原発性副甲状腺機能亢進症, 尿管ポリープおよび腎盂結石を伴ったKlinefelter症候群の1例

副甲状腺機能亢進症, 右尿管ポリープ, および右腎盂結石を合併したKlinefelter症候群の1例を経験し, その臨床経過を報告するとともに文献的考察を加えた。〔症例〕 55歳男性で右側腹部痛を主訴として来院した。理学的所見で外陰部は男性型であるが発育不良で両側睾丸は小さくeunuchoidal habitusを呈した。既往歴に右尿管結石で5回自然排石が認められたほかに, 16年前gynecomastiaのためmastectomyを受けた事がある。身長171 cm, arm span 177.8 cmで体毛は少なく染色体検査は47XXYでKlinefelter症候群と診断した。血清LH, FSHは高値でテストステロン値は低値を示した。排泄性腎盂造影で右水腎症と右腎孟結石が認められ, 逆行性腎盂造影により右尿管上部に水腎症の原因となる腫瘍による多発性陰影欠損がみられ, 尿細胞診も悪性腫瘍を疑わせたため, 右腎尿管全摘術を施行した。尿管腫瘍は組織学的には多発性の尿管ポリープであった。血清Ca, Pは正常範囲内であるがhypercalciuriaがみられ, %TRPも54%と低値で副甲状腺ホルモン値も高いため, 原発性副甲状腺機能亢進症を疑いcervical explorationを施行した。副甲状腺は, diffuse hyperplasiaを呈し3/4副甲状腺亜全摘術と胸腺摘出術を行った。摘出した胸腺内には異所性副甲状腺組織は認められなかった。文献的に本症候群には糖尿病, 甲状腺機能不全, 下垂体牲腺機能不全などのいくつかの内分泌機能障害が報告されている。しかし本症候群に副甲状腺機能充進を合併した報告は過去に1例をみるにすぎない。副甲状腺機能亢進症の診断上, %TRPと静脈カテーテルによる頸部静脈血のmultiple samplingによる副甲状腺ホルモン測定の意義について言及した。Herein is reported a case of 47 XXV-Klinefelter's syndrome associated with ureteral polyps, renal pelvic stone and primary hyperparathyroidism. To the best of our knowledge, this is the second patient reported to have Klinefelter's syndrome coexisting with primary hyperparathyroidism. Frequent endocrinological disorders in the patients with Klinefelter's syndrome and diagnostic problems for hyperparathyroidism and ureteral polyps are discussed

Host selection of hematophagous leeches (Haemadipsa japonica): Implications for iDNA studies

The development of an efficient and cost‐effective method for monitoring animal populations or biodiversity is urgently needed, and invertebrate‐derived DNA (iDNA) may offer a promising tool for assessing the diversity and other ecological information of vertebrates. We studied the host species of a hematophagous leech (Haemadipsa japonica) in Yakushima by genetic barcoding and compared the results with those for mammal composition revealed by camera trapping. We analyzed 119 samples using two sets of primers by Sanger sequencing and one set of primer by next generation sequencing. The proportion of the samples that were successfully sequenced and identified to at least one species was 11.8–24.3%, depending on the three different methods. In all of these three methods, most of the samples were identified as sika deer (18/20, 6/15 and 16/29) or human (2/20, 7/15 and 21/29). The nonhuman mammal host species composition was significantly different from that estimated by camera trapping. Sika deer was the main host, which may be related with their high abundance, large body size and terrestriality. Ten samples included DNA derived from multiple species of vertebrates. This may be due to the contamination of human DNA, but we also found DNA from deer, Japanese macaque and a frog in the same samples, suggesting the mixture of the two meals in the gut of the leech. Using H. japonica‐derived iDNA would not be suitable to make an inventory of species, but it may be useful to collect genetic information on the targeted species, due to their high host selectivity

CIN85 drives B cell responses by linking BCR signals to the canonical NF-κB pathway

CIN85 transduces B cell receptor signals to IKK-β, and its expression in B cells is essential for T cell–independent type II antibody responses in mice

Spatiality Preservable Factored Poisson Regression for Large-Scale Fine-Grained GPS-Based Population Analysis

With the wide use of smartphones with Global Positioning System (GPS) sensors, the analysis of the population from GPS traces has been actively explored in the last decade. We propose herein a brand new population prediction model to capture the population trends in a fine-grained point of interest (POI) densely distributed over large areas and understand the relationship of each POI in terms of spatiality preservation. We propose a new framework, called Spatiality Preservable Factorized Regression (SPFR), to realize this model. The SPFR is inspired by the success of the recently proposed bilinear Poisson regression and the concept of multi-task learning with factorization approach and the graph proximity regularization. Given that the proposed model is written simply in terms of optimization, we achieve scalability using our model. The results of our empirical evaluation, which used a massive dataset of GPS logs in the Tokyo region over 32 M count logs, show that our model is comparable to the stateof-the-art methods in terms of capturing the population trend across meshes while retaining spatial preservation in finer mesh areas

Protein kinase Cδ amplifies ceramide formation via mitochondrial signaling in prostate cancer cells

We studied the role of protein kinase C isoform PKCδ in ceramide (Cer) formation, as well as in the mitochondrial apoptosis pathway induced by anticancer drugs in prostate cancer (PC) cells. Etoposide and paclitaxel induced Cer formation and apoptosis in PKCδ-positive LNCaP and DU145 cells but not in PKCδ-negative LN-TPA or PC-3 cells. In contrast, these drugs induced mitotic cell cycle arrest in all PC cell lines. Treatment with Rottlerin, a specific PKCδ inhibitor, significantly inhibited drug-induced Cer formation and apoptosis in LNCaP cells, as did overexpression of dominant negative–type PKCδ. Overexpression of wild-type PKCδ had an opposite effect in PC-3 cells. Notably, etoposide induced biphasic Cer formation in LNCaP cells. The early and transient Cer increase resulted from de novo Cer synthesis, while the late and sustained Cer accumulation was derived from sphingomyelin hydrolysis by neutral sphingomyelinase (nSMase). Cer, in turn, induced mitochondrial translocation of PKCδ and stimulated the activity of this kinase, promoting cytochrome c release and caspase-9 activation. Furthermore, the specific caspase-9 inhibitor LEHD-fmk significantly inhibited etoposide-induced nSMase activation, Cer accumulation, and PKCδ mitochondrial translocation. These results indicate that PKCδ plays a crucial role in activating anticancer drug–induced apoptosis signaling by amplifying the Cer-mediated mitochondrial amplification loop

重複下大静脈を伴うACDK随伴腎細胞癌に対して腹腔鏡下根治的腎摘徐術を施行した1例

A 59-year-old male patent who had undergone chronic dialysis for 13 years presented with gross hematuria. Radiological examinations showed a cystic renal tumor in the left kidney, multiple renal cysts due to acquired cystic disease of the kidney (ACDK), and a duplicated inferior vena cava (IVC). Although we suspected that the branches of the left IVC might be anormalous with regard to number and location, we could not obtain information about the left renal vein by 3-dimensional computed tomography because of the decreased blood flow in the end-stage kidney. Laparoscopic radical nephrectomy was performed using a transperitoneal approach. We first identified the left IVC and then exposed its surface widely so that we could identify the veins draining into it. We identified and divided two renal veins and also identified an adrenal vein and a gonadal vein draining directly into the left IVC. The enlarged kidney with multiple renal cysts was removed in a purely laparoscopic procedure.症例は13年間透析を行っている59歳, 男性。血尿を主訴に受診し, 画像診断で左腎上極の嚢胞性腫瘤, ACDKによる多発性の腎嚢胞, そして重複下大静脈を認めた。腎静脈を含む左下大静脈からの分枝の数や位置に関して先天的な異常の可能性があると考えられたが, 3次元 CTによる検討では終末腎のため腎血流が減少し, 左腎静脈の情報は得られなかった。経腹膜的に腹腔鏡下根治的腎摘除術を施行した。左下大静脈を最初に同定しその表面に沿って広い範囲で剥離することにより, 左下大静脈に流入する静脈を同定することができた。2本の腎静脈とさらに左下大静脈に直接流入する副腎静脈と性腺静脈を確認できた。腎嚢胞が多発し腫大した腎臓であったが, pure laparoscopicな手技で切除することができた

A Case Report of Renal Cell Carcinoma Producing Alpha-Fetoprotein

We report a rare autopsy case of alpha-fetoprotein producing renal cell carcinoma with multiple metastases to liver, gallbladder, mesenterium, and paraaortic lymphnodes. A 58-year-old male patient was diagnosed as having carcinoma of the right kidney. On admission, the patient showed increased serum level of alphafetoprotein (326 ng/ml) which decreased after resection of the renal tumor. The resected tumor was composed mainly of clear cell type of carcinoma cells and partially of granular cells. At autopsy, the main cell type of the metastatic foci was granular type of carcinoma cells. Immunohistochemistry of alpha-fetoprotein by PAP method revealed more positive reaction on granular cell type of carcinoma cells than clear cell type of carcinoma cells in the primary site of the kidney as well as in the metastatic foci