Abstract
OBJECTIVE: To determine by measuring sitting and standing height, whether growth failure in homozygous β-thalassemia is disproportionate.
METHODS: Management of all patients with homozygous β-thalassemia in the State of Victoria (Australia) is centralised to one major teaching hospital; therefore treatment protocols are relatively standardised, and this group represents a population based cohort. Patients are transfused every 3-4 weeks to maintain hemoglobin values >10 g%, and desferrioxamine 60mg/kg/d, to a maximum of 3g/day, is given S.C. for iron chelation. Measurements were made using Holtain sitting and Harpendon standing stadiometers, in a random sample of 52 of 122 (43%) patients to date. Subischial leg length was determined by subtraction of sitting height from standing height. Standard deviation scores (sds) were used to enable comparisons irrespective of chronological age, and the patient group was analysed according to age: group 1 - <18 y.o., and group 2 - ≥18 y.o.
RESULTS: All results expressed as mean±sd. Sitting height was−3.4±1.9 sds (males <18 y.o., n=19), −4.0±1.0 sds (females<18y.o., n=13); −3.1±1.0 sds (males ≥18 y.o., n=10), −3.1±1.4 sds (females >18y.o., n=10). Sitting height was significantly different from subischial leg length in both sexes and both age groups (p<0.0001) - subischial leg length was −0.1±1.5 sds (males <18 y.o., n=19), −0.5±1.1 sds (females<18y.o., n=13); 0.9±1.3 sds (males ≥18 y.o., n=10), 0.2±1.1 sds (females ≥18y.o., n=10).
CONCLUSION: Short stature is mainly due to truncal shortening in this patient group. Hypogonadism and chelation therapy are possible etiological factors.
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Rodda, C., Reid, E. & Bowden, D. SHORT STATURE IN HOMOZYGOUS β-THALASSEMIA IS DUE TO DISPROPORTIONATE TRUNCAL SHORTENING. Pediatr Res 33 (Suppl 5), S52 (1993). https://doi.org/10.1203/00006450-199305001-00291
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DOI: https://doi.org/10.1203/00006450-199305001-00291