A case report of lung hypoplasia in the patient with limb girdle dystrophy

limb-girdle muscular dystrophy (LGMD) is a hereditary myopathy that is defined by proximal muscle weakness and atrophy. There are two major types of LGMD, type one autosomal dominant and type two autosomal recessive.Pulmonary manifestation in muscular dystrophy included infections, ventilator failure and pulmonary hypoplasia.ventilatory failure is more variable in LGMD compared to other dystrophies. Respiratory muscle strength is commonly impaired in LGMD leading to restrictive lung disease. The common manifestations are dyspnea on exertion, chronic cough and recurrent pulmonary infection. No study has been published about pulmonary complication in LGMD, it may be due to low prevalence of disorderand case slection. Incidence of pulmonary hypoplasia is about 14 per 10,000 births and also is a common cause of neonatal death.

کلیدواژه ها:

Lung hypoplasia ، dystrophy ، embolism ، dyspnea