Janus kinase inhibition in induction treatment of anti-MDA5 juvenile dermatomyositis-associated rapidly progressive interstitial lung disease.

Tofacitinib has an important role in pediatric rapidly progressive interstitial lung disease (ILD) associated with juvenile dermatomyositis (JDM), an otherwise potentially fatal condition. It may be useful in induction of remission and can be used safely to maintain remission. Serum ferritin and interleukin-18 are useful markers for tracking activity and response of JDM-associated ILD.

Impact of work routines on parents' and children's sleep during the COVID-19 pandemic lockdown.

OBJECTIVE: To evaluate the effects of parental sleep and work arrangements on children's sleep duration during the national lockdown period, referred to as 'Circuit Breaker' (CB), due to COVID-19. METHODS: Cross-sectional, anonymous, online questionnaire to parents with school-going children aged between 3 and 16 years. Child and parental sleep duration in relation to change in parental work arrangements, housing type and number of individuals in the household as reported by parents were evaluated. Descriptive statistics and tests of comparison were used to evaluate data. RESULTS: School-going children (n = 593) had a mean age of 8.68 (SD = 3.65; median 7) years. Both, fathers and mothers had gains in sleep during CB (based on self-reported sleep data), compared to pre-CB. Change in both maternal and paternal sleep duration positively correlated with change in child sleep duration (based on parent-reported sleep data) among all children (r2 = 0.27, p < 0.001 and r2 = 0.17, p < 0.001 respectively); pre-schoolers mirrored their mothers' sleep more closely. Parents who changed to working from home during the CB (compared to working from outside home previously) had the greatest gains in sleep during this period. Housing type was not significantly associated with change in child sleep duration from pre-CB to CB. CONCLUSIONS: Greater gains in sleep in parents was associated with working from home during CB. Child sleep duration mirrored gains in parental sleep, especially in pre-school and primary-school-going children. Optimising parental sleep may therefore be one of the means to improve child sleep.

School closure during the coronavirus disease 2019 (COVID-19) pandemic - Impact on children's sleep.

OBJECTIVE: To evaluate the sleep habits of school-going children before and during school closure in the national lockdown period (called 'Circuit Breaker' or CB in Singapore) due to the COVID-19 pandemic. METHODS: Cross-sectional, anonymous, online, population-based survey questionnaire was administered to parents aged 21 years and above with children aged between 3 and 16 years attending pre-school, primary or secondary school (equivalent to kindergarten, middle and-high school) and residing in Singapore. Sleep duration in relation to various daily activities including academic activities, physical exercise, and screen time was evaluated pre-CB and during CB. RESULTS: Data from 593 participants were analyzed. Pre-CB, the overall mean (SD) sleep duration of the study population was 9.01 (1.18) hours on weekdays and 9.99 (0.94) hours on weekends. During CB, mean (SD) sleep duration overall was 9.63 (1.18) hours. Although children generally went to bed later (mean 0.65 h later), they woke up even later during CB (mean 1.27 h later), resulting in longer sleep duration (mean increase of 0.35 h). This was most evident in secondary school children (mean increase of 0.70 h). Children attending private schools (which had later start times) had increased sleep duration (mean 10.01 (SD 0.89) hours pre-CB and 10.05 (SD 0.93) hours during CB) compared to public schools (mean 9.05 (SD 0.91) pre-CB and 9.49 (SD 1.22) hours during CB). CONCLUSIONS: School closure from the COVID-19 pandemic resulted in longer sleep duration in school-going children. Early school/academic activity start times had a significant impact on limiting children's sleep duration.

Rare Lung Diseases: Interstitial Lung Diseases and Lung Manifestations of Rheumatological Diseases.

The concept of Childhood Interstitial Lung Disease (ChILD) is relatively young. There has been tremendous progress in this field in the last decade. The key advance has been the recognition of interstitial lung diseases that are often distinct and occur mainly in infants. Diagnosis is challenging because the incidence is low and no single center in the world has enough cases to promote experience and clinical skills. This has led to formation of international groups of people interested in the field and the "Children's interstitial and diffuse lung disease research network" (ChILDRN) is one such group which contributed to the progress of this field. Clinically, these disorders overlap with those of other common respiratory disorders. Hence, clinical practice guidelines emphasize the additional role of chest imaging, genetic testing and lung biopsy in the diagnostic evaluation. Genetic testing, in particular, has shown tremendous progress in this field. Being noninvasive, it has the potential to help early recognition in a vast majority. Despite progress, definitive therapeutic modalities are still lacking and supportive care is still the backbone of management in the majority. Early recognition of the definitive diagnosis helps in the management, even if, in a significant number, it helps in avoiding unnecessary therapy. Also discussed in this article, is the pulmonary manifestation of rheumatic diseases in children. The incidence and spectrum of pulmonary involvement in rheumatic conditions vary and can be result of the primary disease or its management or due to an concurrent infection.

Pediatric polysomnographic studies at a tertiary-care hospital in Singapore.

OBJECTIVES: To investigate the indications for conducting polysomnography studies and their outcomes. METHODS: Retrospective analysis of pediatric polysomnography studies performed over a four-year period (2009-2012). RESULTS: 425 diagnostic studies and 100 non-invasive positive-pressure ventilation titration studies were conducted. Of these, 389 were performed in male children. Obstructive sleep apnea was the most common diagnosis; 49.6% (211 studies). Other diagnoses included central apnea, narcolepsy, and periodic limb movement disorder. Night time symptoms (snoring, frequent night awakenings, restless sleep) were present in 294 children, and 161 children had daytime symptoms (excessive daytime sleepiness, early morning fatigue, poor concentration at school). 13 studies (2.5%) were inadequate for analysis, reflecting the challenges of conducting studies in children. CONCLUSION: Dedicated pediatric sleep laboratories with properly trained staff are important to minimize failure rates and diagnose these conditions accurately.

Specific antibody deficiency in children with chronic wet cough.

The prevalence and clinical significance of specific polysaccharide antibody deficiency (SAD) in children are poorly understood. The authors sought to determine the prevalence of SAD in children with chronic wet cough, through a retrospective study of all children with chronic wet cough attending our tertiary respiratory clinic over a 12-month period. Antibody levels to 13 pneumococcal serotypes were measured following vaccination with the unconjugated pneumococcal polysaccharide vaccine, Pneumovax II, and clinical data were reviewed. Twenty-four children over 2 years of age with chronic wet cough were vaccinated. Fourteen (58%) failed to mount an adequate antibody response, consistent with SAD. Children with SAD were more likely than children with normal antibody responses to require intravenous antibiotics (p=0.035) and to have abnormal chest radiographs (p=0.029). The authors conclude that SAD is present in a significant number of children with chronic wet cough. The clinical significance and long-term outcome of SAD warrant further investigation in prospective studies.

A prospective review of adverse events during interhospital transfers of neonates by a dedicated neonatal transfer service.

OBJECTIVES: To categorize and quantify adverse events occurring during emergency interhospital transfers performed by a specialized neonatal retrieval team and to assign levels of associated risk. DESIGN: Prospective review of adverse events during emergency interhospital transfers of neonates by the London Neonatal Transfer Service over a 6-month period. The events were categorized based on an adapted retrieval team model from the Paediatric & Neonatal Safe Transfer and Retrieval Course (PANSTAR). Risk levels were measured using a modified risk assessment score. SETTING: Emergency interhospital transfers by a specialized neonatal retrieval team. PATIENTS: Patients were 346 emergency neonatal transfers over 6 months. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We found that 125 transfers (36.1%) had at least one adverse event. There were 205 adverse events in total; 139 events (67%) were perceived as being due to avoidable human errors. Almost a third of events (30%) occurred even before the retrieval team arrived at the referring hospital and made contact with the patient. The largest group of events occurred due to problems in preparation (n = 69) and communication (n = 49). Most events (n = 143) had insignificant impacts on patients, but six events could have potentially caused major harm. CONCLUSIONS: Adverse events commonly occur during neonatal transfers, even if performed by a dedicated transfer service. Early identification of potentially harmful episodes is important. Human error is likely to be a factor in the majority of adverse events; hence, opportunities should be taken to reduce the number of these through education, training, and risk management.