Changes in beef sensory traits as somatic-cell-cloned Japanese black steers increased in age from 20 to 30 months.

Changes in sensory traits of longissimus muscle (LM) from 20-30-month-old cattle were investigated using somatic cell clones of Japanese black steers slaughtered at 20-, 25- and 30-months-old (n=3, 4 and 4 respectively). The fat content of LM samples at 20, 25 and 30 months were 23.7, 38.

Inflammatory changes in infantile-onset LMNA-associated myopathy.

Mutations in LMNA cause wide variety of disorders including Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophy, and congenital muscular dystrophy. We recently found a LMNA mutation in a patient who was previously diagnosed as infantile onset inflammatory myopathy. In this study, we screened for LMNA mutations in 20 patients suspected to have inflammatory myopathy with onset at 2years or younger.

Profiles of blood biomarkers in alternating hemiplegia of childhood--increased MMP-9 and decreased substance P indicates its pathophysiology.

Alternating hemiplegia of childhood (AHC) is a rare disorder characterized by repeated plegic attacks, movement disorders, autonomic phenomena, and developmental delay. To obtain insights into the pathophysiology of AHC, we determined the concentrations of matrix metalloproteinase-9 (MMP-9), tissue inhibitor of MMP-1 (TIMP-1), calcitonin gene-related peptide (CGRP), and substance P (SP) in the serum/plasma of AHC patients (n=6) and control subjects (n=11) by performing enzyme-linked immunosorbent assay (ELISA). Decreased levels of serum SP (382±161 pg/ml), increased levels of plasma MMP-9 (111.

Uteroglobulin-related protein 1 and severity of respiratory syncytial virus infection in children admitted to hospital.

There are several reports suggesting that genetic factors contribute to the severity of infection with the respiratory syncytial virus (RSV). Infants hospitalized with lower respiratory tract infection (LRTI) due to RSV are at a significantly increased risk for both recurrent wheezing and childhood asthma. Uteroglobin-related protein 1 (UGRP1) is a secretory protein expressed in the airways, and speculated to have anti-inflammatory activity.

Quantification of myocardial blood flow using model based analysis of first-pass perfusion MRI: extraction fraction of Gd-DTPA varies with myocardial blood flow in human myocardium.

For the absolute quantification of myocardial blood flow (MBF), Patlak plot-derived K1 need to be converted to MBF by using the relation between the extraction fraction of gadolinium contrast agent and MBF. This study was conducted to determine the relation between extraction fraction of Gd-DTPA and MBF in human heart at rest and during stress. Thirty-four patients (19 men, mean age of 66.

Influence of the FID and off-resonance effects in dense MRI.

Accurate functional measurement in cardiovascular diseases is important as inaccuracy may compromise diagnostic decisions. Cardiac function can be assessed using displacement encoding with stimulated echoes, resulting in three signal components. The free induction decay (FID), arising from spins undergoing T(1) -relaxation, is not displacement encoded and impairs the displacement acquired.

Diagnostic accuracy of 1.5-T unenhanced whole-heart coronary MR angiography performed with 32-channel cardiac coils: initial single-center experience.

Purpose: To compare the imaging time and image quality obtained with whole-heart coronary magnetic resonance (MR) angiography performed with five- and 32-channel coils in healthy subjects and determine the accuracy of MR angiography performed with 32-channel coils in the detection of obstructive coronary artery disease (CAD).

Materials And Methods: The institutional review board approved the study protocol, and all participants provided written informed consent. The authors studied 10 healthy subjects and 67 patients suspected of having CAD who were scheduled for coronary angiography.

Analysis of API2-MALT1 fusion, trisomies, and immunoglobulin VH genes in pulmonary mucosa-associated lymphoid tissue lymphoma.

Pulmonary mucosa-associated lymphoid tissue lymphoma is unique in that chronic inflammation is rare and that API2-MALT1 fusion, resulting from t(11;18)(q21;q21), occurs frequently. In this study, we examined 20 cases for API2-MALT1 fusion using the multiplex reverse-transcription polymerase chain reaction and looked for trisomy 3, trisomy 18, and abnormalities of MALT1 and IGH genes using fluorescence in situ hybridization. In addition, we analyzed VH genes by subcloning of the monoclonal polymerase chain reaction products.

A case of congenital axonal neuropathy associated with West syndrome.

We report the case of an 11-month-old girl with congenital axonal neuropathy and West syndrome. She had generalized hypotonia and an abnormal posture since birth, and apparently, her development was stalled. Deep tendon reflexes were absent, and at 5 months of age, she developed West syndrome followed by refractory seizures.

In vivo SNR in DENSE MRI; temporal and regional effects of field strength, receiver coil sensitivity and flip angle strategies.

Aim: The influences on the signal-to-noise ratio (SNR) of Displacement ENcoding with Stimulated Echoes (DENSE) MRI of field strength, receiver coil sensitivity and choice of flip angle strategy have been previously investigated individually. In this study, all of these parameters have been investigated in the same setting, and a mutual comparison of their impact on SNR is presented.

Materials And Methods: Ten healthy volunteers were imaged in a 1.

[Hereditary spastic paraplegia associated with congenital cataracts, mental retardation and peripheral neuropathy].

A 16-year-old male patient was admitted to our hospital with mental retardation and a gradually increasing gait disturbance. He fell easily at age 6, and lost the ability to jump at age 12. At age 13, he lost the ability to run, and developed pes cavus and hammer toes.

Behçet's disease (Adamantiades-Behçet's disease).

Adamantiades-Behçet's disease (ABD) is characterized by starting with oral aphthous ulceration and developing of the systemic involvements. The pathogenesis of ABD is closely correlated with the genetic factors and the triggering factors which acquire delayed-type hypersensitivity reaction against oral streptococci mediated by IL-12 cytokine family. HLA-B51 is associated in more than 60% of the patients and its restricted CD8+ T cell response is clearly correlated with the target tissues.

Multiple band frequency analysis in a child of medial temporal lobe ganglioglioma.

We report a 1-year 6-month-old girl with ganglioglioma in the right medial temporal lobe who showed epileptic spasms in clusters. Spasms occasionally followed a dazed and fearful gaze. Interictal electroencephalography (EEG) showed diffuse bursts of slightly irregular high-voltage spikes and slow waves without hypsarrhythmia.

Assessment of coronary artery disease using magnetic resonance coronary angiography: a national multicenter trial.

Objectives: This national multicenter study determined the diagnostic performance of 1.5-T whole-heart coronary magnetic resonance angiography (MRA) in patients with suspected coronary artery disease (CAD).

Background: Whole-heart coronary MRA using steady-state free precession allows noninvasive detection of CAD without the administration of contrast medium.

CD4+ CD25(high) regulatory T cell in childhood ocular myasthenia gravis.

Dysfunction of CD4(+)CD25(+) regulatory T cell (Treg) has been demonstrated to play an important role in the development of autoimmune myasthenia gravis. This T cell subset, which has potent regulatory properties against immune response, has been reported to have a numerical or functional defect in patients with myasthenia gravis. We examined various T cell subsets, including CD4(+)CD25(+)Treg in peripheral blood mononuclear cells using flow cytometry in a pediatric patient suffering from ocular myasthenia gravis.

Effects of low-dose hydrochlorothiazide on urolithiasis and bone metabolism in severely disabled individuals: a pilot study.

To clarify the effects of hydrochlorothiazide (HCT) on calcium metabolism in subjects with severe motor and intellectual disabilities (SMID), we examined four patients (16-48years old) with a history of urolithiasis and/or bone fracture and increased urinary calcium/creatinine ratio (U-Ca/Cr). U-Ca/Cr, blood markers of bone turnover, and bone-mineral density (BMD) were measured before and after administration of low-dose HCT (0.25-0.

Acute nonparaneoplastic limbic encephalitis in childhood: a case series in Japan.

Limbic encephalitis not associated with malignancy was investigated in Japanese children, with particular focus on clinical features distinct from adult cases. Clinical, laboratory, and radiographic findings were studied in pediatric nonparaneoplastic limbic encephalitis, based on a literature review and questionnaire-based analyses. Analysis of 14 cases revealed the predominance of seizure occurrence, disturbance in consciousness, and frequent extralimbic signs.

Hypertension impairs myocardial blood perfusion reserve in subjects without regional myocardial ischemia.

Quantitative analysis of myocardial perfusion MRI can provide noninvasive assessments of myocardial perfusion reserve (MPR), which is associated with endothelial function. Endothelial function is influenced by various factors, including hypertension, diabetes, dyslipidemia, renal dysfunction and anemia. The purpose of this study was to evaluate which risk factor is the strongest effector of MPR in subjects without regional myocardial ischemia.

Bromoderma in a patient with migrating partial seizures in infancy.

A 5-month-old boy with migrating partial seizures in infancy acquired bromoderma 4 weeks after initiating potassium bromide (KBr) therapy (60mg/kg/day). Erythematous pustules appeared on the forehead within a few days of attaching electroencephalography electrodes, and on the cheek at the site of nasogastric tube fixation. These later developed into vegetant plaques.

Augmented startle responses in opsoclonus-myoclonus syndrome.

We report a 3-year-old boy with opsoclonus-myoclonus syndrome (OMS) who presented with exaggerated startle responses to unexpected auditory stimuli during an episode of myoclonic status. An augmented blink reflex was also observed clinically and electrophysiologically. Based on the assumption that hyperexcitability in the lower pontine tegmentum may be responsible for the acoustic startle and blink reflex in OMS, we considered that increased excitability of independent but neighboring structures, including the pontine paramedian reticular formation, may cause OMS symptoms.

Analysis of VH gene rearrangement and somatic hypermutation in Sjogren's syndrome and IgG4-related sclerosing sialadenitis.

IgG4-related sclerosing sialadenitis is currently considered as an autoimmune disease distinct from Sjogren's syndrome (SS) and responds extremely well to steroid therapy. To further elucidate the characteristics of IgG4-related sclerosing sialadenitis, we analysed VH fragments of IgH genes and their somatic hypermutation in SS (n = 3) and IgG4-related sclerosing sialadenitis (n = 3), using sialolithiasis (n = 3) as a non-autoimmune control. DNA was extracted from the affected inflammatory lesions.

Evolution of hemiplegic attacks and epileptic seizures in alternating hemiplegia of childhood.

To delineate the evolution of non-epileptic and epileptic paroxysmal events in alternating hemiplegia of childhood (AHC), we reviewed clinical information of nine patients (4-40 years) with AHC. Paroxysmal abnormal ocular movements, head turning, and tonic, clonic, or myoclonic limb movements were the initial symptoms (birth-8m) in each patient. Ictal electroencephalography (EEG) of these episodes, as well as hemiplegic periods that accompanied these symptoms later in infancy showed unremarkable findings or generalized slow background activity.

Electrophysiological evidence of cerebral dysfunction in childhood opsoclonus-myoclonus syndrome.

To address cerebral involvement in childhood opsoclonus-myoclonus syndrome (OMS), electrophysiological investigations including electroencephalograms and evoked potentials were performed in three children affected by nonparaneoplastic OMS. Most patients displayed abnormalities in visual- and somatosensory-evoked potentials, consisting of delayed latency or disorganization of cortical components. Symptoms of OMS have been attributed primarily to an abnormality in the cerebellum and its associated pathway.