Journal of Japan Society of Perinatal and Neonatal Medicine
Online ISSN : 2435-4996
Print ISSN : 1348-964X
Current issue
Journal of Japan Society of Perinatal and Neonatal Medicine
Displaying 1-40 of 40 articles from this issue
Review
  • Kentaro Kurasawa
    2025 Volume 61 Issue 1 Pages 1-13
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     RS virus infection is an important disease in infants and young children, but there is no effective prevention or treatment, and treatment is mainly conservative. Since 2000, the administration of the monoclonal antibody palivizumab has been used for high-risk infants. In 2024, a maternal-infant immune vaccine and the long-acting monoclonal antibody nirsevimab became available. In this paper, after describing the epidemiology of RS virus infection, we will outline the newly added vaccines and monoclonal antibodies, and consider prevention strategies for RS virus infection in Japan.

    Download PDF (6730K)
  • Yasuji Kitabatake
    2025 Volume 61 Issue 1 Pages 14-20
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Down syndrome is caused by trisomy of chromosome 21 and is associated with a wide range of symptoms, including intellectual disability, hematologic abnormalities, and congenital heart disease. Although it is a wellknown condition, the detailed pathological mechanisms remain unclear, and no definitive treatments have been established. In recent years, the invention of induced pluripotent stem cells(iPS cells)and the advent of genome editing technologies have enabled the creation of precise and diverse disease model cells. By differentiating these cells into hematopoietic, neural, and cardiac lineages, researchers anticipate significant progress in elucidating the complex pathology of the syndrome. Regarding the intellectual disability associated with Down syndrome, the underlying mechanisms are gradually being uncovered; however, therapeutic development still faces the major challenge of accurately correcting gene dosage. In the future, combining emerging genome editing techniques with advanced drug delivery systems may provide a solution to this issue.

    Download PDF (2993K)
  • Rie Kato
    2025 Volume 61 Issue 1 Pages 21-30
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     As more parturients request labor analgesia, more delivery facilities introduce labor analgesia. Neuraxial analgesia is the method of analgesia for labor. This review focuses on safety issues in neuraxial analgesia for labor.

     The most serious complications for the mother include high spinal anesthesia, local anesthetic systemic toxicity(LAST), and epidural hematoma. Analgesia providers must take preventive measures against these complications. In order to prevent high spinal anesthesia and local anesthetic systemic toxicity, aspiration before injecting each dose of local anesthetic is useful. More importantly, analgesia provider should inject no more than 2-3 mL of local anesthetic as a single bolus and watch for early signs of high spinal anesthesia and LAST. Neuraxial analgesia should not be offered to parturients with coagulopathy in order to avoid epidural hematoma. If respiratory arrest occurs due to high spinal anesthesia, mask ventilation is mandatory.

     This review also discusses other complications and side effects of neuraxial analgesia, such as post-dural puncture headache, neurological damage, effects on labor progress, maternal fever, and effects on the neonate.

    Download PDF (3339K)
Rapid Communication
Originals
  • Izumi Iitsuka, Shuji Ishida, Rika Sekiya, Kohei Kawada, Yukako Kosaka, ...
    2025 Volume 61 Issue 1 Pages 33-38
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Neonatal lupus, arising from maternal systemic lupus erythematosus or Sjögren’s syndrome, occurs due to the transmission of anti-SS-A and anti-SS-B antibodies from the mother to the fetus via the placenta around 18 to 24 weeks of gestational age. While transient extracardiac conditions like skin lesions, hepatic dysfunction, and cytopenia typically resolve by six months of age, congenital complete atrioventricular block(CCAVB)remains irreversible and carries a poor prognosis. This study aimed to investigate risk factors and short-term outcomes of CCAVB in neonatal lupus managed at Kitasato University.

     This single-center retrospective study included neonatal lupus from 2006 to 2022. Infants were categorized into CCAVB-present and CCAVB-absent groups. Maternal and neonatal backgrounds were analyzed, and short-term outcomes were evaluated up to infancy or school age.

     Among 16 neonatal lupus diagnosed over 14 years, five had CCAVB. Maternal autoimmune disease was absent in all CCAVB cases, contrasting with 73% in the non-CCAVB group. All CCAVB cases received treatment compared to only 27% in the non-CCAVB group. Pacing procedures were performed for all CCAVB, with no fatalities reported. At the final follow-up, four out of five CCAVB cases attended mainstream schools, indicating favorable neurological outcomes.

     Neonatal lupus complicated by CCAVB, often arising from undiagnosed maternal autoimmune conditions, warrant close fetal monitoring upon bradycardia detection. Timely pacing interventions offer promising outcomes, emphasizing the importance of early detection and intervention in improving short-term prognosis.

    Download PDF (1527K)
  • Yurika Okami, Hiroyuki Tsuda, Atsuko Tezuka, Yumiko Ito, Ai Saito, Mio ...
    2025 Volume 61 Issue 1 Pages 39-43
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Objective:Cervical insufficiency is one of the major causes of preterm birth(PTB), and cervical cerclage is commonly used for its treatment. Although there are reports suggesting that cerclage depth(from cerclage to external os)affects the duration of prolonged pregnancy, a consensus has not yet been achieved. Therefore, we retrospectively reviewed cases of cervical cerclage performed in our hospital over the past 5 years. Methods:We reviewed the medical records of 102 cases who underwent cervical cerclage at our hospital from July 2018 to June 2023, and investigated the perinatal outcomes.

     Results:Prophylactic cervical cerclage(prophylactic group)was performed in 50 patients and therapeutic cervical cerclage(therapeutic group)was performed in 52 patients. PTB was defined as less than 36 weeks. 7 cases(14%)in the prophylactic group and 15 cases(28%)in the therapeutic group had PTB. The duration of prolonged pregnancy was 134(±27.0)days in the prophylactic group and 95(±28.0)days in the therapeutic group(P < 0.001).

     In the therapeutic group, cervical length before and after cerclage and cervical depth showed a significant positive correlation with the duration of prolonged pregnancy, respectively. Multiple regression analysis showed that BMI(β=-1.85, P=0.005), gestational age(β=-0.62, P < 0.001), and post-treatment cervical length(β=1.81, P < 0.001)were significant, but the cerclage depth(P=0.715)was not significant.

     Conclusion:In therapeutic cervical cerclage, post-treatment cervical length, not cerclage depth, was significantly associated with the extension of the pregnancy period. Post-treatment cervical length of > 20mm should be achieved when performing cervical cerclage to improve pregnancy outcome.

    Download PDF (1867K)
  • Hidetoki Utsugi, Daisuke Higeta, Ayuko Tanaka, Tomoko Matsuda, Akito M ...
    2025 Volume 61 Issue 1 Pages 44-49
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     【Objective】We use intra-aortic balloon occlusion(IABO)when performing cesarean hysterectomy(CH)for placenta accreta spectrum at our hospital. During CH, we monitor regional oxygen saturation(rSO2)using INVOSTM(Medtronic. Ireland)to evaluate blood flow in the lower limbs. We investigated the usefulness of lower limbs rSO2 in cases with IABO.

     【Methods】We targeted 21 cases in which placenta accrete spectrum was suspected and IAB was placed at our hospital. We investigated lower limbs rSO2 during CH and the presence or absence of complications retrospectively.

     【Results】CH was performed in 13 of the 21 cases in which IAB was placed. In the other 8 cases, we did not extract uterus. There was significant differences in the average value of lower limb rSO2(p < 0.0001). No complications occurred in all cases.

     【Conclusion】It is possible to monitor lower limbs rSO2 during IABO when placenta accreta spectrum is suspected.

    Download PDF (1772K)
  • Seiji Yashima, Norikazu Watanabe, Tomomichi Ito, Urara Idei, Keiko Yam ...
    2025 Volume 61 Issue 1 Pages 50-56
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Objective: In this study, we reviewed the management of retained products of conception(RPOC)among women in rural Japan.

     Methods: We retrospectively analyzed data obtained from women diagnosed with RPOC at our hospital between April 2019 and March 2022.

     Results: The study included 10 women(median age 31.0 years). Four women underwent in vitro fertilization and four were primiparas. Eight women delivered at term, one woman delivered a second-trimester stillbirth, and one woman underwent a first-trimester abortion. The mode of delivery was vaginal in six women and included second-trimester stillbirths, cesarean delivery in three, and manual vacuum aspiration in one woman. After diagnosis, five women underwent only observation, two underwent only uterine artery embolization(UAE), two underwent UAE combined with transcervical resection, and one woman underwent total hysterectomy. Among the four women who lived outside the city where the hospital was located, three underwent an invasive procedure.

     Conclusion: Considering the distance between the hospital and their residence, some women who required only observation opted for prophylactic invasive treatment. Women with RPOC should receive treatment considering the rural medical setting and resource availability.

    Download PDF (2060K)
  • Junya Yamada, Reiji Nakano
    2025 Volume 61 Issue 1 Pages 57-64
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     To test the hypothesis that the effect of being born small for gestational age(SGA)on developmental delay is more pronounced with increasing gestational age in extremely preterm infants, this study analyzed the association between being born SGA and developmental quotient(DQ)at corrected age 3 years, stratified by gestational age. We included 168 children who were admitted to our NICU between 23 weeks 0 days and 27 weeks 6 days of gestation between 2011 and 2019, and who underwent the Kyoto Scale of Physiological Developmental test at corrected age 3 years. Infants with chromosomal anomalies and congenital malformations were excluded. We defined SGA infants as those whose birth weight was less than the 10th percentile and compared the DQs of SGA and non-SGA infants at 3 years of age. The corrected 3-year DQ of SGA children was statistically significantly lower than that of non-SGA children only at 26 and 27 weeks’ gestation. The slope of the regression line showing the correlation between birth weight Z-score and corrected 3-year DQ was greatest at 26 weeks’ gestation. In conclusion, the results of this study did not prove our hypothesis.

    Download PDF (1656K)
  • Miyuki Obata, Mayumi Maruyama, Shota Horikawa, Takeshi Fukunaga, Ayaka ...
    2025 Volume 61 Issue 1 Pages 65-70
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Background: It is unclear the risk and benefit of restrictive or unrestrictive use of episiotomy.

     Objective: To assess maternal and neonatal prognosis when we use episiotomy at delivery restrictively or unrestrictively.

     Design: A retrospective cohort study.

     Setting: Tertiary perinatal medical facility in Japan.

     Study design: The unrestrictive use group included 299 vaginal deliveries between January 1 and December 31, 2015 before restricting episiotomy, and the restrictive use group included 256 vaginal deliveries between January 1 and December 31, 2018 after restricting episiotomy. We assessed perinatal prognosis in mothers and newborns and risk factors for severe lacerations.

     Result: The episiotomy rate was 61% in the unrestrictive use group and 32% in the restrictive use group, which was significantly higher in the routine group(P < 0.001). Third-or fourth-degree lacerations were significantly fewer in the restrictive use group, at 8% in the unrestrictive use group and 2.7% in the restrictive use group(OR 3.104, 95% CI 1.315-7.330, P=0.007). Additionally, labor time of the second stage was significantly longer in the restrictive use group. After adjusting for maternal age and presence or absence of episiotomy, the adjusted odds ratios for unrestricted incision, first birth, and instrumental delivery were 4.187(95% CI 1.658-10.576), 3.823(95% CI 1.355-10.791), and 6.479(95% CI 2.728-15.388), respectively, which were independent risk factors of severe perineal laceration.

     Conclusion: We might be able to reduce severe perineal laceration with restrictive use of episiotomy at deliveries. It is desirable to perform episiotomy with limited indications.

    Download PDF (1389K)
  • Yasufumi Hoshimoto, Mie Hosomizo, Hyuga Matsumoto, Tomoki Nobumasa, Ma ...
    2025 Volume 61 Issue 1 Pages 71-76
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     [Objective]The purpose of this study was to consider the usefulness of culture medium selective in Group B Streptococcus(GBS)at third-trimester screening test in pregnancy.

     [Methods]Since January 2022, we use the culture media selective in GBS at third-trimester screening test in pregnancy, and standardized the specimen collection sites to include vaginal openings and anus. We retrospectively compared the laboratory data of culture tests in 551 cases of delivery after third-trimester screening from January 2022 to December 2023(general medium plus selective medium group), and 676 cases from January 2020 to December 2021(general medium only group).

     [Results]The GBS-positive rates of general medium only group and general medium plus selective medium group were 13.6% and 20.9%, respectively, and significantly higher in the latter group(p=0.00073).

     [Conclusion]We suggest that collecting specimens from the appropriate sites and using the GBS selective media can improve the sensitivity of GBS detection at the third-trimester screening.

    Download PDF (1977K)
  • Akane Honda, Motoyoshi Kawataki, Katsuaki Toyoshima
    2025 Volume 61 Issue 1 Pages 77-84
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Background: Prenatal diagnosis of isolated PCFO/PRFO(premature closure or restriction of the foramen ovale in fetuses with structurally normal heart)is increasing, but there are no reports on severity assessment of the association between fetal echocardiographic findings and postnatal symptoms.

     Aims: To investigate the clinical features and severity prediction of patients prenatally diagnosed with isolated PCFO/PRFO.

     Methods: Twenty-two patients who were prenatally diagnosed with isolated PCFO/PRFO between 2007 and 2014 were included in the study and divided into two groups: a treatment group in which respiratory symptoms were observed after birth and required therapeutic intervention, and a no-treatment group in which no symptoms were observed or respiratory symptoms resolved spontaneously. The timing of prenatal diagnosis, postnatal clinical course, patient background, and fetal echocardiography findings were compared retrospectively from medical records.

     Results: Prenatal diagnosis was made at a median gestational age of 35 weeks in both groups. Among these patients, 10 were treatment group(45%)and 12 were no-treatment group(55%). Respiratory symptoms worsened in 12/22 patients(55%)by 24 hours after birth. Fetal echocardiographic findings were: ASA(atrial septal aneurysm)was more common in the treatment group. RVDd Z-score was not significantly different, but LVDd Z-score was significantly lower in the treatment group. TVD/MVD and PVD/AVD were higher in the treatment group. LVDd Z-score <−2.5, TVD/MVD > 1.50, and PVD/AVD > 1.40 were highly sensitive and specific indicators as cutoff values to predict the treatment group.

     Conclusion: Continuous fetal assessment is necessary, with a focus on the rapid increase in the TVD/MVD in late pregnancy.

    Download PDF (2507K)
  • Naoto Komatsu, Kiichiro Furuya, Yoshitomo Tanaka, Rikuto Hirose, Yuki ...
    2025 Volume 61 Issue 1 Pages 85-92
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     During the COVID-19 pandemic, many pregnant women contracted the virus. In Japan, the severity of COVID-19 is evaluated based on oxygen saturation, respiratory symptoms, and imaging findings. During the fifth wave of the pandemic in 2021, we frequently encountered pregnant women who complained of prolonged shortness of breath after the isolation period was lifted, although the cases were mild to moderate. To quantitatively evaluate the respiratory condition, we conducted pulmonary function tests using a spirometer. Among the 17 pregnant women diagnosed with COVID-19 in the third trimester, 10(58.8%)exhibited restrictive ventilatory impairment immediately after the isolation period was lifted. The percentage predicted vital capacity(% VC)was significantly lower than in non-COVID-19 pregnant women. At the 1-month postpartum check-up, only one of 13 patients(7.7%)who underwent follow-up pulmonary function tests showed restrictive ventilatory impairment; furthermore, % VC had significantly improved. Our findings revealed that restrictive ventilatory impairment is frequent in pregnant women with COVID-19 but usually normalizes by approximately 1 month postpartum. These findings do not support routine respiratory function testing for all pregnant individuals affected by COVID-19. However, if abnormal respiratory function persists beyond the 1-month postpartum checkup, it may serve as a clinical indicator of a potential deviation from the typical natural course of COVID-19 during pregnancy.

    Download PDF (1852K)
  • Saori Unno, Aiko Sasaki, Rina Akaishi, Katusuke Ozawa, Rika Sugibayasi ...
    2025 Volume 61 Issue 1 Pages 93-99
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     【Purpose】To analyze chorionic villus sampling performed at our center.

     【Methods】We retrospectively reviewed the medical records of patients who underwent chorionic villus sampling at our hospital from June 2002 to April 2021.

     【Results】The total number of chorionic villus sampling was 653. The number by year peaked at 81 in 2012 and has been decreasing after the year. Chromosome tests(G band, FISH)were performed in 566 cases(86.7%), and genetic tests in 319 cases(48.8%). The median maternal age was 34.8 years(22 to 48 years), and the median duration of pregnancy was 12 weeks(10 to 22 weeks). As for the method, transabdominal method was used in 468 cases(71.7%)and vaginal method in 185 cases(28.3%). Indications for chorionic villus sampling were, in order of prevalence, family history of genetic disorders 315(48%), Increased nuchal translucency 122(18%), fetal ultrasound abnormalities 94(14%), Positive NIPT result 34(5%), structural abnormalities in previous siblings 33(5%), etc. The results of the chromosome tests were 400(71%)normal karyotypes, 159(28%)abnormal karyotypes, 5(1%)culture failure, and 2(0.3%)results unknown. Abnormal karyotypes were, in order of prevalence, T21 55(33.5%), T18 39(24%), etc. Adverse events are 6(0.91%), pregnancy losses are 4(0.61%).

     【Conclusion】The following is a summary of the status of chorionic villus sampling in our center. It is necessary to understand the advantages and disadvantages of chorionic villus sampling, and to provide adequate genetic counseling before testing.

    Download PDF (1550K)
  • Yuka Kurachi, Kenji Tanimura, Sonoko Suda, Naohisa Masuko, Akiko Uchid ...
    2025 Volume 61 Issue 1 Pages 100-106
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Objective: To determine the risk factors for preterm delivery(PD)before 34 gestational weeks(GWs)following therapeutic cervical cerclages in pregnant women with cervical incompetence(CI)or those with cervical shortening at risk of CI.

     Patients and Methods: In this retrospective study, fifty-one singleton pregnancies diagnosed with CI or those with cervical shortening at risk of CI, who underwent therapeutic cervical cerclage and delivered at our hospital between 2014 and 2022 were enrolled in the final analysis. The participants were divided into two groups: the group of PD < 34 GWs(n=13)and the group of delivery at 34 GWs or later(n=38). We determined the risk factors for PD < 34 GWs following therapeutic cervical cerclages by logistic regression analysis,

     Results: Multivariate logistic regression analysis revealed that the presence of amniotic fluid sludge〔Odds Ratio(OR)12.8[95% Confidence Interval(CI)1.1-150.3], p < 0.05〕and the highest postoperative maternal blood white blood cell(WBC)count(x103 /μL)[OR 1.3(95% CI 1.04-1.6), p < 0.05]were independent risk factors for PD < 34 GWs following therapeutic cervical cerclages.

     Conclusion: Pregnant women who had amniotic fluid sludge and high postoperative maternal blood WBC counts should be intensively managed to prevent PD after therapeutic cervical cerclages.

    Download PDF (1681K)
  • Kota Iwasaki, Satoru Funaki, Kokoro Inoue, Ria Kim, Sota Fukushima, Ko ...
    2025 Volume 61 Issue 1 Pages 107-113
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Retained products of conception(RPOC)are tissues that remain in the uterus after a miscarriage or delivery. Although management guidelines have not been established and treatment methods vary widely, excessive intervention should be avoided to prevent complications such as infertility. This retrospective study evaluated the effectiveness of conservative management for RPOC in our hospital between January 2015 and May 2023. Forty-one cases of RPOC treated initially with conservative management were included. In 37 cases(90.2%), RPOC naturally disappeared with observation alone, whereas 4 cases(9.8%)required additional intervention(all 4 received uterine artery embolization in combination with dilatation and curettage; 2 additionally underwent transcervical resection). The median duration until the disappearance of RPOC with conservative management was 150 days. In conclusion, conservative management is reasonable for most cases of asymptomatic RPOC, although patients should be monitored for severe postpartum hemorrhage.

    Download PDF (2093K)
  • Maho Miki, Kenji Tanimura, Sonoko Suda, Naohisa Masuko, Akiko Uchida, ...
    2025 Volume 61 Issue 1 Pages 114-119
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Objective: The aim of this retrospective study was to determine the factors associated with the success of vaginal delivery(VD)in nulliparous women with a pre-pregnancy body mass index(BMI)of ≥ 30 kg/m2

     Patients and Methods: Nulliparous women with a pre-pregnancy BMI of ≥ 30kg/m2 who attempted VD at our hospital between January 2018 and August 2022 were enrolled for the final analysis. A logistic regression analysis was performed to determine the factors associated with the success of VD.

     Results: A univariate logistic analysis revealed that only pre-pregnancy BMI(kg/m2)was a significant factor associated with the success of VD. Furthermore, receiver operating characteristic analysis demonstrated that the optimal cutoff value of pre-pregnancy BMI for predicting the success of VD in nulliparous women with a pre-pregnancy BMI of ≥ 30kg/m2 was 34.5kg/m2.

     Conclusion: A diet aiming for a pre-pregnancy BMI of ≤ 34.5kg/m2 may be effective in avoiding cesarean delivery in nulliparous obese women with a pre-pregnancy BMI of ≥ 30kg/m2.

    Download PDF (1598K)
  • Keiko Yano, Atsushi Daimon, Seiichiro Nao, Tomoko Matsumoto, Atsushi Y ...
    2025 Volume 61 Issue 1 Pages 120-125
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     The WHO recommends the Robson 10-Group Classification System(RTGCS), which classifies deliveries into 10 groups based on five parameters, for the purpose of evaluating cesarean section rates and comparing with other facilities. In this study, we classified 1,100 deliveries at our hospital using the RTGCS, calculated the number and rate of cesarean sections for each group, and compared them with previous reports from regional perinatal maternal and child medical centers. In our hospital, the cesarean section rate was higher than in other facilities in the group of primiparas who delivered a single fetus in vertex presentation after 37 weeks and multiparas who delivered spontaneously through labor. Our hospital performs vaginal deliveries after cesarean section, and the cesarean section rate was significantly lower in cases of single pregnancy after a previous cesarean section in which the fetus was delivered in vertex presentation after 37 weeks.

     This is the first report to compare the results of using the RTGCS between facilities of the same size in Japan. Further comparative studies of RTGCS in Japan will require multicenter collaborative studies that examine in detail the factors leading to cesarean section in each group.

    Download PDF (1383K)
Case reports
  • Fumi Nagawa, Ikuno Kawabata, Shiho Matsumoto, Jun Ogawa, Momoko Owada, ...
    2025 Volume 61 Issue 1 Pages 126-131
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     May-Hegglin anomaly(MHA)is an autosomal dominant inheritance disorder characterized by varying degrees of thrombocytopenia, giant platelets, and cytoplasmic inclusion bodies in the granulocytes. We report a case of a pregnancy complicated with MHA in which a planned delivery was performed after platelet transfusion with a confirmed diagnosis of MHA by genetic testing.

     A 28-years-old primigravida. At the age of 22, a blood test revealed giant platelets, and genetic testing revealed an MYH9 gene mutation(E1841K), leading to a definite diagnosis of MHA. There was no bleeding tendency or Alport symptoms. After spontaneous conception, her blood platelet count remained at approximately 40,000/μL throughout the pregnancy. After consultation with the hematologist, we decided to proceed with a planned delivery at 38 weeks of gestation, following platelet transfusion to mitigate the risk of excessive bleeding during the peripartum period. Labor induction was initiated at 38 weeks and 0 days of gestation, and the patient successfully delivered vaginally after receiving a total of 30 units of platelet transfusion. The neonate weighed 2,552g, and the maternal blood loss during delivery was 890mL. The neonate’s platelet count was 75,000/μL, and peripheral blood smear revealed the presence of giant platelets, a finding consistent with MHA. Although there are no established guidelines for the treatment of pregnancies complicated with MHA, multidisciplinary management is necessary, paying attention to the risk of hemorrhage during delivery and the inherent characteristic of the genetic disease.

    Download PDF (1833K)
  • Aya Watanabe, Takuya Sugahara, Yoshihiro Kitamura, Suguru Yamashita, H ...
    2025 Volume 61 Issue 1 Pages 132-135
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Uterine rupture without a history of uterine surgery is rare, with approximately 1 case in 15,000 incidences. However, the rupture is associated with poor perinatal outcomes and requires immediate intervention. We report a case of uterine rupture in a multiparous woman with an unscarred uterus that was successfully treated with emergency cesarean section, that resulted in the survival of both the mother and child.

     The patient was a 36-year-old woman(gravida 3 para 2). She had no particular medical history other than a vaginal delivery of twins 12 years previously. No abnormalities were observed during the pregnancy. She visited the hospital at the onset of labor on the 36th week and 4th day of pregnancy. Fetal heart rate monitoring revealed prolonged deceleration after the visit. Therefore, an emergency cesarean section was performed for fetal dysfunction. During laparotomy, the placenta was exposed in the abdominal cavity, leading to the diagnosis of complete uterine rupture. The uterus was successfully sutured and preserved. Although the neonate initially presented with severe acidosis, no neurological abnormalities were noted until 9 months of age. In this case, the patient had no significant medical history other than a previous twin pregnancy. Thus, we considered the possibility that hyperextension of the uterine myometrium associated with a twin pregnancy might have been a risk factor for uterine rupture.

    Download PDF (2622K)
  • Yuko Kubo, Haruki Sassa, Touko Iwase, Mai Ogawa, Yuji Kawamura, Moe Ha ...
    2025 Volume 61 Issue 1 Pages 136-140
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Atypical hemolytic uremic syndrome(aHUS)is a disease that shows pathological condition of thrombotic microangiopathy(TMA)presenting with acute renal failure, hemolytic anemia, and thrombocytopenia, and approximately half of patients with this disease experience progression to end-stage renal failure, indicating poor prognosis. We report our experience with a case of aHUS that developed in postpartum period and well responded to eculizumab. A patient was 38 years old with gravida 4, para 2. The patient had membrane rupture at 39 weeks and two days of pregnancy and had normal delivery on the following day. The patient was discharged on Postpartum Day 4 because the amount of blood loss after the delivery was small and the postpartum course was favorable. On Postpartum Day 7, massive genital bleeding occurred and the patient was thus visited the hospital. As continuous bleeding from the uterus was identified, contrast enhanced CT was performed and it showed contrast medium leakage in the uterus. Therefore, uterine artery embolization was performed. Total blood loss reached 1, 500mL and blood transfusion was provided. After that, no continuous bleeding was identified, but renal function decreased gradually progressed. On Postpartum Day 12, acute renal failure, hemolytic anemia, and platelets decreased were noted, and pregnancy-related TMA was diagnosed. Since oliguria associated with acute renal failure was observed, plasmapheresis and hemodialysis were started. No improvement in the disease condition was obtained although plasmapheresis was performed three times, which suggested aHUS, and therefore treatment with eculizumab was started on Postpartum Day 14. Renal function and platelet count markedly improved thereafter, and the patient was discharged on Postpartum Day 35. The patient received additional administration of eculizumab on an outpatient basis, leading to successful improvement in renal function, and follow-up has been continued.

    Download PDF (2075K)
  • Mayaka Makita, Hiroshi Tomonobe, Junji Ueda, Makoto Nishida
    2025 Volume 61 Issue 1 Pages 141-146
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     The case is a 26-year-old pregnant woman at 11 weeks of gestation. She visited our hospital’s emergency department complaining of abdominal pain. Blood tests indicated inflammatory findings, and a plain CT scan revealed localized dilation and clustering of the small intestine; however, a definitive diagnosis was not achieved. Due to persistent symptoms, a diagnostic laparoscopic examination was performed, revealing band formation around the Meckel’s diverticulum and intussusception of the small intestine at the same site, leading to a diagnosis of intestinal obstruction secondary to Meckel’s diverticulitis. A Meckel’s diverticulectomy was subsequently performed, resulting in the resolution of symptoms. Perinatal management was continued at our facility, where labor induction was carried out for preeclampsia, culminating in a vaginal delivery in the cephalic position at 40 weeks and 1 day of gestation. Acute abdominal conditions due to Meckel’s diverticulum during pregnancy are rare and pose significant diagnostic challenges. Delays in management may adversely affect maternal and fetal outcomes. Therefore, imaging studies such as CT scans should not be hesitated upon, and surgical interventions, including exploratory laparotomy or diagnostic laparoscopy, should be actively considered for cases of unexplained acute abdominal pain during pregnancy.

    Download PDF (2132K)
  • Tomohiro Takahashi, Riki Nishimura, Yu Hironaka, Erina Nagao, Wakako S ...
    2025 Volume 61 Issue 1 Pages 147-151
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Lower urinary tract obstruction(LUTO)with oligohydramnios causes pulmonary hypoplasia, which is associated with high mortality rates. Even if a patient survives, renal and bladder dysfunction remains a long-term issue. Herein, we report the case of a neonate with an anterior urethral valve(AUV), and diverticulum with significant bladder dysfunction after birth, despite the absence of oligohydramnios. At 28 weeks of gestation, a male fetus was noted to have bilateral hydronephrosis, hydroureter, and keyhole signs on prenatal ultrasound. LUTO was suspected, but the amniotic fluid volume was maintained. One day after a full-term birth, urethral diverticulum and AUV were suspected due to an inguinal mass. Although his urine output was sufficient, overflow incontinence was observed, and percutaneous vesicostomy was performed on day 3 of life because of bladder hypertrophy and worsening hydronephrosis. The hydronephrosis did not improve after the procedure, and the patient developed urinary tract infections shortly after discharge. In cases of LUTO without oligohydramnios, there is no indication for fetal therapy or early delivery. However, the present case demonstrates that, even without oligohydramnios, severe bladder dysfunction can develop from intermittent occlusion with increased pressure in the bladder in utero, emphasizing the need for caution.

    Download PDF (1876K)
  • Eri Takahashi, Takao Kobayashi, Ryo Nishimaki, Sota Iwatani, Kento Som ...
    2025 Volume 61 Issue 1 Pages 152-157
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Hemimegalencephaly is a congenital brain malformation often accompanied by symptomatic epilepsy. In patients with refractory epilepsy onset during the neonatal period, psychomotor developmental delay tends to progress. We here report a newborn case who presented with seizure on day 3 of life and was diagnosed as left hemimegalencephaly via head magnetic resonance imaging on day 7 of life. Despite treatment with multiple antiepileptic drugs, including phenobarbital, levetiracetam, and lacosamide, clinical seizures and epileptic discharges on electroencephalogram(EEG)persisted. Slow wave activity in the background EEG of the unaffected hemisphere also progressed. To control the refractory epilepsy, a hemispherectomy was performed at 5 months of age. Following this, the frequency of seizure decreased, and the background EEG activity of the unaffected hemisphere normalized with single-drug therapy using levetiracetam. Her psychomotor developmental resumed. This report details the changes in clinical symptoms and EEG findings from the neonatal to the infancy period. We emphasize the importance of not only early diagnosis in the perinatal period but also subsequent epilepsy treatment in collaboration with neurologists and neurosurgeons in managing hemimegalencephaly.

    Download PDF (4835K)
  • Masaaki Kuda, Emi Higa, Mayumi Tsukayama, Tokuro Baba, Tomohide Yoshid ...
    2025 Volume 61 Issue 1 Pages 158-162
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     We encountered an extremely rare case of an extremely low-birth-weight infant diagnosed with congenital esophageal stenosis. The infant was born at 26 weeks and 0 days of gestation via emergency cesarean delivery because of fetal bradycardia. The birth weight was 778g, necessitating acute care management. At a corrected age of 35 weeks, the infant was transitioned to oral feeding, which resulted in coughing, bradycardia, and a decrease in oxygen saturation(SpO2). By 39 weeks of corrected age, oral secretions increased during feeding while bradycardia and SpO2 worsened. Inserting an esophageal pH-monitoring catheter was difficult. Esophagography revealed stenosis of the lower esophagus. At the corrected age of 43 weeks, balloon dilation was performed under general anesthesia with esophageal endoscopy and fluoroscopic observation. Coughing during feeding improved, and the patient was discharged at a corrected age of 45 weeks. Vomiting symptoms was observed after initiating solid food. Five more dilations were required until age two and a half years. Symptoms disappeared after dilation, and no growth retardation was observed.

    Download PDF (2605K)
  • Chinami Asai, Hiroshi Sato, Yusuke Yamaoka, Yumiko Takeuchi, Yu Takais ...
    2025 Volume 61 Issue 1 Pages 163-168
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Spontaneous septostomy of the dividing membrane in monochorionic diamniotic twins(MD twins)is rare. When there is a spontaneous septostomy of the dividing membrane, in addition to complications unique to MD twins, attention must be paid to umbilical cord entanglement. We recently reported a case of spontaneous septostomy of the dividing membrane in an MD twin. The case involved a 27-year-old, G1P0, naturally conceived pregnant woman who was referred to our hospital at nine gestational weeks and confirmed to have MD twins through ultrasound examination. At 19 gestational weeks, umbilical cord entanglement was visualized on ultrasonography; therefore, we considered the possibility of a spontaneous septostomy of the dividing membrane. She was diagnosed with selective intrauterine growth restriction(selective IUGR)type 3 at 24 weeks of gestation. Considering the risk of umbilical cord entanglement, an elective cesarean section was planned at 33 gestational weeks. At 32 weeks and three days of gestation, frequent variable deceleration was observed in the fetuses, with fetal growth restriction(FGR)during fetal heart rate monitoring. Emergency cesarean section was performed for non reassuring fetal status, which resulted in the birth of live babies. Umbilical cord entanglement was confirmed during surgery, and a dividing membrane was found in the placenta. There are a few reports on the natural rupture of membranes in MD twins, and further knowledge is expected to improve our understanding of this in the future.

    Download PDF (3872K)
  • Rui Yoshii, Kiguna Sei Okawa, Kosuke Miyai, Eri Ueda-Abe, Keiichiro T ...
    2025 Volume 61 Issue 1 Pages 169-173
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     A 36-week pregnant woman was referred to our hospital due to a fetal intrathoracic cyst. Fetal ultrasonography and MRI revealed a 3-cm diameter simple cyst in the mid or posterior mediastinum of the right thoracic cavity, compressing the lung from outside. There was no continuity with other thoracic organs, and there was no obvious blood flow in the relatively thick cyst wall. Most of its content was uniformly hypoechoic. We suspected a foregut duplication cyst from these findings. After birth at 38-week, the newborn required no resuscitation immediately but began to show respiratory disorder on day 6. CT scan revealed that the cyst enlarged slightly and compressed the right main bronchus. An open chest surgery was performed and removed the cyst on day 11. The pathological diagnosis was a gastric duplication cyst. Gastric duplication cyst is one of the enteric duplication cysts, and intra-thoracic solitary type is rare. When an intrathoracic cyst is detected prenatally, it is important to narrow down the diagnosis based on the location and characteristics of the cyst for appropriate management after birth. Evaluation of positional relationship between cyst and bronchus may be useful to estimate risk for postpartum respiratory disorder.

    Download PDF (2692K)
  • Takako Arai, Naoya Morisada, Hitomi Mimura, Tomoko Kihara, Megumi Kane ...
    2025 Volume 61 Issue 1 Pages 174-179
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Silver-Russell syndrome(SRS)is a congenital imprinting disorder characterized by severe fetal growth restriction and postnatal growth retardation, relative macrocephaly, body asymmetry. Most causes of SRS are hypomethylation of the imprinting center region 1(ICR1, H19/IGF2: IG-DMR). We report a case of SRS in only one child who shared the placenta of dichorionic triamniotic triplets. The patient’s mother was 33-year-old nulliparous woman. She was diagnosed dichorionic triamniotic triplets and referred our hospital at 11 weeks of gestation. At 21 weeks of gestation, fetal growth restriction(FGR)was observed in both fetuses sharing one placenta. At 30 weeks of gestation, an emergency cesarean section was performed due to increased uterine contractions and maternal liver dysfunction. Her second child was suspected of having SRS due to postnatal growth retardation and distinct physical features characteristic of the condition, and the diagnosis was definitively confirmed through methylation analysis. It only occurred in one child of the triplets, it was possible that the epigenetic events occurred after the embryo divided into two inner cell masses, or that the twins were dizygotic monochorionic twins.

    Download PDF (2338K)
  • Takanori Miura, Hiroshi Mizumoto, Risa Asui, Mari Sonoda, Kousuke Endo ...
    2025 Volume 61 Issue 1 Pages 180-184
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     The patient was found megacystis from the fetal stage, and after birth, he had dysuria and vomiting repeatedly after feeding, so he was suspected allied disorders of Hirschsprung’s disease. Although full thickness intestinal biopsy is recommended for differential diagnosis, in this case, comprehensive gene analysis early after birth could diagnose ACTG2 visceral myopathy without invasive examination. Although the complications and severity of this disease vary widely, many cases have been reported to have pathological genetic abnormalities. Early diagnosis and prediction of poor prognosis may be useful in determining management strategies.

    Download PDF (1926K)
  • Hanaka Oshiro, Tomomi Shiga, Junko Naito, Masanori Isobe, Tatsuro Furu ...
    2025 Volume 61 Issue 1 Pages 185-191
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Primary nephrotic syndrome during pregnancy is extremely rare and with a high risk of developing perinatal complications. The patient was 34 years old, primipara. At 28 weeks gestation, she was referred to the hospital due to rapid weight gain and generalized edema from 1 week ago, and was then urgently hospitalized due to hypertension. She was found to have a large amount of urinary protein and was started on steroid therapy with a diagnosis of preeclampsia and nephrotic syndrome.

     The medical condition was resistant to treatment and gradually progressed, eventually she became oliguric and had findings of placental insufficiency, so an emergency caesarean section was performed at 31 weeks gestation. She underwent dialysis an hour and a half after the surgery, which caused a large amount of genital bleeding at 7 hours after the start of dialysis and required hemostasis techniques including massive blood transfusion and uterine artery embolization. Later, a kidney biopsy was performed and she was diagnosed with focal segmental glomerulosclerosis. Clinical diagnosis of nephrotic syndrome occurring during pregnancy is important for determining treatment strategy. The timing of dialysis should also be considered, taking into account the condition of the mother and child and the degree of hemorrhage.

    Download PDF (2749K)
  • Yuto Tsuruoka, Ken Takahashi, Osamu Samura, Aikou Okamoto
    2025 Volume 61 Issue 1 Pages 192-196
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Vasa previa is recognized as a perinatal high risk pregnancy resulting in fetal death due to rupture of umbilical cord vessels. The accurate location of the cord vessels is important in perinatal management. However, evaluation of vasa previa with ultrasonography may be inconclusive for accuracy of the vessel location and running vessels. In this case study, we report two patients in whom accurate evaluation using magnetic resonance imaging(MRI)was useful.

     Case 1 was a 30-year-old pregnant woman, para 1. Vasa previa was diagnosed on transvaginal ultrasonography at 30 weeks, but assessment of the distance from the internal cervical os was difficult. However, the distance from the internal cervical os to the vasa previa was accurately evaluated on MRI, allowing careful planning of Cesarean section, allowing safe perinatal care. Case 2 was a 26-year-old pregnant woman, para 3. Vasa previa was diagnosed on transvaginal ultrasonography at 26 weeks. The patient underwent MRI for vessel course evaluation, which revealed five fetal vessels, including the vasa previa, crossing over the anterior wall of the uterus in a complex manner. Consequently, Cesarean section was performed safely, without damaging the vessels.

     These cases indicate that MRI for vasa previa can facilitate the evaluation of the accurate location and vessel course, leading to safe perinatal care.

    Download PDF (3204K)
  • Taiichiro Shida, Junko Yamaguchi, Megumi Koga, So Sugimi, Sachie Suga, ...
    2025 Volume 61 Issue 1 Pages 197-201
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     In Japan, more than 10,000 people are newly diagnosed with tuberculosis each year. We report a case of pregnant women with bronchopulmonary tuberculosis with the difficulties in accurately diagnosis with reflection.

     The patient was a 29-year-old woman with no previous pregnancies. She developed a fever and cough at 15 weeks of gestation, and underwent a thorough examination for persistent fever of unknown origin. However, no abnormalities were found on chest X-ray, and the cause could not be identified even after four weeks. The patient underwent CT at 20 weeks’ gestation, because we thought pulmonary tuberculosis shoud be denied, just in case. Immediately after the diagnosis, a multidrug combination therapy was initiated. She was discharged at 25 weeks’ gestation with negative sputum test and vaginally delivered a male infant weighing 2,850g with Apgar score of 9/9 at 39 weeks’ gestation. The newborn was in good condition with no evidence of tuberculosis infection including the placenta and umbilical cord. In this case, it took four weeks to reach a definitive diagnosis. We should consider pulmonary tuberculosis as one of the differential diagnoses for unexplained cough and febrile condition persisting for more than 2 weeks, even during pregnancy and even with a negative primary screen for pulmonary tuberculosis.

    Download PDF (2837K)
  • Satoshi Komoike, Mariko Kamihigashi, Maiko Matsumae, Kouhei Nakagawa, ...
    2025 Volume 61 Issue 1 Pages 202-206
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     A woman in her 20s, gravida 2, para 0, with a medical history of convulsions and loss of consciousness before pregnancy was referred to our department for concomitant consultation with Neuropsychiatry at 10 weeks. At 31 weeks, the patient was found fallen in the waiting room after prenatal check-up. Subsequently, tonic-clonic seizures occurred frequently, and temporary intubation was performed. However, loss of consciousness, dissociation disorder, and seizures still occurred. The patient experienced chronic events, including hematemesis and allergy-like symptoms with antibiotic administration. At Neuropsychiatry, Munchausen syndrome was diagnosed. As judged, continued hospitalization may lead to aggravation of Munchausen syndrome, the patient’s neuropsychiatric disorder was explained only to her family, and she was discharged from the hospital. On the same day, she was discharged because there were no abnormal findings with herself and the fetus. At 39 weeks, labor started, resulting in normal vaginal delivery. After delivery, the patient lost consciousness, but the frequency improved, and she was discharged. Munchausen syndrome is a complicated disorder that is difficult to diagnose. Healthcare professionals must be informed about the disorder for appropriate management.

    Download PDF (2265K)
  • Hyuga Matsumoto, Mie Hosomizo, Maaya Ono, Tomoki Nobumasa, Yukiko Taga ...
    2025 Volume 61 Issue 1 Pages 207-211
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Hyperthyroidism, including Graves’ Disease, occurs most commonly in women of childbearing age. Treatment of pregnancy complicated by Graves’ disease aims to ensure maternal safety of the mother and prevent the development of thyroid dysfunction in the fetus. The first choice of treatment is oral administration of antithyroid drugs. When it is not possible to continue antithyroid drugs due to side effects or when thyroid function is difficult to control, thyroidectomy is selected. In this study, we report the case of a pregnant woman with T3-dominant Graves’ Disease who underwent subtotal thyroidectomy due to the pain/ arthralgia caused by antithyroid drugs and also due to the difficulty of controlling thyroid functions.

    Download PDF (2106K)
  • Taemi Tsutsui, Soichiro Obata, Mizuho Sawai, Kotone Suzuki, Kazuya Hii ...
    2025 Volume 61 Issue 1 Pages 212-216
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Since pregnancy increases the risk for venous thromboembolism(VTE), anticoagulation by subcutaneous heparin injection is recommended for women with a history of VTE during pregnancy. Venous injection or other anticoagulant medication can be another choice if it is difficult to continue the subcutaneous heparin injection. However, those alternatives have problems such as a long-term hospitalization or off-label use. Hereby, we present a case of 43-year-old women with history of caesarean section who diagnosed deep venous thrombosis(DVT)and started injecting herself with unfractionated heparin subcutaneously at 8 weeks of last pregnancy, but then switched to intravenous injection in hospital due to allergy reaction until selective caesarian section at 37 weeks of pregnancy. During the current pregnancy, she experienced the same allergic response to subcutaneous injection of unfractionated heparin. To avoid long hospitalization, she had total implantable central venous port system(CV port)insertion and initiated injection of heparin through the CV port at home. She continued preventative anticoagulation with no complications until delivery within public healthcare service, while managing the CV port through perinatal checkups and home-visit medical treatment. After delivery, oral warfarin was administered for 6 weeks. Central-venous administration of heparin via CV port at home has advantages in shortening hospitalization and reducing burdens on pregnant women. Therefore, it can be an effective choice for anticoagulation during pregnancy for those who have difficulty in long hospitalization or subcutaneous heparin injection.

    Download PDF (2100K)
  • Hana Okamoto, Kuniaki Ota, Wataru Saito, Mika Sugihara, Yuichiro Nakai ...
    2025 Volume 61 Issue 1 Pages 217-222
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Excessive weight gain is a major health problem in many developed countries. It is clear that obese women are at a disadvantage in terms of reproduction, and there is an increase in various complications during pregnancy, delivery, and the postpartum period. In this case, we experienced a super obese pregnant woman with a BMI of 57.5, and she was suffering from hypertension, impaired glucose tolerance, and a mental disorder. We struggled with how to manage her pregnancy and delivery but were able to succeed in delivering a live birth by cesarean section. The baby was a boy weighing 3,563g(99.9% tile)and was diagnosed as having neonatal asphyxia with an APS score of 2/5/5(1-minute/5-minute/10-minute values). Therefore, the neonate was treated with CPAP because of the marked acidosis in the NICU, and the umbilical artery blood gas consequently was quickly improved. Finally, the neonate was discharged on day 28 after birth. In Japan, extremely obese pregnant women are rare, and there is no standardized method for managing them, so it is necessary to respond to each case individually. Compared to normal-weight pregnant women and other obese pregnant women, extremely obese pregnant women are about twice as likely to experience maternal death or severe complications and therefore, require strict perinatal management.

    Download PDF (1661K)
  • Risa Yamada, Tomoshige Tanimura, Yasuko Togawa, Mari Sugimoto, Takao T ...
    2025 Volume 61 Issue 1 Pages 223-228
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Osteogenesis imperfecta(OI)is a skeletal disorder characterized by generalized bone fragility and a predisposition to fracture. The patient was a female infant suspected of having a skeletal disorder owing to limb deformities detected in utero. She was delivered by cesarean section at 38 weeks and 5 days of gestation with a birth weight of 2,322 g and mild respiratory distress. Genetic testing performed after birth identified pathogenic variants in the P3H1 gene, leading to a diagnosis of OI Type VIII according to the Online Mendelian Inheritance in Man classification. The identified pathogenic variants were c.484delG(p.Ala162LeufsTer22)and c.1453G > T(p.Glu485Ter), the latter of which is a novel variant that has not been reported previously. On day 40 after birth, intravenous disodium pamidronate hydrate was administered. Despite treatment, the infant experienced multiple limb fractures that began at 8 months of age. Additional findings included blue sclerae and dentinogenesis imperfecta in the primary teeth. On the basis of these clinical features, the patient was diagnosed with Sillence Type III. OI caused by P3H1 pathogenic variants is rare, with the present case being the second reported in Japan. Further accumulation of data on genotype-phenotype correlations in P3H1-related OI is essential for a better understanding of this condition.

    Download PDF (1873K)
  • Yu Takeuchi, Atsuhiko Sakai, Takako Sugiura, Kazushige Nakahara, Ryo K ...
    2025 Volume 61 Issue 1 Pages 229-233
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Delayed-interval delivery(DID)is an option for the management of multiple pregnancies when the first fetus is delivered prematurely and no subsequent fetus is delivered. Although DID improves the prognosis of subsequent babies, reported complications include intrauterine infection. The clinical course of cases in which pregnancy can be prolonged without complications and those in which pregnancy cannot be prolonged is not clear, and there are currently no management guidelines for attempting DID. We encountered 4 cases of dichorionic diamniotic twins who delivered their first fetus between 19 and 25 weeks of gestation and underwent DID. Two patients were able to continue their pregnancies without complications, and both delivered at term. In contrast, two cases in which signs of preterm birth, such as intermittent bleeding and uterine contractions, were observed during pregnancy resulted in preterm births. Although no clear clinical signs of infection were observed in these two cases until just before delivery of the subsequent fetuses, pathological examination of the placenta revealed histological chorioamnionitis. In conclusion, in DID, if signs of preterm birth are observed during pregnancy, it is important to consider the presence of an intrauterine infection as the background, regardless of the objective findings.

    Download PDF (2126K)
  • Kazuha Iesaki, Takaharu Yamada, Yukinobu Nomura, Taihei Tanaka
    2025 Volume 61 Issue 1 Pages 234-238
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     The patient was a boy born at 33 weeks and 5 days of gestation with a birth weight of 2,158g. The mother had a history of genital herpes, but no symptoms appeared during the pregnancy. Blood samples taken at the time of admission showed elevated IgM and mild thrombocytopenia, and a search for TORCH syndrome was conducted, which revealed positive cord blood HSV-IgM and IgG levels and positive serum HSV-IgM and IgG levels in the infant. Since the child was doing well and there were no further symptoms suggestive of congenital herpes simplex virus(HSV)infection, he was diagnosed with subclinical congenital HSV infection. After diagnosis, the patient was treated with intravenous acyclovir for 2 weeks. As for the mother, serum HSV-IgM and HSV-IgG were both positive and HSV-2 antibody was negative 9 days after delivery, and HSV-IgM was decreased and HSV-IgG was increased at 35 days after retest, suggesting reactivation of HSV-1 or subclinical re-infection after the mid-term pregnancy. Viral cultures of the pharynx, blood, spinal fluid, and urine were negative before the start of treatment, and HSV-PCR in blood and spinal fluid before the start of treatment and one week after the end of treatment were also negative. At 2 years and 8 months of age, the child’s growth and development are favorable, but subclinical congenital HSV infection is very rare, and more cases are needed to be accumulated regarding its treatment and prognosis.

    Download PDF (1632K)
  • Keisuke Onuma, Mizuho Kadooka, Ami Takesawa, Takahiro Mitani, Soichiro ...
    2025 Volume 61 Issue 1 Pages 239-243
    Published: 2025
    Released on J-STAGE: May 10, 2025
    JOURNAL FREE ACCESS

     Lymphangioleiomyomatosis(LAM)is a rare disease that predominantly premenopausal women. LAM is characterized by growth of smooth muscle-like tumor cells in the lungs and lymph nodes which form multiple cysts in the lungs. Although LAM is known to exacerbate pregnancy, symptoms such as dyspnea may be overlooked because they are common in healthy pregnant women. In this case, we encountered a patient in whom dyspnea during pregnancy was overlooked, resulting in a postpartum diagnosis of LAM.

     The patient was a 33-year-old woman who had experienced but not reported dyspnea since mid-pregnancy, assuming that it was a normal pregnancy-related symptom. When she was admitted to our hospital for a planned painless delivery at 39 weeks’ gestation, hypoxemia was observed. She had vaginal delivery on the same day, and subsequent CT tomography findings were suggestive of LAM. Three months postpartum, a bronchoscopic lung biopsy confirmed the diagnosis of LAM. Her respiratory function worsened six months postpartum, so Laparimus was initiated, and the patient was referred to university hospital for lung transplantation. LAM carries various perinatal risks, but its symptoms may be missed during pregnancy, leading to delayed diagnosis. Therefore, if a pregnant woman complains of dyspnea, it is crucial to perform a pulse oximetry test, and imaging studies should be considered.

    Download PDF (2328K)
feedback
Top