Infants of diabetic mothers are at increased risk for the oculo-auriculo-vertebral sequence: A case-based and case-control approach.

摘要: Abstract Objectives: To determine if infants of diabetic mothers (IDM) are at increased risk for dysplastic ears and the oculoauriculo-vertebral spectrum (OAVS). Study design: Cases IDM with external seen Cedars-Sinai Medical Center were combined case series in medical literature describing similar patients. Data from a large congenital birth defects registry Spain analyzed, odds ratios (OR) born to either gestational or preconceptionally mother tohave one studied malformations calculated 95% confidence intervals. Results: Among 30 patients series, 50.0% (15) had hemifacial microsomia; 46.7% (14) hearing loss; 33.3% (10) facial nerve palsy; vertebral anomalies; 36.7% (11) cardiovascular defects, which 45% (5) conotruncal defects; 26.7% (8) renal 13.3% (4) limb (all radial ray hypoplasia); 10% (3) DiGeorge sequence; 6.7% (2) laterality imperforate anus. Within cases registry, ratio OAVS diabetes mellitus was 2.28 (95% CI, 1.03–4.82, P =.03), OR ear anomalies these 1.21 0.94–1.56, =.13). When diagnosed type 1 2 considered, 1.50 0.08-9.99, =.49), 0.94 0.48–1.81, =.85). Conclusions: Our data indicate that occurs higher incidence than general population. Associated problems include loss, athymia, cardiac, renal, malformations. Therefore, we recommend an features consistent undergo workup including evaluation, skeletal survey, echocardiogram, ultrasonogram, immunodeficiency clinically indicated. Furthermore, noting most occur structures neural crest origin, hypothesize poorly controlled maternal interferes cephalic cell migration. (J Pediatr 2002;141:611-7)