Wednesday, 4 June 2008

hepatosplenomegaly



exam question:
hepatosplenomegaly,signs of chronic liver disease.
asked to discuss causes of portal hypertension.
asked about the management of a patient with acute hepatic encephalopathy.

causes of hepatosplenomegaly:
1-Infection:
Acute viral hepatitis
Infectious mononucleosis
Cytomegalovirus
2-Haematological disease:
Myeloproliferative disorders :CML,Myelofibrosis.
Lymphoproliferative disorders:CLL, Hodgkin's Lymphoma
Anaemia:Pernicious anaemia ,Sickle cell anaemia,Thalassaemia
3-Chronic liver disease and portal hypertension:
Chronic active hepatitis
4-Amyloidosis
5-Acromegaly
6-Systemic lupus erythematosus

another exam question:
anaemic patient with hepatosplenomegaly.
most likely diagnosis:myelfibrosis
discuss.

6 comments:

  1. The causes of portal hypertension can be divided into 3:
    (1) Posthepatic:
    Right-sided heart failure.
    Constrictive pericarditis,.
    Budd-Chiari syndrome (BCS).
    (2) Prehepatic:
    Portal vein thrombosis (PVT).
    Portal compression or occlusion by biliary and pancreatic neoplasms and metastases.
    Increased flow secondary to arterioportal fistula, pancreatic arteriovenous malformations, and massive splenomegaly
    (3) Intrahepatic:
    Cirrhosis.

    The common feature to all the causes is an increased resistance to portal venous flow, although in a few cases, increased inflow into the portal venous system is present.

    what are the causes of cirrhosis?

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    Replies

    1. Causes of Liver Cirrhosis Mnemonic
      Causes of Liver Cirrhosis Mnemonic

      A - Alfa 1 antitrypsin deficiency
      B - Budd Chiary Syndrome - Hepatitis B Virus - Biliary cirrhosis - Bilharzia
      C - Hepatitis C Virus - Cystic fibrosis - copper overload
      D - Drugs
      E - Ethanol
      F - Fe overload
      G - Glycogen storage disease


      and while we are at it, lets review the complications:


      Bruising and bleeding due to decreased production of coagulation factors.
      Jaundice due to decreased processing of bilirubin.
      Itching (pruritus) due to bile salts products deposited in the skin.
      Hepatic encephalopathy.
      Sensitivity to medication due to decreased metabolism of the active compounds.
      Hepatocellular carcinoma
      Portal hypertension
      Ascites
      Esophageal varices
      Immune system dysfunction, leading to infection.
      Hepatorenal syndrome - insufficient blood supply to the kidneys, causing acute renal failure.
      Hepatopulmonary syndrome, leading to cyanosis and dyspnea (shortness of breath), characteristically worse on sitting up.

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    2. F also can be due to fatty liver (diabetes and obesity)

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  2. MYELOFIBROSIS
    Increased fibrosis in the bone marrow is caused by hyperplasia of abnormal megakaryocytes which release fibroblast-stimulating factors such as platelet-derived growth factor.
    clinical features
    The disease presents insidiously with lethargy, weakness and weight loss.
    massive splenomegaly
    bone pain and attacks of gouts.
    Bruising and bleeding occur because of thrombocytopenia or abnormal platelet function. Other physical signs include anaemia and fever .
    management:
    1-Supportive care only is suitable for elderly patients with other medical problems.
    2-'Gentle' chemotherapy (low-dose or single-agent, e.g. azacytidine) may be useful in patients with high WBC counts.
    3-Intensive chemotherapy schedules used for acute myeloblastic leukaemia may be tried in patients under the age of 60, but the remission rate is less, and prolonged pancytopenia may occur owing to poor haemopoietic regeneration because of the defect in stem cells.
    4-Bone marrow transplantation offers the hope of cure in the small proportion of MDS patients who are under the age of 50 and who have an HLA-identical sibling or an unrelated HLA-matched donor.

    ReplyDelete
  3. complications of chronic liver disease: HEPATIK
    HE = HEpatic encephalopathy
    P = Portal hypertension (+ variceal haemorrhage)
    A = Ascites
    T = Tumour (hepatoma)
    I = Infection (spontaneous bacterial peritonitis)
    K = Kidney failure (hepatorenal syndrome)

    ReplyDelete
  4. THIS WAS REALLY HELPFUL! GOD BLESS YO'ALL

    ReplyDelete