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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  • 1. Mauras N, Merimee T, Rogol AD. Function of the growth hormone-insulin-like growth factor I axis in the profoundly growth-retarded diabetic child: Evidence for defective target organ responsiveness in the Mauriac syndrome. Metabolism 1991;40:1106-11.
  • 2. Kapellen TM, Galler A, Raile K, Kiess W. Complications and consequences. Pediatr Adolesc Med 2005;10:329-46.
  • 3. Alemzadeh R, Wyatt DT. Diabetes mellitus in children. In: Behrman RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. Philadelphia: Saunders; 2004. p.1947-72.
  • 4. Şimşek E, Kocabay K. Insulin-like growth factor (IGF-I) in Mauriac syndrome or diabetic dwarfism. Turk J Med Sci 2002;32:421-4.
  • 5. Cheantelau E, Eggert H, Seppel, Schönau E, Althaus C. Elevation of serum IGF-1 precedes proliferative diabetic retinopathy in Mauriac's syndrome. Br J Ophthalmol 1997; 81:168.
  • 6. Saka N. Diabetes mellitus. İçinde: Neyzi O, Ertugrul T, editörler. Pediatri. İstanbul: Nobel; 2002. p.1306-21.
  • 7. Chantelau E, Kohner EM. Why some cases of retinopathy worsen when diabetic control improves? BMJ 1997;315:1105-6.
  • 8. Van Steenbergen W, Lanckmans S. Liver disturbances in obesity and diabetes mellitus. Int J Obes Relat Metab Disord 1995;19Suppl 3:27-36.
  • 9. Lehman RM. Poorly controlled diabetes? Adolesc Med 1996;7:405-8.
  • 10. Beaufrere B, Francois R. Dwarfism and delayed puberty in a child with insulin-dependent diabetes mellitus Pediatrie 1988;43:39-42.
  • 11. Daneman D, Drash AL, Lobes LA, Becker DJ, Baker LM, Travis LB. Progressive retinopathy with improved control in diabetic dwarfism (Mauriac's syndrome). Diabetes Care 1981;4:360-5.
Genel Tıp Dergisi-Cover
  • ISSN: 2602-3741
  • Yayın Aralığı: Yılda 6 Sayı
  • Başlangıç: 1997
  • Yayıncı: SELÇUK ÜNİVERSİTESİ > TIP FAKÜLTESİ
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