Abstract:Hereditary angioedema (HAE) is rare; it is usually a monogenic, genetic disease with autosomal dominant transmission, resulting in C1-inhibitor deficiency (C1InH). Renal involvement associated with HAE has been known for nearly thirty years. We are reporting on two observations of a father and his daughter presenting a glomerulonephritis evolving towards to CKD class V. Renal needle biopsy had evidenced acute vascular lesions associated with segmental and focal hyalinosis. Since these lesions were not describe… Show more
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