Long-Term Effect of Tafamidis on Clinical Parameters and Prognostic Predictors in Patients With Transthyretin Amyloid Cardiomyopathy.

Background: Accurate prediction of prognosis in transthyretin amyloid cardiomyopathy (ATTR-CM) is crucial for optimal treatment selection, including tafamidis, the only approved therapy for ATTR-CM. Although tafamidis has been proven to improve prognosis, the long-term serial changes in comprehensive parameters related to ATTR-CM, including cardiac biomarkers and imaging parameters, under tafamidis remain unknown.

Methods And Results: In this study, we used Cox regression analysis on data from 258 consecutive patients diagnosed with ATTR-CM at Kumamoto University to determine prognostic factors.

CT-derived Extracellular Volume Fraction in Aortic Stenosis, Cardiac Amyloidosis and Dual Pathology.

Aims: To investigate CT-derived extracellular volume fraction (CT-ECV) in patients with lone aortic stenosis (AS), dual pathology of AS and transthyretin cardiac amyloidosis (AS-ATTR), and lone ATTR, and to examine the diagnostic performance and optimal cutoff values of CT-ECV for differentiating between patients with lone AS and AS-ATTR, and between patients with lone AS and lone ATTR.

Methods And Results: This retrospective study included consecutive patients with severe AS (including lone AS and AS-ATTR) and lone ATTR who underwent CT-ECV analysis and technetium 99m pyrophosphate (99mTc-PYP) scintigraphy. The diagnostic performance of CT-ECV for detecting cardiac amyloidosis was evaluated using the area under the receiver operating characteristic curve (AUC).

Early experience with daratumumab-containing regimens in patients with light-chain cardiac amyloidosis.

Background: Immunoglobulin light-chain (AL) amyloidosis is a lethal condition resulting from misfolded immunoglobulin ALs produced by clonal CD38-positive plasma cells. Treatment with daratumumab, an anti-human CD38 monoclonal antibody, led to higher frequencies of complete hematologic response and better clinical outcomes compared with conventional treatment. This study sought to evaluate the survival benefit of daratumumab-containing regimens in patients with AL cardiac amyloidosis.

Cardiac Biomarker Change at 1 Year After Tafamidis Treatment and Clinical Outcomes in Patients With Transthyretin Amyloid Cardiomyopathy.

Background: Although tafamidis treatment improves prognosis in patients with wild-type transthyretin amyloid cardiomyopathy, an optimal surrogate marker monitoring its therapeutic effect remains unclear. This study investigated the association between changes in cardiac biomarkers, high-sensitivity cardiac troponin T (hs-cTnT) and B-type natriuretic peptide (BNP) during the first year after tafamidis treatment and clinical outcomes.

Methods And Results: In 101 patients with wild-type transthyretin amyloid cardiomyopathy receiving tafamidis at our institution, change in cardiac biomarkers from baseline to 1 year after tafamidis administration and its association with composite outcomes (composite of all-cause death and hospitalization attributable to heart failure) was assessed.

Clinical characteristics of patients with high extracellular volume fraction evaluated by cardiac computed tomography for coronary artery evaluation.

Aims: This study aims to evaluate the distribution of extracellular volume fraction detected via computed tomography, clinical characteristics of high extracellular volume fraction detected via computed tomography, and the rate of incidental detection of cardiac amyloidosis in patients undergoing cardiac computed tomography for coronary artery evaluation.

Methods And Results: This study included 874 consecutive patients (mean age, 74.4 ± 7.

Prevalence of Cardiac Amyloidosis in Patients Undergoing Carpal Tunnel Release With Amyloid Deposition.

Background: Carpal tunnel syndrome (CTS) is considered an early sign of cardiac amyloidosis (CA) because amyloid deposition is often confirmed in the tenosynovium removed during carpal tunnel release (CTR); however, the prevalence of concomitant CA is unclear.

Methods and results: We prospectively examined 700 patients who underwent CTR and evaluated amyloid deposition after tenosynovium removal. Amyloid deposition was observed in 261 (37%) patients, who were significantly older and predominantly male (P<0.

Diagnosis of Mucopolysaccharidoses and Mucolipidosis by Assaying Multiplex Enzymes and Glycosaminoglycans.

Mucopolysaccharidoses (MPS) and mucolipidosis (ML II/III) are a group of lysosomal storage disorders (LSDs) that occur due to a dysfunction of the lysosomal hydrolases responsible for the catabolism of glycosaminoglycans (GAGs). However, ML is caused by a deficiency of the enzyme uridine-diphosphate N-acetylglucosamine:lysosomal-enzyme-N-acetylglucosamine-1-phosphotransferase (GlcNAc-1-phosphotransferase, EC2.7.

Validation of Liquid Chromatography-Tandem Mass Spectrometry-Based 5-Plex Assay for Mucopolysaccharidoses.

Mucopolysaccharidoses (MPSs) are rare lysosomal storage diseases caused by the accumulation of undegraded glycosaminoglycans in cells and tissues. The effectiveness of early intervention for MPS has been reported. Multiple-assay formats using tandem mass spectrometry have been developed.

Policy statement of enteral nutrition for preterm and very low birthweight infants.

For preterm and very low birthweight infants, the mother's own milk is the best nutrition. Based on the latest information for mothers who give birth to preterm and very low birthweight infants, medical staff should encourage and assist mothers to pump or express and provide their own milk whenever possible. If the supply of maternal milk is insufficient even though they receive adequate support, or the mother's own milk cannot be given to her infant for any reason, donor human milk should be used.

Male Infants Born to Older Pregnant Women Are Affected by Maternal Physique at the Beginning of the Pregnancy through Birth until 18 Months of Age.

We have previously reported a positive correlation between the body mass index (BMI) of mothers and the Kaup index of infants at birth and that this correlation may have a stronger influence on the male infants, born to older mothers. Therefore, in this study, we aimed to clarify the correlation between maternal age and gender of the infants as maternal BMI and the Kaup index of infants from birth till 18 mo of age. This study was conducted from April 2010 to September 2011 in Japan.

Parental awareness of young children's pattern of ionic beverage consumption.

Background: The aim of this study was to investigate consumer perception of ionic beverage consumption by young children as a potential link to their excessive use.

Methods: We studied parents' awareness of ionic beverages and the current pattern of use in their young children using a self-administered questionnaire.

Results: A total of 424 responses were recorded.

Maternal Body Mass Index Correlates with the Neonatal Physique of Male Infants.

Recently, in Japan, the percentage of leanness has risen in young women, and the average birth weight has decreased. An increase in the risk of low birth weight has been reported in lean expectant mothers. In this study, we aimed to clarify the relationship between mother's physique at the beginning of pregnancy and the infant's physique, by focusing on sex differences.

Factors related to patterns of body mass index in early infancy: 18 month longitudinal study.

Background: Children with early adiposity rebound (AR), measured using individual body mass index (BMI) growth curves, have an increased risk of metabolic disease as adults. The children with early AR, however, are not fully characterized. The aim of this study was to investigate the prevalence and characteristics of the infants who develop early AR.

Case of endophthalmitis following intravitreal injections of bevacizumab.

Purpose: We report a case of infectious endophthalmitis that developed after the third intravitreal injection of bevacizumab. The endophthalmitis was caused by Staphylococcus epidermidis.

Methods: Observational case series.

Genetic relationships between growth and carcass traits and profitability in Japanese Brown cattle.

The objectives of this study were 1) to examine the genetic relationship between growth and carcass traits and carcass price (CaP) and profitability in Japanese Brown cattle, 2) to estimate economic values of carcass and growth traits as regression coefficients of price and profit traits on growth and carcass traits using a multiple regression model, and 3) to compare direct and indirect predictions of the genetic merit of profit obtained from multitrait analysis and selection index, respectively. Growth and carcass traits considered in this study were ADG during the feedlot period, CWT, LM area (LMA), rib thickness (RT), subcutaneous fat thickness (SFT), and marbling score (MS). Carcass price was evaluated as a price trait independent of its influence on profit.

Genome-wide linkage disequilibrium in two Japanese beef cattle breeds.

There is little knowledge about the degree of linkage disequilibrium (LD) in beef cattle. This study aims to perform a genome-wide search for LD in Japanese Black and Japanese Brown beef cattle and to compare the level of LD between these two breeds. Parameter D' (the LD coefficient) was used as a measure of LD, and LD was tested for significance of allelic associations between syntenic and between non-syntenic marker pairs.

Comparison of genetic gains per year for carcass traits among breeding programs in the Japanese Brown and the Japanese Black cattle.

The breeding program for beef cattle in Japan has changed dramatically over 4 decades. Visual judging was done initially, but progeny testing in test stations began in 1968. In the 1980s, the genetic evaluation program using field records, so-called on-farm progeny testing, was first adopted in Oita, Hyogo, and Kumamoto prefectures.

Positional cloning of the gene LIMBIN responsible for bovine chondrodysplastic dwarfism.

Chondrodysplastic dwarfism in Japanese brown cattle is an autosomal recessive disorder characterized by short limbs. Previously, we mapped the locus responsible for the disease on the distal end of bovine chromosome 6. Here, we narrowed the critical region to approximately 2 cM by using linkage analysis, constructed a BAC and YAC contig covering this region, and identified a gene, LIMBIN (LBN), that possessed disease-specific mutations in the affected calves.

Superselective embolisation for intractable idiopathic epistaxis.

37 patients with intractable idiopathic epistaxis were treated with superselective embolisation between 1995 and 1999. A total of 40 embolisations was performed, including three procedures for recurrence. The embolic material was gelatin sponge in 27 procedures, microcoils in 9 and both gelatin sponge and microcoils in 4.

[Antimicrobial susceptibility and serotypes of Streptococcus pneumoniae isolated from the patients with respiratory tract infections in Hokusetsu Area of Osaka].

One hundred and thirteen strains of Streptococcus pneumoniae (S. pneumoniae) were isolated from the clinical specimens of patients with respiratory tract infections between January and December 1998 in three hospitals in Hokusetsu area of Osaka. We investigated susceptibility of 113 strains of S.

Critical value for the index of body fat distribution based on waist and hip circumferences and stature in obese girls.

Objective: To determine the critical value for the standard deviation score (SDS) of waist-hip ratio (WHR)/height (Ht), as an age-adjusted measure of body fat distribution, in relation to occurrence of biochemical complications in obese girls.

Design: Cross-sectional, clinical study. The (WHR/Ht)-SDS was calculated as described previously.

A case of monoclonal gammopathy associated with acute myelomonocytic leukemia with eosinophilia suggested to be the result of lineage infidelity.

Acute myelomonocytic leukemia (AMMoL) accompanied by monoclonal gammopathy is a rare condition, and its pathogenesis and the cytogenetic mechanism of such leukemogenesis have not been determined in detail. A case of AMMoL with eosinophilia accompanied by immunoglobulin G kappa monoclonal gammopathy is described. Immunophenotypic studies of the peripheral blood and bone marrow mononuclear cells revealed no evidence of abnormally proliferating cells of B-lineage.