Luciano Thais Milioni, Stechinni Mônica Freire, Antonini Sonir Roberto Rauber
Universidade de São Paulo (USP) Faculdade de Medicina de Ribeirão Preto (FMRP), Departamento de Puericultura e Pediatria, Divisão de Endocrinologia Infantil, Ribeirão Preto, SP, Brazil.
Universidade de São Paulo (USP) Faculdade de Medicina de Ribeirão Preto (FMRP), Departamento de Puericultura e Pediatria, Divisão de Endocrinologia Infantil, Ribeirão Preto, SP, Brazil.
J Pediatr (Rio J). 2025 Sep 19;101(6):101429. doi: 10.1016/j.jped.2025.101429.
To evaluate the patterns of pubertal development, growth, and adult height in untreated male patients with CDGP.
A retrospective study was conducted at a tertiary care center from 1984 to 2019. Medical records of 46 boys diagnosed with CDGP (after excluding those with hypogonadism) were included for further analysis.
Most patients were born at term (78%) and appropriate for gestational age (85%). A family history of delayed puberty was noted in 50%. The median age at initial evaluation was 14.3 years (range: 4.8-16.2 years). Short stature before puberty was the main reason for seeking medical attention (48%). Short stature was common at the first evaluation (93%) but improved over time; at the final assessment, only 17% of the patients remained short. For those who reached adult height, the height Z-scores were comparable to target height. Predictions using the Bayley-Pinneau method often overestimated adult height. Delayed bone age was present in 82% of patients at initial evaluation. The median age of spontaneous pubertal onset was 15 years, with a median duration of 2.1 years (range: 1.2-4.8 years, n = 33). None of the patients received pharmacological treatment. The median age at Tanner stage G5 was 17.1 years.
In boys with CDGP, transient short stature improved spontaneously during puberty. Most achieved their target height without growth-promoting therapy. However, adult height predictions based on the Bayley-Pinneau method were often overestimated. Spontaneous puberty initiation and completion occurred at approximately 15 and 17 years of age, respectively.
评估未经治疗的体质性青春期发育延迟(CDGP)男性患者的青春期发育模式、生长情况及成年身高。
于1984年至2019年在一家三级医疗中心进行了一项回顾性研究。纳入46例诊断为CDGP的男孩(排除性腺功能减退者)的病历进行进一步分析。
大多数患者足月出生(78%)且出生体重与孕周相符(85%)。50%的患者有青春期发育延迟家族史。初次评估的中位年龄为14.3岁(范围:4.8 - 16.2岁)。青春期前身材矮小是就医的主要原因(48%)。初次评估时身材矮小很常见(93%),但随着时间推移有所改善;在最终评估时,只有17%的患者仍身材矮小。对于达到成年身高的患者,身高Z评分与目标身高相当。使用贝利 - 平诺方法进行的预测往往高估成年身高。初次评估时82%的患者骨龄延迟。自发青春期启动的中位年龄为15岁,中位持续时间为2.1年(范围:1.2 - 4.8年,n = 33)。所有患者均未接受药物治疗。坦纳G5期的中位年龄为17.1岁。
在CDGP男孩中,青春期期间短暂的身材矮小会自发改善。大多数患者在未进行促生长治疗的情况下达到了目标身高。然而,基于贝利 - 平诺方法的成年身高预测往往被高估。自发青春期启动和完成分别发生在约15岁和17岁。