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)

Twenty-seven patients suffering AIDS and disseminated histoplasmosis were included in this study, comprising twenty-three males and four females, from 18 to 46 years of age (mean = 32.9). The most frequent clinical manifestations were fever, weight loss, anaemia, skin lesions, pulmonary micronodules, hepatosplenomegaly and adenomegalies. All of them presented other infectious diseases or neoplasias frequently found in AIDS patients. The diagnosis of histoplasmosis was based upon the finding of Histoplasma capsulatum in microscopic examination or in cultures from the following specimens: skin scrapings, bone marrow aspiration, bronchoalveolar lavage, blood cultures, buccal biopsies and lymph node biopsy. Serologic reactions, searching for antibodies, were positive in 11 cases. Itraconazole by oral route, at a daily dose of 200 mg (24 cases) or 400 mg (3 cases), was administered for 6 months. Those patients who were clinically cured after receiving this scheme of treatment were treated with itraconazole 100 mg day-1 as a suppressive therapy. Twenty-three patients were considered responders, 1 as a non-responder and 3 non-assessable. The average survival time was 7.8 months and eleven cases are still alive. Itraconazole proved to be a useful medication in disseminated histoplasmosis associated with AIDS and it was very well tolerated.
Mycoses
PMID:Itraconazole in the treatment of histoplasmosis associated with AIDS. 133 77

A 2-month-old male Tongan infant presented with fever, severe skin and mucosal infections, hepatosplenomegaly, thrombocytopenia, and normal neutrophil counts. While polymorphonuclear neutrophil (PMN) morphology was normal, several neutrophil motile functions were found to be altered in the patient. Furthermore, two siblings had died in infancy with a similar clinical picture, raising the possibility of an inherited neutrophil defect. Random migration and chemotaxis, assessed by the under agarose method, were profoundly impaired. Actin polymerization, as measured by flow cytometry of N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)phallacidin (NBD-phallacidin)-stained PMNs, showed lower basal F-actin and a 1.75-fold increase in response to 10(-7) mol/L formyl-methionyl-leucyl-phenylalanine (FMLP) compared with a 4.51-fold increase in control. Microscopic examination of NBD-phallacidin-stained PMN spread on glass showed decreased area of spreading and F-actin-rich filamentous projections distinct from control. The early phase of FMLP-induced right angle light scattering was absent, similar to the effect caused by cytochalasin-B (CB), an inhibitor of actin polymerization. Accordingly, FMLP induced secretion of elastase without the addition of CB. Staphylococcus aureus killing was 50% of control whereas superoxide production response to FMLP and surface expression of CD11b were greater than twice normal. Partial defects in actin polymerization and scatter were seen in the parents and release of elastase, in the absence of CB, was also increased in both parents. Sodium dodecyl sulfate-polyacrylamide electrophoresis of whole cell proteins from the patient showed a marked decrease in an 89-Kd protein (8% of control) and a marked increase in a 47-Kd protein (4.2-fold). Both mother and father had decreased 89-Kd (77% and 42% of control) and increased 47-Kd proteins (2- and 3.4-fold), although neither had recurrent infections or chemotactic defects. These studies describe a new inherited actin dysfunction syndrome associated with severe propensity to fungal infection and draw attention to the proteins of apparent molecular weights of 89 Kd and 47 Kd, which may be of great importance in the regulation of actin polymerization in human PMNs.
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PMID:An inherited defect of neutrophil motility and microfilamentous cytoskeleton associated with abnormalities in 47-Kd and 89-Kd proteins. 187 95

90 patients diagnosed as eosinophilia and pulmonary infiltrates with peripheral eosinophilia in PUMC Hospital from 1957 to 1986 were reported. The cause of the eosinophilia was unknown in 42% of the patients. Among these patients, hypereosinophilic syndromes (HES) were suspected in 11 patients. The most common presenting symptoms of HES were fever, skin lesions gastrointestinal symptoms and hepatosplenomegaly, the mean peripheral blood eosinophil count was as high as 25.6 x 10(9) cells/L. Biopsies of bone marrow and involved organs revealed extensive eosinophilic infiltration and severe necrosis. In the past ten years, eosinophilia and pulmonary infiltrates caused by parasitic infections obviously decreased. The most common parasites were ascaris Lumbricoides, Ancylostoma duodenale and Clonorchis sinesis. The other main causes which induced pulmonary infiltrates were allergic bronchopulmonary mycosis (ABPM) and chronic eosinophilic pneumonia. The prevalence of ABPM increased recently; early diagnosis and effective therapy could prevent the irreversible damage resulting in airways obstruction and fibrosis.
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PMID:[Clinical analysis of 90 cases of eosinophilia and pulmonary infiltrates with peripheral eosinophilia]. 208 6

Three children with acute lymphoblastic leukemia developed disseminated fungal disease predominantly involving the liver and spleen. The three patients were undergoing induction chemotherapy and had neutropenia when they presented prolonged fever not responsive to antibiotics. Once neutropenia was recovered, hepatosplenomegaly leukocytosis, elevated serum alkaline phosphatase, and hypoechoic areas in the spleen and liver ultrasound were observed. All fungal blood cultures were negative, with the diagnosis being confirmed by histologic study. One of the patients died without achieving control of the candidiasis. The other two patients received prolonged antifungal treatment concurrently with chemotherapy and both are alive, one of them cured and in complete remission. The increasing frequency of this infection in recent years and the importance of a prompt and prolonged administration of antifungal therapy to obtain the cure are discussed.
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PMID:Hepatosplenic candidiasis in children with acute leukemia. 229 57

We present two cases of paracoccidioidomycosis, one occurring in an AIDS patient and the other in an HIV infected man. This is the first report of such association. The first patient, which was already followed for HIV infection (group IV-A) presented with high fever and hepatosplenomegaly. Plain X-ray, ultrasound and CT-scan of the abdomen showed solid nodules in the spleen, some of them with calcification. Both the direct smear and the culture of a bone marrow aspiration revealed Paracoccidioides brasiliensis. The patient died of acute disseminated Paracoccidioidomycosis. The second patient, a man anti-HIV seropositive presented with a mass on the right lower abdomen and inguinal region. A biopsy of the mass showed the association of Hodgkin's disease of the mixed cellularity type and paracoccidioidomycosis. With the expanding AIDS epidemic we believe this report emphasizes the need to consider Paracoccidioidomycosis in HIV infected persons in countries where this mycosis is endemic. We also suggest the inclusion of Paracoccidioidomycosis as a potential opportunistic infection in these areas.
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PMID:[Paracoccidioidomycosis and infection by the human immunodeficiency virus]. 269 Mar 7

The radiological findings in two cases of hepatic fungal microabscesses (one with concomitant splenic involvement) occurring in children with acute leukaemia are presented, and the previous literature is reviewed. Usually both liver and spleen are involved, though either organ may be affected in isolation. Typical ultrasound features include hepatosplenomegaly with multiple hypoechoic areas throughout the liver and spleen, often with a 'target' configuration. Computed tomography shows these as multiple non-enhancing low attenuation lesions. These findings in a patient with acute leukaemia are strongly suspicious of fungal infection, and percutaneous fine-needle aspiration under ultrasound or computed tomography-guidance is indicated.
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PMID:Fungal liver abscesses in acute leukaemia--a report of two cases. 328 83

Disseminated fungal disease, predominantly involving liver and spleen, developed in eight patients with hematologic malignancies. Because the patients failed to respond to standard antifungal drugs, they were treated with liposomal amphotericin B (L AmpB). Before therapy began, the diagnosis was confirmed histologically and the patients underwent abdominal computed tomography (CT), which indicated hepatosplenomegaly with or without multiple microabscesses in the liver and spleen. After each course of treatment with L AmpB, patients underwent CT, followed by either open or CT-guided percutaneous aspiration biopsy of the liver. Post-treatment CT showed partial regression of lesions in six patients and persistence in two. In all patients a liver biopsy confirmed that the lesions noted after treatment were due to granulomas or focal areas of fibrosis compatible with healing. Thus, the persistence of multiple defects on enhanced scans in two patients was not an indication of persistent abscesses. Clinical response was an additional important factor. Close clinical and pathologic correlation in addition to CT scanning are required in the follow-up of hepatosplenic fungal infections.
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PMID:Hepatosplenic fungal infection: CT and pathologic evaluation after treatment with liposomal amphotericin B. 396 Nov 67

This report describes clinical and immunologic features of five illustrative cases of paracoccidioidomycosis in previously healthy children. All had disseminated disease and two of them died despite treatment. The major clinical presentation in four patients was fever and diffuse superficial and intraabdominal adenopathy, with or without hepatosplenomegaly. Other sites were also affected: three patients had multiple osteoarticular lesions, occasionally with intense tissue destruction; two had cutaneous eruptions; two had pericardial effusions; and two had pulmonary involvement, once considered an organ spared in the young. We detected variable lymphocyte responses to mitogens and to Candida albicans antigen and non-responsiveness to Paracoccidioides brasiliensis cell wall antigen. High concentrations of serum immunoglobulins and anti-P. brasiliensis antibodies were present. These immune alterations tended to resolve with treatment, suggesting a reversible nature of the immune defect. We conclude that this mycosis has a high morbidity and mortality in children, which is probably related to an antigen-specific immunodeficiency. Further studies are needed to increase knowledge of this mycosis in children.
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PMID:Severe acute paracoccidioidomycosis in children. 807 39

The case is described of a 42-year-old patient with acute myeloid leukemia who received two courses of chemotherapy complicated by prolonged bone marrow depression. He was admitted to hospital with fever, hepatosplenomegaly and bilateral nodular pulmonary infiltrates. After admission diffuse cutaneous skin nodules, and hypodense lesions in the hemispheres and cerebellum developed. Cultures of cerebrospinal fluid, bronchoalveolar lavage fluid, skin biopsy specimens and blood revealed Scedosporium prolificans, indicative of disseminated mycosis. Treatment with amphotericin B and fluconazole was unsuccessful and the patient died within five days after admission. Features that may enhance early recognition of Scedosporium prolificans infection by both clinicians and microbiologists, as well as options in the treatment of infection with this fungal agent are discussed.
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PMID:Fatal Scedosporium prolificans infection in a leukemic patient. 924 51

Penicillium marneffei is rarely pathogenic in humans. Most previously reported cases of P. marneffei infection were from Southeast Asia where patients were usually in an immunocompromised state due to human immunodeficiency virus (HIV) infection. The majority of the patients reported in Western countries were immunocompromised by malignancy, especially Hodgkin's lymphoma. In Taiwan, the first case of P. marneffei infection was reported in 1994 and involved an adult with HIV infection. We report a case of systemic P. marneffei infection in a child with common variable immunodeficiency (CVID). The patient, a 4-year, 5-month-old boy, had a 1-year history of oligoarthritis resembling juvenile rheumatoid arthritis (JRA). He developed a low grade fever (38 degrees C) and hepatosplenomegaly 1 month before admission to the hospital. Although cultures of synovial fluid obtained at the time of onset of oligoarthritis did not grow any organisms, cultures of blood, bone marrow, synovial fluid, and lymph node biopsy samples taken during this admission were positive for P. marneffei. Further immunologic studies revealed a profile characteristic of CVID. The fungal infection was finally eradicated by combined therapy with amphotericin B, fluconazole, itraconazole, and regular immunoglobulin replacement. This case reminds us that JRA or JRA-like arthritis should be differentiated from septic arthritis caused by rare pathogens in immunocompromised patients.
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PMID:Systemic Penicillium marneffei infection in a child with common variable immunodeficiency. 987 36


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