Schwaibold H, Wittekind C, Schillinger H, Böhm N
Sektion Kinderpathologie am Pathologischen Institut (Ludwig-Aschoff-Haus).
Klin Padiatr. 1988 Mar-Apr;200(2):135-9. doi: 10.1055/s-2008-1033700.
The diabetic embryopathy syndrome comprises a number of developmental anomalies among fetuses of diabetic mothers. Fetopathia diabetica, on the other hand, is characterized by typical, hormonal, and metabolic dysfunctions and their morphological sequelae in fetuses and offsprings of diabetic mothers. We observed the combination of both these conditions in an immature stillborn fetus. The 34 year-old diabetic mother, who had been treated by insulin since age 16, was first seen at 27 weeks of gestation. Sonography revealed severe congenital malformations of the fetus, and a late abortion was induced. The stillborn female revealed the typical congenital malformations of the diabetic embryopathy syndrome, such as abnormalities of face and skull, skeletal malformations of the thorax, spine, and lower extremities, and malformations of the heart, great vessels and the genitourinary system. We, too, found the characteristic features of fetopathia diabetica, i.e. obesity, macrosomia, increased weight and size of the internal organs, polynesia and macronesia of the pancreas, increased extramedullary hematopoiesis and cellular depletion of lymphoid tissues. A review of the literature revealed various hypotheses about the etiology and pathogenesis of both conditions.
糖尿病胚胎病综合征包括糖尿病母亲所怀胎儿中的多种发育异常。另一方面,糖尿病胎儿病的特征是糖尿病母亲所怀胎儿及后代出现典型的激素和代谢功能障碍及其形态学后果。我们在一个不成熟的死产胎儿中观察到了这两种情况的合并存在。这位34岁的糖尿病母亲自16岁起就接受胰岛素治疗,在妊娠27周时首次就诊。超声检查显示胎儿有严重的先天性畸形,于是引产。死产女婴显示出糖尿病胚胎病综合征的典型先天性畸形,如面部和颅骨异常、胸部、脊柱和下肢骨骼畸形,以及心脏、大血管和泌尿生殖系统畸形。我们还发现了糖尿病胎儿病的特征性表现,即肥胖、巨大儿、内脏器官重量和大小增加、胰腺多发胰岛和大胰岛、骨髓外造血增加以及淋巴组织细胞减少。文献综述揭示了关于这两种情况的病因和发病机制的各种假说。