Cases reported "Diabetes, Gestational"

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1/80. 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)

2/80. Bartter's syndrome and pregnancy.

    Bartter's syndrome is a rare renal tubular disorder and few cases are reported associated with pregnancy. We describe a case of long standing disease with a good obstetric outcome in which the main concern was to maintain normal potassium serum levels. ( info)

3/80. In utero treatment of fetal complete heart block with terbutaline. A case report.

    BACKGROUND: Isolated fetal complete heart block is an uncommon finding, with a mortality rate of 20-30%. Various treatment modalities have been reported, with no consistent success. CASE: Fetal complete heart bock was diagnosed in a 30-year-old woman at 29 weeks' gestation. She had an elevated antinuclear antibody and anti-SSA antibody titer. Fetal cardiac decompensation was detected at 32 weeks. After a trial of intravenous isoproterenol without significant side effects, she was treated with oral terbutaline. An increase in the fetal ventricular rate and complete resolution of hydrops fetalis occurred. A male infant was delivered by cesarean section, at term, and underwent cardiac pacemaker implantation at 4 days of age. CONCLUSION: The treatment of fetal complete heart block is controversial. Premature delivery with cardiac pacing is associated with high morbidity and mortality. We report prenatal treatment with oral sympathomimetic medication, which allowed delivery to be delayed until term, with a successful outcome. ( info)

4/80. 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)

5/80. 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. ( info)

6/80. 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. ( info)

7/80. 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. ( info)

8/80. prenatal diagnosis of total arhinia.

    Complete or total arhinia, in which there is absence of the soft tissue of the nose, is extremely rare. The embryological origin of the defect is thought to be maldevelopment of the paired nasal placodes. Available neonatal case descriptions have reported the frequent coexistence of other facial anomalies. This report is the first to describe the antenatal diagnosis of total arhinia confirmed after delivery. Sonographic views of the fetal profile, showing an absence of the nose led to consideration of the diagnosis. ( info)

9/80. 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. ( info)

10/80. 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. ( info)
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