Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and laboratory features, treatment, prognosis, complications and disability of 45 patients with adult-onset Still's disease were studied. Sixty per cent of the patients were female. Median age at onset was 25 years and median observation period after onset was 41 months. For 28 patients detailed data were available of the first month of illness. In only 43 per cent of these did the disease present with the classical triad of fever, arthritis and rash, although in the whole group eventually, fever with temperatures of 40 degrees C or more occurred in 84 per cent, arthritis in 98 per cent, and the typical rash in 82 per cent. In 32 per cent of the patients with rash, this was pruritic. Other features seen frequently were: lymphadenopathy (71 per cent), splenomegaly (36 per cent), pleuritis and/or pneumonitis (31 per cent), pericarditis (22 per cent), leucocytosis (98 per cent) and hepatic abnormalities (84 per cent). The high spiking fever was reduced to normal in six of 21 cases (29 per cent) by aspirin, in 19 of 27 cases (70 per cent) by indomethacin and in six of seven cases (86 per cent) by naproxen. In 16 of 21 cases (76 per cent) glucocorticoids reduced the systemic and/or joint symptoms. In three patients who remained febrile on glucocorticoids, indomethacin reduced temperature to normal. Eight patients whose joint disease improved on steroids later developed severe joint destructions. Thirteen patients received one or several slow-acting antirheumatic drugs. Only in eight of 18 trials (44 per cent) with one of these drugs did symptoms and signs improve. Fifty-one per cent of the 45 patients had self-limiting disease and 49 per cent had persistent disease with continuous activity for at least one year. At the time of evaluation 47 per cent of the 45 patients were in remission without medication, 33 per cent were in remission while on medication and 20 per cent had active disease. Three life-threatening complications occurred: two patients developed signs of cardiac tamponade and one almost died from diffuse intravascular coagulation. Disability was determined primarily by the course of the arthritis. At the time of review 43 per cent of the 45 patients had no joint destruction (Group 1), 24 per cent had destruction of at least one joint, but had no evidence of disease of the root joints (Group 2) and 33 per cent had destruction in at least one root joint (Group 3).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases. 365 48

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

Visceral leishmaniasis (kala-azar) is an important cause of morbidity and mortality in widely scattered areas of the world. To better characterize the South American form of the disease, the clinical and laboratory manifestations of 29 patients admitted to hospital (18 male and 11 female patients, mean age 4.9 years), were assessed in an endemic area in northeastern Brazil. Fever, weight loss, pronounced splenomegaly, hepatomegaly, anemia, thrombocytopenia, relative neutropenia, hypoalbuminemia and hypergammaglobulinemia were found in the majority of patients. Symptoms were often present for two or more months before diagnosis. Secondary infections complicated many cases; there were ten cases of pneumonia and half of the patients had one or more intestinal parasites. The average length of hospital stay was 27 days; all patients were treated with meglumine antimoniate (Glucantime). The mortality rate was 3%. American visceral leishmaniasis remains an important disease among children living in endemic areas.
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PMID:American visceral leishmaniasis (kala-azar). 402 31

Two patients with the first symptoms of adult onset Still's disease in their 7th decade are reported. In both cases their advanced age was a main reason for a prolonged delay in diagnosis. Besides the characteristic fever pattern, rash and arthritis, our patients showed other typical features of Still's disease such as splenomegaly, pleuritis, pneumonitis, carpal ankylosis and leukocytosis. In addition both showed marked hepatic abnormalities which seemed to be due to the disease itself in one case and at least in part related to exposure to phenylbutazone and diclofenac in the other.
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PMID:Adult onset Still's disease in the elderly: a report of two cases. 405 3

We describe herein the clinical features, diagnosis, and successful treatment of two patients with Legionella micdadei pneumonia, the Pittsburgh pneumonia agent. The rapid pulmonary cavitation and relapse of symptoms after a course of therapy in a renal transplant recipient, and the prolonged illness with hemorrhagic pleural fluid, splenomegaly, and multiple-organ dysfunction in a nonimmunocompromised host, illustrate the clinical spectrum of infections with this organism.
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PMID:Legionella micdadei (Pittsburgh pneumonia agent). Two infections with unusual clinical features. 633 66

A new spontaneous autosomal recessive mutation has recently occurred at the motheaten (me) locus on Chromosome 6 in strain C57BL/6J mice. Homozygotes for the new allele, designated "viable motheaten" (mev), have a mean life span of 61 +/- 2.4 days, compared with only 22 +/- 1.3 days for C57BL/6J-me/me mice. Like the original motheaten mutation, the immediate cause of death in mev/mev mice appears to be severe pneumonitis associated with accumulations of macrophages, granulocytes, and lymphocytes in the lungs. However, because of its longer life span, progression of the disease in mev/mev mice is more amenable to investigation. Eosinophilic crystalline material in alveolar macrophages from mev/mev mice is associated with extravasation of erythrocytes into alveoli. These crystals are morphologically indistinguishable from hematoidin, which results from hemoglobin breakdown following uptake of erythrocytes by macrophages. Severe macrocytic hypochromic anemia with abnormalities in size and shape of erythrocytes develops by 7 weeks. A two-fold increase in peripheral leukocyte count and a five-fold increase in the percentage of neutrophils is seen by 10 weeks. Viable motheaten mice develop focal granulocytic skin lesions by 4 days of age, show depletion of cells from the thymus cortex by 4 weeks, and lack lymphoid follicles in the lymph nodes, spleen, and Peyer's patches. Excessive erythropoiesis and myelopoiesis in the spleen result in marked splenomegaly. Lymph nodes and spleens from mev/mev mice contain increased numbers of plasma cells by 3 weeks; and by 6 weeks, large numbers of atypical plasma cells with Russell bodies are evident. Development of glomerulonephritis by 10 weeks is characterized by granular depositis of immunoglobulin and complement within glomeruli. A twofold increase of blood urea nitrogen levels is present by 15 weeks. Sterility of male mev/mev mice is associated with Leydig cell depletion in the testes, lowered testosterone levels, and impaired spermatogenesis.
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PMID:"Viable motheaten," a new allele at the motheaten locus. I. Pathology. 638 Feb 98

Clinical and morphologic characteristics of hairy cell leukemia at 11 patients have been analyzed. The frequency of this disorder is 2.5% from all leukemias. This disorder is often retrospectively diagnosed--at 7 cases from our study. the disorder is often retrospectively diagnosed--older age (78%). Splenomegaly is the main clinical manifestation, at all 11 patients. Pancytopenia is frequent finding but at 4 patients the leucocyte count was over 10 X 10(9)/1 in the beginning. Hairy cells, although not always with typical appearance, in 90% of the cases are found in peripheral blood over 10%. Bone marrow biopsy cytological and hystological findings at 9 patients were typical for diagnosis. Fibrosis was present in 6 specimens. At 7 patients diagnosis was confirmed with histological examinations of lymphocyte concentrates from peripheral blood on thin and ultrathin sections, as well as with electron microscopy characteristic appearance of hairy cells. Pneumonia as complication was registered in 24 occasions, gastro-intestinal infections at 9, haemorrhagic syndrome at 4 and skin carcinoma at 2 cases. Treatment was variable--2 patients were observed for more than 50 months, 2 were splenectomised, of which one with complete remission longer than a year, while from 8 treated with COP protocol, complete remission was obtained in 5 (62%) patients, and two treated with CHOP protocol entered complete remission for longer than 12 months. Average survival is 51 months (2--144). Three (28%) patients died.
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PMID:[Hairy cell leukemia. Personal experience with 11 cases]. 668 Mar 1

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
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PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

Four cases of acute myelogenous leukemia and six cases of chronic myelogenous leukemia after treatment with azathioprine and prednisone for renal allotransplantation have been described in the literature. We report another two cases of acute leukemia 10 and 5 years after successful renal allotransplantation. Patient 1, a 29-year-old farmer, exhibited the signs of acute lymphatic leukemia resistent to treatment with cytostatic agens. Death was due to pneumonia. Patient 2, a 47-year-old salesman, developed pancytopenia together with splenomegaly. After splenectomy an atypical subacute myeloid leukemia became apparent which was not treated due to withdrawal of the patient. He died 2 months after diagnosis. Both patients received long-term immunosuppressive therapy with azathiopine and prednisone until the leukemia was diagnosed. A relationship between long-term immunosuppression and the occurrence of leukemia is postulated.
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PMID:[Acute leukemia after kidney allotransplantation (author's transl)]. 699 44

Hairy-cell leukemia (HCL) is an unusual malignant hematologic disorder involving splenomegaly, pancytopenia, and circulating mononuclear cells with prominent cytoplasmic projections. As in most forms of leukemia, the risk of pulmonary infection by normal pathogens and opportunistic invaders alike is high. HCL may be associated with granulomatous infections of the lung, especially mycobacterioses. Of the authors' series of 33 patients, 9 had a fungal or mycobacterial infection, including 5 atypical mycobacterial species. Five of the 6 patients with mycobacterioses and 1 of the 3 with fungal pneumonia survived the infection with appropriate therapy. Granulomatous infections, particularly mycobacterioses, should be seriously considered in the differential diagnosis of pulmonary parenchymal disease in a patient with HCl.
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PMID:Pneumonia in hairy-cell leukemia. 720 21


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