Short stature, body disproportion, and endocrine disorders in thalassemia major

الملخص AR

Objectives: Short stature and endocrine disorders are common complications of fi thalassemia major (TM).

The aim of this study was to determine the prevalence and causes of short stature, body disproportion, delayed puberty, and skeletal changes among thalassemia patients in our area.

Methods: A prospective study was done on 314 patients with TM, randomly chosen at the Thalassemic Treatment Center of Al Yarmook Teaching Hospital during the period from January 2, 2001 to April 30, 2001.

Patients ranged from 2-20 years of age and were divided into 5 groups.

The male to female ratio was 1.2:1.

The study included measurements of standing height, ratio of upper body to lower segment, puberty staging according to Tanner staging.

Estimation of the hormones for which assays were available including growth hormone, T4, TSH, LH, and FSH, were performed.

The liver enzymes AST and ALI, and serum calcium were measured.

Radiographs of the head, long bones, vertebrae, and wrist joints were done.

The abnormalities noted were correlated with the duration of the DFX chelation and serum ferritin levels.

Results: The study revealed that 28% of the patients exhibited short stature, predominantly in the age group 15- 20 years, and 25% of the patients had short trunk but normal stature predominantly in the age group of 10-14.

There was a significant relation to the duration of DFX chelation, p=0.0004.

Delayed puberty was present in 33/63 patients (52.4%); 19 were females (57.5%) and 14 were males (42.5%).

Endocrine disorders were present in 25.9%.

Hypo gonadotropic hypogonadism and hypocalcemia were present in 8.4% and 12% of the patients respectively.

There was a significant relation to the duration of the disease, p=0.00003 and 0.0004 respectively.

Skeletal changes including platyspondylosis, maxillary overgrowth, hair-on-end appearance, rectangular metacarpals and metatarsals, and osteoporosis were found in 38.6% of the patients in the age group of 10-14 years and were significantly related to the duration of DFX chelation, p=0.0001.

Conclusion: Short stature, short trunk, skeletal changes and delayed puberty are significant problems in our thalassemic patients.

The role of siderosis and DFX toxicity need further elucidation.