Prediction Models for Two Year Overall Survival and Amputation Free Survival After Revascularisation for Chronic Limb Threatening Ischaemia.

OBJECTIVE: The aim of this study was to create prediction models for two year overall survival (OS) and amputation free survival (AFS) after revascularisation in patients with chronic limb threatening ischaemia (CLTI). METHODS: This was a retrospective analysis of prospectively collected multicentre registry data (JAPAN Critical Limb Ischaemia Database; JCLIMB). Data from 3 505 unique patients with CLTI who had undergone revascularisation from 2013 to 2017 were extracted from the JCLIMB for the analysis. The cohort was randomly divided into development (2 861 patients) and validation cohorts (644 patients). In the development cohort, multivariable risk models were constructed to predict two year OS and AFS using Cox proportional hazard regression analysis. These models were applied to the validation cohort and their performances were evaluated using Harrell's C index and calibration plots. RESULTS: Kaplan-Meier estimates of two year OS and AFS post-revascularisation in the whole cohort were 69% and 62%, respectively. Strong predictors for OS consisted of age, activity, malignant neoplasm, chronic kidney disease (CKD), congestive heart failure (CHF), geriatric nutritional risk index (GNRI), and sex. Strong predictors for AFS included age, activity, malignant neoplasm, CKD, CHF, GNRI, body temperature, white blood cells, urgent revascularisation procedure, and sex. Prediction models for two year OS and AFS showed good discrimination with Harrell's C indexes of 0.73 (95% confidence interval [CI] 0.69 - 0.77) and 0.72 (95% CI 0.68 - 0.76), respectively CONCLUSION: Prediction models for two year OS and AFS post-revascularisation in patients with CLTI were created. They can assist in determining treatment strategies and serve as risk adjustment modalities for quality benchmarking for revascularisation in patients with CLTI at each facility.

Successful launch of robot-assisted mitral valve repair in Japan under universal health coverage.

OBJECTIVES: We compared the clinical outcomes of mitral valve repair for mitral regurgitation via the robot-assisted approach and small right thoracotomy approach 3 years after the reimbursement of the robot-assisted approach in Japan. METHODS: Patients who underwent isolated mitral valve repair by minimally invasive approach between 2018 and 2020 from the Japan Cardiovascular Surgery Database were included. Patients in the robot-assisted approach group were matched to the small right thoracotomy approach group based on propensity scores estimated from patient and surgical characteristics. Perioperative outcomes were compared among all cases as well as in subgroups categorized on the basis of the yearly number of robot-assisted approach cases and small right thoracotomy approach cases (≥10 or <10) at the hospital. RESULTS: We identified 2443 patients who had undergone isolated mitral valve repair at 250 institutions in the database, and analysis of propensity-matched 577 patient-pairs demonstrated that operation time, cardiopulmonary bypass time, and aortic crossclamp time were significantly shorter with the robot-assisted approach. Although the intensive care unit stay was longer in the robot-assisted approach, the time to discharge was significantly shorter in the robot-assisted approach. There was no meaningful difference in the in-hospital mortality. The incidences of postoperative stroke, renal failure, and prolonged ventilation, and the number of patients who converted to mitral valve replacement were similarly low. Procedural time, blood transfusions, explorative procedures for bleeding, postoperative stroke, and prolonged ventilation occurred at a lower rate in the high-volume institutions. CONCLUSIONS: The study found that the robot-assisted approach is just as effective as the small right thoracotomy approach. The introduction of robot-assisted mitral valve repair in Japan has been successful.

Impact of immediate breast reconstruction on perioperative therapy: insights from a Japanese Nationwide Registry.

BACKGROUND: Immediate breast reconstruction (IBR) is a common oncoplastic procedure used in breast cancer surgery. This study aims to investigate compliance with prosthetic breast reconstruction guidelines and its impact on perioperative treatment. METHODS: We reviewed data from the National Clinical Database-Breast Cancer Registry between January 2019 and December 2020. We compared perioperative treatment implementation between the IBR and non-IBR groups by subtype matching for age, menopausal status, T stage, N stage, and histology. RESULTS: A total of 8,860 patients with breast cancer who underwent IBR (6,075 breast prostheses, 2,492 autologous tissues, and 293 others) were identified. The compliance rate with the guidelines for prosthetic breast reconstruction was 97.7%. After matching, chemotherapy for luminal A-like diseases was significantly less frequent in the IBR group than in the non-IBR group (16.3% vs 20.5%, p < 0.001), and radiotherapy was less frequent in luminal A-like and HER2-positive patients (7.2% vs 9.0%, p = 0.010 and 7.1% vs 11.4%, p = 0.005, respectively). Among the 1-3 node-positive cases, fewer patients with prosthetic IBR received radiotherapy than those without IBR (15.7% vs 26.4%, p < 0.001). CONCLUSION: Prosthetic breast reconstruction was performed with strict adherence to the Japanese guidelines. The implementation rates of chemotherapy and radiotherapy were lower in the specific IBR group than those in the non-IBR group. Therefore, large-scale, long-term follow-up data are required.

Annual report of the Japanese Breast Cancer Registry for 2019.

This is an annual report by the Japanese Breast Cancer Society regarding the clinical data on breast cancer extracted from the National Clinical Database-Breast Cancer Registry (NCD-BCR) of Japan. Here, we present an updated summary of 98,300 breast cancer cases registered in 2019. The median age at cancer diagnosis was 61 years (interquartile range 49-72 years), and 30.6% of the breast cancer patients were premenopausal. Of the 93,840 patients without distant metastases, 14,118 (15.0%) and 42,047 (44.8%) were diagnosed with stage 0 and I disease, respectively. Breast-conserving surgery was performed in 42,080 (44.8%) patients. Regarding axillary procedures, 62,677 (66.8%) and 7371 (7.9%) patients underwent sentinel node biopsy and axillary node dissection after biopsy, respectively. Whole breast irradiation was administered to 29,795 (70.8%) of the 42,080 patients undergoing breast-conserving surgery. Chest wall irradiation was administered to 5524 (11.1%) of the 49,637 patients who underwent mastectomy. Of the 6912 clinically lymph node-negative patients who received preoperative therapy, 5250 (76.0%) and 427 (6.2%) underwent sentinel node biopsy and axillary node dissection after biopsy, respectively; however, 602 (8.7%) patients initially underwent axillary node dissection without biopsy.

2020 Annual Report of National Clinical Database-Breast Cancer Registry: 10-year mortality of elderly breast cancer patients in Japan.

The Japanese Breast Cancer Society initiated the breast cancer registry in 1975, which transitioned to the National Clinical Database-Breast Cancer Registry in 2012. This annual report presents data from 2020 and analyzes the ten-year mortality rates for those aged 65 and older. We analyzed data from 93,784 breast cancer (BC) cases registered in 2020 and assessed 10-year mortality rates for 36,279 elderly patients diagnosed between 2008 and 2012. In 2020, 99.4% of BC cases were females with a median age of 61. Most (65%) were diagnosed at early stages (Stage 0 or I). Breast-conserving surgery rates varied with stages: 58.5% at cStage I, 30.8% at cStage II, and 13.1% at cStage III. Sentinel lymph node biopsy was done in 73.6% of cases, followed by radiotherapy in 70% of those post-conserving surgery and chemotherapy in 21.1% post-surgery. Pathology showed that 63.4% had tumors under 2.0 cm, 11.7% had pTis tumors, and 77.3% had no axillary lymph node metastasis. ER positivity was seen in 75.1%, HER2 in 14.3%, and 30% had a Ki67 positivity rate above 30%. Across all stages and subtypes, there was a trend where the 10-year mortality rates increased for individuals older than 65 years. In Stage I, many deaths were not directly linked to BC and, for those with HER2-type and triple-negative BC, breast cancer-related deaths increased with age. Within Stage II, patients older than 70 years with luminal-type BC often experienced deaths not directly linked to BC, whereas patients below 80 years with HER2-type and triple-negative BC, likely had breast cancer-related deaths. In Stage III, breast cancer-related deaths were more common, particularly in HER2 and triple-negative BC. Our prognostic analysis underscores distinct mortality patterns by stage, subtype, and age in elderly BC patients. It highlights the importance of personalized treatment strategies, considering subtype-specific aggressiveness, age-related factors, and comorbidities.

Survival trends and patient characteristics between 2004 and 2016 for breast cancer in Japan based on the National Clinical Database-Breast Cancer Registry.

This is a prognostic report by the Japanese Breast Cancer Society on breast cancer extracted from the National Clinical Database-Breast Cancer Registry of Japan. Here, we present a summary of 457,878 breast cancer cases registered between 2004 and 2016. The median follow-up duration was 5.6 years. The median age at the start of treatment was 59 years (5-95%: 38-82 years) and increased from 57 years between 2004 and 2008 to 60 years between 2013 and 2016. The proportion of patients with Stage 0-II disease increased from 74.5% to 78.3%. The number of cases with estrogen and progesterone receptor positivity increased from 74.8% to 77.9% and 60.5% to 68.1%, respectively. Regarding (neo-)adjuvant chemotherapy, the taxane (T) or taxane-cyclophosphamide (C) regimen increased by 2.4% to 8.2%, but the (fluorouracil (F)) adriamycin (A)-C-T/(F) epirubicin (E)C-T and (F)AC/(F)EC regimens decreased by 18.6% to 15.2% and 13.5% to 5.0%, respectively. Regarding (neo-)adjuvant anti-human epidermal growth factor-2 (HER2)-targeted therapy, the use of trastuzumab increased from 4.6% to 10.5%. The rate of sentinel lymph node biopsy increased from 37.1% to 60.7%, while that of axillary dissection decreased from 54.5% to 22.6%. Improvements in disease-free and overall survival were observed in patients with HER2-positive breast cancer, but there was no apparent trend in patients with hormone receptor-positive, HER2-negative, or triple-negative breast cancers.

Pulmonary Hypertension With Interstitial Pneumonia: Initial Treatment Effectiveness and Severity in a Japan Registry.

Background: Recent guidelines discourage the use of pulmonary arterial hypertension (PAH)-targeted therapies in patients with pulmonary hypertension (PH) associated with respiratory diseases. Therefore, stratifications of the effectiveness of PAH-targeted therapies are important for this group. Objectives: The authors aimed to identify phenotypes that might benefit from initial PAH-targeted therapies in patients with PH associated with interstitial pneumonia and combined pulmonary fibrosis and emphysema. Methods: We categorized 270 patients with precapillary PH (192 interstitial pneumonia, 78 combined pulmonary fibrosis and emphysema) into severe and mild PH using a pulmonary vascular resistance of 5 WU. We investigated the prognostic factors and compared the prognoses of initial (within 2 months after diagnosis) and noninitial treatment groups, as well as responders (improvements in World Health Organization functional class, pulmonary vascular resistance, and 6-minute walk distance) and nonresponders. Results: Among 239 treatment-naive patients, 46.0% had severe PH, 51.8% had mild ventilatory impairment (VI), and 40.6% received initial treatment. In the severe PH with mild VI subgroup, the initial treatment group had a favorable prognosis compared with the noninitial treatment group. The response rate in this group was significantly higher than the others (48.2% vs 21.8%, ratio 2.21 [95% CI: 1.17-4.16]). In multivariate analysis, initial treatment was a better prognostic factor for severe PH but not for mild PH. Within the severe PH subgroup, responders had a favorable prognosis. Conclusions: This study demonstrated an increased number of responders to initial PAH-targeted therapy, with a favorable prognosis in severe PH cases with mild VI. A survival benefit was not observed in mild PH cases. (Multi-institutional Prospective Registry in Pulmonary Hypertension associated with Respiratory Disease; UMIN000011541).

Current status of surgical treatment for acute aortic dissection in Japan: Nationwide database analysis.

OBJECTIVE: To clarify the current status of surgical treatment of acute aortic dissection (AAD) in Japan through the Japan Cardiovascular Database analysis. METHODS: In total, 7194 patients who underwent surgical treatment for AAD in 2021, including type A (TAAAD) (n = 6416) and type B (TBAAD) (n = 778), were investigated. RESULTS: The median age was 70 years, with patients older than age 80 years constituting 21.7% and 23.4% of TAAAD and TBAAD cases. Emergency admission was 88.5% and 78.5%. Shock was found in 11.8% and 6.0%. Rupture/impending rupture occurred in 10.7%/6.0% and 24.0%/11.1%, respectively. Branch malperfusion was complicated in 10.4% and 25.2%. Open repairs were performed in 97.7% and 20.3%, whereas endovascular repairs were performed in 2.3% and 79.7%, respectively. In the increased prevalence of endografting procedures, neurological complications and renal failure occurred frequently after open repair with frozen elephant trunk for 29.9% and 50.3%. The operative mortality rate was 9.8% and 11.5% for open repair and 8.1% and 10.0% for endovascular repair. In patients with TAAAD, age older than 80 years, preoperative critical comorbidities, classical dissection, and coexisting chronic vital organ diseases were independent risk factors for mortality. In frozen elephant trunk procedures, neurologic complications and renal failure were frequent. The operative mortality was higher during the superacute phase within 1 or 2 hours from onset to arrival and between arrival and surgery. CONCLUSIONS: The current status of surgical treatments for AAD including the increased prevalence of endografting of thoracic endovascular aortic repair and frozen elephant trunk were demonstrated with favorable outcomes in the Japan Cardiovascular Database analyses.

Parental age and child growth and development: child health check-up data.

BACKGROUND: The aim of the present study was to determine whether parental age has any influence on child health. METHODS: Well-baby check-up data at 1 month and at 12 months of age were used. The trends of parental age in association with growth measurements, incidence of physical and developmental abnormalities, occurrence of low birthweight, and maternal history of spontaneous abortion were analyzed. RESULTS: Associations between increasing paternal age and incidence of psychomotor developmental delay at 12 months, increasing paternal and maternal age and increasing birthweight, and increasing parental age and higher incidence of history of spontaneous abortion were found. The incidence of low-birthweight infants was significantly decreased with increasing paternal age. CONCLUSIONS: Not only increasing maternal age but also increasing paternal age have influences on child development and growth in the general population.

Clinical correlating factors and cognitive function in community-dwelling healthy subjects with cerebral microbleeds.

We performed brain gradient-echo T2(∗)-weighted magnetic resonance imaging (GE-MRI) in community-dwelling healthy people to investigate the clinical correlates (i.e., possible risk factors) and cognitive function in subjects with cerebral microbleeds (MBs). We examined 368 healthy subjects age 39 years or older living in a Japanese rural community, performing baseline and clinical assessments and brain MRI (T2(∗)-weighted, T1-weighted, T2-weighted, and FLAIR). We assessed global cognitive function in subjects age 60 years or older using the Mini-Mental State Examination (MMSE). An MMSE score >1.5 standard deviations (SD) below the mean score for a particular age group was considered subnormal. MBs were present in 14 of 368 subjects overall (3.8%; 11 males and 3 females) and in 14 of 225 subjects age≥60 years (6.2%). In a logistic regression analysis, older age (odds ratio [OR]=2.649/10 years; 95% confidence interval [CI]=1.465-4.788) and male sex (OR=6.876; 95% CI=1.801-26.248) were significantly related to the presence of MBs. The presence of silent brain infarction and white matter lesions was correlated with MBs, suggesting that MBs were the consequence of small-vessel diseases. There was a significant association between the presence of MBs and subnormal cognition defined by MMSE (OR=5.226; 95% CI=1.463-18.662). Our data suggest that in healthy community-dwelling subjects, MBs may be a consequence of small-vessel disease, which is correlated with aging, male sex, and subnormal cognition.

Alcohol intake and quantitative MRI findings among community dwelling Japanese subjects.

BACKGROUND AND PURPOSE: The relationship between alcohol consumption and subclinical findings on magnetic resonance imaging (MRI) remains uncertain. We examined the relationship between light to moderate alcohol intake and silent brain infarction (SBI), white matter lesions (WMLs), and cerebral atrophy. METHODS: Cranial MRI was performed on subjects>or=40 years residing in a rural community in Japan (n=385; mean age, 67.2). Alcohol intake and type was determined using a detailed questionnaire; subjects were categorized into three groups: non-drinkers, light drinkers (<7 drinks per week), and moderate drinkers (>or=7 drinks per week). Former drinkers were considered non-drinkers. Periventricular WMLs, deep WMLs and cerebral atrophy were measured quantitatively using a computer-assisted processing system (%PVWML, %DWML, and %Brain, respectively). RESULTS: Compared with non-drinkers, the prevalence odds ratios for SBI were significantly higher in light and moderate drinkers, after multivariate adjustment. After adjusting for age, sex, and other related factors, the geometric mean %PVWML volumes in light and moderate drinkers were 1.27% and 1.52%, respectively, significantly larger than those for non-drinkers (0.95%). The geometric mean %DWML volume in light drinkers was 0.10%, which was larger than the value for non-drinkers (0.06%); the value for moderate drinkers (0.13%) was significantly larger than that for non-drinkers. The geometric mean %Brain values for non-, light, and moderate drinkers were 92.1, 91.9 and 90.8%, respectively; a statistically significant difference was found between non-drinkers and moderate drinkers. CONCLUSIONS: The present study indicates that regular drinking, including even low levels of consumption, may be a risk factor for subclinical findings detected on MRI in community-dwelling Japanese people.

Need for thiamine in peripheral parenteral nutrition after abdominal surgery in children.

BACKGROUND: Thiamine blood concentrations of pediatric patients receiving peripheral parenteral nutrition change during the postoperative period. In addition, the need to administer thiamine after surgery has not yet been fully studied in children receiving peripheral parenteral nutrition. OBJECTIVE: The objective of this prospective study is to clarify whether pediatric patients require the administration of thiamine while receiving peripheral parenteral nutrition after abdominal surgery. PATIENTS: Fifteen children were divided into 2 groups; 1 group received peripheral parenteral nutrition without thiamine after surgery (n = 7), whereas the other group received peripheral parenteral nutrition with thiamine after surgery (n = 8). In both groups, thiamine blood concentrations were measured on the preoperative day, and changes in thiamine concentration over time were measured during the starvation period from the first to the fifth postoperative day. RESULTS: Preoperative thiamine blood concentrations were within the normal range in both groups. In the group receiving peripheral parenteral nutrition without thiamine, the thiamine concentration gradually decreased with time after the operation, whereas the concentration remained within the normal range in the group receiving peripheral parenteral nutrition with thiamine. Among the 7 patients receiving peripheral parenteral nutrition without thiamine, the thiamine concentration in 3 patients was below the normal range on the fifth postoperative day. CONCLUSION: During the starvation period after abdominal surgery, thiamine blood concentrations decreased in pediatric patients receiving peripheral parenteral nutrition without thiamine. Therefore, clinicians treating pediatric patients should add thiamine to the peripheral parenteral nutrition solution during the short starvation period after abdominal surgery.

Night-time frequency, sleep disturbance and general health-related quality of life: is there a relation?

OBJECTIVES: We conducted a community-based study to determine the relationship among night-time frequency, sleep disturbance and general health-related quality of life (GHQL). METHODS: A total of 2271 participants, men and women, aged 41-70 and randomly selected in three Japanese towns completed a postal questionnaire survey. This questionnaire included: the International Prostate Symptom Score, the overall incontinence score of the International Consultation of Incontinence Questionnaire Short Form for lower urinary tract symptoms, the Pittsburg Sleep Quality Index for sleep problems, the Medical Outcome Study Short Form-8 for GHQL, and medical history of disease, cigarette smoking, and alcohol consumption. A multiple regression model was used for statistical analysis, and P < 0.05 was considered significant. RESULTS: Although night-time frequency by itself was closely associated with most aspects of GHQL, this association disappeared in four domains (general health perception, vitality, mental health and emotional role) and in the two summary scores of the Medical Outcome Study Short Form-8 after inclusion of the influence of sleep problems represented by the total score on the Pittsburg Sleep Quality Index. However, three domains (physical function, physical role, and social function) remained significantly associated with night-time frequency. Sleep problems were by far the worst risk factor for the deterioration of GHQL. CONCLUSIONS: Night-time frequency appeared to be associated with GHQL mainly by affecting sleep conditions, a symptom that independently influenced some aspects of GHQL.

Improvement in the outcome of patients with antenatally diagnosed congenital diaphragmatic hernia using gentle ventilation and circulatory stabilization.

BACKGROUND/OBJECTIVES: No definitive treatment strategy has been established for patients with an antenatal diagnosed congenital diaphragmatic hernia (AD-CDH). From 1997 to 2003 in this department fetal stabilization (FS) was administered using both morphine and diazepam via the placenta just before delivery of the fetus by cesarean section. In contrast, from 2004 to the present, a combination of gentle ventilation (GV) and a delayed operation was selected, which was performed when the patient's circulatory stabilization (CS) was achieved. PATIENTS AND METHODS: This study included 22 patients in the FS group and 16 patients in the GV + CS group, respectively. The outcomes in both groups were compared and the outcome in AD-CDH patients with a patch repaired operation, liver-up or lower lung-to-thorax transverse area ratio (L/T, <0.10) was further investigated in both groups. RESULTS: The overall survival rate (SR) was 93.8% in the GV + CS group and 59.1% in the FS group, respectively (P = 0.04). For the patients with the lower L/T, the SR was 85.7% in GV + CS group and 53.8% in the FS group (P = 0.33). Regarding the patients using a patch and liver-up, the SR in GV + CS group was better than that in the FS group (patch: FS 44.4%, GV +/- CS 87.5%, P = 0.18; liver-up: FS 57.8 and 87.5%, P = 0.30). CONCLUSION: Our strategy of using GV +/- CS might thus be considered to be more effective than that using FS in the treatment of AD-CDH patients.

Prevention of recurrence with epirubicin and lactobacillus casei after transurethral resection of bladder cancer.

PURPOSE: A prospective, randomized, controlled trial was done to evaluate whether oral administration of a preparation of the probiotic agent Lactobacillus casei (Yakult Honsha, Tokyo, Japan) could enhance the prevention of recurrence by intravesical instillation of epirubicin after transurethral resection for superficial bladder cancer. MATERIALS AND METHODS: Between August 1999 and December 2002, 207 patients clinically diagnosed with superficial bladder cancer were included as study candidates and underwent transurethral resection, followed by intravesical instillation of 30 mg epirubicin/30 ml saline twice during 1 week. After histological confirmation of superficial bladder cancer they were again included as study participants with 102 randomized to receive treatment with 6 additional intravesical instillations of epirubicin during the 3-month period after transurethral resection (epirubicin group) and 100 randomized to intravesical chemotherapy on the same schedule as the epirubicin group plus oral administration of 3 gm Lactobacillus casei preparation per day for 1 year (epirubicin plus Lactobacillus casei group). Patients were evaluated for intravesical recurrence, disease progression, prognosis and adverse drug reactions. RESULTS: The 3-year recurrence-free survival rate was significantly higher in the epirubicin plus Lactobacillus casei group than in the epirubicin group (74.6% vs 59.9%, p = 0.0234), although neither progression-free nor overall survival differed between the groups. The incidence of adverse drug reactions did not significantly differ between the groups and there were no serious adverse drug reactions. CONCLUSIONS: Intravesical instillation of epirubicin plus oral administration of Lactobacillus casei preparation is a novel, promising treatment for preventing recurrence after transurethral resection for superficial bladder cancer.

Validation of Partin tables and development of a preoperative nomogram for Japanese patients with clinically localized prostate cancer using 2005 International Society of Urological Pathology consensus on Gleason grading: data from the Clinicopathological Research Group for Localized Prostate Cancer.

PURPOSE: We validated the 2001 Partin tables and developed an original nomogram for Japanese patients using the 2005 International Society of Urological Pathology consensus on Gleason grading. MATERIALS AND METHODS: Prostatectomy specimens from 1,188 Japanese men who underwent radical prostatectomy for clinically localized prostate cancer (cT1-2) between 1997 and 2005 were analyzed. Polychotomous logistic regression analysis was used to construct a nomogram to predict final pathological stage (organ confined disease, extraprostatic extension, seminal vesicle invasion and lymph node involvement) from 3 variables, including serum prostate specific antigen, clinical stage and biopsy Gleason score. The area under the ROC curve was used to compare the new nomogram with the Partin tables. RESULTS: Preoperative serum prostate specific antigen and biopsy Gleason score were higher in the Japanese cohort than in the Partin cohort. The distribution of clinical and final pathological stages was similar in the 2 cohorts. The AUC for predicting organ confined disease was 0.699 and 0.717 for data applied to the Partin tables and to the new nomogram, respectively. The AUC for predicting lymph node involvement was 0.793 and 0.863, respectively. CONCLUSIONS: To our knowledge this is the first preoperative nomogram developed for clinically localized prostate cancer in Japanese patients. Although the new nomogram predicted the pathological stage of prostate cancer in Japanese patients more accurately than the Partin tables, it did not satisfactorily predict organ confined disease. However, other predictive variables, such as more detailed pathological features of biopsy specimens or magnetic resonance imaging, may further improve prediction accuracy.

Clinical features and outcome of MLL gene rearranged acute lymphoblastic leukemia in infants with additional chromosomal abnormalities other than 11q23 translocation.

The treatment outcome for infant acute lymphoblastic leukemia (ALL) with positive MLL gene rearrangements remains poor. We analyzed whether additional chromosomal abnormalities (ACA) other than 11q23 translocation could affect the disease behavior and its prognosis. Eighteen of seventy-four patients with infant acute lymphoblastic leukemia showed ACA, including three-way translocations in four, other novel translocations in four, and complex structural chromosomal changes in four. Only age less than 6 months and positive central nervous system leukemia were significant prognostic factors by multivariate analysis. However, overall survival rates were worse in patients with ACA compared to those with non-ACA. Genetic alterations induced by additional chromosomal changes may be associated with disease progression and poorer overall survival rates in infants with MLL-rearranged ALL.

Neural basis of photo/chromatic sensitivity in adolescence.

PURPOSE: To determine a psychophysiological basis for age visual sensitivity to chromatic and achromatic stimuli. METHODS: We investigated the effects of achromatic and four isoluminant color combinations (blue/red, blue/green, green/red, and blue/yellow), luminance ratio changes in color combinations (blue/red; 1:1, 3:4, 4:3) and contrast changes (3 to 100%) on steady-state electroretinograms (ERGs) and visual evoked potentials (VEPs) in 32 healthy teenagers and 30 young adults. RESULTS: We found that (1) dual peaks at 9 and 18 Hz with a dip at 12 Hz were observed in VEPs with all isoluminant color combinations, (2) VEP responses were significantly enhanced and the 12-Hz dip became unclear with luminance ratio changes between two colors with a nonantagonistic relationship (blue/red), and (3) VEP amplitudes were significantly increased when the contrast was increased. These characteristics were more evident in teenagers than young adults; however, ERGs were qualitatively similar between the two groups. DISCUSSION: The visual cortex is differently modulated by different color-luminance combinations, and higher sensitivity to color-luminance combinations in the visual cortex in teenagers is responsible for the high prevalence of photo/chromatic sensitivity in adolescence.

Decreased maternal protein S activity is associated with fetal growth restriction.

INTRODUCTION: Protein S (PS) activity has been shown to decrease during normal pregnancy. The aim of this study was to determine any correlation between decreased maternal PS activity and fetal growth restriction (FGR). METHODS: We carried out a retrospective study of maternal PS activity and complement 4b-binding protein (C4BP) concentration in 102 patients with FGR and 58 patients with fetuses that had normal growth. Among pregnancies affected by FGR, 14 diagnoses were made in the second trimester and 88 in the third trimester. Patients whose fetuses had normal growth were matched with FGR subjects for maternal age and gestational age at sampling (29 cases each in the second and third trimester). RESULTS: Mean PS activity of the control group in the third trimester was significantly lower than in the second trimester (56.5+/-16.5% vs 35.8+/-13.8%). PS activity in women with FGR was significantly decreased in both the second trimester (36.6+/-13.2%) and third trimester (30.2+/-12.2%) compared with control group levels. Plasma concentrations of C4BP for the control group were significantly higher in the third trimester than in the second trimester (90.5+/-17.5% vs 81.1+/-13.6%). However, in women with FGR, plasma C4BP concentrations in both the second trimester (84.0+/-14.8%) and the third trimester (86.0+/-17.7%) were comparable with concentrations of the control group. CONCLUSIONS: Maternal PS activity decreased as normal pregnancies progressed but decreased over time in cases with FGR. Excessive decreases in PS activity during pregnancy could contribute to development of FGR.