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)

Fifteen patients (11 males, four females; median age 57) manifested a disease characterized by (1) the histopathologic features of Castleman's disease, plasma cell type, in lymph node biopsies; (2) predominantly lymphadenopathic disease, involving multiple, preferentially peripheral nodal groups; (3) varied manifestations of multisystemic involvement (such as constitutional symptoms; splenomegaly and hypergammaglobulinemia; elevated ESR, anemia, and thrombocytopenia; hepatomegaly and altered liver function tests (LFTs); signs of renal disease); and (4) idiopathic nature. Two main patterns of evolution were recognized: persistent, with sustained clinical manifestations, and episodic, with recurrent exacerbations and remissions. Seventy-three percent of patients had infectious complications, and 27% developed malignancies. Complete remissions were obtained occasionally with antineoplastic agents and with splenectomy but not with glucocorticosteroids alone. The median survival time is 30 months; 60% of patients have died. Median follow-up in the six surviving patients is 97+ months. A review of 50 cases in the literature revealed similar clinical and laboratory features. Despite some similarities with autoimmune diseases, the main features of this process seem to best fit a hyperplastic-dysplastic lymphoid disorder in a setting of immunoregulatory deficit.
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PMID:A systemic lymphoproliferative disorder with morphologic features of Castleman's disease: clinical findings and clinicopathologic correlations in 15 patients. 403 67

Four new cases of adult Still's disease have been observed in the past three years. Clinical and biological features are high fever, polyarthralgia, macular rash on forearm, pericarditis, splenomegaly, lymphadenopathy, alopecia, anemia, hyper-leucocytosis, raised ESR. Transient neurological findings may also be observed with abnormal reflexes, cranial nerve paralysis. High dose steroids treatment can be used in case of systemic involvement. The long term prognosis is usually good with treatment. In conclusion, the diagnosis of Still's disease is essentially clinical and should not be mistaken for septicemia.
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PMID:[Still's disease in the adult (author's transl)]. 627 24

Two patients with adult Still's disease are presented. Both had high "septic" fever, weight loss, mild pharyngitis, evanescent maculo-papular rash, myalgias, arthralgias, splenomegaly and pericarditis, while one of the two patients also had lymphadenopathy and pleurisy. Arthritis, which is a sine qua non for the diagnosis, developed only 5 months after disease onset in one patient. Both ultimately developed severe destructive joint disease requiring hip arthroplasty. Laboratory findings were neutrophilic leukocytosis, normochromic normocytic anemia, elevated ESR, slightly elevated liver enzyme values, negative IgM-rheumatoid factor and antinuclear antibodies, and normal or slightly elevated complement factors. Several biopsies failed to reveal pathognomonic findings. It is of the utmost importance that the exclusion diagnosis of adult Still's disease be posed in order to avoid repeated hospitalizations with undue investigations and unnecessary therapeutic trials with antibiotics.
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PMID:[Still's disease in adults. 2 case reports]. 682 46

A retrospective was designed to analyse the mode of presentation, clinical signs and haematological and biochemical abnormalities in 225 consecutive Black (Zulu) patients who were admitted to a general medical ward between the years 1970 and 1981 and in whom cirrhosis was later diagnosed. The most common presenting complaint was swelling of the body (60% of the patients), followed by abdominal pain (32%) and episodes of bleeding, mainly from the gastrointestinal tract (19%). On examination, hepatomegaly was encountered in 66% of the patients, with moderate to massive enlargement in 40%. Ascites was detected in 56%, with tense abdominal distension in 34%. Jaundice was present in 38% and emaciation, mental disturbance and splenomegaly in over 25%. Spider naevi (found in 2 patients) and Dupuytren's contracture (found in 1) were very rare. Thrombocytopenia and a high ESR were common. Over 90% of patients had low albumin and high globulin concentrations (albumin less than 20 g/dl and globulin greater than 60 g/dl in 25%). Bilirubin and alkaline phosphatase levels and the prothrombin index were found to be within normal limits in 32%, 24% and 52% of cases respectively. Histologically the lesion was most commonly micronodular (73%) with variable deposits of fat and iron. Peritoneoscopy was the most useful special investigation in the diagnosis of cirrhosis, leading to a correct diagnosis in 77% of cases. In conclusion, the clinical signs, biochemical abnormalities and histological features suggest that the factors causing cirrhosis in the community studied are mixed; it may result from the combined effects of alcohol abuse, malnutrition and chronic viral (e.g. hepatitis B) infections.
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PMID:Clinical presentation and biochemical abnormalities in black (Zulu) patients with cirrhosis in Durban. 707 88

The conditions of five adults were eventually diagnosed as juvenile rheumatoid arthritis (Still's disease). Prolonged hospitalization, repetitive roentogenographic examinations, biopsies, laparotomies, and therapeutic trials with toxic agents preceded the establishment of the final diagnosis. Common early findings in all cases were prolonged "septic fever," polyarthralgia, and an elevated ESR. Three patients had a rash, four had splenomegaly, three had a vague history of a similar disease, and four had leukocytosis. Some of the patients were older than others described in the literature. Two received immunosuppressive agents and did relatively well. In view of our experience and the few reports in the literature, we concluded that juvenile rheumatoid arthritis in adults has to be seriously considered in the presence of prolonged septic fever, polyarthralgia, rash, and splenomegaly, before harmful drugs are given or risky procedures are performed.
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PMID:An adult form of juvenile rheumatoid arthritis. 739 11

We conducted a retrospective study to characterize the clinical course, microbiologic spectrum, and risk factors for endocarditis and for associated mortality in a large series of patients with documented pacemaker endocarditis. Using a computerized search through the medical records of 10 major hospitals in Israel from 1982 to 1992, and carefully reviewing the charts, we identified 44 patients with pacemaker endocarditis. The cases were categorized as definite (n = 25), probable (n = 12), or possible (n = 7) infective endocarditis based on strict case definition. Fever and chills were the most common symptoms. Increased ESR, leukocytosis, microscopic hematuria, and anemia were the most common laboratory findings. A relatively high proportion of the patients were diabetic. The most common source of endocarditis was infection acquired by the placement procedure or infection of the pacemaker pouch. Demographic, clinical, and laboratory features were similar to those of endocarditis patients of a similar age range without pacemakers, although the frequency of fever and chills was higher in our patients than in those patients and splenomegaly, vascular embolic phenomena, and new or changing murmurs were rare in our patients. The major pathogens were Staphylococcus aureus and Staphylococcus epidermidis, similar to other series of pacemaker-associated bacteremia and similar to the microbiologic findings of early prosthetic-valve endocarditis. However, this microbiologic profile is different from that of native-valve endocarditis. Although the present series did not show a statistically significant advantage to electrode removal over conservative treatment, when analyzed together with pooled data from other studies, it suggests that the surgical approach is preferable.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pacemaker endocarditis. Report of 44 cases and review of the literature. 798 81

Underlying diseases, complications, clinical findings, and laboratory findings were evaluated in 158 cases of septicaemia admitted to Jikei University Hospital from 1975 to 1994, in order to conjectured factors that prescribe for the prognosis. 50% of the patients had underlying diseases. Malignancy including leukaemia (31 cases, 39.2%) was the most common underlying disease, followed by low birth weight infant (17 cases, 21.5%), aplastic anemia (9 case, 11.4%), and congenital heart disease (7 cases, 8.9%). The death rate for patients with underlying disease (27.8%) was significantly greater than the mortality for normal patients with septicaemia (8.9%) (p < 0.05). Meningitis (24.7%) was the most common complication, followed by DIC (19.6%), shock (15.2%), and pneumonia (10.8%). The mortality rate of septicaemia complicated by shock was 66.7% (p < 0.01), and that complicated by DIC was 45.2% (p < 0.01). The mortality rate for patients with the clinical findings of respiratory distress, cough, abdominal distention, cyanosis, splenomegaly, or peripheral coldness was more than 40% and significantly greater (p < 0.01). Mortality rate in patients with granulocyte counts of < 4.000/mm3, platelet counts of < 5 x 10(4)/ mm3, total protein of < 5.0 g/dl, or ESR of < 20 mm/hr were significantly greater (p < 0.01) than those in patients with normal laboratory findings. Coincidence rate of blood and stool cultures was 57.9% for E. coli, and 28.6% for Klebsiella sp., and that of blood and throat cultures was more than 30% for Pseudomonas sp., Haemophilus influenzae, and Staphylococcus aureus. In the study of antimicrobial susceptibility for microorganisms isolated, the number of drug resistant S. aureus had increased in the last 10 years.
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PMID:[Study on septicaemia in infants and children in the past 20 years. Part 2. An analysis of factors that prescribe for the prognosis]. 889 May 45

Among the chronic myeloproliferative disorders essential thrombocythemia (ET) is known to be a distinct clinical entity in which an excessive number of morphologically and functionally abnormal platelets are produced. The clonal nature of the disease is well established. Based on a review of the literature the present authors propose the following novel criteria for the diagnosis of ET: A1. Platelet count in excess of 600 x 10(9)/L. A2. No increase in red-cell mass (RCM) in the presence of stainable iron in the bone marrow or failure of iron trial (RCM < 36 mL/kg in males and < 32 mL/kg in females; or RCM < 25% above mean normal predicted value*). A3. No Philadelphia chromosome. A4. Megakaryocytic hyperplasia (= increased megakaryocyte number and size) in histological sections of bone marrow and/or increased megakaryocytic ploidy (two-color flow cytometry); no collagen fibrosis. B1. Splenomegaly on isotopic scan or echogram. B2. Unstimulated growth of BFU-E and/or CFU-Meg present. B3. Normal ESR/fibrinogen. The diagnosis of ET is considered to be established if A1 + A2 + A3 + A4 or A1 + A2 + A3 + two B-criteria are fulfilled. (* Br J Haematol 1995; 89:748-756.)
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PMID:Diagnostic and differential criteria of essential thrombocythemia and reactive thrombocytosis. 895 71

There is no objective data on the value of individual clinical symptoms or signs in the diagnosis of enteric fever in a febrile patient. The purpose of the study was to assess the value of some clinical and simple laboratory features in the diagnosis of enteric fever. One hundred & six patients with microbiologically confirmed enteric fever and 170 patients with other established febrile illnesses were included in the evaluation. History of stepladder pattern of rise of temperature, loose motions, relative bradycardia and coated tongue proved to be powerful markers of enteric fever with high specificity (100%, 94.71%, 94.71%, 94.12% respectively), positive and negative predictive values. Headache, hepatomegaly and splenomegaly were moderately powerful. ESR and WBC count appeared to have little value in the diagnosis of enteric fever. Pattern of onset and loose motions did not discriminate between typhoid and paratyphoid fever. Most of these patients had illness persisting beyond one week by which viral infections and infectious enterocolitides were largely excluded. Elucidation of power of these markers in distinguishing enteric fever from other febrile illnesses with the help of better designed prospective studies would lessen our dependence on expensive and time consuming laboratory investigations.
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PMID:Value of clinical features in the diagnosis of enteric fever. 946 34

Seventeen cases of dapsone syndrome were seen in five years from 1992 onwards. Their mean age was 27.8 years (range 11 to 60 years). Male to female ratio was 1.1:1. Of these cases, seven had confirmed leprosy, nine were cases of suspected leprosy and one case had lichen planus. On an average, they developed the symptoms 27 days after the intake of dapsone. The cutaneous lesions were in the form of erythematous papules and plaques (13 cases), eczematous lesions (four cases) and associated bullous lesions (two cases). The other manifestations were: fever (16 cases), pruritus (15 cases), lymphadenopathy (14 cases), hepatomegaly (10 cases), icterus and oral erosions (five cases each), photosensitivity (four cases) and splenomegaly (two cases). Previous drug allergy was present in four cases. Elevated ESR and liver enzyme levels were invariable findings. Raised bilirubin levels and hemolytic anaemia were seen in eight cases. Apart from one case with hepatic encephalopathy, all other cases had a favourable outcome either on conservative management (eight cases) or on oral corticosteroids (eight cases). Oral provocation test was done in two cases with positive response while intradermal test was not very reliable.
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PMID:Dapsone syndrome--a five year retrospective analysis. 980 99


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