Paroxysmal extreme pain disorder (previously familial rectal pain syndrome).

Objective: To describe the clinical phenotype of paroxysmal extreme pain disorder (previously called familial rectal pain syndrome), an autosomal dominant condition recently shown to be a sodium channelopathy involving SCN9A.

Methods: An international consortium of clinicians, scientists, and affected families was formed. Clinical details of all accessible families worldwide were collected, including age at onset, features of attacks, problems between attacks, investigational results, treatments tried, and evolution over time.

Pancuronium during mechanical ventilation speeds recovery of lungs of infants with hyaline membrane disease.

Spontaneous breathing during mechanical ventilation in newborn infants may damage the lung. To find out whether the prevalence of lesions which might be due to trauma was reduced by muscle relaxation, fifty infants who required mechanical ventilation of hyaline membrane disease were randomly assigned to treated and control groups. The treated infants were kept muscle relaxed with pancuronium bromide until they needed a FiO2 of 0.

Continuous monitoring of arterial oxygen tension in infants: four years of experience with an intravascular oxygen electrode.

Two hundred eighty-two umbilical artery catheters with oxygen electrodes on their tips were passed into the aortas of 268 newborn infants with severe respiratory illnesses between 1974-1977; 254 (90%) of the electrodes recorded PaO2 satisfactorily for a mean of 66 hours; 212 (75%) electrodes were still functioning well when removed, because they were no longer clinically needed, after a mean of 87 hours. The 27 electrodes that failed to record PaO2 on insertion, 42 that failed later, and 25 that temporarily malfunctioned were examined after removal from the infants. A structural cause was found which accounted for the failure or malfunction of 43 electrodes.