A case of Prader-Willi Syndrome that monitoring with NAVA was useful for diagnosis and respiratory management

Nakamura Nami, Mori Mari, Takeshita Satoru, Kakita Hiroki, Yamada Yasumasa

doi:10.34456/jjspnm.60.1_151

Bibliographic Information

Other Title

NAVAでのモニタリングが診断と呼吸管理に有用であったPrader-Willi症候群の一例

Abstract

<p> Prader-Willi syndrome（PWS）is a congenital disorder characterized by hypotonia in neonatal period. However, few reports have described cases requiring mechanical ventilation. We report here a case of PWS in which electrical activity of the diaphragm（Edi）on neurally adjusted ventilatory assist（NAVA）was eﬀective in the diagnosis and respiratory management The patient, a female born at 40 weeks and 2 days of gestational age with a birth weight of 2,678 g, was admitted to the hospital after birth with hypotonia and hypoxia. Mechanical ventilation was initiated on day 8, followed by respiratory management of NAVA on day 19. Notably, Edi remained regular within normal range and gradually decreasing. Extubation was performed on day 34, transitioning to non-invasive ventilation. Complete weaning from the respiratory support was achieved on day 54. Genetic testing revealed a diagnosis of PWS. In this case, Edi exhibited regularity and remained within normal range, indicating no evidence of central respiratory disorders or neuromuscular diseases. Edi was considered to be eﬀective in the diagnosis of PWS and as an indicator of respiratory management.</p>

Journal

Journal of Japan Society of Perinatal and Neonatal Medicine

Journal of Japan Society of Perinatal and Neonatal Medicine 60 (1), 151-154, 2024

Japan Society of Perinatal and Neonatal Medicine

Keywords

Details 詳細情報について

CRID: 1390863041926428416

DOI: 10.34456/jjspnm.60.1_151

ISSN: 24354996; 1348964X

Text Lang: ja

Data Source

JaLC

Abstract License Flag: Disallowed

Export