Document Detail


Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature.
MedLine Citation:
PMID:  14747765     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe clinical features, body fat distribution, and prevalence of metabolic abnormalities in 35 patients with acquired partial lipodystrophy (APL) seen by us over 8 years, and also review 220 cases of APL described in the literature. Based on the review and our experience, we propose that the essential diagnostic criterion for APL is the gradual onset of bilaterally symmetrical loss of subcutaneous fat from the face, neck, upper extremities, thorax, and abdomen, in the "cephalocaudal" sequence, sparing the lower extremities. Analysis of the pooled data revealed that female patients were affected approximately 4 times more often than males. The median age of the onset of lipodystrophy was 7 years. Several autoimmune diseases, in particular systemic lupus erythematosus and dermatomyositis, were associated with APL. The prevalence rates of diabetes mellitus and impaired glucose tolerance were 6.7% and 8.9%, respectively. Approximately 83% of APL patients had low complement (C) 3 levels and the presence of polyclonal immunoglobulin C3 nephritic factor. Twenty-two percent of patients developed membranoproliferative glomerulonephritis (MPGN) after a median of approximately 8 years following the onset of lipodystrophy. Compared with patients without renal disease, those with MPGN had earlier age of onset of lipodystrophy (12.6 +/- 10.3 yr vs 7.7 +/- 4.4 yr, respectively; p < 0.001) and a higher prevalence of C3 hypocomplementemia (78% vs 95%, respectively; p = 0.02). The pathogenesis of fat loss and MPGN in patients with APL remains unclear, but activation of an alternate complement pathway has been implicated. Treating the cosmetic disfigurement by surgical procedures has yielded inconsistent results. The use of thiazolidinediones to treat fat loss in patients with APL remains anecdotal. Prognosis is mainly determined by renal insufficiency due to MPGN.
Authors:
Anoop Misra; Aparna Peethambaram; Abhimanyu Garg
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review    
Journal Detail:
Title:  Medicine     Volume:  83     ISSN:  0025-7974     ISO Abbreviation:  Medicine (Baltimore)     Publication Date:  2004 Jan 
Date Detail:
Created Date:  2004-01-28     Completed Date:  2004-02-24     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  2985248R     Medline TA:  Medicine (Baltimore)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  18-34     Citation Subset:  AIM; IM    
Affiliation:
Department of Internal Medicine, Center for Human Nutrition, University of Texas Southwestern Medical Center at Dallas, 75390-9052, USA.
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MeSH Terms
Descriptor/Qualifier:
Adipose Tissue / pathology*,  radiography,  surgery
Adolescent
Adult
Antilipemic Agents / therapeutic use
Body Composition
Child
Diabetes Complications
Diabetes Mellitus / drug therapy
Female
Glomerulonephritis, Membranoproliferative / drug therapy,  etiology,  pathology
Glucose Intolerance / etiology*,  therapy
Humans
Hypertriglyceridemia / drug therapy,  etiology
Leptin / metabolism,  therapeutic use
Lipodystrophy / diagnosis*,  metabolism*,  therapy
Magnetic Resonance Imaging
Male
Middle Aged
Reconstructive Surgical Procedures / methods
Treatment Outcome
Grant Support
ID/Acronym/Agency:
M01-RR00633/RR/NCRR NIH HHS; R01-DK54387/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/Antilipemic Agents; 0/Leptin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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