1/17. 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 = diabetic (Clic here for more details about this article) |
2/17. 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 = 0.33333333333333keywords = diabetic (Clic here for more details about this article) |
3/17. candida albicans chorioamnionitis associated with preterm labor and sudden intrauterine demise of one twin. A case report.BACKGROUND: Although cervicovaginal Candida infections occur in 20-25% of pregnancies, the incidence of ascending infection in these cases is only 0.8%, and such infection rarely causes chorioamnionitis. CASE: Sudden intrauterine fetal demise (IUFD) of twin A occurred in a diabetic primigravida presenting with a twin pregnancy and preterm labor at 33 weeks of gestation. Placental pathology and autopsy of the stillborn twin revealed extensive chorioamnionitis and fetal sepsis in the presence of candida albicans. Twin B was unaffected. CONCLUSION: In this case, C albicans chorioamnionitis seemed to be associated with sudden IUFD.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
4/17. 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 = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
5/17. Neonatal diabetes mellitus: patient report and review of the literature.A female infant born at 33 weeks gestation to a gestationally diabetic mother developed apnea and respiratory distress at 6 hours of age. Laboratory investigation demonstrated persistent hyperglycemia, and the patient was treated with continuous intravenous and subsequent subcutaneous insulin therapy. Detailed laboratory investigation to reveal the etiology of hyperglycemia and further endocrine evaluation were not significant. The baby's insulin requirement has continued thereafter, and she is being followed up in an outpatient clinic still under insulin therapy at 18 months of age. Neonatal diabetes mellitus should be considered in the differential diagnosis of neonatal hyperglycemia, and it may develop in newborns born to diabetic mothers, as well as neonatal hypoglycemia. insulin treatment with close blood glucose monitoring is essential as long as hyperglycemia persists since neonatal diabetes mellitus may be either transient or permanent and it is not possible to differentiate these two outcomes before 18 months of age.- - - - - - - - - - ranking = 0.33333333333333keywords = diabetic (Clic here for more details about this article) |
6/17. Caudal Regression syndrome in twin pregnancy with type II diabetes.Caudal Regression syndrome (CRS) is a rare fetal complication of diabetic pregnancy, which can result in long-term neurological, urologic, and orthopedic complications. Although the exact teratogenic mechanism is not known, hyperglycemia appears to play a crucial role as a teratogen, and therefore, stringent control of diabetes preconceptually and in early pregnancy is presumed to reduce the risk of occurrence. We report an unusual case of CRS affecting only one of a set of monozygotic twins, suggesting that as yet, unidentified factors other than hyperglycemia are included in its causation.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
7/17. intestinal obstruction caused by infarcted splenic hemangioma with renal vein thrombosis in a newborn: a case report.The spleen in newborns, infants, and children is rarely involved in a variety of pathological processes. These processes may involve an isolated splenic disease or may be a part of a systemic illness. Renal vein thrombosis in infants of diabetic mothers has been reported. We report a case of a newborn with intestinal obstruction caused by an infarcted splenic hemangioma and renal vein thrombosis. To the best of our knowledge, this is the youngest case with both above-mentioned diagnoses and causes of intestinal obstruction in the English medical literature.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
8/17. Diabetes, hypertension and birth injuries: a complex interrelationship.Seemingly coincidental occurrence of various pathological conditions may derive from common etiologic denominators. While reviewing 240 malpractice claims involving shoulder dystocia related fetal injuries, we found two antenatal complications in the background conspicuously often. Chronic or pregnancy induced hypertension was identifiable in 80 instances (33%). pregnancy induced or preexisting diabetes was diagnosed 48 times (20%). Many of these patients were poorly controlled. The blood pressure was usually checked during the antenatal visits. However, about one-half of all patients received no diabetic screening. Therefore, this study may underestimate the actual incidence of diabetes. It has been calculated that the frequency of diabetes in pregnancy and that of hypertension, is about 5% in the united states. Thus, the rates of these complications in this selected group of gravidas was severalfold higher than in the general population. Since hypertension causes retarded fetal growth, it cannot be a direct cause of arrest of the shoulders at delivery. The likely common denominator is maternal diabetes a known predisposing factor both for preeclampsia and shoulder dystocia at birth. In the course of litigations for fetal injuries, demonstration of the predisposing role of seemingly unrelated shortcomings of the medical management may profoundly influence the outcome. This principle is demonstrated by the presentation of an actual malpractice action which resulted in a substantial settlement.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
9/17. Caudal dysplasia sequence: severe phenotype presenting in offspring of patients with gestational and pregestational diabetes.The association of maternal diabetes mellitus and congenital anomalies is well established. Children of insulin-dependent diabetic women have an increased risk of congenital malformations, especially major multiorgan defects. The cardiovascular, central nervous, gastrointestinal, genitourinary and musculoskeletal are the most affected body systems. Studies also show that offspring of women with gestational diabetes (specially those with fasting hyperglycaemia) tend to have higher rates of congenital anomalies. We report two cases of infants born to unrelated mothers: one with diabetes mellitus first detected during pregnancy (gestational diabetes) and the other with pregestational diabetes. Both infants had amelia of the lower limbs (suggestive of caudal dysplasia sequence), together with cardiovascular, skeletal, urinary and gastrointestinal defects. While pregestational diabetes seems to leave no doubt about its teratogenicity, the association of gestational diabetes and fetal/newborn malformations is still under discussion. Complete absence of the lower limbs has not been reported in association with gestational diabetes, but it may represent a spectrum of the caudal dysplasia sequence. The presentation of two cases with the same clinical phenotype of mothers with gestational and pregestational diabetes supports the evidence that gestational diabetes can be responsible for the development of the most severe form of the caudal dysplasia sequence.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
10/17. diagnosis of fetal ductus arteriosus aneurysm: importance of the three-vessel view.OBJECTIVE: To present four cases of ductus arteriosus aneurysm (DAA) detected by fetal echocardiography and highlight the value of the three-vessel view in the diagnosis of DAA. methods: In addition to the standard fetal echocardiographic views, we examined the three-vessel view in four cases of DAA. The three-vessel view was achieved by sliding the transducer cranially from the four-chamber plane toward the fetal upper mediastinum to demonstrate cross-sections of the main pulmonary artery, the ascending aorta and superior vena cava arranged in a straight line from the left anterior to the right posterior aspect of the mediastinum. DAA was diagnosed when there was a tortuous ductus arteriosus with a dilation that protruded leftward of the aortic arch. CASE SERIES: In the first case, an insulin-dependent diabetic woman underwent fetal ultrasound examination at 36 weeks' gestation showing right-to-left cardiac disproportion and bidirectional flow in the aorta and main pulmonary artery (PA). The three-vessel view showed a dilated ductus arteriosus (DA) which was stenosed at its distal end. In the second case, a woman had fetal ultrasound scans at 38 and 39 weeks' gestation for suspected intrauterine growth restriction and oligohydramnios. The scans identified an abnormal aortic arch and the three-vessel view showed an elongated vascular structure at the distal end of the PA, which was the DAA. In the third case, a woman with a high-risk obstetric history had multiple scans showing an abnormal PA to aorta relationship, with an apparent 'kink' in the PA. The three-vessel view clarified that this was the DAA. In the fourth case, a woman was scanned because of a 3-kg weight gain in 1 week. The fetal ultrasound scan showed moderate polyhydramnios with normal fetal growth and normal intracardiac anatomy and flow, and the three-vessel view demonstrated a large DA. In each of these cases, the DAA appeared to have occurred in isolation with non-specific clinical findings. CONCLUSION: We advocate the use of the three-vessel view, in conjunction with the standard echocardiography views currently employed, to assist in the diagnosis of DAA.- - - - - - - - - - ranking = 0.16666666666667keywords = diabetic (Clic here for more details about this article) |
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