Mayer-Rokitansky-Kuster-Hauser(MRKH)综合征是一种罕见的先天性疾病,影响女性生殖系统,其特征是子宫和阴道不发达或缺失。一名十七岁未婚女性入住内分泌科,Mymensingh医学院附属医院,孟加拉国于2023年11月评估原发性闭经和控制不佳的糖尿病。她是第5期通过正常阴道分娩分娩的非近亲婚姻。她的成长模式和发展里程碑是正常的。她没有溢乳的病史,慢性或周期性盆腔疼痛,甲状腺功能异常,过度运动,精神病,或药物滥用。她的家人没有这种疾病的历史。两年前,她被诊断出患有糖尿病,没有经典症状,在那个时候,她的血糖为22mmol/L。给她开了二甲双胍和格列齐特的处方。她没有低血糖病史,高血糖危机,或入院。在检查中,她的身体和营养状况都正常。贫血,黄疸,水肿,脱水,淋巴结病,痤疮,多毛症,黑棘皮病,腹部纹和白癜风缺失。她的血压是110/70,没有姿势下降,甲状腺没有肿大,人体测量结果正常,BMI为18.4kg/m2.她的制革舞台是P5和B4。生殖器检查显示女性外生殖器正常,发现了一个盲目的阴道袋。其他全身检查未发现异常。在实验室报告中,她的血糖不受控制(HbA1c-10.2%),糖尿。甲状腺功能及性腺激素正常。腹部超声显示子宫,子宫颈,阴道上部不存在,还有左肾异位.
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital disorder that affects the female reproductive system and is characterized by an underdeveloped or absent uterus and vagina. A 17-year-old unmarried female was admitted into the Department of Endocrinology, Mymensingh Medical College Hospital, Bangladesh in November 2023 for evaluation of primary amenorrhea and poorly controlled diabetes mellitus. She was the 5th issue of non-consanguineous marriage delivered at term by normal vaginal delivery. Her growth pattern and developmental milestones were normal. She had no history of galactorrhea, chronic or cyclic pelvic pain, thyroid dysfunction, excessive exercise, psychiatric illness, or drug abuse. There was no history of such type of illness in her family. She was diagnosed with diabetes mellitus two years back without classic symptoms, and at that time, her blood glucose was 22 mmol/L. She was prescribed metformin and gliclazide. She had no history of hypoglycemia, hyperglycemic crises, or hospital admission. On examination, her body build and nutritional status were normal. Anemia, jaundice, edema, dehydration, lymphadenopathy, acne, hirsutism, acanthosis nigricans, abdominal striae and vitiligo were absent. Her blood pressure was 110/70 without the postural drop, thyroid gland was not enlarged, anthropometric measurements were normal and BMI was 18.4 kg/m2. Her tanner stage was P5 & B4. Genital examination revealed normal female external genitalia, and a blind vaginal pouch was found. Other systemic examinations revealed no abnormality. On laboratory reports, her blood glucose was uncontrolled (HbA1c-10.2%) with glycosuria. Thyroid function test and gonadal hormones were normal. Ultrasonogram of the abdomen revealed uterus, cervix, and upper part of the vagina are absent, and an ectopic left kidney.