title: Short stature and truncal shortening in transfusion dependent thalassemia patients: results from a thalassemia center in Malaysia.
abstract: One of the major complications in patients with transfusion dependent thalassemia is growth impairment secondary to iron overload. We studied the growth status in 66 patients with beta-thalassemia major and HbE-beta thalassemia who were transfusion dependent, aged from 2 to 24 years, and 66 controls matched for sex and age. The prevalence of short stature in transfusion-dependent thalassemics was 54.5% compared to 4.5% in control group (p<0.001). Short stature was more prevalent in those above the age of 10 years in this study group (83.3% vs 16.7%). Transfusion dependent thalassemics with short stature were found to have significantly lower mean standing height standard deviation scores (SDS), sitting height SDS and subischial leg length SDS values (p<0.001). There was also a significant difference between the mean sitting height SDS and the mean subischial leg length SDS in our thalassemics with short stature, suggesting that the short stature was due to disproportionate truncal shortening. Serum ferritin levels were significantly higher in transfusion dependent thalassemics who were short compared to those who were of normal height (p = 0.002). However, the mean pre-transfusion hemoglobin levels did not differ significantly between patients with short stature and those with normal height (p = 0.216). The prevalence of short stature also did not differ significantly between those with beta-thalassemia major and those with HbE-beta thalassemia (p = 0.32). This study highlighted the importance of providing optimal treatment in these patients, including monitoring of growth parameters and optimizing iron chelation therapy.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.
authors.forename: A
authors.initials: A
authors.lastname: Hamidah
authors.forename: R
authors.initials: R
authors.lastname: Rahmah
authors.forename: T
authors.initials: T
authors.lastname: Azmi
authors.forename: J
authors.initials: J
authors.lastname: Aziz
authors.forename: R
authors.initials: R
authors.lastname: Jamal
mesh_terms: D000293:Adolescent; D001803:Blood Transfusion; D001827:Body Height; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008296:Malaysia / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D013789:Thalassemia / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy