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.

Toward a developmental operational definition of autism.

Traditional approaches to diagnosing autism emphasize delays in communication and socialization. Traditional diagnostic schemes typically list symptoms (e.g., lack of eye contact), but provide little guidance on how to incorporate information about developmental level in making a diagnosis. Because standardized measures of adaptive behavior can provide information about children's communication, socialization, and other behavior relative to their age, they may be useful tools for diagnosing autism. This study investigated the ability of the Vineland Adaptive Behavior Scales to identify children with autism. Vineland scores and measures of intellectual functioning were obtained for children with autism, PDDNOS, and other developmental disorders (DD). Discriminant function analyses indicated that the autism and combined nonautism (PDDNOS and DD) groups could be differentiated on the basis of socialization, daily living skills, and serious maladaptive behaviors. Socialization alone accounted for 48% of the variance in diagnosis. Using regression analyses derived from a large normative sample, adaptive behavior scores were predicted from chronological age (CA) and mental age (MA). Socialization scores in the autism group were substantially below the level predicted from CA or MA. An index derived from the ratio of actual to predicted socialization scores correctly classified 86% of both autism and nonautism cases. Findings suggest that comparison of obtained Vineland socialization scores to those predicted by CA or MA may be useful in clarifying the diagnosis of autism.

Footsteps on the road to a positive psychology.

We have argued that psychology as a field has been preoccupied with the negative side of life and has left us with a view of human qualities that is warped and one-sided. Psychology is literally 'half-baked'. We need to bake the other half now. It is time for us to become equally concerned with the qualities and experiences that make life most worthwhile. A balance is needed between work that strives to relieve damage and work that endeavors to build strength. This balance is beautifully exemplified by Jack Rachman's work over the past 40 years. As an astute and compassionate clinician and researcher, Jack developed and evaluated effective treatments for some of the most debilitating anxiety disorders. At the same time, he was impressed by the resiliency of his clients and the courage they exhibited daily. His observations and studies of courage have helped to launch a systematic science of human strengths. They are giant footsteps on the road to a positive psychology.

Disposition of diphenyl sulphoxide in rat.

1. Radiolabelled diphenyl sulphoxide (U-14C- or 35S-) was administered by gavage (1.0 mmol/kg body weight) to the adult male Wistar rat following an overnight fast. 2. For both labelled forms faeces was the major route of excretion of radioactivity (50%) with substantial amounts still being voided during the third and fourth days (13%). Urinary elimination (42%) was similar during the first (20%) and second (17%) days and a small amount of radioactivity (7%) was found within the carcass after 4 days. 3. Plasma data showed a peak concentration at 40 min (tmax), a distribution half-life of 2 h (t1/2 alpha) and an elimination half-life of 22.5 h (t1/2 beta). Biliary studies revealed that 16% of the dose traversed the bile duct during the first day with nearly half of this being excreted in the first 8 h. 4. From urinary data, metabolism occurred via ring hydroxylation with subsequent conjugate formation. Oxidation of the sulphur to form the sulphone also took place. No evidence for sulphoxide reduction, cleavage of the ring structures or exclusion of the sulphur was obtained.

Prevention of depressive symptoms in school children.

This paper describes the development and preliminary efficacy of a program designed to prevent depressive symptoms in at-risk 10-13 year-olds, and relates the findings to the current understanding of childhood depression. The treatment targets depressive symptoms and related difficulties such as conduct problems, low academic achievement, low social competence, and poor peer relations, by proactively teaching cognitive techniques. Children were identified as 'at-risk' based on depressive symptoms and their reports of parental conflict. Sixty-nine children participated in treatment groups and were compared to 73 children in control groups. Depressive symptoms were significantly reduced and classroom behavior was significantly improved in the treatment group as compared to controls at post-test. Six-month follow-up showed continued reduction in depressive symptoms, as well as significantly fewer externalizing conduct problems, as compared to controls. The reduction in symptoms was most pronounced in the children who were most at risk.