Literature DB >> 30805888

Long-term effectiveness and safety of metreleptin in the treatment of patients with partial lipodystrophy.

Elif A Oral1, Phillip Gorden2, Elaine Cochran2, David Araújo-Vilar3, David B Savage4, Alison Long5, Gregory Fine5, Taylor Salinardi5, Rebecca J Brown2.   

Abstract

PURPOSE: To evaluate the effects of metreleptin in patients with partial lipodystrophy (PL).
METHODS: Patients aged ≥ 6 months with PL, circulating leptin < 12.0 ng/mL, and diabetes mellitus, insulin resistance, or hypertriglyceridemia received metreleptin doses (once or twice daily) titrated to a mean of 0.124 mg/kg/day. Changes from baseline to month 12 in glycated hemoglobin (HbA1c) and fasting serum triglycerides (TGs; co-primary endpoints), fasting plasma glucose (FPG), and liver volume were evaluated. Additional assessments included the proportions of patients achieving target decreases in HbA1c or fasting TGs at month 12, long-term treatment effects, and treatment-emergent adverse events (TEAEs).
RESULTS: Significant (p < 0.05) reductions in HbA1c (-0.6%), fasting TGs (-20.8%), FPG (-1.2 mmol/L), and liver volume (-13.4%) were observed in the overall PL population at month 12. In a subgroup of patients with baseline HbA1c ≥ 6.5% or TGs ≥ 5.65 mmol/L, significant (p < 0.05) reductions were seen in HbA1c (-0.9%), fasting TGs (-37.4%), FPG (-1.9 mmol/L), and liver volume (-12.4%). In this subgroup, 67.9% of patients had a ≥ 1% decrease in HbA1c or ≥ 30% decrease in fasting TGs, and 42.9% had a ≥ 2% decrease in HbA1c or ≥ 40% decrease in fasting TGs. Long-term treatment in this subgroup led to significant (p < 0.05) reductions at months 12, 24, and 36 in HbA1c, fasting TGs, and FPG. Metreleptin was well tolerated with no unexpected safety signals. The most common TEAEs were abdominal pain, hypoglycemia, and nausea.
CONCLUSIONS: In patients with PL, treatment with metreleptin was well tolerated and resulted in improvements in glycemic control, hypertriglyceridemia, and liver volume.

Entities:  

Keywords:  Diabetes mellitus; Leptin; Lipodystrophy; Metreleptin; Partial lipodystrophy

Mesh:

Substances:

Year:  2019        PMID: 30805888      PMCID: PMC7340120          DOI: 10.1007/s12020-019-01862-8

Source DB:  PubMed          Journal:  Endocrine        ISSN: 1355-008X            Impact factor:   3.633


  26 in total

Review 1.  Acquired and inherited lipodystrophies.

Authors:  Abhimanyu Garg
Journal:  N Engl J Med       Date:  2004-03-18       Impact factor: 91.245

2.  Clinical presentations, metabolic abnormalities and end-organ complications in patients with familial partial lipodystrophy.

Authors:  Baris Akinci; Huseyin Onay; Tevfik Demir; Şenay Savas-Erdeve; Ramazan Gen; Ilgin Yildirim Simsir; Fatma Ela Keskin; Mehmet Sercan Erturk; Ayse Kubat Uzum; Guzin Fidan Yaylali; Nilufer Kutbay Ozdemir; Tahir Atik; Samim Ozen; Banu Sarer Yurekli; Tugce Apaydin; Canan Altay; Gulcin Akinci; Leyla Demir; Abdurrahman Comlekci; Mustafa Secil; Elif Arioglu Oral
Journal:  Metabolism       Date:  2017-04-27       Impact factor: 8.694

3.  Lipid regulation in lipodystrophy versus the obesity-associated metabolic syndrome: the dissociation of HDL-C and triglycerides.

Authors:  Jalaja Joseph; Robert D Shamburek; Elaine K Cochran; Phillip Gorden; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2014-06-13       Impact factor: 5.958

4.  Partial and generalized lipodystrophy: comparison of baseline characteristics and response to metreleptin.

Authors:  Talia Diker-Cohen; Elaine Cochran; Phillip Gorden; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2015-03-03       Impact factor: 5.958

5.  Comparison of efficacy and safety of leptin replacement therapy in moderately and severely hypoleptinemic patients with familial partial lipodystrophy of the Dunnigan variety.

Authors:  Vinaya Simha; Lalitha Subramanyam; Lidia Szczepaniak; Claudia Quittner; Beverley Adams-Huet; Peter Snell; Abhimanyu Garg
Journal:  J Clin Endocrinol Metab       Date:  2011-12-14       Impact factor: 5.958

6.  Consequences of stopping and restarting leptin in an adolescent with lipodystrophy.

Authors:  F Kamran; K I Rother; E Cochran; E Safar Zadeh; P Gorden; R J Brown
Journal:  Horm Res Paediatr       Date:  2012-09-04       Impact factor: 2.852

7.  Efficacy of leptin therapy in the different forms of human lipodystrophy.

Authors:  A Y Chong; B C Lupsa; E K Cochran; P Gorden
Journal:  Diabetologia       Date:  2009-09-02       Impact factor: 10.122

8.  Efficacy and Safety of Metreleptin in Patients with Partial Lipodystrophy: Lessons from an Expanded Access Program.

Authors:  Nevin Ajluni; Moahad Dar; John Xu; Adam H Neidert; Elif A Oral
Journal:  J Diabetes Metab       Date:  2016-03-23

Review 9.  Diagnosis and treatment of lipodystrophy: a step-by-step approach.

Authors:  D Araújo-Vilar; F Santini
Journal:  J Endocrinol Invest       Date:  2018-04-27       Impact factor: 4.256

10.  Estimating the prevalence of generalized and partial lipodystrophy: findings and challenges.

Authors:  Elaine Chiquette; Elif A Oral; Abhimanyu Garg; David Araújo-Vilar; Praveen Dhankhar
Journal:  Diabetes Metab Syndr Obes       Date:  2017-09-13       Impact factor: 3.168

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  23 in total

1.  Effects of Metreleptin on Patient Outcomes and Quality of Life in Generalized and Partial Lipodystrophy.

Authors:  Keziah Cook; Kelly Adamski; Aparna Gomes; Edward Tuttle; Henner Kalden; Elaine Cochran; Rebecca J Brown
Journal:  J Endocr Soc       Date:  2021-02-16

2.  Comorbidities and Survival in Patients With Lipodystrophy: An International Chart Review Study.

Authors:  Baris Akinci; Elif A Oral; Adam Neidert; Diana Rus; Wendy Y Cheng; Philippe Thompson-Leduc; Hoi Ching Cheung; Pamela Bradt; Maria Cristina Foss de Freitas; Renan Magalhães Montenegro; Virgínia Oliveira Fernandes; Elaine Cochran; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2019-11-01       Impact factor: 5.958

3.  Metreleptin therapy for nonalcoholic steatohepatitis: Open-label therapy interventions in two different clinical settings.

Authors:  Baris Akinci; Angela Subauste; Nevin Ajluni; Nazanene H Esfandiari; Rasimcan Meral; Adam H Neidert; Akin Eraslan; Rita Hench; Diana Rus; Barbara McKenna; Hero K Hussain; Thomas L Chenevert; Marwan K Tayeh; Amit R Rupani; Jeffrey W Innis; Christos S Mantzoros; Hari S Conjeevaram; Charles L Burant; Elif A Oral
Journal:  Med (N Y)       Date:  2021-05-12

4.  Endogenous Leptin Concentrations Poorly Predict Metreleptin Response in Patients With Partial Lipodystrophy.

Authors:  Rasimcan Meral; Noemi Malandrino; Mary Walter; Adam H Neidert; Ranganath Muniyappa; Elif Arioglu Oral; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2022-03-24       Impact factor: 5.958

5.  UNUSUAL PRESENTATIONS OF LMNA-ASSOCIATED LIPODYSTROPHY WITH COMPLEX PHENOTYPES AND GENERALIZED FAT LOSS: WHEN THE GENETIC DIAGNOSIS UNCOVERS NOVEL FEATURES.

Authors:  Natalia Xavier S de Andrade; Suleyman Cem Adiyaman; Berna Demir Yuksel; Carla T Ferrari; Abdelwahab Jalal Eldin; Basak Ozgen Saydam; Canan Altay; Pratima Sharma; Nicole Bhave; Ann Little; Paul McKeever; Huseyin Onay; Sermin Ozkal; Mustafa Secil; Mustafa Nuri Yenerel; Baris Akinci; Elif A Oral
Journal:  AACE Clin Case Rep       Date:  2020-03-04

Review 6.  Leptin in Leanness and Obesity: JACC State-of-the-Art Review.

Authors:  Nikolaos Perakakis; Olivia M Farr; Christos S Mantzoros
Journal:  J Am Coll Cardiol       Date:  2021-02-16       Impact factor: 24.094

7.  Rapid amelioration of anorexia nervosa in a male adolescent during metreleptin treatment including recovery from hypogonadotropic hypogonadism.

Authors:  Jochen Antel; Susanne Tan; Marvin Grabler; Christine Ludwig; Dominik Lohkemper; Tim Brandenburg; Nikolaus Barth; Anke Hinney; Lars Libuda; Miriam Remy; Gabriella Milos; Johannes Hebebrand
Journal:  Eur Child Adolesc Psychiatry       Date:  2021-05-09       Impact factor: 5.349

8.  Effect of Leptin Therapy on Survival in Generalized and Partial Lipodystrophy: A Matched Cohort Analysis.

Authors:  Keziah Cook; Omer Ali; Baris Akinci; Maria Cristina Foss de Freitas; Renan Magalhães Montenegro; Virginia Oliveira Fernandes; Deepshekhar Gupta; Kai-Jye Lou; Edward Tuttle; Elif A Oral; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2021-07-13       Impact factor: 5.958

9.  Leptin Attenuates Cardiac Hypertrophy in Patients With Generalized Lipodystrophy.

Authors:  My-Le Nguyen; Vandana Sachdev; Thomas R Burklow; Wen Li; Megan Startzell; Sungyoung Auh; Rebecca J Brown
Journal:  J Clin Endocrinol Metab       Date:  2021-10-21       Impact factor: 6.134

10.  Case Report: Metreleptin Treatment in a Patient With a Novel Mutation for Familial Partial Lipodystrophy Type 3, Presenting With Uncontrolled Diabetes and Insulin Resistance.

Authors:  Vaia Lambadiari; Aikaterini Kountouri; Eirini Maratou; Stavros Liatis; George D Dimitriadis; Fredrik Karpe
Journal:  Front Endocrinol (Lausanne)       Date:  2021-06-08       Impact factor: 5.555

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