Son Dönem Böbrek Yetmezliğine İlerleyen Fokal Segmental Glomerülosklerozlu Bir Çölyak Hastalığı Olgusu

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