Hepatology Flashcards
When do we use Albumin for SBP? Why?
The ability of albumin to improve intravascular volume and bind inflammatory cytokines has led to the study of albumin therapy in patients with SBP. The published literature suggests that albumin in combination with antibiotics prevents renal impairment and reduces mortality in SBP. The benefit is most appreciated in higher-risk patients with elevated bilirubin levels and evidence of renal dysfunction, and negligible in SBP patients with normal bilirubin levels and renal function.
HFHS criteria: creatinine above 1, BUN above 30, or total bili above 4.
In Acute Alcoholic hepatitis what Liver marker elevations do we see? What major mistake do we want to avoid with these patients?
acute alcoholic hepatitis, the serum AST level is almost never greater than 500 U per L and the serum ALT value is almost never greater than 300 U per L
Patients with alcoholic hepatitis can present with jaundice, abdominal pain, fever and a minimally elevated AST value, thereby leading to a misdiagnosis of cholecystitis. This is a potentially fatal mistake given the high surgical mortality rate in patients with alcoholic hepatitis
Define Acute Liver failure
Acute liver failure (ALF) is defined as rapid development of hepatocellular dysfunction (INR >1.5) and mental status changes (HE, any stage) in a patient without preexisting liver disease within 26 weeks of onset of illness…regardless of ALT level
Non hepatic causes of elevations in Liver enzymes?
- Skeletal muscle damage (rhabdomyolysis)
- Strenuous exercise
- Cardiac muscle damage
- Thyroid disease
- Macro-AST
- Heat stroke
- Hemolysis
- Adrenal insufficiency
- Anorexia nervosa
What is a ratio to use to help determine etiology of liver enzyme elevation?
- LDH reversibly catalyzes conversion of lactate and NAH to pyruvate and NADH
- Serum LDH activity is very high in patients with ischemic hepatitis and neoplasms with hepatic involvement
- ALT:LDH <1.5 ischemic/toxic hepatitis
- ALT:LDH >1.5 viral hepatitis
Sensitivity 94%
Specificity 84%
ALT greater than 1,000 need to think what 3 etiologies?
ALT >1,000 IU/l. In this study, we have confirmed that the main causes of a dramatic ALT rise are ischemic liver injury, DILI and viral hepatitis
What is the treatment for Hereditary hemochromatosis? Explain the general idea and then specific data points hepatology uses?
The most appropriate initial management for patients with hemochromatosis is to lower the total body iron as rapidly as possible through the use of weekly phlebotomy if tolerated. Check patient’s normal hemoglobin, if normal, they will tolerate the removal of 1 unit of blood weekly to help reduce his iron stores.
The goal of treatment in the setting of hemochromatosis is to lower total body iron levels in an effort to prevent worsening organ failure secondary to iron deposition. Although both phlebotomy and deferoxamine may be used to decrease iron stores in patients with suspected hemochromatosis, initiating therapy with weekly phlebotomy is both cost-efficient and effective. In patients without anemia and signs of iron overload, phlebotomy is preferred, as it is easy, cheap, and without significant side effects. Phlebotomy should be performed weekly until ferritin levels fall to 50 to 100 μg/L or until the hemoglobin is less than 11 g/dL. At this time, maintenance phlebotomy can be performed every 2 to 4 months with or without deferoxamine.
The indications for phlebotomy include:
Serum ferritin ≥1000 ng/mL (≥2247 pmol/L) and often ≥500 ng/mL (≥1124 pmol/L)
Tissue injury due to iron overload such as transaminitis or cardiac dysfunction
Increased tissue iron seen on MRI or other study or pathology
Phlebotomy should not be used in asymptomatic individuals, even with known hemochromatosis, if serum ferritin is not ≥500 ng/mL (≥1124 pmol/L) and there are no signs of iron deposition. There is some evidence that phlebotomy may improve hepatic fibrosis, cardiomyopathy, and hypogonadism. It is not known to improve diabetes or arthritis.
What is Fitz-high-Curtis syndrome?
progressive, pleuritic, right upper quadrant pain associated with purulent vaginal discharge and mild elevation in her transaminases without other liver pathology evident on imaging or laboratories. This is consistent with perihepatitis secondary to pelvic inflammatory disease (PID), also called Fitz-Hugh-Curtis (FHC) syndrome.
Fitz-Hugh-Curtis (FHC) syndrome is an extrapelvic manifestation of acute pelvic inflammatory disease (PID) and has been shown to occur in 4-10% of cases. FHC occurs in the setting of PID in which there is the development of perihepatitis or inflammation of the liver capsule and adjacent peritoneum rather than hepatitis that refers to inflammation of the liver stroma itself. The characteristic symptoms of FHC include right upper quadrant pain, often with pleuritic chest pain or flank pain due to diaphragmatic irritation. On occasion, this can be referred to the right shoulder, as in this patient.
Sometimes, the right upper quadrant pain is the presenting symptom and can mask the underlying diagnosis of PID. In the setting of perihepatitis, the liver stroma is usually unaffected and aminotransferases remain relatively normal with mild variation depending on inflammation. FHC can be diagnosed on laparoscopy by visualization of purulent or fibrinous exudate between the liver capsule and abdominal wall (“violin string” adhesions). There are three theories regarding the development of this condition: that there is ascending infection through which microbes travel into the peritoneum through the fallopian tubes, lymphatic spread, or hematogenous spread. It is not clear which of these is to blame for which presentation of this disease.
Solid liver lesions in women of childbearing age?
In women of child-bearing age, the primary considerations for common causes of solid liver lesions include hepatic adenoma, focal nodular hyperplasia, or hepatic hemangioma.
Focal nodular hyperplasia findings?
This lesion has the classic appearance of focal nodular hyperplasia (FNH) and can be remembered by the fact that FNH will have a central scar.
NH is a tumor-like condition that is common in young to middle-aged adults and occurs more commonly in women (90%) than men. It is the second most common benign hepatic tumor, constituting about 0.3 - 3% of primary hepatic tumors based on autopsy series. The pathophysiology of FNH is not fully understood, however, it is thought to be a hyperplastic (proliferation of normal hepatocytes) response to several hepatic abnormalities such as an aberrant dystrophic artery, arterial-venous shunt from a portal tract injury, or congenital vascular malformation. Gross findings of the mass include a poorly encapsulated nodule with a central depressed stellate scar that contains a large central artery that feeds the remainder of the lesion. It is often solitary.
How do you diagnose Wilson’s disease?
A diagnosis of Wilson’s disease is established if the hepatic copper concentration is ≥250 mcg/g dry weight. Molecular testing for an ATP7B mutation can also be helpful in the diagnosis of Wilson’s disease, especially in siblings of affected patients.
Patients with hemochromatosis are at risk for infections with which organisms?
Patients with iron overload are at increased risk of infections from siderophilic organisms. These are bacteria whose virulence is markedly increased in the presence of excess iron. Organisms such as Vibrio vulnificus and Yersinia enterocolitica fall into this category. Y enterocolitica is a gram-negative organism that is contracted through food and can cause mesenteric lymphadenitis, diarrhea, fevers, and abdominal pain, and may mimic appendicitis in children. V vulnificus is also a gram-negative organism but is associated with seawater. It can cause infection both through skin inoculation at the site of a wound or through ingestion. Skin and soft tissue infections can be quite severe, leading to gangrene and often requiring surgical debridement or amputation. Ingestion can lead to severe sepsis. V vulnificus can be ingested in undercooked seafood. For this reason, patients with iron overload should be counseled to avoid undercooked seafood as this raises their risk of systemic infection.
What are the most common benign tumors of the liver?
Cavernous hemangiomas are the most common benign tumors of the liver. They are best diagnosed with contrast-enhanced CT scanning. Rarely, they can rupture and lead to a retroperitoneal hemorrhage.
What is PBC? Treatment?
The most likely diagnosis is primary biliary cholangitis (PBC) given this patient’s elevated liver enzymes and positive antimitochondrial antibody titer. PBC involves granulomatous destruction of the intrahepatic ducts within the portal triads, without affecting the extrahepatic ducts. It is an autoimmune disorder that mainly affects middle-aged women and can increase the risk of developing hepatocellular carcinoma.
Ursodeoxycholic acid has been shown to slow the progression of PBC and is the most important medical therapy. Multiple mechanisms have been proposed including protection of injured cholangiocytes against toxic effects of hydrophobic bile acids and inhibition of apoptosis of hepatocytes. However, the exact mechanism is unknown. Ursodeoxycholic acid does not improve pruritis, which is when cholestyramine or diphenhydramine can be prescribed. Liver biochemical testing will usually show improvement within 3 months of starting therapy. The majority of patients will see sustained improvement with this therapy. For those who do not show improvement, a liver transplant can be considered. Liver transplantation has shown very promising results in patients with end-stage PBC and should be offered after medical therapies have failed.
What are hallmark features in labs of a window period for Hep B?
Isolated antibodies to the core antigen (anti-HBc) seen on her hepatitis panel are the hallmark of the window period and indicate that she was likely admitted for an acute hepatitis B infection multiple months ago and has now convalesced. The fact that she has cleared her hepatitis B surface antigen means she has cleared her infection completely and within the next few weeks will have the appearance of hepatitis B surface antibodies. Remembering that the window period exists is important as patients with prior acute hepatitis B may actually be immune, but not yet show evidence of hepatitis B surface antibodies.
