GI Flashcards

1
Q

2week old male with intermittent bilous vomiting, on PE soft abdomen, ab x-ray normal

A

Malrotation

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2
Q

1month old baby presenting with jaundice, triangular facies, deep set eyes and cardiac murmur

A

Alagille syndrome

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3
Q
Which of the following cause neonatal cholestasis:
A. Hepa A
B. Hepa B
C. Hepa C
D. None of the above
A

D. None

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4
Q
Which of the ff is not usually seen in recurrent cholangitis in pediatric cholestatic liver: 
A. E. Coli
B. Pseudomonas
C. Acinetobacter
D. Klebsiella
A

Seen in recurrent cholangitis: e coli, pseudomonas, Enterococcus, Klebsiella

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5
Q

Identify the condition

Nonbilous vomiting, epigastric distension, abd pain
Radiograph: dilated stomach with characteristic beak sign near lower esophageal junction

A

Volvulus

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6
Q

Pressure of Portal hypertension

A

10-12mmhg

Normal 7mmhg

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7
Q

fetus can start swallowing at

A

12 wks aog

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8
Q

nutritive sucking for fetus at

A

34 wks

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9
Q

bilous vomiting ouccurs if obstruction is found

A

below 2nd part of duodenum

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10
Q

diagnosis of cyclic vomiting

A

ALL MUST BE MET
• At least 5 attacks in any interval, or a minimum of 3 episodes during a 6-mo period
• Recurrent episodes of intense vomiting and nausea lasting 1 hr to 10 days and occurring at least 1 wk apart
• Stereotypical pattern and symptoms in the individual patient
• Vomiting during episodes occurs ≥4 times/hr for ≥1 hr
• Return to baseline health between episodes
• Not attributed to another disorder

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11
Q

cyclic vomiting occurs at what age

A

2-5years old

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12
Q

normal stool output

A

5ml/kg/day

or 200g/24 hours in older kids

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13
Q

diarrhea definition

A

> 10ml/kg/day
or > 200g/24hr in older kids
<14 acute
14 days chronic or persistent

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14
Q

ion gap in secretory diarrhea

A

<100mOsm/kg

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15
Q

calcification of teeth occurs

A

3-4mo AOG

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16
Q

teeth abnormality that occurs in osteogenesis imperfecta
poorly calcified dentin
bluish in appearance

A

dentigenesis imperfecta

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17
Q

tongue becomes lacerated/amputated in neonates with natal teeth

A

Riga Fede disease

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18
Q

surgical closure of cleft lip can be done

A

3months

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19
Q

closure of palate usually done at

A

1yr old for speach

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20
Q
downward sloping palpebral fissures
colobomas
sunken cheekbones
blind fistulas between eyes and mouth
deformed pinnae
receding chin
large mouth
autosomal dominant with incomplete penetrance
A

treacher collins syndrome
mandibulofacial dystosis
franceschetti syndrome

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21
Q

characterized by incomplete development of the ear, nose, soft palate, lip, and mandible
autosomal dominant

A

goldenhar syndrome

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22
Q

organism assoc with dental caries

A

stretococcus mutans

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23
Q

associated with gingival hyperplasia

A

phenytooin
cyclosporine
some calcium channel blockers

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24
Q

most common congenital anomaly of esophagus

A

esophageal atresia

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25
if gap is more than __cm in EA with TEF | primary repoair not possible
3-4cm
26
most encountered foregut duplications
esophageal duplication cysts
27
dysphagia lusoria
dysphagia caused by obstruction either by aberrant right subclavian artery or right sided or double aortic arch
28
most common cause for esophageal obstruction
eosinophilic eosphagitis
29
treatment for achalasia
``` pneumatic dilattation or surgical (Heller) myotomy ```
30
characteristics of infant reflux
peak at 4months | resolves 88% by 12 months and nearly all by 24 months
31
GERD with neck controtions
sanfider syndrome
32
``` Vomiting Feeding problems Epigastric pain Atopy Esophagoscopy granular furrowed with white specks ```
Eosinophilic esophagitis
33
Foreign bodies Lodge at the
Cricopharyngeus | Upper esophageal sphincter, aortic arch or just superior to the diaphragm at the level of the LES
34
How are coins seen at esophagus xray | How is it different from tracheal foreign body
Esophagus: flat surface of coin on AP, edge on lateral Trachea: edge on AP, flat on lateral
35
Button batteries cause
Mucosal injury in 1 hour and involve all layers in 4 hours
36
What can be used to facilitate passage in distal esophagus
Glucagon
37
Foregut gives rise to
Esophagus Stomach Duodenum at level of insertion of common bile duct
38
Midgut gives rise to
Small and large bowel to level of mid transverse colon
39
Hindgut gives rise to
Rest of colon and anal canal
40
rapid growth of the midgut causes it to protrude out of the abdominal cavity through the umbilical ring during fetal development. The midgut subsequently returns to the peritoneal cavity and rotates ______ until the cecum lies in the right lower quadrant. The process is normally complete by the ____ wk of gestation.
Counterclockwise | 8th week
41
Migration of the neural crest tissue is complete by the ___ wk of gestation. Interruption of the migration results in ____
24th | Hirschsprung disease.
42
Carbohydrates, protein, and fat are normally absorbed by the ___
upper half of the small intestine
43
Most of the sodium, potassium, chloride, and water are absorbed in the ___
small bowel
44
Bile salts and vitamin B12 are selectively absorbed in the __
distal ileum
45
iron is absorbed in the ___
duodenum and proximal jejunum
46
Criteria for diagnosis hypertrophic pyloric stenosis include
pyloric thickness 3-4 mm, an overall pyloric length 15-19 mm, and pyloric diameter of 10-14 mm
47
Ultrasonography findings of pyloric stenosis
elongated pyloric channel (string sign), a bulge of the pyloric muscle into the antrum (shoulder sign), and parallel streaks of barium seen in the narrowed channel, producing a “double tract sign”
48
Associated with pyloric stenosis
``` eosinophilic gastroenteritis, Apert syndrome, Zellweger syndrome, trisomy 18, Smith-Lemli-Opitz syndrome, Cornelia de Lange syndrome erythromycin in neonates mostly female infants of mothers treated with macrolide antibiotics during pregnancy ```
49
Treatment pyloric stenosis
Ramstedt | Pyloromyotomy
50
Nonbilous vomiting Ab distention on first day of life Polyhydramnios low birth weight Large stomach on xray
Pyloric atresia
51
Ligaments tethering stomach
Gastrohepatic Gastrosplenic Gastrocolic
52
Associated with cmv vomiting, anorexia, upper abdominal pain, diarrhea, edema (hypoproteinemic protein- losing enteropathy), ascites, and, rarely, hematemesis if ulceration occurs Upper GI series show thickened gastric folds
Hypertrophic gastropathy
53
``` Bilous vomiting No ab distention Seen first day of life Polyhydramnios Double bubble on xrat ```
Duodenal atresia
54
Intestinal duplication in thorax
Neuroenteric cysts
55
typical Meckel diverticulum is a __ outpouching of the ileum along the antimesenteric border ___ from the ileocecal valve
3-6 cm | 50-75 cm (approximately 2 feet)
56
Diagnosis of meckels diverticulum
Meckel radionuclide scan with technetium 99m
57
Aside from antibiotics what else can treat bacterial overgrowth
Low dose octreotide
58
Msot common cause of lower intestinal obstruction in neonate | Predominant male or female?
Hirschprung | Male
59
Histopath findings in hirschprung
Absence meissner and auerbach plexus Hypertrophied nerve bundles High concentration of acetylcholinesterase between muscular layer and submucosa
60
Pathogens in hirschprung
Clostridium Staph Anaerobes Coliform
61
Currarino triad
``` Anorectal malformation (Imperforate or ectopic anus) Sacral bone anomaly Presacral anomaly (meningocele, teratoma,cyst) ```
62
How is rectal biopsy done
Suction no closer than 2 cm above from dentate line
63
Classic finding enema hirschprung
abrupt narrow transition zone between the normal dilated proximal colon and a smallercaliber obstructed distal aganglionic segment
64
compare the diameter of the rectum to that of the sigmoid colon How to know if hirschprung
because a rectal diameter that is the same as or smaller than the sigmoid colon suggests Hirschsprung disease
65
Most common cause of intestinal obstruction 3mo to 6yr
Intussusception
66
Possible lead point for intussusception
Meckel diverticulum, intestinal polyp, neurofibroma, intestinal duplication cysts, inverted appendix stump, leiomyomas, hamartomas, ectopic pancreatic tissue, anastomotic suture line, enterostomy tube, posttransplant lymphoproliferative disease, hemangioma, or malignant conditions such as lymphoma, or Kaposi sarcoma
67
Usual lead point intussusception post op
ileoileal
68
ultrasound findings of intussusception
tubular mass in longitudinal doughnut in transverse coiled spring on contrast enema
69
perforation with foreign body tends to occur at
physiologic sphincters (pylorus, ileocecal valve), acute angulation (duodenal sweep), congenital gut malformations (webs, diaphragms, diverticula), or areas of pre- vious bowel surgery
70
problem with battery ingestion
leakage of alkali or heavy metal | electrical discharges causes burns in intestine
71
gastric ulcer found in
lesser curvature of stomach
72
primary ulcers found in
duodenal
73
ulcer in response to burn
curling ulcer
74
ulcer in response to stress/shock/intracranial mass
cushing ulcer
75
increase gastric acid production
acetylcholine histamin gastrin
76
decrease gastric acid production
prostaglandin
77
h pylori produces
urease catalase oxidase
78
gastric hypersecretion by tumor | associated with MEN multiple endocrine neoplasia
zollinger ellison syndrome
79
ulcerative colitis localized to __
colon
80
blood mucus pus in stool | diarrhea
ulcerative colitis
81
extraintestinal manifestation of ulcerative colitis
pyoderma gangrenosum, sclerosing cholangitis, chronic active hepatitis, and ankylosing spondylitis
82
treatment for ulcerative colitis
sulfasalazine
83
idiopathic, chronic in ammatory disorder of the bowel, involves any region of the alimentary tract from the mouth to the anus.
crohn
84
diarrhea weight loss ab pain failure to thrive
crohn
85
tx for mild crohn
mesalamine
86
edema assoc with
protein losing enteropathy
87
perianal excoriation and gaseous abdominal distention
carbohydrate malabsorption
88
explosive watery diarrhea suggests
carbohydrate malabsorption
89
loose, bulky stools are associated with
celiac disease
90
pasty and yellowish offensive stools suggest an
exocrine pancreatic insufficiency
91
Stool pH and reducing substances for
carbohydrate malabsorption check for acidic stool and >2 reducing substances
92
quantitative stool fat examination
fat malabsorption
93
α1­antitrypsin
protein malabsorption
94
fecal stool elastase­1
exocrine pancreatic insufficiency
95
Breath hydrogen test
to identify the specific carbohydrate that is malabsorbed
96
serum trypsinogen in cystic fibrosis is
elevated early then declines 5-7 years old
97
shwachman syndrome | serum trypsinogen is
low
98
gold standard test for exocrine pancreatic function
duodenal aspirate
99
``` failure to thrive chronic diarrhea vomiting ab distention muscle wasting IDA nonresponsive to iron therapy epilepsy with bilateral occipital classifications ```
celiac disease
100
malignancy main cause of death in celiac disease
non hodgkin lymphoma
101
diagnosis if celiac disease
finding of villous atrophy with hyperplasia of the crypts and abnormal surface epithelium and circulatng igA celiac disease assoicated antibodies while on gluten then complete remission on gluten free diet
102
autosomal recessiev manifests as profuse watery secretory diarrhea light microscopy diffuse thinning of the mucosa with hypoplastic villus atrophy and no inflammatory infiltrate
congenital microvillus atrophy | or microvillus inclusion disease
103
treatment of congenital microvillus atrophy | or microvillus inclusion disease
octreotide
104
small intestinal mucosal biopsy is focal epithelial tufts (teardrop shaoed groups of closely packed enterocytes with apical rounding of plasma membrane presents as persistent watery diarrhea at first week infancy
tufting enteropathy
105
treatment of intestinal lymphangiectasia
restricting long chain fat and using formula with formula and MCT
106
diagnosis of intestinal lymphangiectasia
elevated fecal alpha antitrypsin clearance
107
``` caused by tropheryma whipplei weight loss diarrhea ab pain PAS positive microphages in biopsy ```
whipples disease
108
tx for whipples disease
cotrimoxazole
109
malabsorption of neutral amino acids, including the essential amino acid tryptophan, with aminoaciduria, photosensitive pellagra-like rash, headaches, cerebellar ataxia, delayed intellectual development, and diarrhea
Hartnup disease
110
Tryptophan malabsorption | causes urine to turn blue
blue diaper syndrome (indicanuria, Drummond syndrome)
111
characterized by the absence of high-density lipoprotein cholesterol premature coronary heart disease and accumulation of cholesterol in liver, spleen, lymph nodes (tonsils), and small intestine
Tangier disease
112
useful to differentiate between lack of IF and malabsorption of cobalamin
Schilling test
113
an infant can survive even if __ cm of bowel with ileocecal valve or __cm without
15cm | 20cm
114
t or f | jejunum resection is better than ileal resection
true
115
proximal 100-200cm of jejunum is main site of absorption of
carbohydrates protein iron water soluble vitamin
116
distal ileum absorption
vitb 12 | bile salts
117
net sodium and fluid absorption is at
ileum
118
duodenum and proximal jejunum absorption
``` calcium magnesium phosphorus folic acid iron ```
119
renal stones can occur in malabsorption due to
hyperoxaluria secondary to steatorrhea | calcium binds to fat and to oxalate so it's secreted out and oxalate levels rise
120
severe portal hypertension in liver disease leads to malabsorption due to
portal hypertensive enteropathy | poor absorption of nutrients
121
loss of deep tendon reflexes ophthalmoplegia cerebellar ataxia posterior column dysfunction
vit e deficiency
122
``` tubular nephropathy - high urine Ca and P rickets hepatomegaly failure to thrive fasting hypoglycemia cataracts increased glycogen levels autosomal recessive GLUT2 disorder - glucose transporter problem ```
fanconi bickel syndrom
123
treatment of fanconi bickel syndrom
vit d uncooked conrstarch electrolyte replacement
124
polyhydramnios presents first few weeks with severe diarrhea dilated bowel loops metab alkalosis hypo Cl, Na, K diarrhea tend to regress with age treated with PPI, cholestyramine, butyrate
congenital chloride diarrhea
125
``` polyhydramnios massive secretory diarrhea severe met acid alkaline stools hypoNa ```
congnital sodium diarrhea
126
``` weaned from breastfeeding anorexia diarrhea failure to thrive humoral and cell mediated immunodeficiency dermatitis neuro abnormalitites poor wound healing ```
acrodermatitis enteropathica zinc malabsorption
127
``` kinky hair seizuree hypothermia apnea cutis laxa hypopigmentation ```
menkes disease copper malabsorption
128
most common cause of diarrhea US
rotavirus and norovirus
129
pesticide | organophosphate poisoning
SLUDGEM (salivation, lacrimation, urination, defecation, gastrointestinal motility, emesis, miosis) increase acetylcholine activate parasympathetic
130
cholera and ETEC activate ___ to cause diarrhea
adenylate cyclase
131
diarrhea that have blood and leukocytes in stool | causes ab cramps, tenesmus and fever
``` salmonella shigella campylobacter yersinia enterocolitis EIEC or EHEC or shiga e coli vibrio parahaemolyticus ```
132
shigatoxin producing e coli
e coli o157:h7
133
treatment to minimal dehydration
<10kg 60-120ml for each stool/vomiting | >10kg 120-240ml for each
134
treatment for mild to mod dehydration
50-100ml/kg over 3-4 hours THEN <10kg 60-120ml for each stool/vomiting >10kg 120-240ml for each
135
treatment for severe dehydration
20ml/kg bolus THEN 100ml/kg over 4 hours ORS or D5 0.45Nacl at TWICE FM THEN <10kg 60-120ml for each stool/vomiting >10kg 120-240ml for each via NGT or D5 0.45%Nacl + 20meqkcl
136
WHO ORS
``` 75meq Na 75mmol glucose 20meq K 65meq Cl 10meq citrate 245mosm per liter ```
137
zinc in diarrhea
<6mo 10mg/day >6mo 20mg/day 10-14 days
138
racecadotril is
enkephalinase inhibitor
139
doc for shigella
cipro
140
doc for EPEC, ETEC, EIEC
cotri | cipro
141
doc for salmonella
ceftri
142
doc for yersinia
doxycycline
143
doc for campylobacter
azithromycin
144
doc for clostridium difficile
metro
145
doc for entamoeba
metro
146
doc for giardia
metro
147
doc for cryptosporidium
nitazoxanide
148
doc for blastocystis
metro
149
past history of AGE sensitivity to food allergens disaccharidase deficiency reinfection
postenteritis syndrome
150
``` exocrine pancreas hypoplasia neutropenia bone changes intestinal protein loss low serum trypsinogen ```
shwachmann diamond syndrome
151
recurrent ab pain defined as
3 epsiodes in 3 months with impairment of fxn
152
viscera innervated by
vagal | splanchnic
153
age of appendicitis | sex predominance?
12-18 | boys
154
___ location of appendicitis leads to slower course
retrocecal
155
single most reliable finding in appendicitis
localized abdominal tenderness
156
psoas sign for
retrocecal appendicitis
157
obturator sign for
pelvic appendicitis
158
pediatric appendicitis score
<2 unlikely >8 likely ``` fever 38c 1 anorexia 1 nausea 1 cough tenderness 2 RLQ tenderness 2 migration of pain 1 leukocytosis >10 1 PMN >7.5 1 ```
159
utz criteria to dx appendicitis
``` wall thickness >6mm luminal distention lack of compressibility complex mass RLQ fecalith ```
160
ct scan findings appendicitis
distended thick walled appendix inflammatory streaking of surrounding mesenteric fat pericecal phlegmon or abscess
161
mittelschmerz
rupture of ovarian follicle causes pain
162
pathogens implicated with appendicitis
``` bacteroides clostridia poptostreptococcus e coli psuedomonas enterobacter klebsilla ```
163
antibiotics in appendicitis
simply unperforated: cefoxitin | perforated: ampicillin, gentamycin, clinda or metronidaole)
164
``` Autosomal recessive uridine diphosphogluconurate glucuronosyltransferase (UGT1A1) problem Does not conjugate bili Kernicterus in almost all Jaundice first 3 days of life High unconjugated bili ```
Crigler najjar
165
Autosomal recessive uridine diphosphogluconurate glucuronosyltransferase (UGT1A1) problem Episodes of jaundice may be triggered by stress such as exercise, menstruation, or not eating High unconjugated bili
Gilbert disease
166
anomalies in sacrum assoc with anomalies in kidney/urinary tract
caudal regression
167
organisms in perianal abscess
e coli, klebsiella, staph, bacteroides, clostridium, veillonella
168
difference between internal and external hemorrhoids
internal hemorrhoids above the dentate line present as bleeding and prolapse; external hemorrhoids below dentate line present with pain and itching
169
rectal prolapse where all layers involved
procidentia
170
pathogen in pilonidal abscess
staph and bacteroides
171
hernias occur most at
1st year of life
172
highest risk of srtangulation hernia at
1st year of life
173
predmoinance hernia sex
male
174
testes start to descend by
28wks aog
175
completion of testes descent
28 to 36 weeks aog
176
__% of inguinal hernias occur on right side
60%
177
functional unit of pancreas
acinus
178
mitochondrial DNA mutation affecting oxidative phosphorylation, severe macrocytic anemia, thrombocytopenia, pancreatic insufficiency
pearson syndrome
179
exocrine pan- creatic de ciency, aplasia or hypoplasia of the alae nasi, congenital deafness, hypothyroidism, developmental delay, short stature, ectoder- mal scalp defects, absence of permanent teeth, urogenital malforma- tions, and imperforate anus.
johanson-blizzard syndrome
180
direct testing of pancreas
trypsin, chymotrypsin, lipase, and amylase obtained via triple lumen tube OR 72 hr stool collection for quantitative analysis of fat content is the gold standard for the diagnosis of malabsorption
181
most common etiologies of pancreatitis
blunt ab trauma, multisystem disease, biliary stones, drug toxicity
182
drugs that cause pancreatitis
valproic acid, l-asparaginase, 6 mercaptopurine, azathiprine
183
bluish discoloration around umbilicus in pancreatitis
cullen sign
184
bluish discoloration around flank in pancreatitis
grey turner sign
185
__ is test of choice for pancreatitis
lipase
186
labs associated with pancreatitis
elevated GGT amylase lipase bilis glucose, hemoconcentration, coagulopathy, hypoCa
187
causes of chronic pancreatitis
mutation in PRSS1 gene, cystic fibrosis, hyperlipidemia, hyperparathyroidism, ascariasis, autoimmune pancreatitis, juvenile tropical pancreatitis
188
normal liver size
4.5-5cm at 1week old, 7-8cm for boys and 6-6.5cm in girls at 12 years old
189
normal gallbladder length
1.5-5.5cm (average 3cm) in INFANTS and 4-8cm in adolescents
190
clinically apparent jaundice occurs at level
2-3mg/dL in kids, 5mg/dL in neonates
191
factors favoring ascites
decreased colloid vascular pressure, increased capillary hydrostatic pressure, increased ascitic fluid colloid pressure, decreased ascitic fluid hydrostatic pressure
192
hypoxemia, intrapulmonary vascular dilations, and liver disease
hepatopulmonary syndrome
193
utz finding of biliary atresia
small or absent gallbladder, nonvisualization of common bile duct, trangular cord sign
194
idiopathic familial intrahepatic cholestasis, lymphedemaof lower extremities
aagenaes syndrome
195
autosomal recessive, progressive degeneration of liver and kidneys, generalized hypotonia and retardation, hepatomegaly, renal cortical cysts
zellweger (cerebrohepatorenal syndrome)
196
increased iron deposition in liver heart and endocrine but no increase in iron stores of reticuloendothelial system, alloimmune disorder where maternal antibodies target fetal liver
neonatal iron storage disease or neonatal hemochromatosis
197
final step in bile acid synthesis, catalyzed by
conjugation with glycine and taurine; bile acid coA ligase
198
most common syndrome with intrahepatic bile duct paucity
alagille syndrome
199
broad forehead, deep set widely spaced eyes, long straight nose, underdeveoped mandible, cardiac , tubulointerstitial nephropathy, vertebral defects, short stature, pancreatic insufficiency, defective spermatogenesis
alagille syndrome
200
cardiac lesions associated with alagille syndrome
usually peripheral pulmonic stenosis, tof, pulmonary atresia, vsd, asd,coa
201
most common form of biliary atresia
obliteration of the entire extrahepatic biliary tree at or above porta hepatis
202
histopath biliary atresia
bile ductular proliferation, presence of bile plugs, portal or perilobular edena and fibrosis, basic hepatic lobular architecture intact
203
histopath neonatal hepatitis
diffuse hepatocellular disease with infiltration of inflammatory cells, bile ducts intact
204
kasai
hepatoportoenterostomy
205
best time to do kasai
8 weeks
206
autosomal recessive, defect in hepatocyte secretion of bilirubin glucoronide, liver histopath normal architecture but hepatocytes contain black pigment
dubin-johnson syndrome
207
autosomal recessive, degenrative changes in brain and liver, kayser fleischer rings in cornea, coombs negative hemolytic anemia
wilson disease
208
pathophysio of wilson disease
gene for biliary copper excretion and copper incorporation to ceruloplasmin defective
209
during hemolytic episodes in wilson disease, what are elevated
urinary copper excretion and serum copper levels are elevated
210
treatment of wilsons disease
penicillamine, decrease dietary copper
211
what are side effects of penicillamine
decrease vit b6, goodpasture syndrome, sle, polymyositis