1/9. Moebius syndrome with central hypoventilation and brainstem calcification: a case report.Moebius syndrome (MS) is described in an infant with central hypoventilation and brainstem calcification. The patient had limb defects and bilateral paralysis of the 6th, 7th, 9th, 10th, and 12th cranial nerves. Mechanical ventilation was continued from birth because of shallow spontaneous respiration. Computed tomography revealed brainstem atrophy and four small calcifications restricted to the dorsal portion of the pons and medulla. Prenatal brainstem injury such as ischaemia may have caused MS and central hypoventilation.- - - - - - - - - - ranking = 1keywords = respiration (Clic here for more details about this article) |
2/9. Primary alveolar hypoventilation treated with nocturnal electrophrenic respiration.A case of primary alveolar hypoventilation is described. Despite characteristic clinical findings, the diagnosis was delayed for 4 years. Alleviation of nocturnal hypoxemia and hypercapnia initially by a rocking bed and subsequently by phrenic nerve stimulation was accompanied by reversal of cor pulmonale and polycythemia. Electrophrenic respiration is an effective form of long-term management in primary alveolar hypoventilation.- - - - - - - - - - ranking = 5keywords = respiration (Clic here for more details about this article) |
3/9. Electrophrenic respiration: report of six cases.The development of electrophrenic respiration has permitted freedom from mechanical ventilation for patients who have irreversible respiratory failure in association with high-cervical spinal cord or brainstem lesions. There are three basic criteria for successful diaphragm pacing: (1) the need for long-term mechanical ventilatory assistance, (2) a functionally intact phrenic nerve-diaphragm axis, and (3) chest wall stability. Inability to achieve satisfactory pacing can be due to malfunction of equipment, instability of the chest wall, or inadequate neuromuscular responsiveness. These features of diaphragm pacing are exemplified in a series of six patients. Three achieved independence from mechanical ventilatory assistance with full-time phrenic pacing. In one patient, only limited electrophrenic respiration was achieved, and in another the method was entirely unsuccessful. Although functioning well, pacing systems were removed from the sixth patient because of infection. diaphragm pacing can be a valuable form of respiratory support for carefully selected patients.- - - - - - - - - - ranking = 6keywords = respiration (Clic here for more details about this article) |
4/9. Central hypoventilation syndrome: experience with bilateral phrenic nerve pacing in 3 neonates.Successful long-term phrenic nerve pacing has been reported in adults with acquired central hypoventilation syndrome. This report summarizes our experience with phrenic nerve pacing in 3 infants with congenital central hypoventilation syndrome. The electrodes were implanted in the lower thoracic portion of each phrenic nerve. In all patients. bilateral simultaneous pacing was required to maintain an adequate arterial PO2, tidal volume, and minute ventilation during quiet sleep. Case 1 died of problems primarily related to the severe cor pulmonale that had been present before pacemaker insertion; at autopsy, the pacemaker system was intact and there were no significant phrenic nerve abnormalities. Case 2 later developed failure of awake ventilatory control and died because of extensive phrenic nerve damage incurred by 19 days of continuous pacing. Case 3 has received quiet sleep pacemaker support since September 1977 and has been able to maintain normal quiet sleep ventilation in this manner. phrenic nerve pacing can be successful in infants as long as continuous pacing is not required. Bilateral simultaneous pacing appears to be an appropriate alternative to home-based intermittent positive-pressure breathing for long-term management of children with central hypoventilation syndrome.- - - - - - - - - - ranking = 16.083096675653keywords = positive-pressure (Clic here for more details about this article) |
5/9. almitrine bismesylate in congenital central hypoventilation.We have carried out sequential studies of respiration and sleep state, from two weeks to two years of age in a girl with congenital central hypoventilation. When awake her minute ventilation (VE) and blood gases are normal, and when asleep she shows hypoventilation, with progressive hypoxia, hypercarbia and respiratory acidosis, most marked in NREM sleep. There has been no consistent change in VE (expressed as ml/kg/min) with age, awake or asleep, and she remains ventilator dependent when asleep. growth and development are normal. We report a trial of almitrine bismesylate at the age of 21 months. A dose of 1.5 mg/kg/day orally for eight days produced minimal changes in VE, but when given 2.6 mg/kg/day for a further seven days there was a significant increase in VE in both NREM (27% increase) and in REM sleep (30% increase). These changes were accompanied by substantial improvements in blood gases, with PaCO2 less than 50 mmHg and PaO2 greater than 60 mmHg throughout a four hour study period asleep, off the ventilator, in air. After stopping almitrine bismesylate VE and blood gas values returned to pretreatment values. almitrine bismesylate may be of value as an alternative to artificial ventilation in this condition.- - - - - - - - - - ranking = 1keywords = respiration (Clic here for more details about this article) |
6/9. Congenital central hypoventilation syndrome treated with diaphragm pacing.Congenital central hypoventilation syndrome was diagnosed in an infant who since birth had shallow respiration and CO2 retention during sleep, absent ventilatory response to hypercarbia, and no underlying disease or trauma to account for the symptoms. diaphragm pacing was started at the age of 8 1/2 months and has been successfully carried out at home, guided by end-tidal CO2 monitoring. After 22 months of home treatment, at the age of two years 9 months, linear growth and psychomotor development are progressing normally, while previous symptoms of cor pulmonale have not progressed.- - - - - - - - - - ranking = 1keywords = respiration (Clic here for more details about this article) |
7/9. Insidious onset of acute alveolar hypoventilation following intermittent positive-pressure breathing (IPPB).2 patients developing acute alveolar hypoventilation following IPPB therapy are presented. The hypoventilation occurred insidiously following therapy. It is hypothesized that respiratory center depression from excessive oxygen administration caused the hypoventilation. When the IPPB machines were driven by compressed air instead of oxygen, this complication was revealed in 1 of the patients.- - - - - - - - - - ranking = 64.332386702611keywords = positive-pressure (Clic here for more details about this article) |
8/9. Management of hypoventilation in motor neuron disease presenting with respiratory insufficiency.A 57-year-old man presented with a three-month history of somnolence, hypoventilation, cor pulmonale, and episodes of apnea during sleep. Neurological studies revealed motor neuron disease, mainly confined to the muscles of respiration, that was causing respiratory insufficiency in the supine position. Nighttime treatment of respiratory insufficiency has permitted independent activity during wakefulness.- - - - - - - - - - ranking = 1keywords = respiration (Clic here for more details about this article) |
9/9. hypoventilation during sleep in children who have lymphoid airway obstruction treated by nasopharyngeal tube and T and A.Three children with sleep apnea, alveolar hypoventilation, apparent mental retardation, and poor growth associated with chronically enlarged tonsils and adenoids were treated with the use of a nasopharyngeal tube followed by tonsillectomy and adenoidectomy. The effectiveness of this therapy was documented by polygraphic recording of sleep stages and respirations, and by correlation with serial arterial blood gases and pH. The nasopharyngeal tube was well tolerated, easy to use, and effective in diagnosis and treatment. We suggest that its use be further evaluated in patients with obstructive apnea.- - - - - - - - - - ranking = 1keywords = respiration (Clic here for more details about this article) |