Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is the first reported case of lymphoproliferative disease presenting with adrenal insufficiency after liver transplantation. A 38-year-old white man was admitted 8 months after transplantation for cryptogenic cirrhosis with fever (38-39 degrees C), chills, cough, and dyspnea. His blood pressure was 100/70 mm Hg, there was pallor of the conjunctiva, and a lymph node was palpable in the left groin. Laboratory analyses revealed the following values: serum sodium concentration (112 mmol/L), potassium (5.4 mmol/L), hemoglobin (7.8 g/L), white blood cell count (7.7 x 10(9)/L), glucose 3.9 (mmol/L), and mildly elevated liver functions. Abdominal ultrasound showed multiple hypoechoic solid-appearing lesions throughout the liver and spleen. Results of a biopsy specimen of the groin node confirmed polymorphic B-cell lymphoma. A negative Epstein- Barr virus screen before transplant became positive. The patient's fever increased to 40 degrees C. He subsequently developed sepsis and later, multiple organ failure. Autopsy confirmed extensive abdominal disease. The adrenal glands had been completely replaced by the tumor. Primary Epstein-Barr virus infection is associated with posttransplant lymphoproliferative disease. Replacement of the adrenal glands with a tumor produces a clinical picture of adrenal insufficiency.
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PMID:Posttransplant lymphoproliferative disease presenting as adrenal insufficiency: case report. 1598 81

A 69-year-old man had radiofrequency ablation therapy (following RFA) for type C cirrhosis with hepatoma (following HCC) of S7 in November 2001. Afterward the patient was followed as an outpatient, but he had been admitted to our hospital due to jaundice confirmed in March 2004. His abdominal wall appeared to be soft and flat, and we could not detect a tumor mass by palpating either. Even though he exhibited no actual symptom of anemia, jaundice was found in the bulbar conjunctiva at the time of admission. Laboratory findings showed a mild inflammation and anemia on his admission, and biochemical data showed a rise of hepatobiliary enzyme with jaundice. A rise of tumor marker (AFP, PIVKA-II) was recognized, too. We performed percutaneous transhepatic bile duct drainage (following PTBD) to decrease jaundice because abdominal echography and CT showed an extension of tumor thrombosis in bile duct and right hepatic duct by HCC of S8. However, a check of T-Bil. was 7.29 mg/dl and showed some slight decrease. Therefore, we administered prostaglandin E1 (following PGE1) at first with an intra-arterial injection catheter aiming to protect the hepatocyte. One week later, we performed hepatic artery injection chemotherapy (CDDP+5-FU) for four weeks. We confirmed a manifested improvement in T-Bil to be 1.92 mg/dl at the end of hepatic artery injections as well as a manifested decrease in hepatobiliary enzyme. We confirmed a decrease of HCC of S8 by abdominal CT, and the response rate was PR. Afterward the patient was conservatively treated even though pancytopenia was present, and was discharged from the hospital in June 2004. The hepatic artery injection chemotherapy used together with PGE1 was effective for the HCC patient with jaundice.
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PMID:[The hepatic artery injection chemotherapy and prostaglandin E1 administration for hepatocellular carcinoma invading the biliary tract with jaundice]. 1631 59

A 64-year-old woman was referred to our hospital with jaundice of the bulbar conjunctiva and general fatigue. After admission, she developed hepatic encephalopathy and was diagnosed with fulminant hepatitis based on the American Association for the Study of Liver Disease (AASLD) position paper. Afterwards, additional laboratory findings revealed that serum ceruloplasmin levels were reduced, urinary copper levels were greatly elevated and Wilson's disease (WD)-specific routine tests were positive, but the Kayser-Fleischer ring was not clear. Based on the AASLD practice guidelines for the diagnosis and treatment of WD, the patient was ultimately diagnosed with fulminant WD. Then, administration of penicillamine and zinc acetate was initiated; however, the patient unfortunately died from acute pneumonia on the 28th day of hospitalization. At autopsy, the liver did not show a bridging pattern of fibrosis suggestive of chronic liver injury. Here, we present the case of a patient with clinically diagnosed late-onset fulminant WD without cirrhosis, who had positive disease-specific routine tests.
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PMID:Clinically diagnosed late-onset fulminant Wilson's disease without cirrhosis: A case report. 2937 14

Fibrosis is characterized by excessive deposition of the extracellular matrix and develops because of fibroblast differentiation during the process of inflammation. Various cytokines stimulate resident fibroblasts, which differentiate into myofibroblasts. Myofibroblasts actively synthesize an excessive amount of extracellular matrix, which indicates pathologic fibrosis. Although initial fibrosis is a physiologic response, the accumulated fibrous material causes failure of normal organ function. Cardiac fibrosis interferes with proper diastole, whereas pulmonary fibrosis results in chronic hypoxia; liver cirrhosis induces portal hypertension, and overgrowth of fibroblasts in the conjunctiva is a major cause of glaucoma surgical failure. Recently, several reports have clearly demonstrated the functional relevance of certain types of histone deacetylases (HDACs) in various kinds of fibrosis and the successful alleviation of the condition in animal models using HDAC inhibitors. In this review, we discuss the therapeutic potential of HDAC inhibitors in fibrosis-associated human diseases using results obtained from animal models.
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PMID:HDAC Inhibitors: Therapeutic Potential in Fibrosis-Associated Human Diseases. 3088 85