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)

The clinical pattern of 400 cases of brucellosis in Kuwait is presented. The disease was acute in 77 per cent, sub-acute in 12.5 per cent and chronic in 10.5 per cent of cases. Raw milk was the major source of infection. The major features on presentation, irrespective of the course of the disease, were fever, sweating, headache, rigors, arthralgia, myalgia, and low back pain. Hepatosplenomegaly was present in 41 per cent of cases and in 32 per cent neither liver nor spleen were palpable. The haematologic findings were not specific and hepatic dysfunction (shown by liver enzyme abnormalities) was common. Skeletal (26 per cent) and genital (8.5 per cent) changes and neurobrucellosis (7 per cent) were the major complications. The ELISA was the most sensitive and reliable diagnostic test especially in relation to chronic brucellosis and neurobrucellosis. ELISA allowed the determination of brucella-specific immunoglobulins (Ig)G, IgM and IgA in the CSF, and provided profiles of Ig, in sera, which were different in patients with chronic (elevated IgG and IgA) from those with acute (elevated IgM alone or IgG, IgM and IgA) brucellosis. Treatment with tetracycline, doxycycline or rifampicin gave a cure rate of over 91 per cent in acute and subacute brucellosis. Co-trimoxazole was associated with a relapse rate of 50 per cent. Two drug combinations of streptomycin and tetracycline, streptomycin and rifampicin or streptomycin and doxycycline were effective, but one of five patients with chronic brucellosis relapsed. A combination of streptomycin, tetracycline and rifampicin with or without steroids was used successfully in neurobrucellosis, septicaemic shock and subacute bacterial endocarditis.
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PMID:Human brucellosis in Kuwait: a prospective study of 400 cases. 305 Oct 80

We describe a 19-year-old white male with juvenile galactosialidosis. He presented with hip arthralgia and was found to have facial "coarseness," corneal clouding, mitral and aortic insufficiency, and hepatosplenomegaly. Ultrastructural studies of skin biopsy and peripheral blood lymphocytes showed membrane-bound inclusions containing sparse fibrillogranular material. Biochemical analysis showed elevated urinary sialyloligosaccharides and no free sialic acid. Fibroblast enzyme analysis showed low activities of both alpha-neuraminidase and beta-galactosidase. To date, most patients with juvenile galactosialidosis have been Japanese. However, unlike those patients, our patient did not have macular cherry-red spots, neurologic abnormalities, or mental retardation. We speculate that this young man represents a new subtype of juvenile galactosialidosis with a potentially different molecular defect from that of the Japanese variant.
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PMID:Juvenile galactosialidosis in a white male: a new variant. 314 49

A case of a 45-year-old woman with a prolonged disease (more than 16 years) is reported. The initial manifestations were those of Raynaud's syndrome. Later a very complex combination of symptoms and syndromes developed which included: arthritis, arthralgia, skin eruptions, lymphadenopathy, hepatosplenomegaly, a tendency toward sclerodermic changes of the face and the distal parts of the fingers, Sjogren's syndrome, slight anemia, transient leucopenia, fast ESR, increased serum immunoglobulin G and A levels and presence of high antibody titres against a nuclear ribonucleoprotein. The authors consider the case as a mixed connective tissue disease.
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PMID:[Mixed connective tissue disease (Sharp's syndrome)--a case report]. 341 79

Forty patients with brucellosis were hospitalized and clinical and laboratory findings of patients were recorded, treatment regimens were discussed. Twenty-five of the patients were males and 15 of the patients were females. Leukopenia 21.62%, anemia 27.02% and ESR elevation 83.87% were found. Fever and exhaustion were present all of the patients. Arthralgia, nonproductive cough, gastrointestinal symptoms, headache and night sweating were the other common symptoms. Hepatomegaly, splenomegaly, hepatosplenomegaly and ronchus were imported physical examination findings. First choice treatment solution was tetracycline-streptomycin combination and this combination succeed 89.19 in percent. Relapses were treated with rifampicin.
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PMID:[Brucellosis: clinical and laboratory findings and treatment in 40 patients]. 344 17

The early phase of the disease in 30 children referred to this Clinic with the diagnosis of rheumatic disease and whose condition was ultimately diagnosed as malignancy was analysed. A correct diagnosis was established after a period of 1-22 months. thus acute leukemia, non-Hodgkin's lymphoma, Hodgkin's disease and solid tumours were diagnosed in 13, 7, 3, 7 consecutive patients. It was demonstrated that in an early stage of neoplastic diseases, signs may occur which are typical also of rheumatic diseases. Fever, arthralgia or arthritis, hepatosplenomegaly, lymphadenopathy and signs of cardiac involvement predominated in the early picture of the disease. The need for diagnostic investigations ruling out neoplastic diseases has been emphasized. They should be performed even in children fulfilling the diagnostic criteria for rheumatic diseases. Attention has been called to the importance of carefully performed hematological and morphological analysis.
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PMID:Differential diagnosis of neoplastic and rheumatic diseases in children. 348 89

A 31-year-old woman presented with fever and arthralgia. Despite treatment with antimicrobials and corticosteroids, her symptoms persisted. A diagnosis of myelodysplastic syndrome (MDS)-refractory anemia (RA) was made by pancytopenia, dysplasia, and trisomy 8. Cultures of bone marrow, blood, and gastric juice showed Mycobacterium avium-intracellulare (MAI). She was treated with antimycobacterial drugs and recombinant human G-CSF/M-CSF and showed an initial response, but spike fever recurred and pancytopenia progressed. Hepatosplenomegaly and marked retroperitoneal lymphadenopathy were revealed, indicating further dissemination of MAI. Treatment with recombinant human GM-CSF and very-low-dose cytosine arabinoside, was started but was not effective. This case showed significant reduction in peripheral blood T-lymphocytes, especially the CD4+ population, and low immunoglobulin levels. Immunodeficiency state associated with long-term steroid therapy and MDS seemed to contribute to the development of the disseminated infection with MAI.
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PMID:Disseminated Mycobacterium avium-intracellulare infection in a patient with myelodysplastic syndrome (refractory anemia). 817 3

A 34-year-old Turkish woman presented with septic fever, sweats, arthralgia, and abdominal pain. Further examination revealed generalized lymphadenopathy, hepatosplenomegaly, pancytopenia, and multiple caseous granulomas. Microbiological diagnosis revealed Brucella melitensis type 3 as causative agent. This case report demonstrates that abdominal pain can be a symptom in brucellosis, and caseous granulomas may occur. In our mobile society Brucella infection should be considered as possible differential diagnosis in patients with fever, lymphadenopathy, hepatosplenomegaly, and pancytopenia, although human brucellosis is rare in Germany and other Western countries.
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PMID:Brucellosis: differential diagnosis of acute abdominal pain. 829 Dec 80

Among 276 paediatric cases of brucellosis seen over a 7-year period, 16 patients (5.8%) with pancytopenia were identified. The most frequent presentations were fever, malaise, anorexia, weight loss, arthralgia, and hepatosplenomegaly. Fourteen patients (87.5%) had positive blood and/or bone marrow cultures for Brucella melitensis. Bone marrow aspiration specimens showed hypercellularity in 14 patients and normocellularity in 2. Histiocytes, eosinophils and plasma cells were increased in every marrow aspirate, and haemophagocytosis was observed in 14 patients (87.5%). Non-caseating granulomas were present in the bone marrow biopsy of 11 patients (68.8%). The pancytopenia was transient, and resolved on treatment of the Brucella infection.
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PMID:Pancytopenia in children with brucellosis: clinical manifestations and bone marrow findings. 836 1

An outbreak of louse-borne relapsing fever, due to the return of soldiers to their original recruitment areas, after the end of thirty years of fighting in northern Ethiopia, was reported in Arsi region, southern Ethiopia. The epidemic spread to different members of the community and eventually the schools. We studied 389 patients affected by the epidemic and who were admitted to Asella Hospital between June 1991 and May 1992. Twenty-seven per cent of the patients were ex-soldiers; 28% were students, who were admitted to the hospital since the schools were opened after the summer vacations. The common clinical features of the disease were fever (99%), headache (92%), hepatosplenomegaly (66%), myalgia (55%), arthralgia (51%), petechial rash (43%), epistaxis (24%) and jaundice (23%). Observed complications were pneumonia (10%), pulmonary edema (6%), myocarditis (3%) and 6 abortions in 15 pregnancies. Patients were treated with low dose penicillin and i.v. fluids. The in-hospital case fatality rate was 3.6%. Jarisch-Herxheimer reaction occurred in 43% of the patients. 1.8% of the patients had relapses after treatment.
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PMID:Louse-borne relapsing fever. A clinical and an epidemiological study of 389 patients in Asella Hospital, Ethiopia. 794 Oct 15

Childhood Ki-1 lymphoma is rarely reported in Taiwan. We present two 8-year-old girls with Ki-1 lymphoma diagnosed and treated at the National Taiwan University Hospital. The first patient presented with a neck mass with hepatosplenomegaly. The second patient initially presented with joint pain and pathologic fracture; she was later found to have bone marrow involvement. Histologic studies of both tumors revealed bizarre large cells with pleomorphic nuclei and prominent nucleoli. All these cells were immunolabeled with monoclonal antibody Ki-1. The two patients received different polyregime chemotherapy protocols followed by bone marrow transplantation (BMT); patient 1 had autologous BMT and patient 2 had allogeneic BMT. In addition, local radiotherapy and retinoic acid were tried for patient 1 but she responded poorly and died 2 years after presentation. In patient 2, although bone marrow was initially involved, allogeneic BMT was performed smoothly after tumor loading was reduced and the patient was in remission at last follow-up, 26 months after presentation. Ki-1 lymphoma in children is characterized by clinical presentations, and histologic and immunologic findings. Aggressive polyregime chemotherapy and bone marrow transplantation are treatments for this disease.
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PMID:Ki-1 lymphoma in two children. 950 48


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