Şenay Savaş ERDEVE, Enver ŞİMŞEK, Yıldız DALLAR

Tip 1 diyabetli bir vakada kötü metabolik kontrolün sonucu: Mauriac sendromu ve eklem hareket kısıtlığı sendromu

Mauriac sendromu kötü metabolik kontrollü diyabet hastalarında büyüme genliği ve karaciğer büyüklüğü ile karakterize bir durum olup, metabolik kontrolün düzeltilmesiyle geri dönüşümlü olabilir. Bu yazıda sekiz yıllık kötü metabolik kontrol sonrası eklem hareket kısıtlılığı ile birlikte Mauriac sendromu gelişen on bir yaşında tip 1 diyabetli bir kız vaka sunulmaktadır. Vakanın ağır büyüme geriliği mevcuttu [tartı: 21 kg (-2.8 SDS), boy: 117 cm (-4.7 SDS)]. Karın öne doğru şişkindi ve karaciğer midklaviküler hatta 4 cm palpe ediliyordu. Dua bulgusu denen ellerin palmar yüzeylerinin tam olarak karşılıklı getirilememesi mevcuttu. Hastanın göz muayenesinde katarakt saptandı. Diyette kalori alımı yeniden düzenlendi ve yoğun insülin tedavisiyle kan glukoz ve hemoglobin Alc (HbAlc) değerlerinde belirgin düzelme oldu. Büyüme hızı 5 cm/yıla çıktı ve karaciğer büyüklüğü 2 cm'e indi. El bulgularında düzelme olmadı. Mauriac sendromu büyüme geriliği ve karaciğer büyüklüğü gelişen tip 1 diyabetli çocuklarda akla gelmelidir.

Anahtar Kelimeler:

İnsülin, Vücut ağırlıkları ve ölçüleri, Çocuk, Diabetes mellitus, tip 1, Kadın, Büyüme bozuklukları, Hepatomegali, Sendrom, Tedavi sonucu

A Result of poor metabolic control in a case of type I diabetic patient: Mauriac syndrome and limited joint mobility

Mauriac syndrome is a condition which is characterized by growth failure and hepatomegaly in diabetic patients with poor metabolic control, and may be reversible with improvement of metabolic control is presented. Her weight and height were 21 kg (-2.8 SDS) and 117 cm (-4.7 SDS). Abdomen was protuberant and liver was 4 cm palpable at midclavicular line. There was a failure of opposition of the palmar surfaces of the hands which is called prayer sign. Cataract was determined on eye examination of the patient. Her dietary caloric intke was rearranged and blood glucose and hemoglobin Alc values significantly improved by intensive insulin therapy. Growth velocity increased to 5 cm/year and hepatomegaly decreased to 2 cm. The signs of hand didn't recovered. Mauriac syndrome should be considered in type 1 diabetic children with growth failure and hepatomegaly.

Keywords:

Insulin, Body Weights and Measures, Child, Diabetes Mellitus, Type 1, Female, Growth Disorders, Hepatomegaly, Syndrome, Treatment Outcome,

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