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Internist (Berl). 2016 Oct;57(10):1022-1028.

[Hemolytic kidney failure and transient ischemic attack in a 32-year-old female].

[Article in German]

Author information

1
Department für Innere Medizin, Neurologie und Dermatologie, Klinik für Endokrinologie/Nephrologie, Universitätsklinikum Leipzig, Liebigstr. 20, 04103, Leipzig, Deutschland.
2
Bioscientia, Zentrum für Humangenetik, Ingelheim, Deutschland.
3
Department für Innere Medizin, Neurologie und Dermatologie, Klinik für Endokrinologie/Nephrologie, Universitätsklinikum Leipzig, Liebigstr. 20, 04103, Leipzig, Deutschland. jan.halbritter@medizin.uni-leipzig.de.

Abstract

We report on the case of a 32-year-old female patient who initially presented with oliguric acute renal failure, hemolytic anemia with moderate thrombocytopenia and subsequently developed a transient ischemic attack in the cerebellum. The kidney biopsy revealed clinically suspected atypical hemolytic-uremic syndrome (aHUS), which was confirmed by intraglomerular thrombotic microangiopathy (TMA). Treatment with plasmapheresis and sustained administration of the C5 inhibitor eculizumab resulted in hematological remission but without improvement of kidney function. Further etiological investigations led to reduced plasma levels of inhibitory complement factor I on the basis of a heterozygous CFI mutation. In patients with aHUS molecular genetic investigations are indicated in order to determine the underlying cause, to regulate the therapeutic regimen and to allow prognostic statements with respect to a potential kidney transplantation.

KEYWORDS:

Atypical hemolytic uremic syndrome; Complement pathway; Eculizumab; Kidney failure; Thrombotic microangiopathy

PMID:
27357251
DOI:
10.1007/s00108-016-0092-0
[Indexed for MEDLINE]

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