Proos L A, Dahl M, Ahlsten G, Tuvemo T, Gustafsson J
Department of Paediatrics, Uppsala University Children's Hospital, Sweden.
Arch Dis Child. 1996 Jul;75(1):42-5. doi: 10.1136/adc.75.1.42.
An increased risk of developing precocious puberty has been reported in children with myelomeningocele. In order to evaluate this further and to study factors associated with early or precocious puberty the medical records of all girls with myelomeningocele, born from 1970 onwards (n = 64), who were admitted to our unit were reviewed. Early/precocious puberty (E/PP) was defined as breast development or pubic hair corresponding to Tanner stage 2 before the age of 9.2 years. In 32 out of 62 cases data were sufficient for evaluation of the timing of puberty. Twenty girls had E/PP and 12 girls normal timing of puberty. In the girls who had reached the age of 9.2 years the incidence of E/PP was at least 52%. Girls with E/PP had a higher incidence of hydrocephalus, were treated with intraventricular shunts more often, and had significantly higher frequency of increased intracranial pressure during the perinatal period (p < 0.05, p < 0.01, and p < 0.001, respectively). The group of girls developing E/PP was also more severely disabled with respect to motor and urological function and had more shunt revisions. In conclusion, E/PP in girls with myelomeningocele is strongly associated with increased intracranial pressure particularly during the perinatal period.
据报道，患有脊髓脊膜膨出的儿童患性早熟的风险增加。为了进一步评估这一情况并研究与青春期提前或性早熟相关的因素，我们回顾了1970年以后出生（n = 64）并入住我们科室的所有脊髓脊膜膨出女孩的病历。青春期提前/性早熟（E/PP）被定义为在9.2岁之前出现符合坦纳2期的乳房发育或阴毛。在62例病例中，有32例的数据足以评估青春期的时间。20名女孩患有E/PP，12名女孩青春期时间正常。在达到9.2岁的女孩中，E/PP的发生率至少为52%。患有E/PP的女孩脑积水发生率更高，更常接受脑室内分流治疗，并且围产期颅内压升高的频率显著更高（分别为p < 0.05、p < 0.01和p < 0.001）。出现E/PP的女孩组在运动和泌尿系统功能方面残疾也更严重，并且分流修正次数更多。总之，脊髓脊膜膨出女孩的E/PP与颅内压升高密切相关，尤其是在围产期。
