Long-term effect of growth hormone on sleep-disordered breathing in Malaysian children with Prader-Willi syndrome: a retrospective study.

STUDY OBJECTIVES: The effect of recombinant human growth hormone (rhGH) on sleep-disordered breathing (SDB) in Malaysian children with Prader-Willi syndrome (PWS) is under-investigated. We determined (1) the short- and long-term effects of rhGH and (2) factors associated with worsening SDB in children with PWS receiving rhGH. METHODS: This retrospective study included children with PWS (with and without rhGH) who had undergone at least 1 polysomnography. Outcomes measured were the presence of SDB before and after starting rhGH and the progress of SDB with and without rhGH. Serial insulin-like growth factor 1 (IGF-1) measurements were recorded. RESULTS: One-hundred and thirteen polysomnograms were analyzed. The majority (92.3%) of initial polysomnograms showed SDB, with a median (interquartile range) apnea-hypopnea index of 5.0 (2.6, 16.3) events/h. The age for receiving rhGH was a median (IQR) of 1.9 (0.7, 3.4) years. One-third (36.8%) had worsening SDB after initiating rhGH, which was associated with higher IGF-1 levels post-rhGH (P = .007). After a median of 5 years of rhGH, 73.6% maintained or reduced their positive airway pressure settings. Without rhGH, 80% had increased their positive airway pressure settings. Worsening SDB while on rhGH was associated with higher body mass index, lower rhGH dose, higher IGF-1 levels, and non-15q deletion. CONCLUSIONS: The majority of Malaysian children with PWS had SDB. At initiation of rhGH, one-third of patients had worsening SDB, associated with increased IGF-1 levels. Stabilization of SDB was more frequently seen in those receiving long-term rhGH. Worsening SDB while on rhGH was associated with a higher body mass index, receiving a lower dose of rhGH, higher IGF-1 levels, and non-15q deletion. CITATION: Tan YT, Azanan MS, Hng SY, et al. Long-term effect of growth hormone on sleep-disordered breathing in Malaysian children with Prader-Willi syndrome: a retrospective study. J Clin Sleep Med. 2024;20(8):1291-1299.

Morbidity and treatment costs of cystic fibrosis in a middle-income country.

Introduction: : Asian children with cystic fibrosis (CF) managed in Malaysia have significant morbidity with limited access to life-sustaining treatments. We determined the morbidity and treatment cost of CF in a resource-limited country. Methods: This cross-sectional study included all children diagnosed with CF in our centre. Data on clinical presentation, genetic mutation, serial spirometry results and complications were collected. Out-of-pocket (OOP) and healthcare costs over 1 year were retrieved for patients who were alive. Cohen's d and odds ratio (OR) were used to determine the effect size. Results: Twenty-four patients were diagnosed with CF. Five patients died at a median (range) age of 18 (0.3-22) years. F508deletion (c. 1521_1523delCTT) was found in 20% of the alleles, while 89% of the variants were detected in nine patients. Body mass index (BMI) Z score was >-1.96 in 70.6% of patients. Two thirds (68%) were colonised with Pseudomonas aeruginosa, and this was associated with lower weight (P = 0.009) and BMI (P = 0.02) Z scores. Only 18% had FEV1 Z scores >-1.96. Early symptom onset (d = 0.74), delayed diagnosis (d = 2.07), a low FEF25-75 Z score (d = 0.82) and a high sweat conductance (d = 1.19) were associated with death. Inpatient cost was mainly from diagnostic tests, while medications contributed to half of the outpatient cost.Healthcare utilisation cost was catastrophic, amounting to 20% of the total income. Conclusion: Asian children with CF suffer significant complications such as low weight, low lung function and shortened lifespan. P. aeruginosa colonisation was frequent and associated with poor growth. Healthcare cost to parents was catastrophic.

The outcome of COVID-19 in children with chronic lung disease: Systematic review and meta-analyses.

BACKGROUND: Few studies have examined the impact of Coronavirus disease 2019 (COVID-19) infection on children with chronic lung disease (CLD). OBJECTIVE: To perform a systematic review and meta-analysis to determine the prevalence, risk factors for contracting COVID-19, and complications of COVID-19, in children with CLD. METHODS: This systematic review was based on articles published between January 1, 2020 and July 25, 2022. Children under 18 years old, with any CLD and infected with COVID-19 were included. RESULTS: Ten articles involving children with asthma and four involving children with cystic fibrosis (CF) were included in the analyses. The prevalence of COVID-19 in children with asthma varied between 0.14% and 19.1%. The use of inhaled corticosteroids (ICS) was associated with reduced risk for COVID-19 (risk ratio [RR]: 0.60, 95% confidence interval [CI]: 0.40-0.90). Uncontrolled asthma, younger age, AND moderate-severe asthma were not significant risk factors for contracting COVID-19. Children with asthma had an increased risk for hospitalization (RR: 1.62, 95% CI: 1.07-2.45) but were not more likely to require assisted ventilation (RR: 0.51, 95% CI: 0.14-1.90). The risk of COVID-19 infection among children with CF was <1%. Posttransplant and cystic fibrosis-related diabetes mellitus (CFRDM) patients were at an increased risk for hospitalization and intensive care treatment. CONCLUSION: Hospitalizations were higher in children with asthma with COVID-19 infection. However, using ICS reduced the risk of COVID-19 infection. As for CF, postlung transplantation and CFRDM were risk factors for severe disease.

Poor sleep quality in children with atopic dermatitis and its effects on behavior: A multicenter cross-sectional study from a low-middle-income country.

BACKGROUND: Sleep disturbance in children with atopic dermatitis (AD) frequently goes unnoticed and can be associated with behavioral challenges. The aims of this study were to determine (a) the prevalence and factors associated with sleep disturbance and behavioral problems and (b) the correlation between sleep disturbance and behavioral problems in children with AD. METHODS: This cross-sectional study involved children aged 4-12 years old with moderate to severe AD. Age and sex-matched healthy children were recruited as the comparison group. The Children's Sleep Habits Questionnaire (CSHQ) and the Strengths and Difficulties Questionnaire (SDQ) were used to assess sleep disturbance and behavioral problems, respectively. Higher scores in both questionnaires signify more disturbance. RESULTS: Seventy patients and 141 controls were recruited. Median (interquartile range) age of patients was 5 (4,8) years. Patients had later sleep time (p < 0.001), longer night awakening (p < 0.001), and shorter sleep duration (p < 0.001) compared to controls. CSHQ total scores and all its domains, except for sleep-disordered breathing were significantly higher in patients compared to controls. Patients also had significantly higher SDQ total difficulties scores in all domains, except for peer problems. Severity of AD was significantly associated with high CSHQ and SDQ scores. There was a moderate positive correlation between the total CSHQ score and total SDQ score in patients (r = 0.532). CONCLUSIONS: Children with moderate-to-severe AD had significantly more sleep disturbance and more behavioral problems than their healthy peers. Sleep disturbance had a positive correlation with behavioral problems. Severity of AD was associated with sleep disturbance and behavioral problems.

Colonization of the newborn respiratory tract and its association with respiratory morbidity in the first 6 months of life: A prospective cohort study.

OBJECTIVES: We aimed to determine the association between newborn bacterial colonization and infant respiratory morbidity in the first 6 months of life. METHODS: This prospective study included healthy newborn infants. Nasopharyngeal swabs performed within 72 hours of delivery were analyzed using polymerase chain reaction. We assessed cumulative respiratory morbidity of infants at 6 months. RESULTS: A total of 426 mother-infant pairs were recruited. In 53.3% (n = 225) of newborns, Streptococcus pneumoniae (46%) and Staphylococcus aureus (7.3%) were isolated. None had Haemophilus influenzae nor Moraxella catarrhalis. At the age of 6 months, 50.7% of infants had experienced respiratory symptoms, 25% had unscheduled doctor visits, and 10% were treated with nebulizers. Colonization with S. pneumoniae was associated with reduced risk of any respiratory symptom (adjusted odds ratio [aOR] 0.39, 95% confidence interval [CI] 0.16, 0.50), unscheduled doctor visits (aOR 0.35; 95% CI 0.18, 0.67), and nebulizer treatment (aOR 0.23, 95% CI 0.07, 0.72) at 6 months. Pregnancy-induced hypertension was also associated with increased need for nebulizer treatment (aOR 9.11, 95% CI 1.43, 58.1). CONCLUSION: Colonization of the newborn respiratory tract occurred in 53% of infants. S. pneumoniae was the most common organism, and this was associated with a reduced risk for respiratory morbidity at 6 months of life.

Maternal diet during pregnancy and infant respiratory morbidity: A prospective study.

BACKGROUND AND OBJECTIVES: Maternal diet during pregnancy may impact infant respiratory morbidity. The aim was to determine the association between antenatal maternal diet and respiratory morbidity of their infants during their first 6 months of life. METHODS AND STUDY DESIGN: This prospective cohort study included healthy motherinfant pairs. Maternal diet during the last trimester was determined with a validated food frequency questionnaire. Infant respiratory morbidity was solicited at 1, 3 and 6 months. RESULTS: Three hundred mother-baby pairs were recruited. Maternal consumption of milk and dairy products was associated with reduced respiratory symptoms at 1 month (aOR 0.29 [95% CI: 0.10, 0.86], p=0.03) and 3 months old (aOR 0.43 [95% CI: 0.20, 0.93], p=0.03), while intake of confectionery items was associated with increased unscheduled doctor visits at 3 months (aOR 2.01 [95% CI 1.33, 3.06], p=0.001) and increased nebuliser treatment at both 3 months (aOR 1.88 [95% CI 1.12, 3.17], p=0.02) and 6 months (aOR 1.64 [95% CI 1.05, 2.54], p=0.03). Finally, at 6 months, hypertensive disorders during pregnancy was associated with increased nebuliser treatment (aOR 17.3 [95% CI 1.50, 199], p=0.02) while exclusive breastfeeding was associated with reduced incidence of respiratory symptoms (OR 0.47 [95% CI 0.26, 0.83], p=0.01). CONCLUSIONS: Increased antenatal maternal consumption of milk and dairy products may reduce respiratory morbidity while increased consumption of confectionery items may increase respiratory morbidity in their infants during the first 6 months of life.

Respiratory sequelae and quality of life in children one-year after being admitted with a lower respiratory tract infection: A prospective cohort study from a developing country.

INTRODUCTION: Respiratory tract infections in children can result in respiratory sequelae. We aimed to determine the prevalence of, and factors associated with persistent respiratory sequelae 1 year after admission for a lower respiratory tract infection (LRTI). METHODOLOGY: This prospective cohort study involved children 1 month to 5-years-old admitted with an LRTI. Children with asthma were excluded. Patients were reviewed at 1-, 6-, and 12-months post-hospital discharge. The parent cough-specific quality of life, the depression, anxiety, and stress scale questionnaire and cough diary for 1 month, were administered. Outcomes reviewed were number of unscheduled healthcare visits, respiratory symptoms and final respiratory diagnosis at 6 and/or 12 month-review by pediatric pulmonologists. RESULTS: Three hundred patients with a mean ± SD age of 14 ± 15 months old were recruited. After 1 month, 239 (79.7%) returned: 28.5% (n = 68/239) had sought medical advice and 18% (n = 43/239) had cough at clinic review. Children who received antibiotics in hospital had significantly lower total cough scores (P = .005) as per the cough diary. After 1 year, 26% (n = 78/300) had a respiratory problem, predominantly preschool wheezing phenotype (n = 64/78, 82.1%). Three children had bronchiectasis or bronchiolitis obliterans. The parent cough-specific quality of life (PCQOL) was significantly lower in children with respiratory sequelae (P < .01). In logistic regression, the use of antibiotics in hospitals (adjusted odds ratio, 0.46; P = .005) was associated with reduced risk of respiratory sequelae. CONCLUSION: In children admitted for LRTI, a quarter had respiratory sequelae, of which preschool wheeze was the commonest. The use of antibiotics was associated with a lower risk of respiratory sequelae.