Recurrent pneumonia with mild hypogammaglobulinemia diagnosed as X-linked agammaglobulinemia in adults

<p>Abstract</p> <p>Background</p> <p>X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency caused by disruption of the Bruton's tyrosine kinase (BTK) gene. Typical XLA patients suffer recurrent and severe bacterial infections in childhood.</p> <p>Methods</p> <p>Flow cytometric analysis of the peripheral monocytes using the anti-BTK antibody was used to characterize a 27 year old male patient with mild hypogammaglobulinemia (IgG, 635 mg/dl; IgM, 11 mg/dl; IgA, <5 mg/dl). He had suffered from frequent pneumonia since age 25 but had no history of frequent infections in his childhood or in adolescence. Sequencing of the BTK cDNA obtained from an Epstein–Barr virus-transformed B lymphoblastoid cell line derived from the bone marrow of the patient was performed to confirm a genetic defect.</p> <p>Results</p> <p>Flow cytometric analysis of cytoplasmic BTK protein in peripheral monocytes indicated that the patient presents a rare case of adult-onset XLA and that his mother is an XLA carrier. Sequencing of the BTK gene revealed a deletion of AG in the codon for Glu605 (AGT), resulting in an aberrant stop codon that truncates the BTK protein in its kinase domain.</p> <p>Conclusions</p> <p>This case suggests that some XLA cases may remain undiagnosed because they only show mild hypogammaglobulinemia and they lack repeated infections in childhood. Flow cytometric analysis is a powerful method to screen these patients.</p

Gender representations in East Asian advertising: Hong Kong, Japan, and Shouth Korea

Gender representations in East Asian advertising: Hong Kong, Japan, and South Korea Abstract Gender representations in television advertisements have been a subject of academic research for many years. However, comparatively few studies have looked into television advertising’s gender representations in Confucian societies, particularly from a comparative perspective. This study compares the representation of males and females in 1,694 television advertisements from Hong Kong, Japan, and South Korea. It uncovers stereotypical gender representations related to age (females were predominantly young, males were middleaged), clothing/nudity (females were more suggestively dressed, males were fully clothed), work (females were depicted more often at home, males were typically depicted in the workplace), authority (males were used for voiceovers more than females, with males being the so-called “voice of authority”), and beauty (more females than males advertised for the cosmetics/toiletries product category). Overall, gender representations were highly stereotypical in all three cultures, which may be due to a shared common cultural background based on Confucianism. In terms of the degree of gender stereotyping, Hong Kong was more gender-egalitarian than Japan and South Korea; this finding is consistent with results from Project Globe’s gender egalitarianism index and the Gender-related Development Index (GDI) by the United Nations, but not with Hofstede’s masculinity index. These results suggest a relationship between gender representations and some gender indices. Finally, this article discusses the possible effects of stereotypical gender representations on audiences in relation to social cognitive and cultivation theories

通気量と塩分勾配がオニオコゼ仔稚魚の生残と成長に与える影響

We examined the effects of a flow field in the rearing tank exhibited by different aeration rate and salinity gradient on the larviculture of the devil stinger Inimicus japonicus. Two different rearing experiments using 1 kl rearing tanks were conducted from hatching (day 0) to settlement. In the Experiment 1, fish were reared until day 21 in 5 different aeration rates (0-1200 ml/min). There was a significant and positive relationship between survival andaeration rate, and fish survival became stable at an aeration rate of more than 300 ml/min. In Experiment 2, the salinity gradient in a rearing tank was formed by pumping brackish water (22 ppt) from the surface and seawater (34 ppt) from the bottom at the same time. Control rearing tanks (34 ppt) were aerated at 300 ml/min and fish were reared until day 23. Growth and development was synchronized in the salinity gradient tanks with little standard deviation, although average survival at settlement in the salinity gradient tank (47.3 %) was lower than the aeration tank (68.2 %). The optimal aeration rate for devil stinger larviculture is stronger than 300 ml/min and introducing a salinity gradient can be a novel approach for enhancing performance of the fish.異なる通気量と塩分勾配がオニオコゼ仔魚飼育に与える影響を調べた。容量1klの円形水槽で2つの飼育実験を孵化仔魚（0日齢）より着底まで実施した。実験1では5つの異なる通気量（0-1200 ml/min）で21日齢までの飼育を行った。その結果，最終日の生残と通気量の間に有意な正の相関が検出され，300ml/min以上の通気量で生残が安定した。実験2では飼育水槽に水面から22pptの汽水，底面から34pptの海水をそれぞれ連続注入することで塩分勾配を形成させ，対照に34ppt海水に300ml/minの通気を施す水槽を設け，23日齢まで飼育した。その結果，塩分勾配水槽で飼育した魚の成長と着底が同調したが，飼育終了時の生残（47.3 %）は対照のそれ（68.2 %）より低い傾向があった。1kl規模でのオニオコゼの仔魚飼育には300ml/min以上の強い通気による水流が必要であることが明らかになり，さらに塩分勾配の飼育水槽への導入により種苗生産に新たな可能性を見いだした

Sensitivity of CT perfusion for the diagnosis of cerebral infarction

We aimed to determine the sensitivity of CT perfusion (CTP) for the diagnosis of cerebral infarction in the acute stage. We retrospectively reviewed patients with ischemic stroke who underwent brain CTP on arrival and MRI-diffusion weighted image (DWI) after hospitalization between October 2008 and October 2011. Final diagnosis was made from MRI-DWI findings and 87 patients were identified. Fifty-five out of 87 patients (63%) could be diagnosed with cerebral infarction by initial CTP. The sensitivity depends on the area size (s) : 29% for S<3 cm2, 83% for S≥3 cm2-<6 cm2, 88% for S≥6 cm2-<9 cm2, 80% for S≥9 cm2-<12 cm2, and 96% for S≥12 cm2 (p<0.001). Sensitivity depends on the type of infarction : 0% for lacunar, 74% for atherothrombotic, and 92% for cardioembolism (p<0.001). Sensitivity is not correlated with hours after onset. CT perfusion is an effective imaging modality for the diagnosis and treatment decisions for acute stroke, particularly more serious strokes

Relationship of flow-volume curve pattern on pulmonary function test with clinical and radiological features in idiopathic pulmonary fibrosis.

Background:The flow-volume (FV) curve pattern in the pulmonary function test (PFT) for obstructive lung diseases is widely recognized. However, there are few reports on FV curve pattern in idiopathic pulmonary fibrosis (IPF). In this study, we investigated the relationship between FV curve pattern and clinical or radiological features in IPF.Methods:The FV curves on PFTs and chest high-resolution computed tomography (HRCT) images of 130 patients with IPF were retrospectively evaluated. The FV curves were divided into four groups based on the presence or absence of the convex and concave patterns: convex/concave, non-convex/concave, convex/non-concave, and non-convex/non-concave. Using a computer-aided system, CT honeycombing area (%HA) and subtracted low attenuation area (%sLAA) were quantitatively measured. To assess the distribution of CT findings, the lung area was divided into upper, lower, central, and peripheral areas. The relationships of FV curve patterns with patient characteristics, spirometry results, and quantitative CT findings were evaluated.Results:The patients with convex pattern was identified in 93 (71.5%) and concave pattern in 72 (55.4%). Among the four groups, patients with the convex/non-concave pattern had significantly lower forced vital capacity (FVC) and higher %HA of the upper/peripheral lung area (p = 0.018, and p = 0.005, respectively). The convex/non-concave pattern was a significant predictor of mortality for IPF (hazard ratio, 2.19; p = 0.032).Conclusions:Patients with convex/non-concave pattern in FV curve have lower FVC and poorer prognosis with distinct distribution of fibrosis. Hence, FV curve pattern might be a useful predictor of mortality in IPF