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Background and objective: Aerosol therapies are widely used for mechanically ventilated patients. However, the practice pattern of aerosol therapy in mainland China remains unknown. This study aimed to determine the current practice of aerosol therapy in mainland China. Methods: A web-based survey was conducted by the China Union of Respiratory Care (CURC) from August 2018 to January 2019. The survey was disseminated via Email or WeChat to members of CURC. A questionnaire comprising 16 questions related to hospital information and 12 questions related to the practice of aerosol therapy. Latent class analysis was employed to identify the distinct classes of aerosol therapy practice. Main results: A total of 693 valid questionnaires were returned by respiratory care practitioners from 447 hospitals. Most of the practitioners used aerosol therapy for both invasive mechanical ventilation (90.8%) and non-invasive mechanical ventilation (91.3%). Practitioners from tertiary care centers were more likely to use aerosol therapy compared with those from non-tertiary care centers (91.9% vs. 85.4%, respectively; p = 0.035). The most commonly used drugs for aerosol therapy were bronchodilators (64.8%) followed by mucolytic agents (44.2%), topical corticosteroids (43.4%) and antibiotics (16.5%). The ultrasonic nebulizer (48.3%) was the most commonly used followed by the jet nebulizer (39.2%), the metered dose inhaler (15.4%) and the vibrating mesh nebulizer (14.6%). Six latent classes were identified via latent class analysis. Class 1 was characterized by the aggressive use of aerosol therapy without a standard protocol, while class 3 was characterized by the absence of aerosol therapy. Conclusions: Substantial heterogeneity among institutions with regard to the use of aerosol therapy was noted. The implementation of aerosol therapy during mechanical ventilation was inconsistent in light of recent practice guidelines. Additional efforts by the CURC to improve the implementation of aerosol therapy in mainland China are warranted. [ABSTRACT FROM AUTHOR]

Objective: To systematically describe the short stature of patients with Diamond-Blackfan anemia and to explore factors affecting the height development of patients with Diamond-Blackfan anemia., Study Design: This cross-sectional study was conducted at the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, and the height, weight, and clinical data of 129 patients with Diamond-Blackfan anemia were collected from June 2020 to September 2020., Results: The median height-age-z score (HAZ) of children affected by Diamond-Blackfan anemia was -1.54 (-6.36-1.96). Short stature was found in 37.98% of the patients. Specific Diamond-Blackfan anemia growth curves were developed for weight, height, and body mass index, separately for male and female patients. Multivariable logistic regression models showed that female sex (aOR 4.92; 95% CI 1.29-18.71; P = .0195), underweight (aOR 10.41, 95% CI 1.41-76.98, P = .0217), cardiovascular malformations (aOR 216.65; 95% CI 3.29-14279.79; P = .0118), and RPL11(aOR 29.14; 95% CI 1.18-719.10; P = .0392) or RPS26 (aOR 53.49; 95% CI 1.40-2044.30; P = .0323) mutations were independent risk factors for short stature. In the subgroup of patients who were steroid-dependent, patients with a duration of steroid therapy over 2 years (OR 2.95; 95% CI 1.00-8.66; P = .0494) or maintenance dose of prednisone >0.1 mg/kg per day (OR 3.30; 95% CI 1.02-10.72; P = .0470) had a higher incidence of short stature., Conclusions: Patients with Diamond-Blackfan anemia had a high prevalence of short stature. The risk of short stature increased with age and was associated with sex, underweight, congenital malformations, and RPL11 or RPS26 mutations. The duration of steroid therapy and maintenance dose of steroid was significantly associated with the incidence of short stature in steroid-dependent patients with Diamond-Blackfan anemia., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Several studies on clinical practice for Duchenne muscular dystrophy (DMD) have been conducted in Western countries. However, there have been only a few similar studies in Asia and Oceania. Here, we investigate the steroid therapy-related clinical practice for DMD among the local experts. In 2015, we conducted a DMD expert survey in Asia and Oceania to acquire information regarding patients with DMD and to assess current clinical practice with the cooperation of Asian and Oceanian Myology Centre, a neuromuscular disease research network., Results: We obtained survey responses from 87 out of 148 clinicians (62%) from 13 countries and regions. In China, 1385 DMD patients were followed-up by 5 respondent neurologists, and 84% were between 0 and 9 years of age (15% were 10-19 years, 1% > 19 years). While in Japan, 1032 patients were followed-up by 20 clinicians, and the age distribution was similar between the 3 groups (27% were 0-9 years, 35% were 10-19 years, 38% were >19 years). Most respondent clinicians (91%) were aware of DMD standard of care recommendations. Daily prednisolone/prednisone administration was used most frequently at initiation (N = 45, 64%). Inconsistent opinion on steroid therapy after loss of ambulation and medication for bone protection was observed., Conclusions: Rare disease research infrastructures have been underdeveloped in many of Asian and Oceanian countries. In this situation, our results show the snapshots of current medical situation and clinical practice in DMD. For further epidemiological studies, expansion of DMD registries is necessary., (Copyright © 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Aim: To explore the optimal steroid therapeutic strategy for autoimmune pancreatitis (AIP)., Methods: This study was conducted retrospectively in two large institutions in China. Patients with clinically, radiologically and biochemically diagnosed AIP were enrolled. The performed radiological investigations and biochemical tests, the regimen of the given steroid treatment, remission and relapse whether with and without steroid therapy were analyzed., Results: Twenty-eight patients with AIP received steroid treatment, while 40 patients were treated surgically by pancreatoduodenectomy, distal pancreatectomy and choledochojejunostomy, radiofrequency ablation for the enlarged pancreatic head, percutaneous transhepatic biliary drainage and endoscopic biliary drainage. The starting oral prednisolone dose was 30 mg/d in 18 (64.3%) patients and 40 mg/d in 10 (35.7%) patients administered for 3 wk. The remission rate of AIP patients with steroid treatment (96.4%) was significantly higher than in those without steroid treatment (75%). Maintenance therapy (oral prednisolone dose 5 mg/d) was performed after remission for at least 6-12 mo to complete the treatment course. Similarly, the relapse rate was significantly lower in AIP patients with steroid treatment (28.6%) than in those without steroid treatment (42.5%). Steroid re-treatment was effective in all relapsed patients with or without steroid therapy., Conclusion: Steroid therapy should be considered in all patients with active inflammatory phase of AIP. However, the optimal regimen still should be trailed in larger numbers of patients with AIP.