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)

Sixteen cases of chronic Q fever are described. In eight there was a history of exposure to infection from farms or farm products. All had valvular heart disease, involving the mitral valve in nine and the aortic valve in seven. Infection occurred on a prosthetic valve in two patients. Arterial embolism was common. Venous thrombosis occured in three patients, and pulmonary embolism occurred in three other patients. Complement fixing antibodies to phase 1 antigen were found in a titre of 1:200 or greater in all except two patients. In one of these post-mortem examination revealed rickettsial bodies in mitral valve vegetations, and in the other Coxiella burneti was isolated from heart valve tissue. The majority presented with infective endocarditis but two presented primarily with liver disease. All patients had evidence of liver involvement and in one this led to death from cirrhosis. Abnormal tests of liver function, particularly hyperglobulinaemia, raised alkaline phsophatase and abnormal bromsulphthalein retention were found in all patients. Hepatic histology was abnormal in all eight patients in whom it was studied. The commonest features were mononuclear cell infiltration of the portal tracts and prominence of the sinusoidal Kupffer cells. Patchy focal necrosis of parenchymal cells, granulomata, fatty change, and eosinophilia of the sinusoidal walls were also noted in several patients and cirrhosis developed in one. Six patients had a purpuric rash, and in 12 there was thrombocytopenia. It is suggested that the presence of hepatomegaly and liver involvement and thrombocytopenia may help to differentiate Q fever endocarditis from bacterial endocarditis. Raised serum IgM and IgA levels occured frequently, but with only a moderate dominance of IgM. Sheep cell agglutination and latex fixation tests for rheumatoid factor were occasionally positive. Several features of the disease suggest the possibility that immune-complex mechanisms may play a role in chronic Q fever. Treatment was with prolonged courses of tetracycline usually combined with lincomycin. Seven patients underwent valve replacement surgery for haemodynamic reasons. Five patients died; two from heart failure, one from cirrhosis, one seven days after valve replacement and one from intraperitoneal haemorrhage following percutaneous liver biopsy. Three patients have survived for more than five years, and another six for more than three and a half years after diagnosis. Of these nine patients, three received medical therapy alone and six required valve replacement as well. Antibiotics have been discontinued in four patients who have had valve surgery and three others. Six patients had received antibiotics for continuous periods varying from 29-62 months. In the period after stopping therapy varying from 15-21 months, no relapse has occured. A seventh patient, who had received antibiotics for four months prior to valve replacement, has survived 43 months after the withdrawal of antibiotics...
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PMID:Chronic Q fever. 94 Sep 18

An unexplained increase in the frequency of pyogenic liver abscesses of unknown etiology has, fourtunately, been paralleled by significant advances in diagnostic and therapeutic methods. This report reviews experience with 14 patients operated upon at NYU Medical Center since 1971. Eight cases (57%) were cryptogenic. Other abscesses were associated with biliary disease (3); abdominal sepsis (2); and trauma (1). Abscesses were present on hospitalization in 12 patients. Clinical findings included fever (101-108 F); 100%; leucocytosis, 71%; anorexia and vomiting, 50%; localized tenderness and hepatomegaly, 50%; hypoalbuminemia, 86%; hypocholesterolemia, 78%; elevated SGOT, 71%; and elevated aikaline phosphatase, 43%. Technetium hepatic scintiscans showed focal defects in 10 of 12 patients (83%), but did not detect multiple abscesses in 2 of these. Hepatic arteriography performed in 10 patients was highly accurate, outlining single abscesses in 6 and multiple abscesses in 4. Furthermore, in one patient a false positive scintiscan was demonstrated by negative arteriography, confirmed by autopsy. In 4 patients, arteriography indicated an abscess in the posterior-superior area of the right hepatic lobe. With precise anatomical localization, a trans-thoracic approach permitted uncomplicated drainage in each case. This approach provides excellent exposure and direct drainage for abscesses in this area. An additional therapeutic adjunct in two patients, with 4 and 11 abscesses each, was postoperative intraportal infusion of antibiotics through the umbilical vein. Thirteen patients (83%) recovered, one dying from pulmonary embolism. Primary hepatic abscesses occur with increasing frequency. Primary hepatic abscesses occur with increasing frequency. Primary hepatic abscesses occur with increasing frequency. The methods described allow more precise preoperative diagnosis and direct surgical drainage.
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PMID:New diagnostic and therapeutic techniques in the management of pyogenic liver abscesses. 113 Aug 69

The Fontan operation and its modifications can be relatively safely performed in the treatment of many complex congenital heart diseases, such as univentricular heart and tricuspid atresia. The main postoperative complications following the Fontan procedure and the incremental risk factors involved in their development are investigated. Between January 1984 and January 1988 eleven patients (6 females and 5 males), ranging in age from 2 to 15 years (mean age: 7.3 +/- 3.7) and in weight from 10.8 to 50 Kg (mean weight 22.3 +/- 12.7) underwent the Fontan operation in our Department. No hospital death occurred. The mean postoperative stay in the Intensive Care Unit was 6.3 +/- 3.9 days. There were two surgical re-entries for the same patient: cardiac tamponade (the day of operation) and residual atrial septal defect (2 days following the operation). Eight patients had significant signs of venous stasis, with severe hepatomegaly and pleural effusion. The 11 patients discharged were followed-up for a period of between 3 and 48 months (mean follow-up: 26.4 +/- 18). There were 2 cases of recurrent pleural effusion, 10 to 15 days after discharge. One late death occurred 2 years after the Fontan procedure (massive pulmonary embolism after re-operation). Short and medium-term rate of complications is related to increased post-operative values of mean right atrial pressure (greater than 15 mmHg). Mean right atrial pressures greater than 15 mmHg appear to be correlated with Nakata index values less than 250 mm2/m2. The experience reviewed confirms the excellent results of the Fontan operation, as regards survival and functional recovery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Fontan's operation: results, immediate and long-term postoperative complications]. 227 18

In a 7-year period, transatrial membranotomy was performed in 11 patients with membranous obstruction of the inferior vena cava. There were 5 men and 6 women, ranging in age from 23 to 53 years. Clinical symptoms included jaundice in 4 patients, hepatomegaly in 4, leg edema or varicose veins in 10, and venous collaterals over the abdominal and chest wall in all 11 patients. Transatrial membranotomy was performed through a median sternotomy in all patients. When inferior vena cava venography revealed that the obstruction was accompanied by long segmental thrombosis, additional dilation was performed with a Hegar dilator. There was no surgical mortality. Early operative complications included pulmonary embolism in 2 patients and bleeding requiring reoperation in 1. In a mean follow-up period of 30.6 months (range, 2 to 88 months), 9 patients had no symptoms, transient pericardial constriction developed in 1 patient and resolved 1 month later, and restenosis of the inferior vena cava developed in another patient 1 year after the first operation. This latter patient received a second transatrial membranotomy followed by percutaneous balloon angioplasty of the inferior vena cava, with a satisfactory result at 8 months follow-up. We conclude that transatrial membranotomy is an effective and safe procedure for patients with membranous obstruction of the inferior vena cava.
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PMID:Transatrial membranotomy for Budd-Chiari syndrome. 240 Feb 84

We report the clinical features and outcome of 16 patients with cryoglobulinaemia. Two patients with Type I cryoglobulinaemia both had IgG kappa monoclonal paraproteins. Nine of 10 with Type II disease had monoclonal IgM kappa and polyclonal IgG; one had monoclonal IgG kappa and polyclonal IgG in the cryoglobulin. Underlying disorders identified in 3 of the 4 Type III patients were Sjogren's syndrome, infective endocarditis, and non-A non-B hepatitis and HTLV III infection. The commonest presenting features were rash in 94 p. 100 (ulceration 25 p. 100), arthralgia in 63 p. 100 (erosive arthritis 32 p. 100), renal disease in 63 p. 100, neurological involvement in 56 p. 100, hepatomegaly in 32 p. 100 and splenomegaly in 32 p. 100. Major associated conditions were progressive bronchiectasis in one case, and severe peripheral vascular disease in another; underlying malignancy was found in 2 cases (lymphoma and malignant melanoma). Treatment was with plasma exchange (PE) and immunosuppressive drugs (ID) in 10, PE alone in 3, ID alone in 2 and antibiotics [corrected] in 1. Fourteen of 16 patients showed an initial clinical response and fall in cryoglobulin levels. Four patients have died, one each from gastro-intestinal haemorrhage, sepsis, pulmonary embolism and lymphoma. Of the remaining 12 patients, all are symptomatically controlled and 10 have persisting cryoglobulinaemia (3 on PE and ID, 2 on PE, 2 on ID and 3 on no treatment). Of the two cases in whom cryoglobulinaemia resolved, one (Type II) had received PE and ID and the other (Type III) had been treated with antibiotics and surgery for infective endocarditis.
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PMID:Cryoglobulinaemia: clinical features and response to treatment. 376 96

Among 2175 patients seen over the last three years in a non-specialized department of internal medicine with no intensive care unit, 100 had supranormal serum lactic dehydrogenase activities. These patients' case-reports have been analyzed. Nearly half the patients (47/100) had a malignant disease (cancer or hemopathy). Among the remaining patients, 19 had a hepatic disorder (alcohol hepatitis in 10, viral hepatitis in 8, and isoniazide hepatitis in 1), 7 had a heart disease (heart failure with hepatomegaly in 5, myocardial infarction in 2), and 27 had various other conditions (including hemolysis in 6 and polymyositis en 3). The value of serum LDH assay is obvious in situations other than acute conditions such as myocardial infarction of pulmonary embolism; these are better known and have not been studied here as their prevalence was low among the patients enlisted in our study. In comparison to other enzymes (alkaline phosphatase (AP), gamma-glutamyl transpeptidase (GGT), transaminases (GOT, GPT) that were also routinely assayed in our patients, abnormal serum LDH activities are much less common and their significance is quite different. An increase in serum and their significance is quite different. An increase in serum LDH activity indicates a serious condition, often with a fatal outcome. The "various other conditions" group includes patients with hemolysis, hepatitis and myositis; the other patients in this group either had severe infectious diseases or died suddenly in the first few days of their hospitalization before diagnosis had been established. Each etiologic group has been analyzed to asses the characteristics of patients with increased LDH activity according to each etiology. Analysis of coincident abnormalities of the other enzymes listed above shows marked differences between etiologic groups; diagnostic accuracy can thus be enhanced in certain conditions. Most patients with malignancies had poorly differentiated tumors, with metastases: 28 had an epithelial tumor, with hepatic and/or bone metastases in 23 cases, 5 had cancer of the liver, 10 had a malignant hemopathy (2 lymphomas, 5 myeloproliferative syndromes, 3 acute leukemias), and 4 had a sarcoma. Cancer of the lung is the most common malignancy (10 cases) and may be responsible for increased serum LDH activity even in patients without metastases. Serum LDH assay is of value for monitoring the course in patients with initially increased activities as it falls under effective therapy and rises during exacerbations.
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PMID:[Value and diagnostic significance of serum lactic dehydrogenase in internal medicine (author's transl)]. 628 24

We describe 3 cases of acute graft-versus-host (GVH) disease in patients with acute myeloid leukaemia following transfusions taken from non-HLA-identical healthy donors. The leucocyte transfusions were given because of severe bone marrow aplasia and granulocytopenia following leukaemia induction treatment. The first patient had an acute GVH reaction with an erythrodermia-like skin reaction all over and associated with severe abdominal cramping, enlarged liver and pathological liver function tests. The second patient had a relatively mild skin reaction and enlarged liver. Both died of severe pulmonary infection. The third patient also had a mild skin reaction and enlarged liver. He died of pulmonary embolism. The diagnosis of GVH of the latter 2 cases was made on skin biopsy. The autopsy samples revealed in all cases a heavy lymphocytic infiltration of the kidneys and liver portal area. Until more precise guidelines can be established, irradiation of blood cell products given to patients with neutropenia due to leukaemia induction treatment should be considered.
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PMID:Graft-versus-host reaction in 3 adult leukaemia patients after transfusion of blood cell products. 658 Jul 18

Case report about death due to veno-occlusive liver disease following Dacarbazine treatment: 9 years after surgical treatment of malignant melanoma of the trunk a 68-years old patient developed lymph node metastases in the right axilla, which were removed immediately by surgical excision. One month before the patient had undergone surgical treatment of empyema of the gallbladder: Cholecystectomy and appendectomy were performed, postoperative recovery was uncomplicated. On account of the second lymph node metastasis within nine months adjuvant treatment with Dacarbazine was agreed and started one month later. After having performed the first course of treatment without any hints to intolerance the patient suddenly exhibited severe shock symptoms on the fourth day of the second course. Clinically residual myocardial infarct or pulmonary embolism were assumed, but could not be verified. The patient delivered increasing hepatomegaly. A massive increase in transaminase values was noted. Hemostasiologic changes with decreased Quick value occurred. The patient died of cerebral hemorrhage five days after beginning the second Dacarbazine cycle. Autopsy findings were severe liver cell necroses as well as liver vein thromboses, no metastases of melanoma could be found. Hepatotoxicity of Dacarbazine and the mechanism of liver vein thrombosis are discussed with special regards to possible hemostasiologic changes and sensibilisation due to Dacarbazine and/or previous liver cell damage.
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PMID:[Veno-occlusive syndrome with acute liver dystrophy following decarbazine therapy of malignant melanoma (author's transl)]. 721 35

A 31-year-old woman was admitted because of persistent remittent fever. Tricuspid valve endocarditis due to Staphylococcus aureus was identified as the cause of fever. The patient had no history of intravenous drug abuse, oral contraceptives or predisposing cardiac disease. Huge hepatomegaly was found without any signs of congestive heart failure. Liver enzyme abnormalities were not detected throughout the entire course of therapy. The liver biopsy specimen revealed peliosis hepatis. Treatment with panipenem/betamipron was successful, although recurrent septic pulmonary embolism occurred. The cause of the huge hepatomegaly encountered in the present case may be attributable to peliosis due to severe infection.
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PMID:Tricuspid valve endocarditis in a non-drug addict associated with peliosis hepatis. 939 42

Azathioprine is a drug commonly used for the treatment of inflammatory bowel disease, organ transplantation and various autoimmune diseases. Hepatotoxicity is a rare, but important complication of this drug. The cases reported to date can be grouped into three syndromes: hypersensitivity; idiosyncratic cholestatic reaction; and presumed endothelial cell injury with resultant raised portal pressures, venoocclusive disease or peliosis hepatis. The components of azathioprine, 6-mercaptopurine and the imidazole group, may play different roles in the pathogenesis of hepatotoxicity. The strong association with male sex, and perhaps with human leukocyte antigen type, suggests a genetic predisposition of unknown type. Many of the symptoms of hepatotoxicity, such as nausea, abdominal pain and diarrhea, can be nonspecific and can be confused with a flare-up of inflammatory bowel disease. As well, the subtype resulting in portal hypertension can occur without biochemical abnormalities. A 63-year-old man with Crohn's disease who is presented developed the rare idiosyncratic form of azathioprine hepatotoxicity, but also had a severe disabling steroid myopathy, peripheral neuropathy, resultant deep venous thrombosis and pulmonary embolism related to immobility, and a nosocomial pneumonia. His jaundice and liver enzyme levels improved markedly on withdrawal of the drug, returning to almost normal in five weeks. Treating inflammatory bowel disease effectively while trying to limit iatrogenic disease is a continuous struggle. Understanding the risks of treatment is the first important step. There must be a low threshold for obtaining liver function tests, especially in men, and alertness to the need to discontinue the drug or perform a liver biopsy should patients on azathioprine develop liver biochemical abnormalities, unexplained hepatomegaly or signs of portal hypertension.
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PMID:Cholestatic hepatocellular injury with azathioprine: a case report and review of the mechanisms of hepatotoxicity. 981 67


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