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We report a girl with the syndrome, manifesting left hydronephrosis, proteinuria\/hematuria, and focal segmental glomerular sclerosis (FSGS) resulting in chronic end\u2010stage renal failure, successfully treated with renal transplantation. Microarray comparative genomic hybridization showed the derivative chromosome 6 to have a 6.4\u2010Mb duplication at 6p25.3\u2013p25.1 with 32 protein\u2010coding genes and a 220\u2010Kb deletion at 6p25.3 with two genes of no possible relation to the renal pathology. Review of the literature shows that variation of renal complications in the syndrome is compatible with congenital anomalies of the kidney and urinary tract (CAKUT). FSGS, observed in another patient with 6p duplication syndrome, could be a non\u2010coincidental complication. <jats:italic>FOXC1<\/jats:italic>, located within the 6.4\u2010Mb duplicated region at 6p25.3\u2013p25.2, could be a candidate gene for CAKUT, but its single gene duplication effect would not be sufficient. FSGS would be a primary defect associated with duplicated gene(s) albeit no candidate could be proposed, or might occur in association with CAKUT. \u00a9 2015 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajmg.a.36942","type":"journal-article","created":{"date-parts":[[2015,2,18]],"date-time":"2015-02-18T06:39:36Z","timestamp":1424241576000},"page":"592-601","source":"Crossref","is-referenced-by-count":11,"title":["Renal complications in 6p duplication syndrome: Microarray\u2010based investigation of the candidate gene(s) for the development of congenital anomalies of the kidney and urinary tract (CAKUT) and focal segmental glomerular sclerosis (FSGS)"],"prefix":"10.1002","volume":"167","author":[{"given":"Megumi","family":"Yoshimura\u2010Furuhata","sequence":"first","affiliation":[{"name":"Department of Pediatrics Nagano Red Cross Hospital Nagano Japan"},{"name":"Department of Pediatrics Shinshu University School of Medicine Matsumoto 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