Haffner D, Wühl E, Blum W F, Schaefer F, Mehls O
Sektion für Pädiatrische Nephrologie, Universitäts-Kinderklinik, Heidelberg, Germany.
Eur J Pediatr. 1995 Aug;154(8):610-3. doi: 10.1007/BF02079060.
Three short prepubertal children with X-linked hypophosphataemia were treated with 1 IU recombinant human growth hormone (rhGH)/kg per week sc in addition to calcitriol and phosphate supplementation over a period of 3 years. Improvement of height standard deviation score (SDS) ranged from 1.0-1.7 SD based on an increase in sitting height of 1.5-2.9 SD, whereas subischial leg length improved only slightly by 0.3-0.9 SD. In all three patients, renal phosphate threshold concentration increased slightly and transient hyperparathyroidism was noted.
Treatment of stunted children with X-linked hypophosphataemia is effective in improving growth velocity, but appears to aggravate the pre-existent disproportionate stature of such children.
三名青春期前患有X连锁低磷血症的矮小儿童,除了补充骨化三醇和磷酸盐外,还接受了为期3年的每周每千克体重1国际单位重组人生长激素(rhGH)皮下注射治疗。身高标准差评分(SDS)的改善范围为1.0 - 1.7标准差,这是基于坐高增加了1.5 - 2.9标准差,而坐骨下腿长仅轻微改善了0.3 - 0.9标准差。在所有三名患者中,肾磷酸盐阈值浓度略有增加,并观察到短暂性甲状旁腺功能亢进。
用X连锁低磷血症治疗发育迟缓儿童可有效提高生长速度,但似乎会加重此类儿童已有的身材比例失调。