1. Sedatives such as the benzodiazepines and alcohol reduce upper airway muscle activity. We hypothesized that a sedating antihypertensive, alpha-methyldopa, might have similar effects. To investigate this hypothesis we studied the effect of alpha-methyldopa on alae nasi electromyographic (EMG) activity during hypercapnia. 2. We studied ten healthy subjects and three subjects with obstructive sleep apnoea during CO2-stimulated breathing. In a preliminary study four subjects demonstrated a fall in alae nasi EMG activity 4 h after the ingestion of 500 mg of alpha-methyldopa during CO2 rebreathing. 3. In six additional normal subjects and three subjects with obstructive sleep apnoea, we studied the alae nasi EMG activity during steady-state hypercapnia with PCO2 held constant 5 torr (0.7 kPa) above baseline. On 2 separate days we studied subjects before and 2 h after they had ingested 750 mg of alpha-methyldopa or placebo. 4. In the normal subjects the mean alae nasi EMG activity fell by 34% 2 h after ingestion of alpha-methyldopa (P less than 0.05) without a change in other ventilatory parameters. 5. In the sleep apnoea group the individual mean alae nasi EMG activity fell 16-49%, with ventilation and tidal volume falling in one patient. 6. We conclude that alpha-methyldopa selectively reduces upper airway motor activity.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1042/cs0740547 | DOI Listing |
J Tissue Viability
November 2024
Necmettin Erbakan University Faculty of Medicine Hospital, Division of Surgical Medical Sciences, Department of Anesthesiology and Reanimation, Konya, Turkey. Electronic address:
Johanson-Blizzard syndrome (JBS) is a rare genetic disorder caused by Ubiquitin Protein Ligase E3 Component N-Recognin1 (UBR1) gene mutations. It is characterized by exocrine pancreatic insufficiency, craniofacial deformities, sensorineural hearing loss, and a broad variety of intellectual disabilities. The aim of our study is to report four pediatric cases (three of which are siblings, and the fourth patient is unrelated) that presented some features of JBS.
View Article and Find Full Text PDFCureus
March 2024
Department of Pediatrics, Salmaniya Medical Complex, Manama, BHR.
Johanson-Blizzard syndrome (JBS) is a rare hereditary autosomal recessive disorder caused by a mutation in the ubiquitin protein ligase E3 component n-recognin 1 (UBR1) gene. This syndrome is characterized by the following typical clinical features: hypoplasia or aplasia of the alae nasi, congenital scalp defects, sensorineural hearing loss, hypothyroidism, growth retardation, psychomotor retardation, imperforate anus, genitourinary anomalies, and atypical hair patterns. Here, we describe a case of a 12-year-old girl with JBS of consanguineous parents.
View Article and Find Full Text PDFAm J Med Genet A
July 2024
Hospices Civils de Lyon, GHE, Service de Génétique, Lyon, France.
Anesthesiology
July 2024
Sorbonne Université, Institut National de la Santé et de la Recherche Médicale, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France; Assistance Publique-Hôpitaux de Paris Sorbonne Université, Pitié-Salpêtrière Hospital, Médecine Intensive-Réanimation Unit, Paris, France.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!