Tienboon P, Sanguansermsri T, Fuchs G J
Department of Pediatrics, Chiang Mai University, Thailand.
Southeast Asian J Trop Med Public Health. 1996 Jun;27(2):356-61.
Abnormal linear growth (stunting) is characteristic of children with beta thalassemia major and has been variably and inconsistently attributed to multiple different mechanisms. Despite the coexistence of beta thalassemia with deficits of several micronutrients, global undernutrition as a principle cause of growth abnormalities has not been adequately studied. We prospectively studied 115 nonsplenectomized children (6 months-6 years, 54 males, 61 girls) with beta thalassemia major who has not previously received chelation therapy. Most children had abnormal weight-for-age (WAZ) and height-for-age (HAZ) Z scores, however female children had lower WAZ (p < 0.0001) and HAZ (p < 0.02) compared to males. Mild to moderate degrees of acute wasting was also usual, and two males and one female had severe wasting. Severe weight deficits were more prevalent in the youngest (p < 0.01) and severe stunting in the older (p = 0.01) children. Nearly all children were < 50th percentile for both weight-for-age and height-for-age, and the majority were < 5th percentile. Of note, children were also disproportionately distributed below the 50th percentile for weight-for-height. Pre-transfusion hemoglobin was variably associated with anthropometric measurements. We conclude that not only is linear growth failure pervasive in our population with beta thalassemia major, but varying degrees of wasting are also typical. Further, weight deficits occur at an early age and appear to precede deficits in linear growth. Abnormal growth is not due to chelation therapy and is inconsistently associated with the degree of anemia. These patterns of growth abnormalities indicate general malnutrition as an important cause of growth failure in children with beta thalassemia.
线性生长异常（发育迟缓）是重型β地中海贫血患儿的特征，其病因多样且不一致，涉及多种不同机制。尽管重型β地中海贫血常伴有多种微量营养素缺乏，但作为生长异常主要原因的整体营养不良尚未得到充分研究。我们对115例未行脾切除术的重型β地中海贫血患儿（6个月至6岁，男54例，女61例）进行了前瞻性研究，这些患儿此前未接受过螯合治疗。大多数患儿年龄别体重（WAZ）和年龄别身高（HAZ）Z评分异常，然而，与男性患儿相比，女性患儿的WAZ（p<0.0001）和HAZ（p<0.02）更低。轻度至中度急性消瘦也较为常见，有2名男性和1名女性存在严重消瘦。严重体重不足在最小的患儿中更为普遍（p<0.01），而严重发育迟缓在年龄较大的患儿中更为常见（p = 0.01）。几乎所有患儿的年龄别体重和年龄别身高均低于第50百分位数，大多数低于第5百分位数。值得注意的是，患儿身高别体重低于第50百分位数的比例也不成比例。输血前血红蛋白与人体测量指标的相关性各异。我们得出结论，不仅在我们的重型β地中海贫血患儿群体中普遍存在线性生长失败，而且不同程度的消瘦也很典型。此外，体重不足在早期出现，似乎先于线性生长不足。生长异常并非由螯合治疗引起，且与贫血程度的相关性不一致。这些生长异常模式表明，整体营养不良是重型β地中海贫血患儿生长失败的重要原因。
