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Table 1 Characteristics of the Alström patients studied.

From: Cardiac magnetic resonance imaging in Alström syndrome

 

Normal Values

1

2

3

4

5

6

7

Age yrs

 

37

24

43

19

39

26

23

Gender

 

M

F

M

F

M

F

M

BMI

<25

47

36

34

33

32

29

29

Genetically confirmed AS

 

heterozygote

yes

yes

yes

yes

yes

yes

Infantile cardiomyopathy

 

no

yes

no

no

yes

yes

no

Blood Pressure

 

134/56

90/60

112/72

110/56

145/90

100/64

132/81

eGFR

 

72

57

58

>90

35

44

>90

Ejection Fraction on CMR

>55%

61

25

40

60

59

55

50

Clinical failure in last 12 months

 

no

yes

no

no

no

yes

no

NTBNP ng/L

<150

24

108

65

14

279

495

6

Triglycerides mmol/L

<1.5

7.3

2.6

8.8

2.3

4.0

4.0

1.95

Chol:HDL

 

5.4

5.6

8.3

4.3

4.6

8.5

6.72

HBA1C %

4.0–6.1

11.0

8.5

9.4

5.2

16.8

6.2

5.2