TY - JOUR ID - 39592 TI - Pulmonary Aplasia with Unusual Associations in a Woman JO - Iranian Journal of Medical Sciences JA - IJMS LA - en SN - 0253-0716 AU - Bachh, Arshad Altaf AU - Pulluri, Sridhar AU - Beigh, Aadil AU - Raju, Chippa AU - Deshpande, Ranganath AD - Department of Pulmonary Medicine, Mamata Medical College and Hospital, Khammam, Andra Pradesh, India Y1 - 2014 PY - 2014 VL - 39 IS - 2 SP - 148 EP - 151 KW - Lung agenesis KW - Renal agenesis KW - Müllerian anomalies DO - N2 - Failure of development of the primitive lung bud leads to an extremely rare congenital anomaly with a prevalence of 34 per 10 lac live births termed pulmonary aplasia. In half of such cases, associated congenital malformations of the cardiovascular, skeletal, gastrointestinal, or genitourinary systems are present. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is defined as the congenital aplasia of the uterus and the upper two thirds of the vagina with normal secondary sexual characteristics, ovaries, and a normal karyotype (46, XX). We report an extremely rare association of right lung aplasia, MRKH syndrome, and right renal agenesis with left pelvic kidney, which to the best of our knowledge is the first such association reported. UR - https://ijms.sums.ac.ir/article_39592.html L1 - https://ijms.sums.ac.ir/article_39592_a35442a03c7e818bca7b5ab2cae87434.pdf ER -