Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dapsone has been used since 1976 in the treatment of relapsing polychondritis. A critical analysis of its therapeutic effectiveness based on 2 personal cases and 14 cases reported in the literature, all treated with dapsone alone, showed that treatment had to be discontinued in 4 on account of side effects (haemolytic anaemia, erythema multiforme, somnolence, headache, nausea); 1 patient showed no improvement and 5 relapsed; 6 responded favourably and without relapse during a 3 months' to 4 years' follow-up. Considering the unpredictable course of relapsing polychondritis and the fact that some of its clinical manifestations, notably auricular chondritis, may spontaneously resolve, the response of the disease to dapsone is difficult to establish and requires to be confirmed by a controlled clinical trial.
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PMID:[Chronic atrophic polychondritis. Critical analysis of the therapeutic efficacy of dapsone. 2 cases]. 623 76

This case describes an uncommon acute complication of diffuse thrombotic angiopathy and associated aHUS/TTP in an 11-year-old girl with Danon disease who underwent orthotopic heart transplant. Shortly after transplant, despite an uncomplicated operative course, the patient developed severe kidney injury and progressive altered mental status, culminating in cerebral edema, brain herniation, and death. She had received a single dose of tacrolimus (FK506) and a single dose of antithymocyte globulin. Sources of progressive somnolence, including oversedation from impaired renal clearance of opiates, and severe myopathy as has been previously described in Danon disease, were ruled out, and the patient continued to decline. Initial brain CT scan early after transplant showed no signs of cerebral edema, but repeat CT indicated severe cerebral edema. Based on autopsy, diffuse thrombotic angiopathy, with signs of hemolytic anemia with schistocytes, was likely responsible for her deterioration in the broader condition of aHUS/TTP.
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PMID:Thrombotic microangiopathy following heart transplant in pediatric Danon disease. 3206 23