A new Light on Lung Disease in Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly of the diaphragm with an incidence of approximately 1 per 2500 births. Typically, the abdominal organs will herniate into the chest cavity, with resulting maldevelopment of the alveoli and pulmonary vessels. The defect is usually – reportedly in 84% of the cases – located on the left side of the diaphragm. Right-sided CDH and bilateral CDH, which occur in 14% and 2% of cases, are associated with a worse prognosis. CDH can present as an isolated defect or in combination with other congenital anomalies, such as congenital heart disease or chromosomal anomalies. The condition may be life threatening and key determinants of mortality are the severity of pulmonary hypoplasia and the presence of therapy resistant pulmonary hypertension. Smaller number and generations of airways, thickened alveolar septa, and abnormal architecture of the respiratory acinus characterize pulmonary hypoplasia. Pulmonary hypertension may result from medial hyperplasia and adventitial thickening associated with under- and maldevelopment of the pulmonary vessels. Although survival rates have improved over the years, mortality rates in live-born patients still range from 10-35%, depending on case selection. Moreover, surviving neonates carry a substantial risk of developing secondary morbidity, such as cardiopulmonary, gastro-intestinal and neurological problems

Accounting for self-protective responses in randomized response data from a social security survey using the zero-inflated Poisson model

In 2004 the Dutch Department of Social Affairs conducted a survey to assess the extent of noncompliance with social security regulations. The survey was conducted among 870 recipients of social security benefits and included a series of sensitive questions about regulatory noncompliance. Due to the sensitive nature of the questions the randomized response design was used. Although randomized response protects the privacy of the respondent, it is unlikely that all respondents followed the design. In this paper we introduce a model that allows for respondents displaying self-protective response behavior by consistently giving the nonincriminating response, irrespective of the outcome of the randomizing device. The dependent variable denoting the total number of incriminating responses is assumed to be generated by the application of randomized response to a latent Poisson variable denoting the true number of rule violations. Since self-protective responses result in an excess of observed zeros in relation to the Poisson randomized response distribution, these are modeled as observed zero-inflation. The model includes predictors of the Poisson parameters, as well as predictors of the probability of self-protective response behavior.Comment: Published in at http://dx.doi.org/10.1214/07-AOAS135 the Annals of Applied Statistics (http://www.imstat.org/aoas/) by the Institute of Mathematical Statistics (http://www.imstat.org

An exploration of spousal caregivers’ well-being after the death of their partners who were older cancer patients – A phenomenological approach

Purpose: The aim of this study was to gain more insight into the psychosocial well-being of the recently bereaved spouses who took care of their partners with cancer. Method: A qualitative study was developed, taking a phenomenological approach. Eleven former caregivers and spouses of patients who died of cancer at, or after, the age of 64, participated in individual in-depth interviews. Only caregivers who were bereaved for a minimum of three months and maximum of one year were interviewed. The analysis of the data was based on the Qualitative Analysis Guide of Leuven. Results: The first moments of bereavement included feelings of disbelief, regret and relief. A feeling of being overwhelmed during this time was reported by some, others sought distraction from their grief. Loneliness, emotional fluctuations and a sense of appreciation for the support of loved ones were dominant themes. Also, gratitude and the importance of consolation played a role in the participants' well-being. When participants addressed the matter of moving forward in life, most explained how they wanted to keep the memories of their partner alive while rebuilding their lives. Conclusions: The present study offers insight into the experiences of the bereaved spousal caregiver and highlights the need of social support during the bereavement period. All participants expressed loss-oriented and restoration-oriented coping strategies. Also, loneliness is considered a dominant feeling throughout the bereavement period. Social contact can ease these feelings of loneliness through providing either distraction or possibilities to share the burden. This paper emphasized the importance of improving access to healthcare professionals during bereavement

CBT for people with intellectual disabilities : emerging evidence, cognitive ability and IQ effects

Historically people with intellectual disabilities have not been offered or received cognitive behavioural interventions that have been shown to be effective for mental health and emotional problems experienced by those without such disabilities. This is despite many people with intellectual disabilities having life experiences that potentially result in them having an increased risk to such problems. This paper discusses whether such therapeutic disdain is justified based on the evidence that is available and emerging concerning the application of cognitive behavioural interventions for this population. Issues concerning access to services, the ability of people with intellectual disabilities to engage in and benefit from the cognitive components of CBT, and the effect of cognitive abilities and IQ level on treatment effectiveness are explored in relation to this question

Repeated Cross-Sectional Randomized Response Data Taking Design Change and Self-Protective Responses into Account

Abstract. Randomized response (RR) is an interview technique that can be used to protect the privacy of respondents if sensitive questions are posed. This paper explains how to measure change in time if a binary RR question is posed at several time points. In cross-sectional research settings, new insights often gradually emerge. In our setting, a switch to another RR procedure necessitates the development of a trend model that estimates the effect of the covariate time if the dependent variable is measured by different RR designs. We also demonstrate that it is possible to deal with self-protective responses, thus accommodating our trend model with the latest developments in RR data analysis. Keywords: linear trend, longitudinal data, misclassification, randomized response, repeated cross-sections, self-protective responses Randomized response (RR) is an interview technique that can be used if sensitive questions are posed and respondents are reluctant to answer directly In addition to the RR setting, misclassification probabilities occur in several other fields of research. The one most closely related to RR is the postrandomization method (PRAM, Kooiman, Willenborg, & Gouweleeuw, 1997) that misclassifies values of categorical variables using a computerized process after the data are collected to protect the respondents' privacy. PRAM uses RR after the data collection. Misclassification also plays a role in medicine and epidemiology with the probabilities correctly classified as a case (sensitivity) or noncase (specificity), see This paper proposes a model to measure changes in time whenever RR is used to pose sensitive questions at several time points cross-sectionally. The model is illustrated with data from a Dutch repeated cross-sectional study on noncompliance to rules regarding social benefits. Data are collected every 2 years since 2000 and given that measures to prevent regulatory noncompliance are intensified during this period, the question arises as to whether the prevalence of regulatory noncompliance changes over the years and how the change can be modeled. Considering time a covariate, we propose a method to measure the effect of this covariate if the dependent variable is measured by RR. Several aspects of the cross-sectional study at hand make it impossible to use standard analysis methods and necessitate a new approach in the analysis of RR data to deal with research questions of this type. Firstly, the fact that RR variables represent misclassified responses on categorical variables precludes the use of, for example, the linear logit model (Agresti, 2002, p. 180), to test for a linear trend. Using the framework o

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO consortium consensus

Congenital diaphragmatic hernia (CDH) is associated with high mortality and morbidity. To date, there are no standardized protocols for the treatment of infants with this anomaly. However, protocols based on the literature and expert opinion might improve outcome. This paper is a consensus statement from the CDH EURO Consortium prepared with the aim of achieving standardized postnatal treatment in European countries. During a consensus meeting between high-volume centers with expertise in the treatment of CDH in Europe (CDH EURO Consortium), the most recent literature on CDH was discussed. Thereafter, 5 experts graded the studies according to the Scottish Intercollegiate Guidelines Network (SIGN) Criteria. Differences in opinion were discussed until full consensus was reached. The final consensus statement, therefore, represents the opinion of all consortium members. Multicenter randomized controlled trials on CDH are lacking. Use of a standardized protocol, however, may contribute to more valid comparisons of patient data in multicenter studies and identification of areas for further research

Perioperative management of children with glycogen storage disease type II-Pompe disease

Background: Pompe disease is a rare metabolic disorder caused by a deficiency of the lysosomal enzyme acid α-glucosidase. Glycogen accumulation damages skeletal, cardiac, and smooth muscles, causing a progressive and debilitating muscle weakness and cardiomyopathy. As life expectancy has much improved since the introduction of enzyme replacement therapy an increasing number of patients are referred for surgical procedures. Due to the potential cardiopulmonary complications, these patients form a high-risk group for the anesthesiologist. Aims: In this study, we investigated the incidence of perioperative complications in children with Pompe disease treated in our hospital since the introduction of enzyme replacement therapy. Methods: Anesthetic and perioperative data of children with Pompe disease treated between 1999 and 2015 in the Erasmus MC-Sophia Children's Hospital, University Medical Centre, Rotterdam, The Netherlands, were collected, retrospectively. Results: Of the 65 children with Pompe disease, 34 patients underwent in total 77, mostly low-risk, surgical procedures. Twenty-one children had the classic infantile form and 13 had a nonclassic presentation of Pompe disease. In 13 (16.8%) procedures, 1 or more perioperative complications occurred. Perioperative desaturation was the main complication (12.9%), followed by arrhythmia (3.8%) and heart failure requiring diuretic treatment (2.6%). One child died 2 days postoperatively, but this was considered unrelated to the procedure. Conclusion: Despite the potentially high anesthetic risk for children with Pompe disease under enzym

Особенности деонтологии в сексологической практике

Описаны основные принципы врачебной этики в сексологической практике. Рассмотрены особенности взаимоотношений врача−сексолога и пациента. Подчеркивается, что выполнение врачом деонтологических принципов будет способствовать гармонизации семейно−сексуальных отношений.Basic principles of medical ethics in sexological practice are presented. The peculiarities of mutual relations of the doctor sexologist and the patient are discussed. It is emphasized that adherence of the doctor−sexologist of ethical principles will promote harmonization of family sexual relations

The performance of COBRA, a decision rule to predict the need for intensive care interventions in intentional drug overdose

BACKGROUND: COBRA was developed as a decision rule to predict which patients visiting the emergency department (ED) following intentional drug overdose will not require intensive care unit (ICU) interventions. COBRA uses parameters from five vital systems (cardiac conduction, oxygenation, blood pressure, respiration, and awareness) that are readily available in the ED. COBRA recommends against ICU admission when all these parameters are normal. OBJECTIVE: The primary aim of this study was to determine the negative predictive value (NPV) of COBRA in predicting ICU interventions. Secondary outcomes were the sensitivity, specificity and positive predictive value (PPV), and the observation time required for a reliable prediction. DESIGN: Observational cohort study. SETTINGS AND PARTICIPANTS: Patients with a reported intentional overdose with drugs having potential acute effects on neurological, circulatory or ventilatory function were included, and data necessary to complete the decision rule was collected. The attending physician in the ED made the actual admission decision, on the basis of clinical judgement. COBRA was measured 0, 3 and 6 h after arrival at the ED. OUTCOME MEASURES: Need for ICU interventions (treatment of convulsion; defibrillation; mechanical or noninvasive ventilation; intravenous administration of vasopressive agents, antiarrhythmics, atropine, calcium, magnesium or sedation; continuous hemofiltration or administration of antagonist/antidote and fluid resuscitation). MAIN RESULTS: Of 230 new cases (144 unique patients), 59 were immediately referred to the psychiatric services and/or sent home by the attending physician, 27 went to a regular ward, and 144 were admitted to the ICU. Of these 144 cases, 40 required one or more ICU interventions. By the time the first parameters were collected, the NPV of COBRA was 95.6%. After 3 h of observation, NPV was 100%, while sensitivity, specificity and PPV were 100, 61.1 and 35.1%, respectively. None of these values improved by prolonging the observation time to 6 h. CONCLUSION: In patients with a reported intentional overdose with drugs having potential acute effects on neurological, circulatory or ventilatory function, the COBRA decision rule showed good performances in predicting the need for intensive care interventions, with a NPV of 100% after 3 h of observation