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Year : 2021  |  Volume : 10  |  Issue : 3  |  Page : 355-357

Unilateral pulmonary agenesis with mayer-rokitansky-kuster-hauser syndrome: A rare association

1 Department of Respiratory Medicine, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Psychiatry, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Correspondence Address:
Dr. Manoj Pandey
Assistant Professor, Department of Respiratory Medcine, GSVM Medical College, Kanpur, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijrc.ijrc_59_21

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Unilateral pulmonary agenesis is a rare congenital condition characterized by the absence of one or both lungs. Most of the cases have unilateral presentation as bilateral agenesis does not support life. Simultaneous Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome also has very rare presentation. It is characterized by congenital aplasia of the uterus and the vagina with normal female karyotype (46XX). A 30-year-old female, presenting with dyspnea from last 6 months and amenorrhea for last 15 years was further evaluated with gynecological examination, contrast-enhanced computed tomography thorax with the abdomen and karyotyping. She was eventually diagnosed as a case of Type I pulmonary agenesis with Type I MRKH syndrome. K

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