Lecithin:Cholesterol Acyltransferase (LCAT) Deficiency: Renal Lesions with Early Graft Recurrence

Abstract

Familial lecithin:cholesterol acyltransferase (LCAT) deficiency is a rare metabolic disease with lipid deposition in several organs. The authors report a man with hypertension and proteinuria. Renal biopsy revealed glomerular changes, including peculiar thrombus-like deposits, consistent with LCAT deficiency. He was found to be compound heterozygous for two mutations of the LCAT gene. He received a kidney graft from his father. The authors also describe LCAT deficiency-related lesions in the explanted native kidneys and in biopsies at 2 days, 6 weeks, and 1 year after transplantation. The morphology of this disease is characteristic, and the diagnosis should be suspected from the ultrastructural findings.

KEYWORDS::

• Kidney
• LCAT disease
• Lecithin:cholesterol acyltransferase deficiency
• Nephrotic syndrome
• Renal transplant
• Ultrastructure

ACKNOWLEDGMENT

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.