1/39. 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) |
2/39. Perlman syndrome: four additional cases and review. Perlman syndrome was first described in 1973 and comprises nephromegaly with renal dysplasia and wilms tumor, macrosomia, cryptorchidism, and multiple facial anomalies. polyhydramnios and hypoglycaemia are often found. Twelve children have been described from six different families. Five came from one family whose Yemenite Jewish parents were second cousins. Autosomal recessive inheritance has been suggested. prognosis is severe with neonatal death in most children. We report on 4 new cases of Perlman syndrome from 3 families; all parents were non-consanguineous. Some of the observed manifestations have been described only once in this syndrome (cardiac defect, hepatic fibrosis with portoportal bridging, haemangioma) or never before (volvulus, intestinal atresia, and agenesis of the corpus callosum in 1 patient, a cleft palate in another). All children died within the first year. The 2 sibs were born prematurely with nephromegaly but without hamartomas or nephroblastomatosis. This is consistent with the hypothesis that dysplastic medullary parenchyma in preterm infants develops into nephroblastomatosis and hamartoma and eventually wilms tumor. ( info) |
3/39. Are conventional targets for metabolic control sufficient to prevent fetal macrosomia during diabetic pregnancy? We report the case of a 26 year-old woman, with an uncomplicated type 1 IDDM of 17 yr duration followed for her first pregnancy. At conception, HbA1c (measured by HPLC) was 6.5% and fructosamine was 280 u.mol.l (normal range below 285). During the follow-up, 15-days-interval frutosamine never exceeded the normal range and HbA1c values were under 6.5% excepted in the third trimester (7.0 /- 0.8%) coinciding with a bad control of the 2 hours post-prandial blood glucose. A fetal macrosomy was discovered at 34 weeks of gestation and a heavy-for-date 4680 g baby was delivered by caesarean section at 38 weeks of gestation. Our case report outlines again the need to achieve the recommended target of metabolic control for the diabetic pregnant woman (blood preprandial glucose: 3.9-5.6 mM; post-prandial 2 h < 6.7 mM) specially during the third trimester of pregnancy. The use of computer databases might be helpful for precise monitoring during this narrow window period. ( info) |
4/39. pregnancy in patients with Wegener's granulomatosis: report of five cases in three women. Five cases of pregnancy occurring in three women with previously diagnosed Wegener's granulomatosis are described. The disease was diffuse in one case and localised in the other. Initial treatment consisted of a combination of corticosteroids and intravenous cyclophosphamide in two women, and methotrexate in one. Four pregnancies ended in live births despite pre-eclampsia in two cases. One therapeutic abortion was induced because of encephalocele. Comparable reported cases were reviewed to examine the implications of immunosuppressive treatment on the fetus. A relapse occurred during pregnancy in 40% of the cases, but in 25% if only pregnancies beginning during inactive disease were taken into account. No other indicator for maternal and fetal outcome was obvious. pregnancy should be planned after complete disappearance of disease activity. In the case of a relapse a combination of immunosuppressive drugs and corticosteroids should be chosen rather than corticosteroids alone because the outcome of pregnancy is poor in cases of undertreatment. Prematurity remains common. ( info) |
5/39. Fatal hypertrophic cardiomyopathy in the fetus of a woman with diabetes. BACKGROUND: Hypertrophic cardiomyopathy is recognized in infants of diabetic mothers, and when it occurs it is generally benign and transient. We describe a case of fetal cardiac death caused by hypertrophic cardiomyopathy in an infant of a diabetic mother. CASE: hydrops fetalis caused by hypertrophic cardiomyopathy resulted in the death of a macrosomic male fetus of a young woman who had well-controlled diabetes mellitus and was treated with insulin therapy during pregnancy. CONCLUSION: It is important to monitor fetal heart function in macrosomic infants of diabetic mothers. Hypertrophic cardiomyopathy might explain otherwise unexplained fetal deaths in women with diabetes. ( info) |
6/39. Rupture of the larynx in a newborn. BACKGROUND: Lesions of the laryngotrachea due to repeated intubation trauma after birth are reported. CASE:A macrosomic neonate was born at term after uneventful pregnancy. On the basis of diminished uterine contractions and arrest of descent, the head was delivered by using obstetric forceps. No intubation was attempted after birth. Ten hours later, the infant developed acute life-threatening respiratory distress and subcutaneous emphysema. intubation was impossible. Open surgical exploration of the neck revealed a rupture of the cricothyroid ligament within the larynx. The defect was closed immediately, and after artificial ventilation for 7 days, the infant was extubated. One year later, the child is developing satisfactorily, without stridor or hoarseness. CONCLUSION:In macrosomic neonates after traumatic delivery, a laryngotracheal lesion should be considered when respiratory distress syndrome combined with subcutaneous emphysema occurs. ( info) |
7/39. An unusual case of transitional obstructive acute renal failure during labor. An unusual case of transitional obstructive acute renal failure during labor which resolved after delivery is described. ( info) |
8/39. Large uterine defect found at cesarean section. A case report. BACKGROUND: Uncomplicated uterine perforation has been considered a benign event. Since the advent of operative hysteroscopy, there have been several reports of uterine rupture during pregnancy in patients who have undergone that procedure when complicated by known or unsuspected uterine perforation. Large fundal defects without rupture have also been reported. CASE: A 23-year-old, white woman was admitted for labor induction at 42 weeks' gestation. After an unsuccessful attempt at labor induction, a cesarean section (C/S) was performed. At that time a large (5-cm) fundal defect was noted. A thorough history suggested that the defect was probably the result of unsuspected perforation of the uterus during dilatation and currettage for a late first-trimester fetal death. A follow-up hysterosalpingogram was done and consultation obtained regarding future management. A course of expectant management with C/S prior to the onset of labor was advised. Three years later, after an uncomplicated pregnancy, a repeat C/S was done at 38 weeks' gestation. CONCLUSION: patients with a history of operative hysteroscopy or difficult curettage may have sustained known or unsuspected perforations of the uterus with subsequent scarring or defect, placing them at some risk of uterine rupture during pregnancy. patients should be counseled regarding these risks, and assessment by hysterography might be helpful. ( info) |
9/39. Arrest of descent in second stage of labour secondary to macrosomia: a case report. BACKGROUND: fetal macrosomia, defined as birth weight greater than 4000 g, complicates 10% of pregnancies and is a well-documented cause of prolonged second stage of labour, as well as of arrest of descent of the fetal presenting part. CASE: A multigravida woman with gestational diabetes mellitus was admitted in labour at term, and progressed to full dilatation. The fetal vertex failed to descend beyond -3 station. An emergency Caesarean section was performed and a 6452 g male infant was delivered. CONCLUSION: physicians should be aware of the possibility of macrosomia as the cause of failure of descent in the second stage. A heightened state of suspicion should be maintained, particularly in a multigravida woman with a prior macrosomic baby and the presence of other predisposing factors such as gestational diabetes mellitus. ( info) |
10/39. Abdominal rescue after failed cephalic replacement. BACKGROUND: Since the introduction of cephalic replacement for shoulder dystocia, the management of failures has not been addressed. The purpose of this report is to describe abdominal hysterotomy as an alternative approach to the resolution of shoulder dystocia. CASE: A 21-year-old woman, gravida 1, experienced severe shoulder dystocia following partial delivery of a 4320-g infant. Classical maneuvers as well as cephalic replacement were unsuccessful before and after general anesthesia. A low transverse hysterotomy permitted manual rotation of the anterior shoulder to the oblique diameter and further descent of the posterior shoulder. Delivery of the posterior arm was then completed without difficulty. CONCLUSION: Persistent failed cephalic replacement, especially after general anesthesia, can be successfully resolved with a hysterotomy using a low transverse uterine incision. ( info) |