Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.
Anesthesia, General ; Child ; Child, Preschool ; Chromosome Deletion ; Female ; Genetic Diseases, Inborn ; Heart Defects, Congenital ; Heart Diseases ; Hemodynamics ; Humans ; Intellectual Disability ; Intubation, Intratracheal ; Seizures ; Wolf-Hirschhorn Syndrome*

Objective: The purpose of this study is to identify the problems in the adverse drug report (ADR) system in early post-marketing vigilance phase (EPM phase) in Japan.
Methods: The incidence of all ADRs and the ratio of serious ADRs were compared between the new drug application phase (ND phase) and the EPM phase.  The target medicines were Moxifloxacin (Avelox®tablets, 400mg), Gatifloxacin (Gatiflo®tablets, 100mg) and Prulifloxacin (Sword®tablets, 100mg).
Results: The average incidence of all ADRs in the ND phase was 100-fold greater than that in the EPM phase.  There were also 2-fold differences in the ratio of serious ADRs of individual medicines.
Conclusion: There are several problems with the ADR system in the EPM phase in Japan.  It is currently possible that the implementation of EPM will vary between in individual medicines and companies.  This suggests that the present data cannot be applied universally.  Thus, there is an urgent need to standardize the implementation of EPM.

BACKGROUND/AIMS: Self-expandable metallic stent (SEMS) placement is effective for dysphagia that results from malignant obstruction of the esophagus or gastric cardia; however, stent-related complications may be life-threatening. Thus, the goal of this study was to identify risk factors associated with complications following esophageal stenting. METHODS: Of the 71 patients who underwent SEMS placement for dysphagia as a result of malignant stricture of the esophagus or gastric cardia, 53 patients with squamous cell carcinoma or adenocarcinoma, without previous SEMS placement, without a fistula, and without recurrent tumor after surgery were retrospectively identified. The occurrence of stent-related complications was used as an endpoint. RESULTS: Stent-related complications were identified in 26 patients (49.1%), and major complications occurred in 14 patients (26.4%). The use of an Ultraflex stent (odds ratio [OR], 6.81; 95% confidence interval [CI], 1.54 to 30.00; p=0.011) and prior chemotherapy (OR, 6.13; 95% CI, 1.46 to 25.70; p=0.013) were significantly associated with stent-related complications. Moreover, the use of an Ultraflex stent (OR, 19.60; 95% CI, 2.26 to 170.00; p=0.007) and prior radiation (OR, 25.70; 95% CI, 2.37 to 280.00; p=0.008) significantly increased the risk of major complications. CONCLUSIONS: The use of an Ultraflex stent and prior radiation and/or chemotherapy may represent risk factors for complications following esophageal SEMS placement.
Adenocarcinoma ; Carcinoma, Squamous Cell ; Cardia* ; Constriction, Pathologic ; Deglutition Disorders ; Drug Therapy ; Esophagus* ; Fistula ; Humans ; Retrospective Studies ; Risk Factors ; Stents

Objective: The internet isflooded with drug information; however, some of it isinappropriate and thisinadequate information could expose the public to health hazards. Therefore, we conducted research on the idea of transmitting drug information to the public via the internet and the current state of the information currently provided by each academic society relevant to the field of Medical Informatics.Methods: A questionnaire was mailed to the website managers for the website of 129 specialist medical societies, all members of the Japanese Association of Medical Sciences. We conducted our research between October to November 2018. We investigated each website administrator’s opinion about offering drug information for consumers via the internet and what information each academic society is currently providing.Results: The effective response rate was 43.4% (56/129 groups). Most respondents thought that drug information overflowed in the current Internet society. Moreover, more than half of the respondents thought that the quality of drug information transmitted to the public wasinadequate. Currently, 30 of the academic groupssurveyed are providing information to the public. When providing information, they did not refer to the “Drug Guide for Patients” much. More than 80% of respondents said they would cooperate with linking to the information provided to the public. However, each academic society felt there would be many problems with doing so such as the need for a system to check the contents of the information provided and a system to perform maintenance.Conclusion: The results showed that the website administrators recognized that there is a need to improve the quality of and system for providing drug information to the public. We believe that an integrated information system can be constructed by aggregating the drug information held by each academic society. However, this cannot be realized without first solving many problems.

Objective: We have developed an automatic vigilance system (AVS) that automatically reports adverse drug reactions (ADR) based on laboratory finding abnormalities and symptom keywords in electronic medical records.　In this study, we aimed to evaluate the impact of detecting ADR using AVS on medical treatment.Methods: In AVS, drugs and their ADR signals, which would be detected and reported by AVS to pharmacists, were defined.　Pharmacists evaluated the severity of these signals to identify whether these signals should be discussed with the doctor, continued to be followed up, or ignored.　We investigated detection of ADR at University of Fukui Hospital between April 2016 and March 2017 along with whether prescriptions were modified because of ADR and the contribution of AVS.　Assuming that ADR had worsened without appropriate treatment, medical expenses needed for treating severe ADR were calculated.Results: In total, 325 signals were defined for 146 drugs.　There were 9,103 ADR signals confirmed by pharmacists for 8,531 subjects.　Of these, 12 and 164 signals were discussed with the doctor and continuously observed, respectively.　The pharmacist's suggestions based on AVS led to prescription modifications in 10 cases, corresponding to a reduction of 2.56 million yen in medical expenses in the event that these cases become severe.Conclusion: AVS assisted prescription revisions because of ADR and is thought to contribute to the prevention of worsening of ADR and reduction of medical expenses.

Objective: In this study, we investigated the occurrence of skin damage following the initiation of low-dose lamotrigine.Methods: We retrospectively analyzed the incidence of skin disorders within 8 weeks of the start of lamotrigine administration, prescribing for 3 years from July 2014 to June 2016.In addition, we also confirmed the onset time of skin disorders in the low- and normal-dose groups.Results: The incidence of skin damage was 7.7 and 24.6 % in the low- and normal-dose lamotrigine start groups, respectively. The onset of skin disorders was relatively early in the normal-dose lamotrigine start group.On the other hand, no tendency was found in the low-dose lamotrigine start group because the number of cases was small.Conclusion: The initiation of low-dose lamotrigine and extension of introduction period might reduce the onset of early skin damage.

Objective: We evaluated patients’ degree of understanding of the effects and adverse drug reactions of SGLT2 inhibitors.Methods: We targeted 26 patients who were administered SGLT2 inhibitors during hospitalizations between April 2017 and March 2018. The survey was conducted by interviewing the patients using a questionnaire.Results: In total, 14 patients (53. 8%) were able to explain the term “efficacy.” Although 6 patients (23. 1%) understood “dehydration,” there was little understanding of “urinary tract infection” (7.7%) and “rash/erythema” (2 and 0 patients, respectively). In addition, we confirmed the details of the descriptions of adverse reactions caused by SGLT2 inhibitors with pharmacists, and found that 13 patients (50.0%) clearly received an explanation of “dehydration,” only 3 patients received an explanation of “urinary tract infection” (11.5%), and none of them comprehended “rash/erythema.” Overall, the patients’ awareness of the adverse drug reactions of SGLT2 inhibitors was low.Conclusion: Unlike common drugs for diabetes, SGLT2 inhibitors have been attracting attention as protective agents of the heart and kidneys. Therefore, it is expected that prescriptions for SGLT2 will increase in the future. Pharmacists need to explain the effects and adverse drug reactions of SGLT2 inhibitors to the patients as well as make the patients understand the pharmacological mechanisms of action of SGLT2 inhibitors.

Objective: Two types of symbols have been established as industry standards in terms of two-dimensional (2D) symbols with prescription information: one for objects to be printed on prescriptions and the other for electronic versions of medication diaries. However, no studies have investigated the system for using 2D symbols in pharmacies and hospitals/clinics as well as the quality of the information actually stored in these 2D symbols. Therefore, we conducted a survey to clarify the current status and problems pertaining to prescription information sharing via 2D symbols.Methods: We distributed questionnaires to community pharmacies through the Fukui Pharmaceutical Association and asked them to cooperate with us during the survey. The list of items in the survey　included the installation status of devices necessary for reading 2D symbols at each pharmacy, receipt computer in use, and status of the support issued by hospitals/clinics for reading 2D symbols. At the same time, we received 2D symbols created by community pharmacies and conducted reading tests to examine issues related to the collection of prescription information via 2D symbols at medical institutions.Results: The response rate for the survey was 21.8%. Among the 57 stores that responded to the survey, 26 (45.6%) answered that they could read prescription symbols, and 22 of them had actually used the system till date. In addition, 38 community pharmacies were able to provide the 2D symbols for medication diaries. Of the 30 provided symbols for medication diaries, 16 (53.3%) could be read as Japanese data by the barcode reader used.Conclusions: It has become clear that the 2D symbols with stored prescription information are not being completely utilized at present, as both community pharmacies and hospitals/clinics face several issues such as hardware maintenance, software updates, and time and effort required for the usage.