苗勒管抑制物质在尿道下裂发病中的作用及尿道下裂手术远期效果评价
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摘要
目的:探讨苗勒管抑制物质在尿道下裂发病中的作用。
     方法:同济医院收治的54例单纯型尿道下裂,其中=12月11例,13~24月24例,25~36月19例。同期收治的36例无泌尿生殖系统疾病儿童作为对照,=12月10例,13~24月13例,25~36月13例。检测两组儿童血清苗勒管抑制物质、睾酮及雄烯二酮的水平,对比血清苗勒管抑制物质和睾酮及雄烯二酮的关系。
     结果:尿道下裂组和对照组儿童的血清MIS平均值分别为122.0±21.7和80.5±11.4ng/ml,P<0.01。对照组儿童血清睾酮为1.55±0.42pg/ml,尿道下裂组儿童为0.64±0.31pg/ml,P<0.05。对照组儿童血清雄烯二酮的水平明显高于病例组儿童,分别为1.41±0.28和0.66±0.19ng/ml,P<0.01。根据年龄分三组,=12月(Ⅰ组),13~24月(Ⅱ组),25~36月(Ⅲ组)。Ⅰ组,病儿和正常儿MIS的浓度分别为129.6±11.8和76.7±16.6ng/ml,P<0.01;Ⅱ组分别为100.2±12.3和67.2±7.3ng/ml,P<0.05;Ⅲ组,病儿和正常儿血清MIS的浓度分别为114.8±16.6和90.5±23.6ng/ml,P<0.05。Ⅰ组病儿和正常儿血清睾酮分别为0.91±0.32和2.06±0.10pg/ml,P<0.05:Ⅱ组内尿道下裂病儿和正常儿分别有3例检测不到睾酮,其他儿童的血清睾酮水平分别为0.38±0.13和1.86±0.51pg/ml,P<0.01;Ⅲ组病儿和正常儿分别有6例和4例睾酮检测不到,其他儿童的睾酮水平分别为0.21±0.13pg/ml和0.30±0.19pg/ml,P>0.05。血清雄烯二酮的水平Ⅰ组病例组和对照组分别为0.39±0.21和0.94±0.36ng/ml,P<0.01;Ⅱ组分别为和0.32±0.20ng/ml,0.79±±0.15,P<0.05;Ⅲ组分别为0.25±0.20 ng/ml和0.31±0.18ng/ml,P>0.05。尿道下裂儿童较高的MIS水平和较低的睾酮水平呈较弱的负相关,Ⅰ、Ⅱ组的相关系数分别为-0.31和-0.42。
     结论:尿道下裂儿童血清苗勒管抑制物质的水平与睾酮水平呈负相关,提示苗勒管抑制物质可能在尿道下裂的发病中起作用。
     目的:评价尿道下裂手术的远期效果,探讨影响尿道下裂手术远期效果的因素。
     方法:设计调查问卷,分别寄给1982~1988年收治的尿道下裂手术后病人,对随访结果进行统计学分析。
     结果:142例中,102例将调查表填完整后寄回或直接来门诊接受调查。收回调查表时患者年龄19.2~33.9岁,平均24.5岁。102例中,近端型50例(Ⅰ组),远端型52例(Ⅱ组),Ⅰ、Ⅱ组在文化程度、职业分布及经济状况无差异,性成熟年龄相近(P>0.05)。在对手术总体效果评价上,54例对手术效果满意,48例不满。满意者中,远端型39例,近端型15例;采用一期手术者46例,二期手术者8例;有严重并发症者12例,无并发症者42例。在阴茎外观评价上,29.4%的病例对阴茎外观不满意;其中,近端型病人与远端型病人分别为40%和19.2%。在排尿方面,59.8%有排尿问题;其中,一期手术者中有36.1%,二期手术者有95.1%认为存在排尿方面问题。精神心理方面,49例(48.0%)自诉阴茎曾被人嘲笑:其中,近端型病人占61.2%,远端型占38.8%。60%的近端型病人、36.5%的远端型病人以及78.9%的有并发症的和29.7%无并发症的病人有这种主诉。手术完成时间越晚(r=0.27,t=2.63,P<0.05)、手术次数越多(r=0.23,t=2.25,P<0.05)、阴茎外形越差、病人文化程度越低(r=0.33,t=3.13,P<0.05),病人心理问题出现越多、程度越重。回访的102例中有76例已婚或过同居生活,23.3%有勃起方面的问题,33.0%有射精方面的问题。
     结论:大部分的尿道下裂病人成年后可以获得与正常人一样的精神状态与生理功能。同时也有部分病儿对手术效果不满意,如阴茎外观、排尿异常、心理障碍等。提前手术年龄、选择合适的手术方式、提高第一次手术的成功率、降低手术的并发症及改进术后阴茎外观对提高尿道下裂的远期效果是非常重要的。
Objective To explore the role of mullerian inhibiting substance playing in the
    occurrence of male hypospadias.
    Methods Fifty-four simple hypospadias patients and 36 patients which suffered
    from other diseases were chosen as our research objects. Of the hypospadias patients,
    there were 11patients whose ages were less than 12months, 24 patients were from 13
    to 24months, and 19 patients were from 25 to 36months. Serum mullerian inhibiting
    substance, testosterone, and androstenedion were assayed, respectively. To compare
    the relationgships in testosterone and mullerian inhibiting substance between the the
    two groups.
    Results The concetration of mullerian inhibiting substance in hypospadias group
    was significantly higher than that in control group, P<0.05. However, the concetration
    of testosterone, or androstenedione in group hypospadias was significantly lower than that in control group, P<0.05. Furthermore the two- group patients were divided into 3 subgroups according their ages, respectively. The concetration of testosterone, or
    androstenedione in group hypospadias whose ages were less than 12 months, and patients from 13 to 24 months was significantly lower than that in control group whose ages were less than 12 months, and patients from 13 to 24 months, P<0.05. And the higher concetration of mullerian inhibiting substance was negative relationship with the lower concetration of testosterone in hypospadias patients, and
    the relative coefficient was negative 0.31 and 0.42, respectively.
    Conclusions Mullerian inhibiting substance maybe could inhibit the synthesization
    of testosterone by inhibiting cytochrome P450cl7a hydroxylase/c_(17-20)lase gene
    expreesion. Mullerian inhibiting substance plays a part in the occurrence of
    hypospadias.
    Objective To follow-up the long-term outcome of hypospadias repairs, and explore
    factors which could affect the long-term results of hypospadias repairs
    Methods Questionnaires were designed and mailed respectively to hypospadias
    patients, who underwent surgical treatments during 1982 to 1988 in Tongji hospital
    and The First Pepeole's Hospital of Jinning. The data were analysed using statistical
    methods.
    Results Of 142 hypospadias patients, 102 returned the questionnaires. There were
    no significant differences between the distal group and proximal group in education,
    occupation and economic status. The median age of the first spermatorrhea of diatal
    patient was similar to that of the proximal patient. But the diatal hypospadias patients
    often held more negative attitude than the proximal patients in penile appraisals,
    micturition, sexual psychology, penile erction and ejaculation.
    Conclusions The short-term results of hypospadias repair could affect the long-term
    results significantly, and good short-term results also predict long-term ones. The
    types of hypospadias, procedures, and complications have significant influences on
    predicting the long-term results of hypospadias repairs. In order to achieve good
    long-term results of hypospadias repairs, it is very important to operate earlier,
    enhance the successful rate of the first hypospadias repair, reduce early complications,
    and to improve the penile appearance.
引文
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