Predictors of Katz et al for Nocturnal Hypoventilation in a Pediatric Patient with Progressive Neuromuscular Disease

Patient selection: pediatric patient with a progressive neuromuscular disease

Progressive neuromuscular diseases include: spinal muscular atrophy, congenital myotonia, congenital muscular dystrophy, nemaline myopathy, metabolic myopathy)

Outcome: nocturnal hypoventilation

Predictors:

(1) FVC less than 70% of predicted

(2) FEV1 less than 65% of predicted

(3) scoliosis

Patients who develop nocturnal hypoventilation will develop:

(1) a greater increase in RV to TLV ratio (gas trapping)

(2) decline in muscle strength

(3) worsened perception of health status