Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of the 169 human immunodeficiency virus (HIV)-infected children being cared for at Siriraj Hospital from January 1998 to September 2000, 10 had Mycobacterium avium complex (MAC) infection; seven had disseminated disease and three had MAC pneumonia. Nine children were in the advanced stage of HIV disease at the time of diagnosis with the median CD4 count of 7 cells/mm3 and 127 cells/mm3 and the median age of 65 months and 63 months in disseminated MAC and MAC pneumonia respectively. None of these children had received prior chemoprophylaxis. Common clinical findings included prolonged fever, weight loss, lymphadenopathy, hepatosplenomegaly, diarrhea, anemia and leukopenia. The outcome of MAC infection was poor, with a mortality rate of 60 per cent. In in vitro susceptibility testing, clarithromycin was the least resistant drug. With the incidence rate of 2.15 per 100 person-years, the high rate of antimicrobial resistance, and the poor outcome, primary chemoprophylaxis for MAC infection in conjunction with effective antiretroviral therapy should be considered for Thai children in the advanced stage of HIV infection.
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PMID:Mycobacterium avium complex in HIV-infected Thai children. 1240 47

Visceral leishmaniasis is a worldwide, disseminated intracellular protozoal infection that usually manifests by fever, hepatosplenomegaly, anemia, thrombocytopenia, leukopenia and hypergammaglobulinemia. Although anemia is a usual finding, Coombs' positive hemolytic anemia has rarely been reported in association with this disease. Pentavalent antimonials have been the preferred treatment for this disease for decades, but increasing numbers of treatment failure with antimony are being reported. Liposomal amphotericin B is a new drug which is highly efficacious in the treatment of visceral leishmaniasis and produces minimal toxicity. Here we report an infant with visceral leishmaniasis associated with Coombs' positive hemolytic anemia who was successfully treated with liposomal amphotericin B.
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PMID:Visceral leishmaniasis and Coombs' positive hemolytic anemia: a rare association in an infant treated with liposomal amphotericin B. 1469 14

Dyskeratosis congenita (DC) is a rare inherited disorder characterized by reticulate skin pigmentation, nail dystrophy, mucosal leucoplakia, and bone marrow failure. Pancytopenia is difficult to manage in patients with this disorder. We describe a 13-month-old-boy who presented with reticulate skin lesions, paleness, and hepatosplenomegaly. Anemia and leukopenia developed by the age of 43 months. The patient was treated with granulocyte-macrophage colony-stimulating factor (GM-CSF) (5 microg/kg/d, subcutaneously) for 19 months and erythropoietin (150 U/kg 3 days in a week, subcutaneously) for 8 months, with excellent neutrophil and hemoglobin response. Recurrent infections were not developed after starting GM-CSF, and packed red blood cell transfusion was not given to the patient after starting erythropoietin. GM-CSF combined with erythropoietin may be used in the treatment of bone marrow failure in patients with DC without an HLA-identical donor.
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PMID:Treatment of dyskeratosis congenita with granulocyte-macrophage colony-stimulating factor and erythropoietin. 1267 52

We report a case of a 22 years old type 1 diabetic man with a history of weight loss, weakness, anorexia, fever and recurrent urinary tract infection since February 2001. In April 2001, he presented anuria due to obstructive acute renal failure. Hepatosplenomegaly and lymphadenopathy were absent at physical examination. Laboratory tests revealed a high level of gamma globulin (53.4 g/l) and anaemia (haemoglobin 7.7 g/100 ml) without leukopenia and thrombocytopenia. CT scan showed multiple retroperitoneal lymphadenopathies causing compression of the two ureters, hydro-ureter associated with hydronephrosis, hepatosplenomegaly and multiple pulmonary nodes. Lymphadenopathies, anaemia, high level of gamma globulin, high titres of anti-leishmanial antibodies and the excellent outcome after treatment with meglumine antimoniate (Glucantime) confirmed visceral leishmaniasis. This report documented an unusual clinical presentation of Visceral leishmaniasis in a diabetic patient.
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PMID:[Obstructive acute renal failure revealing visceral leishmaniasis in a diabetic patient]. 1272 15

Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increased serum LDH. Moreover, a chest CT evidenced bilateral multiple pulmonary infiltrates and pleural effusion. After the development of proteinuria, a diagnosis of large B-cell intravascular lymphoma was made with a renal biopsy 10 months after the onset of the clinical manifestations. So far, more than 100 cases of IL have been reported and the diagnosis often turned out to be difficult, as clinical signs did not point to a lymphoproliferative disorder. This report confirms that FUO is not only frequently associated with IL but that it even marks the real onset of the disease. We are then tempted to conclude that undiagnosed fever is not so rare in IL and if we call it FUO, it is only because diagnosis is necessarily elusive and hence time-consuming.
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PMID:Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks. 1458 55

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.
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PMID:Hemophagocytic syndrome associated with bilateral adrenal gland tuberculosis. 1505 49

Anorexia nervosa is a chronic psychiatric process characterized by a restrictive disorder in alimentary habits. Hematologic alterations in the peripheral blood include cytopenias involving one or more hematopoietic lineages. Morphologic changes in the bone marrow and stereologic alterations in bone marrow adiopocytes may also be observed in anorexia nervosa. We present a 12-year-old girl who had chronic anorexia and one third of body weight loss during an 8-month period. She was apathetic and had missed several menstrual cycles. The sex maturity rating was Tanner stage IV. There was no lymphadenopathy, no hepatosplenomegaly, and no identifiable tumor mass. She was not anemic, but was found to have leukopenia, neutropenia and a low level of triiodothyronine. Sections of the bone marrow biopsy showed almost complete serous atrophy (gelatinous degeneration) of the bone marrow. In this patient, the bone marrow alteration is related to nutritional deprivation of anorexia nervosa.
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PMID:Gelatinous degeneration of the bone marrow in anorexia nervosa. 1579 62

Amiodarone therapy is associated with several adverse effects, including hematologic ones such as pancytopenia, hemolytic anemia, and aplastic anemia. Very few cases of amiodarone-associated bone marrow granulomas have been reported. We report 2 cases of amiodarone-associated bone marrow granulomas. Patient 1 was an 81-year-old man who presented with leukopenia, thrombocytopenia, and hepatosplenomegaly after 2 years of amiodarone therapy. Patient 2 was an 80-year-old man who presented with pancytopenia 2 1/2 years after starting amiodarone treatment. Both patients had normal blood counts before amiodarone therapy. Bone marrow biopsies showed noncaseating granulomas in both patients. We reviewed the literature available on Medline for amiodarone-associated bone marrow granulomas and found 8 reported cases of amiodarone-associated bone marrow granulomas. One case also featured amiodarone-associated hepatic granulomas. Amiodarone therapy was stopped in 5 cases, with improvement of the granulomas occurring in 3 cases. We conclude that bone marrow granulomas, although rare, should be considered as a differential diagnosis for patients undergoing amiodarone therapy and presenting with cytopenias.
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PMID:Amiodarone-associated bone marrow granulomas: a report of 2 cases and review of the literature. 1732 85

Visceral leishmaniasis is endemic in southern Saudi Arabia. We prospectively evaluated 121 patients with visceral leishmaniasis at King Fahad Hospital in Gizan. All patients were infants and children. Seasonal variation was observed with more cases presenting in late spring and summer and few in winter. The predominant clinical features in these patients were chronic fever, abdominal distention, weight loss and hepatosplenomegaly. Lymphadenopathy was rare in contrast to African kala-azar. Common laboratory abnormalities included anemia, leukopenia, thrombocytopenia, hypoalbuminemia and hypergammaglobulinemia. Liver function tests were deranged in one-third of patients. Leishmania hemagglutination test was positive in all patients and all of them had positive bone marrow smear or culture for Leishmania donovani. Patients responded well to stibogluconate (Pentostam) therapy with a cure rate of 96.7%. Four patients died in the first few days of therapy. Jaundice and grossly deranged liver function tests were found to be bad prognostic signs.
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PMID:Visceral leishmaniasis in Gizan, Saudi Arabia. 1758 53

Kala-azar (visceral leishmaniasis) is endemic in southern Iran. We retrospectively evaluated 367 infants and children with visceral leishmaniasis at hospitals affiliated to Shiraz University of Medical Sciences in Fars Province (located in the southwestern part of Iran). Seasonal variation was observed with more cases presenting in late winter, spring and a few in summer. The predominant clinical features in these patients were chronic fever, pallor, weight loss, abdominal distention, and hepatosplenomegaly. Lymphadenopathy was less common. Common laboratory abnormalities included anemia, leukopenia, thrombocytopenia, hypoalbuminemia and hypergammaglobulinemia. Liver function tests were deranged in two-thirds of patients. Immuno-fluorescence antibody (IFA) test was positive in all patients and all had positive bone marrow smears or cultures for Leishmania donovani. Patients responded well to Glucantim therapy with a cure rate of 96.7%. Relapse was observed in 8.2%(30) of patients. Mortality in this series was 7.3%. Twenty-three patients died during therapy. Jaundice and grossly deranged liver function tests were bad prognostic signs.
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PMID:Epidemiological, clinical and therapeutic features of pediatric kala-azar. 1788 98


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