Clinical Characteristics Of Japanese Patients Research Articles

Clinical characteristics of Japanese patients with corticobasal degeneration

IntroductionCorticobasal degeneration (CBD) is a clinically heterogeneous neurodegenerative disorder, for which pathological investigations are essential for a definitive diagnosis. This study explored the clinical characteristics of Japanese patients with pathologically confirmed CBD. MethodsWe reviewed the data of Japanese patients with pathologically confirmed CBD who were consecutively autopsied at our institute. Clinical data were obtained from medical records and clinicopathological conferences. ResultsOf the 34 patients initially reviewed, three were excluded because of a lack of detailed clinical data. Of the remaining 31 patients, 16 were men and 15 were women. The mean ages at onset and death were 63.3 ± 6.7 (51–79) years and 69.1 ± 6.9 (54–86), respectively. The median disease duration was 6.0 (2.5–12) years. The clinical phenotypes were as follows: progressive supranuclear palsy syndrome (PSPS; n = 20, 64.5 %), probable or possible corticobasal syndrome (n = 6, 19.4 %), frontal behavioral-spatial syndrome (n = 4, 12.9 %), nonfluent/agrammatic variant of primary progressive aphasia (n = 1, 3.2 %). Furthermore, 28 (90.3 %) patients exhibited dysphagia with a median latency of 3.5 (1.0–10.0) years, and 22 (71.0 %) patients who underwent tube feeding survived longer than those who did not (P = 0.013). ConclusionsCompared with Western populations, a high prevalence of PSPS may be a clinical characteristic of Japanese patients with CBD. Additionally, dysphagia occurs in many patients with early latency and may shorten survival. Tube feeding contributes to the prolonged survival of patients with CBD.

Clinical characteristics in Japanese patients with chronic rhinosinusitis who underwent endoscopic sinus surgery

ObjectiveThere is heterogeneity in the pathophysiology of chronic rhinosinusitis (CRS). Obtaining a detailed understanding of patient profiles in specific regions can provide valuable information not only for clinical practice but also future research plans. The aim of this study was to investigate the characteristics of patients who underwent endoscopic sinus surgery (ESS) for CRS. MethodsThis retrospective, single-center study examined the features of 453 patients with CRS who underwent ESS in the Tokyo area of Japan. The study evaluated various factors in patients with CRS including sex and age, the Japanese Epidemiological Survey of Refractory Eosinophilic Chronic Rhinosinusitis (JESREC) score, the recurrence rate of CRS, comorbidities of asthma and/or allergic diseases, and IgE sensitization to 12 inhaled allergens. ResultsAge-related declines in the sensitization rate to inhaled allergens were observed, and the most notable age-related decrease in specific IgE antibodies was observed for house dust mites (HDM) (p = 8.3 × 10−7). Sensitization to HDM, cat dander, and various types of fungi, including Aspergillus, was frequently observed in the CRS with asthma group, with rates of 54%, 17%, and 17%, respectively. We found that 23% of the patients had recurrence. In the recurrence group, the positive rates of specific IgE antibodies for birch and cat dander were significantly higher than in the no recurrence group. Bronchial asthma was identified as an important factor for recurrence. Among male patients, the recurrence group was younger than the no-recurrence group (p = 0.0032). Severe eosinophilic CRS (ECRS) showed early recurrence after surgery, with over the half of the patients experiencing at least one recurrence within 2 years post-surgery. Among patients with ECRS, the recurrence rate for females was 1.92 times higher than for males. ConclusionOur study revealed the influences of age and sex on various clinical phenotypes of CRS patients undergoing ESS. There was a high sensitization rate to cat dander in both the recurrence and asthma groups. Further research on diverse disease etiologies is necessary to improve therapeutic strategies for patients with CRS.

Clinical characteristics of Japanese patients with myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis.

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a rare disease, which presents with features of myelodysplastic syndromes with ring sideroblasts and essential thrombocythemia, as well as anemia and marked thrombocytosis. SF3B1 and JAK2 mutations are often found in patients, and are associated with their specific clinical features. This study was a retrospective analysis of 34 Japanese patients with MDS/MPN-RS-T. Median age at diagnosis was 77 (range, 51-88) years, and patients had anemia (median hemoglobin: 9.0g/dL) and thrombocytosis (median platelet count: 642 × 109/L). Median overall survival was 70 (95% confidence interval: 68-not applicable) months during the median follow-up period of 26 (range: 0-91) months. A JAK2V617F mutation was detected in 46.2% (n = 12) of analyzed patients (n = 26), while an SF3B1 mutation was detected in 87.5% (n = 7) of analyzed patients (n = 8). Like those with myelodysplastic syndromes or myeloproliferative neoplasms, patients often received erythropoiesis-stimulating agents and aspirin to improve anemia and prevent thrombosis. This study, which was the largest to describe the real-world characteristics of Japanese patients with MDS/MPN-RS-T, showed that the patients had similar characteristics to those in western countries.

PS-C03-4: THE REAL WORLD TRENDS IN BLOOD PRESSURE OF DIALYSIS PATIENTS IN JAPAN USING THE WEB-BASED DATABASE

Objective: Hypertension is common in dialysis patients, which is frequent as well as poorly controlled. Blood pressure (BP) has a J curve relationship with cardiovascular disease and survival in dialysis patients; however, optimal BP is not well defined. We wanted to learn about the clinical characteristics of Japanese patients undergoing dialysis and the prevalence of cardiovascular death based on systolic blood pressure (SBP) using the Web based Analysis of Dialysis Data Archives (WADDA) system database. Design and Methods: We enrolled the patients undergoing dialysis who were recorded pre dialysis SBP from 2012 to 2020 using WADDA system 2.1. According to the Japanese Society of Hypertension guidelines 2019 (JSH 2019), we classified SBP and evaluated their trends over time by gender and age. Moreover, we evaluated the association between SBP and cardiovascular death. Results: Using the 2020 database, we looked at 336,759 Japanese patients undergoing dialysis (222,510 men and 114,249 women). The mean age was 69.4 ± 12.5 years, the mean dialysis history was 7.4 ± 7.7 years, and the mean pre dialysis SBP was 152.3 ± 24.7 mmHg. Among these patients, 72% had hypertension, 56.4% had diabetes, 28.9% had ischemic heart disease, and 25.4% had cerebrovascular disease. From 2012 to 2020, there was no change in the percentage of hypertension in each age group. The rate of grade II and III hypertension was predominantly male in the younger population (the twenties to fifties), but it shifted to females in the older population (over sixties). This gender disparity was more pronounced in the grade III hypertension group. Patients with cerebrovascular death had higher rates of hypertension (60.7 percent [gradeI; 26.2%, gradeII; 17.3%, gradeIII; 17.3%]) than those with heart failure death (32.9 percent [gradeI; 18.3%, gradeII; 8.4%, gradeIII; 6.2%]). Conclusion: Using the WADDA system, we summarized recent trends in BP of Japanese dialysis patients. We discovered that the rate of hypertension in patients undergoing dialysis has remained high (around 70%) and has not changed over the last decade. Furthermore, we discovered higher rates of hypertension, particularly in younger males, implying that optimal BP management for these populations may reduce cerebrovascular mortality.

Clinical characteristics of Japanese patients with polycythemia vera: results of the JSH-MPN-R18 study.

The presence of a JAK2 V617F or JAK2 exon 12 mutation is one of the three major criteria listed for the diagnosis of polycythemia vera (PV) in the 2017 World Health Organization Classification. However, a nationwide study has not yet been conducted in Japan since the discovery of JAK2 mutations. Therefore, the Japanese Society of Hematology (JSH) retrospectively analyzed the clinical characteristics of 596 Japanese patients with PV diagnosed between April 2005 and March 2018. Among the 473 patients with complete data on JAK2 mutations available, 446 (94.3%) and 10 (2.1%) were positive for the JAK2 V617F and JAK2 exon 12 mutations, respectively. During a median follow-up of 46months (range: 0-179months), 47 (7.9%) deaths occurred. The major causes of death were secondary malignancies (23.4%), acute leukemia (12.8%), non-leukemic progressive disease (10.6%) and thrombotic (6.4%) and hemorrhagic complications (6.4%). Thrombotic and hemorrhagic events occurred during the clinical course in 4.0% (n = 24) and 3.5% (n = 21) of patients, respectively. These results show that the international PV prognostic score (age, venous thrombosis and leukocytosis) is applicable to Japanese patients with PV.

An Overview of the Clinical Characteristics of Japanese Patients with Spinal Muscular Atrophy: Data from SMART Consortium

An Overview of the Clinical Characteristics of Japanese Patients with Spinal Muscular Atrophy: Data from SMART Consortium

Clinical Characteristics of Japanese Patients Who Visited a COVID-19 Aftercare Clinic for Post-Acute Sequelae of COVID-19/Long COVID.

IntroductionThe long-term clinical course, prognosis, and optimal management of symptoms and conditions after the acute phase of coronavirus disease 2019 (COVID-19) remain to be elucidated. The purpose of this study was to clarify the characteristics of patients referred to a COVID-19 aftercare (CAC) clinic established at a tertiary academic hospital in Japan.MethodsThis study was a descriptive case series study. All patients who visited the CAC clinic between February 15 and September 17 in 2021 were included. Patients’ background, chief complaints, and clinical courses after the onset of COVID-19 were described.ResultsA total of 87 Japanese patients (median age, 40.0 years; interquartile range [IQR], 26.5-53.0 years; 52.9% women) were referred to the CAC clinic. The median interval between the onset of COVID-19 and the visit to the clinic was 79.0 (IQR, 52.5-112.0) days. Referral sources were hospitals (36 patients), clinics (47 patients), a local healthcare center (3 patients), and other (1 patient). The most common chief complaint was general fatigue (50.4%) followed by dysosmia (28.7%), dysgeusia (26.4%), hair loss (18.4%), headache (17.2%), dyspnea (16.1%), and dyssomnia (13.1%). Respiratory symptoms were common in the early stages of the disease but were less common as the chief complaints when visiting the clinic. On the other hand, neurological, psychiatric, and extremity symptoms were predominant one month after the onset of COVID-19.ConclusionsRegardless of the severity in the acute phase, patients visiting our CAC clinic suffered from a variety of symptoms. General physicians skilled in using a comprehensive approach would be optimal to see patients with such complex symptoms.

Analysis of onset and clinical characteristics in Japanese patients with infantile hemangioma.

Infantile hemangioma (IH) is a common benign tumor during infancy, although the detailed mechanism behind it has not been fully elucidated. Based on previous studies, we hypothesized that formation of hemangioma might be triggered by secondary physiological events (perinatal hypoxia or mechanical stress during delivery) in patients carrying germline risk mutations. We aimed to clarify the mechanism by evaluating whether head and neck lesions were more frequent in patients in who IH appeared after birth compared with those in who it was present at birth. Clinical data of 62 lesions in 51 patients with IH were collected. All patients were analyzed for correlation of onset with gender, localization, family histories, gestational age, birth weight, and clinical subtypes. Distribution of lesions on the head and neck was slightly more frequent in the after-birth IH group, compared with those with IH present at birth, but without significant difference (47.6% vs. 40.0%, p = 0.32). On the other hand, the ratio of superficial and deep type IH at birth was significantly altered compared with that in IH after birth (19:0 vs. 26:7, p = 0.039). In addition, IHs appearing after birth tended to more commonly have multiple lesions than those with IH present at birth, with statistically significant difference (25.8% vs. 0%, p = 0.0164). There may therefore be different triggers for IHs at birth and IH after birth. Further studies with greater number of patients are necessary to validate these findings.

Association of rs3750920 polymorphism in TOLLIP with clinical characteristics of fibrosing interstitial lung diseases in Japanese

TOLLIP polymorphism has been implicated in the development and prognosis of idiopathic pulmonary fibrosis (IPF), mainly in whites. However, ethnic differences in the characteristics of other interstitial pneumonia (non-IPF) subtypes are unclear. We evaluated the association between the rs3750920 genotype and the clinical characteristics of Japanese patients with fibrosing interstitial lung diseases (ILD). We genotyped 102 patients with fibrosing ILD (75 IPF and 27 non-IPF patients) and analyzed the interaction between the rs3750920 genotype distribution and their clinical characteristics. The overall frequencies of the C/C, C/T, and T/T genotypes were 69%, 25%, and 6%, respectively. The proportion of minor T allele carriers was larger in IPF patients than in non-IPF patients (37% vs. 15%, P = 0.031). In addition, survival at 3 years was significantly better for carriers than for non-carriers of the T allele. There was no significant association between genotype distribution and change in pulmonary function after introduction of antifibrotic agents. The frequency of the minor T allele of rs3750920 was low in Japanese patients with fibrosing ILD, particularly in non-IPF patients. Carriers of the minor T allele had better survival than non-carriers. Presence of the T allele might thus be an indicator of better outcomes for fibrosing ILD.

Coronary artery spasm-induced acute myocardial infarction in patients with myocardial infarction with non-obstructive coronary arteries.

Coronary artery spasm-induced acute myocardial infarction (CASIAMI) is one of the etiologies of myocardial infarction with non-obstructive coronary arteries (MINOCA). We retrospectively analyzed the incidence and clinical characteristics of Japanese patients with CASIAMI and non-obstructive coronary arteries. We experienced 62 patients with MINOCA (10 thrombosis, 7 unknown causes, and 45 CASIAMI) among 991 patients with suspected AMI. Pharmacological spasm provocation testing was performed in 37 patients. CASIAMI without obstructive coronary arteries was found in 4.5% of patients with suspected AMI and was observed in 73% of patients with MINOCA. Patients with CASIAMI were frequently males and had relatively small AMIs. Spontaneous spasm was recognized in 8 patients. We could reproduce provoked spasm in 37 patients with MINOCA, including 23 patients with multiple spasm. No patients died during the follow-up period. The clinical outcomes in patients with CASIAMI under optimal coronary vasodilators were satisfactory.

Clinical characteristics of Japanese patients with moderate to severe COVID-19

IntroductionAlthough several reports on the risk factors for severe disease of COVID-19 already exist, reports on effective early indicators are still limited, especially from Japan. This study was conducted to clarify the patient’s characteristics whose disease progressed to severe status. MethodsThe medical records of all consecutive 300 Japanese patients hospitalized at our institution between February and November 2020 were retrospectively reviewed. The clinical characteristics were evaluated to compare between mild (no oxygen needed), moderate (oxygen needs of 1–4 L/min), and severe diseases (oxygen needs of 5 L/min or more). ResultsThe median age was 68 years old, with 123 (41.0%) males and 177 (59.0%) females. Of these, 199 patients (66.3%), 55 patients (18.3%), 46 patients (15.3%) patients were in the mild disease, moderate disease, severe disease groups, respectively. Patients with severe disease were more likely to be older, have more comorbidities, and tended to have higher body mass index. In laboratory data, lymphocyte count, levels of C-reactive protein (CRP), LDH, and AST on admission were significantly associated with the severity. In multivariate analysis, age and CRP were the independent risk factors for severe disease (OR = 1.050, 1.130, respectively). The optimal cut-off value for age was 74 years old and that for CRP was 3.15 mg/dL. ConclusionsAge and CRP were independently associated with disease severity of COVID-19 in multivariate analysis. Additionally, the numbers of underlying disease, lymphocyte count, and inflammatory markers such as LDH and D-dimer may also be related to disease severity.

Clinical manifestations and epilepsy treatment in Japanese patients with pathogenic CDKL5 variants

ObjectivePatients with pathogenic cyclin-dependent kinase-like-5 gene (CDKL5) variants are designated CDKL5 deficiency disorder (CDD). This study aimed to delineate the clinical characteristics of Japanese patients with CDD and elucidate possible appropriate treatments. MethodsWe recruited patients with pathogenic or likely pathogenic CDKL5 variants from a cohort of approximately 1,100 Japanese patients with developmental and epileptic encephalopathies, who underwent genetic analysis. We retrospectively reviewed clinical, electroencephalogram, neuroimaging, and genetic information. ResultsWe identified 29 patients (21 females, eight males). All patients showed severe developmental delay, especially in males. Involuntary movements were observed in 15 patients. No antiepileptic drugs (AEDs) achieved seizure freedom by monotherapy. AEDs achieving ≥ 50% reduction in seizure frequency were sodium valproate in two patients, vigabatrin in one, and lamotrigine in one. Seizure aggravation was observed during the use of lamotrigine, potassium bromide, and levetiracetam. Adrenocorticotrophic hormone (ACTH) was the most effective treatment. The ketogenic diet (KD), corpus callosotomy and vagus nerve stimulation did not improve seizure frequency in most patients, but KD was remarkably effective in one. The degree of brain atrophy on magnetic resonance imaging (MRI) reflected disease severity. Compared with females, males had lower levels of attained motor development and more severe cerebral atrophy on MRI. ConclusionOur patients showed more severe global developmental delay than those in previous studies and had intractable epilepsy, likely because previous studies had lower numbers of males. Further studies are needed to investigate appropriate therapy for CDD, such as AED polytherapy or combination treatment involving ACTH, KD, and AEDs.

Clinical Characteristics of Japanese Patients with Elderly-Onset Adult-Onset Still's Disease.

The aim of this study was to compare the characteristics of Japanese patients with elderly-onset Adult-onset Still's disease (AOSD) and those with younger-onset AOSD. Patients were classified into elderly-onset (≥ 65 years, n = 20) and younger-onset (< 65 years, n = 62) groups according to age at AOSD diagnosis. Analyses included the comparison of clinical features, treatments, and Pouchot and modified Pouchot (mPouchot) scores between the two groups. The frequencies of sore throat, lymphadenopathy, and splenomegaly were significantly lower in the elderly-onset group than in the younger-onset group (30.5% vs. 80.6%, p = 0.0004; 15.0% vs. 54.8%, p = 0.0019; 30.0% vs. 61.3%, p = 0.0203; respectively). There were no significant differences in the frequencies of complications, such as macrophage activation syndrome and disseminated intravenous coagulation, between the patients with elderly-onset or younger-onset AOSD. Serum ferritin levels were higher in the elderly-onset group than in the younger-onset group, albeit without statistical significance (median, 9,423 vs. 4,164 ng/mL, p = 0.1727). Pouchot score was lower in the elderly-onset group than in the younger-onset group (median score, 5.5 vs. 4.0, p = 0.0008); however, there was no significant difference in the mPouchot score between the two groups. Our analyses revealed that elderly-onset AOSD was associated with certain characteristics that were distinct from those of younger-onset AOSD and that the disease severity in patients with elderly-onset AOSD, determined by Pouchot score at the time of AOSD diagnosis, was similar to or less than that in patients with younger-onset AOSD.

Clinical and histopathological characteristics and survival analysis of 4594 Japanese patients with melanoma.

BackgroundThe incidence of melanoma among those of an Asian ethnicity is lower than in Caucasians; few large‐scale Asian studies that include follow‐up data have been reported.ObjectivesTo investigate the clinical characteristics of Japanese patients with melanoma and to evaluate the prognostic factors.MethodsDetailed patient information was collected from the database of Japanese Melanoma Study Group of the Japanese Skin Cancer Society. The American Joint Committee on Cancer seventh Edition system was used for TNM classification. The Kaplan‐Meier method and Cox proportional hazards model were used to estimate the impact of clinical and histological parameters on disease‐specific survival in patients with invasive melanoma.ResultsIn total, 4594 patients were included in this analysis. The most common clinical type was acral lentiginous melanoma (40.4%) followed by superficial spreading melanoma (20.5%), nodular melanoma (10.0%), mucosal melanoma (9.5%), and lentigo maligna melanoma (8.1%). The 5‐year disease‐specific survival for each stage was as follows: IA = 98.0%, IB = 93.9%, IIA = 94.8%, IIB = 82.4%, IIC = 71.8%, IIIA = 75.0%, IIIB = 61.3%, IIIC = 41.7%, and IV = 17.7%. Although multivariate analysis showed that clinical classifications were not associated with survival across all stages, acral type was an independent poor prognostic factor in stage IIIA.ConclusionsOur study revealed the characteristics of melanoma in the Japanese population. The 5‐year disease‐specific survival of each stage showed a similar trend to that of Caucasians. While clinical classification was not associated with survival in any stages, acral type was associated with poor survival in stage IIIA. Our result might indicate the aggressiveness of acral type in certain populations.

Temporal trends in the prevalence of albuminuria and reduced eGFR in Japanese patients with type 2 diabetes.

Changes over time have been shown in renal manifestations in individuals with diabetes in the United States; however, whether the trends are shared across ethnicities is unknown. We conducted this single-center serial cross-sectional study to determine temporal changes in albuminuria and reduced kidney function in Japanese patients with type 2 diabetes. This study included adult Japanese patients with type 2 diabetes who first visited our institute between 2004 and 2013. Temporal changes during the 10years in the frequency of albuminuria ( ≥ 30mg/g creatinine) and reduced eGFR ( < 60mL/min/1.73m2) were analyzed using the univariate and multivariate logistic regression analyses and Granger causality test. 5331 Japanese patients with type 2 diabetes, 1892 women and 3439 men, with the mean age of 56 ± 13years, were studied. There was no change in the prevalence of albuminuria in the univariate analysis; however, a significantly decreasing trend was observed after adjustment for several covariates. On the other hand, patients with reduced eGFR significantly increased over time, although the statistical significance disappeared after adjustment for the covariates, including levels of serum uric acid and hemoglobin and use of renin-angiotensin inhibitors. The Granger causality test showed that time series for use of RAS inhibitors and BMI had a causative role in time series for reduced eGFR. In conclusion, prevalence of albuminuria decreased and that of reduced eGFR remained stable after adjustment for clinical characteristics in Japanese patients with type 2 diabetes during the last decade.

Clinical Characteristics of Japanese Patients with Palmoplantar Pustulosis.

Palmoplantar pustulosis (PPP) is a chronic inflammatory disorder characterized by sterile pustules predominantly involving the palms and soles. The purpose of this review was to describe the characteristics of Japanese PPP patients as PPP is frequently observed within the Japanese population. Most Japanese dermatologists consider PPP a distinct entity, and co-existence of PPP and psoriasis is rare; however, outside Japan, PPP is often considered to be palmoplantar psoriasis, and an extra-palmoplantar lesion associated with PPP is considered to be psoriasis. PPP frequently develops or exacerbates following focal infections such as tonsillitis, odontogenic infection, and sinusitis, either with or without arthralgia. Pustulotic arthro-osteitis (PAO) is a major comorbidity of PPP, most often affecting the anterior chest wall. In Japanese patients, PAO is frequently seen, whereas cases of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome with symptoms other than PPP and sternocostoclavicular joint pain are extremely rare. A difference in incidence depending on race suggests that different genetic backgrounds may be responsible for susceptibility to these disorders. The treatment of focal infections often results in dramatic effects on cutaneous lesions, as well as joint pain. The characteristics of Japanese patients with PPP are female predominance, mostly smokers, rare co-existence with psoriasis, frequent association with PAO, almost no accompanying celiac disease, and closely associated with focal infection. PPP should be separately considered from palmoplantar psoriasis.

Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study

BackgroundEpithelioid hemangioendothelioma is an exceedingly rare sarcoma often occurring as an indolent angiocentric vascular tumor at various anatomic sites. Few reports have evaluated large case series of epithelioid hemangioendothelioma.MethodsWe conducted a retrospective analysis of the clinical data of 42 consecutive patients with epithelioid hemangioendothelioma who were pathologically diagnosed between 1990 and 2014 at 13 Japanese tertiary hospitals. We analyzed their clinical characteristics, tumor features and prognostic factors.ResultsThe study included 22 men and 20 women, with a median age of 54 (range, 18–78) years. Pain was the most common symptom, occurring in 15 (68%) of the 22 symptomatic patients. The median maximum tumor diameter was 4.0 (range, 1.0–12.8) cm. The most commonly involved organs were the liver (81%), lungs (57%), and bones (12%). The overall survival rates were 79.5% at 1 year and 72.0% at 5 years. Substantially better survival was observed in asymptomatic patients than in symptomatic patients (P = 0.03), and better survival was also ovserved in patients with Ki-67 index ≤10% than in those with Ki-67 index > 10% (P = 0.04). By multivariate analysis, tumor size > 3.0 cm was associated with decreased survival (P = 0.049, hazard ratio 13.33).ConclusionsThis study showed the clinical characteristics of Japanese patients with epithelioid hemangioendothelioma. Tumor size > 3.0 cm is an independent indicator of a poor prognosis in epithelioid hemangioendothelioma. The presence of symptoms at the time of diagnosis and high Ki-67 index implied poor survival.

Changing profile of eating disorders between 1963 and 2004 in a Japanese sample.

The purpose of this study was to investigate possible changes in the demographic and clinical characteristics of Japanese patients with eating disorders using a consecutive series of patients who presented at Kyoto University Hospital between 1963 and 2004. We also studied cultural factors related to eating disorders over time. We completed a retrospective review of a cohort of patients using a checklist based on the DSM-5 diagnostic criteria. Patients seen from 1963 to 1974 (Period I, n = 26), 1975 to 1984 (Period II, n = 97), 1985 to 1994 (Period III, n = 540), and 1995 to 2004 (Period IV, n = 700) were compared. In this study, patients with restrictive eating appeared in the early 1960s. Patients with binge eating and purging behaviors appeared in the mid-1970s and thereafter increased over time. The number of patients with anorexia nervosa and bulimia nervosa dramatically increased in Period III. The proportion of patients with binge eating increased, while the proportion of patients with restrictive eating decreased over time. All patients with anorexia nervosa in the 1960s had fat phobia and disturbed body image, but none of them reported dieting for slimness. The prevalence and clinical characteristics of patients with eating disorders significantly changed across the four time periods. In terms of cultural factors, present findings suggest that factors beyond industrialization, modernization and westernization may be necessary for the development of eating disorders, and these factors may change with the times.

Clinical characteristics and ocular complications of patients with scleritis in Japanese.

To investigate the clinical characteristics of Japanese patients with scleritis STUDY DESIGN: Retrospective study METHODS: The clinical records of 123 patients with scleritis, who presented to the University of Tokyo Hospital between January, 2004 and December, 2015, were retrospectively reviewed. The cohort comprised 55 men and 68 women (mean age, 57.8±16.4 years), of which 76 showed anterior diffuse scleritis, 17 showed anterior nodular scleritis, 10 showed anterior necrotizing scleritis, and 20 showed posterior scleritis. The underlying etiology was identified in 39 patients. Autoimmune diseases were present in 32 patients, including eight with rheumatoid arthritis, seven with antineutrophil cytoplasmic antibody-associated vascular disease, and six with relapsing polychondritis. Ocular hypertension was the most common ocular complication (41%), followed by anterior chamber cells (38%). Fifty-three percent of patients required systemic immunosuppressive medication. Systemic corticosteroids were the most commonly used medication (45%), followed by methotrexate (11%). A, decrease in vision was observed in one-third of patients with anterior necrotizing scleritis or posterior scleritis, of which secondary glaucoma and optic neuritis were the major causes. Autoimmune diseases were present in 26% of patients. One-third of patients with anterior necrotizing scleritis or posterior scleritis experienced decreased vision, mostly due to secondary glaucoma and optic neuritis. Therefore, controlling intraocular pressure by methods such as administration of steroid-sparing immunosuppressive medication and appropriate treatment for posterior scleritis are essential for scleritis treatment.

Relationship Between the CYP2C19 Phenotype Using the Voriconazole-to-Voriconazole N-Oxide Plasma Concentration Ratio and Demographic and Clinical Characteristics of Japanese Patients With Different CYP2C19 Genotypes.

Although voriconazole (VRCZ) is metabolized to VRCZ N-oxide principally by CYP2C19, VRCZ clearance is affected by multiple factors. In this study, we investigated the relationship between the CYP2C19 phenotype using the VRCZ-to-VRCZ N-oxide plasma concentration ratio (VRCZ/N-oxide) and demographic and clinical characteristics of Japanese patients taking VRCZ. A total of 65 Japanese patients taking VRCZ for prophylaxis or treatment of fungal infection were enrolled in this study. Stepwise selection multiple linear regression analysis was performed to investigate the effect of factors on the VRCZ/N-oxide ratio. In patients not undergoing concurrent treatment with a drug influencing CYP2C19 activity (n = 54), the VRCZ/N-oxide ratio with definite thresholds for CYP2C19 genotypes, CYP2C19*1/*1, *1/*2 + *1/*3 + *2/*17, and *2/*2 + *2/*3, was specifically identified in patients taking VRCZ (<0.48, ≥0.48 < and <0.82 and ≥0.82). However, the VRCZ/N-oxide ratio could not be predicted based solely on the CYP2C19 genotype (R = 0.053). The route of VRCZ administration, C-reactive protein concentration determined on the same day as VRCZ plasma concentration measurement, CYP2C19 extensive metabolizer, and patient age were independent factors influencing the VRCZ/N-oxide ratio (R = 0.489, standardized regression coefficient = 0.385, 0.380, -0.231, and 0.231; P = 0.001, 0.001, 0.032, and 0.036, respectively). It is possible to comprehensively evaluate CYP2C19 activity using the actual measured value of the VRCZ/N-oxide ratio in patients taking VRCZ. The predictive performance of the VRCZ/N-oxide ratio was improved by including the route of administration, C-reactive protein level, and patient age in addition to the CYP2C19 genotype as predictive factors.