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Thrombotic microangiopathy following heart transplant in pediatric Danon disease
Author(s) -
Hummel Kevin,
Glotzbach Kristi,
Me Shaji,
Griffiths Eric,
Lal Ashwin K.
Publication year - 2020
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.13669
Subject(s) - medicine , thrombotic microangiopathy , schistocyte , angiopathy , cerebral edema , autopsy , brain herniation , complication , tacrolimus , surgery , thrombotic thrombocytopenic purpura , transplantation , disease , platelet , endocrinology , diabetes mellitus
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.