Enfermedad respiratoria y parálisis diafragmática en la enfermedad de Charcot-Marie-Tooth

Ódena, M. Pons; Marimbaldo, I. Piqueras; Oferil, J. Colomer; Matute, S. Segura; Rico, A. Palomeque

doi:10.1016/j.anpedi.2009.11.017

Cited by 1 publication

(1 citation statement)

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“…On the other hand, the incidence of lung infection and bronchial aspiration was higher in Group II than in Group III, although without significance. As described by Pons et al [33], Group II patients frequently present phrenic nerve paralysis, which per se causes respiratory insufficiency. In order to address patients in this group, Darquennes et al [34] have recommended the use of nocturnal NIV to treat respiratory disease, as with Group I.…”

Section: Discussionmentioning

confidence: 78%

Experience in neuromuscular diseases in children and adolescents and their comorbidities in a tertiary hospital

et al. 2021

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Introduction Neuromuscular diseases include a large group of heterogeneous and rare pathologies that affect different components of the motor unit. It is essential to optimize resources to know the prevalence of comorbidities in the most frequent groups to establish an early multidisciplinary approach in a specialized setting. Patients and methods Retrospective descriptive study of pediatric and adolescent patients with neuromuscular diseases (NMDs). The Inclusion criteria were NMDs patients with motor neuron involvement divided into three groups, depending on the affected component of the motor unit. Group I: involvement of the motor neuron; Group II: peripheral neuropathies; Group III: myopathies. Demographic variables, association with comorbidities, need for respiratory support, and rehabilitative treatment were collected in each group. Results Ninety-six patients who met the inclusion criteria were studied. In group I, when compared to the other two groups, a higher incidence of scoliosis (68.3%, p = 0.011), deformity of the rib cage (31.3%, p = 0.0001), chronic respiratory insufficiency (62.5%, p = 0.001) and bronchial aspiration (12.5%, p = 0.03) was detected. In this group, 50%of the patients required non-invasive mechanical ventilation (p = 0.0001). The in-hospital requirement for respiratory physiotherapy was higher in group I (75%, p = 0.001). We observed a higher incidence of scoliosis in Group III compared to Group II. Conclusions Neuromuscular diseases with motor neuron involvement present more comorbidities and require an early approach after diagnosis to improve prognosis.

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Section: Discussionmentioning

confidence: 78%