Clinical Features of COVID-19 in Patients with Congenital Central Hypoventilation Syndrome.

Background: The clinical course of COVID-19 in patients with congenital central hypoventilation syndrome (CCHS) is unknown. Methods: We conducted a cross-sectional questionnaire study in 43 patients with CCHS who had COVID-19. Results: The median age of patients was 11 [interquartile range (IQR) 6-22] years and 53.5% required assisted ventilation (AV) through tracheostomy. Disease severity ranged from asymptomatic infection (12%) to severe illness with hypoxemia (33%) and hypercapnia requiring emergency care/hospitalization (21%), increased AV duration (42%), increased ventilator settings (12%), and supplemental oxygen demand (28%). The median duration to return to baseline AV (n = 20) was 7 (IQR 3-10) days. Patients with polyalanine repeat mutations required increased AV duration compared with those with nonpolyalanine repeat mutations (P = 0.048). Patients with tracheostomy required increased oxygen during illness (P = 0.02). Patients aged ≥18 years took longer to return to baseline AV (P = 0.04). Conclusions: Our study suggests that all patients with CCHS should be vigilantly monitored during COVID-19 illness.

[Development and progress in mechanical ventilation]. / Entwicklung und Fortschritt in der Beatmungsmedizin.

Mechanical ventilation comprises two important sectors: 1) the acute application in intensive care medicine and 2) long-term mechanical ventilation, i.e. home mechanical ventilation. Today, based on increasing scientific evidence the approach to mechanical ventilation is highly sophisticated and this is true for both sectors. For this reason, several guidelines have been established in Germany with specific emphasis on mechanical ventilation. In addition, the current development of mechanical ventilation in Germany is influenced by the corona pandemic and also by the economization of the healthcare system. Notably, home mechanical ventilation is steadily increasing, while intensive care unit (ICU) capacities are currently decreasing. The current article focuses on these developments.

Mechanical ventilation in Duchenne muscular dystrophy: A pilot project in Ukraine.

BACKGROUND: Home mechanical ventilation (HMV) prolongs survival in patients with Duchenne muscular dystrophy (DMD) until ±35 years of age. This study evaluates the implementation of a HMV pilot project in children with DMD in Ukraine. METHODS: Children with DMD were invited to Kirovograd Regional Children's Clinical Hospital, Kropyvnytskyi, Ukraine, for 5 days' training with non-invasive ventilation. Donated equipment comprised second-hand Covidien PB560 ventilators from Belgium. Due to the absence of carbon dioxide pressure and pulse oximetry monitoring, indications for HMV included sleep-related symptoms, restrictive lung function test, loss of ambulation for more than 1 year, or age greater than 17 years. Master class lectures on HMV were conducted for Ukrainian doctors in conjunction with patient training. RESULTS: Twelve Ukrainian physicians took part in face-to-face master classes and 50 Ukrainian physicians participated in online master classes. Simultaneously, eight Duchenne inpatients, mean age 15.4 (SD: 1.8) years and body mass index 25.8 (SD: 4.0), were included in the study. All patients chose nasal masks and volume-pressure-assisted control mode. After 6 weeks, one patient stopped HMV, two others used HMV partially during sleep, and 5/8 used nocturnal HMV increasingly with few complaints. Follow up via phone call was organized after hospitalization. CONCLUSIONS: Implementation of HMV is feasible in DMD inpatients in Ukraine. In the short term, the Ukrainian parliament should recognize official centers for HMV, and define the funding policy of equipment for HMV, and its maintenance. Local distributors should deliver equipment for HMV and devices for monitoring carbon dioxide pressure and pulse oximetry in specialized centers for HMV.