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PPS - From Nelson's > GI > Flashcards

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
Q

if gap is more than __cm in EA with TEF

primary repoair not possible

A

3-4cm

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26
Q

most encountered foregut duplications

A

esophageal duplication cysts

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27
Q

dysphagia lusoria

A

dysphagia caused by obstruction either by aberrant right subclavian artery or right sided or double aortic arch

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28
Q

most common cause for esophageal obstruction

A

eosinophilic eosphagitis

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29
Q

treatment for achalasia

A 
pneumatic dilattation 
or surgical (Heller) myotomy
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30
Q

characteristics of infant reflux

A

peak at 4months

resolves 88% by 12 months and nearly all by 24 months

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31
Q

GERD with neck controtions

A

sanfider syndrome

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32
Q 
Vomiting
Feeding problems
Epigastric pain
Atopy
Esophagoscopy granular furrowed with white specks
A

Eosinophilic esophagitis

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33
Q

Foreign bodies Lodge at the

A

Cricopharyngeus

Upper esophageal sphincter, aortic arch or just superior to the diaphragm at the level of the LES

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34
Q

How are coins seen at esophagus xray

How is it different from tracheal foreign body

A

Esophagus: flat surface of coin on AP, edge on lateral
Trachea: edge on AP, flat on lateral

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35
Q

Button batteries cause

A

Mucosal injury in 1 hour and involve all layers in 4 hours

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36
Q

What can be used to facilitate passage in distal esophagus

A

Glucagon

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37
Q

Foregut gives rise to

A

Esophagus
Stomach
Duodenum at level of insertion of common bile duct

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38
Q

Midgut gives rise to

A

Small and large bowel to level of mid transverse colon

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39
Q

Hindgut gives rise to

A

Rest of colon and anal canal

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40
Q

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.

A

Counterclockwise

8th week

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41
Q

Migration of the neural crest tissue is complete by the ___ wk of gestation. Interruption of the migration results in ____

A

24th

Hirschsprung disease.

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42
Q

Carbohydrates, protein, and fat are normally absorbed by the ___

A

upper half of the small intestine

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43
Q

Most of the sodium, potassium, chloride, and water are absorbed in the ___

A

small bowel

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44
Q

Bile salts and vitamin B12 are selectively absorbed in the __

A

distal ileum

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45
Q

iron is absorbed in the ___

A

duodenum and proximal jejunum

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46
Q

Criteria for diagnosis hypertrophic pyloric stenosis include

A

pyloric thickness 3-4 mm, an overall pyloric length 15-19 mm, and pyloric diameter of 10-14 mm

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47
Q

Ultrasonography findings of pyloric stenosis

A

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”

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48
Q

Associated with pyloric stenosis

A 
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
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49
Q

Treatment pyloric stenosis

A

Ramstedt

Pyloromyotomy

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50
Q

Nonbilous vomiting
Ab distention on first day of life
Polyhydramnios low birth weight
Large stomach on xray

A

Pyloric atresia

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51
Q

Ligaments tethering stomach

A

Gastrohepatic
Gastrosplenic
Gastrocolic

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52
Q

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

A

Hypertrophic gastropathy

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53
Q 
Bilous vomiting
No ab distention
Seen first day of life
Polyhydramnios
Double bubble on xrat
A

Duodenal atresia

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54
Q

Intestinal duplication in thorax

A

Neuroenteric cysts

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55
Q

typical Meckel diverticulum is a __ outpouching of the ileum along the antimesenteric border ___ from the ileocecal valve

A

3-6 cm

50-75 cm (approximately 2 feet)

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56
Q

Diagnosis of meckels diverticulum

A

Meckel radionuclide scan with technetium 99m

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57
Q

Aside from antibiotics what else can treat bacterial overgrowth

A

Low dose octreotide

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58
Q

Msot common cause of lower intestinal obstruction in neonate

Predominant male or female?

A

Hirschprung

Male

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59
Q

Histopath findings in hirschprung

A

Absence meissner and auerbach plexus
Hypertrophied nerve bundles
High concentration of acetylcholinesterase between muscular layer and submucosa

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60
Q

Pathogens in hirschprung

A

Clostridium
Staph
Anaerobes
Coliform

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61
Q

Currarino triad

A 
Anorectal malformation (Imperforate or ectopic anus)
Sacral bone anomaly 
Presacral anomaly (meningocele, teratoma,cyst)
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62
Q

How is rectal biopsy done

A

Suction no closer than 2 cm above from dentate line

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63
Q

Classic finding enema hirschprung

A

abrupt narrow transition zone between the normal dilated proximal colon and a smallercaliber obstructed distal aganglionic segment

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64
Q

compare the diameter of the rectum to that of the sigmoid colon
How to know if hirschprung

A

because a rectal diameter that is the same as or smaller than the sigmoid colon suggests Hirschsprung disease

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65
Q

Most common cause of intestinal obstruction 3mo to 6yr

A

Intussusception

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66
Q

Possible lead point for intussusception

A

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

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67
Q

Usual lead point intussusception post op

A

ileoileal

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68
Q

ultrasound findings of intussusception

A

tubular mass in longitudinal
doughnut in transverse

coiled spring on contrast enema

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69
Q

perforation with foreign body tends to occur at

A

physiologic sphincters (pylorus, ileocecal valve), acute angulation (duodenal sweep), congenital gut malformations (webs, diaphragms, diverticula), or areas of pre- vious bowel surgery

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70
Q

problem with battery ingestion

A

leakage of alkali or heavy metal

electrical discharges causes burns in intestine

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71
Q

gastric ulcer found in

A

lesser curvature of stomach

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72
Q

primary ulcers found in

A

duodenal

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73
Q

ulcer in response to burn

A

curling ulcer

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74
Q

ulcer in response to stress/shock/intracranial mass

A

cushing ulcer

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75
Q

increase gastric acid production

A

acetylcholine
histamin
gastrin

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76
Q

decrease gastric acid production

A

prostaglandin

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77
Q

h pylori produces

A

urease
catalase
oxidase

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78
Q

gastric hypersecretion by tumor

associated with MEN multiple endocrine neoplasia

A

zollinger ellison syndrome

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79
Q

ulcerative colitis localized to __

A

colon

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80
Q

blood mucus pus in stool

diarrhea

A

ulcerative colitis

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81
Q

extraintestinal manifestation of ulcerative colitis

A

pyoderma gangrenosum, sclerosing cholangitis, chronic active hepatitis, and ankylosing spondylitis

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82
Q

treatment for ulcerative colitis

A

sulfasalazine

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83
Q

idiopathic, chronic in ammatory disorder of the bowel, involves any region of the alimentary tract from the mouth to the anus.

A

crohn

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84
Q

diarrhea
weight loss
ab pain
failure to thrive

A

crohn

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85
Q

tx for mild crohn

A

mesalamine

86
Q

edema assoc with

A

protein losing enteropathy

87
Q

perianal excoriation and gaseous abdominal distention

A

carbohydrate malabsorption

88
Q

explosive watery diarrhea suggests

A

carbohydrate malabsorption

89
Q

loose, bulky stools are associated with

A

celiac disease

90
Q

pasty and yellowish offensive stools suggest an

A

exocrine pancreatic insufficiency

91
Q

Stool pH and reducing substances for

A

carbohydrate malabsorption

check for acidic stool and >2 reducing substances

92
Q

quantitative stool fat examination

A

fat malabsorption

93
Q

α1­antitrypsin

A

protein malabsorption

94
Q

fecal stool elastase­1

A

exocrine pancreatic insufficiency

95
Q

Breath hydrogen test

A

to identify the specific carbohydrate that is malabsorbed

96
Q

serum trypsinogen in cystic fibrosis is

A

elevated early then declines 5-7 years old

97
Q

shwachman syndrome

serum trypsinogen is

A

low

98
Q

gold standard test for exocrine pancreatic function

A

duodenal aspirate

99
Q 
failure to thrive
chronic diarrhea 
vomiting 
ab distention
muscle wasting
IDA nonresponsive to iron therapy
epilepsy with bilateral occipital classifications
A

celiac disease

100
Q

malignancy main cause of death in celiac disease

A

non hodgkin lymphoma

101
Q

diagnosis if celiac disease

A

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
Q

autosomal recessiev
manifests as profuse watery secretory diarrhea
light microscopy diffuse thinning of the mucosa with hypoplastic villus atrophy and no inflammatory infiltrate

A

congenital microvillus atrophy

or microvillus inclusion disease

103
Q

treatment of congenital microvillus atrophy

or microvillus inclusion disease

A

octreotide

104
Q

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

A

tufting enteropathy

105
Q

treatment of intestinal lymphangiectasia

A

restricting long chain fat and using formula with formula and MCT

106
Q

diagnosis of intestinal lymphangiectasia

A

elevated fecal alpha antitrypsin clearance

107
Q 
caused by tropheryma whipplei
weight loss
diarrhea
ab pain
PAS positive microphages in biopsy
A

whipples disease

108
Q

tx for whipples disease

A

cotrimoxazole

109
Q

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

A

Hartnup disease

110
Q

Tryptophan malabsorption

causes urine to turn blue

A

blue diaper syndrome (indicanuria, Drummond syndrome)

111
Q

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

A

Tangier disease

112
Q

useful to differentiate between lack of IF and malabsorption of cobalamin

A

Schilling test

113
Q

an infant can survive even if __ cm of bowel with ileocecal valve or __cm without

A

15cm

20cm

114
Q

t or f

jejunum resection is better than ileal resection

A

true

115
Q

proximal 100-200cm of jejunum is main site of absorption of

A

carbohydrates
protein
iron
water soluble vitamin

116
Q

distal ileum absorption

A

vitb 12

bile salts

117
Q

net sodium and fluid absorption is at

A

ileum

118
Q

duodenum and proximal jejunum absorption

A 
calcium
magnesium
phosphorus
folic acid
iron
119
Q

renal stones can occur in malabsorption due to

A

hyperoxaluria secondary to steatorrhea

calcium binds to fat and to oxalate so it’s secreted out and oxalate levels rise

120
Q

severe portal hypertension in liver disease leads to malabsorption due to

A

portal hypertensive enteropathy

poor absorption of nutrients

121
Q

loss of deep tendon reflexes
ophthalmoplegia
cerebellar ataxia
posterior column dysfunction

A

vit e deficiency

122
Q 
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
A

fanconi bickel syndrom

123
Q

treatment of fanconi bickel syndrom

A

vit d
uncooked conrstarch
electrolyte replacement

124
Q

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

A

congenital chloride diarrhea

125
Q 
polyhydramnios
massive secretory diarrhea
severe met acid
alkaline stools
hypoNa
A

congnital sodium diarrhea

126
Q 
weaned from breastfeeding
anorexia diarrhea
failure to thrive
humoral and cell mediated immunodeficiency
dermatitis
neuro abnormalitites
poor wound healing
A

acrodermatitis enteropathica

zinc malabsorption

127
Q 
kinky hair
seizuree
hypothermia
apnea 
cutis laxa
hypopigmentation
A

menkes disease

copper malabsorption

128
Q

most common cause of diarrhea US

A

rotavirus and norovirus

129
Q

pesticide

organophosphate poisoning

A

SLUDGEM (salivation, lacrimation, urination, defecation, gastrointestinal motility, emesis, miosis)

increase acetylcholine
activate parasympathetic

130
Q

cholera and ETEC activate ___ to cause diarrhea

A

adenylate cyclase

131
Q

diarrhea that have blood and leukocytes in stool

causes ab cramps, tenesmus and fever

A 
salmonella
shigella
campylobacter
yersinia enterocolitis
EIEC or EHEC or shiga e coli
vibrio parahaemolyticus
132
Q

shigatoxin producing e coli

A

e coli o157:h7

133
Q

treatment to minimal dehydration

A

<10kg 60-120ml for each stool/vomiting

>10kg 120-240ml for each

134
Q

treatment for mild to mod dehydration

A

50-100ml/kg over 3-4 hours

THEN
<10kg 60-120ml for each stool/vomiting
>10kg 120-240ml for each

135
Q

treatment for severe dehydration

A

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
Q

WHO ORS

A 
75meq Na
75mmol glucose
20meq K
65meq Cl
10meq citrate
245mosm per liter
137
Q

zinc in diarrhea

A

<6mo 10mg/day
>6mo 20mg/day
10-14 days

138
Q

racecadotril is

A

enkephalinase inhibitor

139
Q

doc for shigella

A

cipro

140
Q

doc for EPEC, ETEC, EIEC

A

cotri

cipro

141
Q

doc for salmonella

A

ceftri

142
Q

doc for yersinia

A

doxycycline

143
Q

doc for campylobacter

A

azithromycin

144
Q

doc for clostridium difficile

A

metro

145
Q

doc for entamoeba

A

metro

146
Q

doc for giardia

A

metro

147
Q

doc for cryptosporidium

A

nitazoxanide

148
Q

doc for blastocystis

A

metro

149
Q

past history of AGE
sensitivity to food allergens
disaccharidase deficiency
reinfection

A

postenteritis syndrome

150
Q 
exocrine pancreas hypoplasia
neutropenia
bone changes
intestinal protein loss
low serum trypsinogen
A

shwachmann diamond syndrome

151
Q

recurrent ab pain defined as

A

3 epsiodes in 3 months with impairment of fxn

152
Q

viscera innervated by

A

vagal

splanchnic

153
Q

age of appendicitis

sex predominance?

A

12-18

boys

154
Q

___ location of appendicitis leads to slower course

A

retrocecal

155
Q

single most reliable finding in appendicitis

A

localized abdominal tenderness

156
Q

psoas sign for

A

retrocecal appendicitis

157
Q

obturator sign for

A

pelvic appendicitis

158
Q

pediatric appendicitis score

A

<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
Q

utz criteria to dx appendicitis

A 
wall thickness >6mm
luminal distention
lack of compressibility
complex mass RLQ
fecalith
160
Q

ct scan findings appendicitis

A

distended thick walled appendix
inflammatory streaking of surrounding mesenteric fat
pericecal phlegmon or abscess

161
Q

mittelschmerz

A

rupture of ovarian follicle causes pain

162
Q

pathogens implicated with appendicitis

A 
bacteroides
clostridia
poptostreptococcus
e coli
psuedomonas
enterobacter
klebsilla
163
Q

antibiotics in appendicitis

A

simply unperforated: cefoxitin

perforated: ampicillin, gentamycin, clinda or metronidaole)

164
Q 
Autosomal recessive
uridine diphosphogluconurate glucuronosyltransferase (UGT1A1) problem
Does not conjugate bili
Kernicterus in almost all
Jaundice first 3 days of life
High unconjugated bili
A

Crigler najjar

165
Q

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

A

Gilbert disease

166
Q

anomalies in sacrum assoc with anomalies in kidney/urinary tract

A

caudal regression

167
Q

organisms in perianal abscess

A

e coli, klebsiella, staph, bacteroides, clostridium, veillonella

168
Q

difference between internal and external hemorrhoids

A

internal hemorrhoids above the dentate line present as bleeding and prolapse; external hemorrhoids below dentate line present with pain and itching

169
Q

rectal prolapse where all layers involved

A

procidentia

170
Q

pathogen in pilonidal abscess

A

staph and bacteroides

171
Q

hernias occur most at

A

1st year of life

172
Q

highest risk of srtangulation hernia at

A

1st year of life

173
Q

predmoinance hernia sex

A

male

174
Q

testes start to descend by

A

28wks aog

175
Q

completion of testes descent

A

28 to 36 weeks aog

176
Q

__% of inguinal hernias occur on right side

A

60%

177
Q

functional unit of pancreas

A

acinus

178
Q

mitochondrial DNA mutation affecting oxidative phosphorylation, severe macrocytic anemia, thrombocytopenia, pancreatic insufficiency

A

pearson syndrome

179
Q

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.

A

johanson-blizzard syndrome

180
Q

direct testing of pancreas

A

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
Q

most common etiologies of pancreatitis

A

blunt ab trauma, multisystem disease, biliary stones, drug toxicity

182
Q

drugs that cause pancreatitis

A

valproic acid, l-asparaginase, 6 mercaptopurine, azathiprine

183
Q

bluish discoloration around umbilicus in pancreatitis

A

cullen sign

184
Q

bluish discoloration around flank in pancreatitis

A

grey turner sign

185
Q

__ is test of choice for pancreatitis

A

lipase

186
Q

labs associated with pancreatitis

A

elevated GGT amylase lipase bilis glucose, hemoconcentration, coagulopathy, hypoCa

187
Q

causes of chronic pancreatitis

A

mutation in PRSS1 gene, cystic fibrosis, hyperlipidemia, hyperparathyroidism, ascariasis, autoimmune pancreatitis, juvenile tropical pancreatitis

188
Q

normal liver size

A

4.5-5cm at 1week old, 7-8cm for boys and 6-6.5cm in girls at 12 years old

189
Q

normal gallbladder length

A

1.5-5.5cm (average 3cm) in INFANTS and 4-8cm in adolescents

190
Q

clinically apparent jaundice occurs at level

A

2-3mg/dL in kids, 5mg/dL in neonates

191
Q

factors favoring ascites

A

decreased colloid vascular pressure, increased capillary hydrostatic pressure, increased ascitic fluid colloid pressure, decreased ascitic fluid hydrostatic pressure

192
Q

hypoxemia, intrapulmonary vascular dilations, and liver disease

A

hepatopulmonary syndrome

193
Q

utz finding of biliary atresia

A

small or absent gallbladder, nonvisualization of common bile duct, trangular cord sign

194
Q

idiopathic familial intrahepatic cholestasis, lymphedemaof lower extremities

A

aagenaes syndrome

195
Q

autosomal recessive, progressive degeneration of liver and kidneys, generalized hypotonia and retardation, hepatomegaly, renal cortical cysts

A

zellweger (cerebrohepatorenal syndrome)

196
Q

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

A

neonatal iron storage disease or neonatal hemochromatosis

197
Q

final step in bile acid synthesis, catalyzed by

A

conjugation with glycine and taurine; bile acid coA ligase

198
Q

most common syndrome with intrahepatic bile duct paucity

A

alagille syndrome

199
Q

broad forehead, deep set widely spaced eyes, long straight nose, underdeveoped mandible, cardiac , tubulointerstitial nephropathy, vertebral defects, short stature, pancreatic insufficiency, defective spermatogenesis

A

alagille syndrome

200
Q

cardiac lesions associated with alagille syndrome

A

usually peripheral pulmonic stenosis, tof, pulmonary atresia, vsd, asd,coa

201
Q

most common form of biliary atresia

A

obliteration of the entire extrahepatic biliary tree at or above porta hepatis

202
Q

histopath biliary atresia

A

bile ductular proliferation, presence of bile plugs, portal or perilobular edena and fibrosis, basic hepatic lobular architecture intact

203
Q

histopath neonatal hepatitis

A

diffuse hepatocellular disease with infiltration of inflammatory cells, bile ducts intact

204
Q

kasai

A

hepatoportoenterostomy

205
Q

best time to do kasai

A

8 weeks

206
Q

autosomal recessive, defect in hepatocyte secretion of bilirubin glucoronide, liver histopath normal architecture but hepatocytes contain black pigment

A

dubin-johnson syndrome

207
Q

autosomal recessive, degenrative changes in brain and liver, kayser fleischer rings in cornea, coombs negative hemolytic anemia

A

wilson disease

208
Q

pathophysio of wilson disease

A

gene for biliary copper excretion and copper incorporation to ceruloplasmin defective

209
Q

during hemolytic episodes in wilson disease, what are elevated

A

urinary copper excretion and serum copper levels are elevated

210
Q

treatment of wilsons disease

A

penicillamine, decrease dietary copper

211
Q

what are side effects of penicillamine

A

decrease vit b6, goodpasture syndrome, sle, polymyositis

PPS - From Nelson's (24 decks)

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