Cases Of Exomphalos Research Articles

Pregnancy in patients with well-treated β-thalassemia: Outcome for mothers and newborn infants

Objective: Our purpose was to investigate the course and outcome of pregnancy in women with well-treated β-thalassemia. Study Design: Twenty-two pregnancies, including one twin pregnancy, in 19 women were studied. Pregnancy was advised when patients had received a prolonged intensive treatment with hypertransfusions and iron chelation and had echocardiographically normal resting left ventricular performance. All conceptions were spontaneous. Cardiac function, along with hematologic, endocrinologic, and hepatic parameters were initially assessed and monitored throughout pregnancy and for 2 to 9 years post partum. Babies were delivered by elective cesarean section. Results: Twenty-one healthy newborn infants were delivered. A spontaneous abortion and a case of exomphalos also occurred. Gestation, delivery, and recovery were surprisingly uneventful, and no significant cardiac complications were encountered. Conclusion: Pregnancy can be safe for mothers and babies, provided that women with thalassemia have been started early on intensive treatment and have a normal resting cardiac performance. (Am J Obstet Gynecol 1999;180:360-5.)

Gastroschisis bowel in an intact exomphalos: Implications for etiology and possible prevention

The etiology of changes in the bowel in gastroschisis is unknown. A case of exomphalos is described, in which the bowel had a gastroschisis-like appearance. The possibility of a vascular mechanism as the cause is discussed.

GASTROSCHISIS AND EXOMPHALOS: RECENT TRENDS AND FACTORS INFLUENCING SURVIVAL

The present study examined the treatment and survival of patients with gastroschisis and exomphalos in the 5-year period January 1982-December 1987, at the Prince of Wales Children's Hospital. There were 15 cases of gastroschisis and 17 cases of exomphalos. The influences of temperature on arrival, birthweight, method of repair and associated anomalies on survival were examined. More patients presenting with gastroschisis survived than those with exomphalos (14 of 15 compared with 10 of 17, respectively). Of all the factors examined, the presence and nature of associated anomalies is the most important in determining survival.

A prospective study of amniotic fluid cholinesterases: Comparison of quantitative and qualitative methods for the detection of open neural tube defects

The value of quantitative and qualitative methods of cholinesterase (ChE) analysis in the detection of open neural tube defect (NTD) has been assessed in a prospective survey of 1495 mid-trimester amniotic fluids. Using a quantitative method the mean ChE values were much lower in fluids from pregnancies of normal outcome but it was not possible to discriminate these fluids completely from those associated with NTD pregnancies, particularly when the specimens were contaminated with blood. Similarly, measurement of acetylcholinesterase (AChE) activity alone by three different methods also failed to eliminate the overlap between the two groups. In contrast, polyacrylamide gel electrophoresis revealed only a single band of ChE activity in 1408 out of 1410 fluids from pregnancies with a normal outcome whilst amniotic fluids from all 60 cases of open NTD, 6 out of 7 cases of exomphalos and 3 out of 4 cases of intra-uterine death gave the characteristic second faster-running AChE band. A qualitative gel method which requires the same amount of ChE activity to be loaded from each amniotic fluid is an effective method for pre-natal diagnosis of NTDs.

Acetylcholinesterase and butyrylcholinesterase measurement in the pre-natal detection of neural tube defects and other fetal malformations

Acetylcholinesterase activity in amniotic fluid was measured at 30 °C by a reaction rate method employing acetyl-β-methyl thiocholine as substrate and ethopropazine as a selective inhibitor of butyrylcholinesterase. This assay proved more specific than previously reported methods. Activity was greater in five cases of anencephaly (4.8–9.7 U/l) and nine cases of spina bifida (5.1–8.6 U/l) than in 50 pregnancies with normal outcome (mean activity 2.0 ± 0.9 (S.D.) U/l). There was no overlap between results from normal and neural-tube-defect groups, and the results showed no significant correlation with gestational age. Butyrylcholinesterase activity in amniotic fluid was measured using butyrylthiocholine as substrate. In accordance with previous reports, levels were elevated in pregnancies affected by neural tube defects. The ratio butyrylcholinesterase/acetylcholinesterase activity showed similar values for anencephalic, spina bifida and normal pregnancies; however, the two cases of exomphalos investigated could be clearly distinguished from all other groups on this basis.

Embryonic malformations in rats, resulting from maternal diabetes: preliminary observations

Diabetes mellitus was induced in female Wistar rats by injections of either alloxan or streptozotocin, and their embryos were found to have significantly higher incidences (7.5%) of brain and heart abnormalities (non-closure of neural folds, and deformities of heart chambers) at mid-gestation than controls (2.2%). There were also increased numbers of resorptions (25% in diabetic animals: 7.2% in controls). Both drugs produced similar abnormalities. External and X-ray examination of 488 foetuses from streptozotocin-treated animals at 20 days showed eight cases of exomphalos, two cases of micrognathia with tongue protrusion, and 34 cases of incomplete sacral ossification. This last deformity occurred also in foetuses of mildly diabetic animals, and has been seen occasionally in infants of human diabetic mothers. Other evidence suggests that skeletal deformities may be due to hyperinsulinism in the foetuses of diabetic mothers. Even a mild or pre-diabetic condition may set the foetus at risk.

Exomphalos and gastroschisis: a 10-year review.

Ninety-six cases of exomphalos or gastroschisis managed at The children's Hospital, Sheffield, during the period 1964-74 are analysed. There were 306 cases with minor lesions, 30 with major lesions, 27 with a ruptured exomphalos and only 3 with gastroschisis. The overall mortality rate was 46-9 percent. Survival could be closely correlated with the birth weight, the nature of the primary lesion, the presence or absence of liver within the sac and the presence and severity of associated congenital anomalies. A "scoring system" based on these criteria is proposed to predict the prognosis of an infant with an exomphalos.

An autopsy case of exomphalos containing abdominal viscera organs and cloaca of unknown sexuality

An autopsy case of exomphalos containing abdominal viscera organs and cloaca of unknown sexuality

Exomphalos with macroglossia: A study of eleven cases

In 11 cases of exomphalos with macroglossia other features of the syndrome, which is sometimes familial, included high birth weight, accelerated postnatal growth and skeletal development, facial nevus flammeus, abnormal pinnae and other cranio-facial characteristics, a diaphragmatic anomaly, renal enlargement with abnormal histological features, adrenal cytomegaly and possibly pancreatic islet-cell hyperplasia. The reported occurrence of neo-natal hypoglycaemia in this syndrome is stressed. In 11 cases of exomphalos with macroglossia other features of the syndrome, which is sometimes familial, included high birth weight, accelerated postnatal growth and skeletal development, facial nevus flammeus, abnormal pinnae and other cranio-facial characteristics, a diaphragmatic anomaly, renal enlargement with abnormal histological features, adrenal cytomegaly and possibly pancreatic islet-cell hyperplasia. The reported occurrence of neo-natal hypoglycaemia in this syndrome is stressed. A base de 11 casos de exomphalo con macroglossia il esseva constatate que le syndrome (que es a vices familial) include—como characteristicas additional—alte pesos natal, un accelerate crescentia e disveloppamento skeletic postnatal, facial nevos flammee, anormal pinnas e altere characteristicas cranio-facial, un anormalitate diaphragmatic, allargamento renal con anormal characteristicas histologic, cytomegalia adrenal, e—possibilemente—hyperplasia del cellulas in le insulas pancreatic. Es sublineate le reportate occurrentia de hypoglycemia neonatal in site syndrome. A base de 11 casos de exomphalo con macroglossia il esseva constatate que le syndrome (que es a vices familial) include—como characteristicas additional—alte pesos natal, un accelerate crescentia e disveloppamento skeletic postnatal, facial nevos flammee, anormal pinnas e altere characteristicas cranio-facial, un anormalitate diaphragmatic, allargamento renal con anormal characteristicas histologic, cytomegalia adrenal, e—possibilemente—hyperplasia del cellulas in le insulas pancreatic. Es sublineate le reportate occurrentia de hypoglycemia neonatal in site syndrome.

Case of Exomphalos Closed by Operation, and a Specimen of a Similar Case in Which Closure was Impossible

Case of Exomphalos Closed by Operation, and a Specimen of a Similar Case in Which Closure was Impossible

V. Three Cases of Exomphalos occurring in the Government Maternity Hospital, Madras, within a period of one year, and all presenting and delivered in the same way.

BJOG: An International Journal of Obstetrics & GynaecologyVolume 14, Issue 4 p. 276-278 V. Three Cases of Exomphalos occurring in the Government Maternity Hospital, Madras, within a period of one year, and all presenting and delivered in the same way. Major G. G. Giffard I.M.S., M.R.C.P., M.R.C.S., Major G. G. Giffard I.M.S., M.R.C.P., M.R.C.S. Superintendent, Government Maternity Hospital, Madras.Search for more papers by this author Major G. G. Giffard I.M.S., M.R.C.P., M.R.C.S., Major G. G. Giffard I.M.S., M.R.C.P., M.R.C.S. Superintendent, Government Maternity Hospital, Madras.Search for more papers by this author First published: October 1908 https://doi.org/10.1111/j.1471-0528.1908.tb14602.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Volume14, Issue4October 1908Pages 276-278 RelatedInformation