Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

A study was made of the clinical picture, certain laboratory and immunological findings in pseudotuberculosis patients with involved joints in the presence of opisthorchiasis invasion. The patients suffering from pseudotuberculosis and chronic opisthorchiasis frequently demonstrated icteric sclera, erythema nodosum, skin itch, tenderness in the right hypochondrium, hepatomegaly, liquid stool, leukopenia, eosinophilia, hyperbilirubinemia, activation of aminotransferases. Later they showed the diagnostic titers of the indirect hemagglutination test, an increase in disease duration, delayed disappearance of the main clinical symptoms. As compared to patients suffering from arthritides without invasion, the patients with associated pseudotuberculous arthritides and opisthorchiasis demonstrated a higher activity of the inflammatory process in the joints, an increase of the duration and number of polyarthritis relapses, alterations in the immunological indicators (a stable reduction of the T lymphocyte and T helper count as well as a rise of the IgM level during late convalescence).
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PMID:[The characteristics of pseudotuberculosis with joint involvement in the presence of chronic opisthorchiasis]. 181 61

Thirty-five patients with primary biliary cirrhosis were seen between 1974 and 1989. The median mean age at presentation was 53 years (range: 30-77) and the female to male ratio was 7.8:1. Thirteen (37%) were asymptomatic and nine (26%) had associated auto-immune disorders. Pruritus and hepatomegaly were present in half of the patients. Advanced histological stages of disease (Stages 3 and 4) were present in 57% of patients. The median period of follow-up was 5 years (range: 0.1-20). Twelve patients died, nine from hepatic causes and three from non-hepatic causes. One has undergone liver transplantation. A Kaplan-Meier curve estimated the median survival to be 11 years. Asymptomatic patients developed progressive disease and survival was similar to that of symptomatic patients. Using Cox's proportional hazards model, age, serum bilirubin and serum albumin were found to be independent prognostic variables correlating with reduced survival.
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PMID:Primary biliary cirrhosis: clinicopathological characteristics and outcome. 212 33

Six cases of telangiectatic clinical variant of mastocytosis (TMV) are reported. Patients' age varied from 33 to 62, there were 2 men and 4 women. A number of clinical features of TMV were detected in the course of follow-up: a generalized type of the skin process; abundant maculopapular eruptions colored brownish, sometimes with a cyanotic shade, liable to fuse and form diffuse foci of involvement; development of telangiectasias on the surface of these eruptions; symptoms of elevated blood histamin levels, i.e. regular blood congestion, enhanced itching, tachycardia, gastrointestinal events, etc.; systemic mastocytosis (3 of the 6 patients developed lymphadenopathy and hepatomegaly). The detected features of the condition permit considering TMV as an individual clinical variety of mastocytosis.
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PMID:[A telangiectatic clinical variant of mastocytosis]. 228 58

Primary biliary cirrhosis (PBC) or chronic non-suppurative destructive cholangiohepatitis is rare in southern Africa. Eight patients with this diagnosis were identified and fully investigated at Groote Schuur Hospital between 1980 and 1988. Seven patients were female, all were white or coloured, and their ages ranged from 49 years to 80 years. All patients presented with a history of malaise, fatigue, night sweats and pruritus, which had been present for 3 months--12 years before diagnosis of PBC. Initial misdiagnosis had resulted in unnecessary invasive investigations including laparotomies. Signs of chronic liver disease, such as xantholasmas, evidence of pruritus, the sicca syndrome or hepatomegaly, were invariably present. Marked elevation of serum alkaline phosphatase level and IgM were present in all cases. Antimitochondrial antibodies were positive in significant titre in 7 of the 8 patients. Liver biopsies demonstrated stage II-III disease in all patients. Therapy was chiefly supportive and symptomatic although most patients received immunosuppressive agents. Despite the late presentation, the subsequent course was similar to that seen elsewhere where patients are recognised earlier.
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PMID:Primary biliary cirrhosis. A retrospective survey at Groote Schuur Hospital, Cape Town. 236 77

A thirty year-old man with angio-immunoblastic lymphadenopathy (AILD) is presented. This is the first case recorded from Ethiopia, and few have been reported elsewhere in Africa. The patient presented with generalized lymphadenopathy, fever, weight loss, pruritus, skin rashes, anaemia, hepatomegaly and pulmonary infiltrates. Lymph node histology was typical of AILD. The available literature on this relatively new pathologic entity reveals that little is known about the nature of AILD, and since there is no established therapy, an individualized approach to management is advisable.
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PMID:Angio-immunoblastic lymphadenopathy with dysproteinaemia in an Ethiopian: case report and literature review. 264 18

We conducted a phase II trial of deoxycoformycin (pentostatin [DCF]) in chronic lymphocytic leukemia (CLL). Eligibility criteria included age greater than 18 years, Cancer and Leukemia Group B (CALGB) performance status 0 to 2, lymphocyte count greater than or equal to 15,000 cells/microL, international stage B or C disease (multiple lymph nodes involved and/or hemoglobin [Hgb] less than 11 g and/or platelets less than 100,000/microL) and no more than one prior treatment regimen. DCF dose was 4 mg/m2 intravenously (IV) weekly for 3 weeks and then every 2 weeks. There were 39 eligible patients (35 men and four women; median age, 63 years; median time from diagnosis to study entry, 3 years). Of these 39 patients, 31% were stage B and 33% had no prior treatment. Median laboratory values at entry were Hgb 10.5 g, WBC 96,100/microL, and platelets 93,500/microL. Nodal involvement was present in 90%, splenomegaly in 81%, and hepatomegaly in 47%. Patients received a median of nine DCF injections, with a range of four to 26. Three patients were not evaluable for response. Overall, 3% achieved a complete response (CR), 23% a partial response (PR), 28% showed clinical improvement (CI), and 38% had stable disease (SD). Associated toxicities (grade 2 or worse) observed were infections (52%), worsening of thrombocytopenia (26%) or anemia (33%), nausea and vomiting (31%), rash or pruritus (20%), and stomatitis (8%). We conclude that DCF is an active agent in CLL with acceptable toxicity.
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PMID:Pentostatin in chronic lymphocytic leukemia: a phase II trial of Cancer and Leukemia group B. 278 91

Ten patients with primary biliary cirrhosis (PBC) were clinically followed under the conditions of follow-up care. The diagnosis of the patients was made vie biochemical immunologic, histological and instrumental investigations. The average term for follow up was 4 years and 5 months. All patients were actively checked up by summoning every 6 months. The average incidence of hospitalization at the clinic was 1 year and 5 months. Pruritus preceded all other symptoms--1 year and 11 months on the average. Jaundice was established 1 year and 7 months after the manifestation of pruritus and the dirty greyish pigmentation of the skin--1 year and 11 months after pruritus. Hepatomegaly was established 1 year and 7 months after pruritus. Increased alkaline phosphatase, blood bilirubin and cholesterol were observed in all patients and increase of IgM in 80 per cent, and antimitochondrial antibodies--in 70 per cent. Xanthelasma and xanthoma were found only in patients with high levels of cholesterol and total fats. The treatment with dihydrocortison induced stomach complaints (erosive gastritis), intersifying bone chanes in 20 per cent of the patients. X-ray and instrumental methods have a relative value in making the diagnosis of PBC. It could be concluded from the complex treatment that corticosteroids give complications and the treatment with cholestiramine--to attenuation of pruritus. The average survival after making the diagnosis is 6 years.
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PMID:[Clinical-dispensary finding of patients with primary biliary cirrhosis]. 293 78

We reviewed 82 cases of erythroderma, including clinical, laboratory, and biopsy material. The diseases most commonly associated with erythroderma were drug eruptions (34%), preexisting dermatoses (30%), and lymphoreticular neoplasms (20%), especially cutaneous T cell lymphomas (CTCL). The prognosis was related to the associated process; all deaths occurred in the drug or lymphoma groups. The clinical presentation of erythroderma was fairly uniform, with malaise (34%), pruritus (36%), and a subjective chilly sensation (34%) the most common symptoms. Keratoderma of the palms and soles (37%), mild generalized adenopathy (26%), and hepatomegaly (9%) were also common. Splenomegaly was rare (1%). Skin biopsy revealed the cause in 43% of the patients, but lymph node biopsy was not generally helpful in the initial evaluation. Although numerous laboratory values were abnormal, most findings were nondiagnostic and were related to the inflammatory process. A major exception was the Sezary cell preparation, which was selective for CTCL. A previously undiagnosed chronic erythroderma in a patient with no underlying skin disease may be the initial manifestation of CTCL. Thus, repeated evaluations and close follow-up are recommended.
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PMID:Erythroderma: review of 82 cases. 294 58

Although choledochal cyst is generally considered a disorder of childhood, in approximately 20% of patients a cyst is first recognized in adult life. We reviewed nine adults who underwent operation for choledochal cyst from 1974-1985 at Yale New Haven Hospital. Presenting symptoms included: crampy right upper quadrant pain, jaundice, pruritus, cholangitis, pancreatitis, hepatomegaly, and elevated liver function tests. Seven patients had undergone previous biliary surgery. The eight patients with type I cysts underwent complete excision of the cysts with Roux-Y choledochojejunostomy. One patient with a type IVa cyst underwent Roux-Y cystjejunostomy. All patients had complete resolution of biliary symptoms. There were no deaths, serious complications, or development of biliary malignancy in the 6 months to 13 years of follow-up. Optimum treatment of choledochal cyst is complete excision with reconstruction by Roux-Y choledochojejunostomy.
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PMID:Choledochal cysts in the adult. 298 Jul 68


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