Successful Pregnancy in a Patient with Autosomal Dominant Polycystic Kidney Disease on Long-Term Hemodialysis
A 38-yr-old primigravida female on HD presented with amenorrhea in May 2011. She had been diagnosed with ADPKD 6 yr ago. She started HD 3 times a week after 3 yr of her initial diagnosis. At the time of presentation, she was normotensive and had been prescribed aspirin (100 mg daily), multivitamins, calcium-based phosphate binders, and erythropoietin (1,000 units every HD session). Her dry weight was 53.3 kg and interdialytic weight gain was 1.5-2.5 kg. Her mother had ADPKD and was also on HD.\nShe was confirmed to be 8 weeks pregnant by pelvic ultrasound and beta-hCG test. The physical examination revealed a blood pressure (BP) of 137/85 mm Hg and a regular heart rate of 107 beats per minute. Laboratory findings at presentation were as follows: hemoglobin (Hb) of 10.4 g/dL; hematocrit (Hct) 31.5%; blood urea nitrogen (BUN) 56.9 mg/dL; serum creatinine (Cr) 9.0 mg/dL; total protein 5.9 g/dL; serum albumin 3.7 g/dL; serum calcium 9.3 mg/dL; serum phosphorus 3.3 mg/dL; potassium 5.5 mM/L; and normal liver function. Her 24-hr urine volume was about 1 liter. The computed tomography scan taken 2 yr ago showed bilaterally enlarged kidneys filled with numerous renal cysts along with only a few liver cysts (). Total kidney volume was measured to be 5,970 mL.\nShe was managed by a multidisciplinary team approach to optimize the patient and fetal outcomes. Firstly, the risk of pregnancy in a dialysis patient and the need for intensive dialysis were discussed with her family. Secondly, our team evaluated the risk of stunted intrauterine fetal growth by her massively enlarged kidneys. We considered a unilateral nephrectomy at the second trimester to secure the intraabdominal space, but the risk was determined to outweigh the benefits in this case. Finally, genetic counseling about the fetus's risk of inheriting ADPKD and prenatal genetic diagnosis was provided but declined by the patient.\nAt 10 weeks gestation, HD prescription was changed to 4-hr treatments 4-5 times a week, and a predialysis BUN less than 50 mg/dL was targeted. Throughout pregnancy, predialysis BUN was maintained at 25.1-51.3 mg/dL. Standard unfractionated heparin was used for anticoagulation. Her BP was well controlled without any antihypertensive medication: systolic BP remained at 110-140 mmHg and diastolic was consistently at 60-80 mmHg. Anemia was managed with recombinant human erythropoietin and intravenous iron without transfusion. Erythropoietin doses were adjusted to target a maternal Hb between 10-11 g/dL. The median dose was 18,000 IU/week (range, 5,000-26,000 IU/week) during gestational weeks 12-36. In addition, 100 mg intravenous iron sucrose was administered every session during gestational weeks 19-22. The hemoglobin level was maintained above 9.5 g/dL in the third trimester. However, urine volume gradually decreased to less than 100 mL per day. The follow-up data of dry weight, predialysis BUN and Hb level are shown in .\nFetal growth was appropriate for gestational age. Routine fetal karyotyping was performed at 16 gestational weeks, but mutation screening was not performed. At 34.5 weeks of gestation, the membrane ruptured during HD, and vaginal delivery was performed without any complications. She delivered a healthy female weighing 2,100 g. One week after delivery, the patient was hospitalized for 5 days because of postpartum cardiomyopathy.