Abstract:

Tuberous sclerosis complex (TSC) is a multisystem autosomal dominant disease characterized by the development of benign neoplasia in the skin, brain and kidneys. There are three particular renal disorders in TSC including renal cysts, renal angiomyolipoma and renal cell carcinoma. In the current study we aimed to investigate renal findings of TSC patients followed in our clinic. Methods: Patients' family history, convulsion history, age, gender, physical examination findings, renal function tests, ultrasound and/or magnetic resonance imaging, results of computerized tomography, echocardiography and eye findings were found from hospital records and evaluated. Tuberous sclerosis diagnosis was made by clinical and imaging findings in 19 patients. Results: Nineteen cases were included in the study. Eight were male and the remaining eight were female. The average age was 75.5±65, 1 month (3 months- 18 years) and follow up time was 14.6±7 months. Renal angiomyolipoma was the most commonly seen pathology alone (4 patients-21%) and with renal cysts (5 patients-26.3%). Autosomal dominant polycystic kidney disease was with TSC in two patients. Four patients were presented with only simple renal cysts. Two patients had increased renal echogenicity and one patient had mild pelvicaliectasis. Ureteropelvic junction obstruction, urinary tract infection, nephrolithiasis and hemorrhages are commonly seen complications in TSC. Five patients had history of urinary tract infection. None of the patients had bleeding or rupture complication. Hypertension and end stage renal disease were not seen. The most commonly seen renal lesions in TSC are angiomyolipomas and kidney cysts. At the time of TSC diagnosis, all the children must be screened for renal involvement and we should remember renal findings can change with time and new findings can be added to old ones. Therefore Nephrologist follow up has been done in all patients

Keywords: