Turkiye Klinikleri J Med Sci 2012;32(2) 578 eliac disease (CD) is one of the most common chronic disorders en- countered during childhood. Its prevalence is reported as 0.5-1%. 1 Diagnosis of focal segmental glomerulosclerosis (FSGS) is based on the kidney biopsy findings. The classical lesion of FSGS is characterized by the presence of a scarring lesion in a segment of some, but not all glomeruli. 2 There are two forms of FSGS; idiopathic and secondary forms. Focal prolif- A Case of Celiac Disease with Focal Segmental Glomerulosclerosis Leading to End-Stage Renal Disease ABSTRACT In this case report a 5-year-old girl is presented who had Celiac disease and presented with nephrotic syndrome due to focal segmental glomerulosclerosis. Her intestinal biopsy which had been performed 2 years previously had revealed chronic duodenitis characterized by total villous atrophy and crypt hyperplasia. She presented with abdominal cramps, diarrhea, nausea, vomiting, failure to thrive and generalized edema while she was on gluten-free diet and in clinical remission for the disease. Kidney biopsy was performed considering the probable diagnoses of nephrotic syndrome and renal failure. The histopathological diagnosis was a cellular type focal segmentary glomerulosclerosis. A progressive deterioration could not be prevented in renal functions despite all therapeutic approaches including pulse methylprednisolone and cyclophosphamide administration. An end-stage renal disease (ESRD) developed within eight months, and the patient died due to the cardiovascular complications of ESRD 18 months after the initial presentation of renal findings. Very rare association of progressive glomerular disease and Celiac disease should be kept in mind during the follow-up of any child with Celiac disease Key Words: Renal insufficiency; celiac disease; glomerulosclerosis, focal segmental ÖZET Bu yazıda, çölyak hastalığı olan beş yaşında bir kız olguda nefrotik sendrom ile prezente olan fokal segmental glomerüloskleroz olgusu sunulmuştur. Olgunun iki yıl önceki intestinal biyop- sisinde total villus atrofisi ile birlikte kript hiperplazisi gözlenmiş idi. Hasta glutensiz diyet ile hastalık klinik remisyonda iken abdominal kramplar, diyare, bulantı, kusma, gelişme geriliği ve genel ödem ile başvurdu. Böbrek biyopsisi nefrotik sendrom ve böbrek yetmezliği ile uyumlu olarak rapor edildi. Histopatolojik tanı selüler tip fokal segmental glomerüloskleroz olarak sonuçlandı. Pulse metilprednizolon ve siklofosfamid dâhil yapılan tüm tedavi girişimlerine rağmen olgudaki il- erleme önlenemedi. Son dönem böbrek yetmezliği sekiz ay içinde gelişti ve hasta ilk başvurusun- dan 18 ay sonra son dönem böbrek yetmezliğinin kardiyovasküler komplikasyonlarından dolayı kaybedildi. Çölyak hastalıklı çocuklarda ilerleyici glomerüler hastalık ve çölyak hastalığı birlik- teliği akıldan çıkarılmamalıdır. Anahtar Kelimeler: Böbrek yetmezliği; çölyak hastalığı; glomerüloskleroz, fokal segmental Turkiye Klinikleri J Med Sci 2012;32(2):578-80 Emrah CAN, MD, Msc, a Ilmay BİLGE, MD, Prof., a Aydan ŞİRİN, MD, Prof., a Sevinç EMRE, MD, Prof., a Işın KILIÇASLAN, MD, Prof., b Semra SÖKÜCÜ, MD, Prof., c Veli UYSAL, MD, Prof. b Departments of a Pediatric Nephrology, b Pathology, c Pediatric Gastroenterology and Hepathology, İstanbul University Faculty of Medicine, İstanbul Geliş Tarihi/Received: 16.09.2010 Kabul Tarihi/Accepted: 16.06.2011 Yazışma Adresi/Correspondence: Emrah CAN, MD, Msc İstanbul University Faculty of Medicine, Department of Pediatrics, İstanbul, TÜRKİYE/TURKEY canemrahcan@yahoo.com doi:10.5336/medsci.2010-21128 Copyright © 2012 by Türkiye Klinikleri OLGU SUNUMU