Hostomská L
Cesk Pediatr. 1989 Jun;44(6):322-6.
Nineteen diabetics aged 9 to 18 years with the MODY type were investigated, incl. their families, by the oGTT. Diabetes in the parents was nine times and in siblings four times more frequent than in families of adolescents with IDDM. In parents the manifest form predominated, in siblings PGT. Vertical transmission of diabetes in three consecutive generations was found only in the MODY type (in 35%). Diabetes with the MODY type and their diabetic siblings did not differ significantly as to their mild glucose intolerance (blood sugar level up to 13 mmol/l), and their mild diabetic phenotypes did not differ either. Similarly diabetics with IDDM and their diabetic siblings did not differ substantially as to their severe glucose intolerance (blood sugar level up to 21 mmol/l), and their severe diabetic phenotypes did not differ either. IRI levels revealed five times a hyperinsulinaemic and three times a normal insulinaemic response. Obese diabetics were treated with a reducing diet and physical activity. To non-obese diabetics, if the above procedure was not sufficiently successful, sulphonylurea preparation were also administered. During check-up examinations fasting values and values three hours after a meal lower than 6.1 mmol/l were required. In the course of a four- to ten-year follow up it did not change. Existence of the MODY type already macroangiopathic complications developed; in one diabetic the glucose tolerance improved, in the remainder it did not change. Existence of the MODY type already in adolescents justifies early detection in families with a cumulated incidence of NIDDM and prophylactic procedures ensuring euglycaemia in confirmed diabetics.
对19名9至18岁的MODY型糖尿病患者及其家属进行了口服葡萄糖耐量试验(oGTT)调查。父母患糖尿病的几率是IDDM青少年患者家庭的9倍,兄弟姐妹患糖尿病的几率是其4倍。父母中显性形式占主导,兄弟姐妹中则以糖调节受损(PGT)为主。仅在MODY型糖尿病中发现三代连续垂直传播(占35%)。MODY型糖尿病患者及其患糖尿病的兄弟姐妹在轻度糖耐量异常(血糖水平高达13 mmol/l)方面无显著差异,其轻度糖尿病表型也无差异。同样,IDDM糖尿病患者及其患糖尿病的兄弟姐妹在严重糖耐量异常(血糖水平高达21 mmol/l)方面也无实质性差异,其严重糖尿病表型也无差异。胰岛素释放指数(IRI)水平显示高胰岛素血症反应出现五次,正常胰岛素血症反应出现三次。肥胖的糖尿病患者采用节食和体育活动进行治疗。对于非肥胖的糖尿病患者,如果上述方法效果不佳,也会给予磺脲类制剂。在检查时,要求空腹值和餐后三小时的值低于6.1 mmol/l。在四到十年的随访过程中,情况没有改变。MODY型糖尿病患者已出现大血管病变并发症;一名糖尿病患者的糖耐量有所改善,其余患者则无变化。青少年中存在MODY型糖尿病,这证明在非胰岛素依赖型糖尿病(NIDDM)累积发病率较高的家庭中进行早期检测以及对确诊糖尿病患者采取确保血糖正常的预防措施是合理的。