Case Report: Hepatic Adenomatosis in a Patient With Prader-Willi Syndrome.
Front Endocrinol (Lausanne)
; 13: 826772, 2022.
Article
in English
| MEDLINE | ID: covidwho-1817935
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
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Prader-Willi Syndrome
/
Obesity, Morbid
/
Diabetes Mellitus, Type 2
Type of study:
Case report
/
Observational study
Topics:
Long Covid
Limits:
Child
/
Child, preschool
/
Humans
Language:
English
Journal:
Front Endocrinol (Lausanne)
Year:
2022
Document Type:
Article
Affiliation country:
Fendo.2022.826772
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