Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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