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)

Cytophagic histiocytic panniculitis (CHP) is a recently recognized entity that frequently poses a perplexing diagnostic problem. Although the classical case presents with a relapsing fever, subcutaneous nodules, pancytopenia and liver dysfunction, most patients have in addition a multitude of other manifestations which confuse the clinical picture. Notwithstanding the variable clinical course, the disease frequently terminates in fatal hemorrhage. Diagnosis is based on histological features. A lobular panniculitis with an infiltrate of cytologically benign cytophagocytic histiocytes in skin nodules is the sine qua non of CHP. Hence, a deep skin biopsy which includes subcutaneous fat is mandatory to establish the diagnosis. Published information regarding this newly described entity remains scarce and we report two cases of CHP, one occurring in a 30-year-old Kadazan man and another in a 17-year-old Chinese woman seen at the University Hospital, Kuala Lumpur. The latter case presented with exudative ascites, an unusual feature, possibly due to intra-abdominal panniculitis. In addition, we record the development of cirrhosis in the same patient.
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PMID:Cytophagic histiocytic panniculitis: a diagnostic dilemma. 129 33

Clinically, idiopathic portal hypertension (IPH) is characterized by overt splenomegaly with pancytopenia, portal hypertension and relatively mild abnormalities in liver function tests. Although its etiology is still undetermined, the liver pathology is characterized by occlusive changes of the intrahepatic portal radicles, portal and periportal fibrosis, irregularly distributed parenchyma atrophies and absent of regeneration nodules. The disease is relatively benign and does not progress to cirrhosis. Differential diagnosis between IPH and liver cirrhosis is mandatory. We now report a case with histologically proven IPH, including clinical course, laboratory data, roentgenographic findings of hepatic venogram and celiac angiogram, hepatic hemodynamic features and intravariceal pressure of esophageal varix which has never been reported in Taiwan.
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PMID:Idiopathic portal hypertension: a case report. 132 89

A 36-year-old woman was admitted to our hospital because of general fatigue. The physical and laboratory findings on admission revealed splenomegaly, pancytopenia, hypocoagulopathy, liver hypofunction with a hepaplastin test of 55% and ICG Rmax of 0.6 mg/kg/min. Diagnostic imaging showed a hypoechoic mass 1.5 in diameter a low density area on the CT scan and a faint tumor stain on the AAG in the posterior inferior area of the liver. On a diagnosis of hepatocellular carcinoma with liver cirrhosis and hypersplenism, partial hepatectomy and splenectomy were performed. The resected hepatic specimen revealed a small liver cancer of 1.9 x 1.5 x 1.3 cm with liver cirrhosis. The specimen consisted of a firm rubbery mass. Macroscopically, the tumor appeared oval and was lobulated with a thin capsule. A fibrous scar was observed in the central area. Microscopically, malignant hepatocytes showed various shapes, ranging from polygonal to spindle form, with eosinophilic granular cytoplasm and were surrounded by abundant fibrous stroma. Orcein stain, revealed that these malignant hepatocytes contained many black granules of copper-binding protein. Immunoperoxidase staining for alpha 1-antitrypsin was also positive in the malignant hepatocytes. However, within this lamellar fibrous regions, there were many cords of tumor cells in which nucleoli were absent and abortive biliary differentiation was suggested. Consequently this tumor was diagnosed as an atypical fibrolamellar hepatocellular carcinoma. We think that this case is the 3rd case reported in Japan and the 2nd case in a Japanese person.
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PMID:Fibrolamellar carcinoma of the liver--a case report. 165 47

Infertility and amenorrhea are reported in most cases of Wilson's disease. In this report, we describe a case of Wilson's disease with pancytopenia and liver cirrhosis for over 4 years, without any specific treatment. After 2 years of D-penicillamine therapy, the patient became pregnant and delivered a liver mature female baby with a body weight of 2,800 g. Both before the pregnancy and after delivery, brainstem auditory evoked potential studies showed similar bilaterally abnormal prolongation in the III-V and I-V intervals. In visual evoked potentials, the P 100 latency was delayed bilaterally. Although serial evoked potential studies failed to show any improvement, a successful pregnancy was proven to be possible in a patient with Wilson's disease who had received regular D-penicillamine treatment.
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PMID:Successful pregnancy after D-penicillamine therapy in a patient with Wilson's disease. 168 Oct 22

In a 59-year-old patient presenting in October 1981 with pancytopenia, hairy cell leukemia was diagnosed. Splenectomy, followed by treatments with oncovin, lithium, and prednisone were essentially without effect. Up to July 1984 the patient had been regularly transfused with a total of 62 unit. In June 1984 he acquired a transfusion associated hepatitis C which followed a chronic course and resulted in biopsy proven cirrhosis in 1989. The patient became independent of transfusions in July 1984. Repeated blood counts have shown a complete hematologic remission which has now lasted nearly 6 years, whereas focal leukemic infiltrates have persisted in the bone marrow. The patient has tolerated 20 courses of erythropheresis performed because of biopsy proven severe hepatic siderosis without a fall in hemoglobin. It is suggested that the spontaneous long-lasting hematologic remission of hairy cell leukemia is due to endogenous interferon produced in the course of chronic hepatitis C. Low serum levels of interferon-alpha and -gamma were detected.
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PMID:[Peripheral spontaneous remission of hairy cell leukemia following transfusion-associated hepatitis C]. 190 85

A 58-year-old female was diagnosed as mitral regurgitation and tricuspid regurgitation, combined with liver cirrhosis (T. Bil 2.3 mg/dl, ICG-R 37%) and pancytopenia (Hb 9.4 g/dl, WBC 3,000, Plt 56,000). She underwent mitral and tricuspid annular plications. After extracorporeal circulation, the postoperative platelet count was maintained higher than preoperative one by transfusion of platelet-rich plasma. Administration of FOY was begun during operation. Levels of T. Bil. and BUN were highest at 15th postoperative day and decreased gradually. Postoperative pleural effusion was treated by administration of 25% albumin solution keeping the level of Alb. up 3.5 mg/dl.
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PMID:[A case of open heart surgery associated with liver cirrhosis and pancytopenia]. 194 90

A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.
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PMID:[Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus]. 259 83

A 76-year-old woman, who had suffered from liver cirrhosis, was referred to our hospital because of pancytopenia. Her peripheral leukocyte count was 2,500/microliters with 43% myeloblasts, hemoglobin at 9.0 g/dl and platelet count of 0.9 x 10(4)/microliters. Aspirate from bone marrow showed hypercellular marrow with 52% myeloblasts. No chromosomal abnormality was detected. She was diagnosed as acute myelogenous leukemia (AML, M2). The diagnosis of liver cirrhosis was confirmed by laboratory data and findings of abdominal sonography. Moreover, she had valvular aortic stenosis. These complications made it difficult to treat her with combined chemotherapy containing anthracycline antibiotics, so she was given a small dose of cytosine arabinoside (Ara-C, 10 mg/body/12 hr) for 18 days. After severe myelosuppression, complete remission was achieved. The highest serum concentration of Ara-C was obtained at 15 min after subcutaneous injection of Ara-C; thereafter the Ara-C concentration decreased immediately within 60 min in a pattern similar to that observed in patients without liver cirrhosis. Thus, low-dose Ara-C regimen might be a useful treatment for aged patients with AML, even complicated with liver cirrhosis.
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PMID:[An aged patient with acute myelogenous leukemia complicated with liver cirrhosis: successful treatment with low-dose cytosine arabinoside]. 275 21

Three cases of idiopathic portal hypertension associated with Hashimoto's disease are described. All of the cases were middle-aged Japanese women showing splenomegaly, esophageal varices and pancytopenia in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Two patients were euthyroid with goiter, one of which revealed diffuse lymphocytic infiltration, obliteration of thyroid follicles, and fibrosis on histological examination of the thyroid; the third suffered from myxedema without goiter. Antithyroid microsomal antibody was positive in all patients and antithyroglobulin antibody was positive in none. These findings might imply an immunological role in the pathogenesis of idiopathic portal hypertension.
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PMID:Idiopathic portal hypertension associated with Hashimoto's disease: report of three cases. 294 77

An unusual hepatic disease developed in 3 patients with a well-functioning kidney graft 16-24 months after transplantation. Vague abdominal pain, increased bleeding tendency and edema were initial complaints, and hepato- or splenomegaly and ascites were found as well. Liver function tests were not or only mildly disturbed; hemolysis and pancytopenia were always present. Colloid uptake was absent at liver scintigraphy and the hepatic venous wedge pressure was increased. Esophageal varices were demonstrated. Liver biopsy showed extensive midzonal and pericentral sinusoidal dilatation. After discontinuation of azathioprine the symptoms and the extent of sinusoidal dilatation disappeared gradually, but after 1-3 years fibrosis or micronodular cirrhosis had developed and splenomegaly with hypersplenism remained. These observations strongly suggest an association between chronic use of azathioprine and the development of venous congestion of the liver with sinusoidal dilatation, eventually resulting in chronic liver disease.
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PMID:Hepatic sinusoidal dilatation with portal hypertension during azathioprine treatment after kidney transplantation. 390 52


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