Isolated Agnathia-Otocephaly Complex Diagnosed Prenatally for Ex-Utero Intrapartum Treatment: A Case Report.

BACKGROUND Agnathia-otocephaly complex (AOC) is a rare congenital malformation due to a first-branch arch disorder and has been considered lethal. However, milder variants of the isolated type of AOC have been reported as nonlethal. The ex-utero intrapartum treatment (EXIT) procedure is basically indicated for a fetus with a high risk of airway obstruction immediately after birth; it is not indicated for all AOC cases but is chosen to treat cases until the airway can be evaluated to achieve a better prognosis. CASE REPORT A 37-year-old woman was referred with reported fetal facial deformity and polyhydramnios at 27 weeks of gestation. Our fetal ultrasound scans showed agnathia, microstomia, and synotia, but not holoprosencephaly. Isolated AOC was diagnosed prenatally. Magnetic resonance imaging and microbubble tests revealed delayed fetal lung maturation, although it was not completely unmatured. With patient agreement, an emergency cesarean section with EXIT was performed because of clinical chorioamnionitis at 35 weeks of gestation. Tracheostomy was attempted for 16 min during EXIT and was completed 4 min after delivery. Despite this, the neonate died 12 h after delivery from severe respiratory failure and a tension pneumothorax caused by a hypoplastic lung. CONCLUSIONS There is controversy surrounding the non-lethality of all isolated AOC cases and the non-contraindication of EXIT procedures. Our case was estimated as the milder variant, and the EXIT procedure was indicated; however, the neonate died of the hypoplastic lung. The evaluation methods of lung maturation are inconsistent, and the indication of the invasive EXIT procedure must be carefully considered.

Telemedicine for home care of fetal growth restriction with mobile cardiotocography: A case series.

OBJECTIVE: Modern technological advancements have made it possible to perform cardiotocography at home. Home-based management of high-risk pregnancies using a mobile cardiotocography system has been reported; however, its effectiveness in monitoring cases of fetal growth restriction (FGR) remains unclear. Therefore, the authors aimed to investigate the clinical usefulness of home-based telemedicine for FGR management using the mobile cardiotocography (iCTG). METHODS: The authors conducted a single-center, retrospective case series of patients with FGR. Seventeen women diagnosed with FGR were enrolled. Patients performed iCTG for 1 hour twice daily to examine their fetuses; data were uploaded and saved on the cloud. RESULTS: The median and minimum compliance rates were 93.33 (interquartile range [IQR], 70.00-100.00) and 40.7, respectively. The median and minimum validity rates were 100.00 (IQR, 90.48-100.00) and 36.4, respectively. In this study, many of the patients were managed at home and underwent delivery as planned. However, three patients required emergency visits; one had a nonreassuring fetal status and underwent an emergency cesarean section. CONCLUSION: Even when the fetal prognosis is good, careful pre-evaluation is required before initiating home care management. The current study shows that the economic burden of hospitalization for patients can be reduced by using iCTG.

Open Surgery including Lymphadenectomy without Adjuvant Therapy for Uterine-Confined Intermediate- and High-Risk Endometrioid Endometrial Carcinoma.

Minimally invasive surgery may not be an appropriate surgical approach in intermediate- and high-risk endometrial carcinoma, even though adjuvant therapy is given. The objective of this study was to evaluate the results of open surgery including lymphadenectomy without adjuvant therapy in patients with uterine-confined intermediate- and high-risk endometrioid endometrial carcinoma. Two hundred fifty-six patients with uterine-confined endometrioid endometrial carcinoma were treated with open surgery, including pelvic with or without para-aortic lymphadenectomy. Of the 81 patients with uterine-confined intermediate- or high-risk disease, 77 were treated with systematic lymphadenectomy without adjuvant therapy. Seven patients developed recurrence, comprising 5.5% (3/55) and 18.2% (4/22) of the intermediate- and high-risk patients, respectively. The time to recurrence was 1-66 months. The sites of recurrence were the vaginal apex (n = 2), lung (n = 2), vaginal sidewall (n = 1), pelvic lymph nodes (n = 1), and para-aortic to supraclavicular nodes (n = 1). Of these, five patients were alive without disease after salvage treatment, but two understaged high-risk patients died of disease. The five-year disease-specific survival rates of intermediate- and high-risk patients were 100% and 90%, respectively. The present study indicated that patients with uterine-confined intermediate- and high-risk endometrioid endometrial carcinoma had excellent survival when treated with open surgery, including lymphadenectomy alone. The safety of omitting adjuvant therapy should be evaluated in prospective randomized trials comparing open surgery with minimally invasive surgery.

Carbon and nitrogen depletion-induced nucleophagy and selective autophagic sequestration of a whole nucleus in multinucleate cells of the filamentous fungus Aspergillus oryzae.

Autophagy is a conserved cellular degradation process in eukaryotes, in which cytoplasmic components and organelles are digested in vacuoles/lysosomes. Recently, autophagic degradation of nuclear materials, termed "nucleophagy", has been reported. In the multinucleate filamentous fungus Aspergillus oryzae, a whole nucleus is degraded by nucleophagy after prolonged culture. While developing an H2B-EGFP processing assay for the evaluation of nucleophagy in A. oryzae, we found that nucleophagy is efficiently induced by carbon or nitrogen depletion. Microscopic observations in a carbon depletion condition clearly demonstrated that autophagosomes selectively sequester a particular nucleus, despite the presence of multiple nuclei in the same cell. Furthermore, AoNsp1, the A. oryzae homolog of the yeast nucleoporin Nsp1p, mainly localized at the nuclear periphery, but its localization was restricted to the opposite side of the autophagosome being formed around a nucleus. In contrast, the perinuclear ER visualized with the calnexin AoClxA was not morphologically affected by nucleophagy. The findings of nucleophagy-inducing conditions enabled us to characterize the morphological process of autophagic degradation of a whole nucleus in multinucleate cells.

Age-dependent changes in skeletal muscle mass and visceral fat area in Japanese adults from 40 to 79 years-of-age.

AIM: The age-dependent loss of skeletal muscle mass is highly concerning in diverse aging populations. However, age-dependent changes in muscle mass and the visceral fat area have not been well documented in Asian populations. The aim of the present study was to evaluate the age-dependent changes in skeletal muscle mass and the visceral fat area in Japanese adults from 40 to 79 years-of-age. METHODS: This was a cross-sectional study. Healthy men (n = 16,379) and women (n = 21,660) aged 40-79 years participated in the present study. The skeletal muscle mass and visceral fat area were measured in the study participants by bioelectrical impedance. The muscle mass data were converted into the skeletal muscle mass index (SMI) by dividing the weight by the height squared (kg/m(2)). RESULTS: The SMI showed an age-dependent decrease in both sexes. Between 40 and 79 years, the total SMI decreased by 10.8% in men and by 6.4% in women. The arm SMI decreased by 12.6% in men and 4.1% in women, and the leg SMI decreased by 10.1% in men and by 7.1% in women in the same period. In contrast, the visceral fat area showed an age-dependent increase in both sexes. The visceral fat area increased by 42.9% in men and by 65.3% in women. The multiple regression analysis showed that the SMI was negatively associated with visceral obesity in both sexes. CONCLUSIONS: In Japanese adults, sex-specific changes in skeletal muscle mass are more prominent in the arm than in the leg. Furthermore, the age-dependent increases in visceral adipose tissue might lead to loss of skeletal muscle mass.