Chest ACCP Education Calendar
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Guest Access | Sign In via User Name/Password
This Article
Right arrow Full Text Free
Right arrow Full Text (PDF) Free
Right arrow Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Article Archive
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via ISI Web of Science (13)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Ioos, C.
Right arrow Articles by Estournet-Mathiaud, B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Ioos, C.
Right arrow Articles by Estournet-Mathiaud, B.
(Chest. 2004;126:831-837.)
© 2004 American College of Chest Physicians

Respiratory Capacity Course in Patients With Infantile Spinal Muscular Atrophy*

Christine Ioos, MD; Danièle Leclair-Richard, MD; Slah Mrad, MD; Annie Barois, MD and Brigitte Estournet-Mathiaud, MD

* From the Department of Pediatric Neurology, Hôpital Raymond Poincaré, Garches, France.

Correspondence to: Christine Ioos, MD, Department of Pediatric Neurology, Hôpital Raymond Poincaré, 104, Boulevard Raymond Poincaré, 92380 Garches, France 78-83; e-mail: christine.ioos{at}rpc.ap-hop-paris.fr

Study objectives: To describe the clinical and respiratory course in infantile spinal muscular atrophy (SMA) type I, type II, and type III, and to evaluate the respiratory needs for these patients, using noninvasive or tracheostomy ventilation.

Design: Retrospective cohort study.

Methods: We report 33 patients with SMA true type I (onset before age 3 months), 35 patients with SMA intermediate type I (onset between 3 months and 6 months), 100 patients with SMA type II (onset between 6 months and 18 months), 12 patients with SMA type III (onset after age 18 months). We report the clinical symptoms, respiratory course, and respiratory management: respiratory physiotherapy, periodic hyperinsufflation, nasal nocturnal ventilation (NNV), and tracheostomy. Also, we measured the FVC over several years during childhood and adolescence.

Results: In patients with SMA true type I, 82% of patients died, one third of whom underwent tracheostomy. In patients with SMA intermediate type I, 43% needed NNV, 57% underwent tracheostomy, and 26% died. In patients with SMA type II, 38% needed NNV, 15% underwent tracheostomy, and 4% died. In patients with SMA type III, respiratory impairment was moderate and began during the second decade of life.

Conclusion: This data shows the progressively worsening course of restrictive respiratory insufficiency in patients with SMA, and the importance of early respiratory management to limit pulmonary complications and improve the quality of life for these patients.

Key Words: children • FVC • nasal nocturnal ventilation • periodic hyperinsufflation • respiratory insufficiency • spinal muscular atrophy • tracheostomy




This article has been cited by other articles:


Home page
 ChestHome page
J. R. Bach and C. Ioos
There Are Other Ways to Manage Spinal Muscular Atrophy Type 1
Chest, April 1, 2005; 127(4): 1463 - 1464.
[Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2004 by the American College of Chest Physicians.