Chronic Respiratory Failure in a Case with Juvenile-Onset Acid .ALPHA.-Glucosidase Deficiency; Successful Therapy with Nasal Intermittent Positive Pressure Ventilation(NIPPV).

DOI PubMed 1 Citations 10 References
  • Tanaka Chitose
    Division of Child Neurology, Institute of Neurological Sciences, Tottori University, Faculty of Medicine
  • Maegaki Yoshihiro
    Division of Child Neurology, Institute of Neurological Sciences, Tottori University, Faculty of Medicine

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Other Title
  • ＣＯ２ナルコーシスに対して夜間経鼻間欠的陽圧人工換気（ＮＩＰＰＶ）が有効であった酸性α‐グルコシダーゼ欠損症（若年発症型）の１例

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A 13-year-old boy with juvenile-onset acid a glucosidase deficiency was reported. Proximal muscle weakness including respiratory muscles and scoliosis progressed since nine year of age. He developed nocturnal dyspnea and daytime somnolence at age 13. His arterial blood gas analysis showed hypoxemia (PO, 54.1 mmHg) and hypercapnia (PCO2 72.3mmHg), and spirometry showed significantly decreased vital capacity (%VC 21%). He was treated with nocturnal NIPPV employing a device for delivering bilevel positive airway pressure (Bi-PAP). Nocturnal dyspnea and daytime somnolence rapidly disappeared with nocturnal ventilatory support. Daytime arterial P02 and PCO2 improved after the therapy, namely 74.8 mmHg and 64.1 mmHg respectively. We conclude that NIPPV is a noninvasive and effective therapy for respiratory failure in patients with chronic progressive neuromuscular disorder including acid a glucosidase deficiency.

Journal

  • NO TO HATATSU

    NO TO HATATSU 29 (1), 51-54, 1997

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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