Unilateral localized cystic kidney: Answer
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Priya Verghese & Youngki Kim
- چاپ و سال / کشور: 2011
Description
ADPKD is the clinical manifestation of a genetic defect that is typically thought to be a bilateral renal cystic disease of adults, since children are most often asymptomatic [3]. There are numerous reports of ADPKD presenting in childhood and even in utero [4]. Most of these children have bilateral renal cystic disease, but 17% have been identified with an initial presentation of a unilateral cystic kidney [5, 6]. We present a teenage patient who had a worrisome presentation of a multicystic lesion in the upper pole of his left kidney. Asymmetry of renal involvement has been reported in ADPKD, but our patient had localized tumor-like cysts in one pole of the left kidney with no cysts in the other kidney, which to our knowledge has never been reported before. There is a report of a patient with ADPKD who had a similar multicystic segmental kidney, but that patient had bilateral cystic kidney disease [7]. Despite a negative family history for cystic kidney, imaging of the parents kidneys was essential in allowing us to make the diagnosis of ADPKD in this young man. Due to the timely diagnosis, the patient was spared a surgical procedure with the associated morbidity and mortality. We therefore recommend that the diagnosis of entities such as the asymmetric variant of ADPKD be entertained before surgical management of multicystic segmental renal lesions.
Pediatr Nephrol (2011) 26:715–716 DOI 10.1007/s00467-010-1622-y Received: 26 April 2010 / Revised: 4 June 2010 / Accepted: 29 June 2010 / Published online: 21 August 2010
