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Treating Sleep Disordered Breathing Resolves Failure to Thrive in Child with Congenital Myasthenia
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A5634 - Treating Sleep Disordered Breathing Resolves Failure to Thrive in Child with Congenital Myasthenia
Author Block: M. Hawkins, C. A. Canapari; Pediatric Respiratory Medicine, Yale University, New Haven, CT, United States.
Sleep disordered breathing (SDB) can both complicate and cause growth failure in children. A 4-year-old male was admitted to the hospital for a failure to thrive evaluation. Growth parameters show a weight of 10 kilograms at the age of 4.5 years. Pertinent findings were proximal muscle weakness, subtle chest wall asymmetry, and mild kyphoscoliosis. He was born healthy at term; there were no previous hospital admissions. He had never displayed respiratory difficulties and seemed to have a normal cough and activity tolerance. He sleeps from 10 p.m. to 7 a.m. daily. He was active with no concerns for sleepiness, fatigue, or headaches. He does have a history of gross motor developmental delay from infancy but was able to run. His appetite was normal and there was no history of feeding difficulty. Concern for congenital muscular dystrophy prompted a muscle biopsy which was normal. Sweat test was negative. A sleep study was obtained after discharge and revealed rapid eye movement (REM) related hypoventilation and severe obstructive sleep apnea (OSA) (apnea hypopnea index (AHI) 15/hr) with 29% of time asleep spent with oxygen saturations below 90% and severe episodic desaturations as low as 49%. Non-invasive bi-level positive airway pressure (BiPAP) was quickly initiated. He was subsequently diagnosed with congenital myasthenia gravis and started on pyridostigmine which he continues to take currently. Now at the age of 7 years he shows a weight of 21.9 kilograms and is at the 34th percentile for weight/age. His restrictive lung disease has not progressed since the initiation of BiPAP 15/5 while sleeping which he continues to use. Recent diagnostic sleep study was significant for mild hypoventilation with pCO2 50-55 mmHg and moderate OSA (AHI of 7.8) and oxygen desaturations with a nadir of 82%. Recent otolaryngology evaluation revealed small size of adenoids and tonsils, so surgery was deferred. This case illustrates the importance of screening for SDB despite the lack of purported sleep problems when there is concern for neuromuscular disease. Early initiation of BiPAP and as well as treatment of his underlying disease have resulted in improved growth and development.
Author Block: M. Hawkins, C. A. Canapari; Pediatric Respiratory Medicine, Yale University, New Haven, CT, United States.
Sleep disordered breathing (SDB) can both complicate and cause growth failure in children. A 4-year-old male was admitted to the hospital for a failure to thrive evaluation. Growth parameters show a weight of 10 kilograms at the age of 4.5 years. Pertinent findings were proximal muscle weakness, subtle chest wall asymmetry, and mild kyphoscoliosis. He was born healthy at term; there were no previous hospital admissions. He had never displayed respiratory difficulties and seemed to have a normal cough and activity tolerance. He sleeps from 10 p.m. to 7 a.m. daily. He was active with no concerns for sleepiness, fatigue, or headaches. He does have a history of gross motor developmental delay from infancy but was able to run. His appetite was normal and there was no history of feeding difficulty. Concern for congenital muscular dystrophy prompted a muscle biopsy which was normal. Sweat test was negative. A sleep study was obtained after discharge and revealed rapid eye movement (REM) related hypoventilation and severe obstructive sleep apnea (OSA) (apnea hypopnea index (AHI) 15/hr) with 29% of time asleep spent with oxygen saturations below 90% and severe episodic desaturations as low as 49%. Non-invasive bi-level positive airway pressure (BiPAP) was quickly initiated. He was subsequently diagnosed with congenital myasthenia gravis and started on pyridostigmine which he continues to take currently. Now at the age of 7 years he shows a weight of 21.9 kilograms and is at the 34th percentile for weight/age. His restrictive lung disease has not progressed since the initiation of BiPAP 15/5 while sleeping which he continues to use. Recent diagnostic sleep study was significant for mild hypoventilation with pCO2 50-55 mmHg and moderate OSA (AHI of 7.8) and oxygen desaturations with a nadir of 82%. Recent otolaryngology evaluation revealed small size of adenoids and tonsils, so surgery was deferred. This case illustrates the importance of screening for SDB despite the lack of purported sleep problems when there is concern for neuromuscular disease. Early initiation of BiPAP and as well as treatment of his underlying disease have resulted in improved growth and development.
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