome - check for retinal hemorrhages; CT for subdural hemorrhages; MRI for white matter changes
Q348. Child Abuse - Tx
A348. Document injuries; notify child protective services; hospitalize if necessary
Q349. Epiglottitis - What is it
A349. Serious, rapidly progressive infection of supraglottic; before immunization - from H influ type B; now – Streptococcus, nontypable H flu, viral agents
Q350. Epiglottitis - History/PE
A350. Sudden-onset high fever; dysphagia; drooling; muffled voice; soft stridor; cyanosis; "sniffing dog" position; "tripod" position; insist on sitting up in bed; untreated - life-threatening
Q351. Epiglottitis - Dx
A351. Clinical; DON'T EXAMINE THROAT unless anesthesiologist present; definitive Dx - direct fiberoptic visual of cherry-red, swollen epiglottis & arytenoids; lateral XR - thumbprint sign
Q352. Epiglottitis - Tx
A352. Emergency; call anesthesiologist; transfer patient to OR; endotrach intubation or trach; IV Antibiotics - ceftriaxone or cefuroxime
Q353. Croup (Laryngotracheobronchitis) - What is it
A353. Inflammation of larynx and upper airway, mainly subglottic space => narrowing of airway; kids 3 mos. - 3 yrs. MCC - parainfluenza virus 1, also - PIV-2, PIV-3, RSV, rubeola, influenza, adenovirus, Mycoplasma pneumonia
Q354. Croup (Laryngotracheobronchitis) - History/PE
A354. Prodrome - URI Sxs 1-7 days; stridor - worse by agitation; fever - low grade; hoarseness; barking cough
Q355. Croup (Laryngotracheobronchitis) - Dx
A355. Clinical; XR - steeple sign
Q356. Croup (Laryngotracheobronchitis) - Tx
A356. Mild - cool mist; moderate - oral corticosteroids; severe - (respiratory distress at rest), admit, nebulized racemic epinephrine
Q357. Bronchiolitis - What is it
A357. Acute inflammation of smallest airways; acute viral bronchiolitis; MCC - RSV; infants & kids < 2 y/o; can progress to respiratory failure; risk for severe RSV - < 6 mos. old, premies, heart or lung dis., immunodeficiency
Q358. Bronchiolitis - History/PE
A358. History - low-grade fever, rhinorrhea, cough, apnea - young infants; PE – tachypnea, wheezing, hyperresonance to percussion
Q359. Bronchiolitis - Dx
A359. CXR - hyperinflation of lungs; interstitial infiltrates; atelectasis; ELISA of nasal washings, for RSV - high sensitivity & specificity
Q360. Bronchiolitis - Tx
A360. Mild – outpatient, fluids, nebulizers, O2 if needed; admit if - marked respiratory distress, O2 saturation < 95%, toxic appearance, dehydration/poor oral feeding, premie (< 34 wks), < 3 mos. old, underlying cardiopulmonary dis., unreliable parents; inpatients - contact isolation, hydration, O2, ribavirin; RSV prophylaxis - RespiGam or Synagis, high-risk patients in winter
Q361. Otitis Media - What is it; Risk Factors
A361. Middle ear infection; MCC - #1 - S. pneumoniae, #2 - H. Flu, #3 - Moraxella catarrhalis; kids predisposed - eusta tube; risk factors - viral URIs, trisomy 21, CF, immunodeficiency, smoke exposure, day-care attendance, bottle feeding, cleft palate, prior otitis media
Q362. Otitis Media - History/PE
A362. Fever; ear tugging; hearing loss; irritability; Erythema; bulging; decreased mobility of tympanic membrane; loss of light reflex and bony landmarks; tympanic membrane may be perforated
Q363. Otitis Media - Dx
A363. Clinical
Q364. Otitis Media - Tx
A364. Amoxicillin - 10 days; Tx failure after 3 days - switch to amoxicillin-clavulanic acid, ceftriaxone or cefuroxime
Q365. Otitis Media - Complications
A365. Mastoiditis; meningitis; hearing loss; cholesteatoma; tympanosclerosis; chronic suppurative OM
Q366. Kawasaki Disease; (Mucocutaneous Lymph Node Syndrome) - What is it
A366. Multisystem acute vasculitis; usually kids < 5 y/o, especially Asian; at risk for coronary artery; aneurysms => MI
Q367. Kawasaki Disease; (Mucocutaneous Lymph Node Syndrome) - History/PE
A367. "CRASH and Burn"; Conjunctivitis - b/l; Rash; Adenopathy - cervical lymph; Strawberry tongue; Hands and feet - swollen, red, desquamation; fever > 40C for > 5 days
Q368. Kawasaki Disease; (Mucocutaneous Lymph Node Syndrome) - Dx
A368. Clinical; thrombocytosis - wk 2 or 3; increased ESR
Q369. Kawasaki Disease; (Mucocutaneous Lymph Node Syndrome) - Tx
A369. High-dose aspirin; IVIG - to prevent aneurysms; corticosteroids are contraindicated (they may increased aneurysms)
Q370. FAILURE TO THRIVE - What is it
A370. Persistent weight; below 3rd to 5th percentile or falling off growth curve; organic - medical condition; nonorganic - psychosocial; nonorganic is MC; risk factors - chronic illness, poverty, low maternal age, chaotic environment, genetic dis. (CF), inborn errors of metabolism, HIV
Q371. FAILURE TO THRIVE - History/PE
A371. Low weight for age and height; minimal weight gain or weight loss; plot on growth chart; check for signs of systemic dis. diet History; observe caregiver-child interaction
Q372. FAILURE TO THRIVE - Dx
A372. Calorie count; CBC; electrolytes; Cr; albumin; total protein; sweat chloride test; UA/UC; stool culture; O&P; assess bone age
Q373. FAILURE TO THRIVE - Tx
A373. Tx depends on cause; supplement nutrition if breastfeeding inadequate; admit if – neglect, severe malnourishment
Q374. Atrial Septal Defect (ASD) - What is it
A374. Opening in atrial septum lets blood flow bet. atria; L to R shunting due to lower R pressure; blood flow to lungs increased
Q375. Atrial Septal Defect (ASD) - History/PE
A375. History - usually presents in late childhood or early adult onset & severity depends on size; large defect - tire easy (DOE); freq. respiratory infections; FAILURE TO THRIVE => CHF => cyanosis; PE - RV heave, wide, fixed split S2, systolic ejection murmur; upper left sternal border
Q376. Atrial Septal Defect (ASD) - Dx
A376. Echo with color flow Doppler - diagnostic; ECG - right-axis deviation; CXR - cardiomegaly and increased pulmonary vascular markings
Q377. Atrial Septal Defect (ASD) - Tx
A377. Small defects may close spontaneously (no Tx needed); Antibiotics prophylaxis before dental procedures; surgical closure - infants with CHF, patients > 2:1 pulmonary to systemic blood flow; correct early to prevent – arrhythmia, RV dysfunction, Eisenmenger's syndrome
Q378. Ventric Septal Defect (VSD) - What is it
A378. MC congenital heart defect; More common in patients with - Apert's syndrome; Cri-du-chat; Trisomies 13 & 18
Q379. Ventric Septal Defect (VSD) - History/PE
A379. History - Sxs depends on degree of shunting; small defect usually asymptomatic at birth; large defect – CHF, freq respiratory infections, FAILURE TO THRIVE; PE - pansystolic murmur - lower left sternal border, loud pulmonic S2, in severe defects - systolic thrill, cardiomegaly, crackles
Q380. Ventric Septal Defect (VSD) - Dx
A380. Echocardiogram - diagnostic; EKG - may show RVH or LVH; normal with small VSDs
Q381. Ventricular Septal Defect (VSD) - Tx
A381. Most small defects close spontaneously (no Tx needed); Antibiotics prophylaxis before dental or pulmonary procedures; surgical closure - correct early to prevent - Eisenmenger's syndrome, et al; Tx CHF & respiratory infections
Q382. Patent Ductus Arteriosus (PDA); What is it
A382. Failure of DA to close => L-to-R shunt (aorta to pulmonary artery); risk factors - high altitude (low O2), 1st trimester rubella in mom, premies, females (more common)
Q383. Patent Ductus Arteriosus (PDA); History/PE
A383. History - typically asymptomatic (small PDA); slowed growth; recurrent lower respiratory infections; lower extremity clubbing; CHF Sxs; PE - wide pulse pressure, continuous machine murmur, loud S2, bounding peripheral pulses
Q384. Patent Ductus Arteriosus (PDA); Dx
A384. Small - often no signs of cardiomegaly; Large – echocardiogram - LA & LV enlarged; EKG - LVH; CXR - cardiomegaly; color-flow Doppler- diagnostic
Q385. Patent Ductus Arteriosus (PDA); Tx
A385. Indomethacin; (unless need PDA for survival); if indomethacin fails or; child > 6-8 mos. old - surgical closure preferred
Q386. Coarctation of the Aorta - What is it
A386. Narrowing proximal or distal to DA => increased flow above, decreased flow below coarctation; more common in males; Turner's, 25% have bicuspid aortic valve
Q387. Coarctation of the Aorta - History/PE
A387. History - often presents in childhood with asymptomatic HTN, headache, syncope, epistaxis, DOE, claudication; PE - systolic BP higher in upper extremities, may be greater in right arm; femoral pulses weak or delayed; late systolic murmur in left axilla; apical impulse forceful; advanced cases - well-developed upper body, lower extremities wasting
Q388. Coarctation of the Aorta - Dx
A388. EKG - LVH; echocardiography; color-flow doppler; CXR - "3" sign: rib notching; aortography – diagnostic (cardiac catheterization)
Q389. Coarctation of the Aorta - Tx
A389. Surgery or balloon angioplasty; endocarditis prophylaxis
Q390. Transposition of the; Great Arteries - What is it
A390. Pulmonary & systemic circulation in parallel; aorta connected to RV; pulmonary artery connected to LV; incompatible with life unless septal defect or PDA; risk factors - babies of DM moms, Apert's syn, Down's, cri- du-chat, Trisomies 13 & 18
Q391. Transposition of the; Great Arteries - History/PE
A391. Critically ill; cyanosis immediately after birth; tachypnea; progressive respiratory failure; CHF (some patients)
Q392. Transposition of the; Great Arteries - Dx
A392. Echocardiography; CXR - "egg-shaped sihouette", "egg on a string", "apple on a string"
Q393. Transposition of the; Great Arteries - Tx
A393. Prostaglandin E1 (PGE1) - to keep PDA open; balloon atrial septostomy; arterial or atrial switch op
Q394. Tetralogy of Fallot - What is it
A394. VSD; pulmonary stenosis; RVH; overriding aorta; right-to-left shunting => early cyanosis; risk factors - Down's, cri-du-chat, Trisomies 13 & 18
Q395. Tetralogy of Fallot - History/PE
A395. History – cyanosis, dyspnea, fatigability; profound cyanosis = tet spell; squatting for relief; hypoxemia => FAILURE TO THRIVE, mental status changes; PE - SEM at left sternal border, RV lift, single S2, CHF signs possible
Q396. Tetralogy of Fallot - Dx
A396. Echocardiography; catheterization; CXR - boot-shaped heart, decreased pulmonary vascular markings; EKG - right-axis deviation; RVH
Q397. Tetralogy of Fallot - Tx
A397. PGE1 - keep reopen PDA; for cyanotic spells - O2, propranolol, knee-chest position, fluids, morphine; balloon atrial septostomy before surgical correction
Q398. Cerebral Palsy (CP) - What is it
A398. Group of nonprogressive, nonhereditary neurological disorder; disorder in movement and posture; MC movement disorder in kids; MCC unknown - prenatal, perinatal and post insults; risk factors – prematurity, perinatal asphyxia, intrauterine growth retardation, early infection or trauma, brain malformation, neonatal cerebral hemorrhage
Q399. Cerebral Palsy (CP) - What are the categories
A399. Spastic (pyramidal) - spastic paresis of any limb, 75% of cases, MENTAL RETARDATION up to 90%,; athetoid - extrapyramidal, b. ganglia, uncontrollable jerking, writhing, worse with stress, disappears during sleep; ataxic – cerebellum, hard to coordinate movement, wide- based gait; mixed
Q400. Cerebral Palsy (CP) - History/PE
A400. May be associated with - seizure disorder, behavioral disorder; hearing or vision impaired; learning disabilities; speech deficits; hyperreflexia; Babinski; increased tone/contractures; weakness; underdevelopment; toe walking; scissor gait; hip dislocations; scoliosis
Q401. Cerebral Palsy (CP) - Dx
A401. Clinical; rule out metabolic disorder, cerebellar dysgenesis, spinocerebellar degeneration; EKG (if seizures)
Q402. Cerebral Palsy (CP) - Tx
A402. Special education; physical therapy; braces; surgical relief of contractures; for spasticity – diazepam, dantrolene, baclofen; for severe contractures - baclofen pumps, posterior rhizotomy
Q403. Febrile Seizures - What is it
A403. In kids 6 mos. - 6 y/o; no evidence of intracranial infection or other cause; risk factors - rapid rise in temp, History in close relative
Q404. Febrile Seizures - History/PE
A404. Most are simple seizures; simple - high fever, fever onset within hrs of seizure; generalized seizure, lasts < 15 min., 1 in a 24-hr period; complex - low-grade fever, fever for several days before seizure onset, seizure has focal features, can have postictal paresis, lasts > 15 min., > 1 in a 24-hr period
Q405. Febrile Seizures - Dx
A405. Find source of infection; LP - if signs of CNS infection; No labs if presentation consistent with febrile seizure; Atypical presentation – electrolytes, glucose, BC, UA, CBC with diff, EEG & MRI - complex seizures
Q406. Febrile Seizures - Tx
A406. Simple - aggressive antipyretics; tx underlying illness; complex - thorough neuro exam, chronic anticonvulsants may be necessary
Q407. Febrile Seizures - Complications
A407. Febrile Seizure will recur in 30%; no increased risk of epilepsy, developmental, intellectual or growth abnormality; those with complex seizures - 10% risk of developing epilepsy
Q408. Neonatal Jaundice - What is it; What are the types; What is kernicterus
A408. Increased serum bilirubin from increased production or decreased excretion; conjugated - always pathologic; unconjugated - patholog or physiolog; physiologic jaundice - not present until 72 hrs after birth, bilirubin peaks < 15 mg/dL, resolves by 1 week in term, resolves by 2 weeks in premies; pathologic jaundice - present in 1st 24 hrs of life, bilirubin rises to > 15 mg/dL, persists past 1 week in term, persists past 2 weeks in premies; kernicterus - unconjug hyperbilirubinemia, bilirubin deposits in pons, basal ganglia, cerebellum, irreversible, can be fatal; risk factors – premies, asphyxia, sepsis
Q409. Neonatal Jaundice - History/PE
A409. History - child breastfed or formula? intrauterine drugs; family History of – hemoglobinopathies, enzyme deficiency, RBC defects; Sxs - abdominal distention, delayed passage of meconium, light-colored stools, dark urine, low Apgar scores, weight loss, vomiting; kernicterus – lethargy, poor feeding, high-pitched cry, hypertonicity, seizures; jaundice may be cephalopedal; check for signs of - infection, congenital malformations, cephalohematomas, bruising, pallor, petechiae, hepatomegaly
Q410. Neonatal Jaundice - Dx
A410. CBC; periph blood smear; blood type mom and baby; Coombs' test; bilirubin levels; direct hyperbilirubinemia - LFTs; bile acids; Blood culture; sweat test; tests for aminoacidopathies & a1-antitrypsin deficiency; Sepsis w/u and ICU – jaundice, febrile, hypotensive and/or tachypneic
Q411. Neonatal Jaundice - Tx
A411. Tx underlying cause; unconjugated - severe - exchange transfusion; if mild – phototherapy, start earlier for premies (start at 10- 15 mg/dL)
Q412. Down Syndrome - What is it
A412. Trisomy 21, MC chromosome disorder; #2 cause of congenital MENTAL RETARDATION; risk increased with mom's age, but 80% of kids are born to women < 35 y/o; flat facial profile; prominent epicanthal folds; simian crease; decreased levels of AFP; brushfield spots; duodenal atresia - double bubble on US/XR; congenital heart disease - septum primum-type ASD due to endocardial cushion defect; Alzheimer's > 35 y/o; increased risk of ALL; meiotic nondisjunction
Q413. Edwards' Syndrome - What is it
A413. Trisomy 18 (election age=18); severe MENTAL RETARDATION; rocker bottom feet; low-set ears; micrognathia; congenital heart disease; clenched hands; prominent occiput; death usually < 1 y/o
Q414. Patau's Syndrome - What is it
A414. Trisomy 13 (puberty=13); severe MENTAL RETARDATION; microphthalmia; microcephaly; cleft lip/palate; abnormal forebrain; polydactyly; congenital heart disease; death usually < 1 y/o
Q415. Klinefelter's Syndrome - What is it
A415. XXY (male); inactivated X (Barr body); 1 of MCC of male hypogonadism testicular atrophy; eunuchoid body shape, long extremities; gynecomastia; female hair distribution
Q416. Turner' Syndrome - What is it
A416. XO (No Barr body); short stature; ovarian dysgenesis; webbing of neck; cystic hygroma; coarctaton of aorta; MCC of primary amenorrhea
Q417. Double Y males - What is it
A417. XYY; phenotypically normal; very tall; severe acne; antisocial behavior
Q418. Phenylketonuria (PKU) - What is it
A418. Phenylalanine => tyrosine; In PKU, decreased phenylalanine hydroxylase or tetrahydrobiopterin cofactor; tyrosine becomes essential; phenylalanine builds up => excess phenylketones, phenylketones – phenylacetate, phenyllactate, phenylpyruvate; MENTAL RETARDATION; fair skin; eczema; musty body odor; screened for at birth; Tx - decreased phenylalanine (in Nutrasweet) and increased tyrosine in diet
Q419. Fabry's Disease - (lysosomal storage disease); What is it
A419. X-linked recessive; defect of a-galactosidase A; ceramide trihexoside accumulates; renal failure
Q420. Krabbe's Disease - (lysosomal storage disease); What is it
A420. AR (autosomal recessive); deficiency of B-galactosidase; galactocerebroside accums; in the brain; optic atrophy; spasticity; early death
Q421. Gaucher's Disease - (lysosomal storage disease); What is it
A421. AR; deficiency of B-glucocerebrosidase; glucocerebroside accums in; brain, liver, spleen, bm; Gaucher's cells - "crinkled paper" enlarged; cytoplasm; Type I - more common, compa-; tible with normal life span
Q422. Niemann-Pick Disease - (lysosomal storage disease); What is it
A422. AR; deficiency of sphingomyelinase; sphingomyelin and cholesterol; build up in reticuloendo-; thelial & parenchymal cells; cherry-red spot on macula; death by age 3
Q423. Tay-Sachs Disease - (lysosomal storage disease); What is it
A423. AR; absence of hexosaminidase A; GM2 ganglioside accums; cherry-red spot on macula; death by age 3; MC lysosomal storage disease; that causes MENTAL RETARDATION
Q424. Metachromatic leukodystrophy - (lysosomal storage disease); What is it
A424. AR; deficiency of arylsulfatase A; sulfatide accums in brain,; kidney, liver, periph n.
Q425. Hurler's Syndrome - (lysosomal storage disease); What is it
A425. AR; deficiency of a-L-iduronidase; corneal clouding; MENTAL RETARDATION
Q426. Hunter's Syndrome - (lysosomal storage disease); What is it
A426. X-linked recessive; deficiency of iduronate sulfatase; mild form of Hurler's; no corneal clouding; mild MENTAL RETARDATION
Q427. Fragile X Syndrome - What is it
A427. X-linked; 3rd MCC of MENTAL RETARDATION; FMR1 gene affected; anticipation; triple repeat of CGG; autism; large testes, jaw, ears; floppy/prolapsed mitral valve
Q428. APGAR Score - Chart
A428. Appearance - skin color 0 = blue all over, 1 = blue at extremities, 2 = normal; Pulse - 0 = none, 1 = < 100, 2 = > 100; Grimace - reflex irritability, 0 = none, 1 = grimace, feeble cry, 2 = sneeze, cough, pull away; Activity - muscle tone, 0 = none, 1 = some flexion, 2 = active movement; Respiration - 0 = none, 1 = weak or irreg, 2 = strong
Q429. APGAR Score - What do total scores mean
A429. Score at 1 min. after birth; then at 5 min. score 8-10 - good cardiopulm adaptation; score 4-7 - possible need for resus; observe; stimulate; possible need for vent support; score 0-3 - resus immed
Q430. Erythema Infectiosum - (Fifth Disease); Cause; Characteristics
A430. Cause - parvovirus B19; prodrome none; fever absent or low-grade; "slapped cheek" erythematous; pruritic; maculopapular rash; goes to arms; spreads to trunk and legs; worse with fever and sun
Q431. Erythema Infectiosum - (Fifth Disease); Complications
A431. Arthritis; hemolytic anemia - aplastic crisis in sickle cell; encephalopathy; associated c hydrops fetalis
Q432. Measles - Cause; Characteristics
A432. Paramyxovirus; prodrome - low-grade fever; conjunctivitis; coryza; cough; Koplik's spots - buccal mucosa after 1-2 days; maculopap rash from ears down
Q433. Measles - Complications
A433. Giant cell pneumonia; otitis media; laryngotracheitis; rare - subac scleros. panencephalitis
Q434. Rubella - Cause; Characteristics
A434. Rubella virus; prodrome - asymptomatic or; tender, generalized; lymphadenopathy; erythematous, tender,; maculopapular rash; slight fever; polyarthritis in adolescents
Q435. Rubella - Complications
A435. Encephalitis; thrombocytopenia; congenital infections associated; with congenital anomalies
Q436. Roseola Infantum - Cause; Characteristics
A436. HHV-6; prodrome - acute onset of high fever; no other Sxs for 3-4 days; maculopap rash as fever breaks; starts on trunk; => face and extremities; often lasts < 24 hrs
Q437. Roseola Infantum - Complications
A437. Rapid fever onset =>; febrile seizures
Q438. Rotavirus - Characteristics
A438. Primary cause of diarrhea; in kids < 2; fever and vomiting; then diarrhea; upper respiratory Sxs; lasts < 1 wk; infection confirmed by Elisa; oral rehydration sufficient
Q439. Varicella - Cause; Characteristics
A439. VZV; prodrome - mild fever; anorexia; malaise; precedes rash by 24 hrs; generalized, pruritic,; "teardrop" vesicular rash; starts on trunk; spreads to periphery; lesions often at different; stages of healing; infectious from 24 hrs; before eruption til; lesions crust over
Q440. Varicella - Complications
A440. In immunocompromised kids - progressive varicella with; meningoencephalitis; and hepatitis; congenital infections =>; congenital anomalies
Q441. Varicella Zoster - Cause; Characteristics
A441. Prodrome - reactivation of Varicella; infection; starts as pain along affected; sensory n. pruritic "teardrop"; vesicular rash; in dermatomal distribution; uncommon unless immunocomp
Q442. Varicella Zoster - Complications
A442. Encephalopathy; aseptic meningitis; pneumonitis; TTP; Guillain-Barre; cellulitis; arthritis
Q443. Hand-Foot-and-Mouth Disease - Cause; Characteristics
A443. Coxsackie A; prodrome - fever; anorexia; oral pain; rash - oral ulcers; maculopap vesicular rash on; hands, feet, buttocks
Q444. Hand-Foot-and-Mouth Disease - Complications
A444. None; (self-limited)
Q445. Tracheoesophageal Fistula - What is it
A445. Tract between trachea & esoph; associated with esoph atresia; & VACTERL anomalies - vertebral; anal; cardiac; tracheal; esophagus; renal; limb
Q446. Tracheoesophageal Fistula - Caused by; Presentation
A446. Polyhydramnios in utero; increased oral secretion; inability to feed; gagging; respiratory distress
Q447. Tracheoesophageal Fistula - Dx
A447. CXR after NGT; air in GI tract; bronchoscopy - to confirm
Q448. Tracheoesophageal Fistula - Tx
A448. Surgical repair
Q449. Congenital Diaph Hernia - What is it
A449. GI tract segment protrudes; thru diaph into thorax; 90% are post. lt. Bochdalek
Q450. Congenital Diaph Hernia - Presentation
A450. Respiratory distress from - pulmonary hypoplasia; pulmonary HTN; sunken abdomen; bowel sounds over; lt. hemithorax
Q451. Congenital Diaph Hernia - Dx
A451. US in utero; postnatal CXR - to confirm
Q452. Congenital Diaph Hernia - Tx
A452. Hi-freq ventilation or; extracorporeal membrane; oxygenation (ECMO); (to manage pulmonary HTN); surgical repair
Q453. Gastroschisis - What is it
A453. Herniation of intestine; thru abdominal wall; next to umbilicus; (usually on right); with no sac
Q454. Gastroschisis - Caused by; Associated with
A454. Polyhydramnios in utero; often premie; associated with - GI stenoses; GI atresia
Q455. Gastroschisis - Tx
A455. Surgical emergency; single-stage closure possible; in only 10%
Q456. Omphalocele - What is it
A456. Herniation of abdominal viscera; thru abdominal wall; at umbilicus; into sac covered by; peritoneum and amniotic memb
Q457. Omphalocele - Caused by; Associated with
A457. Polyhydramnios in utero; often premie; associated with; other GI & cardiac defects
Q458. Omphalocele - Tx
A458. C-section - to prevent sac rupture; if sac intact - postpone surg correction; until patient fully resuscitated; keep sac covered/stable; with petroleum & gauze; intermittent NG suction - to prevent abdominal distention
Q459. Duodenal Atresia - What is it
A459. Complete or partial failure; of duodenal lumen; to recanalize; during gestational wks 8-10
Q460. Duodenal Atresia - Presentation; Caused by; Associated with
A460. Bilious emesis within hrs; after 1st feeding; polyhydramnios in utero; associated with - Down's; other cardiac/GI anomalies:; annular pancreas; malrotation; imperforate anus
Q461. Duodenal Atresia - Dx
A461. Double-bubble sign on XR; (prox to site of atresia)
Q462. Duodenal Atresia - Tx
A462. Surgical repair
Q463. Meckel's Diverticulum - What is it
A463. MC congenital GI tract anomaly; vestigial remnant; of omphalomesenteric duct; rule of 2's - 2x's as many males; 2 ft. from ileocecal valve; 2% of people affected; 2 types of mucosa - gastric; pancreatic
Q464. Meckel's Diverticulum - Presentation
A464. MC presentation - painless rectal bleeding; painful diverticulitis; intest. obstruction from - intussusception or volvulus
Q465. Meckel's Diverticulum - Dx
A465. Meckel's scan - for ectopic gastric mucosa; uses IV technetium; pertechnetate
Q466. Meckel's Diverticulum - Tx
A466. Surgery
Q467. Hirschsprung's Disease - (Congenital Aganglionic Megacolon); What is it
A467. Absence of autonomic; innervation of bowel wall; inadeq relaxation; and peristalsis; => intest. obstruction
Q468. Hirschsprung's Disease - (Congenital Aganglionic Megacolon); Presentation
A468. Abdominal distention; bilious vomiting; fail to pass meconium in; 1st 24 hrs of life
Q469. Hirschsprung's Disease - (Congenital Aganglionic Megacolon); Dx
A469. Barium enema - dilated prox segment; narrowed distal segment; rectal Bx - to confirm
Q470. Hirschsprung's Disease - (Congenital Aganglionic Megacolon); Tx
A470. Colostomy prior to; corrective surgery
Q471. Hypospadias - What is it
A471. Abnormal urethral opening; on ventral surface of penis; due to incomplete dev of; distal urethra
Q472. Hypospadias - Presentation; Associated with
A472. Chordee; associated with - hernias; cryptorchidism
Q473. Hypospadias - Tx
A473. Circumcision contraindicated; surgical repair uses; preputial tissue
Q474. X-linked Agammaglobulinemia; (Bruton's Disease) - What is it
A474. B-cell defect; boys only; may present < 6 mos. of age; at risk for life-threatening Pseudomonas infections
Q475. X-linked Agammaglobulinemia; (Bruton's Disease) - Dx
A475. No B cells; low levels of all Ab classes
Q476. X-linked Agammaglobulinemia; (Bruton's Disease) - Tx
A476. IVIG; prophylactic Antibiotics
Q477. Common Variable Immunodeficiency - What is it
A477. Ig levels drop in 2nd-3rd decade of life; increased risk of lymphoma; increased risk of autoimmune dis.
Q478. Common Variable Immunodeficiency; Dx
A478. Ig levels; antibody titers
Q479. Common Variable Immunodeficiency; Tx
A479. IVIG; prophylactic Antibiotics
Q480. IgA Deficiency - What is it
A480. MC immunodeficiency; usually asymptomatic; may have recurrent infections
Q481. DiGeorge Syndrome; (Thymic aplasia) - What is it
A481. CATCH-22; Cardiac defects; Abnormal facies; Thymic hypoplasia; Cleft palate; Hypocalcemia; 22 - microdeletions in chrom22; tetany in first days of life
Q482. DiGeorge Syndrome; (Thymic aplasia) - Dx
A482. Absolute lymphocyte count,; mitogen stimulation response; and delayed hypersensitivity; skin testing
Q483. DiGeorge Syndrome; (Thymic aplasia) - Tx
A483. BMT - if severe; IVIG; thymus transplant
Q484. Ataxia-Telangiectasia - What is it
A484. DNA repair defect; oculocutaneous telangiectasias; progressive cerebellar ataxia
Q485. Ataxia-Telangiectasia; Tx
A485. No effective Tx for CNS abnorm; neuro deterioration progresses; death by 30 y/o
Q486. Severe Combined; Immunodeficiency (SCID) - What is it
A486. Severe lack of B & T cells; frequent severe bacterial infections; chronic Candidiasis; opportunistic organisms
Q487. Severe Combined; Immunodeficiency (SCID) - Tx
A487. BMT or stem cell transplant; IVIG; PCP prophylaxis until BMT; gene therapy may be; future option
Q488. Wiskott-Aldrich Syndrome - What is it
A488. X-linked recessive; T & B cell dysfunction; thrombocytopenia; small-sized platelets; eczema; high IgE; high IgA; low IgM; bloody diarrhea; bleeding gums; prolonged nosebleeds
Q489. Wiskott-Aldrich Syndrome - Tx
A489. Protective helmet; IVIG; aggressive Antibiotics for infections; HLA-identical BMT; if no BMT - rarely survive to adulthood
Q490. Chronic Granulomatous Disease-; (CGD); What is it
A490. X-linked or AR; deficient superoxide production by PMNs & M0s; usual sites of infection – skin, lungs (pneumonia), lymph nodes, liver (abscesses, hepatitis), bones (osteomyelitis); swollen collections of infected tissue obstruct intestines (IBD) and urinary tract (UTIs)
Q491. Chronic Granulomatous Disease-; (CGD); Dx
A491. Absolute neutrophil count and adhesion assays – chemotaxic, phagocytic, bactericidal; diagnostic - negative nitroblue tetrazolium dye reduction test (NBT)
Q492. Chronic Granulomatous Disease-; (CGD); Tx
A492. Daily TMP-SMX; judicious Antibiotics use during infections; IFN-g - can decreased incidence of serious infection
Q493. Chediak-Higashi - What is it
A493. AR; giant lysosomal granules dev. in neutrophils lysosomes can't fuse with phagosomes => ingested bact. can't be lysed; oculocutaneous albinism; neuropathy; neutropenia
Q494. C1 Esterase (Inhib) Deficiency - (Hereditary Angioedema); What is it
A494. C1 inhibitor - acute phase protein inhibits proteinases of: complement pathway, clotting pathway, kinin generator pathway, fibrinolytic pathway; deficiency => hereditary angioedema; AD; can affect - hands & feet - local edema, bowel - extreme abdominal pain, mouth/airway - life-threatening edema; usually lasts 3 days; can be precipitated by trauma, virus; aggravated by stress
Q495. C1 Esterase (Inhib) Deficiency - (Hereditary Angioedema); Dx
A495. Total hemolytic C' assay- CH50; if defect in one component - no CH50 reduction, then detect which component
Q496. C1 Esterase (Inhib) Deficiency - (Hereditary Angioedema); Tx
A496. Daily prophylactic danazol; purified C1 esterase and FFP - prior to surgery
Q497. Terminal Complement Deficiency; (C5-C9) - What is it
A497. AR; recurrent N. meningitides & disseminated gonorrhea infections; rarely - systemic lupus
Q498. Terminal Complement Deficiency; (C5-C9) - Tx
A498. Meningococcal vaccine; appropriate Antibiotics
Q499. Hyper IgM Syndrome - What is it
A499. MC - deficiency in CD40 ligand in T Helper => can't class switch; normal or high IgM; low IgG, IgA, IgE; XL recessive (most common), AR (others); severe upper & lower respiratory; diarrhea - Cryptosporidium
Q500. Hyper IgM Syndrome - Tx
A500. IVIG; PCP prophylaxis - TMP-SMX
Q501. What is the most common cause of bowel obstruction in the first two years of life?
A501. Intussusception
Q502. What is the classic triad for intussusception
A502. Abdominal pain, vomiting, blood per rectum
Q503. Red currant-jelly stools are indicative of what?
A503. Intussusception
Q504. What is diagnostic for intussusception
A504. Air-contrast barium enema
Q505. The string sign on a barium study indicates what?
A505. Pyloric stenosis
Q506. What type of acid/base abnormality can occur with persistent emesis, such as that seen with pyloric stenosis?
A506. Hypochloremic, hypokalemic metabolic alkalosis
Q507. What type of immunodeficiency presents after six months of age with recurrent sinopulmonary, GI, and urinary tract infections with encapsulated organisms (H. influenzae, Streptococcus pneumoniae, Neisseria meningitidis)?
A507. B-cell deficiencies
Q508. What immunodeficiency is seen in boys only and presents with frequent pseudomonas infections?
A508. X-linked agammaglobulinemia (Bruton's)
Q509. With what immunodeficiency will there be a drop in immunoglobulin levels to the 20s and 30s, increased pyogenic upper and lower respiratory diseases, and increased risk of lymphoma and autoimmune disease?
A509. Common variable immunodeficiency
Q510. What is the most common immunodeficiency
A510. IgA deficiency
Q511. What does CATCH-22 stand for in DiGeorge syndrome?
A511. Congenital heart disease,; Abnormal facies,; Thymic aplasia,; Cleft palate,; Hypocalcemia,; 22q deletion
Q512. With what immunodeficiency syndrome will patients have increased infections with fungi and PCP?
A512. Thymic aplasia; (DiGeorge syndrome)
Q513. With what immunodeficiency will patients present with cerebellar ataxia and increased incidence of non-Hodgkin's lymphoma and gastric carcinoma?
A513. Ataxia-telangiectasia
Q514. With what immunodeficiency will patients have severe, frequent bacterial infections; chronic candidiasis, and opportunistic organisms?
A514. Severe combined immunodeficiency
Q515. What is the treatment for SCID?
A515. PCP prophylaxis,; bone marrow transplant or stem cell transplant,; IVIG for antibody deficiency
Q516. With what immunodeficiency will patients present with eczema, increased IgE/IgA, decreased IgM, and thrombocytopenia?
A516. Wiskott-Aldrich syndrome
Q517. What is the classis presentation of Wiskott-Aldrich syndrome (3)
A517. Bleeding,; eczema,; recurrent otitis media
Q518. With what immunodeficiency will patients present with anemia, lymphadenopathy, hypergammaglobulinemia, and infections with catalase-positive organisms?
A518. Chronic granulomatous disease
Q519. Pts with chronic granulomatous disease require daily treatment with what antibiotic?
A519. TMP-SMX
Q520. With what immunodeficiency will patients present with oculocutaneous albinism, neuropathy, neutropenia, and increased incidence of overwhelming infections with S. pyogenes, S. aureus, and Pseudomonas?
A520. Chediak-Higashi syndrome
Q521. What is the defect in Chediak-Higashi syndrome?
A521. Neutrophil chemotaxis (Autosomal recessive)
Q522. What is the diagnostic test for chronic granulomatous disease?
A522. Nitroblue tetrazolium test
Q523. What immunodeficiency presents with recurrent episodes of angioedema lasting 2-72 hours and provoked by stress or trauma
A523. C1 esterase deficiency (autosomal dominant)
Q524. What type of immunodeficiencies tend to present at 1-3 months?
A524. T-cell deficiencies
Q525. With what immunodeficiency are patients unable to form membrane attack complexes
A525. Terminal compliment deficiency (C5-C9)
Q526. With what immunodeficiency will patients get recurrent miningococcal or gonococcal infections?
A526. Terminal compliment deficiency (C5-C9)
Q527. What type of immunodeficiencies are characterized by mucous membrane infections, gram-negative enteric organisms, and delayed umbilical cord separations?
A527. Phagocyte deficiencies
Q528. What type of immunodeficiencies are characterized by recurrent bacterial infections with encapsulated organisms
A528. Complement deficiencies
Q529. What are the subacute-phase manifestations of Kawasaki disease? (2)
A529. Thrombocytosis and increased ESR
Q530. What are the acute-phase manifestations of Kawasaki disease? Hint: Crash and Burn
A530. Conjunctivitis,; Rash,; Adenopathy,; Strawberry tongue,; Hands and feet (red, swollen, flacky skin),; AND Burn=fever >40 for 5 days
Q531. What is the treatment for Kawasaki disease?
A531. High-dose aspirin and IVIG
Q532. What treatment is contraindicated with Kawasaki disease?
A532. Corticosteroids; (may increase aneurysm formation)
Q533. Untreated patients with Kawasaki disease are at risk for what complications?
A533. Coronary artery aneurysms and MI
Q534. What disease is defined as an acute inflammatory illness of the small airways that primarily affects infants and children under 2?
A534. Bronchiolitis; (RSV most common)
Q535. What disease is characterized by the classic barking cough (usually at night)?
A535. Croup; (Laryngotracheobronchitis)
Q536. What is the steeple sign?
A536. Subglottic narrowing of the airway,; commonly seen with Croup
Q537. With what disease will patients age 3-7 present with muffled voice and drooling?
A537. Epiglottitis
Q538. The thumbprint sign on lateral film is characteristic of what?
A538. Epiglottitis
Q539. What three organisms commonly cause otitis media?
A539. S.pneumoniae,; H.influenzae,; Moraxella catarrhalis
Q540. What disease is characterized by a slapped-cheek rash that is worse with fever and sun exposure?
A540. Erythema infectiosum,; caused by Parvovirus B19
Q541. What disease has an acute onset of high fever followed by a maculopapular rash that appears as the fever breaks?
A541. Roseola infantum (HSV-6)
Q542. An abdominal radiograph showing the "double-bubble" sign indicates what?
A542. Duodenal atresia
Q543. What is the most common cause of respiratory distress in infants?
A543. Respiratory distress syndrome
Q544. Ground-glass appearance and air bronchograms on CXR in an infant indicate what disease?
A544. Respiratory distress syndrome
Q545. What are the five Ts and 1P of cyanotic heart disease?
A545. 1. Truncus arteriosus; 2. Transposition of the great arteries; 3. Tricuspid atresia; 4. Tetralogy of Fallot; 5. Total anomalous pulmonary venous return 6. Pulmonary atresia
Q546. What congenital heart defect presents with a harsh holosystolic murmur heard best at the lower left sternal border?
A546. VSD
Q547. What congenital heart defect presents with a wide and fixed split S2 and a systolic ejection murmur heard best at the upper left sternal border?
A547. ASD
Q548. What drug is given to close a patent ductus arteriosus?
A548. Indomethacin
Q549. What congenital defect often presents in childhood with asymptomatic hypertension?
A549. Coarctation of the aorta
Q550. What is the most common childhood malignancy?
A550. ALL
Q551. household and sex contacts of kids/teens with hepatitis A should receive what?
A551. immunoglobulin to prevent hep A
Q552. intracranial trauma without obvious external findings in baby; think?
A552. shaken baby syndrome
Q553. person who gets hives after bee sting. what NSiM?
A553. give Epi-pen at all times (bc had systemic reaction) as well as give prescription for diphenydramine
Q554. hyponasal speech plus mouth breathing, think?
A554. NASAL POLYP
Q555. urinalysis of > 1.015 makes what highly unlikely?
A555. diabetes insipidus
Q556. primary vs secondary enuresis
A556. secondary = bedwetting after 3 or more months of dry nights;; is usually due to psych factors or changes
Q557. therapy for child over 6 with enuresis?
A557. consider conditioning therapy with bedwetting alarm (works in 30-60%)
Q558. any UTI in baby with vomiting and dehydration, think? caused by? tx with ? what follow up tests?
A558. pyelonephritis;; e coli; ceftriaxone; need to follow up ANY UTI in girl under 5 and any boy, with renal US and VCUG to rule out v-u reflux
Q559. rash post GAS pharyngitis, think?
A559. Scarlet fever!
Q560. boggy mass on scalp? tx?
A560. most likely kerion = inflammatory form of tinea capitis (Trich. tonsurans or Microsporum canis). tx = 8-12 weeks oral griseofulvin and shampooing with selenium sulfide
Q561. oral, feet, and mouth ulcers, think?
A561. hand foot and mouth disease!; coxsackie A16
Q562. ehrlichosis contracted how?
A562. by tick bite
Q563. adults with e. infectiousum may also have what sx?
A563. ARTHROPATHY
Q564. parvovirus B19 ( slapped cheek/ E. infectiousum )in hemolytic anemias may cause?
A564. aplastic anemia
Q565. high and persistent fever that is not very responsive to normal doses of paracetamol; with red cracked lips and red mucous membranes?
A565. kawasaki's disease
Q566. swelling of one eyelid + proptosis, limited ocular movements?
A566. orbital cellulitis;; if just swelling of eyelid think periorbital cellulitis
Q567. 1 y/o black baby solely breastfed may develop?
A567. ricketts due to VitD def in breast milk and poor skin conversion of vit D
Q568. torsion of testicular appendix vs torsion of testicle?
A568. Torsion of testicle: in kids over 12; testicular appendix: in kids 2-11
Q569. acute pain in scrotum of sexually active teen, think?
A569. epididymitis
Q570. m/c pathogens of otitis media?
A570. S pneum,; H influ,; M catarrhalis
Q571. what constitutes increased risk for post-traumatic seizure? what % get? tx if these?
A571. depressed cranial fracture; intracranial hemorrhage; cerebral contusion; or unconscious > 24 hrs. 75% of these will seizure; so must tx with anticonvulsants prophylactically
Q572. main pathogen causing croup? epiglotitis?
A572. parainfluenza;; s pneum,; aureus or GBS; H influenzae in non vaccinated
Q573. cow milk feeding causes?
A573. iron def anemia!
Q574. hirsutism, deepened voice, acne, clitoromegaly, think?
A574. CAH
Q575. can you have allergic rhinitis that presents with rhinitis with NO seasonal variation?
A575. YES = perenial allergic rhinitis ie exposure to house dust, pet danders that are there year round
Q576. bilateral PURULENT discharge with fever, cough, sinus tenderness?
A576. sinusitis
Q577. 3 common pathogens in human bites?
A577. eikenella corrodens; peptostreptococcus; alpha streptococcus
Q578. during Antibiotics tx , diaper rash due to?
A578. candida dermatitis!
Q579. m/c cause of round pneumonia?
A579. strep pneumonia
Q580. HUS is most common in what age?
A580. under 2 y/o's!
Q581. live vaccines?
A581. MMR; varicella
Q582. Guillan Barre has what CSF finding?
A582. increased protein;; mild mononuclear pleocytosis (<10 cells/ml)
Q583. when do you treat lead poisoning with chelation?
A583. only if levels over 45 ug/mL !; otherwise just treat by removing source, ie investigating house for lead content, etc
Q584. name 4 advantages of OPV? 1 of IPV?
A584. OPV:; costs less; increased mucuosal immunity; fewer shots; herd immunity through secondary transmission; still recommended for global polio eradication; IPV: less paralytic polio
Q585. m/c cause of HTN in kids?
A585. secondary (not essential) Renal causes!! ie infection, PCKD, glomerulonephritis, vascular anomalies, tumors
Q586. 2 tx's for Increased ICP
A586. intubation with hyperventilation; Mannitol to induce osmotic shift
Q587. after what type of wounds/pts do you give Td, vs Td + Tet Immune Globulin, vs DTaP?
A587. DTaP: give only in kids under age 7; Td only: if patient has had < 3 immunizations or status unknown AND: minor wound w/o contamination; Td + TIG: if patient has had < 3 immunizations or status unknown AND contaminated wounds
Q588. only 2 exceptions to IC kids NOT receiving varicella vacc (b/c it is live)
A588. ALL kid in remission with lymphocyte count over 700;; HIV infected kid in CDC class I =CD4 lymphocytes>25%. All other immunodeficiencies, don’t give!!
Q589. live viruses not to be given to ?
A589. IC pts (see exceptions above) or pregnant women
Q590. m/c organism causing ANY otitis externa?
A590. P. aeruginosa
Q591. causes of intoeing?
A591. adducted great toe,; metatarsus adductus,; medial tibila torson,; femoral anteversion
Q592. m/c back deformity in kids?
A592. idiopathic scoliosis
Q593. tx of b pertussis?
A593. e-mycin
Q594. hacking cough + absolute lymphocytosis, + thick, clear n/p mucus?
A594. think b pertussis
Q595. TCA poisoning?
A595. anticholinergic sx,; mental status changes,; arrhythmias
Q596. otherwise normal 7 y/o with hypospadias. What next step in tx?
A596. renal US to check for enlarged prostatic utricle which predisposes to UTI's
Q597. snuffles =?; acral rash?
A597. mucocutaneous lesions producing persistent, purulent, often bloody nasal discharge!!; acral = on extremities ie hands and feet = syphilis
Q598. febrile seizures do/don’t increase risk for epilepsy?
A598. DO! but by only 7%
Q599. painful swollen fingers and toes for 2 days?
A599. think sickle cell hand-foot syndrome
Q600. most common manifestation of sickle cell disease?
A600. acute PAINFUL episodes ie in legs, arms, in younger kids;; head, chest, back, abdomen in older kids
Q601. shortness from GH deficiency usually shows by how many yrs?
A601. 3 yrs old!
Q602. chest pain in young 12 y/o girl athlete during exercise?
A602. asthma!; give inhaled albuterol
Q603. 5 days of high fever; bilateral bulbar non-exudative conjunctivitis, rash, hand and foot edema-->desquamation, strawberry tongue and mouth erythema, cervical lad?
A603. kawasaki's!
Q604. m/c cyanotic heart disease in newborn?
A604. Transposition!
Q605. presentation of transposition?
A605. blue baby with low sat, no change with 02 given, otherwise looks in no distress; egg on string (narrow mediastinum) chest x-ray...
Q606. a typical, physiological "flow" murmur?
A606. Still's murmur - heard at left lower sternal border
Q607. systolic murmur heard in ASD is really what?
A607. increased flow across pulmonic valve and tricuspid regurgitation
Q608. systole ejection murmur heard best at upper sternal border but radiating to axilla and back? vs carotids?
A608. axilla and back = peripheral pulmonic stenosis (due to flow past sharp angle of pa and its branches = resolves in 6 mo; carotids = aortic stenosis/bicuspid aortic valve
Q609. Most common congenital heart lesions?
A609. VSD
Q610. what's a positive PPD?
A610. 5mm if CLOSE CONTACTS w/known or suspected cases; 10mm if greater risk for disseminated disease ie <4y/o's, DM, CKD, malnourishment, lymphoma, or at greater risk of exposure to TB, ie born in, parents born in high prevalent regions, travel to these regions, exposure to adults at high risk, (ie HIV infected, homeless, drug abusers); 15mm in normal, age over 4 w/o any risk factors
Q611. What are the 5 portions of the APGAR score?
A611. Appearance; Pulse (HR); Grimace; Activity; Respirations
Q612. Dx:; A lump on the face after a foreceps delivery
A612. Subcutaneous Fat Necrosis
Q613. Dx:; rash that starts after the first day of life as a flat reddish area with a central papule that becomes a pustule and persists for a couple of weeks; Test?
A613. Erythema Toxicum; test: Wright stain of vesicular fluid will show Eosinophils
Q614. Dx;; Strawberry rash of newborn with difficulty breathing
A614. Epiglottic Hemangioma
Q615. Dx:; sharply demarcated bruise-like appearance on sacral area of newborn
A615. Mongolian spots; (do not confuse w/ Abuse, which is not sharply demarcated)
Q616. Dx:; orange/yellow warty lesion on the scalp in newborn that displaces hair growth; Tx?; what can it lead to?
A616. Nevus sebaceous; Tx: leave alone until Adolescence (should go away); leads to: 15% chance to become malignant
Q617. Dx:; scalp swelling that crosses suture lines and goes away in a few days
A617. Caput
Q618. Dx:; scalp swelling that does not cross suture lines and goes away in a couple of months; cause?
A618. Cephalohematoma; cause: Subperiosteal bleed
Q619. When do anterior fontanels normally open and close?; Posterior?
A619. Anterior: opens: birth; closes: 9 - 18 months; Posterior: opens: sometimes at birth; closes: 4 - 5 months
Q620. what is possible diagnosis if:; 1. both fontanels are enlarged?; 2. One fontanel only is enlarged?
A620. 1. Hydrocephalus; 2. Hypothyroidism
Q621. Newborn eye:; White reflex
A621. Retinoblastoma; (another source: MCC is Congenital Cataracts)
Q622. Newborn eye:; lens opacity
A622. Cataracts; (possible Galactosemia)
Q623. Newborn eye:; Aniridia and hemihypertrophy (one side of body larger then other)
A623. Wilms Tumor
Q624. Dx:; blue baby pinks up on crying; confirmatory test?
A624. Choanal Atresia; test: Catheter doesn’t pass thru nose
Q625. MC abdominal mass in newborn
A625. Polycyctic Kidney disease
Q626. when should an umbilical hernia close?
A626. by 5 months
Q627. which has a sac--Omphalocele or Gastroschisis?
A627. Omphalocele; (“O” is like a closed sac)
Q628. what should be avoided in a patient with Epispadias or Hypospadias?
A628. do NOT circumsize
Q629. how long can undescended testis remain undescended before you need to bring them down?
A629. one year
Q630. MC type of Ambiguous genitalia
A630. Congenital Adrenal Hyperplasia; (21-hydroxylase deficiency is the MC of the CAH)
Q631. Dx:; newborn has a mass on the right anterior, superior chest and crepitus on exam; Tx?
A631. Clavicle fracture (MC newborn fracture); Tx: None (will heal on its own)
Q632. MC birthing brachial plexus injury?; what nerve roots?; another name for this injury?
A632. Erb-Duchenne; roots: C5-C6 (C4: if ipsilateral diaphragmatic paralysis also); another name: “waiter’s tip” – wrist flexed
Q633. birthing injury where baby has hand extended and fingers flexed; what nerve roots?; another name for this injury?
A633. Klumpke; roots: C8-T1; another name: “claw hand”
Q634. What are the (3) 'diseases' tested for during a newborn screening?
A634. 1. PKU; 2. Galactosemia; 3. Hypothyroidism
Q635. Dx:; baby born with Mental retardation, Eczema, musty odor, fair hair, fair skin and blue eyes
A635. PKU
Q636. Dx:; baby born with Jaundice, Hypoglycemia, and Cataracts; genetic inheritance?; what is contraindicated in this baby?
A636. Galactosemia; (Autosomal Recessive); Contraindication: Breastfeeding
Q637. Dx:; Newborn with Jaundice, mottled, constipation, Large tongue, Umbilical hernia; what is seen on blood test?