PBC\ PSC\ Ascites

Question 1

What is the most common auto-immune disease associated with PBC?

Answer 1

Sjogren

Question 2

PBC causes intra or extra hepatic obstruction?

Answer 2

Intra-hepatic

Question 3

Which antibody positive in PBC?

Answer 3

AMA

Question 4

Survival rate in PBC?

Answer 4

Asx patient: 16 years.
Symptomatic patient: 7 years.

Question 5

Early symptoms of PBC?

Answer 5

Majority of patients are asymptomatic. Itching can appear as early symptom

Question 6

Signs and symptoms of PBC?

Answer 6

1) High cholesterol level.
2) Keratoconjuctivitis.
3) Jaudice.
4) Arthopathy.
5) Kerostomia (Hyperpigmentation)

Question 7

Findings in Biopsy of PBC?

Answer 7

Flouride duct lesion.

Question 8

Management of PBC?

Answer 8

Liver transplant

Question 9

Medical options to treat PBC?

Answer 9

UDCA
- Decrease histological complications and increase survival.
- This will only happen in 25 % of patients.

Question 10

How to monitor patients on medical management of PBC?

Answer 10

Biochemical response:
- Decrease ALT and AST
- Decrease in bilirubin.
- Decrease ALP < 1.67 ULN

Question 11

How to diagnose PBC?

Answer 11

• Increase ALP 1.5 ULN
• AMA +
  If AMA + No need for biopsy.

Note:
- Always do US or MRCP to rule out extrahepatic obstruction

Question 12

What if AMA negative. And you still suscpect PBC. Next step?

Answer 12

• Do other PBC Specific Ab
  1) Sp100 2) Gp210

Question 13

What if other PBC-Specific antibodies are negative?

Answer 13

Liver biopsy.

Question 14

PBC

Any other medical treatment? Other than UDCA

Answer 14

Fibrate can be used as off-label.

Question 15

Cirrhosis

What are the two main aspects of ascites pathophysiology?

Answer 15

1) Portal HTN
2) Na And H2O retention

Question 16

Cirrhosis

How portal hypertension causes ascites? (3)

Answer 16

1) Stellate cell necrosis. Lead to fibrosis.&raquo_space; Vasoconstriction.
2) Production of NO lead of splanchnic vasodilation.&raquo_space; Water retention.

Question 17

Cirrhosis

How Na and H2O retention causes ascites? (2)

Answer 17

1) Water retention due to increase NO.
2) Decrease renal blood flow.
3) Activation of RAAS System.

Question 18

Cirrhosis

How ascites develop in patient with cancer or infection?

Answer 18

Increase albumin pass into the periteneoum and leak of proteneousus material to the peritoneum

Question 19

Cirrhosis

How ascites develop in patient with pancreatitis?

Answer 19

Pancreatic enzyme leak to peritoneum

Question 20

Cirrhosis

What are the causes of Low SAAG < 1.1?

Answer 20

High albumin in ascetic fluid

Low Albumin level in the serum
- Nephrotic syndrome
- Protein losing enteropathy.
High Albumin in the ascitec fluid:
- Malignancy: Meig syndrome, perotoneal mets
- Shistocoma.
- Other infections.
- Inflammation: pancreatitis, serositis, bowel obstruction.

Question 21

Cirrhosis

What are the causes of high SAAG > 1.1?

Answer 21

Divided to
1) Pre-sinosuidal:
- Portal.V thrombosis or splenic V thrombosis
- Shistosoma.
- Sarcoidosis.
2) Sinosuidal: Cirrhosis.
3) Post-sinosoidal:
- SOS, Budd-chiary syndrome and R. CHF

Question 22

Cirrhosis

How to differentiate between high SAAG ascites causes

Answer 22

Protein level. If more than 2.5 = cardiac if less than 2.5 = liver

Question 23

Cirrhosis

Management of ascites

Answer 23

1) Na restriction < 2 g\Day
2) Diuretics

Question 24

Cirrhosis

Options of diuretics in ascites

Answer 24

Spironolactone and Furesamide in ratio of 5 to 2.

Question 25

Cirrhosis

Goal of diuresis per day

Answer 25

1 Liter per day
Urine Na\K Ratio > 1

Question 26

Cirrhosis

Which medications you can stop to ensure good diuresis

Answer 26

NSAID’s and ACE

Question 27

Cirrhosis

What is the maximum dose of diuretics?

Answer 27

Spironolactone 400 mg per day

Question 28

Cirrhosis

Alternative for spironolactone?

Answer 28

Amiloride 5 to 40 mg\D

Question 29

Cirrhosis

What is the definition of refractory ascites?

Answer 29

No adequate diuresis with max dose of diuretics or intolerable SE of diuretics.

Question 30

Cirrhosis

What option to go for in refractory ascites?

Answer 30

1) Paracetesis. If failed.
2) LVP. If failed.
3) TIPS. If failed
4) End-willing catheter (palliative option)

In LVP start 25 % Albumin 6 to 8 g\L for each liter above 5 liter.

Question 31

Cirrhosis

Two conditions to avoid LVP?

Answer 31

• SBP
• AKI

Question 32

Cirrhosis

What if LVP failed? next step?

Answer 32

TIPS

Question 33

Cirrhosis

Mention 4 CI for TIPS?

Answer 33

1) HE Grade II
2) Heart failure
3) P. HTN
4) Biliary disease.
5) Infections.

Question 34

Cirrhosis

4 COMPLICATIONS OF TIPS

Answer 34

1. Bleeding.
2. Thrombosis.
3. Infection.
4. Increase risk of HE by 20 to 30 %

Question 35

Cirrhosis

Palliative option for refractory ascites?

Answer 35

Ind-welling catheter

High risk of complications. Used as palliative option in refractory ascites

Question 36

Cirrhosis

What is the Dx:
- Ascites + Benign Ovarian tumor + R. Sided pleural effusion

Answer 36

Meig syndrome