Successful management of short gut due to vanishing gastroschisis - case report and review of the literature.

Vanishing gastroschisis is a rare in utero complication of gastroschisis. It is associated with a high mortality. We present a case report of an infant with vanishing gastroschisis that was managed with a combination of reconstructive bowel surgery and hepatosparing parenteral nutrition. The technique is described and a review of the literature is provided.

Antenatal detection of cleft lip with or without cleft palate: incidence of associated chromosomal and structural anomalies.

OBJECTIVE: To ascertain how many fetuses with prenatally diagnosed cleft lip with or without cleft palate have associated congenital structural and/or chromosomal abnormalities and whether there is an association with the anatomical type of cleft lip or palate. METHODS: This was a retrospective review of infants referred to the North-West England Regional Cleft Lip and Palate (CLAP) team between January 2000 and January 2006. Referrals made to the Regional Fetal Management Unit (FMU) in the same time period were investigated to identify the corresponding antenatal ultrasound findings and data on termination of pregnancy and intrauterine fetal death. RESULTS: Over the 6-year period investigated, 570 infants were referred to the FMU and/or CLAP team. Among these, there were 24 terminations of pregnancy, two intrauterine fetal deaths and one early neonatal death identified. Data on 69 of the 543 patients that survived were incomplete. Of 188 cases with unilateral and 34 cases with bilateral cleft lip +/- palate there were no karyotypical abnormalities without other structural abnormalities. The incidence of associated structural abnormalities varied with the anatomical type of cleft: that of unilateral cleft lip +/- palate was 9.8% (19/194), that of bilateral cleft lip and palate was 25% (11/44) and that of midline cleft lip and palate was 100% (11/11). None of 252 cases with isolated cleft palate was identified antenatally; of these, 5.6% (n = 14) had either karyotypical or associated structural abnormalities and 21.0% (n = 53) had a genetic syndrome as an underlying diagnosis. CONCLUSIONS: It is essential to tailor the antenatal counseling of patients to the specific scan diagnosis, considering both the anatomical type of cleft and the presence or absence of associated abnormalities. It is inappropriate to offer invasive testing to all patients. The use of three-dimensional ultrasound as an adjunct should be considered in these patients to improve the accuracy of prenatal diagnosis.

TNF-alpha alters nitric oxide- and endothelium-derived hyperpolarizing factor-mediated vasodilatation in human omental arteries.

OBJECTIVE: To investigate the effect of TNF-alpha on the endothelial function of human systemic arteries. METHODS: Omental arteries were obtained from healthy pregnant women undergoing Cesarean section and examined using isometric wire myography. RESULTS: Incubation with TNF-alpha (1nM) alone did not alter bradykinin-mediated endothelium-dependent relaxation of arteries. However, TNF-alpha did attenuate nitric oxide- (NO) and prostacyclin-independent endothelial-mediated relaxation. Similarly, in vessels constricted with a high potassium solution (60 mM), which inhibits vasodilatation via endothelial-derived hyperpolarising factor (EDHF), TNF-alpha incubation also attenuated bradykinin-induced vasodilatation. CONCLUSIONS: The vasorelaxant capacity of human systemic arteries is compromised by TNF-alpha incubation in the presence of NO/prostacyclin or EDHF-blockade.

Regulation of endothelial-dependent relaxation in human systemic arteries by SKCa and IKCa channels.

Blockade of small-conductance Ca (2)(+)-activated K(+) channels (SK(Ca)) and intermediate conductance Ca(2)(+)-activated K(+) channels (IK(Ca)) can cause inhibition of endothelium-dependent hyperpolarizing factor (EDHF) in many vascular beds from animals, but there is a relative paucity of data in human vessels. Systemic arteries, isolated from women with healthy pregnancies, relax to the endothelial-dependent agonist bradykinin via a nonprostacyclin and non-nitric oxide pathway attributable to EDHF. Therefore, in this study, the authors investigated the effect of pharmacological blockade of SK(Ca) and IK(Ca) on EDHF-mediated relaxation of human omental and myometrial arteries preconstricted with either arginine vasopressin or U46619. Human arteries were isolated from omental and myometrial biopsies taken from healthy women undergoing planned cesarean section at term. Endothelial function was assessed using wire myography. In all vessels examined, nonspecific blockade of IK(Ca) with charybdotoxin attenuated EDHF-attributed relaxation. However, when Tram 34 was used to block IK(Ca), the attenuation of relaxation was evident only with U46619 preconstriction. In arteries from both vascular beds, and with either preconstrictor, a combination of either apamin and charybdotoxin or apamin plus Tram 34 almost ablated EDHF-attributable relaxation. These data support the notion that in human systemic arteries, activation of, primarily, SK(Ca) and IK(Ca)K(+) channel subtypes underlies EDHF-mediated relaxation. These results have important implications for future studies ascertaining the molecular mechanisms of hypertensive disorders (eg, preeclampsia, in which EDHF is thought to be aberrant).

An overview of endothelium-derived hyperpolarising factor (EDHF) in normal and compromised pregnancies.

Normal pregnancy is associated with decreased peripheral vascular resistance, although the mechanisms are poorly understood. Pre-eclampsia is characterised by increased vessel resistance and a decreased relaxant capacity, contributing to the associated hypertension, multi-organ damage and intra-uterine growth restriction. An endothelium-derived vasoactive substance-endothelium-derived hyperpolarising factor may be responsible for the physiological changes in the maternal vasculature. This is a review of the current understanding of the complexities of vessel behaviour. Evidence is reviewed to support the possible contribution of endothelium-derived hyperpolarising factor to the compromised vascular state in pre-eclampsia. This is an important area of research which may help to understand further the pathogenesis of this specific pregnancy related disease, that contributes greatly to maternal and fetal morbidity and mortality.