Multilevel Airway Stenosis Being Bypassed by a Customized Tracheostomy Tube in an Infant with Myhre Syndrome.

Background: Myhre syndrome is a rare connective tissue disorder caused by heterozygous pathogenic variants in the SMAD4 gene. Although recognizing Myhre syndrome in early childhood is challenging, it is important to manage airway stenosis in patients with Myhre syndrome. Case Presentation: We report the case of a 2-month-old boy who initially presented with severe multilevel airway stenosis, dysmorphic face, and multiple abnormalities. Lung fibrosis and mild aortic valve stenosis were additionally observed on follow-up examinations. A heterozygous missense variant, c.1499T>C (p.Ile500Thr), in SMAD4 was identified through exome sequencing. Tracheostomy was performed, and the patient has maintained stable respiration through a customized tracheostomy tube with a home ventilator. Conclusions: Patients who have dysmorphic face, airway stenosis, and cardiovascular anomalies that do not fit the diagnosis of common syndromes should be evaluated for rare diseases, including Myhre syndrome. Since respiratory complications can be life threatening, early diagnosis and suitable intervention are necessary.

Short-term efficacy of 1-month and 3-month gonadotropin-releasing hormone agonist depots in girls with central precocious puberty.

PURPOSE: Gonadotropin-releasing hormone agonist (GnRHa) has been the mainstay of central precocious puberty (CPP) treatment for decades, but few reports have compared the efficacy of 1-month and 3-month depot GnRHa formulations. This study investigates the short-term efficacy of 1-month and 3-month GnRHa depots in girls with CPP. METHODS: Overall, 150 girls with CPP were included in a retrospective review of medical records. Subjects in group 1 (n=105) were treated with 1-month GnRHa depots for ≥12 months, and those in group 2 (n=45) were treated with 1-month GnRHa depots for 6 months followed by 3-month GnRHa depots for ≥6 months. Anthropometric and biochemical data were compared between the groups at 3-time points (after 0, 6, and 12 months of GnRHa treatment). RESULTS: Demographic and clinical characteristics did not differ between the groups at baseline or after 6 months of GnRHa treatment. After 12 months of GnRHa treatment, patients in the both groups showed no difference in bone age (BA), chronological age (CA), BA-CA difference, height standard deviation score (SDS) for CA and BA, or body mass index SDS for CA and BA. The sexual maturity rate of the breast was prepubertal at 12 months in most of subjects. GnRH-stimulated luteinizing hormone (LH) level was suppressed during GnRHa treatment in both groups at 6 and 12 months, although the LH level in group 2 was higher than that in group 1. CONCLUSION: Treating CPP with a 3-month GnRHa depot showed short-term efficacy comparable to that with a 1-month depot in anthropometric parameters and pubertal suppression.

Bactericidal and synergistic effects of X-ray irradiation and gallic acid against foodborne pathogens on lettuce.

The objectives of this study were to evaluate the bactericidal effects of X-ray irradiation and gallic acid (GA) against Escherichia coli O157:H7, Salmonella Typhimurium, and Listeria monocytogenes on lettuce leaves and in phosphate-buffered saline (PBS). Inoculated PBS and lettuce were exposed to X-rays (0.05, 0.1, and 0.15; 0.1, 0.2, and 0.3 kGy, respectively), and GA was applied to lettuce leaves as a solution and in PBS at concentrations of 0.5% (w/v). Combined treatment with 0.3 kGy and 0.5% GA reduced E. coli O157:H7, S. Typhimurium, and L. monocytogenes cell counts 5.41, 2.57, and 1.36 log CFU/cm2 on lettuce, respectively. Combined treatment with 0.15 kGy X-ray and 0.5% GA reduced counts for the same species by 6.54, 4.24, and 1.51 log CFU/mL in PBS. The combined treatments exerted a synergistic antibacterial effect against E. coli O157:H7 on lettuce, but not against S. Typhimurium or L. monocytogenes. In PBS, the synergistic effect was confirmed in both E. coli O157:H7 and S. Typhimurium cells. Mechanistic investigations indicated that the synergistic antibacterial effect was associated with intracellular reactive oxygen species (ROS) generation and bacterial cell membrane damage. Additionally, the X-ray and GA combination treatment did not adversely affect the color, total phenol content, and texture of lettuce. These findings demonstrate that treatment with X-ray radiation and GA can enhance the microbiological safety of fresh produce.

Synergistic bactericidal effect and mechanism of X-ray irradiation and citric acid combination against food-borne pathogens on spinach leaves.

In this study, we investigated the antimicrobial activity of the X-ray irradiation and citric acid (CA) combination against Escherichia coli O157:H7 and Listeria monocytogenes on the surface of spinach leaves and elucidated the mechanisms underlying their synergistic interaction. Upon treatment with 0.3 kGy X-ray irradiation and 1% CA combination, the cell counts of E. coli O157:H7 and L. monocytogenes reduced by 4.23 and 3.69 log CFU/mL on spinach leaves, respectively. The synergistic reduction in the cell counts of E. coli O157:H7 and L. monocytogenes by the combination treatment was 0.95 and 1.14 log units, respectively. The X-ray and CA combination exerts its antimicrobial effect by damaging the bacterial cell membrane and enhancing the generation of intracellular reactive oxygen species in the pathogens. The enhanced bactericidal effect of the combination treatment may not be due to the loss of intracellular enzyme activity. We also evaluated the effect of the combination treatment on the quality attributes of spinach leaves. The combination treatment did not result in adverse changes in color and texture of spinach leaves. These results demonstrate the potential of citric acid and X-ray irradiation combination for decontaminating foodborne pathogens on fresh produce.

Inactivating foodborne pathogens in apple juice by combined treatment with fumaric acid and ultraviolet-A light, and mechanisms of their synergistic bactericidal action.

We evaluated the bactericidal efficacy of the simultaneous application of ultraviolet-A (UV-A) irradiation and fumaric acid (FA) against Escherichia coli O157:H7, Salmonella enterica serovar Typhimurium, and Listeria monocytogenes in apple juice and as well as investigated the effects of this treatment on product quality. Further, we elucidated the mechanisms underlying their synergistic bactericidal action. Simultaneous UV-A light irradiation and 0.1% FA treatment for 30 min resulted in 6.65-, 6.27-, and 6.49-log CFU/ml reductions in E. coli O157:H7, S. Typhimurium, and L. monocytogenes, respectively, which involved 3.15, 2.21, and 3.43 log CFU reductions, respectively, and these were attributed to the synergistic action of the combined treatments. Mechanistic investigations suggested that the combined UVA-FA treatment resulted in significantly greater bacterial cell membrane damage and intracellular reactive oxygen species (ROS) generation. UVA-FA treatment for 30 min did not cause significant changes to the color, nonenzymatic browning index, pH, and total phenolic content of apple juice. These results suggest that combined UVA-FA treatment can be effectively used to control foodborne pathogens in apple juice without affecting its quality.