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 62-year-old female patient presented with bullous, intensely itching cutaneous lesions, which clinically and histopathologically resembled dermatitis herpetiformis (Duhring's disease). Therapy with DADPS was unsuccessful. Because of associated cervical lymph node enlargement and splenomegaly, a lymph node biopsy was taken. Histopathology of a lymph node and of the bone marrow confirmed the diagnosis: angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma. Intensely pruritic associated skin eruptions are typical for this peculiar kind of lymphoma. These skin lesions are due to inflammatory cells and not to neoplastic infiltrations. This case report is the first report of AILD with bullous skin lesions to appear in the literature.
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PMID:[Angioimmunoblastic lymphadenopathy accompanied by Duhring disease-like lesions ]. 796 Jul 58

Diaminodiphenyl sulphone (dapsone) is a drug of choice in the treatment of leprosy. It is also useful for the treatment of many neutrophilic and other dermatoses. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato-splenomegaly. Twenty-six patients with dapsone hypersensitivity syndrome were studied for clinical profile, outcome, and prognosis. The male:female ratio was 2.2:1, and the mean age was 33.19 years (range 13 to 64 years). The interval between start of dapsone therapy and appearance of symptoms varied from 2-7 weeks (mean 29.82 days). Twenty-four patients received dapsone as a part of multi-drug therapy for leprosy; the other two patients received dapsone for lichen planus and acne vulgaris. Exfoliative dermatitis was the most common cutaneous manifestation followed by erythematous maculo-papular eruption and Stevens-Johnson syndrome-like lesion. The other common systemic manifestations were: fever (26 cases), itching (22 cases), lymphadenopathy (21 cases), jaundice (21 cases), pallor (20 cases), hepatomegaly (19 cases), and pedal edema (14 cases). Investigation profile revealed elevated levels of serum liver enzymes in 100% of patients, elevated erythrocyte sedimentation rate in 92.3%, raised bilirubin in 84.6%, leucocytosis in 69.23%, low hemoglobin (<9 gm/dl) in 46.15% and hypoproteinemia in 42.3%. Eosinophilia, hemolytic anemia, and reticulocytosis count were found in 4 patients each. All the patients had favorable outcomes except three who died due to hepatic failure. Medical personnel must be aware of this potentially fatal syndrome, because it can cause considerable morbidity and mortality.
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PMID:Dapsone hypersensitivity syndrome: a clinico-epidemiological review. 1636 48

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

Epidemics of Dengue fever (DF) and Dengue hemorrhagic fever (DHF) are common in Southeast Asia. DF is defined & classified according to WHO criteria. Variable clinical manifestations of DF & DHF have been described in earlier studies. But some patients present with unusual clinical features and clinical profile not classifiable according to the present WHO criteria. Some of these complications if not recognized early and treated properly can even prove fatal. So this study was done to describe various clinical features in Dengue fever with special emphasis on unusual manifestations. This study was conducted at University College of Medical Sciences and associated Guru Teg Bahadur hospital; a tertiary care hospital, located in East Delhi in India. It was a retrospective study of 76 patients of probable DF; including 4 cases of DHF (according to WHO classification); 60 males & 16 females above 12 years of age admitted in medical wards of Guru Teg Bahadur hospital in an outbreak of DF which occurred during September-December 2009. The data obtained was analyzed to see clinical and laboratory profile of DF/DHF with special emphasis on unusual manifestations. The mean age of the patients was 28 +/- 9.6 years. Fever was present in all the cases with an average duration of fever being 5.47 +/- 2.2 days with body ache, (84.2%), vomiting (61.8%), abdominal pain (51.3%) and headache (19.7%) being the other presenting complaints. Hemorrhagic manifestations in the form of gum bleeding and epistaxis (35.5%), positive tourniquet test (27.6%); skin rashes (15.8%), melena (15.8%) and hematemesis (5.26%) were also present. In our study a fair no of patients presented with unusual symptoms like pain in abdomen 39 (51.3%), nausea 32 (42.1%), & vomiting 47 (61.8%), which is higher than that reported previous outbreak. Of the 39 patients who presented with abdominal pain; ultrasonography of abdomen was done in 25 patients. Fifteen (38%) of these were found to have acalculous cholecystitis. Amongst the known manifestations of DF, abdominal pain has been well described, but acalculous cholecystitis as a cause of abdominal pain is scantly reported. Another unusual manifestation was the presentation of patients (7.89%) in circulatory failure without the evidence of plasma leakage and not fulfilling all the criterion proposed by WHO for DHF/DSS. All of the above patients had very low platelet counts & tourniquet test was positive in all these six patients. Hepatomegaly and splenomegaly were observed in 34.2% and 7.89% of cases, respectively. Renal dysfunction was observed in 13.1% of cases. Laboratory investigations revealed thrombocytopenia (with a platelet count of < 100,000/microl) in all cases. Leucopenia (WBC < 4,000/mm2) and Hemoconcentration (Hct > 20% of expected for age and sex) were found in 38% and 5.26% of the cases, respectively. Results of our study indicate that apart from usual manifestations, sometimes unusual but clinically extremely important manifestations can occur which if not detected early can prove fatal. So a vigilant and timely approach is warranted.
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PMID:Unusual manifestations in dengue outbreak 2009, Delhi, India. 2247 Nov 94