Ghrelin, leptin and adiponectin levels in Gaucher disease type I patients on enzyme replacement therapy

Doneda, Divair; Lopes, André Luiz; Mittelstadt, Suzana; Moulin, Cileide Cunha; Schwartz, Ida Vanessa Döederlein

doi:10.1016/j.clnu.2014.08.010

Cited by 8 publications

(6 citation statements)

References 38 publications

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“…Doneda et al found, opposite to the results of Langeveld et al [46], that there is no difference between the median levels of adiponectin in GD1 patients treated with ERT and controls. Moreover, they indicated that GD1 patients with splenomegaly have lower levels of adiponectin when compared to GD1 patients without splenomegaly.…”

Section: Main Textmentioning

confidence: 67%

“…A direct correlation between adiponectin and ghrelin levels with HDL-C were found by Doneda et al. Leptin levels were inversely proportional to LDL-C and directly proportional to triglyceride concentrations [46].…”

Section: Main Textmentioning

confidence: 99%

“…Moreover, they indicated that GD1 patients with splenomegaly have lower levels of adiponectin when compared to GD1 patients without splenomegaly. Adiponectin concentrations were inversely proportional to the BMI, waist circumference and TG and directly proportional to HDL-C [46]. These results come from patients with GD1, all of whom were on ERT for at least 6 months and their BMI was higher than in the Lagneveld et al study.…”

Section: Main Textmentioning

confidence: 99%

“…The average concentrations of leptin and ghrelin are comparable in treated GD1 patients and in controls [46]. Doneda et al found that patients with metabolic syndrome and GD1 on ERT had significantly higher levels of leptin, BMI, waist circumference, TG and insulin compared to GD1 cases without metabolic syndrome [46]. A negative correlation between BMI, waist circumference, TG levels and ghrelin concentrations was observed in GD1 patients.…”

Section: Main Textmentioning

confidence: 99%

“…However, serum ghrelin levels were positively correlated with HDL-C. Leptin showed a positive correlation with BMI, waist circumference, TG, insulin and a negative correlation with LDL-C. A strong association between levels of leptin, insulin and homeostatic model assessment of insulin resistance index (HOMA-IR) was also found in GD1 subjects. This association was not present in the controls [46].…”

Section: Main Textmentioning

confidence: 99%

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Endocrine and metabolic disorders in patients with Gaucher disease type 1: a review

Kałużna

Trzeciak

Ziemnicka

et al. 2019

Orphanet J Rare Dis

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BackgroundGaucher disease (GD) is one of the most prevalent lysosomal storage diseases and is associated with hormonal and metabolic abnormalities, including nutritional status disorders, hypermetabolic state with high resting energy expenditures, peripheral insulin resistance, hypoadiponectinaemia, leptin and ghrelin impairments, hypolipidaemia, linear growth deceleration and growth hormone deficiency, delayed puberty, hypocalcaemia and vitamin D deficiency. Specific treatments for GD such as enzyme replacement therapy and substrate reduction therapy display significant effects on the metabolic profile of GD patients.Main body of the abstractHormonal and metabolic disturbances observed in both adult and paediatric patients with Gaucher disease type 1 (GD1) are discussed in this review. The PubMed database was used to identify articles on endocrine and metabolic disorders in GD1. GD1 appears to facilitate the development of disorders of nutrition, glucose metabolism and vitamin D insufficiency. Metabolic and hormonal diseases may have a significant impact on the course of the underlying disease and patient quality of life.ConclusionsConditions relating to hormones and metabolism can be wide-ranging in GD1. Obtained findings were intrinsic to GD either as a deleterious process or a compensatory response and some changes detected may represent co-morbidities. Actively seeking and diagnosing endocrine and metabolic disorders are strongly recommended in GD1 patients to optimize healthcare.

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Section: Main Textmentioning

confidence: 67%

Section: Main Textmentioning

confidence: 99%

Section: Main Textmentioning

confidence: 99%

Section: Main Textmentioning

confidence: 99%

Section: Main Textmentioning

confidence: 99%

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