Literature DB >> 23037578

[Late onset Ondine syndrome: literature review on a case report].

M Butin1, G Labbé, S Vrielynck, P Franco, B Massenavette, G Bellon, P Reix.   

Abstract

Ondine syndrome is the central congenital hypoventilation syndrome (CCHS) caused by the mutation of the PHOX2B gene. In late onset cases, the symptomatology often appears after an acute event (infection, general anesthesia, drug intake), increasing hypoventilation. We report a case of late onset Ondine curse in a 9-year-old girl. The diagnosis was made based on a hypercapnic coma complicating a respiratory infection caused by Mycoplasma pneumoniae and was confirmed by genetic testing. In the patient's history we found symptoms that had not been noted (e.g., enuresis, morning headache, adynamia), attesting to chronic hypoventilation. Through this observation, we review the literature on CCHS, notably late onset cases, which are rare and insidious, emphasizing the pre-existing hypoventilation symptoms in this child. This case underlines the need for all practitioners not to trivialize these symptoms so as to decrease the current delay in diagnosis for late onset CCHS and to introduce optimal care as soon as possible.
Copyright © 2012 Elsevier Masson SAS. All rights reserved.

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Year:  2012        PMID: 23037578     DOI: 10.1016/j.arcped.2012.08.015

Source DB:  PubMed          Journal:  Arch Pediatr        ISSN: 0929-693X            Impact factor:   1.180


  3 in total

1.  Late-onset congenital central hypoventilation syndrome and a rare PHOX2B gene mutation.

Authors:  Joana Magalhães; Núria Madureira; Rita Medeiros; Paula C Fernandes; Myriam Oufadem; Jeanne Amiel; M Helena Estêvão; M Guilhermina Reis
Journal:  Sleep Breath       Date:  2014-05-04       Impact factor: 2.816

2.  Unusual case of central alveolar hypoventilation.

Authors:  Hadil Ak AlOtair; Abdulaziz H Alzeer; Mohammed A Abdou; Shaden O Qasrawi
Journal:  Saudi Med J       Date:  2018-03       Impact factor: 1.484

3.  Sleep Apnea Syndrome after Posterior Fossa Surgery: A Case of Acquired Ondine's Curse.

Authors:  Elnaz Faraji Rad; Mohammad Faraji Rad; Shahram Amini; Reza Zare
Journal:  Iran J Otorhinolaryngol       Date:  2015-01
  3 in total

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