Pediatrics SHELFBlueprints – common favorite XPretest – one of better of the series *BRS – best but longest *UW Case files

Don’t go overboard on vaccine schedules and developmental milestones. You can sink a lot of time into that for little to no benefit

o You should know a couple of big milestones per age group. Know the contraindications for vaccines; don’t learn the actual timelines.

1. Vaccinations given at birth - Hep B2. Vaccinations given at 1 month - Hep B, only if not given at birth3. Vaccinations given at 2 months - Pediarix (HBV, DTaP, IPV) ,Prevnar

(pneumococc), Rotavirus, Hib

4. What is in pediarix - HBV\nDTaP \nIPV5. Vaccinations given at 4 months - Pediarix\nPrevnar \nHib \nRotavirus6. Vaccinations given at 6 months - Pediarix \nPrevnar \nHib\nRotavirus7. Vaccinations given at 12 months - DTaP ,Hib, MMR, VZV, Prevnar,

Influenza, Hep A8. Vaccinations given at 24 months - None9. Vaccinations given at 4–6 yrs - DTaP \nIPV \nMMR\nInfluenza10. Vaccinations given at 11 yo - Tdap \nMeningococcus vaccine11. What is the schedule for well visits starting - 1 mo, 2 mo, 4 mo, 6 mo, 9 mo, 12 mo

from birth 15 mo, 18 mo, 24 mo, then annually12. Why shouldn't babies drink water - 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 szs

13. Plagylocephaly - Mishapen head14. Sunsetting sign - Increased icp, eyes are half closed

2ndary to the increased icp on the cranial nerves

15. Craniosynostosis - Premature closure of the fontanelles16. Rash pattern in RMSF - Palms and soles, then spreads to trunk,

petechial17. Rash pattern in rubella - Rash on face that spreads to the rest

of the body18. Rash pattern in measles - Rash starts at the head and spreads

downwards and disappears in the same manner

19. Rash pattern for erythema infectiosum -slapped cheek rash; lacy, reticular20. VZV rash pattern -begins on trunk, followed by head, face, and extremities21. Gross motor skills at:

a. 1 mo old -Raises headb. 3 mo -Holds head upc. 4–5 mo -Rolls front to back and back/front\nsits supportedd. 6 mo -Sits unsupportede. 9 mo -Crawls\ncruises\npulls to standf. 12 mo -Walks aloneg. 15 mo -Walks backwardsh. 18 mo -Runsi. 24 mo -Walks well up and down stairsj. 3 yrs -Rides tricycle\nthrows ball overhandk. 4 yo -Alternates feet going down stairs \nskips

22. Fine motor skills at: a. 1 mo -follows eyes to midlineb. 3 mo -hands open at restc. 4–5 mo -grasps with both hands togetherd. 6 mo -transfers hand to hand, reaches with either hande. 9 mo -pincher grasp\nfinger feedsf. 12 mo -throws, releases objectsg. 15 mo -builds 2 block towerh. 18 mo -feeds self with utensilsi. 24 mo -removes clothing \nbuilds 5 block towerj. 3 yrs -draws circlek. 4 yrs -catches ball\ndresses alonel. 5 yrs -ties shoes

23. Simple febrile sz -Between 6mo – 6yr: tonic clonic; associated with fever >100.4 sx lasts <15 mins Only 1 sx in 24 hrsminimal post–ictal state

a. Management: Determine the source of the fever, otherwise, no other w/u is needed

24. Complex febrile sz -6 mo – 6 yrsa. Management: full w/u should be done, but no anti–epileptics, no EEG needed ; if

pt is <18 mo, LP25. Focal sz -15 mins; >1 sz/24 hrs26. EEG abnormality associated with infantile spasm -Hypsarrythmia27. Definition of recurrent abdominal pain ->3x in 3 mo28. #1 cause of abdominal pain -Gastroenteritis29. Mesenteric lymphadenitits -Persistent pain following an infx30. Clinical features of HSP -Condition preceded by URI, nnon–

thrombocytopenic palpable purpura, hematuria (good prognosis), proteinuria (poor prognosis) ,

spasmodic abdominal pain, ileus, n/v, Upper GI/Lower GI bleed

31. Tx of Henoch-Schonlein Purpura (HSP) -steroidsa. Inflammation and bleeding in the small blood vessels in your skin, joints,

intestines and kidneys.The most striking feature of Henoch-Schonlein purura is a purplish rash, typically on the lower legs and buttocks

32. Complication of HSP -Intussusception ,kidney probs33. Clinical features of Kawasaki

a. -CRASH and BURN i. Conjunctivitis

ii. Rash (on trunk mostly) iii. Aneurysm (coronary) iv. Skin-peels off, Strawberry tongue, v. Hands/Feet edema,

vi. BURN = FEVER (x 5d)34. Phases of kawasaki dz -acute, subacute (aneurysm

formation) convaslescent -takes 2–3 months to resolve

35. Tx of Kawasaki dz -ASA, IVIG36. What is colicky pain associated with -Constipation37. What type of stool is seen with bacterial enterocolitis -Bloody, mucinous stool38. when will an appendix perforate in appendicitis -W/in 36 hrs39. Which infx can clinically mimic appendicitis -Yersinia; campylobacter40. What imaging study for appendicitis -CT41. Meds used to tx of perforated appendix -Ampicillin, Gentamicin, Flagyl42. Clinical presentation of intussusception -Currant jelly stools, lethargy, palpable

tubular mass, paucity of gas on X-ray or evidence of obstruction

43. Imaging most specific for intussusception -Barium (or air) enema; is also therapeutic

44. Most common location for intussusception -Ileocolic; can also develop at meckel's diverticulum

45. Major complication of intussusception -There is impaired venous return so bowel edema develops ––> ischemia, necrosis – –> perforation

46. Etiology of intussusception -Ileum invaginates into colon at ileocecal valve -Previous viral infx ––> hypertrophy of the peyer's patches... this can develop into a lead point; HSP can be association with an ileal–ileal intussusception

47. Tx of intussusception -Must do fluid resuscitation first, if needed, hydrostatic reduction with air/barium

48. Recurrence rate of intussusception -15%49. At what age would a pt present with -1–3 mo

pyloric stenosis 50. Which medication can be associated with -erythromycin

pyloric stenosis 51. Best imaging for pyloric stenosis -u/s; will also see a string sign Upper GI

study52. Tx of pyloric stenoSIS -MUST correct fluids and lytes first! Then

pyloromyotomy53. Describe malrotation -Small intestines rotate abnormally in utero,

so there is an abnormal fixation posteriorly to the mesentery; it can twist on its vascular supply ––> volvulus

54. Clinical presentation of malrotation -Bilious emesis\npossibly abdominal distention/shock; + guiac test = bowel ischemia, poor prognostic sign

55. Tx of malrotation -Surgery ASAP56. Tx for SCD induced priapism -Sedation57. Dz that SCD can mimic if there is -Appendicitis

abdominal pain 58. When is frontal bossing seen? -Beta–thal or some other hemolytic process

that requires rapid hematopoesis59. Tx for beta–thal -Serial transfusion + chelation therapy

(desferoxamine) b/c of fe overload60. Consequence of Fe overload -Hemochromatosis61. Complications of G6PD deficiency -RBCs are destroyed but there is increased

amounts of Hb liberated in the process ––> Hb–uria

62. Findings in classic hemophilia -Bleeding problems + hemarthrosis63. Battle's sign -Basilar skull fx that leads to bleeding/bruise

behind the ear64. Mechanism behind bell's palsy in neonates -Forceps deliver... usually resovles65. Social Milestones at:

a. 5 yo -Competitive games; understands rules and abides by them

b. 4 yo -imaginative playc. 3 yo -group play\nsharesd. 2 yo -parallel playe. 18 mo -plays around other

childrenf. 12 mo -comes when

called; cooperates with

dressingg. 9 mo -pat–a–cakeh. 6 mo -recognizes strangersi. 4–5 mo -enjoys observing

environmentj. 3 mo -reaches for familiar

objects/pplk. 2 mo -recognizes parentl. 1 mo -fixes on face

66. developmental dysplasia of the hip -abnormal relationship between head of femur and acetabulum ––> instabilility and

dislocation of hip joint develops 2ndary to lack of contact of acetabulum and femur during intrauterine devevlopment

67. Dx of developmental dysplasia of hip -u/s see "false acetabulum" in lateral ileum68. Tx of developmental dysplasia of hip -Pavick harness (keeps hip abducted and

flexed), or body casting on older pts69. Complications of developmental -AVN (avasc nec.), degen arthritis of hip

dysplasia of hip 70. Metatarsus adductus -Dorsiflexion and plantarflexion are

UNRESTRICTED (diff from clubfoot); heels go out and toes go in

71. Tx of metatarsus adductus -Stretching or a brace \nsurgery not usualy needed

72. idiopathic talipes equinovarus (aka) -Congenital clubfoot \nmedial rotation of tibia, appearance fixed plantar flexion, inversion of foot, forefoot

adduction CANNOT DORSIFLEX (unlike metatarsus adductus)

73. Tx of clubfoot -Bracing; serial casting74. Legg–calve–perthes dz -AVN of femoral head; ischemic bone is

eventually resolved and re–ossification occurs ––> limp, pain referred to thigh/knee

75. What movt's are restricted in -Abduction\nflexion\ninternal rotationlegg–calve–perthes dz

76. Tx of legg–calve–perthes dz -Bracing, surgery, observation77. Complications of legg–calve–perthes dz -Collapse of femoral head78. SCFE -Gradual or acute separation of proximal

femoral growth plate \nfem head slipps off of femoral neck and rotates inf–post position (Slipped Capital Femoral Epiphysis)

79. Etiology of SCFE -Common during puberty, could be hormonal80. Presentation of SCFE -Limp, px in hip + groin, px referred to knee

81. Dx of SCFE -Plain film frog–leg, lateral position82. Tx of SCFE -Goal is to prevent further misallignment ; pin

fixation is done acutely -Chronic cases require osteotomy

83. when does osgood schlatter dz occur -During growth spurt, in teens84. Osgood schlatter dz -inflammation, swelling and tenderness

over tibial tuberosity 2ndary to tendonitis of distal insertion of infrapatellar tendon

85. tx of osgood schlatter -Conservative, supportive management86. complication related to achondrodysplasia -Small foramen magnum seen in

homozygotes ––> brainstem compression87. What is achondrodysplasia -d/o of cartilage calcifications and

remodeling88. Why children have an inc risk of fx -Tendons and ligaments are stronger than

bones so in kids injuries often lead to fx when they would only cause sprain in adult

89. Types of fx -Spiral (twisting forces on tibia during fall) ; epiphyseal fx (use salter classification) ; stress fx (hairline crack from repeated activity) ; torus fx (at metaphysis)

90. Nursemaids elbow -Subluxation of radial head91. Define upper airway -Nose ––> carina92. Sx of upper airway dz -inspiratory stridor ; tachypnea; respiratory

distress93. Choanal atresia -Most proximal abnormality of airway ;

bony or membranous septum btwn 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

94. Complications of long–term intubation -Subglottal stenosis95. Laryngeal or tracheomalacia -floppiness that closes off airway

(RAMROD)96. How to confirm dx of laryngeal or -Bronchoscopy

tracheomalacia 97. Ddx for wheezing and respiratory distress -Asthma\nbronchiolitis, foreign body

Aspiration, GERD, TE fistula, vascular sling98. Pathophysiology of sx in cf -Cftr is abnormal ––> altered cl channel, Cl-

stays in cells and Na+/water enter the cell to maintain osmotic balance ––> viscous secretions

99. GI effects in cf -Pancreatic insufficiency, bowel obx, rectal Prolapse, dm, cirrhosis, lage bulky smelly stools, later in life stools ––> distal

obstruction100. Pathognomonic finding in cf -Meconium ileus101. Tx of cf -Chest pt, exercise, frequent cough,

recombinant human DNAse given through nebs to break down thick mucous complexes

102. other than respiratory infx, what are -Hemoptysis (>500 cc/d = emergency) tx w some other complications of cf embolization\nspontaneous ptx (1/2 will

recur unless sclerosis is performemd, but if that is done then transplant is very difficult)

103. Definition of recurrent abdominal pain ->3x in 3 months104. Mesenteric lymphadenitis leads to -Persistent pain after infx105. GI complication from HSP -Ileal–ileal intussusception106. Sx of HSP -Non thrombocytopenic palpable purpura in

dependent areas, an IgA mediated vasculitis involving GI, skin, joints, and kidneys

107. Tx of hsp -Steroids108. What is HSP usually preceded by -URI109. GI sx of HSP -Spasmodic pain, ielus, vomiting, Upper

GI/lower GI bleed110. Embryology behind omphalocele -When midgut loop fails to return to

abdominal cavity; see light gray shiny sac protruding from base of umbilical cord

111. Embryology behind malrotation of midgut -Midgut undergoes partial rotation and ––> abnormal position for abdominal viscera, can be assoc with volvulus ––> compromised blood flow and gangrene

112. Gastroschisis -Weakness in abdominal wall ––> herniation of bowel through the rectus muscle, usually to the right of the umbilicus

113. Clinical features of malrotation -Bilious emesis, possible abdominal distension, X-ray shows gas in stomach, but no gas in intestines

114. Risk factors for developing GERD in babies -Prematurity, esophageal dz, obx lung dz, overdistension of stomach from overeating, meds (theophylline)

115. Dx of GERD -pH probe placement in esophagus or ugi endoscopy, barium swallow to confirm nml anatomy

116. Tx of GER/GERD -Small frequent feedings, keep head up for 20mins p eating, thicken feeds with cereal, metoclopramide (increases gastric

motility), h2 blocker or ppi\nlast resort: nissen fundlopication (fundus of stomach is wrapped around distal esophagus to increase les pressure)

117. Tx of diarrhea in children -If no serious complications, feed through the diarrhea ––> decreased denudement and faster return to nml stooling patterns; don't give antidiarrheals b/c ––> toxic megacolon, unless salmonella, shigella, c diff, or parasites

118. When to do w/u for diarrhea in a child -In infant <3mo, do blood cx ,0–12 mo, do stool cx; do blood and stool cx if >5d of enterocolitis or salmonella exposure; any infant with + stool dx looking toxic or + blood cx should be eval for pyelo, meningiits, pna, osteo

119. When should a pt with diarrhea be ->5% dehydration and can't rehydrate admitted to hospital effectively at home 120. Obstipation and complications -no bm, if after neonatal pd, #1 cause is

voluntary witholding\ncan be caused by pain, on defacation ––> fear for defecation ––> further retention; voluntary holding increases distension of rectum, decreases rectal sensation, requiring increased amounts needed to receive urge

121. Sx of hirschprung's -h/o diarrhea, fecal spotting alternating with constipation

122. Effect on incresaed bll on bm -Constipation123. How to tx functional constipation -incresae fluid, decrease junk food, increase

fiber, increase ingestion of undiluted juice124. Pathophys of hirschsprung's -Failure of ganglion cells of myenteric

plexus to migrate down colon in utero therefore distal colon is tonically contracted and there is obx \nusually limited to rectosigmoid colon

125. When to suspect hirschsprung's -In any infant who doesn't pass meconium, then ––> bilious vomiting, abdominal distension, and poor feeding

126. Tx of hirschsprung's -diverting colostomy with bowel that contains ganglion cells aganglionic segment is removed by pulling ganglionic segment through rectum

127. Sx of meckel's diverticulum -remnant of vitilline duct w/i 2 inches of

ileocecal valve; bleeding from divertic; melena, obx (from intussusception), diverticulitis

128. Tx of meckel's diverticulum -Surgical resection129. Dx of meckel's divertic -Technetium–99 scan p h2 antagonist to

locate hemorrhagic cells130. Encephalocele -Projection of cranial content through bony

skull defect in occiput ––> severe mr, sz, mov't do

131. Myelomeningocele -Protrusion of neural and meningeal tissue132. Meningocele -Meninges protrude133. Spina bifida II is associated with -Chiari II malformation134. Complications of spinal bifida -Caudal end of cord is tehtered to distal

spine and can't ascend to adult position ––> scoliosis, sphincter dysfxn, LE deformities

135. Non–communicating hydrocephalus -Block in exiting ventricles; above blockage, ventricles are big

136. Causes of non–communicating -Secondary to narrowing at 4th hydrocephalus ventricle/aqueduct or malformation at

posterior fossa; chiari II malformation; spina bifida occulta

137. Communicating hydrocephalus -Subarachnoid villi are dysfunctional/obliterated

138. Sx of motor cp -Fixed lesion in immature brain –> nonprogressive d/o of mov't 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

139. Sx of extrapyramidal cp; etiology -From basal ganglia damage ––> choreoathetoid mov't, postural ataxia, spasticity; kernicterus, there is usually some sort of brain insult

140. What GCS is required for a head ct to -<12 be obtained 141. #1 cause of ICH (intracereb. Hemor.) in kids -AVM (arteriovenous malformation) what meds are associated with pseudotumor -Tetracyclines; corticosteroids cerebri 142. Define Encephalopathy -Generalized cerebral dysfxn, MS change,

disorientation143. Risk factors for developing retinopathy -bw <1250 g; age <32 weeks; mechanical of prematurity ventilation; need for supplemental o2 144. Causes of Leukocoria -Retinoblastoma (––> death and visceral

mets in all cases); cataracts (most common cause); retinopathy of prematurity

145. Amblyopia -Visual impairment not corrected by glasses and not due to an ocular lesion; often from strabismus

146. Tx of amblyopia -Occlusion of better seeing eye forces development of affected eye and visual cortex of affected eye

147. At what age will tx not be successful -After 8yo in ambylopia 148. Tx of retinoblastoma -Enucleation (removal of the affected eye);

radiation tx\nchemo149. Complications of neonatal cataracts -If not removed by 3–4 months irreversible

amblyopia150. Course of retinopathy of prematurity -Most regress spontaneously; if not,

cryotherapy can be performed to reduce progression; are still at risk for amblyopia even if treated

151. Dacryostenosis -Congenital nasolacrimal duct obstruction; causes overflow tearing – 6% of neonates; 2ndary to failrue of distal membranous end of nasolacrimal duct to open

152. Tx of dacryostenosis -Probing of nasolacrimal duct at 12–15 mo; although most resolve spontaneously by 1 yo (in 96% of infants)

153. Ophthalmia neonatorum -Conjunctivitis occurring in first month of life; p/w eyelid edema, conjunctival hyperemia and ocular d/c

154. When is it normal for there not to be tears -In the first few weeks of life155. Age of onset of gonococcal ophthalmia -2–4 days neonatorum

a. Clinical features: Eyelid edema; chemosis; purulent d/cb. Complications: sepsis; meningitis; arthritis; corneal ulceration; blindnessc. Tx: Topical erythromycin; IV cefotaxime; tx parents

156. Age of onset of chlamydia ophthalmia -4–10 days neonatorum

a. Complications: Corneal scarring \nPNAb. Tx: Oral and topical erythromycin (despite the risk of developing pyloric stenosis) ; tx parents

157. When should steroid containing be -If hsv–1 is suspected etiology \ncan make avoided in conjunctivitis dz worse158. Hordeolum -Acute infection of sebacous tarsal glands;

staph aureus is usually cause159. Tx of hordeolum -Warm compresses

160. Chalazion -area of sterile lipogranulomatous reaction within meibomian glands that can enlarge; can be chronic and recurrent

161. Is periorbital or orbital cellulitis -Orbital cellulitis an emergency 162. Sx of periorbital cellulitis -skin around eye is indurated, warm, and

tender; no eye pain; may have si/sx of sinus infx

163. Sx of orbital cellulitis -Severe pain with eye movement proptosis; vision changes; decreased ocular mobility

164. Dx of orbital cellulitis -CT scan165. Organisms to cover with orbital cellulitis -Strep; h. flu; m. cat166. Tx of periorbital cellulitis -IV ABX; can ––> meningitis, tx agressively

with Vanco, PCN, 1st gen cephalosporin167. Orgs to tx in periorbital cellulitis -Strep; h. flu; m. cat168. Features of fragile x syndrome -Hyperactive; Mental Retardation; large

body; long face; prominent jaw and ears; thickened nasal bridge; large testes; +/– autism

169. Facial features of xyy -Long asymmetric ears; incresaed length: breadth in hands, feet, and cranium

170. Nutritional deficiencies in goat's milk -Decreased Vit D, Fe2+, folate, B12171. Infectious dz associated with drinking -Brucellosis raw cow's milk 172. What supplements should moms receive -B12 to prevent buildup of MMA if they are vegan 173. Effects of excess Vit D -Hypercalcemia; azotemia; poor growth;

n/v/d174. Vit E deficiency in premies leads to -Hemolytic anemia175. B1 defic –Beriberi; (neuritis, edema, chf);

hoarseness; anorexia176. B2 deficiency –Photophobia; cheilosis; glossitis; corneal

vascularity177. B3 deficiency -Pellagra (dermatitis, dementia, diarrhea)178. Pathophys of primary hypophosphatemia -Defective po4 resorption\nno conversion

of 25–vit D ––> 1,25–vit D in proximal tubules

179. Lab studies in primary hypophosphatemia 1. Ca2+ (LOW)2. PO4

2- (LOW) 3. Alk phos (HIGH)

180. Clinical findings in primary -Smooth LE bowing (not angular, as is seenhypophosphatemia in ca defic) \nintraglobulin dentin

deformities (ca deficiency ––> enamel defects) \ncoarse trabecular bone and fraying

181. Somogyi phenomenon -Nocturnal hypoglycemia manifested as night terrors, early am sweating, then later has hyperglycemia, ketonuria, glucosuria (sugars increase because of glucagon release)

182. What is an absolute contraindication -if first dose ––> encephalophaty or for DTaP encephalitis183. Clinical presentation of pb poisoning -Emotional lability; abdominal pain; achy

bones; intermittent vomiting and constipation

184. At what BLL should tx be initiated ->45185. Clinical presentation of acute hg poisoning -GI pain; fever; chills; HA; visual changes;

cough; cp186. Clnical presentation of chronic hg poisoning -Gingivostomatitis; tremor; neuropsych

features187. Clinical presentation of acute -N/V/D, abdominal pain; 3rd spacing; arsenic poisoning Hemorrhage; Hypovolemic shock; vtach ;

QT prolongation188. Narcosis -Deep stupor, unconsciousness189 Clinical presentation of cerebral palsy -Postural hypotonia; failure to reach for

toys; gross and fine motor delay; cognitive and sensory deficits

190. Complications of cerebral palsy -Seizures and mental retardation191. Differences in etiology for quadriplegia -Quadriplegia: UMN damage; Paraplegia: and paraplegia LMN or spinal cord damage 192. Most likely organisms PNA in CF -Staph or Pseudomonas193. Clinical presentation of meconium ileus -Vomiting; abdominal distension; distended

bowel loops; "soap bubble" on Axial X-ray194. Pathophys of meconium ileus -Begins in utero ––> underdeveloped distal

lumen195. Tx of meconium ileus -Surgical emergency; gastrograffin enema196. Vitamin A deficiency -pseudotumor cerebri (among other things);

will see bulging fontanelles, HA, n/v; may be seen as first presenting sign in a pt with CF

197. What effect does phenobarb -It improves it by increaseing gluocoronyl have on jaundice transferase

198. Cancer that is associated with (germline) retinoblastoma 1. Osteosarcoma (MC) 2. Melanoma 3. Squamous cell ca199. Conditions associated with aniridia -Congenital glaucoma; surge–weber;

marfan; neurofibromatosis200. Presentation of malrotation -Obstruction typically normal for first few

days of life than malrotation worsens ––> abdominal fullness, especially in RUQ ––> bilious vomiting ––> ischemia and necrosis

201. #1 malrotation -Volvulus202. Pathophys of volvulus -cecum fails to move to RLQ 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 obx

203. Presentation of children with 2o HTN -HA; epistaxis; visual sx; easy fatiguability204. Describe how biliary atresia can -From scarring and inflammation of occur post–natally intrahepatic or extrahepatic biliary ducts;

etiology is unclear 205. Describe findings of prenatal biliary atresia -Gall Bladder is absent206. Sx of congenital toxoplasmosis -Chorioretinitis; hydrocephalus; intracranial

calcifications207. Complement levels in post–strep -Decreased C3 glomerular nephritisn 208. What are "currant jelly stools" -bloody stools; indicative of intussusception209. Tx of intussusception -Barium/air enema210. Germinal matrix -Embryonic tissue present near caudate

nucleus, often gets damaged by hypoxia/ischemia; #1 place for intraparenchymal bleed

211. Progression of intraparenchymal bleed -Blood can flow into ventricles w/in 3 days in a newborn of life212. What blood vessels are damaged in -Bridging veins shaken baby syndroem 213. What BV are involved in SAH -Circle of Willis214. Signs of SVT -HR 220–270\nno p waves215. Pathophys of strawberry hemangioma -Vascular tissue fails to communicate with

adjoining tissue; enlarges ––> raised tumor216. MCC for PNA in a child > 6 yo -Mycoplasma; S.Pneumoniae217. MC orgs in peritonsillar abscess -Anaerobes\nGAS218. Physical findings of patau syndrome -Forebrain fails to develop;

holoprosenceph; midface developmental abnormalities; abnml genitalia; severe MR

219. Which chromosomes are associated with a. Edwards Edwards Edwards = Election age (18)b. Patau Patau = Puberty age (13)

220. Porencephaly -Cyst/cavity in brain that communicates with ventricles

221. Causes of euvolemic hyponatremia -SIADH; glucocorticoid deficiency; hypothyroid; water intoxication

222. VACTERL -Vertebral; Anal; Cardiac; Trach; Esophageal; Renal; Limb

223. Gastrografin -Way to dx meconium ileus; is demonstates unused microcolon and pellets of meconium higher up; can draws water into lumen and meconium gets unplugged (dx and tx)

224. Features of neonatal listerosis -Respiratoyr distress at 5 days; meningitis225. Clinical presentation of congenital -Constipation; jaundice; ftt; enlarged hypothyroid fontanelle; umbilical hernia226. Presentation of neuroblastoma -Asymptomatic abdominal mass; horner's ;

dancing eyes; dancing feet; blueberry muffin lesions; HTN

227. Patau's or Edwards syndrome or both:Microcephaly -patauProminent occiput -edwardsNarrow forehead -edwardsMicrophthalmia -patauCutis aplasia -patauMicrognathia -edwardsLow–set malformed ears -edwardsCleft lip -patauCongenital heart dz -bothomphalocele -patauclenched hands with overlapping fingers -bothrocker bottom feet -edwardspolydactyly -pataupolycystic kidney dz -patauhorseshoe kidney -edwardscrytorchidism -patauagenesis of corpus callosum -patau

228. Genetic changes in prader willi -Paternal deletion, 2 defective maternal chromosomes

229. Physical appearance of pts with prader willi -obese; almond shaped eyes; downturned mouth; small hands and feet

230. Genetic changes in angelman's -Maternal deletion, 2 deffective paternal chromosomes

231. Physical appearance of angelman's -Large mouth; short stature; tiptoe walk; szs232. Galactosemia -Galactose 1–p builds up and accumulates

in liver and brain233. Complications of galactosemia -Increased risk of e coli sepsis; LD;

premature ovarian failure234. Tx of galactosemia -eliminate galactose foods from diet235. Complications of PKU during pregnancy -If diet not followed, baby can develop

microcephaly, MR, and congenital heart dz236. Physical features of homocystinemia -Marfan like appearance; dislocaed lens237. What effect does sepsis have on bilirubin -Disrupts the BBB so it can cause diffusion

of bili into the brain238. What effect does temp have on bili -Can cause bili dissociation from albumin239. Protocol for neonates whose mom -Mom has sx w/in 2 days of delivery or developed varicella infection just after delivery during end of pregnancy treat baby with

IVIG and acyclovir. -If > Amt of time no tx needed

240. Consequences of neonatal asphyxia -Cerebral edema; irritability; sz; cardiomegaly; renal and heart failure ; DIC; RDS

241. What is the APT test -Used to differentiae fetal from maternal blood

242. Which drugs are contraindicated for 1. lithium 2. cyclosporine 3. antineoplastic breastfeeding drugs 4. ergots 5. bromocriptine 6.

6. tetracyclines

243. Classic finding on X-Ray for necrotizing -Pneumanitis intestinalis enterocolitis244. Consequences of cold temparature -Increased metabolic rate in order to raise in a premie body temp, but their ventilation rate

increased as well; but, b/c of respiratory problems in premies they can't oxygenate enough so lactic acid accumulates ––> metabolic acidosis

245. Which is worse: ABO incompat or -Rh incompat Rh incompat

246. Lab findings in ABO incompat -Increased retic count; weakly + coomb's247. Lab findings in Rh incompat -strongly + coombs248. Pathophys of transient apnea of the newborn -immat. respiratory centers, esp in premat249. How to ID the underlying cause of -Iodine uptake scan congenital hypothyroid

250. Late development of clavicle fx during -Callous formation in anterior shoulder; 1 delivery... when does this occur? week251. Physical findings of subgaleal hemorrhage -Feels like cephalohematoma that crosses

midline; can lose 1/3 of252. Onset of gonococcal conjunctivitis in a newborn -DOL 2–5253. Onset of chlamydia conjunctivitis -DOL 5–14254. What effects does surfactant deficiency -Decreases both; R––>L shunthave on lung compliance and lung volume and cardiac effect255. Newborn w/mom + for HepB give: -Hep B Ig and HepB vaccine256. Effect of propanolol in utero -IUGR; decreased ability of asphyxiated new

born to incresae hr and CO; associated with hypoglycemia and apnea

257. When is surgery for cleft lip done -3 months258. When does transient tachypnea of new -DOL #3 born resolve 259. Significance of 5th finger polydactyly (in black infants)no consequence Significance of 5th finger polydactyly (in white infants)Can be associated with cardiac

abnormalities, must do an echo260 . Twin twin transfusion:

a. donor twin ––> oligohydramnios, anemia, ypovolemiab. Recipient twin ––> polyhydramnios, larger size (20% difference in body weight),

hyperviscocity, respiratory distress, hyperbili, hypocalcemia, renal vein thrombosis261. When does serum bili peak -DOL 3–5

Friedreich Ataxia – Wide based gait; dec. vib sense in LE; dec. reflexes in LE; atrophy of spinal cord along with cerebral atrophy; Autosomal recessive – excess trinucleotide repeats results in abnormal tocopherol protein; wheelchair bound by 25 dead by 35. DO GENETIC COUNSELING FOR PARENTS WITH ONE AFFECTED CHILD.

12 mo – “mama” “dada” follow simple commands with gestures15 mo - “mama” “dada” +3 OTHER WORDS, understand simple commands WITHOUT gesture18 mo – 5-20 words24 mo – combine words into short sentences and half understood by stranger36 mo – 1000 words; 75% understood by stranger, 3-5 word sentences

Thalassemia – Absent or deficient >or= 1 alpha or beta-globulin chains of Hb

Factor V Leiden – Autosomal Dominant w/incomplete penetrance. Prone to blood clots. V activates X to form thrombin. In def. pts. Protein C cannot cleave the leiden form for factor V leading to hypercoagulability (potentially).

OBXObstruction

-Pathophysiology of Reye syndrome: Swelling of mitochondria

-Points for DKA tx: IV insulin 0.1 U/kg/hr +/- bolus; Add dextrose when glucose<250; Replace HCO3- if pH<7.2; When ketones get clear or HCO3->20: stop IV n give SQ insulin

-Ant mediastinal mass: Thymoma, thyroid CA, Teratoma, T cell lymphoma

-Lacunar cells: Hodgkin’s lymphoma nodular sclerosis type

-Kid w/ known cardiac defect now presents w/ fever, seizure and focal neurologic deficit : brain abscess; dx by brain CT, tx is surgical drainage and IV antibiotics. If abscess <2.5cm n pt is stable: antibiotics & observe

-70-75% of kids w/ HTN have renal etiology so check BUN & creatinine

-Intussusception: dx & tx is barium enema-Meconium ileus: dx & tx is gastrografin enema

-Contact dermatitis covering 15-20% of body surface: tx is PO steroids

-Infant w/ sinus tachycardia (QRS complexes -Bag of ice on face for 5 secw/ no preceding p waves) initial best step:

-Tx of peritonsillar abscess: -IV antibiotics (PCN; if allergic: clindamycin & metronidazole) & needle aspiration in ER setting

-Patau syndrome or Trisomy 13 : -Associated w/ holoprosencephaly (failure of forebrain to dev. Into 2 hemispheres)

-Infant abdominal difficulties & CF TOW: -Meconium ileus

-Alpha drugs given for nasal congestion can -Rebound congestion & rhinits have SE of: medicamentosa

-William syndrome: Overfriendly pt w/ supravulvular aortic stenosis-DiGeorge syndrome: Associated w/ truncus arteriosus & total anomalous pulmonary venous

return

-Surgery for cleft lip prerequisites -Rule of 10 (age10wks, weight 10 lb, Hb10)

-Tx for tet spell of TOF: -Knee chest position, phenylepherine drip, oxygen

-To know if TOF pt is in trouble: -Check O2 oximetry (E.g., pH 7.38> ok, but if pt has metabolic acidosis> means not enough O2 to tissues)

-Normal newborn Hb 50 Polycythemia >65. -Conginetal cyanotic disease of polycythemia (twin-twin transfusion, Intrauterine hypoxia, DM infants) mostly asx but can get stroke.

-Dx : Heel stick blood. -Tx: Partial exchange transfusion (taking whole blood out n putting in saline)

-Reactive Mantoux and positive CXR in 2 yr old, next step? Early morning Gastric aspiration to id acid fast bacilli swallowed at night.

-As a general rule gm-ve organisms need to be covered with 2 antibiotics. E.g, CF pt w/ pneumonia (suspected pseudomona infect) needs 2 IV drugs w/anti-pseudomonal activity like ceftazidime & tobramycin; an alternative combo is ticarcillin & tobramycin. Adjunctive tx w/chest physical therapy & bronchodilators is also used.

-Precocious puberty w/ normal ht & wt, next step? XR of head & wrist

-Tx of hypophosphatemic rickets: combined oral phosphate & 1,25-dihydroxyvitamin D3

-Short PR interval & delta waves w/ slow QRS upstroke : Wolf-Parkisnson-White syndrome. Tx: ablation of bypass tract

-Neuroblastoma: small round cell tumor w/ Homer-Wright Rosettes

-sickle cell pt w/ dilute urine & dehydration : think secondary enuresis

-Decrease C3: SLE, Membranoproliferative GN, Post strep GN

-Alport syndrome: X linked D, AD, mutations; biopsy> glomerular sclerosis, lamellation of basement membrane (onion layers w/breaks)

-MCC of non-anion acidosis in kids: diarrhea

-Bartter syndrome: (opposite of renal tubular acidosis type 4) hyper-renin, hyperaldosteronism, hypokalemia, normal BP, Jexta-glomerular apparatus doesn’t work, hypocholeremic metabolic acidosis

-Most common cause of ARF in kids: Hemolytic-Uremic syndrome

-No stool pass in the nursery: think CF, kid of DM (microcolon), Hirshsprung

-All kids w/ rectal prolapse should get a sweat chloride test

-Werdnig Hoffman: Autosomal Recessive, survival motor neuron gene (SMN) 5q13

-Pes cavum: high arched foot, peroneal muscle atrophy, recurrent ankle sprains

-Allelic expansion ( I allele has too many copies) seen in: Fragile X synd, myotonic dystrophy, Huntington’s chorea

TEMPCystic fibrosis flareup tx - IV Ceftazidime and gentamicin to cover P. aeroginosa

Intussuception - Most common cause of intestinal obstruction in first 2 yrs of life-Red currant jelly stools, severe sudden onset abd pain

Meckel's diverticulum - Presents at 2 yo w/ painless rectal bleeding

Duodenal atresia - Bile stained vomitus, no abd distension, dehydration, Down syndromeTx: decompress w/ NG tube, correct electrolytes+ surgery

Tracheoesophageal fistula - Presents in infancy w/ coughing, vomiting, gagging after feeding; Resp distress and frothy bubbles in oral cavityVACTERL association

Malrotation - Distended abdomen w/ dilated loops of bowel

Ewing's sarcoma - *Onion skin periosteal reaction!*-Lytic, central and accompanied by endosteal scalloping-Moth eaten mottled appearance + extension into soft tissue.

Osteomyelitis - Central lytic bone defect w. sclerosis= Brodie's abscess

Chondrosarcoma - Not common as 1 tumor-Mostly in *older people*-Fusiform defect w/ scalloping

Fibrosarcoma - Malignant spindle cell neoplasm in ??*30-60 yo* presenting as painful mass

Manifestations of CF - anemia, recurrent infections, rectal prolapse, clubbing, hemoptysis, heat intolerance, wheezing, steatorrhea

Most common mutation in CF - Deletion of a 3base pair encoding for phenylalanine in the CFTR gene located obn chromosome 7

Meconium ileus - Diagnostic for CF Obstruction of the small intestine in the newborn caused by impaction of thick, dry, tenacious meconium.

Chlamydial conjunctivitis - Most common causative agent of neonatal conjunctivitis Presents few days-->weeks post birth-Can present w/ pneumonia w/ rales, but no fever and no wheezing- May present w/ comjunctivits as well as pneumonia

Gonococcal conjunctivitis - Purulent eye discharge between 2-5 days post birth

Pertussis prevention - All close contacts should be given erythromycin for 14 days *regardless of age, immunisation or symptoms*

RSV infection - Increase risk of asthma later in life

Bronchiolitis - First wheezing episode associated w/ a URI

Failure to pass stool in first 24 hrs of life - Meconium ileus

Epinephrine 1= arteries constrict2= Bronchiles relax 1 agonist that vasoconstricts to increase BP2 agonist- SM relaxation to counter bronchospasm

Night Terrors - seen between ages 2-12Occur during REM sleep and are caharcterised by fear, crying, ↓ consciousness, amnesia

Pyelonephritis - fever, chills, N/V, flank pain, suprapubic pain, CVA tendernessTx: hospitalisation and IV antibiotics

Acquired torticollis - relatively common in kids, usually caused by URI, minor trauma, cervical lymphadenitis, or retropharyngeal abscess, first step is xray to rule out cervical spine injury

Treat Meningitis in adolescents d/t DETERIORATION - SHiN : N. meningitidis, S. pneumo, H. flu*N. meningitidis:*-QUICK DETERIORATION, associated rash/mucosal hemorrhage+ DIC (thrombocytopenia,↑ PT/PTT, bleeding from IV sites)Tx: Ceftriaxone 2g IV and Vanc (to cover resistant S. pneumo)-Get an LP (CSF glu low; protein ↑), Blood culture, CT scan; chemoprophylaxis of close contacts

Growth in infants Weight: Double by 5 months; triple by 1 yrHeight: Double by 4 years; triple by 13 yrsOther: Say 1 or 2 words; walk at least dependently

Hypophasphatemic Rickets - X-linked dominant. - Deficiency in PHEX. Messed up bone formations.

MCC of short stature and pubertal delay - Constituional grwoth delay: dealyed growth spurt, delayed puberty, delayed bone age

Viral meningitis - Nothing in Gram stain; fever+ URI sx; Normal glucose (40-80) and protein (10-30); Increased lymphocytes; Tx is supportive and sx will resolve in 7-10 daysCommon cause: echo, non-polio, cocksackie

Fetal alcohol syndrome - *MCC of MR in children*; - Facial hypoplasia, epicanthal folds, palpebral fissures, heart abnormalities, long philtrum, thin upper lip; normal karyotype (ruling out Down syndrome)

Congenital rubella - Hearing defects, Cataract defects + CHD defects (PDA), microcephaly(CataractHeart&HearingDefects-CHD)

Vit D deficiency rickets - Thickening of bonesWide open anterior fontanelle, rachitic rosary, craniotabes, rachitic rosary, thickening of the lower end of the long bones

Spondylolisthesis - forward slip of vertebrae (usually L5 slips over S1) that usually manifests in preadolescent children w/ back pain, a palpable step off, possible urinary incontinence

Iron poisoning - Symptoms: Vomiting & Diarrhea (often bloody) - Massive blood and fluid loss; Remission (may be asymptomatic for up to 12 hrs); Shock, seizures, Liver failure and deathTx: deferoxamine

Risk factors for neurological damage from hyperbilirubinemia 1. Hypoalbuminemia- Having a decreased albumin, means there will be more free unconjugated bilirubin which would normally bind to albumin. More free UCG bili-->free bili in body which can easily cross BBB to cause neurological damage2. Neonatal sepsis: Sepsis makes the BBB more crossable, and as such, there is an increased chance for neurological damage in such infants3. Metabolic acidosis decreases bilirubin binding, and as such, this free bili can cause an increase in neurological damage.

Choanal Atresia - Blocked posterior nostrils on one or both sides due to congenital septum. Feedings by mouth are either difficult or impossible b/c infant would have to breathe through their mouth. Crying makes respiration better b/c they use their mouths Surgery opens the airways.

When to not breastfeed - HIV infection, active pulmonary TB, malaria, typhoid fever, septicemia, women undergoing cancer tx. -Very few contraindications. Everything else, BREASTFEed

Apt test - Used to differentiate fetal blood (alkali resistant) from maternal blood (alkali reactant)Qualitative test used in simple cases of vaginal bleeding

Symptomatic congenital disease - Occurs when a mother has a primary CMV infection in the 1st trimester of pregnancy-->death of baby

Asymptomatic congenital disease - Occurs in the event of reactivation of CMV infection during pregnancy, maternal IgG passes transplacentally, protecting the infant from serious infection

Phenobarb on bili - Phenobarb-->↑ UGDP enzyme-->↑ conjugation of bilirubin-->↓ unconjugated bilirubin. Using phenobarb can help ↓ neonatal jaundice

Beck's triad - Test set for cardiac tamponade: ↓BP, muffled heart sounds over pericardium, distended neck veins

Scarlet fever and tx - fever, pharyngitis, sandpaper rash, strawberry tongue-Caused by Grp A strep-Tx: Penicillin V

Lyme Disease, RMSF, , trat - Give Doxycycline. If pt is <9yo, give Amoxicillin for 21 days

Angiofibroma - *Nasal obstruction, visible mass, frequent nosebleeds* -benign tumor of nasal mucosa composed of large blood vessels and fibrous tissue; only seen in adolescent males***-presents with profuse epistaxis

Measles - Cephalocaudal rash + leukopenia +thrombocytopenia-Vit A helps decrease mortality and morbidity

Biliary Atresia - light colored stools, hepatomegaly, increased direct hyperbilirubin (direct hyperbilirubinemia)Direct hyperbilirubinemia (>2) is an indication for evaluating neonatal jaundice even if the infant seems normal otherwise

Crigler Najjar - Absence of UDGP causing an increase in indirect bilirubin. These infants present in the first 24 hrs and die within the 1st yr of life(Gilbert's syndrome-milder form of this)

Whooping cough - Coughing spells lasting 20-30mns. These spells are so severe that they can cause rectal prolapse, epistaxis, or even pneumothoraces-May worsen w/ eating/drinking cold liquids

Parathyroid hormone - phosphate trashing hormone, increased phosphate in urine. in rickets, calcium and phophate pulled into blood, but phosphate is wasted in urine (hypocalciuria)

Milstones:Gross Motor2 - lifts head4 - front to back 5 back to front ( just think back to front is more muscle)6 - imagine the "6" as baby sitting9 - number "9" doesnt have good base so crawls11 - "11" has 2 good base so baby is able to stand/cruise/walk12 - now good base plus 2 legs can walk for sure (alone)15 - "5" is mirror image of "2" so baby can walk backwards2yr - u need to use both legs to go up/down stairs3yr - "TRI-cycle"4yr - HOPS 4 letters

Fine Motor"9ince12" = PINCER Before pincer comes raking which is more immature9 mo = non specific ( 3 finger) also nonspecific mom and dad12 mo = specific (2 fingers) also specific mom and dad 15 mo = 2block18 = 3blocks2yr = 6 blocks Also playstation controler 0 --> X --> Square --> triangle 3 4 5 6 yrs

Language/social2 coos and smiles because recognizes parents.4 coos and laughs6 babbles and anxiety, because recognizes strangers1 yr alone so plays ALONE/ follow ONE step command, One word besides mom dad2 yrs now 2 so PARALELL play, follows TWO step command, 2 word PHRASES3 4 5 6 number of sentences with yearsBowel control at 4 Pee control at 5

Birthweight x 2 every 6mo/ 1 yr Then x2 the next year = so x4 by 2yrs Lenght doubles every 4 yrs so x4 by puberty

ReflexPalmar is first to come ( US baby always has his palm close) Rooting is first to go Parachute always stays