The outcome of pulmonary hypertension and its association with pulmonary artery dilatation.

BACKGROUND: Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality. METHODS: Patients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N­terminal pro-brain natriuretic peptide (NT-proBNP) level and 6­min walking distance (6MWD)). RESULTS: In total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22-92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure ( r = 0.23, p < 0.001). Male sex, higher age, shorter 6MWD and higher NT-proBNP level were independently associated with mortality, but PA diameter was not. CONCLUSION: The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients.

[Financial compensation for asbestosis patients]. / Financiële compensatie voor patiënten met asbestose.

Asbestosis in the Netherlands is a rare work-related form of pulmonary fibrosis caused by long-term, intensive exposure to asbestos. It can have a great impact on patients' quality of life and life expectancy even 20-30 years after initial exposure. The Dutch Institute of Asbestos Victims (IAS) mediates between the victims and their employers or former employers about payment of compensation. Liability procedures against a previous employer are long and stressful. Since 1 April 2014 it has, therefore, been possible to receive financial aid from the state. The IAS and the Netherlands Asbestosis Panel determine who is eligible for this. In this article we look in detail at the conditions for, and the process of, application for this financial aid. Since the introduction of this arrangement, more than 250 asbestosis victims have applied for aid; so far, 65 applicants have met the required conditions.

Residential exposure to mould and dampness is associated with adverse respiratory health.

BACKGROUND: Indoor exposure to mould and dampness is frequently associated with asthma symptoms with and without lung function changes. However, the mechanisms contributing to this threat to respiratory health are only partly understood. OBJECTIVE: To investigate the contribution of recent exposure to mould and dampness in the living room or bedroom to respiratory health in a general practice-based cohort of 526 asthmatic children. METHODS: Parents were questioned about home characteristics, including moulds and dampness. The level of asthma control was evaluated in their participating children by means of asthma symptoms, peak expiratory flow (PEF) variability, severity of airway hyperresponsiveness (AHR), and medication usage. RESULTS: Children exposed to indoor moulds and dampness more often had severe AHR compared with non-exposed (42% vs. 16%; P< or =0.001). They also showed an increased PEF variability (11.3% vs. 8.4%; P=0.03) and, however, not significant, more frequent asthma symptoms. The use of controller medication was not significantly different between exposed and non-exposed children. After adjustment for gender, age, smoking, exposure to parental smoking, parental education, pet ownership, presence of inhalant allergy, use of controller medication, health care center, and season of study assessment, the odds ratio for severe AHR in exposed children was 3.95 [95% confidence interval (CI): 1.82-8.57]. CONCLUSION: We found a consistent association between reported moulds and dampness in the living room or the child's bedroom and an increased risk for severe AHR in a general practice-based cohort of asthmatic children, even after adjustment for gender, presence of inhalant allergy, and use of controller medication.

Severe airway hyperresponsiveness was not predictable with the use of current tools in asthmatic children in general practice.

OBJECTIVE: To evaluate whether moderate to severe airway hyperresponsiveness (AHR) could be suspected with the use of routinely available clinical and environmental information. STUDY DESIGN AND SETTING: Cross-sectional study of asthma in 526 asthmatics aged 7-17 years and treated in general practice. RESULTS: Moderate to severe AHR was present in 48% (n=253) of the participants. The presence of inhalation allergy, nocturnal symptoms, and usage of beta2-mimetics were significantly associated with moderate to severe AHR. If all three factors were present, the probability of the presence of moderate to severe and severe AHR was 76% and 36%, respectively. If all three were absent, the probability decreased to 11% and 5%, respectively. In 319 subjects (64%) AHR could not be adequately predicted with routinely available information. CONCLUSION: Moderate and severe AHR could not be suspected with the use of routinely available clinical and environmental information in the majority of children. Except for a subgroup of children, our models were not helpful in deciding in which child an inhaled corticosteroid should be started or whether the dose should be increased or decreased. We recommend measuring the severity of AHR in these children by means of an inhalation challenge test.

Life-threatening heart failure in meningococcal septic shock in children: non-invasive measurement of cardiac parameters is of important prognostic value.

UNLABELLED: Heart failure is a life-threatening complication of fulminant meningococcal septic shock (MSS). Depression of left ventricular function, in particular, is thought to be due to circulating meningococcal endotoxin. Myocardial failure leads to ventricular dilation expressed by an increased left-ventricle end-diastolic diameter (LVED). With ultrasonography, LVED can be accurately measured as well as the shortening fraction (SF). In an evaluative study we investigated the accuracy of the SF and compared it to the accuracy of the Glasgow meningococcal septicemia prognostic score (GMSPS) in the prediction of mortality in children with fulminant MSS. In 27 children admitted in a 4-year period with a presumptive clinical diagnosis of fulminant MSS, hypotension persisted for more than 1 h despite volume loading and inotropic therapy. Seven of these children died (26%); all had an SF <0.30 and a GMSPS > or =10 (the sensitivity of both scores was 100%). Positive predictive values of the SF and GMSPS were 41% and 58% respectively. CONCLUSIONS: SF can be used in addition to other severity scores in clinical decision-making and contribute to the selection of children with the worst prospects for inclusion in experimental treatment studies.