Dear members,From this year, I was appointed as the editorial chairman as the successor to chairman Satoru Takeda. At the time of publication in 2013, I remember having a lot of discussions with the ...editorial office and struggling to decide on the format and peer review systems.At the beginning, not enough papers were collected, and with the cooperation of the members, the publication of two volumes a year were maintained. Since last year, it has been published only online, and the number of publications has increased to four volumes a year. In this way, thanks to everyone’s help, the significance of the existence of this journal has been established.In 2022, we will feature the guidelines as a special issue, and hope that it will be useful as an opportunity to disseminate the JSSHP guidelines to the world.We are looking forward to many submissions from our members.All the best.
Among the various coagulation-related factors, fibrinogen levels are the first to decrease below critical levels during massive hemorrhage. Therefore, in obstetric hemorrhage, the lost fibrinogen ...should be replaced at an early stage to restore hemostatic blood levels. Recently, the use of fibrinogen concentrates for critical obstetric hemorrhage has been increasing in Japan, and its efficacy in Japanese patients is similar to that reported in patients from other countries. According to the survey conducted by the Japanese Society of Obstetrics and Gynecology on the use of fibrinogen concentrates, a median dose of 3 g is generally administered, and if the effect is inadequate, additional doses are administered. The Japanese Clinical Practice Guide for Critical Obstetrical Hemorrhage (2017 revision) states that blood transfusion should be initiated as soon as possible and recommends the prompt administration of fresh-frozen plasma and red blood cells because obstetric hemorrhage can easily result in disseminated intravascular coagulation. Moreover, the use of fibrinogens is also recommended for severe hemorrhage cases. Evaluation of the efficacy of fibrinogen concentrates in severe cases, such as a placebo-controlled, randomized and double blind study, cannot be carried out because it is unethical. However, since it is clear that fibrinogen concentrates have a high potential for reducing maternal mortality, there is an urgent need to expand the indications for fibrinogen concentrate administration.
Japanese obstetrical hemorrhage recommendations state that not only pregnant women with an obstetrical disseminated intravascular coagulation (DIC) score ≥ 8 points but also those with fibrinogen ...levels ≤ 1.5 g/L have a high risk of maternal death and warrant blood transfusion. Our aim was to demonstrate the potential of fibrinogen levels ≤ 1.5 g/L as predictors of a Japanese obstetrical DIC score of ≥ 8. We included 595 participants with blood loss ≥ 1000 mL during vaginal delivery or ≥ 2000 mL during cesarean delivery. The frequency and volume of red blood cell (RBC), fresh-frozen plasma, platelet concentrate (PC), and fibrinogen administration in women with a DIC score of ≥ 8 and fibrinogen levels of ≤ 1.5 g/L were significantly higher than controls (P < 0.0001). Multivariate analysis demonstrated that a score of ≥ 3 was associated with RBC or fibrinogen administration and a score of ≥ 5 was associated with PC transfusion. Fibrinogen levels ≤ 1.89 g/L and ≤ 2.44 g/L were associated with PC transfusion and fibrinogen administration, respectively. Fibrinogen levels ≤ 1.5 g/L may have similar potential to a DIC score of ≥ 8 points for detecting obstetrical DIC in Japan.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
TET3 at 2p13.1 encodes tet methylcytosine dioxygenase 3, a demethylation enzyme that converts 5-methylcytosine to 5-hydroxymethylcytosine. Beck et al. reported that patients with TET3 abnormalities ...in either an autosomal dominant or recessive inheritance fashion clinically showed global developmental delay, intellectual disability, and dysmorphisms. In this study, exome sequencing identified both mono- and biallelic TET3 variants in two families: a de novo variant NM_001287491.1:c.3028 A > G:p.(Asn1010Asp), and compound heterozygous variants NM_001287491.1:c.2077 C > T;2896 T > G,p.Gln693*;Cys966Gly. Despite the different inheritance modes, the affected individuals showed similar phenotypic features. Including these three patients, only 14 affected individuals have been reported to date. The accumulation of data regarding individuals with TET3-related disorder is necessary to describe their clinical spectrum.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Since July 2022, obstetrical disseminated intravascular coagulation (DIC) in Japan has been diagnosed based on the new criteria (tentative version), which assesses the main underlying disease, ...fibrinogen level, and fibrin/fibrinogen degradation products or D-dimer level. In June 2024, the tentative version underwent minor revision and the final version was released. The previous Japanese criteria assessed underlying disease, clinical symptoms, and various laboratory findings. This study aimed to prove the effectiveness, reliability, and validity of the new criteria (final version). We analyzed 212 women with singleton pregnancies who delivered after 22 gestational weeks and experienced blood loss ≥ 1000 mL during vaginal delivery or ≥ 2000 mL during cesarean section. Those with missing laboratory findings before receiving blood transfusion at delivery were excluded. In the obstetrical DIC group, the frequency of fibrinogen levels < 150 mg/dL was significantly higher than in the control group (90% vs. 5%, p < 0.0001), as was the frequency of scores ≥ 8 according to the previous Japanese criteria (100% vs. 10%, p < 0.0001). Cronbach alpha was 0.757 and Spearman’s rank-order correlation was 0.558 between the new and previous criteria. In conclusion, we proved the effectiveness, reliability, and validity of the Japanese new criteria (final version) to diagnose obstetrical DIC.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
TNNI2 at 11p15.5 encodes troponin I2, fast skeletal type, which is a member of the troponin I gene family and a component of the troponin complex. Distal arthrogryposis (DA) is characterized by ...congenital limb contractures without primary neurological or muscular effects. DA is inherited in an autosomal dominant fashion and is clinically and genetically heterogeneous. Exome sequencing identified a causative variant in TNNI2 NM_003282.4:c.532T>C p.(Phe178Leu) in a Japanese girl with typical DA2b. Interestingly, the familial study using Sanger sequencing suggested a mosaic variant in her healthy father. Subsequent targeted amplicon-based deep sequencing detected the TNNI2 variant with variant allele frequencies of 9.4-17.7% in genomic DNA derived from peripheral blood leukocytes, saliva, hair, and nails in the father. We confirmed a disease-causing variant in TNNI2 in the proband inherited from her asymptomatic father with its somatic variant. Our case demonstrates that careful clinical and genetic evaluation is required in DA.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
The objective of this study was to evaluate the efficacy of whole exome sequencing (WES) for the genetic diagnosis of cases presenting with fetal structural anomalies detected by ultrasonography. WES ...was performed on 19 cases with prenatal structural anomalies. Genomic DNA was extracted from umbilical cords or umbilical blood obtained shortly after birth. WES data were analyzed on prenatal phenotypes alone, and the data were re-analyzed after information regarding the postnatal phenotype was obtained. Based solely on the fetal phenotype, pathogenic, or likely pathogenic, single nucleotide variants were identified in 5 of 19 (26.3%) cases. Moreover, we detected trisomy 21 in two cases by WES-based copy number variation analysis. The overall diagnostic rate was 36.8% (7/19). They were all compatible with respective fetal structural anomalies. By referring to postnatal phenotype information, another candidate variant was identified by a postnatal clinical feature that was not detected in prenatal screening. As detailed phenotyping is desirable for better diagnostic rates in WES analysis, we should be aware that fetal phenotype is a useful, but sometimes limited source of information for comprehensive genetic analysis. It is important to amass more data of genotype-phenotype correlations, especially to appropriately assess the validity of WES in prenatal settings.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
This study was to investigate the employment status of pregnant women in Japan and identify risk factors associated with resigning from work during pregnancy. We conducted a cross-sectional survey ...with 975 postpartum women in Tokyo and its suburbs, focusing on those who were employed during pregnancy. Women who were employed when they became pregnant were selected and divided into two groups: those who did not resign and those who resigned; the groups were analyzed separately. Multiple logistic regression analyses were performed to examine associations between employment resignation and risk factors. The analysis revealed that 79% continued working, while 8.1% resigned. Risk factors for resignation included non-regular employment (OR: 13.1, 95% CI: 6.6–25.9), fewer employees (OR: 3.4, 95% CI: 1.8–6.4), and shorter employee tenure (OR: 2.4, 95% CI: 1.1–5.2). Non-regular employment status, a smaller number of employees, and shorter employee tenure were identified as risk factors for working women resigning from their job during pregnancy. In work situations and work environments that encourage pregnant women to leave the workforce, developing systems to improve these conditions for pregnant employees may help women to progress in the labor force.
This study aimed to clarify the effectiveness of cervical cerclage in preventing recurrent preterm births.
A retrospective study was conducted using the perinatal registration database of the Japan ...Society of Obstetrics and Gynecology for the Perinatal Center from 2014 to 2016. The efficacies of history-indicated, ultrasound-indicated, and physical examination-indicated cerclage as preventive treatments for women with a history of preterm birth were evaluated by comparing cerclage and non-cerclage cases. Fisher's exact test was performed to evaluate any significant differences in patient backgrounds. Prior to the evaluation, propensity score matching was performed for history-indicated and ultrasound-indicated cerclage patients.
For this study, 6060 multiparous women with a history of preterm birth were reviewed. After excluding 17 patients with unknown indications for cervical cerclage, 6043 patients were included in the study. History-indicated and ultrasound-indicated cerclage did not reduce the risk of preterm birth in subsequent pregnancies for any of the pregnancy periods (p = 0.413, p = 1.000). In contrast, physical examination-indicated cerclage significantly reduced the risk of subsequent preterm births for all pregnancy periods (p < 0.001).
Subsequent preterm births were effectively prevented only in physical examination-indicated cerclage cases. For history- or ultrasound-indicated cerclage, statistically significant differences in subsequent preterm births were not evident.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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