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Case Report: Hepatic Adenomatosis in a Patient With Prader-Willi Syndrome.
Dauleh, Hajar; Soliman, Ali; Haris, Basma; Khalifa, Amal; Al Khori, Noor; Hussain, Khalid.
Affiliation
  • Dauleh H; Department of Pediatric Medicine, Division of Endocrinology, Sidra Medicine, Doha, Qatar.
  • Soliman A; Department of Pediatric Medicine, Division of Endocrinology, Sidra Medicine, Doha, Qatar.
  • Haris B; Department of Pediatric Medicine, Division of Endocrinology, Sidra Medicine, Doha, Qatar.
  • Khalifa A; Department of Pediatric Medicine, Division of Endocrinology, Sidra Medicine, Doha, Qatar.
  • Al Khori N; Department of Pediatric Medicine, Division of Diagnostic Radiology, Sidra Medicine, Doha, Qatar.
  • Hussain K; Department of Pediatric Medicine, Division of Endocrinology, Sidra Medicine, Doha, Qatar.
Front Endocrinol (Lausanne) ; 13: 826772, 2022.
Article in En | MEDLINE | ID: mdl-35355562
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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Prader-Willi Syndrome / Obesity, Morbid / Diabetes Mellitus, Type 2 Limits: Child / Child, preschool / Humans Language: En Journal: Front Endocrinol (Lausanne) Year: 2022 Document type: Article Affiliation country: Qatar

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Prader-Willi Syndrome / Obesity, Morbid / Diabetes Mellitus, Type 2 Limits: Child / Child, preschool / Humans Language: En Journal: Front Endocrinol (Lausanne) Year: 2022 Document type: Article Affiliation country: Qatar