Rationale: There are limited data on mechanical discontinuation practices in Asia. Objectives: To document self-reported mechanical discontinuation practices and determine whether there is clinical equipoise regarding protocolized weaning among Asian Intensive Care specialists. Methods: A survey using a validated questionnaire, distributed using a snowball method to Asian Intensive Care specialists. Results: Of the 2,967 invited specialists from 20 territories, 2,074 (69.9%) took part. The majority of respondents (60.5%) were from China. Of the respondents, 42% worked in intensive care units (ICUs) where respiratory therapists were present; 78.9% used a spontaneous breathing trial as the initial weaning step; 44.3% frequently/always used pressure support (PS) alone, 53.4% intermittent spontaneous breathing trials with PS in between, and 19.8% synchronized intermittent mandatory ventilation with PS as a weaning mode. Of the respondents, 56.3% routinely stopped feeds before extubation, 71.5% generally followed a sedation protocol or guideline, and 61.8% worked in an ICU with a weaning protocol. Of these, 78.2% frequently always followed the protocol. A multivariate analysis involving a modified Poisson regression analysis showed that working in an ICU with a weaning protocol and frequently/always following it was positively associated with an upper-middle-income territory, a university-affiliated hospital, or in an ICU that employed respiratory therapists; and negatively with a low-income or lower-middle-income territory or a public hospital. There was no significant association with "in-house" intensivist at night, multidisciplinary ICU, closed ICU, or nurse-patient ratio. There was heterogeneity in agreement/disagreement with the statement, "evidence clearly supports protocolized weaning over nonprotocolized weaning." Conclusions: A substantial minority of Asian Intensive Care specialists do not wean patients in accordance with the best available evidence or current guidelines. There is clinical equipoise regarding the benefit of protocolized weaning.
Objectives: To determine whether children with neuromuscular disorders using long-term noninvasive ventilation (NIV), continuous or bilevel positive airway pressure, have improved health outcomes compared with alternative treatment strategies. Data Sources: This systematic review is an extension of a scoping review. The search strategy used Medical Subject Headings and free-text terms for "child" and "noninvasive ventilation." Studies of humans from 1990 onward were searched in MEDLINE (Ovid), Embase (Ovid), CINAHL (Ebsco), Cochrane Library (Wiley), and PubMed. The results were reviewed for articles reporting on neuromuscular disorders and health outcomes including mortality, hospitalization, quality of life, lung function, sleep study parameters, and healthcare costs. Data Extraction: Extracted data included study design, study duration, sample size, age, type of NIV, follow-up period, primary disease, and primary and secondary outcome measures. Studies were grouped by primary disease into three groups: spinal muscular atrophy, Duchenne muscular dystrophy, and other/multiple neuromuscular diseases. Data Synthesis: A total of 50 articles including 1,412 children across 36 different neuromuscular disorders are included in the review. Mortality is lower for children using long-term NIV compared with supportive care across all neuromuscular disease types. Overall, mortality does not differ when comparing the use of NIV with invasive mechanical ventilation, though heterogeneity suggests that mortality with NIV is higher for spinal muscular atrophy type 1 and lower for other/multiple neuromuscular diseases. The impact of long-term NIV on hospitalization rate differed by neuromuscular disease type with lower rates compared with supportive care but higher rates compared with supportive care use for spinal muscular atrophy type 1, and lower rates compared with before NIV for other/multiple neuromuscular diseases. Overall, lung function was unaltered and sleep study parameters were improved from baseline by long-term NIV use. There are few data to assess the impact of long-term NIV use on quality of life and healthcare costs. Conclusions: Long-term NIV for children provides benefit for mortality, hospitalizations, and sleep study parameters for some sub-groups of children with neuromuscular disorders. High risk of bias and low study quality preclude strong conclusions.
Rationale: Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed.Objectives: To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.Methods: We performed a retrospective chart review of 199 deidentified patients with NEHI from 11 centers. Data were collected in a centralized Research Electronic Data Capture registry and we performed descriptive statistics.Results: The majority of patients with NEHI were male (66%). The sensitivity of the NEHI Clinical Score was 87% (95% confidence interval [CI], 0.82-0.91) for all patients from included centers and 93% (95% CI, 0.86-0.97) for those with complete scores (e.g., no missing data). Findings were similar when we limited the population to the 75 patients diagnosed by lung biopsy (87%; 95% CI, 0.77-0.93). Of those patients evaluated for comorbidities, 51% had gastroesophageal reflux, 35% had aspiration or were at risk for aspiration, and 17% had evidence of immune system abnormalities.Conclusions: The NEHI Clinical Score is a sensitive tool for clinically evaluating NEHI; however, its specificity has not yet been addressed. Clinicians should consider evaluating patients with NEHI for comorbidities, including gastroesophageal reflux, aspiration, and immune system abnormalities, because these can contribute to the child's clinical picture and may influence clinical course and treatment.