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)

Two patients with Q fever endocarditis are described. Both patients demonstrated some of the characteristic features of Q fever endocarditis, i.e. the long course of the disease before diagnosis, persistently negative blood cultures, resistance to conventional antibiotic therapy and a dramatic response to tetracycline therapy. Complications included arteriovenous thrombo-embolism and hepatic enlargement, and 1 patient developed an immune complex form of glomerulonephritis. The possibility of Q fever endocarditis should be considered in all patients with infective endocarditis in whom blood cultures are negative and who fail to respond to conventional antibiotic therapy.
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PMID:Q fever endocarditis: a report of 2 cases. 65 35

33 children (22 girls) with brucellosis seen between 1972-1988 were studied retrospectively. All but 1 were Bedouins. The mean age at diagnosis was 9.8 years (range: 17 months-17 years). Duration of illness prior to diagnosis was less than 1 week in 13 (39%), 1-4 weeks in 8 (24%) and 1-3 months in 10 (30%). In 2 cases the symptoms lasted 6 and 8 months, respectively, before diagnosis. Presenting symptoms included fever (85%), articular involvement (65%), hepatomegaly (45%) and splenomegaly (33%). Less common manifestations were anorexia (30%) and weight loss (15%) cases. Meningoencephalitis developed in 2 patients and uveitis and glomerulonephritis in 1 each. Diagnosis was based on positive agglutination titers (greater than 160), which were found in all. Brucella melitensis was isolated in blood cultures in 8 of the 33. 18 were treated with tetracycline and 9 with tetracycline and streptomycin, all of whom responded well. 3 of the 6 treated with trimethoprimsulphamethoxazole were only cured when therapy was changed to tetracycline in 2 and tetracycline plus streptomycin in 1. All patients recovered without sequelae. We conclude that brucellosis due to Brucella melitensis is endemic among the Bedouin of the Negev. An increased incidence of brucellosis among hospitalized children has been noted in the past 2 years, indicating the need for diagnostic awareness.
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PMID:[Childhood brucellosis in the Negev]. 228 18

An allergic disease may develop in any organ or system. The respective etiological factors include foreign proteins, infectious agents such as various microbes, viruses, moulds, parasites, chemical compounds often in the form of drugs usually designated as haptens, polysaccharides, benign and malignant neoplasms. Of the factors operating in the causal pathogenesis of such diseases the most important one is an exaggerated formation of antibodies, which appears to be uncontrolled and occurring irrespective of the demands of the organism. The essential morphological features in allergic inflammation are rather variegated, their diagnostic value differing in a wide range but being never absolute. The above features include eosinophilic leucocytes, allergic arteritis and phlebitis, fibrinoid necrotic glomerulonephritis, histiocytic granulomatous inflammation or histiocytic granuloma. Granulomatous capsulitis and trabeculitis affecting the spleen and lymph nodes are believed to be of major diagnostic significance. The immunofluorescent and immunoperoxidase methods and electron microscopy are important diagnostic tools. It has been generally acknowledged that many drugs operate as antigens. They may cause death of the respective patient, but allergic manifestations may subside after withdrawal of such drugs. On occasion they operate as a trigger mechanism with the allergy progressing even after treatment had been interrupted. Therefore they have been receiving extreme attention. Our collection of cases a case of giant-cell myocarditis following sulfamethoxypyridazine, anaphylactic shock has been reported to occur after intravenous administration of novocaine, and generalized cutaneous vasculitis developed in the same patient during the subsequent phase. A similar cutaneous finding was reported to have developed after penicillin injection, granulomatous inflammation developed owing to sulfonamide treatment. Allergic tumour-like lymphadenitis developed after administration of anti-anthracic serum; an anticonvulsive syndrome developed after hydantoinate administration. The latter consisted of generalized exanthema, hepatomegaly, splenomegaly and generalized lymphadenopathy. The lymph nodes showed tumour-like lymphadenitis mimicking lymphogranuloma or reticulosis. Allergic diseases appear as either isolated organ lesions or systemic diseases. Thus, isolated and systemic polyarteritis nodosa, isolated nasal, pulmonary or systemic Wegener's granulomatosis have been recognized. Temporal arteritis has been recognized as a localized form of systemic giant-cell arteritis. The haemolytic-uraemic syndrome appears to be a milder variety of thrombotic thrombocytopenic purpura. Allergic diseases or manifestations occasionally affect two or more organs or systems.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Pathology of allergic diseases. 248 27

In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.
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PMID:Liver disease associated with anti-liver-kidney microsome antibody in children. 395 Aug 19

At autopsy, massive nonamyloid fibrillar deposits, immunoreactive to IgG and kappa light chain, were found in glomeruli, liver, and bone marrow of a 72-year-old woman. The patient suffered from severe nephrotic syndrome, hepatomegaly and cholestasis, normochromic anemia, and IgG kappa monoclonal gammopathy. Fibrillary glomerulopathies, most often denoted as fibrillary glomerulonephritis or immunotactoid glomerulopathy, are generally considered to have deposits restricted to the glomeruli. However, this study indicates that fibrillary deposits may be a systemic manifestation of fibrillary glomerulonephritis or immunotactoid glomerulopathy, at least when the patient is suffering from a monoclonal gammopathy.
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PMID:Immunotactoid-like glomerulopathy with massive fibrillary deposits in liver and bone marrow in monoclonal gammopathy. 895 65

Four cases of typhoid fever complicated by both acute oliguric renal failure and hepatitis are presented. Two patients had type II hepatitis according to criteria proposed by Khosla et al. (30) with hepatomegaly, hyperbilirubinaemia and markedly elevated asparate transaminase (AST); the others had type III hepatitis, characterized clinically and biochemically by profound jaundice, hepatomegaly, hepatic encephalopathy (one case only), hyperbilirubinemia and markedly elevated serum AST. Renal biopsy was not performed in any of our patients. However, a combination of proteinuria and abnormal urinary sediments containing red cell casts and granular casts, as noted in these patients, is considered highly suggestive of glomerulonephritis. Although isolated renal failure and hepatitis with hepatomegaly and deranged liver enzyme values have been reported previously in typhoid fever, their occurrence simultaneously in the same patient in distinctly rare, having been reported only twice in the English language literature.
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PMID:Typhoid fever complicated by acute renal failure and hepatitis: case reports and review. 964 38

The Severe Chronic Neutropenia International Registry (SCNIR) was established in 1994 following four phase I/II and one phase III clinical trial on the use of filgrastim (recombinant human granulocyte colony-stimulating factor [r-metHuG-CSF]) as a treatment for severe chronic neutropenia (SCN). A primary purpose of the SCNIR is to monitor SCN patients treated with filgrastim for adverse events that might occur over time. As of December 31, 2000, 832 patients with SCN (384 congenital, 160 cyclic, 288 idiopathic) were enrolled. Clinical trial and Registry data show that filgrastim is an effective treatment for SCN; more than 90% of patients treated respond with normalization of blood neutrophil counts. The SCNIR has collected data on bone pain, splenomegaly, hepatomegaly, thrombocytopenia, osteopenia/osteoporosis, vasculitis, glomerulonephritis, growth and development, pregnancy and fertility, leukemic transformation, and mortality. Analysis of data from patients who received filgrastim for up to 11 years did not identify any adverse events associated with increased duration of treatment.
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PMID:Risk and benefit of treatment of severe chronic neutropenia with granulocyte colony-stimulating factor. 1195 97

History and presenting signs and symptoms in 26 Hungarian Gaucher patients are described in order to promote recognition of non-diagnosed patients. Macrophages accumulating glucocerebrosidase due to deficient glucocerebrosidase activity may be present in many organs leading to a wide variety of clinical manifestations. The most common presenting signs and symptoms are splenomegaly, hepatomegaly, hypersplenism with anemia and thrombocytopenia, and bone deterioration. Rare manifestations of Gaucher disease including glomerulonephritis, pericarditis, pericardial calcification, haemorrhagic colitis and amyloidosis are also discussed.
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PMID:[Presenting signs and symptoms in Gaucher disease]. 1243 34

Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor the clinical course, treatments, and disease outcomes for SCN patients. This report summarizes data for 853 patients, almost all treated with daily or alternate-day recombinant human granulocyte colony-stimulating factor (G-CSF or Filgrastim). G-CSF treatment increased the ANC overall from 0.34 x 10(9)/L +/- 0.018 pre-treatment to 3.70 x 10(9)/L +/- 0.18 during the first year of treatment. For most patients, the responses were durable with patients remaining on the same dose of G-CSF for many years. Long-term hematological observations showed stable mean leukocyte and neutrophil counts and gradually increasing hemoglobin levels. Thrombocytopenia developed in 4% of patients. As of January 1, 2000, myelodysplasia (MDS) or acute myelogenous leukemia (AML) has occurred in 35 of 387 patients with congenital neutropenia with a cumulative risk of 13% after 8 years of G-CSF treatment. This event occurred without a predictable relationship to the duration or dose of G-CSF treatment. No patients with cyclic or idiopathic neutropenia developed MDS or AML. Other important adverse events included hepatomegaly, osteoporosis, vasculitis, glomerulonephritis, and deaths in 4 of 14 cases requiring splenectomy. Growth and development and the outcome of pregnancy appeared to be unaffected by G-CSF treatment. These data indicate that congenital, cyclic, and idiopathic neutropenia can be effectively treated with long-term G-CSF. The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long-term observations.
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PMID:Severe chronic neutropenia: treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry. 1255 10

Generalized lipodystrophy is characterized by adipose tissue absence, hypoleptinemia, hypertriglyceridemia, insulin resistance, diabetes, hepatomegaly, and nonalcoholic steatohepatitis. In the course of recruiting patients for treatment with recombinant leptin, we were struck by the frequency and severity of proteinuria. We evaluated 25 patients with generalized lipodystrophy. Eighteen were treated with recombinant leptin, and we have followed 15 on leptin for 4-36 months. We followed renal parameters at baseline and during follow-up visits. Renal biopsies were performed as clinically indicated. At baseline, 22 of 25 patients (88%) had elevated urine albumin excretion (>30 mg/24 h), 15 (60%) had macroalbuminuria (>300 mg/24 h), and five (20%) had nephrotic-range proteinuria (>3500 mg/24 h). Twenty-three (92%) had elevated creatinine clearance (>125 ml/min.1.73 m(2)). Eleven of 15 patients (73%) treated with recombinant leptin exhibited reduction in proteinuria, associated with reduction of hyperfiltration. Four patients who did not improve are discussed individually. Renal biopsy findings were remarkable for focal segmental glomerulosclerosis in four patients, membranoproliferative glomerulonephritis in two patients, and diabetic nephropathy in one patient. In conclusion, generalized lipodystrophy is associated with proteinuria and unique renal pathologies, including focal segmental glomerulosclerosis and membranoproliferative glomerulonephritis. The majority treated with recombinant leptin demonstrated reduction in proteinuria and hyperfiltration.
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PMID:Proteinuric nephropathy in acquired and congenital generalized lipodystrophy: baseline characteristics and course during recombinant leptin therapy. 1524 May 93


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