Pedi Gi

Front 1

1. is direct or indirect bilirubin insoluble in water
2. which if present is ALWAYS pathologic?
3. which is most likely physiologic?

Back 1

1. indirected -> unconjugated w/ water soluble thing
2. conjugated or direct needs to be worked up r/o TORCHES
3. indirect -> due to ^ production or low conjugation

Front 2

1. direct or indirect is likely an obstruction?
2. location if jaundice reaches probably pathologic
3. best way to confirm yellow color is jaundice?

conjugated bile is not reabsorbed from the gut

Back 2

1. direct or conjugated
2. belly button
3. look at the sclerae if they are yellow

Front 3

baby should not be jaundiced in first 24 hours; see it in the first 2-3 days; peaks 3-4 days; better by 5-7 days ; gone entirely by 10-14 days

Seen 1st in face/head then spreads to trunk

Back 3

Unconjugated jaundice due to physiologic ( b/c ^RBC mass, decreased RBC life span, decreased conjugation, low perfusion/excretion), breastfeeding, breast milk, inheritied defects in it

Front 4

1. enzyme in breast milk that ^ physiologic jaundice
2. when does breastfeeding jaundice peak?
3. primary problem w/ it?

Back 4

1. breast, - beta glucuronidase uncconjugates bile so it is reabsorbed which ^ risk
2. DOL 3-5
3. ineffective breastfeeding leading to wt loss, hypovolemia, hypernatremia, jaundice

Front 5

-Jaundice risk factors: male gender, excesssive cutaneous bleeding or subQ, asian origin
-Severe hyperbilirubinemia RiFa: jaundice in 1st 24 hrs; ABO incompatibility direct coombs, asicanetc

Back 5

concerning if jaundice in 1st 24 hrs; serum bili increases more than 5 mg/dL/24 hrs; lasts longer than 2 weeks; direct bili is > 2 mg/dL at any time

Front 6

1. is breastfeeding jaundice due to breast milk or the lack of breast milk?
2. when does breast milk jaundice peak?
3. what might cause breast milk jaundice

Back 6

1. lack of it
2. 2nd to 3rd week of life
3. beta glucuronidase

Front 7

1. what occurs 1st breast feeding or milk jaundice
2. form of bilirubin that crosses the BBB
3. what does bilirubin bind to in the blood stream
may be free if binding sites are occupied or displaced by drugs

Back 7

1. breast feeding then milk
2. free unconjugated billirubin
3. albumin
-- bile may increase after albumin transfusion b/c of translocaiton of bili from hepatic to vasculature

Front 8

1. term for the depo and accumulation of unconjugated bilirubin in basal ganglia & brain stem
2. term for agnrml posturing of body often involving rigidity and hyperextension
3. treatment for hyperbilirbuinemia?

Back 8

1. kernicterus - chronic and permanet
2. opisthotonus
3. phototherapy - change bilirubin to lumirubin via fluorescent blue light

Front 9

Dubin-Johnson, Rotor, Gilbert, Crigler Najjar
1. which are unconjugated hyperbilirubinemia
2. which are conjugate hyperbilirubinemia
3. term for obliteration of entire biliary tree between porta hepatis and duodenum; 50% of pedi liver transplants

Back 9

1. Gilbert & Criggler-Najjar
2. Dubin-Johnson & Rotor; after Gilbert alphabetical
3. extrahepatic biliary atresia; more common in females usu extrahepatic; see splenic defects; portal vein abnormalities, CV & GU abnormalities

Front 10

1. If persistent jaundice > 2 wks of age; conjugate hyperbilirubinemia, persistent acholic stools (ltly colored), epatosplenomegaly think of?
2. findings on US?
3. Rx for this

Back 10

1. Extrahepatic biliary atresia
2. micro or absent gallbladder
exclude ddx: TORCH, sepsis
3. Kasai portoenterostomy - reconstruction of bile duct w/ small intestine b4 2 months of age

Front 11

1. most common genetic cause of liver disease in kids present w/ jaundice at 1-2 mo; acholic stools, hepatomegaly,
2. how is dx made?
Rx - supportive, liver transplant, avoid smoking

Back 11

1. alpha 1 antitrypsin deficiency
2. serum alpha 1 AT levels and liver Bx ww/ PAS+ diastase resistant globules

Front 12

1. synd. charac by noninflammatory encephalopathy & hepatic failure; dx of exclusion. Typically preceded by viral illness that appears to be resolving then worsen mb correlated w/ salicylate use

Back 12

Reye's syndrome: altered mental status, elevated intracranial pressure (ICP), increased liver enzymes and hyperammonemia, but are anicteric and have normal serum bili levels.
--> involves mitochondrial dysfxn

Front 13

1. what is ass w/ maternal polyhydraminos, Downs, and imperforate anus?
2. what does the sudan stain stain for
3. most common pancreatic insufficiency syndroem

Back 13

1. Annular pancreas
2. Fat - see red globules
3. cystic fibrosis - most common in caucasian pop

Front 14

1. chromosome for cystic fibrosis?
CF causes bronchiecstasis; GERD, pancreatic duct obstruciton steatorrhea; deficiency of ADEK poor growth, hepatic cirrhosi Meconium ileus; rectal prolapse

Back 14

1. chromosome 7 - inbetween cystic = 6 fibro-8

can see azospermia, congenital bilateral absence of vas deferens; dx w/ sweat clhlorid test

Front 15

1. term for obstruction of terminal ileum by thick meconium; colon distal to obstruction is often narrow and empty
-failure to pass meconium in first 24 hrs of life; see abdominal distention; bilious emesis

Back 15

1. meconium ileus 90% of cases associated w/ CF; pathognomic for CF

see a soap bubble appearance w/ microcolon; Rx w/ enema; N acetylcysteine

Front 16

1. how does texas screen for CF?
these kids need more food/energy; support w/ ADEK vitamins

2. primary cause o fdeath in CF?

Back 16

1. immunoreactive typsinogen test; ^ trypsinogen in blood 2ndary to blocked pancreatic ducts
2. Cardiorespiratory causes