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The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline.

The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline.
Author Information (click to view)

Brown RJ, Araujo-Vilar D, Cheung PT, Dunger D, Garg A, Jack M, Mungai L, Oral EA, Patni N, Rother K, von Schnurbein J, Sorkina E, Stanley T, Vigouroux C, Wabitsch M, Williams R, Yorifuji T,


Brown RJ, Araujo-Vilar D, Cheung PT, Dunger D, Garg A, Jack M, Mungai L, Oral EA, Patni N, Rother K, von Schnurbein J, Sorkina E, Stanley T, Vigouroux C, Wabitsch M, Williams R, Yorifuji T, (click to view)

Brown RJ, Araujo-Vilar D, Cheung PT, Dunger D, Garg A, Jack M, Mungai L, Oral EA, Patni N, Rother K, von Schnurbein J, Sorkina E, Stanley T, Vigouroux C, Wabitsch M, Williams R, Yorifuji T,

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The Journal of clinical endocrinology and metabolism 2016 Oct 6() jc20162466

Abstract
OBJECTIVE
Lipodystrophy syndromes are extremely rare disorders of deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia, and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and management. This practice guideline summarizes diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs.

PARTICIPANTS
Seventeen participants were nominated by worldwide endocrine societies or selected by the committee as content experts. Funding was via unrestricted educational grant (Astra Zeneca) to the Pediatric Endocrine Society. Meetings were not open to the general public.

EVIDENCE
Literature review was conducted by the committee. Recommendations of the committee were graded using the system of the American Heart Association. Expert opinion was used when published data were not available or scarce.

CONSENSUS PROCESS
The guideline was drafted by committee members, and reviewed, revised, and approved by the entire committee during group meetings. Contributing societies reviewed the document and provided approval.

CONCLUSIONS
Lipodystrophy syndromes are heterogeneous, and are diagnosed by clinical phenotype, supplemented by genetic testing in certain forms. Patients with most lipodystrophy syndromes should be screened for diabetes, dyslipidemia, and liver, kidney, and heart disease annually. Diet is essential for management of metabolic complications of lipodystrophy. Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized lipodystrophy, and selected patients with partial lipodystrophy. Other treatments not specific for lipodystrophy may be helpful as well (e.g. metformin for diabetes, statins or fibrates for hyperlipidemia). Oral estrogens are contraindicated.

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