Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Nutritional Status of Patients With Glycogen Storage Diseases – Polish Experience

Ewa Ehmke vel Emczyńska - Seliga
* ORCID logo ,
Małgorzata Hajdacka
,
Maciej Jaworski
,
Maria Kobylińska
,
Magdalena Kaczor
,
Dorota Wesół-Kucharska
ORCID logo ,
Milena Greczan
,
Małgorzata Średzińska
,
Dariusz Rokicki
ORCID logo ,
Janusz Książyk
*
Version 1 : Received: 4 September 2023 / Approved: 4 September 2023 / Online: 6 September 2023 (15:10:23 CEST)

How to cite: Ehmke vel Emczyńska - Seliga, E.; Hajdacka, M.; Jaworski, M.; Kobylińska, M.; Kaczor, M.; Wesół-Kucharska, D.; Greczan, M.; Średzińska, M.; Rokicki, D.; Książyk, J. Nutritional Status of Patients With Glycogen Storage Diseases – Polish Experience. Preprints 2023, 2023090248. https://doi.org/10.20944/preprints202309.0248.v1 Ehmke vel Emczyńska - Seliga, E.; Hajdacka, M.; Jaworski, M.; Kobylińska, M.; Kaczor, M.; Wesół-Kucharska, D.; Greczan, M.; Średzińska, M.; Rokicki, D.; Książyk, J. Nutritional Status of Patients With Glycogen Storage Diseases – Polish Experience. Preprints 2023, 2023090248. https://doi.org/10.20944/preprints202309.0248.v1

Abstract

Background: Glycogen storage diseases (GSDs), known as glycogenoses, belong to inborn metabolic defects in the glycogen metabolism pathway. Several types of GSDs are distinguished, including Ia, Ib, III, VI, and IX. GSDs manifest as excessive glycogen deposition in the liver and muscles, resulting in the dysfunction of these organs. Therefore, the treatment of choice is multitherapy that, due to dietary restrictions, may lead to nutritional deficiencies and organ complications. This study aims to assess anthropometric, body composition, skeletal status, results of selected laboratory tests, dietary, lifestyle and physical capacity analyses in patients with glycogenoses and compare the results between GSD I and GSD III-VI-IX groups. Results: The results indicate that GSD patients were overweight or obese (44% of patients had BMI +1SD or +2SD) with high percentage of fat tissue (50% of patients had above 30% of body fat) and inadequate bone mineralisation (total body less head: median z-score -0.9, L2-L4 segment: median z-score -1.65). GSD I patients had the highest risk of developing obesity (67% of them had above 30% of body fat) and osteoporosis (total body less head: median z-score -1.0, L2-L4 segment: median -1.7). Conclusions: Special attention should be paid to appropriate diet and supplementation in patients with glycogenoses.

Keywords

glycogen storage diseases; glycogenoses; body composition; obesity; fat tissue; bone mineral density

Subject

Medicine and Pharmacology, Other

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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