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 total of eighty-one consecutive cases of Kala-azar admitted in all four medicine units of Mymensingh Medical College Hospital during the period from January 2002 to mid August 2002 were included in this study. The number of the patients clearly indicates that the burden of Kala-azar in this region is significant and expanding, which constituted 1.90% of total admission in all 4 medicine units during this period. Majority of the patients were of 20-29 years of age. Male to female ratio was 1.38:1. Maximum number of the patients were of poor socio-economic group with history of housing made up of mud and having close proximity with cattle house. Fever and splenomegaly (100%) were the predominant features. Hepatomegaly was found in 91.36% of the cases. Other clinical manifestations were weight loss (79.01%), normal or increased appetite (65.43%), generalized weakness (72.84%), pallor (69.13%), cough (25.92%), jaundice (17.28%), abdominal Pain (12.34%), hyperpigmentation (9.88%), ascites (4.94%) and bleeding manifestations (4.94%). Notable concomitant illnesses were urinary tract infection (7.40%), pulmonary tuberculosis (3.70%), malaria (1.23%), scabies (4.94%), heart failure (3.70%) and chronic liver disease (2.47%). Due to wide diversity of clinical presentations, clinical features of kala-azar should be evaluated in details which will pave the hidden cases into light.
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PMID:Clinical profile of Kala-azar in adults: as seen in Mymensingh Medical College Hospital, Mymensingh, Bangladesh. 1271 42

Leishmanioses are widespread in 88 countries of the tropical and subtropical zone, including regions of the Mediterranean Sea basin of Southern Europe. Actually, approximately 350 million of people live in Leishmania endemic areas and about 12 million of individuals are infected. Visceral leishmaniosis (kala-azar disease, tropical splenomegaly) is caused by at least 3 species of Leishmania protozoa: L. donovani, L. infantum and L. chagasi. The incidence of the disease is estimated at 500,000 new cases annually. The infection is transmitted by Phlebotomus or Lutzomyia mosquitos bites, in which intestines forms invasive to humans are developed. Leishmania spp. have a predilection to the reticulo-histiocytary system cells, leading to their proliferation and disruption, and after spreading to the circulation they invade spleen, liver and bone marrow. Visceral leishmaniosis should be suspected in travelers returning from tropical and subtropical areas with signs of splenomegaly and twice temperature spikes in a day. We reported a case of the kala-azar disease in the 22 year-old Polish patient seasonally working in Italy. The clinical picture was expressed by two daily pikes of fever proceeded by chills, excessive sweat, hepatosplenomegaly, lymphadenopathy, general weakness, abdominal pain and nausea. The Leishmania infection was complicated by candidiosis. Laboratory tests showed anaemia, thrombocytopenia, leucopenia, hypergammaglobulinaemia and a suppression of immunological cellular response. The diagnosis was confirmed by a presence of amastigota forms in macrophages of the bone marrow aspirate and a detection of specific antibodies to L. infantum by Westernblotting. The patient was successfully treated with Glucantime.
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PMID:[Visceral leishmaniasis]. 1291 Jun 4

A 30-year-old homosexual man presented with anemia and a several months history of recurrent fever, night sweats and weakness. His travel history included several stays in mediterranean countries during the recent years. Abdominal ultrasound showed massive splenomegaly, hepatomegaly and abdominal lymphadenopathy. A bone marrow aspirate revealed the presence of numerous Leishmania amastigotes, and bone marrow culture and polymerase chain reaction were also positive for Leishmania. In this case report epidemiological, immunological, diagnostic and therapeutic aspects of HIV-Leishmania coinfection are discussed with special emphasis on the impact of liposomal amphotericin B and highly active antiretroviral therapy on the treatment of HIV-leishmania-coinfection.
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PMID:[HIV positive patient with pancytopenia and massive splenomegaly]. 1467 17

Hereditary elliptocytosis (HE) is a common disorder of erythrocyte shape, occurring especially in individuals of African and Mediterranean ancestry, presumably because elliptocytes confer some resistance to malaria. The principle lesion in HE is mechanical weakness or fragility of the erythrocyte membrane skeleton due to defects in alpha-spectrin, beta-spectrin, or protein 4.1. Numerous mutations have been described in the genes encoding these proteins, including point mutations, gene deletions and insertions, and mRNA processing defects. Several mutations have been identified in a number of individuals on the same genetic background, suggesting a "founder effect." The majority of HE patients are asymptomatic, but some may experience hemolytic anemia, splenomegaly, and intermittent jaundice.
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PMID:Hereditary elliptocytosis: spectrin and protein 4.1R. 1507 91

From February, 1981 to May, 2001, 63 children under 15 y old (ages 2 - 15 y, median = 8 y, mean +/- 1 SD = 8 +/- 3 y) presenting 70 episodes of Paracoccidioidomycosis were admitted. The main clinical manifestations and laboratory features observed upon admission were: lymph node enlargement (87.1%), fever (75.7%), weakness (48.6%), pallor (41.4%), hepatomegaly (40%), splenomegaly (35.7%), anemia (90%), hypergammaglobulinemia (88.5%), eosinophilia (75.5%) and hypoalbuminemia (72.5%). Moderate to severe malnutrition was detected in 35.7% of the episodes (Gomez's criterion). Radiographic and technetium studies showed bone lesions in 20 of the episodes, most of them being multiple lytic lesions, involving both long (70%) and plain bones (30%). First line treatment consisted of an association of sulfametoxazole-trimethoprin, which was used, exclusively, in 50 episodes. Follow-up of hemoglobin levels, number of eosinophils in the peripheral blood, albumin and gammaglobulin serum levels revealed significant sequential improvement one and six months after hospital admission, being quite useful to evaluate treatment effectiveness. Six patients died (9.3%) and four developed sequelae (6.3%). In conclusion, the juvenile and disseminated forms can be observed in about 70% of the episodes of PCM occurring in children younger than 15 y old, most of them presenting with a febrile lymphoproliferative syndrome associated to anemia, eosinophilia and hypergammaglobulinemia.
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PMID:Paracoccidioidomycosis in children: clinical presentation, follow-up and outcome. 1528 12

A middle-aged man presented with progressive dyspnea and a pleural effusion. He had slowly progressive bilateral symmetric extremity weakness, mediastinal lymphadenopathy, and splenomegaly. The diagnosis of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein and Skin changes was suspected clinically and confirmed with a bone marrow biopsy. He responded symptomatically to corticosteroids and is awaiting a stem cell transplant.
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PMID:POEMS syndrome. 1538 41

The aim of the study was to the clinical features and long term follow up after treatment with Cladarbine in a tertiary care hospital. Seven patients with hairy cell leukemia were diagnosed between January 1990 till December 2003. Diagnosis in all the patients was established by bone marrow aspirates and trephine biopsy along with TRAP. In two patients the diagnosis was supplemented by flowcytometry and in another two patients by splenectomy. Six patients were male while one was female. Mean age was 47.7 years (range 36-64). Most common presenting features were pallor and weakness (n=5). All patients had splenomegaly. Blood count at presentation revealed that one patient had bicytopenia, two had isolated thrombocytopenia, and three had pancytopenia. Treatment responses were evaluable in seven patients. Complete response was seen in six patients (85.7%). One patient died after two months due to sepsis while 3 (50%) patients relapsed. Those who relapsed received another course of CDA and have maintained remission with a median duration of response of 48 months (20-48). From this small series we can conclude that CDA is an effective treatment for HCL and even it works very well in relapsed cases.
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PMID:Hairy cell leukemia: clinical presentation and long term follow up after treatment with 2-chlorodeoxyadenosine (2-CdA). 1596 Feb 89

T- cell Prolymhocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy that is usually reported in the elderly and follows an aggressive course. A 68 year old male presented with a history of weakness and weight loss of two months duration. Clinical examination revealed pallor, enlarged cervical and axillary lymph nodes and splenomegaly. He also had a maculo- papular skin rash. There was marked leucocytosis, anemia and thrombocytopenia (WBC 445 x 103 sub/ml, Hb 8.5 gm/dl, Platelet 25 x 103 sub/microl) with 60% prolymphocytes in the peripheral blood. Bone marrow was hypercellular with an excess of prolymphocytes. Flow cytometric analysis of the bone marrow showed positivity for CD2, CD3, CD4, CD5 and CD 7. T- PLL is a rare T cell disorder with characteristic clinical and laboratory features. Currently, no optimal treatment exists although there has been some success with 2'- deoxycoformycin or Campath-1H.
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PMID:T- cell prolymphocytic leukemia - a rare case. 1614 12

A 74-year old woman had been suffering from chronic idiopathic myelofibrosis (CIMF) for three years before noticing skin rash and subsequent muscle weakness. On admission, purplish and erythematous skin rash was seen spreading over large parts of her body, including the face, chest, back and extremities. She could not stand up without assistance due to weakness. On laboratory examination, her hemoglobin was found to be 11.8g/dl, platelet 17,000/microl, WBC 22,500/microl (with blast cells), and CK 1,757 IU/I. Auto-antibodies including antinuclear antibody, Jo-I antibody and rheumatoid factors were negative. Abdominal CT revealed giant splenomegaly. She was diagnosed as having dermatomyositis (DM) associated with CIMF. Although administration of prednisolone followed by methyl-prednisolone pulse therapy ameliorated the weakness and skin rash, WBC increased to 35,000/ microl. In case of worsening of CIMF, azathioprine (AZP) administered. This decreased the WBC count to 13,700/microl and the CK to 49 IU/I 40 days after the administration. Patients with CIMF have an increased incidence of complications of other autoimmune diseases, indicating that the immunological mechanism plays some roles in the progression of the disease. There has been only one other reported case of DM complication. This case suggests usefulness of AZP treatment combined with prednisolone in these patients.
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PMID:[A case of dermatomyositis associated with chronic idiopathic myelofibrosis]. 1664 32

Two patients, a 36-year-old female and a 36-year-old male, separately experienced new onset nausea, vomiting, diarrhea, abdominal pain, muscle weakness and pallor. Over a period of 14-16 h these symptoms continue and progress to include hypotension refractory to therapy, pulmonary edema and cardiovascular collapse. Autopsies show hemorrhagic pulmonary edema, splenomegaly and lack of anatomical cause for sudden death. Postmortem analysis, in one case post-embalming and exhumation, revealed elevated selenium concentrations and a determination of the cause of death. These two cases present several important features associated with selenium toxicity, two of which are previously unreported: (1) selenium as a potential homicidal agent, (2) the toxidrome and time frame of selenium toxicity, (3) selenium determination in exhumed, embalmed tissues, (4) postmortem urinary selenium concentration, and (5) decrease in tissue concentrations over time.
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PMID:Two fatal cases of selenium toxicity. 1689 Oct 71


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