title: Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.
abstract: BACKGROUND
  Lipodystrophy syndromes are characterized by generalized or partial absence of adipose tissue.
  
  
  OBJECTIVE
  We conducted a systematic review to synthesize data on clinical and metabolic features of lipodystrophy (age at onset, < 18 years).
  
  
  METHODS
  Sources included Medline, Embase, Cochrane Library, Scopus and Non-Indexed Citations from inception through January 2016.
  
  
  METHODS
  Search terms included lipodystrophy, and age 0 to 18 years. Patients with unambiguous diagnosis of lipodystrophy were included. Lipodystrophy secondary to HIV treatment was excluded.
  
  
  RESULTS
  We identified 1141 patients from 351 studies. Generalized fat loss involving face, neck, abdomen, thorax, and upper and lower limbs was explicitly reported in 65% to 93% of patients with congenital generalized lipodystrophy (CGL) and acquired generalized lipodystrophy (AGL). In familial partial lipodystrophy (FPL), fat loss occurred from upper and lower limbs, with sparing of face and neck. In acquired partial lipodystrophy (APL), upper limbs were involved while lower limbs were spared. Other features were prominent musculature, acromegaloid, acanthosis nigricans and hepatosplenomegaly. Diabetes mellitus was diagnosed in 48% (n = 222) of patients with CGL (mean age at onset, 5.3 years). Hypertriglyceridemia was observed in CGL, AGL and FPL. Multiple interventions were used, with most patients receiving ≥ 3 interventions and being ≥ 18 years of age at the initiation of interventions.
  
  
  CONCLUSIONS
  To our knowledge, this is the largest reported pooled database describing lipodystrophy patients with age at onset < 18 years. We have suggested core and supportive clinical features and summarized data on available interventions, outcomes and mortality.
authors.affiliation: Evidence-Based Practice Center and.|Division of Pediatric Endocrinology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905.
authors.forename: Nidhi
authors.initials: N
authors.lastname: Gupta
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Noor
authors.initials: N
authors.lastname: Asi
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Wigdan
authors.initials: W
authors.lastname: Farah
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Jehad
authors.initials: J
authors.lastname: Almasri
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Patricia
authors.initials: P
authors.lastname: Barrionuevo
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Mouaz
authors.initials: M
authors.lastname: Alsawas
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: Zhen
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, Children's Nutrition Research Center, Baylor College of Medicine, Houston, Texas 77030; and.
authors.forename: Morey W
authors.initials: MW
authors.lastname: Haymond
authors.affiliation: Diabetes, Endocrinology and Obesity Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892.
authors.forename: Rebecca J
authors.initials: RJ
authors.lastname: Brown
authors.affiliation: Evidence-Based Practice Center and.
authors.forename: M Hassan
authors.initials: MH
authors.lastname: Murad
mesh_terms: D002648:Child; D039682:HIV-Associated Lipodystrophy Syndrome; D006801:Humans; D008060:Lipodystrophy / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D052497:Lipodystrophy, Congenital Generalized / Q000175:diagnosis / Q000401:mortality / Q000628:therapy