1/59. 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.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
2/59. 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.- - - - - - - - - - ranking = 2.5keywords = diabetes (Clic here for more details about this article) |
3/59. A novel syndrome of diabetes mellitus, renal dysfunction and genital malformation associated with a partial deletion of the pseudo-POU domain of hepatocyte nuclear factor-1beta.Mutations in the homeodomain-containing transcription factor hepatocyte nuclear factor (HNF)-1beta are the cause of one form of maturity-onset diabetes of the young (MODY), type 5 (MODY5). We have studied a Norwegian family, N5, with a syndrome of mild diabetes, progressive non-diabetic renal disease and severe genital malformations. The sequence of the HNF-1beta gene ( TCF2 ) revealed a 75 bp deletion in exon 2 (409-483del) which would result in the synthesis of a protein lacking amino acids Arg137 to Lys161 (R137-K161del). This deletion is located in the pseudo-POU region of HNF-1beta, a region implicated in the specificity of dna binding. Functional studies of R137-K161del HNF-1beta revealed that it could not bind an HNF-1 target sequence or stimulate transcription of a reporter gene indicating that this is a loss-of-function mutation. The R137-K161del allele co-segregated with diabetes and renal disease in pedigree N5. In addition, two of four female carriers with this mutation had vaginal aplasia and rudimentary uterus (Mullerian aplasia). These studies strongly suggest that heterozygous mutations in the HNF-1beta gene are associated with a syndrome characterized by MODY and severe, non-diabetic renal disease. Moreover, the presence of internal genital malformations in two females suggests that additional clinical features may be associated with HNF-1beta mutations.- - - - - - - - - - ranking = 3.5keywords = diabetes (Clic here for more details about this article) |
4/59. uterine rupture in a multiparous woman during labor induction with oral misoprostol.A multigravida with gestational diabetes, mild pregnancy-induced hypertension and a previous curettage received four doses of misoprostol (100 microg) at three hourly intervals for induction of labor at term. Vaginal delivery of a live healthy baby occurred 1 h after the fourth dose. Hindwaters were bloodstained. Three hours later, she had excessive bleeding. Examination showed that the left lateral uterine wall had ruptured. She recovered after hysterectomy and blood transfusions.- - - - - - - - - - ranking = 0.5keywords = diabetes (Clic here for more details about this article) |
5/59. Controversies in the diagnosis and treatment of gestational diabetes.Uncontrolled gestational diabetes is associated with infant macrosomia and a lifelong risk of developing diabetes. Prompt diagnosis and aggressive management is therefore critical. All pregnant women should be screened for carbohydrate intolerance. Women with even minor abnormalities in blood sugar levels should be trained to monitor their glucose levels, rigorously control their diet, and use insulin if necessary. exercise is also very beneficial.- - - - - - - - - - ranking = 3keywords = diabetes (Clic here for more details about this article) |
6/59. Amylophagia presenting as gestational diabetes.Amylophagia, or the practice of consuming purified starch, is a particular expression of the more general phenomenon of pica. This compulsive dietary aberration, observed in many pregnant patients worldwide, is common among rural African American women in the southern united states. The effect this practice has on the course of gestational diabetes has not been examined. We report 2 cases of gestational diabetes, refractory to initial dietary management, in which the patients were consuming one-half to 1 box of cornstarch per day. Following cessation of amylophagia, the gestational hyperglycemia spontaneously resolved. Amylophagia is a complex behavioral phenomenon arising from the interplay of biochemical, hematological, psychological, and cultural factors. In some patient populations, it may represent an often overlooked etiologic or exacerbating factor in the condition of gestational diabetes. family physicians practicing obstetrics should inquire about amylophagia in patients who are at risk for this behavior and in patients who present with gestational hyperglycemia.- - - - - - - - - - ranking = 3.5keywords = diabetes (Clic here for more details about this article) |
7/59. Conjoined twins in a triplet pregnancy.Conjoined twins in a triplet pregnancy is an extremely rare occurrence. We present here, a 27-year-old multigravida with gestational diabetes and a conjoined twins in a triplet pregnancy.- - - - - - - - - - ranking = 0.5keywords = diabetes (Clic here for more details about this article) |
8/59. Unilateral bowing of long bones and multiple congenital anomalies in a child born to a mother with gestational diabetes.We report on a new-born girl with multiple congenital anomalies consisting of major skeletal anomalies restricted to the left side, cleft palate, ventricular and atrial septal defect, retromicrognathia, short neck, dysplastic low-set ears and large birth weight. The left-side bony anomalies include shortening and bowing of the femur and tibia, hypoplasia of the fibula, hip dislocation, clubfoot and mild shortening of the long tubular bones in the left arm with elbow dislocation. The pregnancy was complicated by insulin-dependent gestational diabetes mellitus in the mother. The radiographic features were not consistent with the diagnosis of campomelic dysplasia, kyphomelic dysplasia or other skeletal dysplasias characterized by bowing and shortening of the long bones. To our knowledge, the multiple congenital anomalies, including major skeletal malformations, present in our case have never been simultaneously reported until now. A maternal diabetes syndrome in this infant is probable. The occurrence of major congenital malformations in offspring of women with gestational diabetes is reviewed and discussed. We provide evidence that gestational diabetes mellitus could be teratogenic. We recommend a careful diabetic control in every woman with a history of gestational diabetes.- - - - - - - - - - ranking = 4.5keywords = diabetes (Clic here for more details about this article) |
9/59. Twin pregnancy following gonadotrophin therapy in a patient with Sheehan's syndrome.A case of Sheehan's syndrome presented with secondary amenorrhea and was put on L-thyroxine, prednisolone and cyclical estrogen and progestin. ovulation induction with gonadotrophins and intrauterine insemination with husband's semen resulted in a twin pregnancy. Antepartum course was complicated by bronchial asthma, gestational diabetes and pregnancy-induced hypertension. Cesarian section was done at 34 weeks gestation for preterm rupture of membranes and breech presentation. Both babies and their mother were doing well at 6 months of follow-up.- - - - - - - - - - ranking = 0.5keywords = diabetes (Clic here for more details about this article) |
10/59. Identification of mtDNA mutation in a pedigree with gestational diabetes, deafness, wolff-parkinson-white syndrome and placenta accreta.Mitochondrial dna (mtDNA) defects are associated with a number of human disorders. Although many occur sporadically, maternal transmission is the hallmark of diseases due to mtDNA point mutations. The same mutation may manifest strikingly different phenotypes; for example, the A to G substitution at np 3243 was first reported in patients with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (the melas syndrome), but is also found in patients with diabetes and deafness. Here we present a case of gestational diabetes, deafness, premature greying, placenta accreta and Wolff-Parkinson-White (WPW) syndrome associated with a mtDNA mutation. Although this is the first report of such an association, study of 27 other patients with WPW syndrome failed to confirm that this mtDNA mutation is a common cause of such pre-excitation disorders.- - - - - - - - - - ranking = 3keywords = diabetes (Clic here for more details about this article) |
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