The aim of this study was to assess the impact of the COVID-19 pandemic on the mental wellbeing of children 8-18 years old with chronic conditions, by comparing pandemic data with pre-pandemic data ...and with healthy peers. Data were obtained from two ongoing longitudinal cohorts: the PROactive cohort study following children with a chronic condition, and the WHISTLER population cohort. Mental wellbeing was assessed by three indicators: life satisfaction, internalising symptoms, and psychosomatic health. The stringency of the COVID-19-related lockdown was considered a moderating factor. Data on chronic patients were recorded before (n = 934, 65% girls) and during (n = 503, 61% girls) the pandemic, and compared to healthy peers during the pandemic (n = 166, 61% girls). Children with a chronic condition reported lower life satisfaction, but no clinically relevant changes in internalising symptoms or psychosomatic health, during the pandemic compared to before. In comparison to healthy peers, children with a chronic condition experienced decreased life satisfaction and psychosomatic health, but internalising symptoms did not differ between groups during the COVID-19 pandemic. The lockdown stringency was negatively associated with all indicators of mental wellbeing-worse life satisfaction, more internalising symptoms, and more psychosomatic symptoms.
Background
Data on the impact of the number and nature of perceived asthma triggers on health‐related quality of life (HRQL) in children are scarce.
Objective
To investigate the impact of perceived ...asthma triggers on both asthma‐specific and generic HRQL in children.
Methods
A cross‐sectional study was conducted among children (7‐18 years) with asthma in secondary and tertiary care. Children were screened with electronic questionnaires regarding respiratory and allergic symptoms. Asthma‐specific HRQL was assessed using the Pediatric Asthma Quality of Life Questionnaire (PAQLQ) (score range 1‐7) and generic HRQL using the RAND questionnaire (score range 7‐32). The Kruskal‐Wallis test and one‐way ANOVA were used to test the difference of, respectively, the PAQLQ and RAND scores across the number of perceived asthma triggers (0, 1‐2, 3‐4, or ≥ 5). Univariable and multivariable linear regression analyses were performed to evaluate the association between individual triggers and HRQL.
Results
A total of 527 children with a mean (SD) age of 12.1 (2.9) years were included. Children with a higher number of perceived triggers had significantly lower PAQLQ and RAND scores (ie poorer HRQL). The difference in PAQLQ scores was clinically relevant between children with 0 versus 3‐4 or ≥ 5 triggers and 1‐2 versus ≥ 5 triggers (mean difference 0.66, 1.02 and 0.63, respectively). Especially, non‐allergic triggers (physical exercise, the weather, (cigarette) smoke and emotions) were significantly associated with reduced PAQLQ scores. Emotions and food/drinks were associated with reduced RAND scores.
Conclusion and Clinical Relevance
A higher number of perceived triggers of asthma were associated with reduced HRQL in children with asthma. Especially, non‐allergic triggers were associated with reduced HRQL.
Many adolescents worldwide (indirectly) grow up with a chronic disease, which may impact their functioning and wellbeing. The objective of this study is to assess whether adolescents with a (family ...member with a) chronic disease differ from their healthy counterparts in terms of psychosocial functioning. Data from the Dutch 2013 HBSC-survey were used, including 7168 adolescents (
Mean
age
= 13.7, SD = 1.57, 50.5% female). Participants indicated whether they or one of their family members had a long-term (> 3 months) disease or disability (mental/physical) and were categorized into four groups based on disease presence (none, other, self, both). Psychosocial functioning was assessed in terms of life satisfaction, self-rated health, psychosomatic health, mental health problems, support, substance use, physical exercise, screen time, and school liking. Chronically diseased adolescents (
n
= 162) reported lower life satisfaction, self-rated and psychosomatic health, more mental health problems, lower peer support, more substance use, and less physical exercise compared to healthy peers. Chronically diseased adolescents who also had a family member with a chronic disease (
n
= 74) showed comparable outcomes on these life domains, although they did not differ from their healthy peers regarding peer support, substance use, and physical activity. Healthy adolescents with a chronically diseased family member (
n
= 737) reported significantly lower life satisfaction, self-rated and psychosomatic health, more mental health problems, and less family support compared to healthy peers who grew up in healthy families; however, they reported more positive outcomes than adolescents who had a chronic disease themselves.
Conclusion
: Having a (family member with a) chronic disease is associated with impaired psychosocial functioning on various life domains. Our findings aid in understanding the psychosocial associates of chronic disease and imply that caregivers should be observant of psychosocial problems among vulnerable adolescents to provide appropriate guidance.
What is Known:
• Adolescents who grow up with a (family member with a) chronic disease encounter numerous challenges that may be related to poorer developmental outcomes on the long term.
What is New:
• This study adds a comprehensive overview of the psychosocial functioning of adolescents with a (family member with a) chronic disease, as compared to healthy counterparts that grow up in a healthy family.
Introduction
Spirometry plays an important role in the assessment of possible respiratory failure in children with neuromuscular diseases (NMDs). However, obtaining reliable spirometry results is a ...major challenge. We studied the relation between oscillometry and spirometry results. Oscillometry is an easy, noninvasive method to measure respiratory resistance R and reactance X. We hypothesized an increased R and reduced X in patients with more reduced lung function.
Methods
In this prospective single‐center study, we included all children with NMDs able to perform spirometry. We consecutively measured R and X at 5, 11, and 19 Hz and (forced) vital capacity, peak expiratory flow. Spearman correlation coefficients and positive and negative predictive values were calculated. Regression curves were estimated.
Results
We included 148 patients, median age 13 years (interquartile range: 8–16). A negative correlation was found between R and spirometry outcomes (Spearman correlation coefficient ρ: −0.5 to −0.6, p < 0.001). A positive correlation was found between X (i.e., less negative outcomes) and spirometry outcomes (ρ: 0.4–0.6, p < 0.001). Highest correlation was found at lower frequencies. Regression analysis showed a nonlinear relation. Measurement of inspiratory and expiratory R and X did not provide added value. Positive predictive values of 80%–85% were found for z‐scores of R measured at 5 Hz versus (F)VC ≤ 60%.
Conclusion
We found a nonlinear relation between oscillometry and spirometry results with increased R and reduced X in patients with more restrictive lung function decline. Given the difficulties with performing spirometry, oscillometry may be a promising substitute.
Introduction
Children with neuromuscular diseases develop cough impairment. Airway clearance techniques (ACTs) may help to prevent recurrent respiratory tract infections (RTIs). A commonly used ACT ...is mechanical insufflation‐exsufflation (MI‐E), but evidence for efficacy is limited. We hypothesize that MI‐E has beneficial effect on RTI related hospital admission rate.
Methods
In this single‐center retrospective study, we reviewed all children who used daily MI‐E between 2005 till June 2019. Primary outcome studied was the number of RTIs requiring hospital admission. Patient satisfaction and burden experienced by MI‐E use were explored by questionnaires using a Likert scale. The relative number of RTIs requiring admission and the number of admission days per eligible period before and after the introduction of MI‐E were compared using the Friedman test and the Wilcoxon signed‐rank test.
Results
Thirty‐seven children were included.
The median number of RTI related hospital admissions per 1000 eligible days after the introduction of MI‐E was 0.9 (interquartile range IQR 0.0‐3.1) compared to the 3 preceding years (median 3.7; IQR 1.4‐5.9; P = .006). The median number of RTI related admission days per 1000 eligible days after the introduction of MI‐E was significantly lower with a median of 2.7 (IQR 0.0‐17.4) compared to the 3 preceding years (median 33.6; IQR 15.0‐51.1; P = .001). Patient satisfaction was high with low burden, even in patients who discontinued treatment.
Conclusion
A significantly lower number of RTIs requiring hospital admission and shorter admission duration after the introduction of MI‐E was found, with high patient satisfaction and low burden.
Respiratory complications are the most important cause of morbidity and mortality in spinal muscular atrophy (SMA). Respiratory muscle weakness results in impaired cough, recurrent respiratory tract ...infections and eventually can cause respiratory failure. We assessed longitudinal patterns of respiratory muscle strength in a national cohort of treatment-naïve children and adults with SMA, hypothesizing a continued decline throughout life.
We measured maximal expiratory and inspiratory pressure (PE
and PI
), Sniff Nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), and peak cough flow (PCF) in treatment-naïve patients with SMA. We used mixed-models to analyze natural history patterns.
We included 2172 measurements of respiratory muscle function from 80 treatment-naïve patients with SMA types 1c-3b. All outcomes were lower in the more severe phenotypes. Significant differences in PEF were present between SMA types from early ages onwards. PEF decline was linear (1-2%/year). PEF reached values below 80% during early childhood in types 1c-2, and during adolescence in type 3a. PE
and PI
were severely lowered in most patients throughout life, with PE
values abnormally low (i.e. < 80 cmH
O) in virtually all patients. The PE
/PI
ratio was < 1 throughout life in all SMA types, indicating that expiratory muscles were most affected. All but SMA type 3b patients had a lowered PCF. Patients with types 2b and 3a had PCF levels between 160 and 270 L/min, those with type 2a around 160 L/min and patients with type 1c well below 160 L/min. Finally, SNIP was low in nearly all patients, most pronounced in more severely affected patients.
There are clear differences in respiratory muscle strength and its progressive decline between SMA types. We observed lower outcomes in more severe SMA types. Particularly PEF may be a suitable outcome measure for the follow-up of respiratory strength in patients with SMA. PEF declines in a rather linear pattern in all SMA types, with clear differences at baseline. These natural history data may serve as a reference for longer-term treatment efficacy assessments.
Introduction
Understanding the impact of scoliosis surgery on lung function is important for counseling patients about risks and benefits of surgery. We prospectively compared the trends in lung ...function test (LFT) results before and after scoliosis surgery in children with neuromuscular diseases or dysmorphic syndromes. We hypothesized a stabilization.
Methods
We prospectively included children with neuromuscular or syndromic scoliosis able to perform LFTs. We studied (forced) vital capacity (FVC), ratio of forced expiratory volume in 1 s (FEV1) and FVC, and peak expiratory flow (PEF). Preoperative LFT results were compared with results 3–4 months after surgery. The mean monthly change in LFT results up to 2 years after surgery was compared with the preoperative natural history using linear mixed‐effects models.
Results
We included 43 patients. No significant change was observed in absolute values of (F)VC, FEV1/FVC, and PEF before and after surgery. In 23 neuromuscular patients median standardized VC, FVC, and PEF decreased significantly after surgery from 43% to 33%, 42% to 31%, and 51% to 40%, respectively. In 20 syndromic patients, median FVC decreased from 68% to 65%. The monthly rate of change in FVC did not change significantly in both groups with a mean difference of 0.18% (95% CI: −0.27, −0.61) and −0.44% (95% CI: −1.05, 0.16).
Conclusion
No stabilization of lung function 3–4 months after scoliosis surgery was observed in children with neuromuscular and syndromic scoliosis with restrictive lung function disease. The effect on the rate of lung function decline remains inconclusive.
Background
We aimed to assess whether final height in children with cystic fibrosis (CF) is affected by body mass index (BMI), BMI increase, pulmonary function, and cystic fibrosis‐related diabetes ...(CFRD).
Study design
A longitudinal, retrospective study was performed in a cohort of 57 patients with CF (30 boys, 27 girls) born between 1997 and 2001. Height and weight were recorded annually from ages 0.5 to 10 years and biannually up to the age of 18. Measurements were converted to height‐for‐age‐adjusted‐for‐target‐height (HFA‐TH) and BMI‐for‐age z‐scores. Analyses were performed using the independent t tests and the Pearson's correlation.
Results
For both boys and girls, HFA‐TH and BMI‐for‐age z‐scores were significantly lower in the first year of life, these scores increased rapidly until the age of 11 and 8 years, respectively. In boys, HFA‐TH z‐scores declined during puberty, with subsequently significantly impaired final height (z‐score, −0.56, n = 30, standard deviation SD = 0.81, P = 0.001). In girls, HFA‐TH z‐scores briefly declined after the age of 8 years, but then increased to a z‐score of −0.21 (n = 27, SD = 0.87) at age 18, which is not significantly lower than the national average (P = 0.22). Pulmonary function and the presence of CFRD were not associated with final height. However, rapid BMI increase between ages 1 and 6 was negatively associated with final height in boys (n = 29, r =−0.420; P = 0.023) and girls (n = 25, r =−0.466; P = 0.019).
Conclusions
In boys and girls, early BMI increase was associated with impaired final height. We suggest that early childhood serves as a “window” in which nutritional variations may program subsequent growth. Further refinement of nutritional strategies could be needed.
Progressive lung function decline, resulting in respiratory failure, is an important complication of spinal muscular atrophy (SMA). The ability to predict the need for mechanical ventilation is ...important. We assessed longitudinal patterns of lung function prior to chronic respiratory failure in a national cohort of treatment-naïve children and adults with SMA, hypothesizing an accelerated decline prior to chronic respiratory failure.
We included treatment-naïve SMA patients participating in a prospective national cohort study if they required mechanical ventilation because of chronic respiratory failure and if lung function test results were available from the years prior to initiation of ventilation. We analyzed Forced Vital Capacity (FVC), Forced Expiratory Volume in 1 s (FEV
), Peak Expiratory Flow (PEF) and Maximum Expiratory Pressure (PE
). We studied the longitudinal course using linear mixed-effects models. We compared patients who electively started mechanical ventilation compared to patients who could not be weaned after acute respiratory failure.
We analyzed 385 lung function tests from 38 patients with SMA types 1c-3a. At initiation of ventilation median age was 18.8 years (IQR: 13.2-30.1) and median standardized FVC, FEV
and PEF were 28.8% (95% CI: 23.5; 34.2), 28.8% (95% CI: 24.0; 33.7) and 30.0% (95% CI: 23.4; 36.7), with an average annual decline of 1.75% (95% CI: 0.86; 2.66), 1.72% (95% CI: 1.04; 2.40) and 1.65% (95% CI: 0.71; 2.59), respectively. Our data did not support the hypothesis of an accelerated decline prior to initiation of mechanical ventilation. Median PE
was 35.3 cmH
O (95% CI: 29.4; 41.2) at initiation of mechanical ventilation and relatively stable in the years preceding ventilation. Median FVC, FEV
PEF and PE
were lower in patients who electively started mechanical ventilation (p < 0.001).
Patterns of lung function decline cannot predict impending respiratory failure: SMA is characterized by a gradual decline of lung function. We found no evidence for an accelerated deterioration. In addition, PE
remains low and stable in the years preceding initiation of ventilation. Patients who electively started mechanical ventilation had more restrictive lung function at initiation of ventilation, compared to patients who could not be weaned after surgery or a respiratory tract infection.
The diagnostic value of peanut components is extensively studied in children, but to a lesser extent in adults with suspected peanut allergy. The use of peanut components in daily practice may reduce ...the need for double-blind placebo-controlled food challenges (DBPCFCs); however, validation studies are currently lacking.
To evaluate the diagnostic value of (combined) peanut components and validate a previously found Ara h 2 cutoff level with 100% positive predictive value (PPV) in adults with suspected peanut allergy.
Adults who underwent a peanut DBPCFC were included: 84 patients from a previous study (2002-2012) and 70 new patients (2012-2019). Specific IgE (sIgE) to peanut extract, Ara h 1, 2, 3, 6, and 8 was measured using ImmunoCAP. Diagnostic value was assessed with an area under the curve (AUC) analysis.
In total, 95 (62%) patients were peanut allergic. sIgE to Ara h 2 and Ara h 6 were the best predictors with an AUC (95% confidence interval) of 0.85 (0.79-0.91) and 0.85 (0.79-0.92), respectively. The Ara h 2 cutoff level with 100% PPV (≥1.75 kUA/L) was validated in the 70 new patients. Thirty percent of all included patients could be classified correctly as peanut allergic using this validated cutoff level.
sIgE to Ara h 2 and Ara h 6 have equally high discriminative ability. Peanut allergy can be predicted accurately in one-third of adults using a validated cutoff level of sIgE to Ara h 2.
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