Pseudo Hypoparathyroidism Narrative Revue

The term pseudohypoparathyroidism was first introduced in 1942 by Albright to describe patients who presented with hormonal parathyroid disease (PTHb-resistant hypocalcemia and hyperphosphatemia) with characteristic skeletal and developmental changes. Pseudohypoparathyroidism is a group of rare, related and highly heterogeneous disorders characterized by target organ resistance to the action of parathyroid hormone. PHP and related disorders are caused by genetic and/or epigenetic changes leading to downregulation of a cyclic adenosine mono phosphate generator, primarily linked to the Nucleotide binding protein, Alpha Stimulating activity gene. As a story on the disease; It is under the name of a curious eponym referring to an old breed of dwarf chickens created by Sir John Sebright, in the 19th century, that the American endocrinologist Fuller Albright (1900-1969) described for the first noted in 1942 the “ Sea brightbantam syndrome”, a morphological syndrome characterized by short stature, obesity, rounded facial features, finger length abnormalities, often accompanied by mental retardation, associated with hypocalcemia and hyperphosphatemia.

A criteria-based narrative systematic literature review PRISMA-P has summer carried out in leaning methodologically on there publication of the article of Zaugg And al as well as the guide to recommendations for journals systematic narrative of the Economic and Social Research Council. There review of there literature has summer performed In THE basics of data international MEDLINE and Cochrane data base of systematic reviews by their search engine (respectively Pub Med). The data French speakers were searched with the search engines of the Database of the Scientific Literature in Health site (LiSSa), of the University Documentation System (SUDoc), the Catalog and Index sites Medical of language French (CISMeF). Selection of the double studies _ blind _ results products by the app of the equations of research In the different search engines made it possible to obtain a list of publication. The selection was initially made on the title and the abstract and In A second time on there reading of Full Text. As Criteria of inclusion And of exclusion studies were to be written in language English or French. Criteria major of inclusion were to be found In THE title or THE summary: Subject treating pseudo hypoparathyroidism. The exclusion criteria for the first selection phase based on title and summary were non-response to the inclusion criteria and the absence of summary available. The extraction of data After pooling publications included or excluded by title and summary, THE studies have summer read in their entirety of manner independent. Data was extracted using a data extraction grid developed from the Cochrane and PRISMA-P repositories. This second stage has permit there selection definitive of the items Following the same methodology as in the first step. We have could formalize THE points analyzes following: Title, Author Journal, Population of the study (kind actor, country, number of topics) ; the year of the study (or by default, year of publication); Objective main of the study as well as the Methodology used. The analysis of there quality of studies: The level of quality of each study was assessed using different scales: COREQ guidelines for qualitative research, criteria STROBE for descriptive studies, the ENTREQ criteria for qualitative research syntheses and the PRISMA-P criteria for THE journals of literature. description _ of the studies selected with search equations and key words used in the different bases data made it possible to reference 239 publications. After analysis 113 documents have been retained by consensus. The elements retained were distributed as follows: 55 articles and 58 academic documents (thesis or memory) obtained from the research equations. As results found:

In Japan, a national epidemiological study in 1998 estimated that there were 430 (95% confidence interval 330–520) patients with PPH in Japan, equivalent to a prevalence of 0·34/100,000.The study showed that a majority of women (58%), and the majority of patients were diagnosed during the first 4 decades of life.

In Pathophysiology, indirect evidence makes it possible to eliminate a defect in renal alpha-hydroxylase as a determining factor in this condition. Clinical signs: signs of hereditary Albright osteodystrophy, progressive bone heteroplasia, acrodysostosis, cognitive disorders Intracerebral calcification. PTH resistance should be treated with activated forms of vitamin D, for example calcitriol or alfacalcidol, to increase serum calcium levels and thereby reduce PTH levels. The authors recommend targeting a serum calcium level that is in the low-normal range and not normalizing the serum PTH concentration, to avoid the risk of hypercalcemia and/or hypercalciuria.