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Table 2 Summary of clinical features observed between severe Hb S/β+, Hb S/β0 and Hb SS groups

From: Sickle cell disease in Sri Lanka: clinical and molecular basis and the unanswered questions about disease severity

Clinical feature/Complication

severe Hb S/β+

group

n = 37

Hb S/β0 group

n = 14

Hb SS group

n = 9

Cumulative Figure

N = 60

p value1 between two SBT groups

p value2 between all three groups

Joint pain

20 (54.05%)

12 (85.70%)

7 (77.78%)

39 (65.0%)

0.053

0.080

Palpable spleen

26 (70.27%)

7 (50.0%)

3 (33.33%)

36 (60.0%)

0.204

0.086

Hospital admission due to pain

20 (54.05%)

10 (71.43%)

5 (55.56%)

35 (58.33%)

0.346

0.562

Jaundice

14 (37.84%)

9 (64.28%)

6 (66.67%)

29 (48.33%)

0.120

0.109

Major infections

13 (35.14%)

4 (28.57%)

4 (44.44%)

21 (35.0%)

0.749

0.744

Recurrent headaches

11 (29.73%)

1 (7.14%)

2 (22.22%)

14 (23.33%)

0.142

0.281

Pica

6 (16.22%)

3 (21.43%)

2 (22.22%)

11 (18.33%)

0.692

0.711

Abdominal pain

6 (16.22%)

4 (28.57%)

0

10 (16.67%)

0.432

0.217

Dactylitis

3 (8.11%)

3 (21.43%)

4 (44.44%)

10 (16.67%)

0.327

0.027*

Gallstones

6 (16.22%)

3 (21.43%)

1 (11.11%)

10 (16.67%)

0.692

0.888

Pallor

4 (10.81%)

2 (14.28%)

3 (33.33%)

9 (15.0%)

0.661

0.220

Acute chest syndrome

3 (8.11%)

3 (21.43%)

2 (22.22%)

8 (13.33%)

0.327

0.290

Vision impairment

8 (21.62%)

0

0

8 (13.33%)

0.088

0.074

Abdominal distension

3 (8.11%)

1 (7.14%)

1 (11.11%)

5 (8.33%)

1.000

1.000

Facial deformities

1 (2.70%)

2 (14.28%)

0

3 (5.0%)

0.179

0.189

Nocturnal enuresis

2 (5.40%)

1 (7.14%)

0

3 (5.0%)

1.000

1.000

Leg ulcers

2 (5.40%)

1 (7.14%)

0

3 (5.0%)

1.000

1.000

  1. 1Fisher’s exact test p < 0.05 was taken as significant between two SBT groups
  2. 2Fisher’s exact test p < 0.05 was taken as significant between all three groups