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Management of pseudohypoparathyroidism
1Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Center for Rare Bone Disorders and Albright Center, University of Connecticut School of Medicine, Connecticut Children's Medical Center, Farmington, Connecticut, USA.
Current Opinion in Pediatrics
|May 31, 2019
Summary
This review synthesizes current knowledge on pseudohypoparathyroidism (PHP) and its subtypes, PHP1A, pseudopseudohypoparathyroidism (PPHP), and PHP1B. It offers practical guidance for recognizing and managing these rare genetic disorders.
Area of Science:
- Endocrinology
- Genetics
- Rare Diseases
Background:
- Pseudohypoparathyroidism (PHP) and related disorders encompass a spectrum of genetic conditions.
- Recent advancements include the 2018 international Consensus Statement for diagnosis and management.
- Understanding of PHP mechanisms and phenotypes is continually evolving.
Purpose of the Study:
- To provide essential knowledge for recognizing and managing PHP1A, PPHP, and PHP1B.
- To offer an overview of the entire PHP spectrum.
- To summarize management strategies for clinical application.
Main Methods:
- Review of recent literature.
- Synthesis of personal clinical experience with numerous PHP patients.
- Incorporation of findings from the international Consensus Statement.
Main Results:
- Detailed elucidation of PHP phenotypes, including short stature, brachydactyly, and metabolic disturbances.
- Progress in understanding the underlying mechanisms of PHP abnormalities.
- The international Consensus Statement provides systematic guidance for improved management.
Conclusions:
- Continued research is uncovering PHP mechanisms and improving patient management.
- The 2018 Consensus Statement is expected to drive significant progress in PHP care.
- This review consolidates current understanding for practical clinical use.
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