Non-invasive APV in a child with the syndrome of central alveolar hypoventilation

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Abstract

Introduction: the syndrome of congenital central alveolar hypoventilation (SCCAH) is characterized by disturbed autonomic control of breathing (most frequently, while sleeping) in the structure of the autonomic nervous system general dysfunction. SCCAH is an orphan disease. In Russia, 29 children were diagnosed the same in 2018 (based on data provided by the Association of parents who have children with SCCAH). Purpose: successful experience of using non-invasive pulmonary ventilation as a method of restoring the central respiratory function in a child with SCCAH is of clinical interest. Materials and methods: we present a clinical case of managing a child with genetically confirmed SCCAH from the pediatric department of anesthesiology and resuscitation at Almazov National Medical Research Center prior to discharge. Conclusion: in the presented case, stable gas exchange was achieved against the background of 18-day staged adaptation to non-invasive pulmonary ventilation while sleeping only. The adaptation was selected as a method of chronic respiratory support. The child was discharged from the hospital at the age of 4 months and 14 days in a stable condition having equipment for respiratory support and monitoring. In every case of SCCAH, selection of additional ventilation method must be individually tailored.

About the authors

D. V. Gorshkov

Almazov National Medical Research Centre

Author for correspondence.
Email: dmitrijgors21@yandex.ru

Dmitrii V. GORSHKOV – Anesthesiologist‑resuscitator of Department of Anaesthesiology and Resuscitation for children

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341
phone: +7(812)702–37–49 

Россия

D. V. Novoseltsev

Almazov National Medical Research Centre

Email: fake@neicon.ru

Dmitrii V. NOVOSELTSEV – Head of Department of Anaesthesiology and Resuscitation for children

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341

Россия

M. A. Pritsan

Almazov National Medical Research Centre

Email: fake@neicon.ru

Mariya A. PRITSAN – Anesthesiologist‑resuscitator of Department of Anaesthesiology and Resuscitation for children

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341

Россия

N. A. Petrova

Almazov National Medical Research Centre

Email: fake@neicon.ru

Natal’ya A. PETROVA – Ph D., assistant of the Faculty of children’s diseases

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341

Россия

N. Yu. Kolbina

Almazov National Medical Research Centre

Email: fake@neicon.ru

Natal’ya Yu. KOLBIN – Head of Department of Pediatrics and Rehabilitation № 1

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341

Россия

M. S. Shchekoldina

Almazov National Medical Research Centre

Email: fake@neicon.ru

Margarita S. SHCHEKOLDINA – Pediatrician of Department of Pediatrics and Rehabilitation № 1

Kolomyazhsky Av., 21, b.2, Saint‑Petersburg, 197341

Россия

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Copyright (c) 2019 Gorshkov D.V., Novoseltsev D.V., Pritsan M.A., Petrova N.A., Kolbina N.Y., Shchekoldina M.S.

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