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我院近期收治同胞兄弟4人中,3人为男性假两性畸形Ⅱ型,报告如下:[例1]男,11岁,于1986年12月15日以尿道口位置异常入院。患儿双亲非近亲通婚,双亲体检未发现异常.患儿体检:BP94/60mmHg,体重27kg,身高113 cm。智力发育正常。乳房未发育,唇须、腋毛及阴毛缺如。两侧皮下环处可扪及睾丸,阴茎小,阴囊分叶状、中空,尿道口位于分叶阴囊间呈漏斗状(假阴道)。前列腺小。性染色质(X 小体)阴性,染色体核型46,XY。诊断为不完全性男性假两性畸形第Ⅱ型——假阴道型尿道下裂综合征。剖腹探查未见子宫及卵巢,双睾各为2×1×1 cm,附睾、输精管存在,双睾取活检后,将其固定于阴囊肉膜外,行尿
In our hospital recently treated siblings 4, 3 were male bisexual deformity type Ⅱ, the report is as follows: [Example 1] Male, 11 years old, on December 15, 1986 to the urethra abnormal position. Parents of non-relatives of children with intermarriage, parents found no abnormalities in physical examination. Children’s physical examination: BP94 / 60mmHg, weight 27kg, height 113cm. Normal mental development. Breast undeveloped, lips, armpit hair and pubic hair missing. On both sides of the subcutaneous ring palpable testis, penis, scrotum lobulated, hollow, urethral folds between the scrotal funnel-shaped (false vagina). Small prostate. Sexual chromatin (X corpuscles) negative, chromosome karyotype 46, XY. Diagnosed as incomplete male pseudohermaphroditism Type II - Fake vaginal hypospadias syndrome. Splenectomy no uterus and ovaries were found, each of the 2 × 1 × 1 cm of each testis, epididymis, vasectomy, biopsy biopsy, it is fixed in the scrotum flesh outside the line of urine