Síndrome de Turner como causa de cirrosis hepática

Machlab, Salvador; Miquel, Mireia; Voltà, Tània; Escoda, M.; Vergara, Mercedes

doi:10.1016/j.gastrohep.2017.05.014

Cited by 5 publications

(2 citation statements)

References 9 publications

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“…As far as hypogonadism is concerned, Klinefelter’s syndrome is exceptionally associated with primary biliary cholangitis rather than with NASH–cirrhosis [170,171]. Turner syndrome is rarely associated with NASH–cirrhosis [172]. However, we are not aware of any cases of NAFLD-related hepatocellular carcinoma occurring in hypogonadic patients.…”

Section: Discussionmentioning

confidence: 99%

NAFLD in Some Common Endocrine Diseases: Prevalence, Pathophysiology, and Principles of Diagnosis and Management

Lonardo

Mantovani

Lugari

et al. 2019

IJMS

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Secondary nonalcoholic fatty liver disease (NAFLD) defines those complex pathophysiological and clinical consequences that ensue when the liver becomes an ectopic site of lipid storage owing to reasons other than its mutual association with the metabolic syndrome. Disorders affecting gonadal hormones, thyroid hormones, or growth hormones (GH) may cause secondary forms of NAFLD, which exhibit specific pathophysiologic features and, in theory, the possibility to receive an effective treatment. Here, we critically discuss epidemiological and pathophysiological features, as well as principles of diagnosis and management of some common endocrine diseases, such as polycystic ovary syndrome (PCOS), hypothyroidism, hypogonadism, and GH deficiency. Collectively, these forms of NAFLD secondary to specific endocrine derangements may be envisaged as a naturally occurring disease model of NAFLD in humans. Improved understanding of such endocrine secondary forms of NAFLD promises to disclose novel clinical associations and innovative therapeutic approaches, which may potentially be applied also to selected cases of primary NAFLD.

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Section: Discussionmentioning

confidence: 99%

NAFLD in Some Common Endocrine Diseases: Prevalence, Pathophysiology, and Principles of Diagnosis and Management

Lonardo

Mantovani

Lugari

et al. 2019

IJMS

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“…The hepatic histological changes reported in TS patients vary and include minimal abnormalities, steatosis, steatohepatitis, biliary involvement, nodular regenerative hyperplasia and even cirrhosis (5,6,7,8,9,10,11,12,13,14,15,16,17,18,19). Potential causes and mechanisms are not clear and may include autoimmune processes, venous malformations, obesity and sex hormone replacement therapy (HRT) (5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20). However, some basic and animal studies point to the crucial a role of estrogen deficiency or estrogen receptor malfunction in the development of liver impairment (21,22,23,24,25,26,27).…”

Section: Introductionmentioning

confidence: 99%

Liver Biochemical Abnormalities in Adolescent Patients with Turner Syndrome

Wójcik¹,

Ruszała²,

Januś³

et al. 2019

Jcrpe

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Objective:Elevated liver function tests (LFTs) are common in adult Turner syndrome (TS) patients. Data regarding children and adolescents are lacking. To investigate the prevalence of abnormal LFTs in children and adolescents with TS during several years of observation; to evaluate the potential impact of increased body mass index (BMI) and sex hormone replacement therapy (HRT) on LFTs.Methods:The analysis included 100 girls with TS, aged 4-16 years, all of whom were receiving recombinant human growth hormone therapy. A longitudinal study was conducted which included 81 patients.Results:Mean BMI-standard deviation (SD) score of the subjects was 0.63 (SD: 1.53). Forty-four were being treated with HRT. Elevated LFTs were found in 34% of the patients overall (32% not receiving HRT vs 36% on HRT). The relative risk of increased LFTs was not higher in obese vs normal weight [odds ratio (OR): 0.2; 95% confidence interval (CI): 0.1-0.36, p=0.38 vs OR: 0.16; 95% CI: 0.08-0.3, p=0.1]. HRT did not increase the risk of abnormal LFTs activity (OR: 0.8; 95% CI: 0.5-1.2, p=0.37 vs OR: 0.7; 95% CI: 0.4-1.1, p=0.27). During the follow-up period (mean±SD=4.31±0.82 years), no patient developed overt liver disease. There was no significant increase nor decrease of abnormal LFT frequency in the subsequent years of follow up.Conclusion:Constantly elevated LFTs in TS are common in children and adolescents with TS. However the causes and clinical significance remain unclear. This study suggests that obesity and HRT do not increase the risk of elevated LFTs.

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