Table 1.
Clinical Findings of β thalassemia intermedia cohort in Sri Lanka.
| Clinical Findings | All β thalassemia intermedia n = 50 |
β thalassemia intermedia disease severity | P value | ||
|---|---|---|---|---|---|
| Mild n = 26 |
Moderate n = 12 |
Severe n = 12 |
|||
| Age at first presentation (years) |
8.5 (5.0–26.0) |
10.0 (5.4–25.8) |
19.5 (6.0–46.2) |
4.0 (0.83–8.0) |
0.010 |
| No. patients received > = 1 blood transfusion |
43/50 (86%) |
19/26 (38%) |
12//12 (100%) |
12/12 (100%) |
0.021 |
| Age at first transfusion (years) |
10.5 (5.0–28.8) |
17.0 (10.0–31.0) |
19.5 (6.5–51.2) |
4.0 (2.3–8.0) |
0.007 |
| Average No. blood transfusions per year |
1.3 (0.31–5.0) |
0.41 (0.0–1.2) |
3.3 (1.2–4.6) |
7.8 (5.9–9.0) |
<0.001 |
| Age at clinical review (years) |
23.5 (12.8–38.2) |
25.5 (15.2–38.5) |
37.5 (18.8–57.8) |
18.0 (6.8–22.8) |
0.014 |
| Splenectomised |
6/50 (12.0%) |
2/26 (7.7%) |
2/12 (16.7%) |
2/12 (16.7%) |
0.605 |
| Splenomegaly |
30/44 (68.2%) |
11/24 (45.8%) |
10/10 (100.0%) |
9/10 (90.0%) |
0.002 |
| Spleen size (cm) |
7.0 (4.8–10.2) |
6.0 (9.0–13.0) |
4.5 (2.8–8.0) |
8.0 (5.0–10.0) |
0.110 |
| Hepatomegaly |
19/50 (38.0%) |
6/26 (23.1%) |
4/12 (33.3%) |
9/12 (75%) |
0.010 |
| Jaundice |
34/50 (68.0%) |
21/26 (80.8%) |
10/12 (83.3%) |
3/12 (25.0%) |
0.002 |
| Gall stones |
5/50 (10.0%) |
3/26 (11.5%) |
2/12 (16.7%) |
0/12 (0.0%) |
0.410 |
| Cholecystectomy |
3/50 (6.0%) |
2/26 (7.7%) |
1/12 (8.3%) |
0/12 (0.0%) |
0801 |
| Leg ulcers |
4/50 (8.0%) |
2/26 (7.7%) |
2/12 (16.7%) |
0/12 (0.0%) |
0.526 |
| Severe Facial changes |
7/50 (14%) |
2/26 (7.7%) |
0/12 (0.0%) |
5/12 (41.7%) |
0.011 |
| Pulmonary Hypertension | |||||
| No pulmonary Hypertension |
26/39 (66.7%) |
9/19 (34.6%) |
6/9 (50%) |
11/11 (91.7%) |
0.004 |
|
Mild pulmonary Hypertension (PAP 25–40 mmHg) |
13/39 (33.3%) |
10/19 (38.5%) |
3/9 (25%) |
0/11 (0%) |
0.029 |
| BMI |
17.6 (15.3–21.0) |
18.7 (15.8–20.7) |
17.2 (14.7–19.9) |
16.6 (14.4–19.2) |
0.112 |
| Completed Growth |
35/50 (79%) |
19/26 (73.1%) |
9/12 (75.0%) |
7/12 (58.3%) |
0.71 |
| Age appropriate Tanner staging |
37/50 (74.0%) |
22/26 (84.6%) |
9/12 (75.0%) |
6/12 (50.0%) |
0.091 |
| Delayed puberty |
6/50 (12.0%) |
1/26 (3.8%) |
2/12 (16.7%) |
3/12 (25.0%) |
0.113 |
| Children with Tanner 1 (3–8 years) |
7/50 (14%) |
3/26 (11.5%) |
1/12 (8.3%) |
3/12 (25%) |
0.486 |
| Number on Iron chelation |
23/50 (46%) |
6/26 (23%) |
6/12 (50%) |
11/12 (91.6%) |
<0.001 |
Data are median (IQR) or number (%).