Surgical treatment of aortic coarctation associated with multi-vessel brachiocephalic involvement in takayasu's arteritis.

In Takayasu's arteritis (TA), both atypical coarctation (CO) and brachiocephalic involvement are common features that occasionally require operative correction. A combination of these abnormalities could duplicate underlying illness in patients, posing an increased risk of operative morbidity. We present, herein, two TA patients in which hypertensive heart disease secondary to CO was surgically corrected. Both patients had multi-vessel brachiocephalic disease. One patient who showed occlusion of all brachiocephalic arteries underwent aorto-aortic bypass, while another with two-vessel lesion underwent axillo-bifemoral bypass grafting. Subclavian reconstruction was supplemental to each procedure, resulting in relief of neurologic stigmata. Strategies to avoid intraoperative cerebral ischemia played an important role in the surgical repair of such TA-related extensive vascular lesions.     

Inguinal herniation in two patients with continuous ambulatory peritoneal dialysis

We report two cases of subacute inguinal swelling in uremic patients on continuous ambulatory peritoneal dialysis (CAPD). Computed tomography, scintigraphy demonstrated a mass in the right groin. Surgical repair of an inguinal hernia resulted in complete resolution of the inguinal swelling. Both patients could restart continuous ambulatory peritoneal dialysis, without complication.     

Clinical characteristics after surgery of non-small cell lung cancer which measures 20 mm or less in diameter

Today's advances in diagnostic image-technologies often enable us to find small lung cancers. However, we have few definite strategies including how to diagnosis and treat them. In this study, we performed a retrospective analysis of 122 consecutive patients who underwent surgery for non-small cell lung cancer 20 mm or less in diameter to clarify the clinical features of small lung cancer. Of 122 patients, there were 114 patients of pN0, and 8 patients with lymph node metastasis. Seventy three patients underwent lobectomy, 45 underwent segmentectomy, and 4 underwent wedge resection based on the findings of preoperative CT and anatomical and oncological view during operation. Overall survival rate( OS) and progression free survival( PFS) at 3-year was, 94% and 84%, respectively. There were no differences in OS or PFS between lobectomy group and limited resection group, which might suggest that we adapted appropriate surgical procedures. Multivariate analysis revealed that pathological pleural invasion, lymphatic vessel invasion, and vascular vessel invasion were likely to be unfavorable prognostic-factors. We believe that further investigations should be required to clarify the characteristics of small lung cancer.     

Detection of sub-centimeter lesions using digital TOF-PET/CT system combined with Bayesian penalized likelihood reconstruction algorithm

Annals of Nuclear Medicine - Many advances in PET/CT technology can potentially improve image quality and the ability to detect small lesions. A new digital TOF-PET/CT scanner based on silicon...     

Evaluation of PiB visual interpretation with CSF Aβ and longitudinal SUVR in J-ADNI study

The objectives of the present study were to investigate (1) whether trinary visual interpretation of amyloid positron emission tomography (PET) imaging (negative/equivocal/positive) reflects quantitative amyloid measurements and the time course of 11C-Pittsburgh compound B (PiB) amyloid accumulation, and (2) whether visually equivocal scans represent an early stage of the Alzheimer’s disease (AD) continuum in terms of an intermediate state of quantitative amyloid measurements and the changes in amyloid accumulation over time. From the National Bioscience Database Center Human Database of the Japanese Alzheimer’s Disease Neuroimaging Initiative, we selected 133 individuals for this study including 33 with Alzheimer’s disease dementia (ADD), 52 with late mild cognitive impairment (LMCI), and 48 cognitively normal (CN) subjects who underwent clinical assessment, PiB PET, and structural magnetic resonance imaging (MRI) with 2 or 3-years of follow-up. Sixty-eight of the 133 individuals underwent cerebrospinal fluid amyloid-β1-42 (CSF-Ab42) analysis at baseline. The standard uptake value ratio (SUVR) of PiB PET was calculated with a method using MRI at each visit. The cross-sectional values, longitudinal changes in SUVR, and baseline CSF-Ab42 were compared among groups, which were categorized based on trinary visual reads of amyloid PET (negative/equivocal/positive). From the trinary visual interpretation of the PiB PET images, 55 subjects were negative, 8 were equivocal, and 70 were positive. Negative interpretation was most frequent in the CN group (70.8/10.4/18.8%: negative/equivocal/positive), and positive was most frequent in the LMCI group (34.6/1.9/63.5%) and in the ADD group (9.1/6.1/84.8%). The baseline SUVRs were 1.08 ± 0.06 in the negative group, 1.23 ± 0.15 in the equivocal group, and 1.86 ± 0.31 in the positive group (F = 174.9, p < 0.001). The baseline CSF-Ab42 level was 463 ± 112 pg/mL in the negative group, 383 ± 125 pg/mL in the equivocal group, and 264 ± 69 pg/mL in the positive group (F = 37, p < 0.001). Over the 3-year follow-up, annual changes in SUVR were − 0.00 ± 0.02 in the negative group, 0.02 ± 0.02 in the equivocal group, and 0.04 ± 0.07 in the positive group (F = 8.4, p < 0.001). Trinary visual interpretation (negative/equivocal/positive) of amyloid PET imaging reflects quantitative amyloid measurements evaluated with PET and the CSF amyloid test as well as the amyloid accumulation over time evaluated with PET over 3 years. Subjects in the early stage of the AD continuum could be identified with an equivocal scan, because they showed intermediate quantitative amyloid PET, CSF measurements, and the amyloid accumulation over time.     

Objective assessment of facial skin aging and the associated environmental factors in Japanese monozygotic twins

Twin studies, especially those involving monozygotic (MZ) twins, facilitate the analysis of factors affecting skin aging while controlling for age, gender, and genetic susceptibility. The purpose of this study was to objectively assess various features of facial skin and analyze the effects of environmental factors on these features in MZ twins. At the Osaka Twin Research Center, 67 pairs of MZ twins underwent medical interviews and photographic assessments, using the VISIA^® Complexion Analysis System. First, the average scores of the right and left cheek skin spots, wrinkles, pores, texture, and erythema were calculated; the differences between the scores were then compared in each pair of twins. Next, using the results of medical interviews and VISIA data, we investigated the effects of environmental factors on skin aging. The data were analyzed using Pearson's correlation coefficient test and the Wilcoxon signed‐rank test. The intrapair differences in facial texture scores significantly increased as the age of the twins increased (P = 0.03). Among the twin pairs who provided answers to the questions regarding history differences in medical interviews, the twins who smoked or did not use skin protection showed significantly higher facial texture or wrinkle scores compared with the twins not exposed to cigarettes or protectants (P = 0.04 and 0.03, respectively). The study demonstrated that skin aging among Japanese MZ twins, especially in terms of facial texture, was significantly influenced by environmental factors. In addition, smoking and skin protectant use were important environmental factors influencing skin aging.     

A HIGH-GRADE MALIGNANCY BRONCHIAL MUCOEPIDERMOID CARCINOMA WITH FEATURES OF GIANT CELL CARCINOMA

A high-grade mucoepidermoid carcinoma associated with giant cell carcinoma of the bronchus was found in a 41-year-old man. Light and electron microscopic examinations showed glandular and squamous elements. The histology of the giant cell carcinoma was similar to those of previously reported giant cell carcinomas. It was assumed that the giant cell carcinoma in the present case is the result of transformation of mucoepidermoid carcinoma originating in the bronchial gland.     

Geographic distribution of specialist orthodontists and orthodontic providers in Japan

ObjectiveThis study aimed to clarify the geographic distribution of specialist orthodontists and dentists who provide orthodontic services in Japan.MethodsWe obtained data on the populations of 1750 municipalities in Japan in 2010 by referring to the census. We obtained data on the number of dentists who mainly provide orthodontic services (specialist orthodontists) and the number of dentists, including general dentists, who provide orthodontic services (orthodontic providers), by referring to the Survey of Physicians, Dentists, and Pharmacists. Furthermore, we referred to the directory on the website of the Japanese Orthodontic Society (JOS) to obtain data on JOS-qualified orthodontists. To assess the distribution of specialist orthodontists and orthodontic providers, we used Lorenz curves and Gini coefficients.ResultsThe median value for the number of specialist orthodontists and number of JOS-certified orthodontists per 100,000 persons aged between 5 and 40 years old was 0, while that of orthodontic providers was 27.5. Gini coefficients for specialist orthodontists and JOS-certified orthodontists were 0.523 and 0.615, respectively. On the other hand, the Gini coefficient for orthodontic providers was 0.258.ConclusionsRegional inequalities in the availability of specialist orthodontists are high, and medical access to specialist orthodontic services may be limited in areas other than urban districts. In municipalities with a population of fewer than 50,000 inhabitants, the number of specialist orthodontists was very low, but orthodontic providers were relatively evenly distributed. Our research results suggested that studying the distribution of specialist orthodontists and orthodontic providers can provide valuable information for developing dental care policies.     

Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report

Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p.R249*) in FLAD1. In the asymptomatic male infant born at full term, newborn screening showed positive results with elevated C5 and C14:1 acylcarnitine levels and an increased C14:1/C2 ratio. Biochemical studies were unremarkable except for lactic acidosis (pH 7.197, lactate 61 mg/dL). A diagnosis of MADD was suspected because of mild abnormalities of the acylcarnitine profile and apparent abnormalities of urinary organic acids, although mutations in the ETFA, ETFB, ETFDH, and riboflavin transporter genes (SLC52A1, SLC52A2, and SLC52A3) were not detected. Administration of riboflavin and L-carnitine was initiated at one month of age based on the diagnosis of “biochemical MADD” despite a lack of symptoms. Nevertheless, the acylcarnitine profile was not normalized. Symptoms resembling bulbar palsy, such as vocal cord paralysis and dyspnea with stridor, were present from 3 months of age. At 4 months of age, he became bedridden because of hypoxic-ischemic encephalopathy due to fulminant respiratory failure with aspiration pneumonia. At 2 years and 5 months of age, a homozygous c.745C > T (p.R249*) mutation in the FLAD1 gene was identified, confirming the diagnosis of FADS deficiency. His severe clinical course may be caused by this nonsense mutation associated with poor responsiveness to riboflavin. Persistent lactic acidosis and neuropathy, such as bulbar palsy, may be important for diagnosing FADS deficiency. Although the biochemical findings in FADS deficiency are similar to those in MADD, their clinical symptoms and severity may not be identical.