Wernicke’s encephalopathy, an acute neuropsychiatric syndrome caused by thiamine (vitamin B1 ) deficiency, is associated with serious clinical disease and can be fatal. It has rarely been reported in infants and children. We report a case of a 3-year-old girl with Wernicke’s encephalopathy. The patient’s diet had been severely unbalanced since the age of 2 years, and for about a month prior to admission to our hospital had consisted almost exclusively of polished white rice and noodles. Her clinical symptoms supported thiamine deficiency-related neuropathy. Brain MRI findings revealed abnormalities consistent with pediatric Wernicke’s encephalopathy with involvement of the putamen. The diagnosis prompted thiamine replacement therapy, to which the patient showed an excellent response.
Central Nervous System

Recently, the problem of the high incidence of throwing injuries in young people has been gaining attention. Identifying high-risk players before the onset of the throwing injury is important for prevention. One of the most widely used screening tests for sports-related injuries is the Functional Movement Screen (FMS), which assesses the quality of movement; however, its correlation with throwing injuries has not been established. The purpose of this study was to investigate the correlation between the FMS score and throwing injuries. The FMS was used during the medical check for two hundred and thirty junior high school baseball players. We allotted those who had experienced throwing injuries multiple times to the injury group and those who had never experienced throwing injury to the control group. We then calculated the FMS cutoff value using the receiver operating characteristic curve. In addition, we investigated differences in the incidence of throwing injury between above and below the cutoff value using chi-square test. The FMS cutoff score was 17. Players who scored ≤17 had a significantly higher incidence of throwing injuries than those who scored ≥18. Conclusion: We believe that FMS score is correlated to throwing injuries. In addition, the results suggest that throwing injuries might be prevented in junior high school baseball players who scored ≤17 on the FMS if they undergo training in the correct movement patterns.

Endoscopic retrograde cholangiopancreatography (ERCP) in patients with surgically altered anatomy is technically challenging. For example, scope insertion, selective cannulation, and intended procedures, such as stone extraction or stent placement, can be difficult. Single-balloon enteroscopy (SBE)-assisted ERCP has been used to effectively and safely address these technical issues in clinical practice. However, the small working channel limits its therapeutic potential. To address this shortcoming, a short-type SBE (short SBE) with a working length of 152 cm and a channel of 3.2 mm diameter has recently been introduced. Short SBE facilitates the use of larger accessories to complete certain procedures, such as stone extraction or self-expandable metallic stent placement. Despite the development in the SBE endoscope, various steps have to be overcome to successfully perform such procedure. To improve success, the challenging factors of each procedure must be identified. At the same time, endoscopists need to be mindful of adverse events, such as perforation, which can arise due to adhesions specific to the surgically altered anatomy. This review discussed technical tips regarding SBE-assisted ERCP in patients with surgically altered anatomy to increase success and reduce the risk of adverse events associated with ERCP.

Abstract: We report a case of intractable pain in a patient with amyotrophic lateral sclerosis (ALS) that was successfully managed by administering a fentanyl transdermal patch. Case: A 75-year-old man was diagnosed with ALS in 2013 after he became aware of difficulty in walking and systemic pain since 2010. In 2019, he underwent gastrostomy and tracheostomy, and intractable generalized pain necessitated the administration of morphine hydrochloride six times a day; however, it could not provide adequate pain relief. Later, morphine was replaced with a fentanyl patch under a very strict safe-monitored setting and the pain became bearable. Discussion: Although high-level evidence is lacking, pain experts have reported the effectiveness of morphine for intractable pain in patients with ALS. Frequent short-acting morphine dosing is often burdensome due to the complexity of its administration and it also causes end-of-dose pain. A fentanyl patch may possibly improve these drawbacks.

Background/Aims: Endoscopic ultrasound-guided tissue acquisition (EUS-TA) using Franseen needles is reportedly useful for its high diagnostic yield. This study compared the diagnostic yield and puncturing ability of EUS-TA using 22-gauge cobalt-chromium (CO-Cr) needles with those of stainless-steel Franseen needles in patients with solid pancreatic lesions. Methods: Outcomes were compared between the 22-gauge Co-Cr Franseen needle (December 2019 to November 2020; group C) and stainless-steel needle (November 2020 to May 2022; group S). Results: A total of 155 patients (group C, 75; group S, 80) were eligible. The diagnostic accuracy was 92.0% in group C and 96.3% in group S with no significant intergroup differences (p=0.32). The rate of change in the operator (from training fellows to experts) was 20.0% (15/75) in group C and 7.5% (6/80) in group S. Stainless-steel Franseen needles showed less inter-operator difference than Co-Cr needles (p=0.03). Conclusions Both Co-Cr and stainless-steel Franseen needles showed high diagnostic ability. Stainless-steel Franseen needles are soft and flexible; therefore, the range of puncture angles can be widely adjusted, making them suitable for training fellows to complete the procedure.

Purpose: Accelerated atherosclerosis is a major complication in patients with end-stage renal disease and it plays an important role in the pathogenesis of erectile dysfunction (ED). However, the association between aortic calcification burden and the severity of ED remains unclear. The aim of the present study was to investigate this association in men undergoing dialysis. Materials and Methods: This cross-sectional study included 71 men undergoing peritoneal dialysis and/or hemodialysis between July 2016 and May 2018 at Mutsu General Hospital. ED was assessed with the Sexual Health Inventory for Men (SHIM). Patients were divided into the mild/moderate (SHIM score ≥8) and severe ED groups (SHIM score ≤7). Aortic calcification index (ACI) was examined as a clinical indicator of abdominal aortic calcification. Multivariable logistic regression analysis was performed to identify the significant factors associated with severe ED. Results: The median age of the study participants was 64 years; all had ED, with 64.8% having severe ED. In the multivariable analyses, a slight association was observed between ankle-brachial index and severe ED (odds ratio [OR], 0.058; p=0.072), whereas ACI was significantly associated with severe ED (OR, 1.022; p=0.022). Conclusions Aortic calcification burden was independently associated with severe ED.

Purpose: To evaluate the impact of severe erectile dysfunction (ED) on future major adverse cardiovascular events (MACE) in men on dialysis. Materials and Methods: This prospective cohort study included 71 men on dialysis. ED was assessed using the Sexual Health Inventory for Men (SHIM). Men were divided into the mild/moderate ED (SHIM score ≥8) and severe ED (SHIM score ≤7) groups. The primary endpoint was MACE-free survival. MACE was a composite of myocardial infarction, cardiovascular death, and stroke. The secondary endpoints were cardiac event-free survival and overall survival (OS). Moreover, the predictive abilities of severe ED for 5-year MACE, 5-year cardiac events, and 5-year overall mortality were evaluated. Results: The median age and follow-up period of the included men were 64 years and 58 months, respectively. The median SHIM score was 4.0; all had a degree of ED, and 64.7% had severe ED. In the background-adjusted multivariable analyses, severe ED was not significantly associated with shorter MACE-free survival (hazard ratio [HR], 1.890; 95% confidence interval [CI], 0.533–6.706; p=0.324), cardiac event-free survival (HR, 2.081; 95% CI, 0.687–6.304; p=0.195), and OS (HR, 0.817; 95% CI, 0.358–1.863; p=0.630). Severe ED did not significantly improve the predictive abilities for 5-year MACE, 5-year cardiac events, and 5-year overall mortality (p=0.110, p=0.101, and p=0.740, respectively). Conclusions ED severity was not associated with shorter MACE-free survival, cardiac event-free survival, or OS, and ED severity could not improve the predictive abilities for these outcomes in men undergoing dialysis.

OBJECTIVETo investigate the clinical and neurodevelopmental profiles of patients with biotinidase deficiency and to determine the efficacy of current therapy with respect to outcome.

METHODSSix patients aged from 3 months to 14 years with biotinidase deficiency were confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS) and biotinidase assay on dried blood spots. Biotin was supplemented individually (10-40 mg/d). Their clinical features, laboratory findings, and treatment regimen were reviewed.

RESULTSAll the 6 patients presented with some extent of neurological abnormalities and dermatological lesions. Cases 1 - 3 had poor feeding, vomiting, seizures, mental retardation, and lethargy onset from their early infancy, with varied degree of anemia, ketosis, acidosis, and hypoglycemia. Case 2 exhibited eczema and dermatitis from his age of 7 months. Case 4 displayed motor deficit and ataxia after 6 months of age, and generalized pustular psoriasis when he was 8 months old. Cases 5 and 6 gradually showed muscle weakness and paraplegia at the age of 7 years and 5 years, respectively. Inflammatory demyelination changes of cervical cord were evident on magnetic resonance imaging in these two patients. Case 6 had progressive optic atrophy, eczema and alopecia. Remarkable elevations of urinary lactate, pyruvate, 3-OH-propionate, methylcitrate, propionylglycine, 3-OH-isovalerate, 3-methylcrontonylglycine were confirmed in cases 1, 2, 3 and 5. Slight increase of urinary lactate, pyruvate, and 3-methylcrontonylglycine was observed in cases 4 and 6. Biotinidase activities assayed on dried blood spots from all the patients were below 0.1 pmol/(min.3 mm) Biotin supplementation for all the patients, except for case 3 who was not treated, resulted in pronounced and rapid clinical and biochemical improvement. Cases 4 and 6 had residual neurological damage comprising ataxia and motor handicap of legs, due to prolonged disease course.

CONCLUSIONSBiotinidase deficiency intensively impairs nervous system and skin in the affected patients. Urinary organic acid analysis and blood biotinidase assay are crucial to the diagnosis. Early diagnosis and biotin supplementation can contribute significantly to the improvement of prognosis.

Adolescent ; Biotin ; administration & dosage ; therapeutic use ; Biotinidase Deficiency ; diagnosis ; drug therapy ; urine ; Child ; Child, Preschool ; Gas Chromatography-Mass Spectrometry ; Humans ; Infant ; Male ; Treatment Outcome

The importance of promoting self-medication is increasing, and the active participation of pharmacies is required to support this. We developed a training program based on the Attention, Relevance, Confidence, and Satisfaction (ARCS) model, which is a motivational design process to train pharmacists who support the proper use of OTC drugs, and for insurance pharmacists who usually perform dispensing work. In the six months of training, we conducted six sessions for insurance pharmacists on the subject of colds and constipation. In addition to conducting lectures and exercises on OTC drugs, we provided pharmacists with an opportunity to practice the training content during their daily work, and discuss solutions to the problems that they encountered. Of the eight participants who attended all workshops and were part of the final evaluation, seven provided OTC drugs after considering whether it was necessary to provide them. A total of six advised patients who came to the pharmacy to have their prescription medications dispensed that there were OTC medications that they should be careful about taking. These results suggest the usefulness of this training program based on the ARCS motivational design model.

This case report describes our first experience performing percutaneous epicardial catheter ablation for Burugada syndrome in our hospital. We describe the good results achieved in this case. The patient was a man in his 30s with no remarkable medical history. However, his family history was notable for the sudden death of his grandfather at age 37 years and his father at age 27 years. While asleep, the patient experienced convulsions and lost consciousness. During emergency transportation, defibrillation was performed 7 times by the ambulance crew. When the patient arrived at our hospital, sinus rhythm was observed on ECG. During resuscitation, Burugada syndrome was diagnosed based on ECG findings. On hospital day 6, an internal cardioverter defibrillator was implanted. After discharge, the defibrillator operated 10 times, so we opted for ablation treatment. Fractionated potential of over 150 ms was confirmed in the right ventricular outflow tract. A low voltage zone of <1 mV could be mapped, and the same site was cauterized a total of 46 times. As a result, ST segment amplitude decreased significantly in lead V1 on ECG. Percutaneous epicardial catheter ablation performed with reference to Nademanee’s report achieved good results in this case of Burugada syndrome.