Characterization of Digestive Manifestations in Patients with Impaired PTH/PTHrP Signaling Disorder/Pseudohypoparathyroidism
Authors' conclusion
Our work shows for the first time that digestive manifestations, including constipation, occur frequently in children followed for iPPSD, suggesting a potential role of Gsα and G protein receptors in the digestive tract. It is well known that constipation and digestive symptoms alter quality of life. Early management is therefore essential to improve the quality of life of children followed for iPPSD. Our data need to be confirmed on a larger cohort.
Study context
- Population
- Children aged 2–18 years followed for iPPSD type 2 or 3 at the French national reference center for rare calcium and phosphate metabolism diseases
- Participants
- 36 children
- Exposure
- iPPSD2 vs iPPSD3
- iPPSD2n = 32
- iPPSD3n = 4
- Outcome
- Frequency and characteristics of digestive manifestations
Methodological detailssingle reference centre, parent questionnaire
- Single reference centre
- Parent questionnaire
Main finding
As reported in the abstract
Parent-reported constipation (most frequent digestive disorder)
22 of 36
over 60%
Bristol score 1–2 confirming constipation, among constipated children
19
87%
Neonatal vomiting and eating disorders (lack of satiety, food selectivity)
18
50%
Gastroesophageal reflux in neonatal period
14
40%
Observational data: associations, not effects.
Further findings
Treatment of constipation
- Dedicated treatment initiated
- 10 (55%)
- Families considering treatment effective
- 3 (16%)
Original publication
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