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)

Rickettsia is emerging in the subcontinent and clinically presents as non-specific febrile illness. At present there is no cheap & easily available diagnostic tool in our hand. Beside this, Weil-Felix test is becoming abandoned. So, high index of clinical suspicions is essential to diagnose rickettsia at early stage and to prevent mortality & morbidity. 40 cases were recorded among the admitted febrile patients in MMCH since 2003 to 2005. Cases were selected by clinical suspicions; exclusions of other common febrile illness & thereafter supported by lab. Investigations, specially by positive Weil-felix test. Cases were distributed through out the year but 19 (47.5%) cases were detected in March to May. 12 (30%) cases were found in August to October. The remaining 9 cases were detected in the rest 6 months. All (40) cases were presented with fever (100%), headache was present in 33 (82.5%) cases, rashes were present in 15 (37.5%) cases, isolated splenomegaly was found in 15 (37.5%) cases & hepatosplenomegaly in 12 (30%) cases, arthralgia in 13 (32.5%) cases, lymphadenopathy in 5 (12.5%) cases; 2 (5%) cases attended with unconsciousness & epistaxis in 1 (2.25%) case. Scrub typhus were 19 (47.5%), Indian tick typhus 16 (40%), 5 (12.5%) cases were with dual pathology and were associated with enteric fever. 15 (37.5%) cases were treated with tetracycline only. 20 (50%) cases with only doxyclycline & 5 (12.5%) cases with tetracycline and ceftriaxone as these cases were associated with enteric fever. All patients (100%) cured with treatment.
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PMID:Study on 40 cases of rickettsia. 1734 87

We report a 12-year-old girl with sickle cell disease who presented with pain in abdomen, fever, joint pain and hematemesis. On examination she had mild jaundice and splenomegaly. Upper GI endoscopy showed esophageal varices. She was treated with variceal band ligation and is well on folic acid supplements and propranolol.
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PMID:Portal hypertension associated with sickle cell disease. 1843 Oct 24

Sjogren's syndrome (SS) is characterized by an increased risk of developing non-Hodgkin's lymphoma (NHL). Optimal treatment for NHL-complicating SS is not clearly established. NHL, which expresses the CD20 antigen on tumor cell surfaces, is a disease entity candidate to treatment with anti-CD20 monoclonal antibodies. We report clinical and immunological data of a patient with SS and NHL who was treated with a regimen consisting of cyclophosphamide/vincristine/prednisone (CVP) plus rituximab. A 68-year-old women had a 26-year history of SS and autoimmune thyroiditis. The clinical course of SS was complicated with severe splenomegaly. An increased percentage of CD19+ B cells (up to 30%) was detected in peripheral blood during follow-up. Clonal rearrangement of immunoglobulin heavy chain was detected. Low-grade B marginal zone lymphoma was diagnosed (peripheral blood immunophenotype: CD19+CD20+CD23+sIg+Kappa; bone marrow immunophenotype: 25% lymphocytes; CD19+CD20+CD79A/BCL2+). She received a total of six cycles of CVP plus rituximab (375 mg/m2). Therapy was well tolerated, and B lymphocytes were depleted from the peripheral blood. Splenomegaly normalized. No evidence of neoplastic infiltration was detected in bone marrow after completion of therapy, while certain symptoms of SS (sicca and arthralgia) improved with treatment. CVP plus rituximab proved effective in a patient with SS with NHL.
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PMID:Combined therapy with rituximab plus cyclophosphamide/vincristine/prednisone for Sjogren's syndrome-associated B-cell non-Hodgkin's lymphoma. 1827 Aug 61

Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). Schnitzler syndrome is not wellknown and very likely under-recognized. Comprehensive diagnostic examinations are necessary to rule out other diseases, especially those of hematologic origin. Close interdisciplinary collaboration is mandatory. The etiology of Schnitzler syndrome is unclear, but the rapid response to the interleukin-1 receptor inhibitor anakinra underlines the pivotal role which the proinflammatory cytokine interleukin-1 may play in the pathophysiology of this potentially autoinflammatory disorder.
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PMID:The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy--an autoinflammatory syndrome? 1837 Oct 52

The aim of this study was to evaluate the clinical, laboratory findings and therapeutic features of patients with brucellosis. The diagnosis was made by clinical findings, automated blood culture, serology (Rose Bengal plate agglutination test, standard tube agglutination (Wright) and immunofluorerescence). The susceptibility of 13 strains was tested in vitro. The base sequence was determined for four strains. Forty-five cases were collected (31 acute and 14 sub-acute). Contamination was digestive in 62%. Symptoms of patients were fever (93%), sweating (82%), arthralgia (78%) and splenomegaly (51%). Elevated erythrocyte sedimentation rate was determined in 80%, leukopenia in 49% and anaemia in 37% of cases. Blood cultures were positives in 39% of cases. The four sequenced strains were identified as Brucella melitensis biovar abortus. Six strains were resistant to sufomethoxazol-trimetoprim (54%). In 93% of cases, the treatment was associated rifampicin and doxycyclin. One patient died. No relapse was reported.
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PMID:[Clinical manifestations, complications and treatment of brucellosis: 45-patient study]. 1838 52

Adult onset Still's disease (AOSD) is an uncommon disorder of unknown cause. The clinical symptoms of AOSD are a spiking fever, a typical rash, arthralgia or arthritis, sore throat, lymphadenopathy, and splenomegaly. Pleuropulmonary and cardiac involvement are rare. We report a patient with a two-year history of AOSD with myocarditis refractory to cyclosporine and glucocorticoid. Significant congestive heart failure due to left ventricle dysfunction and hyperferritinemia developed during the hospital course. After therapy with etanercept, the patient's clinical manifestations recovered and she regained normal left ventricular systolic function.
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PMID:Etanercept as a rescue agent in patient with adult onset Still's disease complicated with congestive heart failure. 1850 Apr 57

The present work is a prospective, observational, hospital based study on 100 sero positive cases of dengue infection, admitted to Dhaka Children Hospital, Dhaka, Bangladesh during the period 2000 -2001. The patients were in the age group 8 months to 14 years with a mean age of 8.3 years. The serological tests were performed by rapid strip test. Primary dengue infection (only Ig M positive) was observed in 15% cases while rest 85% were secondary dengue infection (either Ig G or both Ig M and Ig G positive). Classical dengue fever (DF) was noted in 11% patients and 89% children presented with dengue hemorrhagic fever / dengue shock syndrome (DHF / DSS). Common clinical presentations were fever, headache, retro- orbital pain, arthralgia / bone pain, vomiting, abdominal pain and bleeding manifestations. Other presentations were tachycardia, bradycardia, hypotension, hepatomegaly, splenomegaly, pleural effusion, ascites, thrombocytopenia and high hematocrit values. The incidences of tachycardia, hypotension, hepatomegaly, high hematocrit and thrombocytopenia were significantly higher in DHF / DSS cases. The tourniquet test was positive in significantly higher percentage of DF cases. The tourniquet test and thrombocytopenia did not correlate well with other bleeding manifestations suggesting alternate pathogenesis for bleeding. In an epidemic setting, if a child presents with fever, vomiting, musculoskeletal pain and bleeding along with hepatomegaly, low platelet count and high hematocrit, a strong possibility of DHF/ DSS should be kept.
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PMID:Clinical and laboratory profile of dengue infection in children. 1860 66

Schnitzler's syndrome (SS) is defined by monoclonal gammopathy and chronic urticaria combined with at least two of the following features: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, palpable lymph nodes, elevated ESR, and leukocytosis. We report a 49-year-old man with monoclonal IgM gammopathy and a 4-year history of recurrent urticarial rash, unexplained fever and arthralgias. The skin biopsy from an acute lesion revealed perivascular lymphocytic infiltrates consisting of CD4+ and CD8+ T lymphocytes. To our knowledge, this is the first report of an immunophenotypic characterization of skin infiltrates in SS. A lower CD4+/CD8+ ratio of circulating T lymphocytes was also detected. SS usually has a benign course, but in 15% of patients a lymphoproliferative disorder develops.
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PMID:Schnitzler's syndrome: monoclonal gammopathy associated with chronic urticaria. 1861 98

The aim of the present study was to evaluate the clinical features of childhood-onset Familial Mediterranean fever (FMF) patients and to assess the phenotype-genotype correlation. The study included patients with childhood-onset FMF that followed up over a period of 18 years in the Division of Pediatric Allergy and Immunology clinic. Twelve MEFV mutations were investigated in all patients. The patients were classified into four groups according to mutations: 1, M694V homozygote; 2, M694V heterozygote; 3, compound heterozygote for M694V; and 4, other-other gene mutation group. The following parameters were evaluated: gender, age of onset, age at diagnosis, time interval between disease onset and diagnosis, fever, abdominal pain, chest pain, arthralgia, arthritis, myalgia, vomiting, diarrhea, constipation, headache, erysipela-like erythema, protracted febrile myalgia, splenomegaly, hepatomegaly, consanguinity, number of attacks before and after treatment, severity score, response to colchicine treatment. Of the 124 patients included in the study, 105 had at least one MEFV gene mutation. M694V homozygosity was the most common mutation, followed by M694V heterozygotes and M694V-M680I compound heterozygotes. Severity score was found significantly higher in patients with M694V homozygote and compound heterozygote for M694V compared with other groups. The data supported the findings in literature that FMF patients with M694V homozygote and compound heterozygote for M694V gene mutations experience a more severe clinical course.
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PMID:The clinical and genetical features of 124 children with Familial Mediterranean fever: experience of a single tertiary center. 1911 56

Hepatitis C is caused by the hepatitis C virus (HCV) infection. According to World Health Organization data, 3% of the world population (approximately 170 million people) is infected with HCV; in Poland there are over 700,000. Over 70% of those infected manifest no symptoms in the acute phase of the disease, and in about 70-80% the acute phase progresses into a chronic form. Patients with symptoms in the acute phase of HCV infection most commonly present with unspecific signs and symptoms that may develop in other viral liver infections, e.g. malaise, fatigue, abdominal pain, mild hepato- and splenomegaly and arthralgia. These symptoms usually persist for 2 to 12 weeks. In the chronic phase a subset of patients complain of malaise, nausea, abdominal pain and itching. With time, chronic hepatitis C may develop into liver cirrhosis. The basic diagnostic methods in HCV infection involve determination of anti-HCV antibodies using the ELISA immunoassay and examination of HCV-RNA with the RT-PCR method. The current treatment of HCV infection involves administration of pegylated interferon a and ribavirin over a period of 48 weeks in HCV-1 genotype infection, and 24 weeks for HCV-2 and 3 genotypes. Effectiveness of therapy depends on the HCV genotype. HCV elimination can be achieved in 78% of patients with HCV-2 and 3 genotypes, and in 55% of patients with HCV-1 genotype.
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PMID:Viral hepatitis C. 1920 52


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