Autoimmune and Cholestatic Flashcards
How do you treat Hep D?
Per IFNalpha for 12 months/ until undetectable HDV RNA/ALT normalization
What happens in liver transplant and HDV?
HDV confection is a risk factor for HepB viral replication, So need to give both HBIG and NA
What is treatment for Hep E
ribavirin
How do you test immunocompetent vs immunocompromised patients for Hep E?
immunocompetent: start with anti HEV IgM and IgG and RNA
immunocompromised, started with HEV RNA
What are extrahepatic manifestations of Hep E?
Mostly neurologic and renal:
Guillan Barre
Meningoencephalitis
Myosisitis
Renal complications: IgA nephropathy, membranoproliferazive
So if you see these and elevated LFTS, think Hep E
What is Harvoni?
sofo+led
genotype 1,4,5,6
What is epclusa
sofo+velpa
pangenotypic
what Is mayvret
glecaprevir+ pibrentsavir
pangenotypic
Can’t use in decompensated
What are options for HCV without cirrhosis?
Mayret (Glec +pib) 8 weeks
Epclusa (sof +vel) 12 weeks
How do you treat HCV in decompensated cirrhosis?
Ribavirin eligible:
Epclusa +ribavirin for 12 weeks
Harvoni + ribavirin for 12 weeks (1,4,5,6 only)
Ribavirin ineligible:
HArvoni (1,4,5,6) for 24 weeks
Epclusa for 24 weeks
Avoid protease inhibitors (previr) and interferon
What is the definition of ALF?
What is the UNOS criteria for ALF?
Definition: INR >1.5, encephalopathy (any degree), without pre-existing liver disease, duration of illness <26 weeks
UNOS criteria for 1A:
-HE within 8 weeks of the first symptoms of liver disease
-absence of pre-exisiting liver disease
-must be in the ICU
- must have life expectancy of less than 7 days
- must have one of the following
—ventilator dependence
—dialysis
— INR >2.0
What are factors that influence outcome of ALF?
- early recognition
- administration of NAC
- transfer to liver transplant center
- listing for liver transplant
What are the kings college criteria for ALF due to Tylenol?
It is a prognostic model to help identify those who should be referred to transplant. There is a criteria by etiology (Tylenol vs non Tylenol)
For Tylenol ALF:
List for OLT if
- pH<7.3 after resuscitation and >24 hours post ingestion
or
- lactic >3 after IVF
Strongly consider listing if
- Lactic >3.5 after IVF
List if all three occur within 24 hour period
1. HE>grade 3
2. Cr >3.4
3. INR>6.5
What is the kings criteria for ALF not due to Tylenol
INR>6.5 and HE present
OR three of the following 5 criteria
- indeterminate etiology, non acetaminophen drug induced, unfavorable etiology
- IND >3.5
- Interval from jaundice to encephalopathy >7 days
- Bilirubin >17
- Age <10 or >40
What are favorable etiologies of ALF? And what are unfavorable etiologies of ALF?
Favorable:
- Tylenol
- Pregnancy associated
- Hep A
- ischemic hepatitis
Unfavorable:
- wilson- won’t recover if presents with alf without transplant
- non-a viral hepatitis- majority will recover with supportive care but 1% present with alf and need transplant even if on antiviral
- mushroom intoxication- can treat with penicillin g and nac, but still need lt
- budd chiari syndrome
- yellow phosphorus
- non acetaminophen drug induced
- Indeterminate
Can lactulose be used in ALF HE?
no role, just causes ileum and bowel edema
What are predictors of cerebral edema in ALF?
- persistent ammonia >150-200
- younger age - decreased free space within cranium
- hyper acute ALF phenotype
What are non - invasive methods of monitoring ICP
trans-cranial doppler
optic nerve sheath diameter
pupillometry
What are general management for elevated ICP
- HOB elevation to 30
- Avoidance of excessive stimulation
- avoidance of unnecessary or routine tracheal suctioning
- avoidance of fever
- clamping of serum sodium to 140-145
sustained elevation:
- hypertonic saline 23.4% bolus
- mannitol - in those without renal dysfunction
-indomethicin
- mechanical ventillation- only as rescue
Per AASLD:
- in ICP, can give mannitol bolus as first line therapy, but prophylactic administration is not recommended
- in ALF patients at highest risk for cerebral edema (ammonia >150, HE, acute renal failure, vasopressors), prophylactic induction of hypernatremia with hypertonic saline to a sodium level of 145-155 is ok
When can charcoal be given in Tylenol overdose?
For patients with known or suspected tylenol overdose within four hours of presentation, given activated charcoal just prior to starting NAC
How to manage ALF from mushroom poisoning?
Amanita phalloides
No blood test
look for severe GI symptoms (nausea, vomiting, diarrhea, abdominal cramping)
Tx: penicillin G and NAC
Patients with ALF from mushroom poisoning should be listed for transplantation as this procedure is often the only lifesaving option
When is liver biopsy recommended in AIH and ALF?
when AIH is suspected as cause of ALF and autoantibodies are negative.
Can still treat with steroids but need to start workup for transplant, while getting steroids
How do you treat seizures in ALF?
phenytoin and benzodiazepines with short half lives, prophylactic phenytoin is not recommended
What type of PH is portal vein thrombosis and what hepatic vein pressure measurements do you see?
prehepatic
Free: normal
Wedged: normal
Gradient/HVPG: normal
What type of portal hypertension is heart failure or SVC?
post hepatic
Free: elevated
Wedged: elevated
HVPG: normal
What type of ph is seen in cirrhosis? pressure measurements?
Sinuoidal (intrahepatic PH)
Free: normal
Wedged: Elevated
Gradient: Elevated
What type of PH Is seen in cholestatic cirrhosis or schisto?
Intrahepatic, pre-sinusoidal
Free: normal
Wedged: normal
Gradient: normal
What type of portal hypertension is seen in sinusoidal obstruction syndrome?>
Intrahepatic, post sinusoidal
Free: normal
Wedged: elevated
Gradients: elevated
What does free hepatic vein measure?
pressure in hepatic vein
what does wedged pressure measure?
portal and sinusoidal pressure
What are the cutoff for LSM of CSPH?
LSM>25 alone
LSM 20-25 + Plt <150
LSM 15-20 +plts <110
Who needs a screening EGD at time of diagnosis of cirrhosis?
everyone unless LSM <20 and Plts >150 (these have a very low probability of having high risk varices (<5%)
When should someone with compensated cirrhosis get a repeat screening endoscopy for prophylaxis?
- At time of diagnosis
- No varices –> 2 (with ongoing liver injury) to 3 years (if liver injury is quiescent)
- Small varices –> 1 to 2 years
- at time of decompensation
When should someone with decompensated cirrhosis be screened for varices?
yearly and at time of decompensation
How do you give propranolol for varices?
20-40 g orally twice a day
Adjust every 2-3 days until treatment goal (HR of 55-60) is achieved
Max dose is 320 mg/day without ascites or 160 mg/day with ascites
SBP should not fall <90
Continue indefinitely
No need for follow up EGD, but assess HR at every visit
How do you give nadolol for varices?
20-40 mg once a day (not for propranolol is it twice a day)
adjust every 2-3 days until treatment goal is achieved
Max dose is 160 mg/day in patients with ascites, 80 mg/day in patients with ascites
Goal is HR of 55-60
Continue indefinitely, making sure HR
No need for follow up EGD
How to give carvedilol for varices?
Start with 6.25 mg once a day
After three days, increase to 6.5 mg twice a day
Max dose is 12/5 mg/day
Goal is to have SBP not decrease <90
No need for follow up EGD, continue indefinitely
How often do you do EGD for EVL?
Every 2-8 weeks until eradication of varices
First EGD is 3-6 months after eradication and every 6-12 months thereafter
What does the new Baveno guidelines say re use of co-reg?
non selective beta blockers (particularly co-reg) may be considered in patients with compensated cirrhosis with CSPH (remember compensated cirrhosis can be without CSPH or with CSPH i.e presence of varices) to prevent decompensation. So if compensated cirrhosis, and evidence of varices –> can start on co-reg to prevent future decompensation
Once NSBB started, serial endoscopy not needed
How do you treat patients with compensated cirrhosis and mild pH?
objective is to prevent development of CSPH/decompensation and maybe even achieve regression of cirrhosis
NSBB are not as helpful, because they act on portal flow, and at this stage the hyper dynamic circulatory state is not fully developed
What type of portal hypertension in seen in PBC
pre-sinsuoidal
free: normal
Wedged: normal
Gradient: normal
Why is coreg better than other NSBB
has more anti alpha adrenergic vasodilatory properties that contribute to its greater portal pressure reducing effect (**I think it causes systemic vasodilation, which decreased cardiac output and therefore decreases portal pressures)
What do you do if you see medium to large varices on EGD in someone who has not bleed?
What about small varices with high risk stigmata
can do NSBB or EVL for the prevention of first VH. Should not do both EVL and BB for prophylaxis
Small varices with high risk stigmata is rare, but can do BB. May be too small for EVL
How long should you continue antibiotics with GIB?
max of 7 days, consider stopping once hemorrhage has resolved and vasoactive drugs discontinued
ceftriaxone 1g/24 hr
When should vasoactive be started in a bleed
vasoactive (octreotide 50 micrograms bolus and then continuous infusion of 50 micrograms/hr, SMT, vasopressin, terlipressin) should be initiated as soon as VH is suspected
all is for 2-5 days
When should EGD be performed for VH?
within 12 hours of admission and once stable
When is early/pre-emptive TIPS recommended?
in those with high risk of failure or rebleeding
CTP C
CTP B with active bleeding
TIPS should be within 72 hours from EGD/EVL
Once someone is banded for VH, how long to keep vasoactive on? When to start NSBB?
if no early TIPS performed, then keep vasoactive on for 2-5 days and NSBB started once vasoactive discontinued. Rescue TIPS indicated if hemorrhage can’t be controlled or if bleeding recurs despite vasoactive drug + EVL
If rescue TIPS is performed, vasoactive drugs can be discontinued
How do you treat GOV 1? What about prophylactic?
GOV 1 = extension of EV allow lesser curve
Same as that for EV
Prophy or prevention of first bleeding from GOV1 varices is the same as that for EV
Treatment for GOV2 or IGV1?
NSBB
GOV 2= extension of EV along great curve
IGV1 = gastric varies along fundus
What is treatment of bleeding GOV1?
EVL if feasible or glue
Can do combination of EVL and NSBB for prevention of re-bleed
What is treatment of GOV2 and IGV1?
TIPS
What do you do with NSBB in someone who has bleed from varices and has ascites or SBP?
refractory ascites and SBP are NOT contraindications for treatment with NSBB. Just avoid high doses (over 160 of propranolol and 80 of nadolol)
If the SBP <90 or NA <130, then dose of NSBB should be decreased, or drug temporarily held. NSBB can be re-introduced if circulatory dysfunction improves
Which are better to prevent shunt dysfunction in TIPS? Bare metal or PTFE covered stents?
PTFE covered lowers the risk
Can TIPS be used to prevent rebreeding in patients who have bled only once from EV? GV?
No- its use should only be limited to those who fail pharmacologic and endoscopic therapy
Can be used to prevent rebreeding from gastric varices and is preferred approach for the prevention of rebleeding in this group
What are absolute contraindications to TIPS?
- Primary prevention of variceal bleeding
- CHF
- Multiple hepatic cysts
- Uncontrolled systemic infection or sepsis
- Unrelieved biliary obstuction
- Severe pulmonary hypertension
What are the indications for status 1a for adults?
- Fulminant Liver failure
a. in ICU
b. no pre-exisiting liver disease
c. HE within 56 days of onset AND ONE of the following
–vent dependent
– on HD, CVVH,
– or INR >2.0 - Anheptic
- Primary nonfiction of allograft within 7 day LTx
a. AST>/= 3000 IU/L AND ONE of the following
– INR >/=2.5
– pH /= 4
* all labs must be from same lab draw - HAT
– AST>/= 3000 AND one of the following
- INR >/= 2.5
–arterial pH /= 4
** all labs must be from same lab draw - Acute decompensated Wilson disease
What are indications for pediatric 1A status?
- Fulminant liver failure, no pre-existing liver disease
– do not need to be in ICU
- HE within 56 days of onset AND ONE of the following
- vent dependent
- CVVH/HD
- INR >2.0 - Primary non function of allograft within 7 days LTx
– Two of the following
—- ALT>/=2000
— INR >/= 2.5
— Bili >/= 1-
— Acidosis defined by ONE of the following
—– arterial pH <7.3, venous pH<7.25, lactate >4 - HAT within 14 days of LTx
- no defined lab tests for HAT in Peds and note 14 days instead of 7 days as seen in adults - acute decompensated Wilson disease
What are indications for pediatric status 1B?
- hepatoblastoma (biopsy proven) without metastatic disease
- no size limit - Organic academia or urea cycle defect AND approved exception for >/= 30 days
- Sick kid: chronic liver disease with MELD/PELD >/=25 AND ONE of the following
- mechanical ventilation
- GIB ?30 mL/kg RBC in 24 hours
- on HD, CVVH
- glasgow coma score <10 48 hours prior to status 1B
What are component of MELD-Na
Cr
INR
Bili
What is max Cr in MELD score
Cr set to 4 when
- cr >/=4
- two or more dialysis treatments in last 7 days
- 24 hours of CVVHD in last 7 days
Which is used for candidates with MELD
MELD
When is MELD-NA used? Why is it used?
when MELD>/= 12
-wait list mortality higher in those with hyponatremia
When is PELD score used?
for candidates <12 years old
What are components of PELD?
age
Bili
albumin
INR
***eventually Cr will be added
growth failure = 2 standard deviations below expected growth for age and gender