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Obstructive Sleep Apnea and Otolaryngologic Manifestations in Children with Pseudohypoparathyroidism

Kathleen L Curley1, Sachini Kahanda2, Katia M Perez3

  • 1The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.

Hormone Research in Paediatrics
|February 19, 2018
PubMed

Insights

Children with pseudohypoparathyroidism (PHP) have a higher risk of obstructive sleep apnea (OSA). This study found increased OSA prevalence in PHP type 1A and 1B patients, suggesting screening is needed.

Area of Science:

  • Pediatric Endocrinology
  • Sleep Medicine
  • Genetics

Background:

  • Pseudohypoparathyroidism (PHP) is a rare genetic disorder.
  • Patients with PHP may have a higher prevalence of obstructive sleep apnea (OSA).
  • This association has not been prospectively studied in children.

Purpose of the Study:

  • To prospectively investigate the prevalence of OSA in children with PHP.
  • To compare OSA incidence in children with PHP type 1A (PHP1A) and type 1B (PHP1B) against matched controls.
  • To identify associated conditions such as otitis media and adenotonsillar hypertrophy in PHP patients.

Main Methods:

  • Enrolled children aged 6-18 years with PHP and matched controls.
  • Conducted physical examinations, reviewed medical histories, and performed polysomnography (sleep studies).
  • Evaluated 15 children with PHP1A, 15 controls, and 3 children with PHP1B.

Main Results:

  • Children with PHP1A showed a significantly higher obstructive disturbance index (1.8 ± 2.3 vs. 0.6 ± 0.5) and OSA prevalence (60.0% vs. 13.3%) compared to controls.
  • PHP1A patients had high rates of tympanostomy tube placement (86.7%) and adenotonsillectomy (73.3%).
  • Two of three PHP1B participants (66.7%) had OSA, with a similar obstructive disturbance index (2.0 ± 2.3).

Conclusions:

  • Children with PHP1A face an increased risk of OSA compared to obese peers.
  • PHP1A patients exhibit higher incidences of otitis media and adenotonsillar hypertrophy.
  • Screening for OSA is recommended for PHP1A patients and potentially for PHP1B patients, pending further research.
Abstract

Keywords:
PseudohypoparathyroidismSleep apnea

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