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)

A prospective study of 100 children aged 2-17 years (68 girls) with juvenile rheumatoid arthritis (JRA) was initiated to follow changes in the temporomandibular joint (TMJ), growth of the craniofacial complex and development of the dental occlusion, and to relate the findings to clinical and laboratory parameters. The mean age at onset was 5.5 years. The debut type was acute febrile in 14, pauciarticular in 64, and polyarticular in 22 patients. At examination, at mean age 9 years, 45 patients had pauci- and 55 polyarticular JRA. Definite radiographic TMJ changes were found in 41 patients (unilaterally in 17). 130 children without joint disease served as controls. Of the patients with radiographic TMJ changes, 65% had clinical symptoms and/or signs, the most frequent being restricted mouth opening. Of the patients without radiographic TMJ changes, 19% had uncertain clinical findings (pain, tenderness or slightly restricted mouth opening). Radiographic TMJ changes were significantly related to early onset of JRA, long disease duration, actual polyarticular type, high disease activity, impaired functional capacity and general health, splenomegaly and kidney involvement, low hemoglobin and high gammaglobulin concentrations. A significant relationship was also found with radiographic changes of the cervical spine. Radiographic examination seemed essential for the diagnosis of TMJ arthritis.
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PMID:The temporomandibular joint in juvenile rheumatoid arthritis. Radiographic changes related to clinical and laboratory parameters in 100 children. 706 12

The records of eleven patients who underwent splenectomy for Gaucher's disease at The Mount Sinai Hospital from 1967 to 1981 have been reviewed. The major indications for operation were hypersplenism, pain, and mechanical problems associated with a massively enlarged spleen. There were no operative mortalities and the hematologic picture returned to normal in all cases. Because of the danger of overwhelming sepsis following splenectomy, partial splenectomy should be considered as a therapeutic modality in these patients.
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PMID:The surgical management of Gaucher's disease. 712 40

Sarcoidosis involves the spleen in a significant percentage of patients afflicted with this disease. The most commonly applied modality for studying such patients is the (99m)Tc-sulfur colloid scan. Different patterns have been recognized: "normal" spleen and nonspecific splenic enlargement, with or without focal areas of photon deficiency, which was thought to represent an infarction in a previous report. We have encountered two patients showing splenomegaly and multifocal photon deficiencies secondary to sarcoid granulomata. These cases are presented to inform clinicians of a new pattern. Our patients were managed with systemic corticosteroids and palliation of pain. If symptoms persist or worsen, splenectomy may be necessary to avoid spontaneous rupture. An abbreviated differential diagnosis of the enlarged spleen with multiple focal defects is discussed.
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PMID:Multifocal defects and splenomegaly in sarcoidosis: a new scintigraphic pattern. 713 74

The literature on diaphragmatic hernia in cattle and buffaloes is reviewed. A heifer showed accelerated respiration, symptoms of dyspnoea, occasional coughing and groaning, a variable appetite and emaciation unaccompanied by an increase in temperature for sixteen days prior to parturition. A diagnosis was not established. During parturition which occurred at term, marked symptoms were not observed, and it was decided to perform caesarian section because of the unduly large foetus. During the operation, performed in the standing animal, the heifer offered violent resistance when the abdominal cavity had been opened, showing symptoms of severe dyspnoea and/or pain, an fell down. In this state of excitement, which subsequently came to resemble shock, the heifer showed cyanotic mucosae and a very quick pulse. The heart sounds on the left side were inaudible on auscultation. The operation could only be completed in the recumbent position after sedation. Nothing which could account for the symptoms was detectable throughout treatment. The animal then remained quiet, the mucosae having regained their pink colour, the pulse rate decreased. Respiration continued to be too rapid and it became plain that it was obviously of the pendular type. The animal having been slaughtered in agreement with the owner, diaphragmatic hernia was found to be present on the left side. The enlarged spleen was largely situated in the thorax and partly adhered to the lung. The history of the case is discussed.
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PMID:[Diaphragmatic hernia as a rare complication during caesarean section in a cow (author's transl)]. 719 6

In 1984, Van der Meer first reported six patients with a long history of recurrent attacks of fever of unknown cause and a constantly elevated polyclonal IgD (> 100 U/mL); he suggested the acronym of "hyper-IgD syndrome" (HIDS). A recent literature review identified 60 cases (59 from Europe and 1 from Japan). The mean age was 27 years (range: 3-69 years). The family studies have shown a positive family history for periodic fever (40% out of the patients) but not for hyper-IgD. The median age at onset was 0.5 years (range from the first weeks of life to 53 years). The length of the febrile attacks, though variable, lasted from 3 to 7 days. The frequency of the attacks varied among the individual patients, but in general it was once a month or bimonthly. The fever was sustained in all 60 patients (from 38 degrees C to 41 degrees C), with a rapid rose, a plateau and a slow decline to normal values over 5 days. The associated clinical findings involve abdominal symptoms (pain, vomiting, diarrhea), recurrent peritonitis, lymphadenopathy, splenomegaly, articular manifestations (non-destructive recurrent arthritis) and skin lesions (vasculitis). The prognosis is benign. The aetiopathogenesis of HIDS is unknown. The role of IgD in the pathogenesis remains to be elucidated. The therapy is only supportive.
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PMID:[Hyper-IgD syndrome (HIDS)]. 762 86

From 1980 to 1993, 39 splenectomies were performed in the Department of Visceral Surgery of Saint-Louis Hospital, in patients referred for myelofibrosis associated with myeloid splenomegaly. The short term morbidity was considerable: 33 serious haemorrhagic, infectious or thrombotic complications including 5 fatal accidents were observed in 18 patients. Severe thrombotic or infectious complications leading to 6 further deaths occurred in 8 patients over the two years following splenectomy, while six cases of acute leukaemia appeared between 6 months and 3 years after splenectomy. In 40% of cases with regular follow-up, the operation did not provide any haematological improvement and all these patients died. Only patients with minimally progressive or stable myelofibrosis and residual marrow activity in isotope studies showed an amelioration of general status with relief of pain and reduction of transfusional requirements. The indication for splenectomy should therefore probably be limited to such cases.
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PMID:Risks and benefits of splenectomy in myelofibrosis: an analysis of 39 cases. 789 30

Laparoscopic splenectomy was attempted in 16 patients and was performed successfully in 13 (81%) patients with the diagnosis of idiopathic thrombocytopenic purpura (ITP), AIDS-related thrombocytopenia, Hodgkin's disease, or lymphoma. The operative time averaged 157 min, and autologous transfusion was required in four patients. The postsurgical stay averaged 3 days in patients with completely laparoscopic splenectomies and 4 days in patients whose spleens were removed through small counterincisions. No major complications secondary to the procedure itself occurred postoperatively. Conversion to open operation was necessary in three (19%) patients because of bleeding or splenomegaly. With careful selection of patients and mastery of the technique, laparoscopic splenectomy can be safely performed on normal or slightly enlarged spleens. The advantages are less pain, shorter hospitalization, and reduced disability as compared to "open" splenectomy.
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PMID:Laparoscopic splenectomy. 799 69

The authors report about 4 cases of splenic metastases in patients with the following neoplasms: urothelial carcinoma of the left kidney, adenocarcinoma of the uterine body, adenocarcinoma of the left flexure of the colon, adenocarcinoma of the sigmoid loop. On large autopsy series, metastases to the spleen are not rare (7% in average), and rank 10th among the 44 metastatic sites described in the literature. Cancers of the breast, lung, skin (melanoma), ovaries and stomach (cardia) make up more than 60% of the primary tumors. The diagnosis is most often accidental (surveillance of operated patients), rarely clinical (pain, splenomegaly), and it is based on medical imaging (ultrasound, CT). In the absence of hemostatic disorders, percutaneous needle biopsies are possible. Treatment, when at all possible, is surgical (splenectomy) and must comply with carcinological rules.
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PMID:[Splenic metastases. Apropos of 4 cases]. 816 2

Nineteen consecutive patients of pyogenic liver abscess (13 males, 6 females, mean age 45.2 +/- 6, 5 years) were studied over a period of 5 years for the clinical profile and therapeutic efficacy of percutaneous aspiration. Majority of them presented with spiking fever (94.7%), pain over right upper quadrant of abdomen (53.8%) and often with prostration and shock (31.6%). The onset has been rather acute in patients with multiple abscesses (7 cases). Ascites (10.5%), clubbing of fingers (15.8%) and splenomegaly (10.5%) were observed in cases with long duration of illness. There has been the polymorphonuclear leucocytosis (89.5%) and mild to moderate anaemia (52.6%). Jaundice (42.1%) was usually mild degree (serum bilirubin 4.6 +/- 2.4 mg/dl). The serum transaminases and alkaline phosphatase were raised in 94.9% of cases. Ultrasonography revealed predominantly hypoechoic (54.1%) areas with frequent distal acoustic enhancement and internal echoes (21.6%). Seven patients had multiple abscesses with 25 lesions (size 5.2 +/- 4.6 cms) situated mainly over antero-inferior segment of the right lobe (45.9%) of the liver. The blood and pus cultures demonstrated the micro-organisms (positive in 63.9% and 86.6% respectively) predominantly of bowel flora including facultative gram negative rods and anaerobes. Mortality was 26.5% and pleuro-pulmonary complications were the commonest (26.4%) one. 15 cases were treated by percutaneous aspiration, proper antibiotic and metronidazole with encouraging results (only two deaths). Complete resolution of abscess took place in 14.2 +/- 1.2 weeks (range 6 weeks to 7.5 months). It is emphasized that percutaneous needle aspiration is useful both for diagnosis and treatment of pyogenic liver abscess.
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PMID:Clinico-therapeutic profile of pyogenic liver abscess. 822 94

The case of an 18-year-old female, with mild left hypochondrial pain and an abdominal mass mimicking gross splenomegaly, with severe small and large bowel fibrosis and adhesions and numerous mononuclear cellular infiltrate on histology, is presented. A clear picture of intestinal obstruction only unfolded terminally. She died within 48 hours of presentation. Our patient illustrates the fact that severe intestinal adhesions may present with very mild symptoms and signs and therefore a high index of suspicion is necessary, to reduce the attendant high mortality in intestinal obstruction in our environment.
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PMID:Severe large and small bowel fibrosis clinically mimicking gross splenomegaly. 834 48


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