Hideo Matsui

Risk factors associated with altered fetal growth in patients with pregestational diabetes mellitus.

Objective: To assess the risk factors for abnormal fetal growth in patients with pregestational diabetic mellitus (DM). Methods: A retrospective study was performed in 336 patients with pregestational DM. Small-for-gestational-age (SGA) and large-for-gestational-age (LGA) infants were defined as newborns with birth weights < 10th percentile and > 90th percentile, respectively. Logistic regression analysis was performed to identify risk factors for SGA and LGA. Results: Multivariate analysis of the patients with pregestational DM revealed a significant difference between patients who delivered SGA and appropriate-for-gestational-age (AGA) infants in terms of retinopathy (OR = 5.73, 95%CI = 1.39–23.59) and hemoglobin A1C (HbA1C) before delivery (OR = 0.80, 95%CI = 0.68 – 0.94, with a 0.1% increase in DCCT unit). Multivariate analysis revealed a significant difference between patients who delivered LGA and AGA infants in terms of primipara (OR = 3.40, 95%CI = 1.47–7.87) and HbA1C before delivery (OR = 1.14, 95%CI = 1.07–1.21, with a 0.1% increase in DCCT unit). Conclusions: HbA1C before delivery influenced both SGA and LGA infants in patients with pregestational DM. Tight glycemic control might be harmful to fetal growth in pregestational diabetic patients, especially when complicated with retinopathy.

Agranulocytosis associated with intravenous ritodrine hydrochloride therapy: Two case reports by different mechanisms

Ritodrine hydrochloride has been widely used for tocolysis, although serious side‐effects have been reported. We report two cases of agranulocytosis induced by ritodrine hydrochloride, which probably occurred by different mechanisms. Two patients were hospitalized because of preterm labor and were given intravenous ritodrine hydrochloride. The nadir of neutrocytes was 199/mm3 and 13/mm3 in the two cases, respectively. The total dose of ritodrine hydrochloride was calculated to be 7800 mg for 26 days and 2500 mg for 22 days, respectively. The total doses were heavier and administration duration was longer in Case 1, which suggested a toxic mechanism of agranulocytosis, while in Case 2, they were smaller and shorter, suggesting an immunological mechanism. For patients receiving ritodrine hydrochloride, the white blood cell count should be checked frequently regardless of the duration of therapy and a drug lymphocyte stimulation test should be performed.

Sexual Satisfaction of infertile couples assessed using the Golombok-Rust Inventory of Sexual Satisfaction (GRISS)

Recently, infertility treatment-related psychological effects are receiving increased attention. However, whether sexual satisfaction is reduced amongst infertile couples remains to be elucidated. In this study, sexual satisfaction of Japanese infertile couples was assessed using a validated questionnaire designed to assess the male and female partner individually, and the couple as a whole for the first time. This study randomly included 170 infertile couples seen at the outpatient clinic and 170 couples that had recently achieved spontaneous pregnancy. All couples were given the Japanese version of the Golombok-Rust Inventory of Sexual Satisfaction (GRISS). In couples aged 35 years or older, the male partners showed significantly worse sexual satisfaction scores than the female partners. Sexual satisfaction also deteriorated with therapeutic interventions, with mental factors affected more than physical factors. Therapeutic interventions such as timed sexual intercourse and assisted reproductive technology were considered emotionally stressful for infertile couples, with sexual satisfaction accordingly lower in this group than in couples achieving spontaneous pregnancy. GRISS successfully evaluated lower sexual satisfaction associated with infertility, and hence is a useful tool for identifying couples whose sexual satisfaction could be enhanced by counselling or other stress-reduction modalities.

Primary amenorrhea due to juvenile granulosa-cell tumor of the ovary: A case report

In general, primary amenorrhea is caused by gonadal dysgenesis, anomalies of internal or external genitalia with or without chromosomal anomalies, and sometimes by hormonal abnormalities that affect the hypothalamus, pituitary, ovaries, adrenals or thyroid, or by chronic or metabolic diseases. We report a rare case of a juvenile granulosa‐cell tumor of the ovary that caused primary amenorrhea in a 16‐year‐old girl. Her hormonal profiles before the operation were characterized by an extremely low level of follicle‐stimulating hormone (FSH), a relatively low level of estradiol and a high level of inhibin B. The patient had menarche after the removal of the tumor. Her elevated serum FSH after the operation was the result of a decreased serum level of inhibin that had been produced by the tumor. The present case highlights that a granulosa‐cell tumor, known as an inhibin‐secreting tumor, should be considered when treating primary amenorrheic girls.

Enhanced expression of myogenic differentiation factors and skeletal muscle proteins in human amnion-derived cells via the forced expression of MYOD1

OBJECTIVES Mesenchymal stem cells are expected to be an ideal cell source for cellular and gene therapy. We previously showed that cells derived from the human placenta can be induced to differentiate into myotubes in vitro and to express dystrophin in mdx/scid mice in vivo. In this study, we examined whether amnion-derived cells can be efficiently transduced and differentiated using lentiviral vectors carrying human MYOD1. METHODS The amnion-derived cells were isolated from human preterm placentas. They were transduced with the MYOD1 vector, and mRNA levels for MYOD1, MYF5, MYOG, MYH2 and DMD were determined by quantitative-reverse transcriptase-polymerase chain reaction, and also examined immunocytochemically. RESULTS Approximately 70% of amnion-derived cells were efficiently transduced by the lentiviral vectors. MYOD1 activates MYF5 and MYOG, MYH2 and DMD after a 7-day culture. The concerted upregulations of these myogenic regulatory factors enhanced MYH2 and DMD expressions. PAX7 was below the detectable level. Both myosin heavy chain and dystrophin were demonstrated by immunocytochemistry. CONCLUSIONS MYOD1 activates MYF5 and MYOG, the transcription factor genes essential for myogenic differentiation, and the concerted upregulation of these myogenic regulatory factors enhanced MYH2 and DMD expressions. The amniotic membrane is an immune-privileged tissue, making MYOD1-transduced amnion-derived cells an ideal cell source for cellular and gene therapy for muscle disorders. This is the first report showing that amnion-derived cells can be modified by exogenous genes using lentiviral vectors. Furthermore, MYOD1-transduced amnion-derived cells are capable of the dystrophin expression necessary for myogenic differentiation.

Prenatal diagnosis of osteogenesis imperfecta type II by three‐dimensional computed tomography: The current state of fetal computed tomography

We report a case of osteogenesis imperfecta (OI) (OMIM166210) type II, in which a prenatal diagnosis was made by three‐dimensional computed tomography (3D‐CT). Subsequent molecular analysis revealed a recurrent, heterozygous mutation in COL1A2. Fetal CT is a powerful tool for visualizing the fetal skeleton and can provide a definitive diagnosis of fetal skeletal dysplasias; however, whether or not its employment for prenatal diagnosis is warranted in terms of fetal radiation risks remains controversial, both medically and ethically. Based on our experience, we review the current state of fetal CT for the diagnosis of skeletal dysplasias, with a discussion of the relevant literature.

Risk factors associated with preterm delivery in women with cardiac disease

BACKGROUND The purpose of this study was to identify clinical characteristics of preterm delivery at less than 37 weeks of gestation (PD37G) and prenatal events associated with preterm delivery at less than 35 weeks of gestation (PD35G) in women with cardiac disease (WCD). METHODS A case-control study was conducted of 599 pregnancies in 479 single pregnant women with congenital or acquired cardiac lesions or cardiac arrhythmias. The relevant variables were compared between women who had PD35G (n=37) and the controls (n=562). Cardiac dysfunction was defined as the appearance of clinical symptoms of heart failure, abnormal electrocardiogram, or cardiac ultrasonography. RESULTS PD37G occurred in 77 cases (12.9%). The spontaneous and indicated preterm delivery was 26 (33.8%) and 51 (66.2%) cases, respectively. The presence of cardiac dysfunction [odds ratio (OR) 21.82, 95% confidence interval (CI) 8.3-57.49], New York Heart Association class II (OR 3.96, 95% CI 1.05-14.93), cardiomyopathy (OR 7.74, 95% CI 1.69-35.45) and pregnancy-induced hypertension (PIH) (OR 3.15, 95% CI 1.37-7.24) was significantly associated with an increased risk of PD35G. No maternal death was seen within one year after delivery. CONCLUSIONS Although pregnancy and delivery are generally safe in WCD, it is necessary to be aware of the risk factors of cardiac dysfunction, cardiomyopathy, and PIH from the aspect of PD35G.

Levels of soluble endothelial selectin in umbilical cord serum are influenced by gestational age and histological chorioamnionitis, but not by pre-eclampsia: sE-selectin elevates with gestational age

Aims:  The aim of this study was to examine the factors that influence soluble endothelial selectin (sE‐selectin) levels in umbilical cord serum.

A case of rapidly-growing atypical polypoid adenomyoma which was histologically diagnosed before operation and removed by a laparoscopic resection

OBJECTIVE Atypical polypoid adenomyoma (APAM) is an epithelial-mesenchymal mixed tumor which often develops in the uterine cavity of reproductive age women, requiring preservation of the reproductive functions. Preoperative endometrial biopsy may not yield histological diagnosis as the tumor is a solid smooth muscle tumor. The standard treatment option is a hysteroscopic resection for the diagnosis and the treatment at the same time. CASE REPORT We report a case of rapidly-growing APAM successfully diagnosed preoperatively via transcervical punch biopsy followed by a laparoscopic resection. The mass was relatively large, had been located in the lower segment of the uterus, and the area of contact with the muscular layers was large. It was a complete removal and no recurrence had been observed 9 months after the operation. CONCLUSION This is the first report of APAM treated by laparoscopic resection. The method may be a useful alternative when hysteroscopic surgery is inappropriate.

Gorlin syndrome with an ovarian leiomyoma associated with a PTCH1 second hit

We describe a Gorlin syndrome (GS) case with two different second hit mutations of PTCH1, one in a keratocystic odontogenic tumor (KCOT) and the other in an ovarian leiomyoma. GS is a rare genetic condition manifesting as multiple basal cell nevi associated with other features such as medulloblastomas, skeletal abnormalities, and ovarian fibromas. A 21‐year‐old Japanese woman with a history of two KCOTs was diagnosed with GS according to clinical criteria. A PTCH1 mutation, c.1427del T, was detected in peripheral blood. A novel PTCH1 mutation, c.264_265insAATA, had been found in the maxillary KCOT as a second hit mutation. More recently, the ovarian tumor was detected during a gynecological examination. Laparoscopic adnexectomy was performed, and the pathological diagnosis of the ovarian tumor was leiomyoma. Interestingly, another novel mutation, loss of heterozygosity spanning from 9q22.32 to 9q31.2, including PTCH1 and 89 other genes, was detected in this ovarian tumor, providing evidence of a second hit mutation. This is the first report describing a GS‐associated ovarian tumor carrying a second hit in the PTCH1 region. We anticipate that accumulation of more cases will clarify the importance of second hit mutations in ovarian tumor formation in GS.