A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major.
OBJECTIVE
Iron overload is a major problem in patients with beta-thalassemia major, and it has many structural and metabolic consequences. The aim of this study was evaluation of endocrine disturbances in patients with beta-thalassemia major who were older than 10 years of age.


METHODS
In this cross-sectional study, investigators collected demographic data and medical histories, as well as menstrual history in females, from the medical records of 56 patients with beta-thalassemia major. Patients were examined to determine their pubertal status and the standard deviation score for height for evaluation of short stature. For evaluation of glucose tolerance, a fasting blood glucose and oral glucose tolerance test were performed. Evidence for diabetes mellitus was based on American Diabetes Association and World Health Organization criteria. Serum levels of calcium, phosphorous, thyroid-stimulating hormone, free thyroxin, luteinizing hormone and follicular-stimulating hormone, and estradiol in girls and testosterone in boys were measured.


RESULTS
The mean and standard deviation for age in the 56 patients (36 males and 20 females) was 15.62+/-4.44 years. Diabetes mellitus was present in 5 patients (8.9%), impaired fasting glucose was found in 16 patients (28.6%) and an impaired glucose tolerance test was found in 4 patients (7.1%). Short stature (standard deviation score <-2) was seen in 25 (70%) boys and 14 (73%) girls. Impaired puberty was found in 40 patients (71%). Hypocalcaemia and primary overt hypothyroidism were present in 23 (41%) and 9 patients (16%), respectively.


CONCLUSIONS
Despite therapy with deferoxamine to treat iron overload, the risk of secondary endocrine dysfunction remained high. Hypogonadism was one of the most frequent endocrine complications. Impaired glucose tolerance, short stature, hypocalcemia, subclinical and overt hypothyroidism are also frequent.
Najafipour
Farzad
F
Endocrinology and Metabolism section, Department of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. najafipourf@tbzmed.ac.ir
Aliasgarzadeh
Akbar
A
Aghamohamadzadeh
Naser
N
Bahrami
Amir
A
Mobasri
Majid
M
Niafar
Mitra
M
Khoshbaten
Manouchehr
M
D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D003430:Cross-Sectional Studies; D003676:Deferoxamine / Q000627:therapeutic use; D003920:Diabetes Mellitus / Q000453:epidemiology*; D005260:Female; D005951:Glucose Tolerance Test; D006058:Gonadal Disorders / Q000453:epidemiology / Q000209:etiology*; D006130:Growth Disorders / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D006996:Hypocalcemia / Q000453:epidemiology / Q000209:etiology; D007037:Hypothyroidism / Q000453:epidemiology / Q000209:etiology; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology; D008297:Male; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications*
D002614:Chelating Agents; D003676:Deferoxamine
Arch Iran Med. 2006 Oct;9(4):329-34
17061604
J Pediatr Endocrinol Metab. 1998;11 Suppl 3:835-44
10091154
Acta Haematol. 1990;84(2):72-6
2120889
Haematologica. 2004 Oct;89(10):1187-93
15477202
J Trop Pediatr. 1994 Oct;40(5):261-6
7807619
Indian Pediatr. 1992 Aug;29(8):997-102
1459722
Eur J Pediatr. 2003 Mar;162(3):139-146
12655415
Br J Haematol. 2006 Aug;134(4):438-44
16822284
Ceylon Med J. 1999 Dec;44(4):166-8
10895267
Br J Haematol. 2006 Nov;135(4):574-82
17054676
Clin Endocrinol (Oxf). 1984 Apr;20(4):445-50
6424976
Acta Paediatr Suppl. 1994 Dec;406:111-3
7734806
Diabetes Metab. 2001 Apr;27(2 Pt 1):149-54
11353881
Pediatr Diabetes. 2004 Sep;5(3):126-32
15450007
Clin Endocrinol (Oxf). 1993 Jan;38(1):55-61
8435886
J Pediatr Endocrinol Metab. 2000 Jun;13(6):651-6
10905390
BMC Endocr Disord. 2003 Aug 12;3(1):4
12914670
Diabetologia. 2001 Sep;44(9):1094-101
11596662
Clin Endocrinol (Oxf). 1995 Jun;42(6):581-6
7634497
J Pediatr Endocrinol Metab. 2003 Mar;16 Suppl 2:259-66
12729401
Diabetes Care. 2001 May;24(5):850-4
11347742
Pediatr Endocrinol Rev. 2004 Dec;2 Suppl 2:249-55
16462705
Acta Haematol. 1992;88(2-3):105-8
1466190
Folia Med (Plovdiv). 1994;36(1):29-34
7867989
J Trop Pediatr. 1999 Feb;45(1):23-30
10191589
J Pediatr Endocrinol Metab. 1997 Mar-Apr;10(2):175-84
9364350
J Pediatr Endocrinol Metab. 2006 Apr;19(4):471-80
16759032
J Pediatr Hematol Oncol. 2003 Nov;25(11):880-4
14608198
Ann Pediatr (Paris). 1985 Nov;32(9):809-11
4091451
Ann N Y Acad Sci. 1998 Jun 30;850:475-8
9668589
J Pediatr Endocrinol Metab. 2006 Dec;19(12):1397-404
17252692