Category Archives: Gastroenterology and Hepatology

VA Ambulatory Report 12.13.17 – Flatulence!

Thanks to Vaibhav who presented his patient’s case of bothersome flatulence found to have positive celiac serologies, but negative EGD now being treated for H. Pylori.  This case allowed us to have an excellent discussion about approaching a less common chief complaint such as flatulence and how we diagnose celiac disease and IBS.

Learning Pearls    Screen Shot 2017-10-11 at 12.58.11 PM

  • Patients may experience belching (also called eructation), flatulence, or abdominal bloating due to decreased intestinal motility or increased sensitivity to normal gas production.  Reference the Rome IV criteria for diagnostic criteria of functional gastrointestinal disorders.
  • Healthy individuals are not able to completely digest some carbohydrates which leads to increased H2 production.  This is the basis for the H2 breath test for carbohydrate malabsorption
  • A low FODMAPs diet has good evidence for symptom reduction in patients with IBS. Some studies have shown benefit in other functional gastrointestinal disorders and in patients with IBD with symptoms such as bloating not explained by their inflammatory disease
  • Anti-TTG is testing for IgA antibodies and may be falsely negative in individuals with IgA deficency.  Consider co-testing with IgA levels or testing for Gliaden.  No test is perfect and the gold standard is small bowel biopsy.

 

More than you want to know about Intestinal gas

  • There is about 200 mL of gas in the intestinal system
  • The main elements in gas are nitrogen, oxygen, carbon dioxide, hydrogen, and methane
  • The odor in gas comes from minor elements such as methanethiol, dimethyl sulfide, hydrogen sulfide, short-chain fatty acids, skatoles, indoles, volatile amines, and ammonia.
  • Where does intestinal gas come from?
    • 1 – Swallowed air
    • 2 – Digestion of fat and protein leads to production of CO2 in the small bowel
    • 3 – Bacterial fermentation leads to production of Hydrogen and Methane
      • Hydrogen
        • Normal in the colon
        • Produced in the small bowel in SIBO
      • Methane
    • 4 – Carbohydrate malabsorption
      • In healthy individuals, foods with high concentrations of oligosaccharides (such as wheat, oats, potatoes, and corn, legumes) cannot be completely digested by enzymes within the normal small bowel, leading to increased H2 production
        • This increased colonic H2 production from carbohydrate ingestion is why we perform the H2 breath test for carbohydrate malabsorption
      • Fructose malabsorption may be an underappreciated cause of gastrointestinal symptoms. Up to one-half of the population cannot completely absorb the load of fructose consumed in the average diet
    • 5 – Diffusion from the blood
  • Gas Disorders (Look to the Rome IV criteria for diagnosing these conditions)
    • Belching (also called Eructition)
      • Involuntary belching occurs after meals
      • Can be increased by foods that decrease LES tone such as chocolate, fats, mints
      • Chronic, repetitive belching due to habitual air swallowing
    • Flatulence
      • May patients reporting bothersome flatulence produce a normal amount of gas but may be experiencing symptoms from it due to: alteration in intestinal motility or bacteria, dietary factors, or physiologic factors that heighten awareness of gas
    • Functional Abdominal Bloating and Distention
      • Correlation between symptoms of bloating and amount of gas production is inconsiente
      • Similar underlying contributors as flatulence: decreased gut motility, increased sensitivity to gas,
      • Rome IV criteria for diagnosis: recurrent bloating or distention at least on day/week and insufficient criteria for diagnosis of IBS, functional constipation, functional diarrhea, or postprandial distress syndrome

 

What is up with the FODMAPs diet?

  • FODMAPs = Fermentable, Oligo, Di, Monosaccharides and polyols
  • Studies have consistently shown improvement in symptoms on a low FODMAPs diet for patients with IBS including this recent RCT conducted in the US demonstrated a low FODMAP diet reduced IBS symptoms reduced symptoms
    • There is evidence it can be helpful in symptom reduction for other functional gastrointestinal disorders and in patients with IBD but symptoms such as bloating or discomfort that are not related to inflammatory disease
    • There is some evidence
  • Key concepts to teach to your patients
    • Elimination or limiting of all FODMAPs (not just one component)
    • FODMAPs foods are not the cause of the symptoms, but help reduce symptoms related to visceral hypersensitivity or decreased gut motility
  • Here is an excellent review article on FODMAPs

 

Diagnosing Celiac disease – why are Celiac Serologies so confusing?

  • When we check anti-TTG this is traditionally testing for IgA antibodies
  • Given the co-occurence with IgA deficiency, you may also want to test for IgA to ensure there is also not IgA deficiency leading to a false negative TTG
  • Gliaden is an IgG test and therefore avoids the problem with IgA testing
  • Gold standard is duodenal biopsy looking for
  • HLA testing is helpful in very specific instances (possibly in high probability population and discordance on serology and biopsy)
  • Remember, patients should be on a gluten-rich diet (yum!) when these tests are performed

 

What are the recommendations for “screening labs”

  • HIV: Screen in adults age 15-65 or at other ages if increased risk or pregnant
  • Screening for diabetes: USPSTF recommends screening in adults age 40-75 who are obese or overweight
  • Lipids: USPSTF recommends calculating ASCVD risk in all people 40-75
  • HBV, HCV: Screen in high risk individuals
  • No recommendations to get screening CBC, metabolic panel, or UA

Evernote link: https://www.evernote.com/l/AMrAvmVoGvRAdYlVY8T3cXx5RJIktWhm-Nw

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Double Moffitt Pearls 11/3 & 11/6 – ALF, Toxic ingestions and Mesenteric Lymphadenopathy

Moffitt Pearls 11.3.17 – Saipan Case

Thank you to Amy for presenting a case from Saipan!! We learned about a middle-aged man presenting with encephalopathy and jaundice found to have acute liver injury and renal failure of unclear etiology. The leading thoughts were possibly leptospirosis vs biliary colic that self resolved. The patient was managed with IV abx and IVFs and improved over 2 weeks. We discussed the limitations of practicing in this setting and inability to transfer this patient to a center for transplant evaluation.

Key Pearls

  • EtoH Hepatitis: jaundice, anorexia, fever, and tender hepatomegaly. Labs: transaminases (typically less than 300 int. unit/mL), AST/ALT ratio > 2. Patients may also present with right upper-quadrant/epigastric pain, hepatic encephalopathy, and signs of malnutrition.
  • Acute liver failure is defined as the presence of coagulopathy (INR > 1.5), encephalopathy and no pre-existing liver disease. See more details below.
  • Leptospirosis has a broad range of manifestations, from subclinical illness or mild self-limited disease (approximately 90% of infections) to Weil’s syndrome (Weil’s disease), which is characterized by renal failure, jaundice, and hemorrhage and has a 5 to 15% mortality rate (1).
  • See this NEJM article where HH crushes the diagnosis in the first couple of sentences (it is related to this case)

Overview of Acute Liver Failure 

ALF = coaguapathy INR > 1.5, encephalopathy, and no signs of chronic liver disease

Key History: 

  • IVDU, travel, sexual, ingestions, Fmhx Wilson’s, (note: Hemochromatosis – no acute liver failure)

Physical Exam:  

  • Vitals, stigmata of liver disease, neuro exam, optho exam

Etiology of Acute Liver Failure

  • Ischemia*
  • Toxins* – Tylenol (most common cause in USA), Amanita
  • Acute viral hepatitis*
    • Professional: HAV, HBV, sometimes HCV, HEV
    • “Moonlighters”: HSV, CMV, VZV, parvovirus
  • Autoimmune Hepatitis
  • Acute Budd Chiari – esp if concomitant portal vein thrombosis
  • HELLP
  • Reactivation of HBV or HDV on chronic HBV
  • Wilsonian crisis – often with concomitant hemolytic anemia
  • Malignant infiltration – breast, small cell lung, lymphoma, melanoma, myeloma
  • HLH
  • Heat stroke 
  • Remember -> NOT causes of acute liver failure – ETOH, NAFLD, iron overload, alpha-1 def, PSC, PBC
  • * denotes are most likely to cause AST/ALT in the 1000s

 

Moffitt Pearls 11/6 – Toxic Ingestions

Thank you to Tim for presenting a fascinating case of a young woman 9 weeks post-partum presenting with a profound gap acidosis and osm gap initially c/f ethylene glycol vs methanol ingestion found to have severe starvation ketosis. We discussed the evaluation of possible ingestions and treatment for suspected Ethylenel Glycol ingestion with fomepizole (below).

Thank you HH for presenting a mini-case of a young engineer returning from India p/w abdominal pain, n/v and mild hepatocellular transaminitis (300s) found to have mesenteric lymphadenopathy 2/2 hepatitis E!!

Key Pearls

  • Per EM guidelines send Serum Osm, Salicylates, APAP and EtoH levels in any suspected ingestion.
  • Fomepizole is used in ethylene glycol and methanol toxic ingestion. It is a competitive inhibitor of alcohol dehydrogenase and prevents formation of glycolic acid which is responsible for both the acidosis and oxalate crystal formation. See this NEJM article for more info.
  • We learned the ddx for mesenteric lymphadenopathy include hepatitis E in addition to those listed below!!

Effect of Fomepizole on Metabolism of Eythlene Glycol and Methanol (Brent J. N Engl J Med 2009;360:2216-2223)Picture1

Differential Diagnosis for Mesenteric Lymphadenopathy

  1. Malignancy – almost any intraabdominal malignancy and metastatic process can cause mesenteric lymphadenopathy, however the following are more common:
    1. Lymphoma
    2. Carcionid Tumors
    3. Kaposi Sarcoma
    4. Carcinoma of pancreas, colon or small bowel
  2. Infection
    1. TB or MAC
    2. Salmonella and Yersinia
    3. T. Whippelii
    4. Viral Infections –EBV and Hepatitis E
  3. Inflammatory

SFGH Morning Report 10.6.17 – Cirrhosis – HCV Treatment and altered mental status

Thanks Dan for presenting an interesting case of a middle age man with HCV cirrhosis c/b HCC in the ICU after a motor vehicle accident with altered mental status, hyperkalemia, and volume overload.  Dr. Cello gave us some awesome pearls!

Learning Pearls

  • Of all the causes of cirrhosis there is one that will not progress to HCC: Congestive hepatopathy from heart failure
  • There is no role for beta-blocker treatment to prevent recurrent variceal bleeds after banding has been performed because the varices have a very low incidence of bleeding.  These patients may have upper GI bleed from other causes (gastritis, PUD, portal hypertensive gastropathy)
  • Treating individuals with HCV who are actively using drugs is part of the End Hep C SF Initiative! Patients should be counseled about the risk of re-infection
  • There are 5 reasons a cirrhotic will have worsening hepatic encephalopathy: increased gut protein load, infection, drugs, azotemia, or hypokalemia

 

Physical Exam in cirrhosis

  • Spider angiomata: the number and size of spider angiomata correlate with the severity of liver disease
  • Nail findings
    • Muehrcke nails = horizontal white bands
    • Terry’s nails = proximal 2/3 of the nail bed is white and distal 1/3 of nail bed is red
      • This is different from half-and-half nails seen in renal failure where the proximal 1/2 of the nail bed is white and the distal 1/2 of the nail bed is red
    • Both are due to hypoalbuminemia
  • Per Dr. Cello: A cirrhotic liver from alcohol is often big and feels like cooked ham whereas a cirrhotic liver from viral causes is often small and not palpable below the costal margin

Treatment of Varices

  • Pre-primary prophylaxis: Preventing variceal bleed by preventing varices.
    • Current goal is to treat underlying cause of cirrhosis leading to portal hypertension.  There is no data suggesting beta-blockers can prevent the developemnt of varices
    • We should continue screen patients with cirrhosis for varices
  • Primary prophylaxis: Preventing variceal bleeding by treating varices
    • Can treat with beta-blockers or endoscopic variceal ligation. Studies have shown similar efficacy
  • Beta-blockers reduce portal hypertension by decreasing portal venous inflow
  • Variceal ligation should be repeated until the varices are obliterated.  After they are obliterated they have a low risk of re-bleeding.

 

Treating HCV in IVDU

  • If the goal is to eliminate HCV then we need to treat the population most affected
  • Studies have shown successful (~95%) sustained virologic response in individuals treated with the direct-acting antivirals even when actively using drugs.
  • After treatment positive antibodies do not project against from re-infection
  • Reinfection rates in perviously cured individuals using drugs are ~5-10 per 100 person-years
  • SFDPH is actively treating individuals using drugs as part of their End Hep C SF Initiative

 

5 causes to look out for in a patient with hepatic encephalopathy

  • Protein load in the gut either from diet but more commonly from GI bleed
  • Infection
  • Drugs – especially drugs that have their first pass through the liver such as benzos
  • Azotemia
  • Hypokalemia worsening uremia

 

 

Moffitt Pearls 10.2.17 – Upper GI Bleed

Key Pearls for Upper GI Bleed Evaluation and Management

  • JAMA Rational Clinical Exam series: Does this patient have a severe upper GI bleed? A great resource for evaluation of a patient with suspected upper GI bleed attached below!
  • Bleeding sources proximal to the ligament of Treitz are considered Upper GI bleeds by definition

-> Top 3 causes include peptic ulcer bleed, gastritis and variceal bleed.

  • NG lavage with blood or coffee grounds has a positive LR of 9.6 for UGIB, but sensitivity is only 44%! If it’s there, you’re likely dealing with UGIB, but if it’s not, you still might be!
  • The other features that have solid likelihood ratios for UGIB are melena on exam (LR, 25) and BUN/Cr ratio >30 (LR, 7.5).
  • Blood clots in the stool make UGIB much less likely (LR 0.05).
  • The Blatchford prediction score efficiently identifies patients who do NOT need urgent scope. Score components are HR, BP, hgb, BUN, melena, syncope (as in our patient today), liver disease, and heart failure.
  • Remember: We usually use a transfusion threshold of hgb 7.0, but if someone is unstable and actively bleeding, transfuse regardless of hgb level!

Capture

http://jamanetwork.com/journals/jama/fullarticle/1105075

VA Ambulatory Report Pearls 9.6.17 – Drug Induced Liver Injury

LT Pearls from the daily ditty about bacterial meningitis

  • Listeria meningitis is the one cause of bacterial meningitis that can have a lymphocytic predominant pleocytosis
    • CSF findings in Listeria meningitis can range from 100% PMNs to 100% monocytes

Thank you to Colin for presenting a patient seen in liver clinic with a history of alcoholic cirrhosis who recently stopped drinking found to have hepatocellular injury likely due to Disulfiram.

Learning Pearls:

  • DILI can be due to many different medications. To help you narrow your list, focus on the pattern of injury (hepatocellular vs. cholestatic) and the time course (acute vs. chronic)
  • Disulfiram can cause an acute hepatocellular injury and should be used with caution and monitoring in patients with a cirrhosis
  • In patients with cirrhosis with thrombocytopenia without anemia consider other sites of Epo production such as in HCC
  • First line medication assisted treatment for alcohol use disorder include Naltrexone, Acamprosate, and Disulfiram. Choose a medication based on patient goals and side effect profile.

 

Drug Induced Liver Injury (DILI)

  • Presentation
    • Depending on the offending agent:
      • Can see acute (< 3 months) or chronic liver injury (>3 months) dependi
      • Can see hepatocellular injury or cholestatic injury
    • Symptoms can vary based on severity of injury and pattern of injury
      • Patients are often asymptomatic
      • Can have non-specific symptoms including nausea, malaise, anorexia,
      • Can also cause present with symptoms similar to other causes of acute or chronic liver injury such as jaundice, pruritus, dark urine, pale stools
    • Diagnosis
      • Clinical diagnosis which can be hard to make
        • Known offending medication started prior to laboratory abnormalities
        • Stopping the offending agent leads to improvement in liver injury
        • Negative work-up for other causes
          • Assessment of other causes should be guided by history, physical exam, labs
        • If still uncertain the patient may need a liver biopsy
  • Medications that can cause DILI
    • There are over 1000 known medications and supplements that can cause DILI
    • Most common: Acetaminophen followed by antibiotics
    • Use the pattern and time course of liver injury to help you narrow your list of offending agents
    • Great resource for medications and supplements that can cause liver injury by the NIH: https://livertox.nlm.nih.gov/index.html
  • Disulfiram induced liver injury
    • Seen within 2-12 weeks of starting Disulfiram
    • Disease severity can range from asymptomatic elevations in aminotransferases to acute liver failure and death
    • Can have immuno-allergenic fevers with fever, eosinophilia and rash
    • No hepatic dose adjustment for patients with liver failure but should be used with caution and should monitor for hepatotoxicity

 

Lab abnormalities in cirrhosis

  • Pearl from LT: In patients with cirrhosis who have a thrombocytopenia with a normal hemoglobin think about Epo production from sites other than bone marrow (such as in HCC)
  • The classic LFT pattern in patients with alcoholic cirrhosis of AST:ALT of >2 is often maintained when there is another acute insult not related to alcohol
    • There is a hypothesis that the AST:ALT ratio in alcoholic use is due to a deficiency of  pyridoxal 5′-phosphate in alcoholics, a cofactor for the enzymatic activity of ALT.  This may explain why the pattern is maintained when there is a separate liver insult.
  • Shoutout to Rabih for these awesome pearls about patterns of LFT abnormalities.

 

Medications for Alcohol Use Disorder (AUD)

Medication Mechanism of Action Adverse drug effects and contraindications Evidence Who should I use this in?
Oral Naltrexone Blocks of the mu-opioid receptor involved in the rewarding effects of drinking ADE:

Transaminitis

 

Contraindications:

ALT or AST > 150s

Use of Opiates

Reduces relapse to heavy drinking (See 1, 2)

 

Reduces alcohol consumption compared to placebo

Helpful in reducing the amount of alcohol

 

Less effective for abstinence

Depot Naltrexone (Vivitrol) Same as above, but long acting requiring monthly IM injection Same as above Decreased rate of heavy alcohol use

 

Increased the number of abstinent days

If covered by insurance and patient prefers long acting form
Acamprosate Acts at GABA and glutamate neutrotransmitters –> reduce symptoms of protracted abstinence Three times daily dosing

 

Safe to use in patients with liver failure

 

Renal dose adjustment required

 

Contraindicated in renal failure

Increased proportion of heavy drinkers who maintained abstinence in European study

 

Not demonstrated in these two US studies

Consider if the goal if abstinence and patient cannot tolerate other medication options
Disulfiram Inhibits aldehyde dehydrogenase –> accumulation of  acetaldehyde –> flushing, nausea, palpitations, headache  if alcohol is consumed Contraindicated in severe heart failure or CAD

 

Avoid in pregnant or nursing women

 

Monitor for hepatotoxicity

Most studies have shown it to be no more effective than placebo in maintaining abstinence, but may reduce drinking days

 

Most effective when given in monitored setting such as by spouse (27)

Patients highly motivated to have complete abstinence

 

Patients can use periodically for high risk social situations

Topiramate Acts at propionic acid, GABA and glutamate receptors SE: Cognitive impairment, weight loss, headache, depression.

 

Off label use

 

Titrate gradually over several weeks

Decreased consumption in patients with SUD, similar effect as naltrexone Off label use, second line medication
Gabapentin Structurally related to GABA SE: Sedation and dizziness

 

Abuse potential

Higher abstinence rates and decreased alcohol consumption in small trials Off label use, second line medication
Baclofen   SE: nausea, vertigo, sleepiness

 

Generally well tolerated

Mixed results Limited evidence of efficacy, off label use
Nalmefene Opioid antagonist SE: nausea, insomnia, fatigue, psychiatric symptoms

 

 

 

 

Taken as needed shown to reduce number of heavy drinking days Not available in the US
SSRIs Inhibits breakdown to serotonin   Reduced alcohol intake in patients with depression and alcohol use No efficacy demonstrated in patients without depression
Ondansetron 5-HT3 receptor antagonist SE: diarrhea, headache

 

QT-prolongation

Some demonstrated effectiveness in subgroups: early onset alcohol use and patients with specific genetic variant of 5-HT3 receptor Off label use, only demonstrated efficacy in sub-groups

 

The VA has lots of resources for patients with substance use disorders including: PES, Addiction consult, OTOP, and inpatient rehab.

Check out the California Society of Addiction Medicine’s guide for primary care physicians regarding patients who drink too much.

 

Moffitt Pearls – 8.11.17 – Saddle Nose Deformity, Upper GI Bleeding &Munchausen Syndrome

Thank you to HH and Neil for both presenting today! HH first presented a mini case of a patient who came in for a gout flare that was found to have a saddle nose deformity.

Neil then presented the interesting case of a young woman with a hx of gastric ulcers presented with epigastric pain and hematemesis who after extensive work-up including CT and EGD was found to have Munchausen syndrome!************************************************************************************

Key Pearls

  1. The differential diagnosis for saddle nose deformity falls into the classic triad of infectious, inflammatory and malignancy per table below.
  2. 80% of upper GU bleeds are due to four causes: peptic ulcer disease (35%), esophagogastric varices (30%), esophagitis (10%) and Mallory-Weiss tears (~5%).
  3. The management strategy for a pt. w/ munchausen syndrome is VERY difficult, but should include a single provider (w/ help from psychiatry) and goal to limit interventions + discuss diagnosis with patient in a supportive manner.

*************************************************************************************

Differential Diagnosis for Saddle Nose Deformities

Infectious Inflammatory Malignancy Other
Syphilis GCA (formerly Wegner’s) NK T-cell Lymphoma Trauma
Leprosy Sarcoidosis Locally invasive tumor (BCC) Cocaine
TB Relapsing polychondritis Lymphomatoid Granulomatosis Surgery
Cutaneous Leishmaniosis      
Septal abscess      

 Differential Diagnosis for Hematemesis AND Fever

  • Mallory-Weiss Tear
  • Peptic ulcer bleeds c/b perforation
  • Hemosuccus pancreaticus (pseudoaneurysm/aneurysm)
  • Upper GI Malignancy – hemobilia, widespread esophageal/gastric malignancy

Munchausen Syndrome or Factitious d/o Imposed on Self

  • Definition: Falsified general medical or psychiatric symptoms
  • Risk Factors: Females, Unmarried, Healthcare professional
  • Diagnostic Criteria (DSM-5):
  1. Falsification of physical or psychological signs or symptoms, or induction of injury or disease, associated with identified deception
  2. The individual presents himself or herself to others as ill, impaired, or injured
  3. The deceptive behavior is evident even in the absence of obvious external rewards
  4. The behavior is not better explained by another mental disorder, such as delusional disorder or another psychotic disorderPrognosis: Very poor even as multiple studies have shown limited benefit even with psychotherapy
  • Management: One provider should oversee pt with help of psychiatry w/ goal to limit interventions. One should be sure to exclude all possible medical conditions and then discuss diagnosis w/ pt in supportive manner

 

Moffitt Pearls 7.5.17 – Wilson’s Disease & Hemolytic Anemia

Thank you to Vincent for presenting a challenging case of a young woman with anxiety and depression presenting with unexplained jaundice and cirrhosis, later representing with a severe, hemolytic anemia. Although similar to a prior case presented last month then this was a second presentation of possible Wilson’s disease

 Top Pearls:

  1. Acute alcoholic hepatitis is a chronic disease (despite the name!). Presentation is based on predominantly cumulative burden – a single binge in a patient with otherwise minimal alcohol intake is unlikely to cause severe alcoholic hepatitis.
  2. In a young patient (< 35 yo) with new diagnosis of cirrhosis, consider genetic causes including Wilson’s, hemochromatosis, alpha-1 antitrypsin disease, autoimmune hepatitis, PBC, PSC. Vascular disease and infectious hepatitis should also be considered.
  3. The combination of liver disease and hemolysis raises concern for Wilsonian Crisis, which can herald impending acute liver failure.

Laboratory Diagnosis of Tumor Lysis Syndrome

Alcoholic Hepatitis

The clinical syndrome of acute alcoholic hepatitis includes the following compilation of laboratory and clinical features:

  • Moderately elevated transaminases in a 2:1 ratio of AST/ALT
  • Typically less than 300, rarely greater than 500
  • Elevated bilirubin and Jaundice
  • Jaundice generally develops within 3 months prior to presentation
  • Fever & Neutrophilic Leukocytosis
  • Both should only be ascribed to alcoholic hepatitis after ruling out infection!
  • Right upper quadrant pain – can often palpate tender hepatomegaly
  • Clinical history of chronic drinking with or without recent bing
  • It’s not uncommon that patients have actually decreased their drinking in the weeks-months preceding acute alc hep due to the onset of symptoms with alcohol intake.

See this RCT from NEJM regarding pentoxifylline vs. prednisolone for the treatment of alcoholic hepatitis. Bottom line:  Acute alcoholic hepatitis is a profoundly morbid disease with very high mortality (30-40% in 6 months).  Prednisolone was associated with a reduction in 28-day mortality, but did not reach significance and there were no improvements in 90-day or 1 year mortality.

Autoantibodies Associated with Causes of Cirrhosis

 

Autoimmune hepatitis

IgG, ANA, antismooth muscle Ab, anti-liver-kidney microsome-1 Ab, anti-liver cytosol Ab-1

Primary biliary cirrhosis

ANA, anti-mitochondrial Ab

Primary sclerosing cholangitis

IgM (40-50%), p-ANCA (30-80%)

Wilson Disease (WD) & Hemolytic Anemia

  • Wilson’s disease (WD) is an inherent disease, caused by mutations in the ATP7B gene leading to decreased excretion of copper into the bile
  • Copper accumulation results in injury to the liver and the central nervous system (thank you Muazzum for you amazing neuro ROS)
  • WD presents in a fulminant form with hepatocellular dysfunction, hemolysis and various multiorgan failures (Wilson’s crises)
  • Wilson disease can cause a Coombs-negative hemolytic anemia WITHOUT schistocytes reported in the literature
  • The exact mechanism of the haemolytic process has yet to be defined. copper inhibits sodium potassium ATPase in the erythrocyte leading to haemolysis (and likely without schistocytes as in this case).
  • Medical treatment includes chelating agents and/or zinc, but these have not proved effective in fulminant liver failure, where only liver transplantation (LTx) is regarded as lifesaving.
  • Some limited data for plasmapheresis for rapid copper removal
  • Portends very poor prognosis, often leading to acute liver failure

 (see attached an article on a review of Wilson’s and Hemolytic anemia compliments of HH!!)

Blast from the past!!! Approach to Hemolytic Anemia (thank you Katie!!)

 

 

https://www.evernote.com/shard/s307/sh/16c1d172-fb95-4da7-93b0-d85e5c25854f/3768012329b5e8d8e358cacd312f033a