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The Journal of Clinical Endocrinology... Feb 2017Lipodystrophy syndromes are characterized by generalized or partial absence of adipose tissue. (Review)
Review
CONTEXT
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).
DATA SOURCE
Sources included Medline, Embase, Cochrane Library, Scopus and Non-Indexed Citations from inception through January 2016.
STUDY SELECTION
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.
DATA SYNTHESIS
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.
Topics: Child; HIV-Associated Lipodystrophy Syndrome; Humans; Lipodystrophy; Lipodystrophy, Congenital Generalized
PubMed: 27967300
DOI: 10.1210/jc.2016-2271 -
Danish Medical Journal Jan 2017Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations:... (Review)
Review
INTRODUCTION
Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations: acanthosis nigricans (AN), skin tags (ST), diabetic dermopathy (DD), rubeosis faciei (RF), pruritus (PR), granuloma annulare (GA), necrobiosis lipoidica (NL), scleroedema diabeticorum (SD) and bullosis diabeticorum (BD). These conditions possibly relate to underlying diabetogenic mechanisms. Our aim was to determine whether skin signs are feasible as cutaneous markers for the prediabetic or diabetic state.
METHODS
Data were collected from the databases PubMed, Embase and Cochrane. Articles were excluded if the populations presented with comorbidities or received treatment with drugs affecting the skin. Also, animal studies, studies with poor methodology and pilot studies were excluded.
RESULTS
Among the 34 included original articles, an association with diabetes was shown as follows: in eight articles with AN, five articles with ST, three articles with GA, two articles with NL, PR and SD respectively and in one article with RF. Three papers indirectly showed an association of DD with diabetes. Association between bullous skin lesions and diabetes was only documented by case reports and case series.
CONCLUSION
The results indicate a benefit of diabetes screening in individuals presenting with AN, ST or BD. Further studies are required to enlighten a possible association with RF, GA, SD or NL. Until such studies are available, it is advisable to screen individuals with the skin lesions presented by measuring their glycated haemoglobin.
Topics: Acanthosis Nigricans; Biomarkers; Blister; Blood Glucose; Diabetes Complications; Facial Dermatoses; Glycated Hemoglobin; Granuloma Annulare; Humans; Necrobiosis Lipoidica; Prediabetic State; Pruritus; Scleroderma, Localized; Skin Diseases
PubMed: 28007053
DOI: No ID Found