Abstract
Our case report is the only reported case in literature of transplanting a black coloured kidney successfully from a albino donor. Oculocutaneous albinism is similar to hermansky–pudlak syndrome and chediak higashi syndrome and could lead to more widespread lysosome excretory defects. These defects could lead to accumulation of some intra cellular material, leading to the gross discolouration of the kidney. Hermansky pudlak syndrome is a triad of albinism, platelets lacking dense bodies and storage of ceroid like material in tissues. The manifestations of storage disease are variable and include granulomatous colitis, restrictive lung disease, kidney failure and cardiomyopathy. The autofluorescent material stored in the hermansky pudlak syndrome contains dolichols. Our patient was a living related donor who was having oculocutaneous albinism. Her kidneys were normal functioning but on exposure was black coloured. Post transplant the graft showed ATN and parenchymal dysfunction which later recovered.
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More From: Journal of Transplantation Technologies & Research
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