Miscellaneous Liver And Gastrointestinal Diseases Part 1

Front 1

What are the two types of jaundice and three causes of each?

What is the point at which you can see scleral icterus in adults? Children?

Back 1

Adults = 3

Children = 5

Front 2

Is jaundice common in neonates? Why? How can you treat severe hyperbilirubinemia?

Back 2

Front 3

What is a progressive inflammatory and
fibrosing disease of extrahepatic
bile ducts, of unknown etiology,
with gradually complete obstruction
of bile flow caused by destruction
of extrahepatic bile ducts?

Causes 1/3 of neonatal ______.

Most common cause of death in early child hood from _____.

Back 3

Front 4

Back 4

Front 5

What is the condition? How do you know?

Back 5

Biliary atresia

Fibrosis obliterating and extra-hepatic bile duct.

Front 6

Condition?

Back 6

Biliary atresia

Front 7

BILIARY ATRESIA:

typically become _____
at 3-6 weeks of age
have _____ hyperbilirubinemia
disproportionately high _____
mild-moderately high ALT and AST
_____ prognosis improved by early
surgery (hepatoportoenterostomy)
but many still need liver transplant

(footnote: Alkaline phosphatase in children is more from _____, less useful in diagnosing liver disease)

Back 7

BILIARY ATRESIA:

typically become jaundiced
at 3-6 weeks of age
have conjugated hyperbilirubinemia
disproportionately high GGT
mild-moderately high ALT and AST
poor prognosis improved by early
surgery (hepatoportoenterostomy)
but many still need liver transplant

(footnote: Alkaline phosphatase in children is more from
bone, less useful in diagnosing liver disease)

Front 8

What is a syndromic paucity of intrahepatic bile ducts? What are the three major symptoms?

Back 8

Front 9

ALAGILLE SYNDROME
also called _____ dysplasia

autosomal _____

70% sporadic new mutations

1 in 70,000 live births

due to mutation in _____ gene
for ligand for _____ receptor

Back 9

ALAGILLE SYNDROME
also called arteriohepatic dysplasia

autosomal dominant

70% sporadic new mutations

1 in 70,000 live births

due to mutation in JAGGED 1 gene
for ligand for NOTCH 1 receptor

Front 10

What is the condition shown? Trichrome stain of portal triad
Blood vessels,
but no bile ducts.

Good or poor prognosis?

Back 10

Front 11

hereditary conjugated hyperbilirubinemia

defective excretion of bilirubin
conjugates and other organic
anions across hepatocyte
canalicular membrane

What is the condition?

Back 11

Dubin-Johnson Syndrome

Front 12

What is the condition? What is the brown-black pigment in the liver?

Back 12

Front 13

What is the life expectancy of Dubin-Johnson syndrome?

Fluctuating or recurrent what?

Back 13

Front 14

Gilbert Syndrome:

Autosomal recessive deficiency of what enzyme? What does this enzyme do?

More common in which sex?

How is it diagnosed?

Back 14

Front 15

Is Gilbert syndrome serious? What are some "benefits" of the disease?

Back 15

Front 16

Primary Biliary Cirrhosis:

What type of disease, fast or slowly progressive?

Age? Sex?

What do PBC patients usually present with? Progress to?

Back 16

Front 17

What is the condition?

Back 17

Primary biliary cirrhosis

Front 18

What is the condition?

Back 18

Front 19

What is shown here?

Back 19

Front 20

BUDD-CHIARI SYNDROME:

_____ hypertension due to
thrombosis of _____ veins
manifestations: hepatomegaly,
___, abdominal pain
due to polycythemia vera, other
myeloproliferative disease,
_____, postpartum state,
oral contraception, paroxysmal
nocturnal hemoglobinuria,
______ cancer (especially
within the liver itself)

Back 20

BUDD-CHIARI SYNDROME:

portal hypertension due to
thrombosis of hepatic veins
manifestations: hepatomegaly,
ascites, abdominal pain
due to polycythemia vera, other
myeloproliferative disease,
pregnancy, postpartum state,
oral contraception, paroxysmal
nocturnal hemoglobinuria,
abdominal cancer (especially
within the liver itself)

Front 21

What is the condition?

Back 21

Budd-Chiari syndrome

Front 22

What is the diagnosis?

Back 22

Budd-Chiari syndrome

Front 23

If patient has pain and 4 Fs, what is the diagnosis?

Back 23

Acute cholecystitis

Simply having stones does not cause pain.

(Cholelithiasis)

Front 24

BUDD-CHIARI SYNDROME

30% _____ (cause)

associated with hypertrophy of
_____ lobe.

commonly _____ if develops acutely

_____% 5-year survival if develops
chronically

Back 24

BUDD-CHIARI SYNDROME

30% idiopathic

associated with hypertrophy of
caudate lobe

commonly fatal if develops acutely

50% 5-year survival if develops
chronically

Front 25

What is an infection of the intrahepatic biliary ducts?

What bacteria cause the acute form?

What organisms cause the chronic form?

Back 25

Front 26

Ascending cholangitis:

Where is it located?

Back 26

Small tan areas

Front 27

What are the symptoms of ascending bacterial cholangitis?

Describe the pathology:

What is the treatment?

Back 27

Front 28

What is shown at the arrows? Diagnosis?

Back 28

Front 29

What is shown at the arrows?

Back 29

Front 30

Sigmoid Colonic Diverticular Disease:

Diverticular disease refers to acquired pseudo-diverticular ______ of the colonic _____ and _____. Unlike true diverticula, such as _____diverticulum, they are not invested by all _____ layers of the colonic wall.
Diverticula are generally multiple and the condition is referred to as _____.
Colonic diverticula occur primarily in those over age __, in the US.

Colonic diverticula result from the unique structure of the colonic _____ and elevated intraluminal _____ in the sigmoid colon. There are ___ in the colonic muscle wall where nerves, blood vessels and their connective tissue sheaths penetrate the inner circular muscle coat. In other parts of the intestine, these gaps are reinforced by the external longitudinal layer of the _____, but, in the colon, this muscle layer is gathered into the three bands termed _____. Increased intraluminal pressure is probably due to exaggerated peristaltic contractions, with spasmodic sequestration of bowel segments, enhanced by low _____ diets, which reduce soft bulky stool to small rock-like pellets, particularly in the _____ colon.

Back 30

Sigmoid Colonic Diverticular Disease:

Diverticular disease refers to acquired pseudo-diverticular outpouchings of the colonic mucosa and submucosa. Unlike true diverticula, such as Meckel diverticulum, they are not invested by all three layers of the colonic wall.
Diverticula are generally multiple and the condition is referred to as diverticulosis.
Colonic diverticula occur primarily in those over age 60, in the US.

Colonic diverticula result from the unique structure of the colonic muscularis propria and elevated intraluminal pressure in the sigmoid colon. There are holes in the colonic muscle wall where nerves, blood vessels and their connective tissue sheaths penetrate the inner circular muscle coat. In other parts of the intestine, these gaps are reinforced by the external longitudinal layer of the muscularis propria, but, in the colon, this muscle layer is gathered into the three bands termed taeniae coli. Increased intraluminal pressure is probably due to exaggerated peristaltic contractions, with spasmodic sequestration of bowel segments, enhanced by low fiber diets, which reduce soft bulky stool to small rock-like pellets, particularly in the sigmoid colon.

Front 31

Sigmoid Colonic Diverticulosis:

Colonic diverticula are small, flask-like outpouchings, usually __ to __ cm in diameter,
that occur alongside the _____, often in a regular distribution.
Colonic diverticula have a thin wall composed of a flattened or atrophic mucosa,
compressed _____, and attenuated or, most often, totally absent ______. Hypertrophy of the _____ layer of the muscularis propria in the affected bowel segment around the diverticula is common.

Back 31

Sigmoid Colonic Diverticulosis:

Colonic diverticula are small, flask-like outpouchings, usually 0.5 to 1 cm in diameter,
that occur alongside the taeniae coli, often in a regular distribution.
Colonic diverticula have a thin wall composed of a flattened or atrophic mucosa,
compressed submucosa, and attenuated or, most often, totally absent muscularis
propria. Hypertrophy of the circular layer of the muscularis propria in the affected
bowel segment around the diverticula is common.

Front 32

Back 32

Front 33

What can sigmoid colonic diverticular disease lead to?

Back 33

Inflammation => diverticulitis => shown by bile staining or hemorrhage in mucosa

Peri-diverticulitis => hyperemia or exudate on serosa (right)

Front 34

What can obstruction of diverticula lead to? What is the condition?

Back 34

Front 35

Sigmoid Colonic Diverticulitis and Abscess:

Because the wall of the diverticulum is supported only by the _____
and a thin layer of subserosal adipose tissue, inflammation and increased pressure
within an obstructed diverticulum can lead to _____.
With or without perforation, diverticulitis may cause segmental diverticular
disease-associated colitis, _____ thickening in and around the colonic wall,
or _____ formation.
Perforation is uncommon but it can result in pericolonic _____, sinus tracts,
and peritonitis, as in this 78-year-old woman, who had sigmoid diverticulitis
with perforation and peritonitis
prompting surgical resection:

Back 35

Front 36

Most people with diverticular disease remain _____ throughout their lives,

but about 20% develop intermittent _____, continuous lower abdominal
discomfort, _____, distention, or a sensation of never being able to completely
empty the rectum. Of course, the _____ is more likely the cause than the
effect of diverticulosis. Patients sometimes experience alternating constipation and
diarrhea that can mimic _____ or coexist with it.

Diverticulosis can cause chronic low-grade or intermittent acute high grade ______ loss,
and, rarely, massive hemorrhage. The high pressure that can erode the diverticula
can also erode the co-traveling _____ vessels in the tunnel they share. When present,
bleed­ing is typically visible in the ______ and red since it is a short distance to the exit.
Whether a high-fiber diet protects against bleeding or diverticulitis is unclear,
but diets supplemented with fiber may provide _____ improvement.
Even when diverticulitis occurs, it most often resolves _____
and relatively few patients require surgical intervention.

Back 36

Most people with diverticular disease remain asymptomatic throughout their lives,

but about 20% develop intermittent cramping, continuous lower abdominal
discomfort, constipation, distention, or a sensation of never being able to completely
empty the rectum. Of course, the constipation is more likely the cause than the
effect of diverticulosis. Patients sometimes experience alternating constipation and
diarrhea that can mimic IBS or coexist with it.

Diverticulosis can cause chronic low-grade or intermittent acute high grade blood loss,
and, rarely, massive hemorrhage. The high pressure that can erode the diverticula
can also erode the co-traveling blood vessels in the tunnel they share. When present,
bleed­ing is typically visible in the stools and red since it is a short distance to the exit.
Whether a high-fiber diet protects against bleeding or diverticulitis is unclear,
but diets supplemented with fiber may provide symptomatic improvement.
Even when diverticulitis occurs, it most often resolves spontaneously
and relatively few patients require surgical intervention.

Front 37

Why is GGT so much higher in biliary atresia?

Back 37

INDUCED by the disease as opposed to other enzymes that are merely released from cells by disease.

Front 38

When will you have high alk phos levels in males? Females?

Back 38

Puberty!

12-14 in males

Around 10 in females