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Type of hyperbilirubinemia Diagnostic Indicators Management Recommendations
Managment of isolated unconjugated jaundice, hemolytic Any H/O recent trauma, hematoma or blood transfusion
Any recent travel
Inquire about medications that can cause hemolysis
Any positive family history of hemolytic anemia
 Work-up and detect the cause of hemolysis, if low Hb, High LDH, Low haptoglobin, and reticulocytes present
 G6PD deficiency - mostly recover on its own, if progresses to hemolytic anemia, oxygen therapy or blood transfusion may be required. Avoid of precipitants and etiological factors
 Spherocytosis, phototherapy and/or exchange transfusion for infants, Folic acid for maintaining erythropoiesis. Splenectomy is the definitive treatment
 Sickle cell anemia reduce pain and prevent complications, blood transfusions and supplemental oxygen, as well as a bone marrow transplant.
 Immune-related hemolysis – corticosteroids, folic acid is the main line of treatment
 Parasitic Infections like malaria are treated with antimalarial drugs like chloroquine, artesunate, lumefantrine, amodiaquine
 Ineffective erythropoiesis- iron and folic acid & B12 supplementation, repeated blood transfusions
Managment of isolated unconjugated jaundice, Non-hemolytic  Gilbert's syndrome does not produce symptoms or adverse effects and patients have a normal life span
Crigler-Najjar type I is fatal in early life due to development of kernicterus.
Crigler-Najjar type II is compatible with a normal life.
 Phenobarbital can decrease serum bilirubin by enzymatic induction of UDPGT in Crigler-Najjar type II.
Managment of isolated conjugated jaundice ❑Dubin- Johnson syndrome doesn't produce any symptoms and compatible with a normal life span.
❑Rotor's syndrome is a harmless chronic hyperbilirubinemia
❑Suspect for Dubin-Johnson syndrome before considering surgery if the healthy patient with long-standing conjugated hyperbilirubinemia, other normal liver function tests, and a non visualized gallbladder.
Hepatic architecture is normal but there is an accumulation of hepatic pigment compatible with melanin in patients with Dubin-Johnson syndrome
❑In Rotor's syndrome the gallbladder opacifies normally with cholecystographic dye and no pigmentation be seen in the liver.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ⇈AST/ALT out of proportion to ALK-P' ❑H/O recent travel
❑ Social history: Alcohol, sexual history
❑ Family history of Wilson's disease or hemochromatosis
❑Review medications
❑Pregnancy test
HELLP ,AFLD
❑Toxicology screen
Acetaminophen level
❑Consider work-up for rare cases
Liver biopsy if results negative
❑Viral hepatitis
Hepatitis A: mostly self-limiting
Hepatitis B treated with antiviral medications
Hepatitis C is treated with interferons
Other Viral infections like EBV, CMV, HSV are treated with Antiviral medications
Alcohol hepatitis: Alcohol abstinence, glucocorticoids, pentoxifylline
 Wilson"s disease: chelating agents such as D-penicillamine
 Drug toxicity treatment(e.g. Acetaminophen, Isoniazid
 Autoimmune hepatitis treatment with glucocorticoids
 Avoid drugs associated if any(e.g.kavakava)
❑Mild elevation in aminotransferase levels in female patients with concomitant autoimmune disorders (e.g., autoimmune thyroiditis, connective tissue diseases) is suggestive of autoimmune hepatitis.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ⇈ Alk-P out of proportion to AST/ALT H/O intermittent right upper quadrant pain radiating to the back or right shoulder favors gallstones, fever and chills suggest cholangitis.
H/O biliary tract surgery within 2 years should alert the physician to possible biliary stricture.
H/o recent weight loss, constant epigastric or right upper quadrant pain radiating to the back suggests malignancy
icteric patient with extrahepatic obstruction due to gallstones or postsurgical biliary stricture has usually had acute symptoms for less than 2 weeks
Those with carcinoma, chronic pancreatitis, or primary sclerosing cholangitis have had symptoms of longer duration
❑ A middle-aged women with a history of itching and autoimmune disease raises the suspicion of primary biliary cirrhosis
More than half the people with primary biliary cholangitis do not have any symptoms when diagnosed. Symptoms develop over the next five to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes.
Commonly used drugs such as antihypertensives (e.g., angiotensin-converting enzyme inhibitors) or hormones (e.g., estrogen) may cause cholestasis
Abnormal ALk-p levels may be a sign of metastatic cancer of the liver, lymphoma or infiltrative diseases such as sarcoidosis.
H/O inflammatory bowel disease (most commonly ulcerative colitis) suggest the presence of primary sclerosing cholangitis since about 70% of these cases are associated with inflammatory bowel disease
Obstruction removal by ERCP, PTC, Surgery (e.g.Cholecystectomy or Palliative Bypass procedures such as hepaticojejunostomy if stenting has failed in patients with tumors)
Primary biliary cholangitis management: no cure for primary biliary cholangitis, but medications are available for slow the progression and prevent complications of the disease: Ursodeoxycholic acid (UDCA), Obeticholic acid (Ocaliva), Fibrates, Liver transplantation may help prolongs life.
Managment of conjugated & unconjugated hyperbilirubinemia jaundice with ↑ INR,↓ Alb,↓ PLt Compensated Cirrhosis Mangament
Alochol abstinence
Antiviral medications for viral hepatitis
avoidance of hepatotoxic medications
vaccination
Decompensated Cirrhosis Managment
Managment of complications:
Varices,Asciets,Hepatorenal syndrome,Hepatic encephalopathy