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Case Reports
. 2022 Mar 9:13:826772.
doi: 10.3389/fendo.2022.826772. eCollection 2022.

Case Report: Hepatic Adenomatosis in a Patient With Prader-Willi Syndrome

Hajar Dauleh  1 , Ali Soliman  1 , Basma Haris  1 , Amal Khalifa  1 , Noor Al Khori  2 , Khalid Hussain  1
Affiliations
Case Reports

Case Report: Hepatic Adenomatosis in a Patient With Prader-Willi Syndrome

Hajar Dauleh et al. Front Endocrinol (Lausanne). .

Abstract

Prader-Willi syndrome (PWS) is a genetic disorder caused by the lack of expression of genes on the paternally inherited chromosome region 15q11.2-q13. It is a multisystem disorder that is characterized by severe hypotonia with poor suck and feeding difficulties in early infancy, followed in early childhood by excessive eating and gradual development of morbid obesity. The incidence of type 2 diabetes mellitus is high, particularly in obese patients. Non-alcoholic fatty liver disease has also been reported in some patients with PWS. Liver adenomatosis is a benign vascular lesion of the liver, defined by the presence of >10 adenomas, in the otherwise healthy liver parenchyma. We report the first case of a patient with PWS with severe obesity, type 2 diabetes mellitus, and non-alcoholic fatty liver who also developed liver adenomatosis, review the pediatric literature on liver adenomatosis, and discuss the potential underlying mechanisms.

Keywords: Glycogen Storage Disease; Prader Willi syndrom; hepatic adenomatosis; liver adenoma; oral contraception pills.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
MRI liver findings. (A) Contrast enhanced liver MRI image showing multiple small liver lesions showing early arterial enhancement. (B) Lesions showing retention of contrast after 6 minute delay. (C) Lesions showing retention of contrast after 31 minute delay.
See this image and copyright information in PMC

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References

    1. Muscogiuri G, Barrea L, Faggiano F, Maiorino MI, Parrillo M, Pugliese G, et al. . Obesity in Prader-Willi Syndrome: Physiopathological Mechanisms, Nutritional and Pharmacological Approaches. J Endocrinol Invest (2021) 44(10):2057–70. doi: 10.1007/s40618-021-01574-9 - DOI - PMC - PubMed
    1. Brambilla P, Crinò A, Bedogni G, Bosio L, Cappa M, Corrias A, et al. . Metabolic Syndrome in Children With Prader-Willi Syndrome: The Effect of Obesity. Nutr Metab Cardiovasc Dis (2011) 21(4):269–76. doi: 10.1016/j.numecd.2009.10.004 - DOI - PubMed
    1. Talebizadeh Z, Butler MG. Insulin Resistance and Obesity-Related Factors in Prader-Willi Syndrome: Comparison With Obese Subjects. Clin Genet (2005) 67(3):230–9. doi: 10.1111/j.1399-0004.2004.00392.x - DOI - PMC - PubMed
    1. Haqq AM, Muehlbauer MJ, Newgard CB, Grambow S, Freemark M. The Metabolic Phenotype of Prader-Willi Syndrome (PWS) in Childhood: Heightened Insulin Sensitivity Relative to Body Mass Index. J Clin Endocrinol Metab (2011) 96(1):E225–32. doi: 10.1210/jc.2010-1733 - DOI - PMC - PubMed
    1. Barbier L, Nault JC, Dujardin F, Scotto B, Besson M, de Muret A, et al. . Natural History of Liver Adenomatosis: A Long-Term Observational Study. J Hepatol (2019) 71(6):1184–92. doi: 10.1016/j.jhep.2019.08.004 - DOI - PubMed

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