Pediatric Endocrinology Unit, Department of Pediatrics, Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR), Curitiba, PR, Brazil.
Department of Pediatrics, Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR), Curitiba, PR, Brazil.
J Pediatr Endocrinol Metab. 2020 Nov 26;33(11):1449-1455. doi: 10.1515/jpem-2020-0285.
Objectives Adequate treatment of congenital hypothyroidism (CH) is required for normal growth and sexual development. To evaluate pubertal development in patients with permanent CH detected by a statewide Neonatal Screening Program of Paraná and, secondly, to evaluate adult height (AH) in a subgroup of patients. Methods Clinical, laboratory, and auxological data obtained from medical records of 174 patients (123 girls). Results Median chronological age (CA) at treatment initiation was 24 days, and mean initial levothyroxine dose was 11.7 ± 1.9 μg/kg/day; mean CA at puberty onset was 11.5 ± 1.3 years (boys) and 9.7 ± 1.2 years (girls); mean CA in girls who underwent menarche (n=81) was 12.1 ± 1.1 years. Thyroid-stimulating hormone (TSH) values above the normal range were observed in 36.4% of the boys and 32.7% of the girls on puberty onset, and in 44.6% around menarche. Among 15 boys and 66 girls who had reached the AH, the median height z-score value was significantly greater than the target height (TH) z-score value in boys (p=0.01) and in girls (p<0.001). Boys with normal TSH values at puberty onset had greater mean AH z-score compared with boys with TSH values above the normal range (p=0.04). Conclusions In this group, pubertal development in girls with CH was not different from that reported in healthy girls in the general Brazilian population. Boys with higher TSH at puberty onset may have an increased risk of not reaching their potential height compared with those with normal TSH during this period. In a subgroup who attained AH, the median AH z-score was greater than the median TH z-score.
为了正常生长和性发育,先天性甲状腺功能减退症(CH)需要得到充分治疗。本研究评估了巴西南里奥格兰德州新生儿筛查计划检测出的永久性 CH 患者的青春期发育情况,并评估了部分患者的成人身高(AH)。
从 174 名患者(123 名女孩)的病历中获取临床、实验室和生长数据。
治疗开始时的中位年龄(CA)为 24 天,初始左甲状腺素剂量的平均值为 11.7±1.9μg/kg/天;青春期开始时的平均 CA 为男孩 11.5±1.3 岁,女孩 9.7±1.2 岁;81 名女孩开始月经初潮时的 CA 为 12.1±1.1 岁。青春期开始时,36.4%的男孩和 32.7%的女孩 TSH 值高于正常范围,而在接近月经初潮时,44.6%的患者 TSH 值高于正常范围。在达到 AH 的 15 名男孩和 66 名女孩中,男孩的身高 z 评分中位数显著大于目标身高(TH)z 评分(p=0.01),女孩也如此(p<0.001)。青春期开始时 TSH 值正常的男孩的平均 AH z 评分大于 TSH 值高于正常范围的男孩(p=0.04)。
在本研究中,CH 女孩的青春期发育与巴西普通人群中健康女孩的青春期发育无差异。与青春期 TSH 值正常的男孩相比,青春期 TSH 值较高的男孩可能无法达到潜在身高的风险增加。在达到 AH 的亚组中,AH 的中位数 z 评分大于 TH 的中位数 z 评分。