The long-term effect of recombinant methionyl human leptin therapy on hyperandrogenism and menstrual function in female and pituitary function in male and female hypoleptinemic lipodystrophic patients

Musso, Carla; Cochran, Elaine; Javor, Edward D.; Young, Janice Ryan; DePaoli, Alex M.; Görden, Phillip

doi:10.1016/j.metabol.2004.08.021

Cited by 128 publications

(122 citation statements)

References 29 publications

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“…Therefore, it would be reasonable to assume that leptin may be applicable for clinical use in other non--lipodystrophy leptin-deficient conditions. Indeed, leptin has previously been investigated in hypothalamic amenorrhea with positive results (40,41). Finally, age-and sex-specific effects cannot be discounted as possible sources of variation and error, and future clinical trials should investigate these aspects, as some studies assessed here included only children, and others only women ( Table 2).…”

Section: Discussionmentioning

confidence: 99%

Leptin replacement therapy for the treatment of non-HAART associated lipodystrophy syndromes: a meta-analysis into the effects of leptin on metabolic and hepatic endpoints

Rodríguez

Neeman

Giles

et al. 2014

Arq Bras Endocrinol Metab

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RESUMOAs manifestações clínicas das síndromes lipodistróficas (SL) incluem hipoleptinemia, hiperglicemia, resistência insulínica, dislipidemia e esteatose hepática. A terapia de reposição de leptina (TRL) melhora tais parâmetros, mas atualmente não há dados compilados demonstrando tal efeito. Uma revisão sistemática dos bancos de dados MEDLINE e Cochrane Library identificou estudos avaliando os efeitos da TRL sobre parâmetros metabólicos e hepáticos em pacientes com SL não associadas ao uso de antirretrovirais. Diferenças médias padronizadas (DMP) e intervalos de confiança de 95% foram calculados a partir dos resultados, para os efeitos da TRL sobre a homeostase da glicose, perfil lipídico, e morfologia/função hepática, usando um modelo de variação inversa e efeitos randômicos. Após a triagem, 12 estudos foram incluídos para revisão. A metanálise dos resultados de 226 pacientes mostrou que a TRL reduziu a glicemia de jejum [0,75 DMP (amplitude 0,13), p = 0,0001], HbA1c [0,49 (0,17-0,81)

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Section: Discussionmentioning

confidence: 99%

Leptin replacement therapy for the treatment of non-HAART associated lipodystrophy syndromes: a meta-analysis into the effects of leptin on metabolic and hepatic endpoints

Rodríguez

Neeman

Giles

et al. 2014

Arq Bras Endocrinol Metab

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“…Leptin has been used to treat other features of lipodystrophy, such as steatohepatitis [27] and polycystic ovary syndrome [28,29]. It has also been shown to have beneficial effects in congenital leptin deficiency, the RabsonMendenhall syndrome and functional hypothalamic amenorrhoea [30][31][32][33].…”

Section: Discussionmentioning

confidence: 99%

Efficacy of leptin therapy in the different forms of human lipodystrophy

et al. 2009

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Aims/hypothesis Lipodystrophy is a rare disorder characterised by loss of adipose tissue, hypoleptinaemia, severe insulin resistance, diabetes and dyslipidaemia. The aims of this study were to determine whether leptin replacement in lipodystrophy patients ameliorates their metabolic abnormalities over an extended period of time and whether leptin therapy is effective in the different forms of lipodystrophy.

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“…Cushing's syndrome, characterized by endogenous or exogenous hypercortisolism, is another important differential diagnosis of FPLD2 [29]. The main symptoms associated with the excess of glucocorticoids are weight gain, lethargy, weakness, menstrual irregularity, loss of libido, hirsutism, acne, purple cutaneous stretch marks, hyperpigmentation, depression, and psychosis.…”

Section: Differential Diagnosismentioning

confidence: 99%

Dunnigan-Type Familial Partial Lipodystrophy: Understanding and Treating the Syndrome

Santos¹,

Ferreira²,

Benazzi³

et al. 2017

OJEMD

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Dunnigan-type partial lipodystrophy, which is characterized by a number of metabolic alterations, change in body fat distribution, and autosomal dominant inheritance pattern, is rare in the general population. Objective: To report the case of an adolescent with clinical and laboratory findings suggestive of Dunnigan-type partial lipodystrophy. Methods: Case report and literature review. Results: A 15-year-old adolescent presented at the clinic complaining of darkening of skin folds on her trunk and back. During physical examination, the presence of serious acanthosis nigricans in her cervical region, axillae, and intergluteal space was noted. Hirsutism in androgen-dependent areas was also observed, as well as relevant reduction of subcutaneous adipose tissue in the limbs, gluteal region, abdomen, and trunk and fat accumulation in the face and chin. Discussion. Dunnigan-type familial partial lipodystrophy is a rare dominant autosomal disease resulting from a heterozygous missense mutation in the LMNA gene, known as LPF type 2 (Dunnigan variant), which encodes the nuclear protein A/C-type lamin. It is characterized by the progressive disappearance of the subcutaneous adipose tissue in the limbs, gluteal region, abdomen, and trunk, with onset in puberty, followed by fat accumulation in other areas such as the face, chin, labia majora, and intra-abdominal region, leading to hypertrophy that may mimic the Cushing's syndrome phenotype. Affected patients display marked insulin resistance and may consequently develop diabetes mellitus, acanthosis nigricans, hirsutism, and polycystic ovary syndrome. Conclusion: This case report highlights the importance of suspecting Dunnigan-type familial partial lipodystrophy in clinical practice. Early clinical diagnosis allows for measures that minimize the severe metabolic disorders associated with this disease and, consequently, these adolescents' self-esteem issues.

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The long-term effect of recombinant methionyl human leptin therapy on hyperandrogenism and menstrual function in female and pituitary function in male and female hypoleptinemic lipodystrophic patients

Cited by 128 publications

References 29 publications

Leptin replacement therapy for the treatment of non-HAART associated lipodystrophy syndromes: a meta-analysis into the effects of leptin on metabolic and hepatic endpoints

Leptin replacement therapy for the treatment of non-HAART associated lipodystrophy syndromes: a meta-analysis into the effects of leptin on metabolic and hepatic endpoints

Efficacy of leptin therapy in the different forms of human lipodystrophy

Dunnigan-Type Familial Partial Lipodystrophy: Understanding and Treating the Syndrome

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