Good obstetric outcome of consecutive pregnancies in a woman with Alport syndrome

Abstract

Alport syndrome is characterized by progressive glomerulonephritis, which is an inherited disorder, with 80% of patients as an X-linked fashion [1]. How pregnancy aVects this disorder or vice versa is unclear. No reports, to our knowledge, described the course of consecutive pregnancies of Alport patients. We previously reported a primiparous woman with Alport syndrome of very suggestive X-linked type, who had mild but stable proteinuria during pregnancy [1]. Hospitalization for bed rest and close observation did not deteriorate her condition: proteinuria 1.0–2.0 g/day, serum albumin 2.4 g/dL (normal range 3.8–4.9), serum creatinine 0.70 mg/dL (normal range 0.4–0.8), and blood pressure 110/60 mmHg. She had given birth to a healthy term male infant via cesarean section because of failure to progress. Literature review using Pubmed and Igakuchuouzasshi (Internet-based medical literature retrieval system for Japanese) had yielded only three case reports describing the pregnancy course of three primiparous women with Alport syndrome, which we had summarized [1]. We had concluded that (1) pre-existing hypertension and renal dysfunction and (2) serum creatinine elevation, proteinuria increment, and hypertension deterioration during pregnancy may predict a poor outcome of both mothers and infants. On the other hand, if these signs are absent, a good obstetric outcome may be expected. We ended the previous article, “when the urinary protein level and blood pressure are stable, it is not certain whether hospitalization is still necessary” [1]. The eVect of the Wrst pregnancy on the subsequent pregnancy also remained unclear. We wish to brieXy report her second pregnancy course: to our knowledge, this is the Wrst report of consecutive pregnancy course of Alport patients. After her Wrst delivery, she received no therapy. Proteinuria (1.0 g/day), serum albumin (3.5 mg/dL), creatinine level (0.67 mg/dL), and blood pressure (118/62 mmHg) remained stable. Genetic analysis was not performed. Two years later, she became pregnant. During her second pregnancy, mild proteinuria (0.8–2.0 g/day), slightly low albuminemia (2.5–2.8 mg/dL), serum creatinine level (0.67–0.70 mg/dL), and blood pressure (102–123/58–78 mmHg) remained stable throughout the three trimesters. We decided to follow her on an outpatient basis. At 37 weeks, she had labor initiation and she gave birth to a healthy female infant weighing 2,458 g, appropriate for date infant in Japanese standards, via repeat cesarean section. Follow-up for 2 months postpartum indicated that proteinuria, creatinine level, and blood pressure remained stable. Since our 2009 report [1], only one Japanese report [2] has been added to the previously summarized three reports [1]: a primiparous woman with suggestive X-linked Alport syndrome with low albuminemia and slight edema gave birth to a healthy term infant without her condition deteriorating during pregnancy [2]. Undoubtedly, many women with Alport syndrome become pregnant; however, their outcome has been rarely reported. Rather, many women with this disease may have good obstetric outcome, which may be the reason why reports regarding this issue have been few. However, as previously described [1], Matsuo et al. [3] reported that pregnancy may have badly aVected S. Matsubara (&) Department of Obstetrics and Gynecology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498, Japan e-mail: matsushi@jichi.ac.jp