Prenatal high-dose immunoglobulin treatment for neonatal hemochromatosis: a case report and review of the literature

Neonatal hemochromatosis is a difficult disorder to cure, and it has a high rate of recurrence. High-dose immunoglobulin treatment is very effective as prenatal treatment for recurrent neonatal hemochromatosis. A 34-year-old pregnant Japanese woman underwent high-dose immunoglobulin treatment for recurrent neonatal hemochromatosis. High-dose non-specific intravenous immunoglobulin (1 g/kg bodyweight) was administered to the mother intravenously every week from 18 until 36 gestational weeks. A male infant was delivered at 37 weeks of gestation, and his condition was favorable, including hepatic function. The use of γ-globulin for neonatal hemochromatosis appears adequately validated by experience.     

Reliability of the lung to thorax transverse area ratio as a predictive parameter in fetuses with congenital diaphragmatic hernia

Purpose

An accurate prenatal assessment of the patients?? severity is essential for the optimal treatment of individuals with congenital diaphragmatic hernia (CDH). The purpose of this study was to clarify the reliability of the lung to thorax transverse area ratio (L/T) as a prenatal predictive parameter.

Methods

A multicenter retrospective cohort study was conducted on 114 isolated CDH fetuses with a prenatal diagnosis during the period between 2002 and 2007 at five participating centers in Japan. The relationship between the gestational age and the L/T was analyzed. The most powerful measurement point and accurate cutoff value of the L/T was determined by an analysis of a receiver operating characteristic curve, which was verified by comparing the patients?? severity.

Results

There was a negative correlation between the gestational age and the L/T in the non-survivors, and no correlation in the survivors. There were significant differences in the parameters which represented the patients?? severity including the respiratory and circulatory status, the surgical findings, and the final outcomes between the groups divided at 0.080 in the minimum value of the L/T during gestation.

Conclusion

The L/T was not strongly influenced by the gestational age, and it was found to be a reliable prenatal predictive parameter in fetuses with isolated CDH.     

Occupational safety and health in Japan: current situations and the future

The Industrial Safety and Health Law enacted in 1972 has contributed much to the progress of occupational safety and health (OSH) activities. Many indicators including death and illness statistics show continued improvement up to date. The establishment of OSH organization within enterprises and 5-yr administrative programs formulated by the Ministry of Health, Labour, and Welfare (MHLW) were important factors for satisfactory management. The past programs indicate that the weight of self regulation in comparison to legal control gradually increased since late 1990s. In spite of the past achievement, many hazards such as overwork, mental stress, chemical agents and others still remain to be prevented. The systematic risk assessment of unregulated chemicals by the MHLW proved to be an effective scheme for risk-based management and to deserve continued implementation. The size of human resources for OSH was estimated at 1.5 million. In view of the adverse effect on OSH by economic, social and political environment in the future, the importance of the efficiency of OSH management was indicated. Since the efficiency depends on the competence of OSH personnel and the level of scientific basis, it was concluded that the fundamental policy for the future should give high priority to education and research.     

Down syndrome live births following assisted reproductive technology in Japan: a nationwide survey between 2007 and 2016

Journal of Assisted Reproduction and Genetics - The use of assisted reproductive technology (ART) and prenatal genetic testing have become more widespread in line with an increase in maternal age....     

Stimulatory effect of pituitary adenylate-cyclase activating polypeptide (PACAP) and its PACAP type I receptor (PAC1R) on prolactin synthesis in rat pituitary somatolactotroph GH3 cells

In this present study, we investigated the role of pituitary adenylate cyclase-activating polypeptide (PACAP) and its receptor, PACAP type I receptor (PAC1R) on prolactin synthesis in pituitary somatolactotroph GH3 cells. PACAP increased prolactin promoter activity up to 1.3 ± 0.1-fold. This increase, while significant, was less than the increase resulting from thyrotropin-releasing hormone (TRH) stimulation. By transfection of a PAC1R expression vector to the cells, the response to PACAP on prolactin promoter activity was dramatically potentiated to a degree proportional to the amount of PAC1R transfected. In the PAC1R expressing GH3 cells, TRH and PACAP alone increased prolactin promoter up to 3.3 ± 0.3-fold and 4.9 ± 0.2-fold, respectively, and combined treatment with TRH and PACAP further increased prolactin promoters up to 6.8 ± 0.6-fold. PACAP binds both Gs- and Gq-coupled receptors and stimulates adenylate cyclase/cAMP and protein kinase C/extracellular signal-regulated kinase (ERK) signaling pathways. PACAP increased ERK phosphorylation in PAC1R expressing cells to the same degree as TRH. Combined treatment with TRH and PACAP had a synergistic effect on ERK activation. GH3 cells produce both prolactin and growth hormone. Stimulation of GH3 cells with TRH significantly increased the mRNA level of prolactin and attenuated growth hormone mRNA expression. PACAP increased both prolactin and growth hormone mRNA levels, particularly in PAC1R expressing cells. In addition, increasing amount of PAC1R in GH3 cells potentiated the action of TRH on prolactin promoter activity, as well as on ERK phosphorylation. PAC1R was induced by PACAP itself, but not by TRH. Our current study demonstrates that PACAP and its PAC1R, functions as a stimulator of prolactin alone or with TRH in prolactin producing cells.     

Correlation between endocytoscopy and conventional histopathology in microstructural features of ulcerative colitis

Background  

Routine diagnosis of the histopathological activity of ulcerative colitis (UC) requires multiple biopsy samples, and an endocytoscopy system (ECS) provides real-time ultra-magnifying microscopic imaging in vivo.     

Utility of contrast-enhanced ultrasonography with Sonazoid in radiofrequency ablation for hepatocellular carcinoma

Background and Aims: Kupffer imaging in contrast‐enhanced ultrasonography (CEUS) with Sonazoid, which lasts for 60 min or longer, may be useful in ultrasound‐guided percutaneous tumor ablation. The utility of Sonazoid in radiofrequency ablation (RFA) of hepatocellular carcinoma (HCC) was investigated in this study. Methods: We analyzed a total of 716 HCC nodules that were detected on dynamic computed tomography in 316 patients. Detectability of these nodules was compared between CEUS and conventional ultrasonography. The effectiveness in the treatment was assessed by comparing the mean numbers of treatment sessions of RFA in patients treated with CEUS and that in historical controls matched for tumor and background conditions. Results: Detectability of tumor nodule was 83.5% in conventional ultrasonography and 93.2% in CEUS (P = 0.04). Sixty‐nine nodules in 52 patients were additionally detected with CEUS. The number of additionally detected tumor nodules was positively correlated with serum albumin level (P = 0.016). The number of RFA sessions was 1.33 ± 0.45 with CEUS as compared to 1.49 ± 0.76 in the historical controls (P = 0.0019). Conclusions: CEUS with Sonazoid is useful for HCC detection in patients with a well‐conserved liver function reservoir. The decrease in RFA session numbers indicated the utility of Sonazoid in RFA treatment of HCC.     

A case of sinobronchial allergic mycosis; possibility of basidiomycetous fungi as a causative antigen

We herein report a case of sinobronchial allergic mycosis (SAM) caused by basidiomycetous (BM) fungi (probably Phanerochaete velutina). The patient with bronchial asthma that accompanied allergic fungal sinusitis (AFS) fulfilled all 6 criteria for diagnosing SAM. In this case, the BM fungus may act as an allergen, reacting continually in both the upper and lower respiratory tract. The antifungal drug (itraconazole 50 mg/day) seemed to achieve a partial response. Basidiomycetous fungi may attract attention because of the possibility as a causative antigen in this new clinical concept of SAM.