Chowdhury Tanvir K, Chowdhury Md Zonaid, Mili Fahmida, Hutson John M, Banu Tahmina
Department of Pediatric Surgery, Chittagong Medical College and Hospital, Chittagong, Bangladesh.
Pediatr Surg Int. 2014 May;30(5):527-32. doi: 10.1007/s00383-014-3486-x. Epub 2014 Feb 25.
In developing countries like Bangladesh, delayed presentation for disorders of sex development (DSD) is common, and provides some special problems for management. There remains significant controversy about appropriate sex assignment in this group. We aimed, therefore, to assess gender identity (GI) in 50 consecutive patients with DSD presenting to a referral centre in Chittagong, Bangladesh, and correlate it with Prader score, to see if the latter could be used to predict GI.
A cross-sectional, case-control study of 50 consecutive children with DSD and 50 children with vascular anomalies was conducted in the Pediatric Surgical Clinic, Chittagong Medical College and Hospital. After informed consent, patients and controls provided oral answers to a GI questionnaire and had a detailed history and physical examination. Sex-typed activities were assessed by observations of a structured toy play and the child's selection of a toy to keep. Both patients and parents then completed the Child Game Participation Questionnaire.
There were no differences in age (2-16 years, mean 8.74) between controls and DSD patients (11 46, XX DSD, 32 46, XY DSD, 4 MGD, 3 ovo-testicular DSD). Fifteen of the DSD patients (30 %) came from consanguineous marriages and only 2 of the control patients had consanguinity of their parents. For the 13-question GI interview, there was no overall difference between DSD cases and controls. For the 46, XX DSD subgroup, there was a significantly higher score (11.1 ± 7.1) compared with control girls (4.5 ± 4.7) (p < 0.05), while for DSD and control boys, there was a positive correlation with age and GI (p < 0.01). Prader score correlated with GI score in both control (r = 0.91) and DSD patients (r = 0.75) (p < 0.01), DSD girls played significantly less with girls' toys than controls (p < 0.01), but there was no differences for the boys. Composite scores on GI and gender-related behaviour correlated with Prader score for DSD patients (r = 0.61) (p < 0.01).
This study supports the view that GI and gender-role behaviour should be assessed routinely in DSD patients presenting after the neonatal period, so that sex assignment is in accordance with behaviour. Prader scores showed a good correlation with GI and gender role behaviour.
在孟加拉国这样的发展中国家，性发育障碍（DSD）患者延迟就诊的情况很常见，这给治疗带来了一些特殊问题。对于这一群体的合适性别指定仍存在重大争议。因此，我们旨在评估连续50例就诊于孟加拉国吉大港一家转诊中心的DSD患者的性别认同（GI），并将其与普拉德评分相关联，以查看后者是否可用于预测GI。
在吉大港医学院和医院的儿科外科诊所对50例连续的DSD患儿和50例血管异常患儿进行了一项横断面病例对照研究。在获得知情同意后，患者和对照组对GI问卷进行了口头回答，并进行了详细的病史询问和体格检查。通过观察结构化玩具玩耍和孩子选择保留的玩具来评估性别类型活动。然后患者和家长都完成了儿童游戏参与问卷。
对照组和DSD患者（11例46,XX DSD，32例46,XY DSD，4例MGD，3例卵睾性DSD）在年龄（2至16岁，平均8.74岁）上没有差异。15例DSD患者（30%）来自近亲婚姻，而对照组患者中只有2例父母有血缘关系。对于13个问题的GI访谈，DSD病例和对照组之间没有总体差异。对于46,XX DSD亚组，其得分（11.1±7.1）显著高于对照组女孩（4.5±4.7）（p<0.05），而对于DSD和对照组男孩，年龄与GI呈正相关（p<0.01）。在对照组（r = 0.91）和DSD患者（r = 0.75）中，普拉德评分与GI评分均相关（p<0.01），DSD女孩玩女孩玩具的次数明显少于对照组（p<0.01），但男孩之间没有差异。DSD患者的GI和性别相关行为综合评分与普拉德评分相关（r = 0.61）（p<0.01）。
本研究支持这样的观点，即对于新生儿期后就诊的DSD患者，应常规评估GI和性别角色行为，以便性别指定与行为相符。普拉德评分与GI和性别角色行为显示出良好的相关性。
