記述言語：英語   掲載種別：研究論文（学術雑誌）  

We performed 3 kidney biopsies in a 71-year-old man. At the first biopsy, we made the diagnosis of immunoglobulin G4 (IgG4)-related interstitial nephritis characterized by the simultaneous presence of IgG4-positive plasma cells and characteristic fibrosis with a bird's-eye pattern. At the second biopsy, rather than finding fibrosis as a post-inflammatory scar, we noted that steroid treatment had caused the simultaneous disappearance of IgG4-positive plasma cells and fibrosis and had restored the normal tubular structure. The third biopsy showed the recurrence of the disease with inflammatory cells accompanied by fibrosis. These findings suggest that IgG4-positive plasma cells and fibrosis occur simultaneously.

DOI： 10.2169/internalmedicine.1121-22

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掲載種別：研究論文（学術雑誌）   出版者・発行元：MDPI AG  

The histopathological findings of the glomeruli from whole slide images (WSIs) of a renal biopsy play an important role in diagnosing and grading kidney disease. This study aimed to develop an automated computational pipeline to detect glomeruli and to segment the histopathological regions inside of the glomerulus in a WSI. In order to assess the significance of this pipeline, we conducted a multivariate regression analysis to determine whether the quantified regions were associated with the prognosis of kidney function in 46 cases of immunoglobulin A nephropathy (IgAN). The developed pipelines showed a mean intersection over union (IoU) of 0.670 and 0.693 for five classes (i.e., background, Bowman’s space, glomerular tuft, crescentic, and sclerotic regions) against the WSI of its facility, and 0.678 and 0.609 against the WSI of the external facility. The multivariate analysis revealed that the predicted sclerotic regions, even those that were predicted by the external model, had a significant negative impact on the slope of the estimated glomerular filtration rate after biopsy. This is the first study to demonstrate that the quantified sclerotic regions that are predicted by an automated computational pipeline for the segmentation of the histopathological glomerular components on WSIs impact the prognosis of kidney function in patients with IgAN.

DOI： 10.3390/diagnostics12122955

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記述言語：日本語   出版者・発行元：日本医科大学医学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J04260&link_issn=&doc_id=20220831380006&doc_link_id=10.1272%2Fmanms.18.272&url=https%3A%2F%2Fdoi.org%2F10.1272%2Fmanms.18.272&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Serum amyloid A (SAA) is an acute phase protein, which undergoes structural changes and deposits in the extracellular matrix, causing organ damage. Systemic AA amyloidosis is a relatively common amyloid subtype among the more than 30 amyloid subtypes, but the mechanism of amyloid fibril formation remains unclear. In this study, we investigated the tissue distribution of SAA derived peptides in formalin-fixed paraffin embedded (FFPE) specimens of human myocardium with amyloidosis using matrix-assisted laser desorption/ionization imaging mass spectrometry (MALDI-IMS). In the whole SAA protein, four trypsin-digested peptides in the range of SAA2-67 were visualized and the N-terminal peptide; SAA2-15, was selectively localized in the Congo red-positive region. The C-terminal peptides; SAA47-62, SAA48-62, and SAA63-67 were detected not only in the Congo red-positive region but also in the surrounding negative region. Our results demonstrate that the N-terminal SAA2-15 plays a critical role in the formation of AA amyloid fibril, as previously reported. Roles of the C-terminal peptides require further investigation.

DOI： 10.1371/journal.pone.0275993

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記述言語：英語  

An 85-year-old man with a history of aortic dissection suddenly fainted, underwent cardiac heart arrest, and died. An autopsy was performed, but the cause of death was not grossly identified. Congo red staining detected amyloid deposits in systemic organs, including the heart, lungs, liver, and kidneys. Immunohistochemical (IHC) analysis revealed immunoglobulin (Ig) λ light chain (-λ) in systemic blood vessels and transthyretin (TTR) in the heart and lungs. Ig-λ was predominantly positive in the blood vessels of the lungs, while TTR was detected in the alveolar septum. In the heart, Ig-λ was positive in the endocardium and blood vessels, and TTR was positive in nodular deposits between cardiomyocytes. The concurrent deposition of Ig-λ and TTR in the heart was further substantiated by laser microdissection (LMD)-liquid chromatography-tandem mass spectrometry (LC-MS/MS) at each deposition site. Despite systemic deposition of Ig-λ, bone marrow biopsy findings were not diagnostic for multiple myeloma. In summary, we present an autopsy case of concurrent Ig-λ and TTR deposition as revealed by IHC and LC-MS/MS. When Congo red staining and IHC results are indeterminate due to the deposition of multiple amyloid proteins, LMD-LC-MS/MS is useful for determining the precursor protein.

DOI： 10.1111/pin.13179

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記述言語：日本語   出版者・発行元：(株)文光堂  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Overlap syndrome refers to a group of conditions that have clinical features of more than one well-characterised rheumatic disease and meet the respective classification criteria. There are no typical renal histological findings in overlap syndrome. When patients with overlap syndrome develop renal dysfunction, various potential causes, including lupus nephritis (LN), renal crisis by systemic sclerosis, interstitial nephritis, and so on, need to be distinguished. Here, we report a 44-year-old woman with overlap syndrome involving systemic lupus erythematosus (SLE), diffuse cutaneous systemic scleroderma, and Sjogren's syndrome, who was also positive for anti-mitochondrial M2 antibody. She developed glomerular haematuria, proteinuria, and increase in creatinine appeared gradually. Suspecting LN, renal biopsy was performed. However, in the interstitium, mild infiltration of lymphocytes and plasma cells and very partial fibrosis were observed. Immunofluorescence microscopy revealed predominant mesangial immunoglobulin M, C3, and λ light chain staining. Overall, LN was not diagnosed based on these findings. Renal dysfunction was normalised by glucocorticoid treatment for 3 months. This case suggests the importance of a renal diagnosis based on renal pathological findings, especially in a case of overlap syndrome including SLE.

DOI： 10.1093/mrcr/rxab017

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Histiocytic sarcoma is a relatively new disease category and the gastrointestinal origin is sporadic. We report a case of a 74-year-old woman who underwent chemotherapy and proximal gastrectomy for extremely rare, advanced gastric histiocytic sarcoma. The resected specimen was subjected to numerous immunostainings to meet the diagnostic criteria of histiocytic sarcoma and was positive for the histiocyte markers' cluster of differentiation 68 and lysozyme. The markers of Langerhans cells, follicular dendritic cells, and myelocyte were all negative. Six reports of surgical resection of histiocytic sarcoma originating in the stomach exist, including our case. We reviewed the clinical course and the histological and immunohistochemical diagnostic features of surgically resected gastric histiocytic sarcoma.

DOI： 10.1007/s12328-021-01438-y

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor (TKI) had clinical success in the treatment of non-small cell lung carcinoma (NSCLC). An effect of this drug on kidney has not been clarified and the occurrence of glomerulonephritis related to EGFR-TKI has rarely been reported. We present the case of a 71-year-old man with NSCLC who developed proteinuria and microscopic hematuria with the rise in a titer of MPO-ANCA, when 2 years and 3 months passed since the initiation of erlotinib, one of oral EGFR-TKI. Two serial biopsies support that ANCA-associated vasculitis may have been modified by the persistent use of erlotinib. We initiated intravenous pulse therapy with methylprednisolone followed by oral prednisone. The proteinuria has decreased and serum CRP was normalized. However, the serum creatinine level and hematuria did not change during the treatment period. While EGFR inhibition is implicated in protective control for glomerulonephritis, it may exacerbate vasculitis. Close monitoring of the kidney function and urinary findings is required during the use of EGFR inhibitors, such as erlotinib, because it may cause renal adverse events.

DOI： 10.1007/s13730-021-00632-8

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Recessive mutations in the Myosin regulatory light chain 2 (MYL2) gene are the cause of an infantile-onset myopathy, associated with fatal myocardial disease of variable macromorphology. We here present the first Japanese family affected with recessive MYL2 myopathy. Affected siblings manifested typical features and the proband's autopsy findings were compatible with the diagnosis of noncompaction cardiomyopathy. The rapidly progressive clinical course of this recessive MYL2 cardiomyopathy highlights the crucial role of c-terminal tails in MYL2 protein in maintaining cardiac morphology and function.

DOI： 10.1536/ihj.20-639

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Introduction: Diagnosing renal pathologies is important for performing treatments. However, classifying every glomerulus is difficult for clinicians; thus, a support system, such as a computer, is required. This paper describes the automatic classification of glomerular images using a convolutional neural network (CNN). Method: To generate appropriate labeled data, annotation criteria including 12 features (e.g., "fibrous crescent") were defined. The concordance among 5 clinicians was evaluated for 100 images using the kappa (κ) coefficient for each feature. Using the annotation criteria, 1 clinician annotated 10,102 images. We trained the CNNs to classify the features with an average κ ≥0.4 and evaluated their performance using the receiver operating characteristic-area under the curve (ROC-AUC). An error analysis was conducted and the gradient-weighted class activation mapping (Grad-CAM) was also applied; it expresses the CNN's focusing point with a heat map when the CNN classifies the glomerular image for a feature. Results: The average κ coefficient of the features ranged from 0.28 to 0.50. The ROC-AUC of the CNNs for test data varied from 0.65 to 0.98. Among the features, "capillary collapse" and "fibrous crescent" had high ROC-AUC values of 0.98 and 0.91, respectively. The error analysis and the Grad-CAM visually showed that the CNN could not distinguish between 2 different features that had similar visual structures or that occurred simultaneously. Conclusion: The differences in the texture or frequency of the co-occurrence between the different features affected the CNN performance; thus, to improve the classification accuracy, methods such as segmentation are required.

DOI： 10.1016/j.ekir.2020.11.037

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Aquagenic urticaria (AU) is a rare skin condition in which dermal contact with water serves as a trigger of urticaria and pruritus. To make a diagnosis, a water challenge test is useful. We herein report a 16-year-old Japanese boy diagnosed with AU by water provocation test. The induced skin lesion histologically showed prominent degranulation of mast cells and lymphocytic infiltration throughout the dermis. This is the second report documenting the histopathological features of AU.

DOI： 10.1111/1346-8138.15615

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記述言語：日本語   出版者・発行元：日本脳神経CI学会  

生前画像では異常と判断すべき所見であっても、死後画像では正常と判断すべき所見がある。臨床医もこのような死亡時画像診断におけるピットフォールを知っておく必要がある。本稿では、頭部の死亡時画像診断について、正常の死後変化所見や代表的な頭蓋内病変の所見を中心に、以下の項目で解説した。1)死亡時画像診断の社会的背景、2)死亡時画像診断の対象・撮影・画像の成り立ち、3)正常な頭部の早期死後CT所見、4)死後晩期の正常な頭部の死後CT所見、5)頭部の死後MRI、6)病的所見のある頭部の死後CT、7)その他の先進的な死後脳MRI研究。病的所見として、脳出血、くも膜下出血、低酸素虚血脳症、脳梗塞、凍死、脳の形態異常、焼死をとりあげた。

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

腎生検病理診断は、病型診断(光学顕微鏡的分類)、病因診断(蛍光抗体法、電子顕微鏡)を臨床経過と照らしあわせて病態を推定し、臨床医に説明する行為である。この診断行程が広く普及されるためには、所見の把握から臨床病態の推定に至るまで、その作業過程を標準化する必要があるとの考えから、腎生検病理診断取扱い規約第1版が出版された。本稿はこの取扱い規約の概要とともに、腎生検病理診断の考え方を概説した。(著者抄録)

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 49-year-old man was diagnosed with acute cardiac insufficiency based on evidence of congestive heart failure. The non-invasive measurement of the cardiac output using an AESCULON® mini showed low cardiac output (CO, 3.9 L/min). We administered an intravenous diuretic for cardiac edema and dobutamine drip for low cardiac output. Soon after starting dobutamine at 3.2 γ (microg/kg/min), the CO improved to 6.8 L/min. Combination therapy of diuretic and dobutamine resolved the heart failure. CO measurement by an AESCULON® mini was safe, cost-effective, and convenient. Data output correlates with the CO by Swan-Ganz catheterization. The non-invasive measurement of the CO permitted a smooth recovery without recurrence in this patient.

DOI： 10.2169/internalmedicine.4271-19

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

In this study, we compared the postmortem computed tomography (PMCT) findings among nonpathological lungs, lungs with bacterial pneumonia, and lungs with pulmonary edema in patients following non-traumatic in-hospital death. We studied 104 consecutive adult patients (208 lungs) who died in our tertiary care hospital and underwent PMCT and pathological autopsy (both within 2.5 days after death), and were pathologically diagnosed with nonpathological lungs, bacterial pneumonia, and pulmonary edema. Thirteen pulmonary features were assessed on the CT scans. We also examined the association between the time elapsed since death and the pulmonary findings. We observed increased lung opacities with horizontal plane formation, diffuse opacities, diffuse bronchovascular bundle thickening, symmetric opacities to the contralateral lung, and decreased segmental opacities with time elapsed since death (Cochran-Armitage test for trend). Multiple logistic regression revealed that the presence of opacities without horizontal plane formation or centrilobular opacities, and the absence of diffuse bronchovascular bundle thickening were associated with histopathological pneumonia, whereas the presence of opacities with horizontal plane formation, diffuse opacities, and interlobular septal thickening were associated with histopathological pulmonary edema. In conclusion, specific pulmonary PMCT findings increased with time elapsed since death, and some lung findings may facilitate the diagnosis of bacterial pneumonia and pulmonary edema.

DOI： 10.1016/j.legalmed.2020.101716

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

To evaluate the utility of unenhanced postmortem computed tomography (PMCT) for the investigation of in-hospital nontraumatic death in children up to 3 years of age.This study included the cadavers of children who died from intrinsic diseases before 3 years of age. The major underlying disease and the main organ-disease systems associated with the immediate causes of death were determined by clinical evaluation, PMCT, and autopsy, which were used as a reference standard. The rates of concordance between the former two methods and autopsy were calculated for all cases.In total, 22 cadavers (12 male and 10 female; mean age, 6.1 ± 8.2 months) were included. The rates of concordance between clinical evaluation/PMCT and autopsy for diagnosis of the major underlying disease and main organ-disease systems associated with the immediate causes of death were 100%/36% (P = .0015) and 59%/41% (P = .37), respectively. In cases where the respiratory system was associated with the immediate cause of death, PMCT showed greater diagnostic sensitivity (90%) than did clinical evaluation (20%). In contrast, the diagnostic sensitivity of PMCT was lower than that of clinical evaluation in cases involving disorders of the cardiac system and multiple organ systems (0% vs 100% for both).The findings of this study suggest that the use of unenhanced PMCT with clinical evaluation can result in improved detection of the immediate cause of death in select cases of in-hospital nontraumatic death before 3 years of age.

DOI： 10.1097/MD.0000000000020130

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Immune checkpoint inhibitors (CPIs), including pembrolizumab, are becoming common oncological treatments. CPIs have been associated with a significant risk of developing immune-related adverse events (irAEs), such as nephritis and interstitial nephritis. However, the occurrence of glomerulonephritis has only rarely been reported. We herein present the case of a 75-year-old woman with non-small cell lung carcinoma (NSCLC) who developed proteinuria and microscopic hematuria during treatment with pembrolizumab. Renal biopsy revealed tubulointerstitial nephritis and IgA nephropathy. Considering that a urinalysis showed no abnormality before treatment, the condition might have been induced by pembrolizumab. In this report, we focus on the correct diagnosis and management of renal irAEs, which remain controversial.

DOI： 10.2169/internalmedicine.3928-19

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担当区分：筆頭著者   記述言語：英語   掲載種別：研究論文（学術雑誌）  

To clarify the significance of quantitative analyses of amyloid proteins in clinical practice and in research relating to systemic amyloidoses, we applied mass spectrometry-based quantification by isotope-labeled cell-free products (MS-QBIC) to formalin-fixed, paraffin-embedded (FFPE) tissues. The technique was applied to amyloid tissues collected by laser microdissection of Congo red-stained lesions of FFPE specimens. Twelve of 13 amyloid precursor proteins were successfully quantified, including serum amyloid A (SAA), transthyretin (TTR), immunoglobulin kappa light chain (IGK), immunoglobulin lambda light chain (IGL), beta-2-microglobulin (B2M), apolipoprotein (Apo) A1, Apo A4, Apo E, lysozyme, Apo A2, gelsolin, and fibrinogen alpha chain; leukocyte cell-derived chemotaxin-2 was not detected. The quantification of SAA, TTR, IGK, IGL, and B2M confirmed the responsible proteins, even when the immunohistochemical results were not decisive. Considerable amounts of Apo A1, Apo A4, and Apo E were deposited in parallel amounts with the responsible proteins. Quantification of amyloid protein by MS-QBIC is feasible and useful for the classification of and research on systemic amyloidoses.

DOI： 10.1371/journal.pone.0235143

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men, 15/84; mean age 67.3±9.5 years), 33 were diagnosed based on the histopathological findings of perivascular/retroperitoneal lesions, 50 were diagnosed based on the characteristic imaging findings of perivascular/retroperitoneal lesions and the presence of definitive IgG4-related disease in other organ(s), and the remaining 16 patients were diagnosed by experts based on the characteristic imaging findings of perivascular/retroperitoneal legions, serological findings, response to glucocorticoid treatment, and/or the presence of suspected IgG4-related disease in other organ(s). According to the new organ-specific criteria proposed by experts, 73 (73.7%) diagnoses were categorized to be definitive, and 6 (6.1%) and 17 (17.2%) diagnoses were categorized to be probable and possible, respectively. Further analyses are needed to clarify the optimal diagnostic and therapeutic strategy of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. (This is a translation of J Jpn Coll Angiol 2018; 58: 117-129.).

DOI： 10.3400/avd.oa.19-00085

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

This study evaluated myocardial nuclear staining for the DNA damage markers poly(ADP-ribose) (PAR) and γ-H2A.X in 58 patients with dilated cardiomyopathy. Patients with left ventricular reverse remodeling (LVRR) showed a significantly smaller proportion of PAR-positive nuclei and γ-H2A.X-positive nuclei in biopsy specimens compared with those without LVRR. Propensity analysis showed that the proportion of both PAR-positive and γ-H2A.X-positive nuclei were independent prognostic factors for LVRR. In conclusion, we showed the utility of DNA damage-marker staining to predict the probability of LVRR, thus revealing a novel prognostic predictor of medical therapy for dilated cardiomyopathy.

DOI： 10.1016/j.jacbts.2019.05.010

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 21-year-old woman was referred to our hospital because of proteinuria and hematuria. She had occasional flank pain. A renal biopsy was performed and revealed a thin basement membrane. Therefore, she was diagnosed with thin basement membrane disease. However, the frequency of her flank pain increased. Since her left kidney was slightly larger than the right, nutcracker syndrome (NCS) was suspected. Renal vein ultrasonography and venography were performed, and NCS was confirmed. Her hematuria was multifactorial, and NCS can go unnoticed if there is a comorbidity that also causes hematuria.

DOI： 10.2169/internalmedicine.1433-18

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We report the first case of coronary artery fistula with aneurysmal change in a patient with immunoglobulin G4-related disease (IgG4-RD). This case revealed concomitant coronary artery dilation, pericardial inflammatory nodules, and coronary-pulmonary fistula aneurysm in addition to several IgG4-RD lesions. Each of these features was located in close proximity to the thickened pericardium. These lesions might result from inflammation of the pericardial space, which extended to the coronary-pulmonary artery vessels, leading to aneurysmal formation. This case will enhance our understanding of the pathological mechanisms of IgG4-RD inflammation.

DOI： 10.1016/j.cjca.2018.11.030

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掲載種別：研究論文（学術雑誌）  

DOI： 10.3390/jimaging4070091

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVES: Although lymphovascular space invasion is a prognostic factor for the recurrence of resectable endometrial cancer, the differential impacts of lymphatic vessel invasion (LVI) and blood vessel invasion (BVI) on the recurrence of endometrial cancer are poorly described. We investigated the prognostic significance of LVI and BVI on the recurrence of endometrial cancer and their association with patterns of recurrence. METHODS: We retrospectively reviewed 376 patients with stage I to III endometrial cancer who underwent surgery with curative intent at our institution between 2007 and 2015. The associations of the presence of lymphovascular space invasion or LVI and BVI with recurrence-free survival and patterns of recurrence were evaluated. RESULTS: Lymphovascular space invasion positivity was an independent prognostic factor for recurrence-free survival (hazards ratio [HR], 3.070; 95% confidence interval [CI], 1.404-6.824; P = 0.0048). However, when categorized by LVI versus BVI, the latter was a strong independent prognostic factor (HR, 2.697; CI, 1.288-5.798; P = 0.0081), whereas the former was not (HR, 1.740; CI, 0.795-3.721; P = 0.1637). Hematogenous metastasis was the most prevalent form of recurrence in endometrial cancer (24 [50%] of all 48 recurrent cases). Notably, 17 (19.5%) of 87 patients with BVI developed hematogenous metastases, compared with 7 (2.4%) of 289 without BVI (χ test, P < 0.0001). CONCLUSIONS: Blood vessel invasion rather than LVI was a strong predictor of postoperative recurrence in stage I to III endometrial cancer, probably due to its predisposition to hematogenous metastases.

DOI： 10.1097/IGC.0000000000001262

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Postmortem computed tomography (PMCT) of the brain has an important role in detection of subarachnoid hemorrhage (SAH), which has a high mortality rate. However, a phenomenon known as "pseudo-SAH," or high-attenuation areas along the cisterns mimicking SAH, may be seen on CT. The aim of this study was to evaluate the diagnostic accuracy of brain PMCT for SAH and to identify the characteristics of pseudo-SAH. Findings on PMCT (sulcal effacement, asymmetry, maximum thickness of SAH signs, presence of acute/subacute intraventricular/intraparenchymal hemorrhage) and clinical history (left ventricular assist device [LVAD] implantation, anticoagulation therapy/coagulation disorder, global ischemia) were compared between subjects with true SAH and those with pseudo-SAH. Twenty eight of 128 enrolled subjects had positive signs of SAH on PMCT, 20 (71.4%) had SAH on autopsy, and 8 (28.6%) did not. The sensitivity, specificity, positive predictive value, and negative predictive value of SAH signs seen on PMCT were 95.2, 94.6, 71.4, and 99.3%, respectively. Asymmetry of SAH signs and acute/subacute intraventricular and intraparenchymal hemorrhage were significantly more common in true SAH cases than in pseudo-SAH cases. The maximum thickness of SAH signs was significantly greater in true SAH cases. A history of LVAD implantation, anticoagulation therapy, and/or a coagulation disorder were more common in true SAH cases but not significantly so. A history of global ischemia was significantly more common in pseudo-SAH cases. If signs of SAH are observed on PMCT, it is important to look for other signs on PMCT and carefully review the clinical history to avoid a diagnostic error.

DOI： 10.1007/s00414-017-1651-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Fulminant myocarditis is a life-threatening disease, and myocardial damage expands the right ventricle as well as the left ventricle in some cases. There is a mortality rate of over 40% in patients with fulminant myocarditis who need mechanical circulatory support by peripheral venoarterial extracorporeal membrane oxygenation. CASE PRESENTATION: We report a case of a 27-year-old Japanese woman who was successfully bridged to recovery by using a biventricular assist device. She was diagnosed with fulminant myocarditis, and peripheral venoarterial extracorporeal membrane oxygenation was established on the same day. Her left ventricular ejection fraction rapidly decreased from 40% to 5% in 3 days and weaning from venoarterial extracorporeal membrane oxygenation was deemed difficult. Therefore, we performed a ventricular assist device implantation on day 4. A left ventricular assist device was implanted first. However, adequate blood flow did not circulate to the left side of her heart because of right-sided heart failure. Thus, an additional implant of a right ventricular assist device was performed during the operation. Her left ventricular ejection fraction recovered to 50% on day 10. The biventricular assist device was successfully removed on day 14. She has not experienced worsening of biventricular function during her follow-ups for 4 years. CONCLUSIONS: Ventricular assist device therapy should be considered if there is no improvement in cardiac function in patients with fulminant myocarditis regardless of several days of support by venoarterial extracorporeal membrane oxygenation. A right ventricular assist device should always be implemented when necessary because biventricular involvement is not uncommon in fulminant myocarditis.

DOI： 10.1186/s13256-017-1466-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Nivolumab is a monoclonal antibody against programmed death 1 and has become a standard treatment of advanced melanoma because of its durable response and survival benefits. In this report, we present a case of severe intestinal perforation after nivolumab immunotherapy for malignant melanoma. CASE PRESENTATION: A 73-year-old man with stage IV malignant melanoma underwent nivolumab therapy. The patient presented to our hospital because of a progressing abdominal pain. Radiological evaluation revealed evidence of free intraperitoneal air. Therefore, we diagnosed the patient as having an intestinal perforation, which was successfully resolved after surgical treatment. CONCLUSION: Although intestinal perforation after nivolumab immunotherapy is rare, it can be severe and requires early diagnosis and emergency surgery to ensure a favorable prognosis.

DOI： 10.1186/s40792-017-0370-7

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Desmin-related cardiomyopathy is a heterogeneous group of myofibrillar myopathies characterized by aggregates of desmin and related proteins in myocytes. It has been debated how the expression and protein structure are altered in the aggregates and other parts of myocytes in patients. To address this question, we investigated the proteome quantification as well as localization in formalin-fixed and paraffin-embedded specimens of the heart of patients by imaging mass spectrometry and liquid chromatography-mass spectrometry analyses. Fifteen tryptic peptide signals were enriched in the desmin-related cardiomyopathy myocardium, twelve of which were identified as desmin peptides with 14.3- to 27.3-fold increase compared to normal hearts. High-intensity signals at m/z 1032.5 and 1002.5, which were desmin peptides 59-70 at the head portion and 213-222 at the 1B domain, were with infrequent colocalization distributed not only in desmin-positive intracytoplasmic aggregates but also in histologically normal cytoplasm, indicating that desmin protein is fragmented and different types of naturally-occurring truncated proteins ectopically assemble throughout the heart of patients. Thus, in addition to conventional histological identification of protein aggregates, specific desmin peptides show a marked difference in quantity and localization in a tissue section of desmin-related cardiomyopathy and differentiate from other cardiomyopathies. This article is part of a Special Issue entitled: MALDI Imaging, edited by Dr. Corinna Henkel and Prof. Peter Hoffmann.

DOI： 10.1016/j.bbapap.2017.03.006

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma. He was treated with hemodialysis and chemotherapy but died of the disease 15 months after admission. Autopsy demonstrated a huge amount of crystal deposition in the subintimal layer of the vascular wall throughout the thoracic to abdominal aorta. The characteristic deposition extended to the iliac arteries, common carotid arteries, and subclavian arteries but did not affect the bilateral renal arteries. Antemortem computed tomography demonstrated higher intensity in the wall of the abdominal aorta but not in the walls of the renal arteries, suggesting that a finding of high intensity on computed tomography could be a clinical marker of systemic crystal deposition.

DOI： 10.1111/pin.12526

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Perforation of jejunal diverticulum is a rare complication. Here, we report a case of jejunal diverticulum penetration with surrounding ectopic pancreas. An 83-year-old female patient was admitted to our department with acute onset of severe abdominal pain lasting for half a day. Abdominal computed tomography showed outpouching of the small intestine that contained air/fluid, with multiple surrounding air bubbles in the mesentery of the small intestine. She was diagnosed with penetration of the small intestine, and an emergency laparotomy was indicated. The penetrated jejunal diverticulum was identified ~20-cm distal to the ligament of Treitz. Partial resection of the jejunum was performed, and her postoperative course was uneventful. The pathological findings confirmed diverticulum penetration into the mesentery and severe inflammation at the site, with surrounding ectopic pancreas. Furthermore, the pancreatic ducts were opened through the penetrated diverticulum. This rare case shows that the ectopic pancreas might have caused penetration of jejunal diverticulum owing to the pancreatic duct opening through the diverticulum.

DOI： 10.1007/s12328-017-0712-9

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

As postmortem imaging has gained prominence as a supplement to traditional autopsy, it is important to understand the normal postmortem changes to enable the accurate evaluation of postmortem imaging. No studies have evaluated the postmortem changes in cardiothoracic ratio (CTR) compared with antemortem images in the same subjects. We studied 147 consecutive subjects who underwent antemortem and postmortem CT, and autopsy. Postmortem CT was performed <23h after death and was followed by autopsy. The subjects were divided into three groups: normal heart, old myocardial infarction, and CPR-treated hearts. CTR was compared between antemortem and postmortem CT using paired t tests, which revealed that the CTR was greater on postmortem CT than on antemortem CT in all groups (mean CTR: 0.53±0.06vs. 0.50±0.06, respectively; P<0.01). Sex, age, time elapsed since death, and the causes of death were examined as potential confounding factors for the postmortem changes in CTR, but no significant associations were found. Receiver-operating characteristic (ROC) curves were used to determine CTR values for cardiomegaly, which was defined according to the autopsy weight of the heart. The area under the ROC curve was 0.71 (95% confidence interval 0.63-0.79). The CTR threshold of 0.54 identified cardiomegaly with the greatest accuracy, compared with the general threshold of 0.50. In conclusion, the CT-determined CTR increases after death, irrespective of the heart's condition. We should be cautious of overdiagnosis of cardiomegaly on postmortem CT, and new criteria for interpreting cardiomegaly on postmortem CTR are needed.

DOI： 10.1016/j.legalmed.2016.12.006

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

This is the first report of Danon cardiomyopathy managed with a left ventricular assist device (LVAD). Danon disease is an X-linked dominant inheritance disorder. Heart failure with Danon cardiomyopathy results in a poor prognosis, and heart transplantation is the treatment of choice. We present two cases of successful implantation of an LVAD for Danon cardiomyopathy. Patient 1 was in the dilatated phase of hypertrophic cardiomyopathy (HCM) with Danon cardiomyopathy, and she underwent LVAD implantation. She is waiting for transplantation. Patient 2 had dilatated cardiomyopathy with Danon cardiomyopathy and received transplantation 990 days after LVAD implantation without myopathy or intellectual disability.

DOI： 10.1016/j.athoracsur.2016.07.022

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記述言語：英語  

DOI： 10.1016/j.ijcard.2016.08.011

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The purpose of this study was to evaluate the usefulness of brain postmortem computed tomography (PMCT) findings for the detection of global hypoxia or hypoperfusion leading to hypoxic-ischemic encephalopathy (HIE) prior to death. Cadavers of individuals who died from non-traumatic causes were subjected to PMCT and pathological autopsy. Cases with an episode of cardiopulmonary arrest, hypoxia, or hypoperfusion that required intensive respiratory management at least 24 h before death and exhibited findings of HIE in conventional autopsy (HIE group, n = 6) were compared with those without such episodes prior to death (control group; overall, n = 37; age-matched, n = 8) with regard to four parameters: (1) width of the central sulcus (CS), (2) attenuation difference at the basal ganglia (BG) level, (3) attenuation difference between cerebral gray matter (GM) and cerebral white matter (WM), and (4) attenuation difference between cerebellar GM and cerebral GM. The results revealed significant differences in the width of the CS (P < 0.001), attenuation difference at the BG level (P < 0.001), and attenuation difference between cerebral GM and cerebral WM (P = 0.009) between the HIE group and the overall control group. When the age-matched control group and the HIE group were compared, there was a significant difference in the width of the CS (P = 0.026) and attenuation difference at the BG level (P < 0.001). Our results suggest that effacement of the sulcus of the cerebral hemisphere and the loss of contrast at the BG level on brain PMCT indicate the existence of HIE prior to death.

DOI： 10.1007/s12024-016-9787-8

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.ijcard.2016.04.137

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVES: The purpose of this study is to compare the postmortem changes in computed tomography (CT) findings between normal spleen, splenic infarct, and splenic tumor infiltration. METHODS: The institutional review board approved this study, and informed consent was obtained from the next of kin. We studied 63 consecutive subjects who underwent antemortem CT, postmortem CT, and autopsy between February 2012 and December 2013. Postmortem CT was performed within 1678 min after death and was followed by pathological studies. The subjects were divided into three groups based on the pathological findings: normal, splenic infarct, and splenic tumor infiltration. The volume and attenuation of the spleen were compared between antemortem and postmortem CT using paired t tests. Gender, age, time elapsed since death, and the causes of death were examined as potential confounding factors of the postmortem changes in volume and attenuation. RESULTS: In all groups, the spleen decreased in volume and attenuation increased on postmortem CT compared with antemortem CT. The postmortem changes in spleen volume and attenuation were not significantly associated with sex, age, time elapsed since death, or causes of death. CONCLUSIONS: Spleen volume decreased and attenuation increased on postmortem CT compared with antemortem CT in subjects with a normal spleen, splenic infarct, or splenic tumor infiltration. These results should caution us against underestimating the significance of splenomegaly on postmortem CT, misinterpreting reduced splenic volume as the presence of hypovolemic or distributive shock in the subject while alive, and confusing postmortem splenic hyperattenuation with diseases characterized by this finding.

DOI： 10.1007/s00414-016-1337-0

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

An up-regulated CXCR3 pathway and affluent plasma cell infiltration are characteristic features of Hunner type interstitial cystitis (HIC). We further examined these two features using bladder biopsy samples taken from 27 patients with HIC and 15 patients with non-IC cystitis as a control. The number of CD3-positive T lymphocytes, CD20-positive B lymphocytes, CD138-positive plasma cells, and CXCR3-positive cells was quantified by digital image analysis. Double-immunofluorescence for CXCR3 and CD138 was used to detect CXCR3 expression in plasma cells. Correlations between CXCR3 positivity and lymphocytic and plasma cell numbers and clinical parameters were explored. The density of CXCR3-positive cells showed no significant differences between HIC and non-IC cystitis specimens. However, distribution of CXCR3-positivity in plasma cells indicated co-localization of CXCR3 with CD138 in HIC specimens, but not in non-IC cystitis specimens. The number of CXCR3-positive cells correlated with plasma cells in HIC specimens alone. Infiltration of CXCR3-positive cells was unrelated to clinical parameters of patients with HIC. These results suggest that infiltration of CXCR3-positive plasma cells is a characteristic feature of HIC. The CXCR3 pathway and specific immune responses may be involved in accumulation/retention of plasma cells and pathophysiology of the HIC bladder.

DOI： 10.1038/srep28652

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

In a case of acquired Fanconi syndrome associated with smoldering myeloma, we confirmed the deposition of protease-resistant κ light chain proteins in a proximal tubular injury and found the decreased expression of apical tubular transporters including sodium glucose co-transporter, sodium phosphate co-transporter, uric acid transporter 1, and a decrease of Na(+)/K(+)-ATPase in the basolateral membrane. The protease-resistant kappa light chain has a pathological role in the expression of tubular transporters in the proximal tubule and causes Fanconi syndrome associated with smoldering myeloma.

DOI： 10.1007/s00795-015-0114-3

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The development of nephrotic syndrome (NS) after umbilical cord transplantation (UBT) has been reported in only four cases to date. We herein report the case of a 50-year-old woman who developed NS 94 days after UBT. She fell into oliguria and required dialysis. A kidney biopsy revealed focal and segmental glomerulosclerosis. Although glucocorticoid monotherapy did not improve her condition, the addition of low-density lipoprotein (LDL) apheresis resulted in remission of NS, a drastic improvement in her renal function, and withdrawal from dialysis. To the best of our knowledge, this is the first report of UBT-associated NS treated with LDL apheresis.

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掲載種別：研究論文（学術雑誌）  

DOI： 10.9738/INTSURG-D-15-00130.1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We aimed to confirm whether postmortem adrenal volumetric changes occur by measuring adrenal volumes on computed tomography (CT). Fifty-five adrenal glands from 28 subjects who died were included. All subjects underwent antemortem CT (AMCT) and postmortem CT (PMCT) within 94-1,191 min after death, followed by conventional autopsy. CT volumetry was performed using freely-available software. Differences between AMCT and PMCT adrenal volumes were evaluated statistically along with differences in the degree of volume change, elapsed time to PMCT, and presence of underlying malignant disease. The mean volume of the right adrenal gland decreased from 3.8 cm(3) on AMCT to 2.6 cm(3) on PMCT (P < 0.001); the left adrenal gland decreased from 4.2 cm(3) on AMCT to 3.1 cm(3) on PMCT (P < 0.001). Conventional autopsy revealed decreased intracellular lipid components in portions of the adrenal glands. No correlation between the adrenal gland reduction rate and the elapsed time from AMCT to death or from death to PMCT was observed (P = 0.99 and 0.79; P = 0.28 and 0.59 for the right and left adrenal glands, respectively). Significant differences in both the bilateral adrenal gland reduction rates and underlying malignant disease were found for the left adrenal gland (P = 0.015), but not for the right (P = 0.74). Adrenal volume reduction was observed on PMCT compared to AMCT. This highlights the need to further elucidate the mechanism of adrenal shrinkage during the agonal stage and after death. This may be explained by pathological findings of intracellular lipid depletion.

DOI： 10.1007/s11547-014-0449-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Although membranous nephropathy (MN) is a commonly observed cause of post-transplant glomerulonephritis, distinguishing de novo from recurrent MN in kidney allograft is often difficult. Phospholipase A2 receptor (PLA2R) staining is useful for diagnosing recurrent MN in allografts similarly to idiopathic MN in native kidney. No specific treatment strategy has been established for MN, especially when accompanied with HCV infection in kidney transplant recipients. This report describes a 66-year-old man who was diagnosed as having PLA2R positive membranous nephropathy accompanied with already-known IgA nephropathy and HCV infection 26 years after kidney transplantation conducted between identical twins. PLA2R was detected along capillary loops, implying that this patient is affected by the same pathogenic mechanism as idiopathic MN, not secondary MN associated with other disorders such as HCV infection. The patient successfully achieved clinical remission after steroid therapy.

DOI： 10.1111/nep.12458

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KOREAN RADIOLOGICAL SOC  

Computed tomography (CT) is widely used in postmortem investigations as an adjunct to the traditional autopsy in forensic medicine. To date, several studies have described postmortem CT findings as being caused by normal postmortem changes. However, on interpretation, postmortem CT findings that are seemingly due to normal postmortem changes initially, may not have been mere postmortem artifacts. In this pictorial essay, we describe the common postmortem CT findings in cases of atraumatic in-hospital death and describe the diagnostic pitfalls of normal postmortem changes that can mimic real pathologic lesions.

DOI： 10.3348/kjr.2015.16.4.798

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記述言語：英語  

DOI： 10.3109/13506129.2014.987865

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The purpose of this study was to evaluate the brain by postmortem computed tomography (PMCT) versus antemortem computed tomography (AMCT) using brains from the same patients. We studied 36 nontraumatic subjects who underwent AMCT, PMCT, and pathological autopsy in our hospital between April 2009 and December 2013. PMCT was performed within 20 h after death, followed by pathological autopsy including the brain. Autopsy confirmed the absence of intracranial disorders that might be related to the cause of death or might affect measurements in our study. Width of the third ventricle, width of the central sulcus, and attenuation in gray matter (GM) and white matter (WM) from the same area of the basal ganglia, centrum semiovale, and high convexity were statistically compared between AMCT and PMCT. Both the width of the third ventricle and the central sulcus were significantly shorter in PMCT than in AMCT (P < 0.0001). GM attenuation increased after death at the level of the centrum semiovale and high convexity, but the differences were not statistically significant considering the differences in attenuation among the different computed tomography scanners. WM attenuation significantly increased after death at all levels (P<0.0001). The differences were larger than the differences in scanners. GM/WM ratio of attenuation was significantly lower by PMCT than by AMCT at all levels (P<0.0001). PMCT showed an increase in WM attenuation, loss of GM-WM differentiation, and brain swelling, evidenced by a decrease in the size of ventricles and sulci.

DOI： 10.1371/journal.pone.0143848

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Congenital contractural arachnodactyly (CCA) is a rare connective tissue disorder characterized by marfanoid habitus with camptodactyly. However, cardiac features have rarely been documented in adults. We herein report a sporadic case of CCA in a 20-year-old woman who developed decompensated dilated cardiomyopathy. The patient did not have any mutations in the FBN1 or FBN2 genes, which are most commonly associated with Marfan syndrome and CCA, respectively. Although whether these two diseases are caused by a mutation(s) in the same gene or two different genes remains unknown, this case provides new clinical insight into the cardiovascular management of CCA.

DOI： 10.2169/internalmedicine.54.4280

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

IgG4-related pericardial involvement has rarely been reported and its clinical features remain unknown. We herein report a case of a 50-year-old woman with pericarditis who presented with a fever, elevated C-reactive protein levels, elevated serum IgG4 concentrations, and thickened pericardium with a patchy (18)F-fluorodeoxyglucose (FDG) uptake. A biopsy specimen of (18)F-FDG accumulated in the mediastinal lymph nodes revealed an abundant infiltration of IgG4-bearing plasma cells without fibrosis. Moderate-dose glucocorticoids promptly resolved the physical, serological, and imaging abnormalities, thus indicating a relatively acute and reversible nature of IgG4-related pericardial involvement.

DOI： 10.2169/internalmedicine.54.4340

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

ヒトの大動脈壁は死後に恐らく死後硬直によって厚くなることを発見しました。

DOI： 10.1007/s00414-013-0955-z

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

To evaluate radiographic features of endotracheal/endobronchial fluid in the airway (FA) observed on postmortem computed tomography (PMCT). We studied 164 subjects who died at our hospital between April 2009 and September 2012. Fluid in the airway was considered positive when fluid was identified in the lumen of 1 of the 2 main bronchi in continuity with a segmental bronchus. Pleural effusion and atelectasis/consolidation of the lung lower lobes were also evaluated. Fluid in the airway was observed in 60 (71%) of 84 subjects with unilateral or bilateral pleural effusion, and in 44 (55%) of 80 subjects without pleural effusion (P = 0.029). Of the latter, 41 (93%) had atelectasis/consolidation of the lower lung lobes. Among subjects without pleural effusion, average times after death to PMCT of subjects with and without FA were 501 and 314 minutes, respectively (P = 0.01). Time-course analysis showed that cases with FA on PMCT largely correlated with time after death (R = 0.7966). Fluid in the airway is frequently observed on PMCT in subjects with pleural effusion or atelectasis/consolidation of the lung. No FA in subjects without pleural effusion correlated to shorter times after death. In addition, FA frequency on PMCT increased over time after death.

DOI： 10.1097/PAF.0000000000000083

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: We evaluated the postmortem changes of striated muscle by comparing computed tomography (CT) images obtained postmortem and antemortem in the same patients. MATERIALS AND METHODS: We studied 33 consecutive patients who underwent antemortem CT, postmortem CT, and pathological autopsy in our tertiary care hospital between April 2009 and December 2010. Postmortem CT was performed within 20 h after death and was followed by pathological autopsy. Pathological autopsy confirmed the absence of muscular diseases such as amyotrophic lateral sclerosis, muscular dystrophy, myositis, and myasthenia, in all of the patients. The CT attenuation values of four cardiac muscle sites (anterior wall of the left ventricle, left ventricular free wall, posterior wall of the left ventricle, and the ventricular septum) and two skeletal muscle sites (the pectoralis major muscle and the erector spinae muscle) were compared between antemortem and postmortem CT using paired t test. RESULTS: Striated muscle had significantly greater attenuation on postmortem CT than on antemortem CT (P<0.001) in all six tissue sites. No significant association was found between postmortem change in the CT attenuation of striated muscle and gender, age, or elapsed time since death. CONCLUSION: This is the first longitudinal study to show hyperattenuation of striated muscle on postmortem CT images compared with antemortem CT images in the same patients.

DOI： 10.1371/journal.pone.0111457

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掲載種別：研究論文（学術雑誌）  

DOI： 10.1007/s00277-013-1935-6

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：9  

Objective:To evaluate the postmortem changes of the heart wall on postmortem (PM) computed tomography (CT) in comparison with those on ante mortem CT (AMCT), and in comparison with the pathological findings, obtained in the same patients.Materials and Methods:We studied 57 consecutive patients who had undergone AMCT, PMCT, and pathological autopsy in our tertiary care hospital between April 2009 and December 2010. PMCT was performed within 20 hours after death, followed by pathological autopsy. The cardiac chambers were measured at five sites on both AMCT and PMCT by two board-certified radiologists who were not provided with clinical information. The differences in heart wall thickness between AMCT with and without contrast medium, between AMCT and PMCT, and between PMCT and pathological anatomy were evaluated statistically. Confounding factors of postmortem change such as gender, presence of arteriosclerosis, the organ related to cause of death, age, and elapsed time since death were examined statistically.Results:No significant differences were observed on AMCT in comparison of contrasted and non-contrasted images. The heart wall was significantly thicker on PMCT than on AMCT (p &lt
0.0001) at all five measurement sites. The heart wall was significantly thicker on PMCT than on pathology specimens when measured in accordance with pathological standard mensuration. However, no significant difference was observed between PMCT measurements and those of pathology specimens at any site when the papillary muscles and epicardial fat were included. No significant association was found between postmortem change in heart wall thickness and gender, presence of arteriosclerosis, the organ related to cause of death, age, or elapsed time since death.Conclusion:This is the first longitudinal study to confirm greater thickness of heart wall on postmortem images compared with ante mortem images, in the same patients. Furthermore, the postmortem changes on CT were supported by the pathological findings. © 2013 Okuma et al.

DOI： 10.1371/journal.pone.0076026

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Mitral and aortic valve regurgitation is commonly found in osteogenesis imperfecta (OI) patients, however, little is known about the myocardial involvement in this disorder. An 82-year-old man with OI developed heart failure and was admitted to our hospital. Echocardiogram revealed severe mitral regurgitation without left ventricular (LV) dilatation, but with LV wall thickening. Histological analysis exhibited interstitial fibrosis of the myocardium in addition to myxoid changes of the mitral leaflet. These findings suggest that OI patients may develop LV remodeling together with diastolic dysfunction.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER ASSOC CANCER RESEARCH  

ERV-associated gastric carcinoma is a distinct subset of gastric carcinoma infected with EBV, which shows latency I type expression of EBV latent genes (EBNA1, EBER, BARF0, and LMP2A). To clarify the role of EBV in this type of gastric carcinoma, the cell biological characteristics (growth, apoptosis, and migration) were evaluated in gastric carcinoma cell lines (MKN-1, TMK1, MKN-74 and MKN-7) with and without infection of recombinant EBV harboring the neomycin resistance gene. The infection reiterated the latency I type infection, and the only difference observed in EBV-infected gastric carcinoma cell lines was the resistance to serum deprivation-induced apoptosis. Comparative analyses of transcripts of apoptosis-associated genes in MKN-1 and EBV-MKN-1 and subsequent quantitative reverse transcription-PCR analysis showed up-regulation of the cellular survivin gene in EBV-infected gastric carcinoma cell lines. Small interfering RNA-mediated knockdown of survivin increased apoptosis in EBV-MKN-1 to the level of the original MKN-1 cells. Transfection of EBV-latent genes into MKN-1 showed that LMP2A, but not EBNA1, EBER, or BARF0, up-regulated survivin gene expression. LMP2A-mediated survivin up-regulation in gastric carcinoma cells was inhibited with a nuclear factor-kappa B (NF-kappa B) inhibitor, Bay 11-7082. In parallel with these findings in vitro, survivin expression was frequent in carcinoma tissues of gastric carcinoma by immunohistochemistry, and significantly more in EBV-associated gastric carcinoma (12 of 13) than in EBV-negative gastric carcinoma in the advanced stage (P = 0.0307). Thus, EBV uses its latent protein, LMP2A, to activate the NF-kappa B-survivin pathway to rescue EBV-infected epithelial cells from serum deprivation, and up-regulation of survivin may play a role in the progression of this specific type of gastric carcinoma infected with EBV.

DOI： 10.1158/0008-5472.CAN-07-3027

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(公社)日本眼科手術学会  

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記述言語：日本語   出版者・発行元：(公社)日本眼科手術学会  

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記述言語：日本語   出版者・発行元：日本医科大学医学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：日本医科大学医学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本医療情報学会  

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記述言語：日本語   出版者・発行元：(NPO)日本肺癌学会  

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記述言語：日本語   出版者・発行元：(一社)日本脈管学会  

エキスパートによりIgG4関連の大動脈周囲炎/動脈周囲炎および後腹膜線維症と診断された99例のデータを解析し、臨床像や診断に至った経緯を検討した。33例は動脈周囲/後腹膜の病理組織学的所見から、50例は他臓器のIgG4関連疾患の確診から、残りの16例は臨床経過などから総合的に診断されていた。IgG4関連疾患の厚生労働省研究班により策定された新しい臓器特異的診断基準を用いた検討では、73例(73.7%)が確診、6例(6.1%)が準確診、17例(17.2%)が疑診に分類された。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本脈管学会  

エキスパートによりIgG4関連の大動脈周囲炎/動脈周囲炎および後腹膜線維症と診断された99例のデータを解析し、臨床像や診断に至った経緯を検討した。33例は動脈周囲/後腹膜の病理組織学的所見から、50例は他臓器のIgG4関連疾患の確診から、残りの16例は臨床経過などから総合的に診断されていた。IgG4関連疾患の厚生労働省研究班により策定された新しい臓器特異的診断基準を用いた検討では、73例(73.7%)が確診、6例(6.1%)が準確診、17例(17.2%)が疑診に分類された。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J01422&link_issn=&doc_id=20180816440002&doc_link_id=10.7133%2Fjca.18-00018&url=https%3A%2F%2Fdoi.org%2F10.7133%2Fjca.18-00018&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：日本臨床外科学会  

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記述言語：日本語   出版者・発行元：(NPO)日本頭頸部外科学会  

神経鞘腫は身体のあらゆる部分に発生し、舌根部の発生はまれとされる。今回、偶然発見された、無症候性の舌根部神経鞘腫に対して、合併症なく経口的に切除できた症例を経験したので報告する。症例は42歳、男性で、消化管造影検査を受けた際に舌根部の隆起性病変を指摘され、当科を受診した。中咽頭を占拠する粘膜下腫瘍を認め、全身麻酔下の迅速病理検査で悪性が否定されたので、FK-WOリトラクターと先端彎曲型硬性内視鏡を併用した経口法で腫瘍を摘出した。永久病理検査で最終的に神経鞘腫と診断された。明らかな神経脱落症状を認めず、術後経過は良好である。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J04210&link_issn=&doc_id=20180309480023&doc_link_id=10.5106%2Fjjshns.27.387&url=https%3A%2F%2Fdoi.org%2F10.5106%2Fjjshns.27.387&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：日本語   出版者・発行元：(一社)日本医療安全学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本透析医学会  

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記述言語：日本語   出版者・発行元：(株)東京医学社  

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記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

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記述言語：日本語   出版者・発行元：(株)学研メディカル秀潤社  

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記述言語：日本語   出版者・発行元：(株)文光堂  

病理解剖1052例の病理解剖報告書を対象に、「突然」のキー・ワードで検索を行い症例を抽出した。得られた症例について臨床推定死因、病理解剖診断、死後CT画像による診断を検討した。その結果、突然死は全症例中40例(3.8%)であった。生前の臨床診断と剖検診断の一致は20例(50%)、死因が生前不明・解剖で判明または生前診断と解剖診断が不一致は計13例(32.5%)、剖検を行っても死因が不明であったのは7例(17.5%)であった。急変した場所(院内か院外)、急変までの期間(10日以上か10日未満)では一致率に差はなかった。急変後に画像検索(CT等)が行われた症例、また消化器疾患や脳疾患を有する症例は全例とも生前の臨床診断と剖検診断が一致した。死後CTは8例で行われ、うち3例は死後CTのみでは死因推定が困難で、剖検で死因が判明した。以上、突然死の死因の不一致率は過去の報告における不一致率と同程度であり、突然死で特に不一致率が高いわけではなかった。突然死であるか否かに関わらず、病理解剖は重要であると考えられた。

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記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

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記述言語：日本語   出版者・発行元：日本臨床外科学会  

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記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：日本語   出版者・発行元：(公社)日本医学放射線学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：医学図書出版(株)  

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記述言語：日本語   出版者・発行元：日本胸部外科学会-関東甲信越地方会  

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記述言語：日本語   出版者・発行元：(一社)日本透析医学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(株)インナービジョン  

オートプシー・イメージング(以下、Ai)は、死後に施行されるCT、MRI、超音波検査など、あらゆる画像モダリティを包括した概念である。本邦の死後画像検査はCTが一般的であり、本稿では単純(非造影)CTで見られる代表的所見について概説する。なお、われわれは院内死亡の病理解剖前に死後CTを撮影しており、遺体は室温、仰臥位での静置が標準である。死後画像所見は、(1)死後変化、(2)蘇生術後変化、(3)病変(死因関与は不問)の3つに大別される(表1)。読影時にはこれらを意識することが肝要であるが、(1)と(2)はAiに一般的な所見で、特に(1)はAiでしか見ない。死後画像は生前画像と比べていくつかの相違点が存在するため、Aiで特徴的に認められる所見を押さえることが、正確なAi読影を行う際に大切である。(著者抄録)

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記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：日本語   出版者・発行元：(公社)日本医学放射線学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：日本胸部外科学会-関東甲信越地方会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：日本内科学会-関東地方会  

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記述言語：日本語   出版者・発行元：日本臨床外科学会  

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記述言語：英語   出版者・発行元：(公社)日本医学放射線学会  

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記述言語：英語   出版者・発行元：(公社)日本医学放射線学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(株)日本臨床社  

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記述言語：英語   出版者・発行元：(一社)日本癌学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：英語   出版者・発行元：(一社)日本癌学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

十二指腸乳頭部腫瘍を疑われ、膵十二指腸切除術で摘出された膵組織に、多数の肉芽腫形成を認めた67歳の男性例を経験した。膵管周囲を中心に、多核巨細胞を伴う多数の肉芽腫形成がみられた。膵内に軽度の線維化、軽度の炎症細胞浸潤、膵管上皮に強い扁平上皮化生が観察され、膵周囲に軽度の脂肪壊死を伴っていた。周囲のリンパ節に肉芽腫形成は認めず、他の肉芽腫形成性疾患の合併もなかった。膵に限局した肉芽腫形成をみることはまれであり、その発生機序や意義について考察した。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2006&ichushi_jid=J03623&link_issn=&doc_id=20061107260006&doc_link_id=%2Fcd9jjodp%2F2006%2F002304%2F006%2F0274-0276%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcd9jjodp%2F2006%2F002304%2F006%2F0274-0276%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：日本語   出版者・発行元：(福)聖隷福祉事業団総合病院聖隷三方原病院  

急性冠症候群に対して行っている血栓吸引療法の成績と費用効果を検討した.従来のPCIに血栓吸引療法を追加し,最終的に全症例でTIMI3 flowを獲得し,末梢塞栓も発生しなかった.また,バルーンカテの使用率も低く,入院費用,PCI費用の軽減が予想された

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