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Table 1 Clinical and demographical data of patients with BSS

From: Immunological features of patients affected by Barraquer-Simons syndrome

VariableBSS1BSS2BSS3BSS4BSS5BSS6BSS7BSS8BSS9BSS10BSS11BSS12BSS13Reference
SexFFFFFFFFMMFFF 
Age (years)7613158494241445211621012 
Lipodystrophy onset (years)N/A2549417367877 
BMI (kg/m2)24.319.31816.319.822.333.822.723.521.326.116.522.718–25
% total fataN/AN/A17.72220.327.841.82328.733.229.131.242.625–31 (Women)
18–24 (Men)
AcanthosisNoNoNoNoNoNoNoNoNoNoNoNoNoYes/No
Glucose (mg/dL)798569868393821028490107948460–110
Insulin (μU/mL)15.810.410.713.5N/AN/A18.59.81314.333.394.742.6–24.9
DiabetesNoNoNoNoNoNoNoNoNoNoYesNoNoYes/No
HbA1c (%)N/A4.8N/A5.35.2N/A5.4N/AN/A5.26.45.15.24.0–5.5
HypoleptinemiaNoNoYesNoNoNoNoNoNoNoNoNoNoYes/No
Triglycerides (mg/dL)1067911089786610521044561265795325–115
Total colesterol (mg/dL)129145122163238183158120245112175167159110–230
HDL-cholesterol (mg/dL)5762N/A4269706335454537446640–70
LDL-cholesterol (mg/dL)120109N/A112153.4100741111636411012610150–129
Fatty liverbNoNoNoNoNoNoYesNoNoYesYesNoNoYes/No
ALT (UI/L)332813243120602121152422285–31
AST (UI/L)512423332619391515242525205–32
HypertensionYesNoNoNoNoNoNoNoYesNoYesNoNoYes/No
Renal diseaseYes (DDD)NoYes (IgAN)NoNoNoNoNoYes (C3GN)NoNoNoNoYes/No
  1. Abbreviations: F female, M male, BMI Body Mass Index, HbA1c Glycohemoglobin, ALT Alanine aminotransferase, AST Aspartate aminotransferase; DDD Dense deposit disease, IgAN IgA nephropathy, C3GN C3 glomerulonephritis, N/A Not available
  2. a Percentage of total fat was measured using dual-energy x-ray absorptiometry
  3. b Liver steatosis was assessed by means of ultrasonography