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

Heeft het BW een politieke kleur? Een kwantitatief onderzoek naar politieke kleuring van BW-wetgeving sinds 1992.

Samenvatting Van veel wetgeving kan men zeggen dat deze door politieke ideologie is ingegeven, maar van het Burgerlijk Wetboek hoort men dat toch zelden. Is dat terecht? In deze bijdrage wordt onderzocht of wijzigingen van het BW sinds 1992 in verband te brengen zijn met de politieke kleur van het indienende kabinet. Als dat inderdaad mogelijk is, is het BW misschien minder technisch-juridisch van aard dan soms wordt gedacht. Maar hoe valt politieke kleur van privaatrechtelijke wetgeving te meten? In deze bijdrage wordt een veelbelovende manier voor meting geïntroduceerd, die tot de conclusie leidt dat de onderzochte wijzigingen van het BW inderdaad een politieke kleur hebben. Summary With many products of the legislative process, one can easily identify the political ideology underlying the substantive policies contained in those vehicles for societal change. The Dutch Civil Code is not one of these. In fact, the 1992 Civil Code was commonly considered to be a technical recodification of private law rather than a vehicle for societal change. But how true is that of changes and amendments of the Code since its promulgation in 1992? In this contribution, we seek to find whether there is a relationship between political colours of governing coalitions and the ideological colour of the legislative bills concerning the Code introduced into Dutch Parliament. After a review of the literature, we introduc

Exhaled nitric oxide measurements in the first 2 years of life: methodological issues, clinical and epidemiological applications

Fractional exhaled nitric oxide (FeNO) is a useful tool to diagnose and monitor eosinophilic bronchial inflammation in asthmatic children and adults. In children younger than 2 years of age FeNO has been successfully measured both with the tidal breathing and with the single breath techniques. However, there are a number of methodological issues that need to be addressed in order to increase the reproducibility of the FeNO measurements within and between infants. Indeed, a standardized method to measure FeNO in the first 2 years of life would be extremely useful in order to meaningfully interpret FeNO values in this age group. Several factors related to the measurement conditions have been found to influence FeNO, such as expiratory flow, ambient NO and nasal contamination. Furthermore, the exposure to pre- and postnatal risk factors for respiratory morbidity has been shown to influence FeNO values. Therefore, these factors should always be assessed and their association with FeNO values in the specific study population should be evaluated and, eventually, controlled for

Annual lung function changes in young patients with chronic lung disease

Reference equations for ventilatory function that use different statistical models may introduce artifacts that affect the estimated change of lung function during growth in young subjects. The effect of differently modelled reference equations on the estimated annual change of forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) in young patients with chronic lung disease was assessed. Four frequently used reference equations were used to describe the longitudinal changes of FEV1 and FVC in 52 patients (23 females) with cystic fibrosis (CF) during a mean follow-up of 3.9 yrs. Choice of reference equations directly affected value and, most importantly, estimated annual change of FVC and FEV1. Mean+/-SD annual change of FEV1 varied from 2.2+/-6.2 to -2.2+/-3.6% of predicted. For two reference equations the estimated individual changes of FEV1 and FVC in CF were positively correlated wit

Paraneoplastic neurological syndromes with anti-hu antibodies

__Abstract__ Paraneoplastic neurological syndromes (PNS) are remote effects of cancer that are neither caused by invasion of the tumor or its metastasis, nor by infection, ischemia, metabolic and nutritional deficits, surgery or other forms of tumor treatment.1 PNS cause severe neurological disability and carry a poor prognosis. In PNS patients, various antibodies (Abs) have been found directed at neuronal antigens that are also expressed by the associated tumors in most cases. In 2004, the term ‘well characterized onconeural Ab’ was defined based on (1) Abs for which there are recognisable patterns on routine immunohistochemistry and for which immunoblotting on recombinant proteins must be used to confirm their specificities; (2) the number of cases reported associated with tumours; (3) the description of well characterised neurological syndromes associated with the antibodies; (4) the unambiguous identification of the Abs among different studies, and (5) the frequency of these Abs in patients without cancer.2 These ‘well-characterized’ onconeural Abs are by definition almost exclusively found in patients with cancer and include anti-Hu, Yo, CV2, Ri, Ma2 and amphiphysin.2 Recently, the anti-Tr antigen has been identified as the transmembrane protein Delta/ Notch-like epidermal growth factor-related receptor (DNER). Anti-DNER is thightly associated with Hodgkin lymphoma, and can therefore be considered a ‘well-characterized’ onconeural Ab, although DNER is not expressed by Hodgkin lymphoma.3 Anti-Hu is the most frequent well characterized onconeural Ab and usually associated with paraneoplastic encephalomyelitis and sensory neuronopathy.4 This thesis will focus on the immunopathogenesis of PNS with well-characterized onconeural Abs, in particular those with anti-Hu Abs (Hu-PNS)

A family with extrinsic allergic alveolitis caused by wild city pigeons: A case report

We describe a family in which the mother died of unresolved lung disease and whose 5 children, some of whom had previous signs of asthma, were subsequently affected by extrinsic allergic alveolitis caused by contact with wild city pigeon antigens. The children received systemic corticosteroids for 1 month and inhaled steroids for 24 months, while antigen exposure was reduced as much as feasible. This was followed by a quick clinical recovery and a slow normalization of chest radiographs and pulmonary function indices, especially of diffusion capacity, during a follow-up of 24 months. Because pigeon-breeder's lung caused by free-roaming city pigeons has not been previously described, it remains unclear whether this family developed the disease because of high antigen exposure or because of increased susceptibility. None of the supposedly high-risk human leukocyte antigen types were found in the children. Whether human leukocyte antigen B7 in 1 child played a role in the course of the illness remains speculative. It is unknown to what extent pigeon-breeder's lung caused by nondomestic birds remains undetected and misdiagnosed as difficult or steroid-resistant asthma. The question remains whether free-roaming city pigeons are indeed a public health risk. We suggest that atypical outdoor antigens be considered in all patients with nonresolving chest disease or therapy-resistant asthma