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Purpose The aim of this study was to review previous studies on infant skin problems and to summarize 1) the types of skin problems, 2) body area observed depends on the skin problem, and 3) the method of scoring for severity of skin problems. Method A search was conducted on PubMed for literature related to infant skin problems in August 2017. Among the 429 papers identified, 13 were included in this review. Result There were nine papers on diaper dermatitis, one on infant eczema, and three on seborrheic dermatitis. Four papers about diaper dermatitis used scales that were developed or used in previous studies, including the diaper rash and erythema scoring scale, Neonatal Skin Condition Score (NSCS), and Diaper Rash Severity Scale. Only the NSCS was validated. Although, erythema was assessed in all papers about diaper dermatitis, most of the types of skin problems were different among papers. Dryness, scaling, papules, and erythema were assessed in the literature on infant eczema, whereas erythema, scaling, and crusting were assessed in the literature about seborrheic dermatitis. Conclusion There was not the validated scale which was specialized the one skin problem. Also, the skin conditions related to each skin problem were different. Therefore, along with the scales for assessing the skin conditions of the entire body, the scales specialized for each skin problem or body area that have been examined for reliability and validity are preferable. [ABSTRACT FROM AUTHOR]

We analyzed 20 patients diagnosed with autoimmune neurological diseases with seizure predominance. In these patients, we examined the usefulness of Antibody Prevalence in Epilepsy and Encephalopathy (APE 2 ) score and Antibodies Contributing to Focal Epilepsy Signs and Symptoms (ACES) score in autoimmune encephalitis (AE) for facilitating early treatment. APE 2 score was positive in 19 of 20 patients. ACES score was positive in 15 of 20 patients, and 4 of 5 of the patients with negative ACES score did not have AE. Comprehensive assessment including the use of the above scores is desirable in the early stage of AE.

In the management of ulcerative colitis (UC), colonoscopy (CS) is considered essential for diagnosis;however, its invasiveness poses a challenge. Conversely, recent advancements in ultrasound diagnostic devices have improved imaging quality for the digestive tract, rendering them valuable in UC management. Therefore, this study aimed to elucidate the correlation between abdominal ultrasonography (AUS) and CS in assessing UC activity. The indices adopted for UC evaluation using AUS were as follows:1) bowel wall stratification, 2) bowel wall thickness, 3) bowel wall flow at power Doppler, 4) presence of increased brightness of inflammatory fat, and 5) presence of mesenteric lymph node swelling greater than 5mm. Subsequently, we developed a new AUS index for UC, termed the UCUS score, which comprises the aforementioned five indices. Finally, we compared the UCUS score with representative endoscopic indices, the Mayo endoscopic sub-score, and the Ulcerative Colitis Endoscopic Index of Severity. The results demonstrated that our proposed UCUS score better reflected disease activity than individual items assessed separately. ROC curve analysis revealed a UCUS score cutoff of 3 points. Therefore, a UCUS score of ≥3 points indicates the need for further examination with CS. Conversely, a score below 3 points suggests low disease activity, and in situations when evaluating treatment effectiveness, AUS could potentially substitute for CS. We believe that the UCUS score is an important source of information to understand the patient's condition and to motivate the patient to undergo endoscopy.

Background: Itch is the most troublesome symptom of atopic dermatitis, and it is important to assess it appropriately for optimal treatment. We discussed issues regarding itch and the most appropriate methods of assessment at the Atopic Itch Consensus Meeting (AICOM), attended by physicians and researchers with expertise in itch treatment and research., Methods: The AICOM participants prepared a draft consensus statement that addressed the most appropriate itch assessment methods for age groups <2 years, 2-6 years, 7-14 years, and ≥15 years. Consensus was defined as agreement by ≥80% of the participants., Results: Votes were cast by 20 participants (8 dermatologists, 7 pediatricians, and 5 researchers), and a consensus on the best current methods of itch assessment was reached with 95% agreement. For infants and preschool children, because subjective evaluation is difficult, a checklist for itch assessment was developed for caregivers., Conclusion: For itch assessment, we recommend subjective evaluation by the patient using a rating scale. For infants and preschoolers, evaluation should be done by the caregiver using a checklist, combined with objective evaluation (of skin lesions, for example) by a physician. We anticipate that more objective itch assessment indices will be established in the future.

A 72-year-old male presented with severe mitral regurgitation, moderate tricuspid regurgitation, and chronic atrial fibrillation. One month prior, he encountered difficulties with dialysis and was subsequently referred to our department for cardiac surgery. The patient's medical history includes living-donor liver transplantation for type C cirrhosis associated with acquired hemophilia A. The preoperative liver function was categorized as Child-Pugh grade B, with a model for end-stage liver disease( MELD) score of 23. His factor Ⅷ activity was close to the lower limit of the normal range. The patient underwent mitral valve replacement, tricuspid valve repair, and left atrial appendage closure. Initially, he experienced intractable bleeding, but hemostasis was easily achieved after administrating a factor Ⅷ preparation. Upon admission to the intensive care unit, his factor Ⅷ clotting activity was slightly below the normal range. Therefore, in cases where a patient with remission stage hemophilia A resulting in liver transplantation undergoes open heart surgery, it is crucial to have coagulation factor medication readily available, regardless of normal preoperative factor Ⅷ levels.

This study aimed to investigate the feasibility of estimating functional ischemia information from coronary artery computed tomography (CACT) data (i.e., morphological information). Fifty-five suspected ischemic heart disease patients were included in this study. To calculate the ischemic myocardium percentage (LV myocardial territories volume of distal portion the stenotic lesion/total LV myocardial volume) from CACT data with "coronary territories analysis, Ziostation2", and compared with the ischemic LV myocardium percentage and the functional flow reserve (FFR). The results showed that ischemic LV myocardium percentage was correlated with the FFR (r=-0.57). The median ischemic LV myocardium percentage of the FFR-positive group (n=33) was 37.1% (interquartile range [IQR] 33, 41.4%) and that of the FFR-negative group (n=22) was 24.8% (IQR 19.6, 30.6%). The ischemic LV myocardium percentage was significantly higher in the FFR-positive group (p<0.01) than in the FFR-negative group. The receiver operating characteristic (ROC) curve showed that the cutoff value for the ischemic LV myocardium percentage was 30%, with the sensitivity of 90.9% and the specificity of 77.3%. In conclusion, myocardial ischemia to diagnosis of FFR may occur when ischemic LV myocardium percentage is over than 30% and is unlikely to occur when it is less than 30%. This study suggests that the analysis of CACT data may contribute to the diagnosis of functional ischemia.

A 15-year-old female patient was diagnosed with a fulminant-type Wilson's disease. She had severe illness with a Model for End-Stage Liver Disease score of 25 and new Wilson Index score of 11. She underwent plasma exchanges, hemodiafiltration, and administration of fresh frozen plasma on consecutive days. Finally, she had recovered from severe illness and was discharged from the hospital. After 18 months of waiting time, she underwent deceased liver transplantation and returned to normal daily life. In Japan, the critical shortage of donated organs requires a long waiting time. Previous studies demonstrated that artificial liver support systems, including plasma exchange and hemodiafiltration, could be useful for a fulminant-type Wilson's disease. For such a disease, multidisciplinary bridging treatments are crucial for a successful liver transplantation.

The patient was an 81-year-old man. Transcatheter aortic valve implantation( TAVI) was performed for severe aortic stenosis using Evolut R. The patient moved to intensive care unit without an adverse event after the operation. But repeated acute heart failure occurred several times during hospital stay. Mitral regurgitation (MR) was worsened from mild at baseline to moderate or more by transthoracic echocardiography. Various factors that worsened MR after TAVI have been reported, and treatment strategy for severe aortic stenosis patients with MR should be carefully developed.

Damage of the central and peripheral nervous systems associated with systemic lupus erythematosus (SLE) is termed neuropsychiatric SLE (NPSLE). In this review, we have discussed SLE encephalopathy, which is associated with neurological symptoms in particular. At the time of diagnosis, disease severity should be evaluated based on clinical findings, imaging, laboratory tests, including cerebrospinal fluid tests and neurophysiological tests, of the patient. Subsequently, treatment involving both definitive therapy and symptomatic treatment is initiated. After introducing definitive therapy, it is further desirable to adopt an appropriate treatment approach by identifying the predominant type of pathogenesis (inflammatory or vascular). A collaborative approach involving specialists in collagen vascular disease, neurologists, and psychiatrists is important for appropriate management of NPSLE.

A 78-year-old woman was diagnosed with herpes zoster in the first branch of the trigeminal nerve and was treated with amenamevir. Subsequently, she was hospitalized for postherpetic neuralgia. Fever and unconsciousness were observed, and a diagnosis of varicella-zoster virus meningoencephalitis and vasculitis was made. In addition to the antithrombotic therapy, she was treated with intravenous acyclovir and steroid pulse therapy; however, her unconsciousness persisted. Amenamevir was not transferrable to the spinal fluid and resulted in an incomplete treatment of herpes zoster in the cerebral nerve region, suggesting that this case may be related to the severe course of the disease.

Perampanel is an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antagonist that has been marked as an antiepileptic drug for partial-onset and primary generalized tonic-clonic seizures. There have been some recent reports of perampanel being effective against cortical myoclonus by Lafora disease and Unverricht-Lundborg disease. We herein report a 49-year-old man who presented with myoclonus due to Lance-Adams syndrome (LAS) after cardiopulmonary arrest caused by a severe bronchial asthma attack. Perampanel was very effective against myoclonus induced by LAS even in the chronic state, over 10 years after the remote onset. Perampanel should be considered for the treatment of extremely refractory myoclonus due to LAS.

Denosumab is a fully monoclonal antibody against the receptor activator of nuclear factor kappa-B ligand (RANKL), and prevents skeletal-related events by bone metastasis. Hypocalcemia is the most typical adverse effect of denosumab use. We have developed a management system for the more efficient and safer management of denosumab administration, and evaluated pharmaceutical interventions for the better control of hypocalcemia. All pharmaceutical interventions in the system from April 2016 to March 2020 were retrospectively evaluated. We have also assessed the incidence of hypocalcemia in 158 patients who were administered denosumab for six months or more in the period. A total of 282 pharmaceutical interventions (7.0% of the total administration) were conducted. The most conducted intervention was regarding hypocalcemia, which involved the suspension of the injection and/or the increase of calcium and vitamin D supplement with 65% adoption and 17% temporary treatment suspensions. Other interventions were about hypercalcemia, request of laboratory examination and ordering supplements, dental consultation, and poor renal function. A total of 199 interventions (70.6%) were adopted, with 33 administrations suspended. The frequency of hypocalcemia was 27.8% with just one patient having grade 2 hypocalcemia, suggesting that there were no severe cases. Moreover, hypocalcemia was significantly normalized following pharmaceutical intervention and/or handling by physicians (p=0.02) according to the system. Conversely, the normalization rate in hypercalcemia did not differ according to the countermeasures. In conclusion, pharmaceutical interventions according to our management system benefit safe denosumab treatment, especially in severe hypocalcemia prevention.

(Objectives) This study aimed to evaluate the clinical characteristics and the mortality risk factors of 15 patients with Fournier gangrene in the past decade at Teikyo University Hospital. (Materials and methods) We retrospectively assessed 15 patients with Fournier gangrene between May 2009 and April 2019. We compared the demographic characteristics along with several clinical variables including Fournier Gangrene Severity Index of the survivors and nonsurvivors. We also assessed the risk factors associated with mortality. (Results) All patients were men with a median age of 67 years. Among the 15 patients, 9 had diabetes mellitus (60%). Furthermore, 14 patients (93%) underwent surgical debridement, 5 (33%) required orchiectomy, 3 (20%) were treated with cystostomy for urinary diversion, and 3 (20%) needed temporary colostomy for fecal diversion. Three patients died of the disease with a mortality rate of 20%. The nonsurvivors were significantly older (p = 0.043) and had a smaller body mass index (p = 0.038) than the survivors. The scores of clinical risk models, such as the Fournier Gangrene Severity Index, were higher in nonsurvivors than in survivors, with no statistical significance presumably due to the small sample size. (Conclusions) The mortality rate for Fournier gangrene during the past decade at our institution was 20%. Fournier gangrene was a potentially fatal disease even in the 2010s.

Transcatheter aortic valve replacement(TAVR) in the treatment of patients with severe aortic valve stenosis (AS) has evolved on the basis of evidence from clinical trials. A 84-year-old woman with a complaint of dyspnea was diagnosed with severe AS. A preoperative computed tomography (CT) revealed huge mural thrombus at descending aorta, therefore we planned direct aortic access for TAVR to avoid embolism. Transesophageal echocardiography revealed fluttering echogram at left ventricular outflow tract. After TAVR the fluttering echogram disappeared. A postoperative CT revealed spleen infarction. In such cases, we should keep in mind that surgical AVR can be a treatment option.

We report a case of severe sensory-motor axonal neuropathy on the lower extremities associated with diabetic ketoacidosis (DKA). A sixteen-year-old boy developed coma and admitted to our hospital. We diagnosed him with DKA based on remarkable hyperglycemia, severe acidosis with hyperketonemia. Intensive glycemic control with insulin was immediately started. He had complications of heart failure, rhabdomyolysis, and renal failure, which required intensive care including mechanical ventilation and hemodialysis. When recovered from the critical condition, he noticed severe weakness, numbness, and pain on the lower limbs, and urinary retention. On nerve conduction studies, both motor and sensory action potentials were absent. Serum anti-ganglioside antibodies were negative. Albuminocytologic dissociation was evident in the cerebrospinal fluid. MRI study revealed marked gadolinium enhancement of the cauda equina. After high-dose intravenous immunoglobulin treatment, he was relieved from leg pain, but the leg weakness and bladder bowel dysfunction did not show immediate improvement. It took approximately six months until he became able to stand and walk using ankle orthosis. Acute neuropathy is a rare complication of diabetes mellitus. Painful neuropathy is known to emerge in association with diabetic treatment, but it seldom causes severe motor disturbance. On the other hand, motor-dominant polyneuropathy has been reported to occur acutely along the treatment of DKA and hyperosmolar hyperglycemia syndrome (HHS). Present case and previous cases with DKA and HHS suggest that rapid correction of glucose level is one of the underlying factors of acute neuropathy related with diabetic treatment.

Liver cirrhosis is a major risk factor in patients requiring cardiac surgery. Although current evidence is limited to reports coming mostly from small case series, it is clear that the surgical risk increases with the severity of the liver disease. Hemodynamic instability caused by hyperdynamic circulation, systemic fluid retention, infection, and bleeding is frequently observed postoperatively in severely cirrhotic patients. Preoperative optimization, including correction of coagulopathy and poor nutrition, is therefore crucial for minimizing the predictive postoperative complications in those patients. Postoperative management should focus on bleeding and infection control, body fluid management, adequate nutrition, and hemodynamics, particularly hepatic circulation. Multiple studies have shown that patients who are diagnosed as Child-Pugh class B or C liver cirrhosis have a high surgical mortality rate, with most reports suggesting class C as inoperable. Recently, the model for end-stage liver disease( MELD) score has been gaining attention for its reliability in identifying patients at high risk for open heart surgery. Off-pump surgery may be beneficial in improving the surgical outcomes, but the evidence is weak and further studies are required. A thorough preoperative evaluation is thus mandatory in cirrhotic patients scheduled for cardiac surgery, with a particular attention to the risks and benefits of performing the surgery itself.

The patient was a 40-year-old woman who was previously diagnosed with systemic lupus erythematosus and myasthenia gravis and had received prednisolone and tacrolimus for more than 7 years. In February 2017, she noticed pain in her lower back and weakness of the lower limbs, and was referred to our hospital on day 5. She had shingles in the right lower thoracic dermatomes and Brown-Séquard syndrome with right-sided dominant weakness in her lower limbs and left-sided superficial sensory disturbance below the L1 level. Varicella zoster virus (VZV)-associated myelopathy was suspected because of her symptoms and clinical findings. Despite the immediate administration of intravenous acyclovir after hospitalization, she lost consciousness and experienced a seizure related to cerebral hemorrhage in the left temporal lobe on the night of day 5. MRI showed enhanced lesions along the spinal cord and leptomeninges of the brainstem and temporal lobe. VZV-IgG and VZV-DNA were positive in the cerebrospinal fluid. Based on these clinical features and laboratory findings, she was diagnosed as VZV-associated vasculopathy and myelopathy. She subsequently had multiple cerebral infractions and hemorrhage, and developed sudden cardiopulmonary arrest on day 6, culminating in death on day 17. Autopsy showed that inflammatory mononuclear cells had infiltrated the vascular walls of the spinal cord. Immunohistochemistry revealed that some neurons and macrophages in the white matter of the spinal cord were positive for VZV. In addition, atrophic neurons, satellite cells surrounding these neurons, and infiltrating macrophages were immune-positive for VZV at the L2 dorsal root ganglia. These findings were consistent with VZV-associated vasculopathy and myelitis. Under immunosuppressive conditions, VZV can cause shingles and neuronal complications such as vasculopathy and myelitis, which are sometimes fatal despite the immediate administration of intravenous acyclovir. New treatment drugs or drugs to prevent VZV activation are desired.

A 16-year-old male with language disorders, such as motor aphasia or mutism, was hospitalized on day 4 after the onset of fever. Magnetic resonance imaging (MRI) on admission revealed lesions of the corpus callosum and brain white matter. Brain single photon emission computed tomography (99mTc-ethyl cysteinate dimer) on day 7 shows hypoperfusion (with right dominance) of bilateral upper parietal region. His condition improved gradually with symptomatic treatments alone, and he was discharged on day 13. The lesions on the MRI disappeared by day 15. Although this case might have suffered from leukoencephalopathy, clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) type II was suspected from the reversible splenial lesion. Except for the elevation (640 times) of mycoplasma pneumonia antibody titer (particle agglutination) in the serum, the blood tests and cerebrospinal fluid findings showed no significant abnormalities. We then considered this encephalopathy was related to mycoplasma pneumonia infection. Since no symptoms of mycoplasma infection except for neurologic symptoms were observed, indirect mechanism, such as immune-mediated reactions, is suggested to cause encephalopathy in this case.

Hereditary myopathy with early respiratory failure (HMERF) with heterozygous mutations in the titin gene (TTN) is characterized by respiratory failure developing from the early phase of limb weakness or gait disturbance. Here, we describe a characteristic distribution of muscle involvement in three members of a HMERF family with a TTN mutation. Despite the differences in severity exhibited among the father, daughter and son, the systemic imaging studies showed a similar pattern among these individuals. The semitendinosus and fibularis longus muscles were selectively affected, as described previously. In addition, we found marked atrophy in the sternocleidomastoid and psoas major muscles, regardless of the disease severity. The atrophy in selective trunk muscles observed in routine CT scans can be useful for the differential diagnosis of hereditary myopathies with heart and respiratory failure.

Guillain-Barré Syndrome (GBS) is an acute monophasic immune-mediated neuropathy, generally considered to be of good prognosis. However, 15-20% of GBS patients cannot walk independently at six months from onset. Poor prognostic factors for long-term functional disability included old age, preceding diarrhea, muscle weakness on admission and on day 7 from admission, severe GBS disability score at two weeks from admission and IgG antibody against GD1a/GD1b ganglioside complex. Factors related with requirement of mechanical ventilation included the time from onset to admission <7 days, muscle weakness on admission, facial and/or bulbar weakness and IgG antibody against GQ1b. Recently modified Erasmus GBS outcome score (mEGOS) and Erasmus GBS respiratory insufficiency score (EGRIS) were reported as prognostic factors for the long-term functional disability and respiratory insufficiency. Those were designed on Dutch patients. The usefulness of these tools in Japan or other countries remained unknown. The authors validated mEGOS and EGRIS on Japanese GBS patients in Japanese GBS outcome study, which revealed that these tools were also adaptable on Japanese GBS patients. To identify clinical and biological factors of GBS in more detail, such a large scale prospective study as International GBS outcome study (IGOS) is warranted.

We report a case of a 29-year-old woman with spinal muscular atrophy (SMA) type II who developed severe ketoacidosis after short-term starvation. She was hospitalized with lower respiratory tract infection. Although her symptoms improved after administration of intravenous antibiotic agents, her food intake gradually decreased. On the 7th day of hospitalization, she experienced abdominal pain followed by vomiting, after which she was unable to eat. Approximately 12 h later, she suffered from shock, accompanied with disturbance of consciousness, and she was admitted to the intensive care unit. She was diagnosed with ketoacidosis based on arterial blood gas analyses and urine test results. On receiving continuous infusion of glucose and insulin, her ketoacidosis was rapidly resolved and her symptoms completely recovered by the next day. To prevent the recurrence of ketoacidosis, we provided a diet plan based on indirect calorimetry results. However, ketoacidosis recurred twice, at 12 months and 16 months after discharge, both within 24 h of the onset of the fasting state. In addition to insufficient glycogen storage because of chronic malnutrition, poor gluconeogenesis or poor ketone body consumption due to skeletal muscle atrophy was believed to increase the risk of acute-onset, severe ketoacidosis after short-term starvation. Clinicians must note that patients with SMA are prone to ketoacidosis and that they must be promptly treated.

Objectives　Atopic dermatitis (AD) is a common childhood disease with an increasing prevalence, affecting the quality of life of afflicted children. The onset of AD at an early age may disrupt normal sleep patterns, behavior, and relationships. Increased behavioral and discipline problems associated with AD have been documented. However, there are insufficient studies on AD at early ages, especially in Japan. This study aimed to examine the association between AD and the mental and behavioral health of children of preschool age.Methods　This study was a part of a prospective cohort study (n=20,926), and children born after April 2008 (n=7,386) were the target population of this study. Those who answered the Japanese version of the International Study of Asthma and Allergies in Childhood (ISAAC) at 4 years of age (n=4,228) and answered the Strength and Difficulties Questionnaire (SDQ) at 5 years of age were included in this study (n=3,862). The subscale scores of SDQ, namely, emotional symptoms, conduct problems, peer relationship problems, and hyperactivity/inattention, along with the sum of these 4 subscale scores, (total difficulties score (TDS), were investigated in relation to the prevalence of AD. SDQ scores were treated as continuous values for linear regression analysis and as dichotomized values for logistic regression analysis. The parental history of AD was adjusted in the final models.Results　The prevalence of AD at 4 years of age was 20.7% (n=799). The mean scores of emotional symptoms, conduct problems, and TDS were significantly higher among children with AD than among those without AD. Linear regression analysis revealed increased scores for emotional symptoms, conduct problems, and TDS in association with AD. Logistic regression analysis revealed a significantly increased risk for conduct problems in children with AD.Conclusion　This study found associations between AD at 4 years of age and emotional symptoms, conduct problems and TDS at 5 years of age. Further studies to assess the severity of AD and children's mental and behavioral problems at older ages are essential.

Background: Transabdominal ultrasound is a promising technique to evaluate inflammatory bowel disease. Several studies have demonstrated a relationship between ultrasound findings and colonic inflammation. However, the applicability of transabdominal ultrasound in patients with ulcerative colitis (UC) has not been elucidated. The aim of this study was to clarify the relationship between the transabdominal ultrasound findings and endoscopic activity in patients with UC., Methods: Patients with active and underwent transabdominal ultrasound and colonoscopy were enrolled in this retrospective single-center analysis. Blood flow in the bowel wall was evaluated by power Doppler ultrasound. Both the thickness and stratification of the bowel wall were assessed by B-mode ultrasound imaging. The endpoints were the correlations between the ultrasound appearances (i.e., blood flow, thickness, and stratification of the bowel wall) and endoscopic activity (endoscopic Mayo Score)., Results: There were 34 lesions in 26 patients evaluated. Blood flow and thickness of the bowel wall were positively significantly correlated with the endoscopic Mayo Scores (r=0.43, p=0.011 and r=0.503, p=0.002, respectively). According to the bowel stratification, the endoscopic Mayo Scores were significantly higher in unclear and diminished bowel wall stratifications than in the clear bowel wall stratifications (p<0.001 and p<0.001, respectively). When focusing on the endoscopic Mayo Scores of three lesions, blood flow was lower in ulcer lesions with a diameter of ≥10mm than in those with a diameter of <10mm., Conclusion: All transabdominal ultrasound findings of bowel blood flow, wall thickness, and wall stratification reflected colonic inflammation.

We report a case of a 77-year-old male who had been diagnosed with normal-flow high-pressure gradient severe aortic stenosis (AS) two years previously. In accordance with his wishes, it was decided not to perform surgery. He visited our hospital with anorexia and weight loss and was diagnosed with gastric cancer. Echocardiography showed a change to paradoxical low-flow low-pressure gradient severe AS (PLFLPG AS). A decrease in stroke volume is typically associated with a smaller LV size, but the reason for a smaller LV size in PLFLPG AS remains unclear. In this case, the change to PLFLPG AS was thought to be due to a decrease in whole body oxygen consumption, and this may help to understand the pathology.

Non-steroidal anti-inflammatory drugs (NSAIDs) are widely known as painkillers. The analgesic action of NSAIDs is attributable to the inhibition of prostaglandin synthesis that occurs in response to blocking cyclooxygenase activity. The effective dose of NSAIDs can vary depending on pain intensity and administration timing; however, there are few studies on this. This study aimed to elucidate whether the analgesic effect of NSAIDs changes depending on the situation in which they are taken and we focused on the NSAID, aspirin (ASP). In a rat model of brewer's yeast-induced inflammation, pain caused by 20% (w/v) brewer's yeast-treatment was defined as "strong pain" and that caused by 2.5% (w/v) was defined as "weak pain". The analgesic effect of ASP (low-dose; 44 mg/kg or high-dose; 66 mg/kg) against strong pain was dose-dependent, but that against weak pain was the same. Furthermore, we defined drug administration after 3 h of brewer's yeast-treatment as "late administration" and that after 20 min as "early administration". In the case of strong pain, the analgesic effect of "late ASP administration" was dose-dependent, but that of "early ASP administration" was the same. These results suggest that low-dose NSAIDs have an analgesic effect against weak pain or when taken early.

A 45-year old man with untreated diabetes mellitus (HbA1c 11.0%) was admitted with headache and left limb weakness. Findings of diffusion-weighted and FLAIR MR images of the brain were unremarkable. However, cortical vein dilation and occlusion of the upper sagittal sinus were visualized on T 2 * and magnetic resonance venography images, respectively. Perfusion CT revealed increased mean transit-time in the right frontal lobe. Cerebral venous thrombosis was diagnosed and treated with intravenous heparin. The neurological symptoms disappeared on post onset day (POD) 8. Contrast CT on POD 13 revealed sagittal sinus recanalization and he was discharged 10 days later. Perfusion CT helped to identify cerebral venous thrombosis that might have been associated with untreated diabetes mellitus.

We analyzed the registration data of inpatients with facioscapulohumeral muscular dystrophy (FSHD) receiving care at 27 specialized institutions for muscular dystrophy in Japan from 1999 to 2013 using data from October 1 of each year. The number of inpatients of each year ranged from 63 to 72 (67.1 ± 3.3) throughout the study period. Those aged over 50 years gradually increased during the study period, while the oldest inpatient was 82.8 years old. Most could not walk. The rate of respirator dependency increased from 21.0% in 1999 to 71.0% in 2013, while the rate of patients receiving oral nutrition was 98.4% in 1999 and then reduced to 75.4% in 2013. There were 36 death cases reported in the database, including 15 patients with respiratory failure and 4 with heart failure. Our findings indicate that FSHD patients in a severe condition are impacted by respiratory and nutritional problems and their prognosis for survival is related to respiratory failure.

A 17-year-old woman was admitted to our hospital because of a high fever, consciousness disturbance, and delirious behavior. Methicillin susceptible Staphylococcus aureus (MSSA) infection was confirmed by blood culture. Transthoracic echocardiogram showed no abnormality at first. Diffusion-weighted brain MRI showed a high intensity lesion in the middle portion of the splenium, which was shown as low intensity on apparent diffusion coefficient map. Then, antibiotics therapy was started against suspected bacterial meningitis, while the lumbar puncture was not performed because of the decreased number of platelets. Since the systolic murmur appeared at the apex on day 12, the diagnosis with infectious endocarditis was made by transthoracic echocardiogram. The MRI abnormalities disappeared on day 16 and we diagnosed her with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) associated with infectious endocarditis. This case suggests that MERS can occur associated with infectious endocarditis caused by Staphylococcus aureus.

Many cases of acute flaccid paralysis occurred during an enterovirus D68 (EV-D68) outbreak in North America in the fall of 2014, and this epidemic has been newly defined as a distinct disease entity named acute flaccid myelitis (AFM). This disease entity is relatively popular among pediatricians, whereas it remains little-known among neurologists in Japan. We reported a 7-year-old girl with AFM, in whom severe limb weakness and respiratory failure developed five days after appearance of respiratory symptoms. Clinical features of our case were mimicked by those of acute axonal motor neuropathy at early stage of the disease, and this resulted in delayed diagnosis of AFM. DNA of EV-D68 was not detected. There are few reported cases of severe AFM, in which artificial ventilation is needed for a long time including both acute and recovery phases of the illness, and functional prognosis of AFM is discussed by literature.

Wilson disease is an autosomal recessive disorder based on inborn error of copper metabolism. The copper accumulates in the liver, brain, cornea, kidney, and other organs. This disease should be considered any individual with liver abnormality except infant, any patient older than teenage with neurological (especially for extra pyramidal signs) or neuropsychiatric disorder with or without liver disease and sibling of Wilson disease patient. Typically, a combination of low serum ceruloplasmine levels and high levels of urinary copper contents is sufficient to establish a diagnosis. As other diagnostic tests, measurement of hepatic copper content and ATP7B gene analysis are available. The key strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both copper-chelating agents, such as D-penicillamine or Trientine, and/or zinc acetate. The author recommend zinc acetate monotherapy for mild to moderate hepatic disorder, Trientine mono therapy for mild to moderate neurologic disorder and combination therapy of Trientine and zinc acetate for sever hepatic or neurologic disorder.

Corticosteroid insensitive airway inflammation is one of major barrier to effective managements of chronic airway diseases, such as chronic obstructive pulmonary disease (COPD) and severe asthma. The role of nonreceptor tyrosine kinase Src is important in airway inflammation in mice models of atopic asthma and COPD. Thus, in this study, we determined the effects of Src inhibitor, dasatinib, on airway inflammation induced by repeated intranasal exposure to lipopolysaccharide (LPS). Male mice (A/J strain, 5 weeks old) were intranasally exposed to LPS twice daily for 3 d, and dasatinib was intranasally treated 2 h prior to each LPS exposure. A day after the last stimulation, lungs and bronchoalveolar lavage fluid (BALF) were collected. Dasatinib attenuated the accumulation of inflammatory cells in lungs, and the increase in the numbers of inflammatory cells and the accumulation of cytokines/chemokines in BALF in a dose dependent manner. Therefore, this study suggested that targeting the Src can provide a new therapeutic approach for corticosteroid insensitive pulmonary diseases.

Modified Rankin Scale (mRS) is widely used as an indicator of physical disability after the onset of stroke and a main evaluation scale in clinical stroke trials but sometimes has discrepancies among raters. We designed a Japanese version of simplified mRS questionnaire (J-RASQ) to evaluate mRS using closed question to assess the difference from the original. Currently there are no Japanese version of mRS questionnaire and its validity and reliability assessment has been warranted. Patients with planned visit to our hospital between 60 and 120 days after the onset of cerebral hemorrhage or infarction between August 2017 and March 2018 were recruited. J-RASQ was distributed before the outpatient visit to stroke patients, families or caregivers, and response was obtained. Thereafter, outpatient attending physicians qualified as stroke or neurological specialists evaluated mRS according to the Japanese version of guidance scheme for mRS. Subsequently, 130 patients (mean age, 72 ± 12 years old) were enrolled, and the difference between the two scale scores (mRS vs. J-RASQ) were statistically examined by the κ statistic and the weighted κ statistic. The κ statistic was 0.42 (95% CI, 0.31-0.52), and the weighted κ statistic calculated taking into account the extent of disagreement was 0.78 (95% CI, 0.70-0.86). We conclude that the J-RASQ appears to have decent reliability and is similar to the existing mRS.

A 61-year-old woman presented with acute intense lower back pain and weakness in her left leg. She also presented with throbbing headache on the same day. On admission, muscle weakness in her left leg, lower left quadrantanopia and left lower extremity deep sensory disturbance were observed. Laboratory data showed no coagulopathy and autoimmune antibody was negative. Cerebrospinal fluid examination showed bloody and inflammatory findings. Brain MRI revealed cerebral infarction with multiple intracranial arterial stenosis and convexal subarachnoid hemorrhage. Spinal MRI revealed spinal hemorrhage in the cervical, thoracic, and part of the lumbar spine. Because these lesions occurred simultaneously, we made a diagnosis of vasculitis. After high dose corticosteroids therapy was undertaken, the multiple arterial stenosis improved. Primary angiitis of the central nervous system is sometimes difficult to distinguish from reversible cerebral vasoconstriction syndrome in its initial stage; although symptoms, examination findings and treatment differ in both.

A 68-year-old man visited our hospital emergency department with consciousness disturbance. He was diagnosed as bacterial meningitis with septic shock, and initial empirical antibacterial therapy was initiated immediately. Streptococcus pneumoniae. was cultured from the cerebrospinal fluid (CSF), and brain MRIs showed pyogenic ventriculitis. Even though CSF findings improved, he was still in coma and finally died with pneumonia. It is unknown how pyogenic ventriculitis affects the course of bacterial meningitis. We analyzed total 11 inpatients with bacterial meningitis associated with or without the pyogenic ventriculitis, including the present patient, in our hospital. Severity of clinical symptoms and CSF findings might determine the duration of antimicrobial administration, regardless of whether pyogenic ventriculitis existed or not.

We divided acute ischemic stroke patients into an oldest old group (≥90 years, n = 414) and an elderly group (75-89 years, n = 1,927), and compared the clinical characteristics between the two groups. Female sex, early admission, severe stroke on admission, atrial fibrillation, and cardioembolic stoke were significantly more frequent in the oldest-old group. On the other hand, risk factors associated with atherosclerosis were less frequent in the oldest-old group. There were no differences in hyper-acute recanalization therapy between the two groups, whereas antithrombotic therapy for secondary prevention at discharge was introduced less frequently in the oldest-old group than in the elderly group. Death prior to discharge from the acute care hospital , and death at 3 months were significantly more frequent in the oldest-old group. Moreover, in the oldest old group, severe stroke and atrial fibrillation were independent predictors for both death prior to discharge from the acute care hospital and at 3 months after onset. Oldest-old stroke patients had poor outcome, although they had received aggressive treatment as same as elderly patients. Therefore, as the number of oldest-old stroke patients is increasing, the demand for cooperation in the healthcare system is likely to grow, including acute care hospitals.

A significant number of patients with minor or mild stroke symptoms on initial presentation subsequently develop neurological deterioration and poor clinical outcomes at hospital discharge. The presence of an underlying large vessel occlusion is a strong predictor of both clinical worsening and poor outcome. Although patients with a low baseline National Institutes of Health Stroke Scale (NIHSS) could have been included in some randomized controlled trials, the benefits of the mechanical thrombectomy for patients with a low NIHSS score are unknown. The causes of neurological deterioration in patients with underlying large vessel occlusion are heterogeneous, but include collateral failure, and no straightforward mechanisms are found in the majority of cases. Patients with internal carotid artery occlusion, but with a patent middle cerebral artery (MCA), can occasionally have good collateral circulation and develop only minor or mild stroke. These patients exhibit collateral MCA flow via the circle of Willis despite ipsilateral internal carotid artery occlusion. However, thrombus migration may cause occlusion of collateral MCA flow, leading to dramatic neurological deterioration. Careful observation and detailed assessment are required for the management of these patients. Recent studies have examined the efficacy and optimal timing of thrombolysis or mechanical thrombectomy for patients with minor or mild stroke. Herein, we review the mechanisms of neurological deterioration, and the efficacy of reperfusion therapy, for patients with minor or mild stroke.

From 2014 to 2015, the five pivotal stroke trials (MR CLEAN, ESCAPE, EXTEND-IA, SWIFT PRIME and REVASCAT) have shown that stroke thrombectomy clearly improved functional outcome of patients with occlusion of the internal carotid artery or the M1 portion of the middle cerebral artery, with a baseline National Institutes of Health Stroke Scale score of ≥6, with a baseline Alberta Stroke Program Early Computed Tomography Score of ≥6, and who could receive thrombectomy within a 6-hour window of symptom onset. In 2018, the efficacy of stroke thrombectomy for patients with late-presenting stroke up to 16 to 24 hours of onset and who had clinical imaging mismatch or target mismatch was also established by the DAWN and the DEFUSE3 trials. Nowadays, stroke thrombectomy, therefore, have become a "standard of care" and acute stroke patients with large vessel occlusion should receive stroke thrombectomy if they meet top-tier evidence criteria.

Here we report a case of a 12-year-old girl who was referred to our department because of marked short stature of more than -5 SD below the median. Although her growth failure began suddenly at 6 years of age, she never had an examination because she had no other symptoms. Brain MRI examination suggested a tumor in the suprasellar region, and endocrine examination revealed combined pituitery hormone deficiency due to the tumor. Before surgery, the supplementation with hydrocortisone and levothyroxine was initiated. The pathological diagnosis of the surgically removed tumor was xanthogranuloma. The pattern of her growth curve showed a growth failure with sudden onset, which is a typical pattern of short stature secondary to pituitary disfunction including growth hormone deficiency associated with brain tumors. This case suggests that growth failure could be the only symptom in pediatric cases with brain tumors. Improved awareness regarding the association of growth failure with brain tumors is needed for earlier diagnosis and treatment. Furthermore, the growth curves should be carefully evaluated in regular health examinations at school.

Inactivated quadrivalent influenza vaccine (IIV4) has been used as seasonal influenza vaccine since 2016 in Japan. This study examined the safety of IIV4 in comparison with the AH1pdm monovalent vaccine used for novel influenza in 2009. Questionnaire surveillance associated with adverse events (AEs) was conducted at Chiba University Hospital, Japan. After being vaccinated, all health care workers (HCWs) were given a daily AEs check sheet on which they recorded solicited events, the same surveillance program used after AH1pdm vaccination in 2009. The frequency of injection site AEs with IIV4 was significantly higher than with the monovalent vaccine, but there was no significant difference with systemic AEs. Injection site and systemic AEs were reported as 83.7% and 25.5%, respectively, with IIV4. The grades of AE, mild, moderate and severe, were 67.2%, 16.4% and 0.1% with IIV4, respectively, indicating that almost all of the AEs reported with IIV4 were mild or moderate. Systemic AEs with IIV4 and monovalent vaccine were reported to be 25.5% and 23.1%, respectively, with the difference not being significant. The grade of AEs with IIV4, mild, moderate and severe, was 19.1%, 5.6% and 0.9%, respectively. The ratio of HCWs reporting AEs peaked at around 80% on day 1, then decreasing to less than 5% by day 7. AEs with IIV4 were reported more frequently compared with the AH1pdm monovalent vaccine. However, in consideration of the grade and duration of AEs, IIV4 was a well-tolerated, safe vaccine.

Tazobactam/piperacillin (TAZ/PIPC) is a useful antimicrobial agent with broad antibacterial activity. Hypokalemia is considered a rare side effect of TAZ/PIPC; however, it may occur more often than previously thought. In this study, hypokalemia frequency and risk factors were examined in 420 patients treated with TAZ/PIPC. Our results demonstrated that the hypokalemia incidence was 24.8% (grade 1-2: 18.3%, grade 3-4: 6.4%). In addition, multivariate analysis revealed that age [odds ratio 1.057, 95% confidence interval 1.024-1.090, cutoff value 80.5 years] is a risk factor. Although the "Daily dosage/creatinine clearance" was not significant in multivariate analysis, univariate analysis indicated it be to be significant, with a cutoff value of 294.9 mg/mL/min. Furthermore, a "body mass index of 19.7 kg/m 2 or higher", "serum potassium level before administration of 3.95 mEq/L or more", and "no empirical treatment for administration purposes" appeared to prevent the hypokalemia development. Overall, the hypokalemia incidence rate in TAZ/PIPC-administered patients was as high as 20%, with patients aged >80.5 years considered a high-risk group. Thus, careful monitoring of potassium levels in patients treated with TAZ/PIPC, particularly those aged >81 years, is warranted.

Direct oral anticoagulants (DOACs) are safe and efficacious when compared to warfarin for patients with venous thromboembolism (VTE). However, bleeding is a major side effect of anticoagulant therapy in VTE patients. Discontinuation of the DOACs associated to adverse events such as bleeding. The HAS-BLED score predicts warfarin-associated hemorrhage. However, little is known about risk factors for DOAC-associated minor bleeding in VTE patients. We aimed to identify risk factors for minor bleeding in VTE patients that were treated with edoxaban, rivaroxaban, or apixaban. We retrospectively evaluated the data of 212 VTE patients who received treatment with a DOAC. The study endpoint was defined as the occurrence of minor bleeding. Logistic regression analysis was used to determine risk factors that were significantly associated with minor bleeding. A total of 36 (17.0%) patients experienced minor bleeding, with rates of 15.7%, 0%, and 21.3% for edoxaban, rivaroxaban, and apixaban, respectively. In the multivariate analysis, bleeding history or predisposition [odds ratio (OR) 6.083, 95% confidence interval (CI) 2.131-17.364, p=0.001] and cancer (OR 6.397, 95% CI 2.858-14.317, p<0.001) were significantly associated with minor bleeding. Bleeding history or predisposition and cancer were the most important risk factors for DOAC-induced minor bleeding in VTE patients in this study. To continue anticoagulant therapy of the DOACs, further management systems by minor bleeding risk factors for patients with VTE will be required.

Objective　This study aimed to clarify the effectiveness of the Stepping Stones Triple P (SSTP) for mothers of developmentally delayed children.Methods　The participants were 36 mothers of children using a development support classroom after a medical examination in A city. The children, aged 2 to 6 years old, were suspected to have autism spectrum disorder (ASD) based on Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) scores of 9 points or more. The investigation randomly assigned them to two groups-"the intervention group" and "the control group"-and carried out SSTP. The intervention group answered the questionnaire before and after intervention, three months later. The control group also answered the questionnaire 2 months before the intervention, before and after intervention. Thus, each group answered three times. The questionnaire used the Strengths and Difficulties Questionnaire (SDQ), a Parenting Style scale (PS), the Relationship Quality Index (RQI), and the Japanese version of a Maltreatment scale (JM). For effectiveness of the intervention before and after, an analysis of covariance was carried out on the scores from the first and second questionnaires in the intervention and control groups. For effectiveness 3 months after the intervention, a repeated-measures analysis of variance was performed on the scale scores from the first to the third questionnaire in the intervention group.Results　The average age of the children was 3.7±1.4 years old, and the average PARS score was 20±6.8. They were suspected to have ASD. The average SDQ score was 76.1±18.8, and their intelligence was borderline. A significant difference was seen before and after the intervention in SDQ (the issue of action, the total of the difficulty), PS (overreaction, gab, general score), and JM scores; no significant difference was seen in RQI scores. Three months after the intervention, a lasting effect was seen in SDQ (the issue of action, the total of the difficulty, hyperkinetic) and PS (all items) scores in the intervention group.Conclusions　Receiving SSTP caused a positive change in the mothers' parenting and improved the problem behavior of the children. It was suggested that SSTP was connected to the prevention of child abuse because it helped stop parents from hitting their children.

(Purpose) Both administration of antibiotics and drainage of urine are necessary for the treatment of acute pyelonephritis associated with urinary obstruction by the ureteral calculi. Though most patients get better after the treatment, some patients deteriorate accompanying with low blood pressure, and need the intensive care. Such patients sometimes visit small hospital, even at night with a few medical staffs. It is sometimes difficult to predict the patient's outcome. The disease severity prediction index for the patients was investigated. (Object and method) We examined 134 patients, who visited our hospital from 2001 to 2013, retrospectively. Ureteral stenting or nephrostomy was undergone within 24 hours in principle. If the blood pressure became under 90 mmHg, or lowered more than 40 mmHg than usual, the case was defined as serious. Blood data and physical findings were compared between serious and non-serious cases. The factors affecting the seriousness were found. Multiple logistic analysis was done to make a disease severity prediction index. (Result) 42 cases were judged as serious and 92 cases as non-serious. Six factors consisting of heart rate, serum creatinine, platelets, ages, PS and CRP affected the consequence significantly (p<0.05), however, white blood cells did not.Multiple logistic analysis was done, four factors consisting of serum creatinine, platelets, PS and CRP affected the consequence significantly (p<0.05), and the standardizing coefficients of each points were found to be 2, 2, 1, 1, respectively.The disease severity prediction index was proposed. If the index was 4 or more, the sensitivity and specificity were found to be 73.8% and 82.6%, respectively. (Conclusion) This index is useful and reliable for the prediction of the outcome of the disease.

Paclitaxel (PTX)-associated acute pain syndrome (P-APS) is characterized by disabling but transient arthralgia and myalgia in up to 80% of patients administered with PTX. Non-steroidal anti-inflammatory drugs (NSAIDs) are widely administered to patients with cancer who have pain or fever, and are mainly used to manage P-APS. In this study, we investigated how P-APS appear in the patients who were administered NSAIDs prior to PTX injection. The incidence or severity and duration of P-APS in patients previously administered NSAIDs were compared to those of patients who were not administered NSAIDs. The relationship between previously administered NSAIDs and rescue administration for the relief of P-APS was also evaluated. It was revealed that the incidence and duration of P-APS were 72% and 4.67±2.30 d, respectively, in the control group and 84% and 6.19±3.30 d, respectively, in the NSAIDs group. There was no significant difference in the incidence and duration and the severity of P-APS between the two groups. Patients who were previously administered NSAIDs tended to obtain less pain relief from NSAIDs administered as rescue medications, and needed other medication. Univariate and multivariate analysis revealed no correlation between previously administered NSAIDs or patient characteristics and the incidence of P-APS. In this study, it was found that clinical condition that needs NSAIDs and previously administered NSAIDs prior to PTX injection do not affect the incidence, severity, and duration of P-APS. These results will help in educating patients about their medications and will contribute to the management of P-APS.

Aim: This study aimed to clarify the effectiveness of using the complete lateral position method to treat elderly patients with severe dysphagia., Methods: We enrolled 47 patients >65 years of age who had been diagnosed with severe dysphagia using a video endoscopic examination of swallowing at Hida City Hospital between February 1, 2015, and October 31, 2017. We collected and analyzed data pertaining to patient characteristics, the onset of aspiration pneumonia, and treatment outcomes., Results: Although all patients had severe dysphagia, adopting the complete lateral position method enabled 25 patients (53.2%) to safely perform oral ingestion and be discharged home or to a nursing home. Thirteen (52.0%) of the patients who were discharged were able to safely receive oral intake in the sitting position again. In addition, the serum albumin level and Barthel index were significantly improved. In the patients whose condition worsened due to senility, the fasting period in the complete lateral potion group was significantly shorter than in the control group (7.3 days vs. 17.3 days)., Conclusions: The present study showed that the complete lateral position method enabled safe oral ingestion in elderly patients with severe dysphagia. Safe oral ingestion contributed to improved nutrition and rehabilitation. The complete lateral position method is easy to assume and does not require the use of special appliances or techniques. We believe that the complete lateral position method will prove to be a breakthrough approach in the care of elderly patients with severe dysphagia.

The case is a 64-year-old male who had a past history of herpes simplex virus encephalitis (HSE) two years prior to his admission. He was admitted to our hospital due to severe pneumonia and sepsis. Several days later, he developed HSE again. It has been known that immunosuppressive state called immune paralysis occurs in the patient with sepsis due to the amplification of anti-inflammatory responses after the initial hyper-inflammatory phase, which increases the susceptibility to various latent viruses including herpes simplex virus. In the present case, we consider that the severe infection may trigger the recurrence of HSE through the viral reactivation due to immune paralysis. When we see a patient suffering from severe infection who had a past history of HSE, we should keep in mind that such a patient may have a risk of the recurrence of HSE.