دورية أكاديمية

Insulin-like growth factor-1 deficiency caused by hepatocellular adenoma leads to growth arrest, primary amenorrhea and metabolic syndrome: a case report and 4 years follow up.

التفاصيل البيبلوغرافية
العنوان: Insulin-like growth factor-1 deficiency caused by hepatocellular adenoma leads to growth arrest, primary amenorrhea and metabolic syndrome: a case report and 4 years follow up.
المؤلفون: Huang Z; Department of Endocrinology and Diabetes Center, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P.R. China. hzhim@mail.sysu.edu.cn., Li Y; Department of Hyperbaric Oxygen, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P.R. China., Chen W; Department of Pathology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P.R. China., Deng W; Department of Endocrinology and Diabetes Center, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P.R. China., Li Y; Department of Endocrinology and Diabetes Center, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, P.R. China. liyb@mail.sysu.edu.cn.
المصدر: BMC endocrine disorders [BMC Endocr Disord] 2024 Sep 12; Vol. 24 (1), pp. 188. Date of Electronic Publication: 2024 Sep 12.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: BioMed Central Country of Publication: England NLM ID: 101088676 Publication Model: Electronic Cited Medium: Internet ISSN: 1472-6823 (Electronic) Linking ISSN: 14726823 NLM ISO Abbreviation: BMC Endocr Disord Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : BioMed Central, [2001-
مواضيع طبية MeSH: Metabolic Syndrome*/complications , Insulin-Like Growth Factor I*/metabolism , Liver Neoplasms*/pathology , Liver Neoplasms*/surgery , Liver Neoplasms*/complications , Adenoma, Liver Cell*/pathology , Adenoma, Liver Cell*/surgery , Adenoma, Liver Cell*/complications , Adenoma, Liver Cell*/etiology , Amenorrhea*/etiology, Humans ; Female ; Adolescent ; Follow-Up Studies ; Growth Disorders/etiology ; Growth Disorders/complications ; Growth Disorders/pathology ; Prognosis ; Insulin-Like Peptides
مستخلص: Background: Hepatocellular adenoma (HCA) is a rare benign neoplasm, seldom ascribed as the cause of endocrine and metabolic derangement. We herein report a case of primary amenorrhea, growth arrest and metabolic syndrome. En bloc resection of the tumor normalized all the disturbances.
Case Presentation: A 16-year-old girl complained of primary amenorrhea and growth arrest for the past 2 years. Her height and weight were at the 3rd percentile, whereas waist circumference was at the 90th percentile for chronological age. She was hypertensive on admission. Plasma cholesterol, triglyceride and uric acid were elevated. Evaluation of GH/IGF-1 axis showed extremely low IGF-1 concentration, which was unresponsive to hGH stimulation. Computer tomography identified a huge liver mass (18.2 cm×13.7 cm×21 cm). The patient underwent an uneventful open right hepatic lobectomy. The tumor was en bloc resected. Immunohistochemistry indicated an unclassified HCA, which was confirmed by genetic screening. IGF-1 concentration, blood pressure, lipid profile and ovarian function were all normalized after surgery, and the girl had reduction in waist circumference and gain in height during the follow up.
Conclusion: We provide evidence that liver-derived IGF-1 has a direct effect on skeletal and pubertal development, blood pressure, visceral adiposity and dyslipidemia independent of insulin resistance and obesity in the circumstance of undernutrition. Though rare, we propose the need to look into HCA cases for the existence of IGF-1 deficiency and its impact on metabolic derangement.
(© 2024. The Author(s).)
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معلومات مُعتمدة: 82070918 National Natural Science Fund of China; 2019B020230001 Key Field Research and Development Program of Guangdong Province, China
فهرسة مساهمة: Keywords: Adiposity; Hepatocellular adenoma; Insulin-like growth factor-1; Metabolic syndrome
المشرفين على المادة: 67763-96-6 (Insulin-Like Growth Factor I)
0 (IGF1 protein, human)
0 (Insulin-Like Peptides)
تواريخ الأحداث: Date Created: 20240912 Date Completed: 20240913 Latest Revision: 20240915
رمز التحديث: 20240915
مُعرف محوري في PubMed: PMC11391839
DOI: 10.1186/s12902-024-01716-z
PMID: 39266979
قاعدة البيانات: MEDLINE
الوصف
تدمد:1472-6823
DOI:10.1186/s12902-024-01716-z