1/15. 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.- - - - - - - - - - ranking = 1keywords = diabetes mellitus, diabetes (Clic here for more details about this article) |
2/15. Neonatal diabetes mellitus and neonatal polycystic, dysplastic kidneys: Phenotypically discordant recurrence of a mutation in the hepatocyte nuclear factor-1beta gene due to germline mosaicism.Mutations in the gene coding for hepatocyte nuclear factor-1beta (HNF-1beta) have been known to cause a form of maturity-onset diabetes of the young (MODY5), which is usually characterized by dominantly inherited adolescence-onset diabetes mellitus associated with renal cysts. This report, however, describes recurrence of a novel missense mutation in the HNF-1beta gene, S148W (C443G), in two sibs, one with neonatal diabetes mellitus and the other with neonatal polycystic, dysplastic kidneys leading to early renal failure. The former patient had only a few small renal cysts with normal renal functions, and the latter had only a transient episode of hyperglycemia, which resolved spontaneously. Interestingly, both parents were clinically unaffected, and PCR restriction fragment length polymorphism analysis showed that the mother was a low-level mosaic of normal and mutant HNF-1beta, which suggested that the recurrence was caused by germline mosaicism. This is the first report of permanent neonatal diabetes mellitus caused by a mutation of the HNF-1beta gene as well as the first report of germline mosaicism of this gene. In addition, the two cases described here show that additional factors, genetic or environmental, can have a significant influence on the phenotypic expression of HNF-1beta mutations.- - - - - - - - - - ranking = 7.4331482109848keywords = diabetes mellitus, diabetes (Clic here for more details about this article) |
3/15. Neonatal toxicity and transient neurodevelopmental deficits following prenatal exposure to lithium: Another clinical report and a review of the literature.I report the case of an infant girl who was exposed to lithium during gestation and her follow-up at the age of 1 year. She presented with transient neurodevelopmental deficits including lethargy, hypotonia, and poor oral feeding ability in the neonatal period. She required supportive treatment and made gradual improvement in neurologic functioning. On examination at the age of 1 year, physical findings and psychomotor development were normal. The English literature from 1978 to 2004 is reviewed. A total of 30 patients who were exposed to lithium during gestation with adequate clinical description were identified. A significant number of these babies presented with neurodevelopmental deficits and depressed neurological status including hypotonia, respiratory distress syndrome, cyanosis, lethargy, and weak suck and Moro reflexes in the neonatal period. The majority of these abnormalities resolved and most babies made full recovery. Other abnormalities were structural as well as functional involvement of the cardiovascular system, macrosomia, prematurity, jaundice, diabetes insipidus, and involvement of the thyroid gland. While the use of lithium during pregnancy does not appear to significantly increase the risk of congenital anomalies, it is frequently associated with perinatal complications and reversible neonatal toxicity. Suggested guidelines for appropriate monitoring of infants and breast-feeding of exposed babies are presented. In addition, prenatal surveillance of women with bipolar disorders who are being treated with lithium is briefly discussed.- - - - - - - - - - ranking = 0.43314821098484keywords = diabetes (Clic here for more details about this article) |
4/15. Monozygous triplets discordant for transient neonatal diabetes mellitus and for imprinting of the TNDM differentially methylated region.Transient neonatal diabetes mellitus (TNDM) is associated with paternal over-expression of an imprinted locus on chromosome 6q24, which contains one differentially methylated region (DMR); maternal demethylation at the DMR accounts for approximately 20% of cases. Here we report female monozygous triplets, two of whom have TNDM arising from loss of maternal methylation within the TNDM DMR.- - - - - - - - - - ranking = 5keywords = diabetes mellitus, diabetes (Clic here for more details about this article) |
5/15. polyhydramnios with maternal lithium treatment.Severe polyhydramnios, probably due to fetal lithium toxicity, is described. The mother had been treated with lithium because of manic-depressive psychosis. The plasma lithium level during the pregnancy was in or below the therapeutic range. From the 26th week of gestation, polyhydramnios developed. In the 35th week, 11.5 L of amniotic fluid was removed over a period of 12 hours by transabdominal amniocentesis. A cesarean delivery was performed in the 39th week of gestation because of fetal distress. The infant presented with the following symptoms, which in previous reports have been associated with lithium toxicity: asphyxia, apnea, cardiac decompensation, respiratory distress, hypoglycemia, thrombocytopenia, diabetes insipidus, hypotonia, and convulsions. The polyhydramnios was probably caused by fetal diabetes insipidus, possibly combined with cardiac decompensation. lithium can be toxic to the infant and the fetus even though the mother is not affected and has a normal or low plasma lithium level. polyhydramnios may be a sign of fetal lithium toxicity.- - - - - - - - - - ranking = 0.86629642196968keywords = diabetes (Clic here for more details about this article) |
6/15. Fractured femur of the newborn at cesarean section. A case report.Midshaft femur fractures are not expected in the newborn, particularly with the currently increased cesarean section rate. Such injuries have occurred when the mother had uterine myomas or diabetes or when the fetus had intrauterine growth retardation or osteoporosis secondary to copper deficiency, but they can also occur in newborn infants with normal weights and healthy mothers.- - - - - - - - - - ranking = 0.43314821098484keywords = diabetes (Clic here for more details about this article) |
7/15. Pancreatic isleitis with coxsackie virus B5 infection.Coxsackie group B virus infection may be responsible for some cases of insulin-dependent diabetes mellitus. However, Coxsackie B5 virus is rarely implicated in this respect. The authors observed striking pancreatic islet cell damage in an infant who died of a Coxsackie B5 virus infection. The inflammatory response consisted of mononuclear cells, and the acinar tissue was completely uninvolved.- - - - - - - - - - ranking = 1keywords = diabetes mellitus, diabetes (Clic here for more details about this article) |
8/15. Disorders of the endocrine pancreas.Hyperinsulinemic hypoglycemia in the neonate or in early infancy may be caused by an islet cell adenoma or a diffuse organic lesion of the endocrine pancreas such as nesidioblastosis. The 2 disorders cannot be distinguished by biochemical means but only by immunohistochemical analysis after subtotal pancreatectomy. An aggressive therapeutic approach is mandatory to avoid permanent neurological damage. In nesidioblastosis, 20% of patients are healed by subtotal pancreatectomy and the remainder may be controlled by postoperative treatment with diazoxide and hydrochlorothiazide. Some patients may need total pancreatectomy which leads to permanent insulin-dependent diabetes in most of them. When hyperinsulinemic hypoglycemia first manifests after 1 year of age, it is always caused by an islet cell adenoma, which is cured by enucleation.- - - - - - - - - - ranking = 0.43314821098484keywords = diabetes (Clic here for more details about this article) |
9/15. Pancreatic islet cell function and metabolic control in an infant with permanent neonatal diabetes.A girl with typical clinical manifestations of neonatal diabetes was observed for 16 months with consecutive evaluations of pancreatic beta- and alpha-cell function and metabolic control. At the diagnosis both the plasma immunoreactive insulin (IRI) and c-peptide concentrations were inappropriate for the contemporaneous hyperglycemia. During the follow-up, the c-peptide fell twice below the detection limit but the beta-cell function recovered partially on both occasions. Based on 24-hour urinary c-peptide excretion, the endogenous insulin secretion was less than 10% of that in non-diabetic infants. When diagnosed the patient had plasma immunoreactive glucagon (IRG) and glucagon-like immunoreactivity (GLI) concentrations below the reference range for normal neonates. The IRG normalised within the first month, while the GLI increased to a level exceeding the reference range. Hemoglobin A1 had already risen at the time of diagnosis and subsequently rose to a level indicating poor metabolic control. The findings indicate an immature function of both beta- and alpha-cells at the diagnosis with the alpha-cells maturing within the first month. The recovery of the beta-cell function, after two failures in this patient with permanent neonatal diabetes, suggests that the beta-cell damage was at least partially reversible.- - - - - - - - - - ranking = 2.598889265909keywords = diabetes (Clic here for more details about this article) |
10/15. Continuing neonatal morbidity in infants of women with class A diabetes.Despite having implemented rigorous glucose control for women with gestational diabetes early in the third trimester, we found excessive morbidity among the neonates of these women. To accurately assess the risk of newborn complications, we did a five-year review (1977 to 1981) of infants of class A diabetic mothers to determine the incidence and scope of morbidity in these infants. Fifty-one infants of class A mothers were identified (group 1) and randomly matched with 102 infants of nondiabetic mothers (group 2). The distribution of morbidity between the two groups was as follows: hypoglycemia 9/51 (18%) vs 0/102 (P less than .001); birth injuries 4/51 (8%) vs 1/102 (2%) (P less than .05); pulmonary edema 3/51 (6%) vs 0/102 (P less than .05); respiratory distress 4/51 (8%) vs 7/102 (7%) (NS); macrosomia 18/51 (35%) vs 23/102 (23%) (NS); and hyperbilirubinemia 3/51 (6%) vs 8/102 (8%) (NS). There were two fetal deaths and three infants with major congenital anomalies among the diabetic pregnancies compared to none from the nondiabetic pregnancies. Compared to insulin-dependent diabetes, class A diabetes is accompanied by relatively mild metabolic disturbances in the mother. On the other hand, the infant of a mother with class A diabetes appears to be at risk for serious and life-threatening complications, both before and after birth. These results raise the question of whether earlier identification, subsequent meticulous diabetic management, and altered timing of delivery might reduce the complications experienced by these infants.- - - - - - - - - - ranking = 3.4651856878787keywords = diabetes (Clic here for more details about this article) |
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