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Table 1 Summary of genotypic, metabolic and cardiovascular data of patients with CGL (n = 10)

From: Association between cardiovascular autonomic neuropathy and left ventricular hypertrophy in young patients with congenital generalized lipodystrophy

Case

Gender/age

Pubertal stage

Subtype mutation (gene)

Comorbidities, microvascular complications

Drugs

LVMI (g/m2.7)

cIMT (mm)

cQT (s)a

CAN

LVH

1

♀, 7 years

M2P2

Type 2 CGL

BSCL2

High HOMA-IR score, ↓HDL, ↑TG, nephropathy (moderate albuminuria), ↑BP

MTF

63.9

0.61

0.43

Clinical

LVH

2

♀, 7 years

M2P2

Type 1 CGL

AGPAT2

High HOMA-IR score,

↓HDL, ↑TG

None

32.8

0.66

0.45a

Absent

No LVH

3

♂, 9 years

G1P1

DNA

Not available

High HOMA-IR score,

↓HDL, ↑TG

None

36.2

0.57

0.41

Absent

No LVH

4

♀, 10 years

M2P2

Type 2 CGL

BSCL2

DM, high HOMA-IR score, ↓HDL, ↑TG

MTF

44.5

0.53

0.41

Clinical

No LVH

5

♂, 10 years

G2P1

Type 2 CGL

BSCL2

DM, ↓HDL, ↑TG, ↑cholesterol, nephropathy (severe albuminuria)

MTF, insulin

29.9

0.54

0.44a

Absent

No LVH

6

♂, 14 years

G4P4

DNA

Not available

DM, ↓HDL, ↑TG, ↑cholesterol

MTF

37.8

0.54

0.45a

Absent

No LVH

7

♀, 14 years

M4P4

Type 2 CGL

BSCL2

DM, ↓HDL, ↑TG, ↑cholesterol, nephropathy (severe albuminuria), peripheral neuropathy, ↑BP

MTF, acarbose, insulin, ciprofibrate

88.7

0.75

0.49a

Clinical

LVH

8

♂, 14 years

G5P5

Type 2 CGL

BSCL2

DM, high HOMA-IR score, ↓HDL, ↑TG, nephropathy (moderate albuminuria)

MTF

65.3

0.57

0.41

Absent

LVH

9

♀, 25 years

M5P5

Type 1 CGL

AGPAT2

DM, ↓HDL, ↑TG, ↑cholesterol, ↑BP, nephropathy (severe albuminuria), peripheral neuropathy

MTF, insulin, ciprofibrate

154.0*

0.64

0.47a

Clinical/

Advanced

LVH

10

♀, 30 years

M5P5

DNA

Not available

DM, ↓HDL-c, ↑TG, ↑BP, nephropathy (severe albuminuria)

MTF, insulin, losartan

88.6*

0.60

0.42

Incipient

No LVH

  1. CGL, congenital generalized lipodystrophy; CAN, cardiovascular autonomic neuropathy; HDL, high-density lipoprotein; HOMA-IR, homeostasis model assessment-insulin resistance; LVH, left ventricular hypertrophy; LVMI, left ventricular mass index; TG, triglycerides; BP, blood pressure; DM, diabetes mellitus; MTF, metformin
  2. * g/m2
  3. acQT prolongation