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KMID : 1101620230340010028
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Perinatology
2023 Volume.34 No. 1 p.28 ~ p.35
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Prenatal Diagnosis of a Fatal Case of Fetal Autosomal Dominant Polycystic Kidney Disease
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Suh Kah-Won
Jeong Ji-Eun
Hong Seong-Yeon
Bae Jin-Young
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Abstract
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The polycystic kidney disease (PKD) is a genetic disease that can be classified into 2 groups: autosomal dominant and autosomal recessive. Autosomal dominant PKD has a mild to moderate clinical course with an average onset after adulthood. Autosomal recessive PKD can have life-threatening manifestations during the neonatal period, even before birth. Daegu Catholic Universtiy Medical Center had a prenatally diagnosed case of a known autosomal dominant PKD and the fetus suffering fetal hydrops due to severely enlarged polycystic kidneys. A cesarean section was performed at 34 weeks of gestation due to severe fetal hydrops. To relieve abdominal compartment syndrome, the neonate underwent unilateral nephrectomy just after birth but expired 4 days later due to respiratory distress. Hence, there can be a rare but possible case of fatal neonatal outcome in a mother with autosomal dominant PKD. Therefore, we recommend close observation and multifaceted counseling for the patient with family history of autosomal dominant PKD.
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KEYWORD
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Polycystic kidney diseases, Postnatal care, Prenatal diagnosis, Nephrectomy
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