Resumen

GIMENEZ, Gloria Concepción et al. Decannulation in a Pediatric Patient with Neuromuscular Disease. Med. clín. soc. [online]. 2021, vol.5, n.2, pp.106-110. ISSN 2521-2281.  https://doi.org/10.52379/mcs.v5i2.167.

Multiminicores disease is a hereditary neuromuscular disorder characterized by the presence of multiple 'nuclei' on muscle biopsy and clinical features of a congenital myopathy. The present case concerns a 10-year-old patient, diagnosed with multiminicores disease, tracheostomized since she was seven due to failed weaning and muscle weakness. The patient was referred to the Department of Cardio-respiratory Rehabilitation of the Clínicas Hospital from the National University of Asunción, presenting in her first evaluation chronic oxygen dependence (for more than 12 months), weak and non-functional cough with cough peak flow less than 160 L / m, chronic respiratory failure and hypercapnia (52 mmHg CO2ET). We did the follow-up in order to the Respiratory Rehabilitation guidelines contemplated in the Project for the evaluation, treatment and follow-up of patients with Neuromuscular Diseases, which was approved by the Superior Council of the Medical Sciences School from the National University of Asunción, these guidelines are based on scientific studies and publications done by Dr. John Bach (Rudgers University, Newart, New Jersey-USA) and his collaborating team from the Ibero-American Group for Respiratory Care in Neuromuscular Diseases. As a result, the patient was successfully decannulated, in an outpatient clinic, without hemodynamic decompensations, with excellent tolerance and without the requirement of hospital admissions.

Palabras clave : Neuromuscular Disease; Tracheotomy; Respiratory Rehabilitation; Decannulation; Multicores Disease..

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