ISSN: 1305-385X
Hakkında: Özel sayılar şeklinde yayınlanır.
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Medullary Cystic Kidney Disease Andmedullar Sponge Kidney
Dr. Serhan Z. TUĞLULARa
aİç Hastalıkları AD, Nefroloji BD, Marmara Üniversitesi Tıp Fakültesi, İSTANBUL
Medullary cystic kidney disease (MCKD) is an inherited kidney disease characterized by development of corticomedullary cysts leading to impairment of tubular structure and function. MCKD, leads to end-stage renal disease (ESRD), most frequently, in adulthood. Two different genetic loci have been described for MCKD (MCKD1 and MCKD2). Diagnosis is made through a definitive family history and clinical findings in addition to imaging of the cysts. Although MCKD has several similarities with with nephronoftizis, they differ by genetic features, time to ESRD and concomitant extrarenal findings. MCKD has also been associated with familial juvenile hyperuricemic nephropathy (FJHN). Medullary sponge kidney is a renal disorder characterized by structural dialatation of papilalry collecting tubules thought to be caused by a developmental defect. It is generally asymptomatic but urolithiasis secondary to hypercalciuria and recurrent urinary infections are the main long term complications.Keywords: Medullary cystic kidney disease, medullary sponge kidneyTurkiye Klinikleri J Int Med Sci 2005, 1(4):52-55
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