Short stature and truncal shortening in transfusion dependent thalassemia patients: Results from a Thalassemia Center in Malaysia

Hamidah Alias, Rahmah Rasat, Azmi Mohd. Tamil, Abdul Aziz Jemain, A. Rahman A. Jamal

Research output: Contribution to journalArticle

6 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)625-630
Number of pages6
JournalSoutheast Asian Journal of Tropical Medicine and Public Health
Volume32
Issue number3
Publication statusPublished - 2001

Fingerprint

Thalassemia
beta-Thalassemia
Malaysia
Leg
Growth
Chelation Therapy
Iron Overload
Physiologic Monitoring
Ferritins
Hemoglobins
Iron
Control Groups
Serum

ASJC Scopus subject areas

  • Medicine(all)

Cite this

@article{6cef682c65d34f90bb7d3e51b1e2f4cb,
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.",
author = "Hamidah Alias and Rahmah Rasat and {Mohd. Tamil}, Azmi and Jemain, {Abdul Aziz} and {A. Jamal}, {A. Rahman}",
year = "2001",
language = "English",
volume = "32",
pages = "625--630",
journal = "The Southeast Asian journal of tropical medicine and public health",
issn = "0125-1562",
publisher = "Southeast Asian Ministers of Education Organisation",
number = "3",

}

TY - JOUR

T1 - Short stature and truncal shortening in transfusion dependent thalassemia patients

T2 - Results from a Thalassemia Center in Malaysia

AU - Alias, Hamidah

AU - Rasat, Rahmah

AU - Mohd. Tamil, Azmi

AU - Jemain, Abdul Aziz

AU - A. Jamal, A. Rahman

PY - 2001

Y1 - 2001

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0035469472&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0035469472&partnerID=8YFLogxK

M3 - Article

C2 - 11944728

AN - SCOPUS:0035469472

VL - 32

SP - 625

EP - 630

JO - The Southeast Asian journal of tropical medicine and public health

JF - The Southeast Asian journal of tropical medicine and public health

SN - 0125-1562

IS - 3

ER -