Mauriac sendromu: Bir olgu sunumu
Amaç: Mauriac sendromu kötü kontrollü diyabet, belirgin gelişme geriliği ve hepatomegali triadı ile tanımlanır. Düşük insülinizasyon ile ilişkili olan bir sendromdur. Olgu sunumu: Bu yazıda dokuz yaşında Mauriac sendromu tanısı alan bir erkek hasta sunulmaktadır. Dört yıl önce tip 1 diyabetes mellitus tanısı alan hasta diyabetik ketoasidoz koması ile hastaneye kabul edildi. Dört yıldır 0.35 U/kg/G dozunda karışım insülin ve 1400 kcal diyabetik diyeti almaktaydı. Ağırlığı 23.7 kg (10–25. persentil), boy 107 cm (
Mauriac syndrome: A case report
Objective: Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation, and hepatomegaly. The syndrome is related to underinsulinization. Case report: In this case report, we present a nine-year-old boy diagnosed as Mauriac syndrome. He was admitted because of diabetic ketoacidosis. He had been followed-up with a history of type 1 diabetes mellitus for four years and treated by a two-dose insulin regimen. He had been taking mixture insulin with a dose of 0.35 units/kg/day, and was on a 1400 kcal diet for four years. His weight was 23.7 kg (10-25th percentile) and his height was 107 cm (<3rd percentile, -4.2 SDS). Physical examination revealed that he had protuberant abdomen, hepatomegaly, stage I pubertal development according to the Tanner grading system, and limited-joint mobility. Laboratory analyses were as follows: blood glucose 538 mg/dl, HbA1c 16.5%, blood ketones (+++) positive, urine ketones positive, and IGF-I 75 ng/mL. Microalbumin excretion rate in urine collected for twenty-four hours was 84 µg/min. The ketoacidosis was successfully treated by short-acting insulin infusion and he has followed-up with intensive insulin regimen. Nine months after the adjustment of his diet and insulin regimen, physical examination revealed that his height was 113 cm (<3 percentile; -3.7 SDS) and hepatomegaly had regressed. Blood glucose values over a nine months period were between 60 and 190 mg/dl, HbA1c 9.8%, and IGF-I 148 ng/ml. But microalbuminuria had persisted. Conclusion: Metabolic controls of diabetic patients should be done regularly and the patients should be followed up closely for development of possible complications.
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