ABSTRACT
The relationship between the level of hemoglobin A1 (Hb A1) in the first trimester and major malformations and spontaneous abortions was examined in 303 insulin-requiring diabetic gravidas. During the study period, all patients with insulin-requiring diabetes mellitus antedating pregnancy who registered for prenatal care prior to 12 weeks' gestation and who had a known outcome were included. Thirty-five percent of the patients entered with a first-trimester Hb A1 of greater than 11.0% of total hemoglobin (9 standard deviations above the mean for a nondiabetic population). A broad spectrum of glycemic control was therefore represented. The risk of spontaneous abortion was 12.4% with first-trimester Hb A1 less than or equal to 9.3% and 37.5% with Hb A1 greater than 14.4% (risk ratio 3.0; 95% confidence interval 1.3-7.0). The risk for major malformation was 3.0% with Hb A1 less than or equal to 9.3% and 40% with Hb A1 greater than 14.4% (risk ratio 13.2; 95% confidence interval 4.3-40.4). Although the risks for both adverse outcomes were markedly elevated following a first trimester in very poor metabolic control, there was a broad range of control over which the risks were not substantially elevated. To keep malformations and spontaneous abortions to a minimum among diabetic women does not require "excellent" control; there seems to be a fairly broad range of "acceptable" control.
Subject(s)
Abortion, Spontaneous/etiology , Congenital Abnormalities/etiology , Glycated Hemoglobin/metabolism , Pregnancy in Diabetics/blood , Female , Humans , Pregnancy , Pregnancy Trimester, First , Pregnancy in Diabetics/complications , Prospective Studies , Risk FactorsSubject(s)
Congenital Abnormalities/etiology , Glycosides/analysis , Hemoglobin A/analogs & derivatives , Pregnancy in Diabetics/blood , Diabetic Angiopathies , Female , Hemoglobin A/analysis , Humans , Insulin/therapeutic use , Pregnancy , Pregnancy Trimester, First , Pregnancy in Diabetics/drug therapySubject(s)
Fetal Diseases/etiology , Infant, Newborn, Diseases/etiology , Pregnancy in Diabetics/complications , Birth Weight , Carbohydrate Metabolism , Congenital Abnormalities/etiology , Delivery, Obstetric , Diabetic Angiopathies/etiology , Female , Fetal Death/epidemiology , Fetal Death/etiology , Fetal Monitoring , Gestational Age , Humans , Infant Mortality , Infant, Newborn , Insulin/therapeutic use , Labor, Obstetric , Pregnancy , Pregnancy Complications , Pregnancy in Diabetics/therapy , Respiratory Distress Syndrome, Newborn/etiologyABSTRACT
Study of a 4 1/2-year-old boy with the unusual combination of acute infantile hemiplegia, ectopia lentis and the absence of homocystinuria showed large amounts of abnormal sulfur-containing metabolites (sulfite, thiosulfate and S-sulfocysteine) in the urine. Sulfite and S-sulfocysteine were also present in the plasma. His inorganic sulfate excretion was only 50 per cent of total sulfur, as compared with 75 to 95 per cent by controls. Loading with L-cysteine hydrochloride and L-methionine further increased the excretion of sulfite and thiosulfate, but not inorganic sulfate excretion. Sulfite oxidase activity in skin fibroblasts average 1.07 nmol of cytochrome d reduced per milligram of protein per minute in control lines; it was not detectable (less than 5 per cent) in the patient. Activity was reduced in both parents (0.50 in the father and 0.32 in the mother)--compatible with autosomal recessive inheritance. Good biochemical responses to a low sulfur amino acid diet suggest that early treatment may benefit the patient.
