GROWTH (G) AND SEXUAL MATURATION (SM) PATTERNS IN THALASSEMIA MAJOR CHILDREN (Th.)

Abstract

G and SM are frequently delayed in Th., hut data are disomogeneous because of different transfusional ard iron-chelation protocols. Height(H), weight(W), Tanner stages (T), penis lenght, testis volume(TV), serum ferritin (SF) and bone age(BA) were evaluated in 83 Th., aged 3-22y, transfused to mantain pretransfusional Hb levels >9 and >11 g/dl before and after 1980, respectively, treated with DFO, im until 1978, sc later on. Th. were divided by sex and age: A(3-8y), B(8-12y), C(>12 y). MALES(43): H was<3 centile(c) in 60%A, 50%B, 72%C;>25c in 0%A, 29%B, 6%C. H delay(HD) and age (CA) (r=0.69), BA and CA(r=0.95> and H age(HA) (r=0.93) were correlated*. W was<3c in 10%A, 22%B, 55%C;>25c in 40%A, 44% B, 11%C. HA and WA(r=0.69), HD and WD(r=0.92) were correlated*. In C, 63% had a penis lenght<5 cm, 51% a TV<5 ml and 45% T I pubic hair (PH). FEMALES(40): H was < 3c in 91A, 9%B, 67%C;>25c in 54%A, 45%B, 16%C. HD and CA(r=0.85), BA and CA(r= 0.93) and HA(r=0.96) were correlated*, w was <3c in 0%A, 18%B, 44%C; >25c in 70%A, 64%B, 39%C. HA and WA(r=0.94), HD and WD(r=0.90) were correlated*. In C, 58% had no menarche, 11% T I breast and 21% T I PH. In both sexes auxological parameters were not correlated with iron stores (=SF). Our data show a precocious and progressive impairment of growth and bone maturation, especially in males, worst after 12 years in both sexes because of the lack of pubertal growth spurt.*=p40.001.

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