UNASSIGNED: We identified 26 children aged <3 years who were on dialysis and treated with cinacalcet between 2009 and 2021 in 8 European pediatric centers.
UNASSIGNED: Median (interquartile range) age at the start of cinacalcet was 18 (interquartile range: 11-27) months, serum parathyroid hormone (PTH) was 792 (411-1397) pg/ml, corresponding to 11.6 (5.9-19.8) times the upper limit of normal (ULN). Serum calcium was 2.56 (2.43-2.75) mmol/l, and serum phosphate 1.47 (1.16-1.71) mmol/l. Serum 25-OH vitamin D (25-OHD) was 70 (60-89) nmol/l, 3 children were vitamin D deficient (<50 nmol/l). The initial cinacalcet dose was 0.4 (0.2-0.8) mg/kg/d and the maximum dose was 1.1 (0.6-1.2) mg/kg/d. The median follow-up under cinacalcet was 1.2 (0.7-2.0) years. PTH decreased to 4.3 (2.2-7.8) times the ULN after 6 months, to 2.0 (1.0-5.3) times ULN after 12 months, and to 1.6 (0.5-3.4) times thereafter (P = 0.017/0.003/<0.0001, log-transformed PTH). Seven of the 26 infants developed 10 hypocalcemic episodes <2.10 mmol/l. Oral calcium intake was 84% (66%-117%) of recommended nutrient intake at start, 100% (64%-142%) at 3 months and declined to 78% (65%-102%) at 12 months of therapy. Three children developed clinical signs of precocious puberty.
UNASSIGNED: Cinacalcet efficiently controlled severe sHPT in children aged <3 years and was associated with hypocalcemic episodes (similar to what is observed in older children) and precious puberty, thereby mandating meticulous control of calcium (considering nutrition, supplementation, and dialysate) and endocrine changes.
■我们在8个欧洲儿科中心确定了2009年至2021年期间接受透析并接受西那卡塞治疗的26名3岁以下儿童。
■西那卡塞开始时的中位数(四分位距)年龄为18(四分位距:11-27)个月,血清甲状旁腺激素(PTH)为792(411-1397)pg/ml,对应于正常上限(ULN)的11.6(5.9-19.8)倍。血清钙为2.56(2.43-2.75)mmol/l,和血清磷酸盐1.47(1.16-1.71)mmol/l。血清25-OH维生素D(25-OHD)为70(60-89)nmol/l,3名儿童维生素D缺乏(<50nmol/l)。西那卡塞初始剂量为0.4(0.2-0.8)mg/kg/d,最大剂量为1.1(0.6-1.2)mg/kg/d。西那卡塞治疗的中位随访时间为1.2年(0.7-2.0年)。PTH在6个月后下降到ULN的4.3(2.2-7.8)倍,至12个月后ULN的2.0(1.0-5.3)倍,至1.6(0.5-3.4)次(P=0.017/0.003/<0.0001,对数转化PTH)。26名婴儿中有7名出现10次低钙血症发作<2.10mmol/l。开始时口服钙摄入量为推荐营养素摄入量的84%(66%-117%),3个月时为100%(64%-142%),12个月时下降到78%(65%-102%)。三个孩子出现了性早熟的临床症状。
■Cinacalcet有效控制了3岁以下儿童的严重sHPT,并与低钙血症发作(类似于年龄较大的儿童)和宝贵的青春期有关,从而要求精心控制钙(考虑到营养,补充,和透析液)和内分泌变化。