1/266. Aphallia as part of urorectal septum malformation sequence in an infant of a diabetic mother. A male patient with aphallia, anal stenosis, tetralogy of fallot, multiple vertebral anomalies including sacral agenesis and central nervous system (CNS) malformations was born after a pregnancy complicated by poorly controlled maternal diabetes. Aphallia is an extremely rare abnormality and can be part of the urorectal septum malformation sequence (URSMS). While aphallia has not been reported in infants of diabetic mothers, urogenital malformations are known to occur with increased frequency. Two female products of pregnancies complicated by diabetes presented with multiple malformations including anal atresia and recto-vaginal fistula consistent with the diagnosis of URSMS. The three patients share CNS, cardiac, and vertebral anomalies, abnormalities secondary to abnormal blastogenesis and characteristic of diabetic embryopathy. URSMS is also caused by abnormal blastogenesis. Therefore, this particular malformation should be viewed in the context of the multiple blastogenetic abnormalities in the cases reported here. The overlap of findings of URSMS in our cases with other abnormalities of blastogenesis, such as VATER association or sacral agenesis is not surprising, as these associations are known to lack clear diagnostic boundaries. ( info) |
2/266. pregnancy outcome in aboriginal women with NIDDM in the Sioux Lookout Zone. PURPOSE: To review the pregnancy outcomes of Aboriginal women with non-insulin-dependent diabetes mellitus (NIDDM) in the Sioux Lookout Zone of Northwestern ontario, canada. METHOD: Retrospective chart review of deliveries of all women with a confirmed diagnosis of NIDDM was carried out between 1989 and 1992. RESULTS: During this period, 26 infants were born to 19 women with the diagnosis of NIDDM. Mean birth weight was 4,075 grams, with an average gestational age at delivery of 38 weeks. Three newborns required cesarean delivery, one required forceps, and one a vacuum extraction. There were four cases of shoulder dystocia. There were one stillbirth, one maternal death, and two cases of congenital heart disease. Ten newborns had neonatal jaundice and only two had neonatal hypoglycemia. These results suggest there is significant risk associated with NIDDM in pregnancy. ( info) |
3/266. Successful obstetric outcome after simultaneous pancreas and kidney transplantation. A 34-year-old woman became pregnant two years after having a simultaneous pancreas and kidney (SPK) transplantation, necessitated by type 1 diabetes and end-stage renal disease. The pregnancy was uneventful until 30 weeks' gestation, when she developed pancreatitis and a worsening of mild hypertension. A healthy 1700 g boy was delivered by caesarean section at 34 weeks' gestation. This is the first report of a successful pregnancy after SPK transplantation in australia. ( info) |
4/266. Hemodynamic concomitants of glycemia in diabetes mellitus: working hypothesis. This report illustrates a working hypothesis that proposes a simple-to-use, noninvasive hemodynamic system to provide myocardial contractility and arterial compliance patterns that may be clinically useful adjuncts to insulin and glycemic measurements in diabetes mellitus. This proposition is based on the concept that biochemical cellular milieu may only obliquely predict cellular function, whereas in the biophysical domain, it may be more clearly delineated. ( info) |
5/266. The management of hypertension in a diabetic pregnancy. Pregnancy in a woman with Type 1 diabetes poses several clinical challenges. In addition to meticulous glycaemic control, careful attention must be paid to the management of developing and pre-existing diabetic complications which may progress in severity during pregnancy. Pregnancy-induced hypertension is more common in women with diabetes and especially in those with diabetes of long duration. Diabetic renal disease is associated with hypertension which often deteriorates during pregnancy. The management of hypertension is difficult because of limited therapeutic options and the need to consider the implications for the developing fetus as well as the mother. This case report details the clinical management of a young woman with Type 1 diabetes whose pregnancy was complicated by the development of hypertension. ( info) |
| We encountered a pregnant woman with transient diabetes insipidus which developed during the third trimester. A hypertonic saline infusion study did not concentrate the osmolality of urine. Her laboratory data showed hypokalemia, hyperreninemia, an increased concentration of plasma aldosterone and an increased urinary excretion rate of prostaglandin E2, which resembled hyperprostaglandin E-syndrome. T1-weighted magnetic resonance imaging of the posterior pituitary gland revealed decreased intensity. polyuria reached 4-6 L daily, and urine osmolality remained dilute despite a lapse of four days since treatment with intranasal 1-desamino-8-D-arginine vasopressin (dDAVP: 10-25 microg every 12 h). The patient was conservatively managed without medical treatment, then delivered in the 38th week of pregnancy without complication. The osmolality of the patient's urine was higher than that of the plasma when tested 3 days postpartum. The abnormality of magnetic resonance imaging of the posterior pituitary gland disappeared at 6 months after delivery. We consider that subclinical nephrogenic diabetes insipidus in our patient was exacerbated during pregnancy. ( info) |
7/266. Spinal anaesthesia for caesarean section in a patient with systemic sclerosis. We describe the management of a diabetic primigravid woman with systemic sclerosis and thrombocytopaenia who required Caesarean section for pre-eclampsia. This was performed successfully under spinal anaesthesia. ( info) |
| Fetal bradycardia is a recognized response to maternal hypothermia but has not previously been reported in conjunction with diabetes. A 30-year-old insulin-dependent diabetic was admitted at 35 weeks gestation for control of her diabetes. She developed maternal hypothermia and hypoglycemia and the fetal heart rate fell to 100 beats per minute (b.p.m.). However, the fetal heart rate gradually returned to normal after rewarming the patient. ( info) |
9/266. Episodic hyperglycaemia in pregnant women with well-controlled Type 1 diabetes mellitus: a major potential factor underlying macrosomia. AIMS: To test the common assumption that pregnant women who are sufficiently motivated to achieve very good HbA1c levels will record home blood glucose data accurately. methods: A new device was used to download information from electronic blood glucose meters to assess the extent of selectivity in patient glucose diary-keeping. RESULTS: In an index case, a woman with excellent ambient HbA1c (5.9%; upper limit of normal 6.1%) was observed to have 68% of preprandial blood glucose readings above the target range of 3.5-6.5 mmol/l and a mean ( /- SD) level of 8.9 /-3.9 mmol/l in the corresponding period. No such impression was conveyed by the home monitoring diary. Six pregnant women with well controlled Type 1 diabetes (mean HbA1c 6.6 /-0.2%) exhibited between 42 and 68% of preprandial readings above the target range. CONCLUSIONS: The frequency of hyperglycaemia has hitherto been underestimated in well controlled pregnant women whose near-perfect home monitoring record is apparently corroborated by near-normal HbA1c levels. These observations provide a hypothesis for understanding of the disappointing continuance of macrosomia despite excellent HbA1c levels throughout pregnancy. ( info) |
10/266. Neonatal diabetes mellitus and cerebellar hypoplasia/agenesis: report of a new recessive syndrome. Classical neonatal diabetes mellitus is defined as hyperglycaemia occurring within the first six weeks of life in term infants. Cerebellar agenesis is rare. We report three cases of neonatal diabetes mellitus, cerebellar hypoplasia/agenesis, and dysmorphism occurring within a highly consanguineous family. This constellation of abnormalities has not previously been described. Two of these cases are sisters and the third case is a female first cousin. The pattern of inheritance suggests this is a previously undescribed autosomal recessive disorder. prenatal diagnosis of the condition in this family was possible by demonstration of the absence of the cerebellum and severe IUGR. ( info) |