Physical growth in children with transfusion-dependent thalassemia

Abstract

Objective: To describe physical growth and related factors in transfusion-dependent thalassemia patients. Methods: This is a cross-sectional analysis of the records of the patients registered at and being followed up by the Thalassemia Day Care Center (TDCC) at Kalawati Saran Children's Hospital, New Delhi, India. Clinical and laboratory parameters were recorded on a spreadsheet for analysis. Clinical parameters included weight, height, sexual maturity ratings, and general and systemic physical examination. Laboratory parameters included pretransfusion hemoglobin (Hb), periodic serum ferritin, and tests for viral markers of human immunodeficiency virus (HIV) and hepatitis B and C. Z-scores for weight, height, and body mass index (BMI) were calculated using World Health Organization reference data. Statistical analysis was carried out using Microsoft Excel ® and Stata ® software. Results: Out of 214 patients registered at the TDCC since 2001, 154 were included in this study. The mean age of patients was 9.19 years (range 0.5-20 years). Pretransfusion Hb was well maintained (mean 9.21 g/dL; 95% confidence interval [CI]: 9.06-9.36), but the mean serum ferritin levels were approximately three times (3112 ng/mL) the desired value despite the patients being on deferiprone (72%) or deferasirox (25%). One-third (33.11%) of the patients had short stature, 13% were thin, and 10.82% were very thin (BMI z-score ,-3). No patient was overweight or obese. Linear regression coefficient showed that for every 1-year increase in age, the mean ferritin value increased by 186.21 pg/mL (95% CI: 143.31-228.27). Height z-scores had significant correlation with mean ferritin levels, whereas correlation with mean pretransfusion Hb was not significant statistically. Mean ferritin levels were significantly higher in patients with short stature than in the patients with normal height. Regression analysis showed that an increase of 3571 units of serum ferritin was associated with a decrease of one point in height z-scores. One-fifth (19.40%) of adolescent patients had delayed puberty. Conclusion: Approximately one-third (33.11%) of patients with transfusion-dependent thalassemia major were of short stature. In this group of patients with pretransfusion Hb levels maintained at desired levels, physical growth was correlated with status of iron overload.

Impact of Ferritin Load on Gonadal Reserve among Regular Transfused β-Thalassemia

“…[3]. Harish et al, (2011); also was reported that minimal siderosis, with reduced number of primordial follicles, and thickened ovarian capsules was noticed in these patients [28]. The reason for why the correlation between serum ferritin and sex hormones pattern or gonadal reserve was non-significant in this study and other studies, we can postulate that there are other etiological factors responsible for hypogonadism in these patients, the most important one is chronic persistent anemia; this can confirmed by the fact that; the single patient who had high LH post stimulation test, his Hb level was highest one.…”

Subclinical renal abnormalities in young thalassemia major and intermedia patients and its relation to chelation therapy

“…However, other studies [38] reported significant short stature for age among their poorly chelated group (serum ferritin P2500 ng/ ml) when compared to the well chelated group (serum ferritin <2500 ng/ml) (P < 0.001). Pemde et al [39], classified their thalassemic patients into two groups, one with serum ferritin <2000 ng/ml and the other with serum ferritin >2000 ng/ ml, and they reported that the height of the patients with serum ferritin >2000 ng/ml was significantly lower than those with serum ferritin <2000 ng/ml. However, in an earlier study done in India by Gomber and Dewan [40], no relation between physical growth and serum ferritin level was found.…”

Anthropometric measurements in children having transfusion-dependent beta thalassemia

“…Physical growth is relatively normal up until 9-10 years of age but the majority of thalassemia patients fail to achieve the same final height as their normal peers [4]. Growth retardation in thalassemia is a well-observed phenomenon with a frequency between 25-66% globally [3,[5][6][7][8] and 20-57% nationally [9][10][11]. Growth retardation is multifactorial and the etiology varies with age.…”