The second wind phenomenon in very young McArdle’s patients

Pérez, Margarita; Ruiz, Jonatan R.; Fernández del Valle, María; Nogales-Gadea, Gisela; Andreu, Antoni L.; Arenas, Joaquín; Lucía, Alejandro

doi:10.1016/j.nmd.2009.04.010

« Previous Next »Neuromuscular Disorders
Volume 19, Issue 6 , Pages 403-405, June 2009

The second wind phenomenon in very young McArdle’s patients

Margarita Pérez
- Universidad Europea de Madrid (Polideportivo), 28670 Villaviciosa de Odón, Madrid, Spain
- These authors contributed equally to this work.
Jonatan R. Ruiz
- Department of Biosciences and Nutrition at NOVUM, Unit for Preventive Nutrition, Karolinska Institutet, Huddinge, Sweden
- These authors contributed equally to this work.
María Fernández del Valle
- Universidad Europea de Madrid (Polideportivo), 28670 Villaviciosa de Odón, Madrid, Spain
Gisela Nogales-Gadea
- Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Spain
Antoni L. Andreu
- Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Spain
Joaquín Arenas
- Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Spain
Alejandro Lucía
- Universidad Europea de Madrid (Polideportivo), 28670 Villaviciosa de Odón, Madrid, Spain
- Corresponding author. Tel.: +31 91 664 78 00; fax: +34 91 616 82 65.

Received 7 December 2008; received in revised form 10 April 2009; accepted 16 April 2009.

Abstract

We investigated the phenomenon of second wind in four patients with McArdle’s disease: a brother and sister (aged 4 and 12 years respectively) and two unrelated patients, a boy of 14 and a 17-year-old girl. We also studied the siblings’ healthy 6-year-old sister. Each patient performed a 15-min exercise test at a constant workload and a subsequent graded exercise test until exhaustion. Overall the healthy girl and the youngest McArdle’s patient, the 4-year-old boy, did not show a second wind phenomenon. Further, the peak cardio-respiratory capacity of the young McArdle’s boy was normal for his age (32.3mL02/kg/min) and he did not report any function limitations during physical education classes.

Keywords: Glycogenosis type V, Myophosphorylase deficiency, Exercise intolerance, Peak cardiorespiratory capacity, Children, Heart rate