Epidemiology and outcomes of pediatric autosomal recessive polycystic kidney disease in the Middle East and North Africa

Article


Salman, M. A., Elgebaly, A. and Soliman, N. A. 2024. Epidemiology and outcomes of pediatric autosomal recessive polycystic kidney disease in the Middle East and North Africa. Pediatric Nephrology. 39, pp. 2569-2578. https://doi.org/10.1007/s00467-024-06281-0
AuthorsSalman, M. A., Elgebaly, A. and Soliman, N. A.
Abstract

The incidence of rare diseases is expected to be comparatively higher in the Middle East and North Africa (MENA) region than in other parts of the world, attributed to the high prevalence of consanguinity. Most MENA countries share social and economic statuses, cultural relativism, religious beliefs, and healthcare policies. Polycystic kidney diseases (PKDs) are the most common genetic causes of kidney failure, accounting for nearly 8.0% of dialysis cases. The development of PKDs is linked to variants in several genes, including PKD1, PKD2, PKHD1, DZIP1L, and CYS1. Autosomal recessive PKD (ARPKD) is the less common yet aggressive form of PKD. ARPKD has an estimated incidence between 1:10,000 and 1:40,000. Most patients with ARPKD require kidney replacement therapy earlier than patients with autosomal dominant polycystic kidney disease (ADPKD), often in their early years of life. This review gathered data from published research studies and reviews of ARPKD, highlighting the epidemiology, phenotypic presentation, investigations, genetic analysis, outcomes, and management. Although limited data are available, the published literature suggests that the incidence of ARPKD may be higher in the MENA region due to consanguineous marriages. Patients with ARPKD from the MENA region usually present at a later disease stage and have a relatively short time to progress to kidney failure. Limited data are available regarding the management practice in the region, which warrants further investigations.

JournalPediatric Nephrology
Journal citation39, pp. 2569-2578
ISSN0931-041X
1432-198X
Year2024
PublisherSpringer
Accepted author manuscript
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Anyone
Digital Object Identifier (DOI)https://doi.org/10.1007/s00467-024-06281-0
Publication dates
Online23 Jan 2024
Publication process dates
Accepted02 Jan 2024
Deposited06 Aug 2024
Copyright holder© 2024, The Author
Additional information

This version of the article has been accepted for publication, after peer review (when applicable) and is subject to Springer Nature’s AM terms of use, but is not the Version of Record and does not reflect post-acceptance improvements, or any corrections. The Version of Record is available online at: http://dx.doi.org/10.1007/s00467-024-06281-0

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License: Springer Nature Terms of Use for accepted manuscripts of subscription articles, books and chapters
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