Budd-chiari syndrome and unsuspected gastric adenocarcinoma

A síndrome de Budd-Chiari não tem sido descrita em pacientes jovens portadores de câncer gástrico. Relata-se caso de síndrome de Budd-Chiari e linfangite carcinomatosa em homem branco de 28 anos, portador de câncer gástrico insuspeito, apresentando icterícia, hematêmese e dispnéia. O estudo de autopsia revelou adenocarcinoma gástrico infiltrando vasos das camadas submucosa e serosa, com sangramento do estômago e intestino, além de metástases hepáticas e pulmonares. Múltiplos microtrombos mistos (fibrina, plaquetas e células tumorais) foram observados em pequenos vasos sangüíneos pulmonares e tanto os vasos linfáticos subpleurais quanto o interstício pulmonar continham células tumorais metastáticas.The Budd-Chiari syndrome has not been described in young patients with gastric cancer. A case of Budd-Chiari syndrome and carcinomatous lymphangitis is reported in a 28 years-old white man with unsuspected gastric cancer, presenting jaundice, hematemesis and dyspnea. Autopsy disclosed gastric adenocarcinoma invading vessels of the submucous and serous layers, with gastric and intestinal bleeding, liver and lung metastases. Multiple mixed (fibrin, platelets and tumor cells) microthrombi were observed in small pulmonary blood vessels, and both subpleural lymph vessels and lung interstitium contained metastatic tumor cells

Collagenofibrotic Glomerulopathy: Three Case Reports in Brazil

<p>Abstract</p> <p>Background</p> <p>We are reporting the first Collagenofibrotic Glomerulopathy (CG) in South America. So, this collagen type III glomerulopathy is not limited to Japan but may be found throughout the world.</p> <p>Case Reports</p> <p>We describe three patients that presented some factors in common, such as sex, age and the presence of non-nephrotic proteinuria associated with microscopic hematuria. The findings with the immunofluorescence microscopy, of immunoglobulins, and components of the complement were usually negative. The picrosyrius staining showed the presence of reddish material in the mesangium, when it was seen under standard microscopy; however, when it was seen with birefringence, it became greenish under polarized light, showed the collagen found in this area of the glomerulus. The identification of CG was made through electronic microscopic scanning, and curved and disorganized fibers were found.</p> <p>Conclusion</p> <p>These cases are the first from South America to be reported, and they are about an idiopathic renal disease that is not related to any specific races or locations. The reports contribute to a better understanding of this disease, which although not so prevalent, should be considered as an importantly differential diagnostic of cases of proteinuria.</p

CD28 Family and Chronic Rejection: “To Belatacept...and Beyond!”

Kidneys are one of the most frequently transplanted human organs. Immunosuppressive agents may prevent or reverse most acute rejection episodes; however, the graft may still succumb to chronic rejection. The immunological response involved in the chronic rejection process depends on both innate and adaptive immune response. T lymphocytes have a pivotal role in chronic rejection in adaptive immune response. Meanwhile, we aim to present a general overview on the state-of-the-art knowledge of the strategies used for manipulating the lymphocyte activation mechanisms involved in allografts, with emphasis on T-lymphocyte costimulatory and coinhibitory molecules of the B7-CD28 superfamily. A deeper understanding of the structure and function of these molecules improves both the knowledge of the immune system itself and their potential action as rejection inducers or tolerance promoters. In this context, the central role played by CD28 family, especially the relationship between CD28 and CTLA-4, becomes an interesting target for the development of immune-based therapies aiming to increase the survival rate of allografts and to decrease autoimmune phenomena. Good results obtained by the recent development of abatacept and belatacept with potential clinical use aroused better expectations concerning the outcome of transplanted patients

Beta-fibrilose encefálica e cardíaca em idosos autopsiados

INTRODUCTION: Amyloidosis in elderly individuals can be an independent alteration and a characteristic of aging. However, the clinical, pathophysiologic, and biochemical characteristics of amyloidosis related to age remain uncertain. OBJECTIVE: The purpose of this study was to determine the extent to which the heart and/or the brain of individuals aged 60 years or over exhibits amyloid deposits. MATERIALS AND METHODS: The autopsy findings of individuals who were at least 60 years old were studied. The autopsies took place between the years of 1976 and 2000. A total of 10 cases were selected that had hearts without cardiopathies, had negative serology for Chagas' disease, and had brains without morphological changes related to encephalopathies. Slides with fragments of heart and brain were processed and analyzed using polarized and common light microscopy. RESULTS: Of the 10 cases, 4 were positive for amyloidosis. All had positive findings in the brain, and 1 case also had positive findings in the heart. Among the positive cases, 50% were of people aged 60 to 69 years. There appeared to be a relationship between the presence of amyloid deposits and the ratio of brain and body weight, with the ratio in the positive cases being smaller than in the negative cases. CONCLUSIONS: The analysis of amyloid deposits in the brains and hearts of elderly individuals shows that such deposits may lead to a systemic attack of senility, common to natural aging. It is not certain that beta-amyloid deposits would alone bring such drastic repercussions to the individual. Some additional disorders of the organism could cause the breakdown of the natural balance related to the accumulation of these proteins, leading the way to the pathological contexts of amyloidosis.RESUMO INTRODUÇÃO: A amiloidose em idosos pode ser uma alteração independente e própria do envelhecimento. Entretanto, as características clínicas, fisiopatológicas e bioquímicas da Amiloidose relacionada à idade ainda permanecem incertas. OBJETIVO: Verificar se o coração e o encéfalo de indivíduos acima de 60 anos apresentavam depósito amilóide. MATERIAL E MÉTODOS: Foram estudados laudos consecutivos de autópsias de indivíduos acima de 60 anos realizadas entre 1976 e 2000, que apresentavam corações sem cardiopatias, com sorologia negativa para Doença de Chagas e encéfalos sem alterações morfológicas de encefalopatias, chegando a um n de 10 casos. Lâminas de fragmentos do coração e de encéfalo foram processadas e analisadas em microscopia de luz comum e polarizada. RESULTADOS: Dos 10 casos, 3 apresentaram depósito amilóide no encéfalo e 1 no encéfalo e no coração. Em 50% dos casos, os indivíduos tinham entre 60 e 69 anos. A relação entre o peso encefálico e o peso corporal mostrou ter uma associação significativa com os casos positivos, sendo esta menor em relação aos negativos. CONCLUSÃO: A análise conjunta de depósitos amilóides em encéfalo e coração de indivíduos idosos talvez direcione para um acometimento sistêmico comum ao envelhecimento natural. Alguma alteração adicional do organismo poderia determinar a quebra de um equilíbrio natural sobre o acúmulo dessas proteínas, levando dessa forma aos contextos patológicos da amiloidose

Adipose Tissue-Derived Stem Cell Treatment Prevents Renal Disease Progression

Adipose tissue-derived stem cells (ASCs) are an attractive source of stem cells with regenerative properties that are similar to those of bone marrow stem cells. Here, we analyze the role of ASCs in reducing the progression of kidney fibrosis. Progressive renal fibrosis was achieved by unilateral clamping of the renal pedicle in mice for 1 h; after that, the kidney was reperfused immediately. Four hours after the surgery, 2 x 10(5) ASCs were intraperitoneally administered, and mice were followed for 24 h posttreatment and then at some other time interval for the next 6 weeks. Also, animals were treated with 2 x 10(5) ASCs at 6 weeks after reperfusion and sacrificed 4 weeks later to study their effect when interstitial fibrosis is already present. At 24 h after reperfusion, ASC-treated animals showed reduced renal dysfunction and enhanced regenerative tubular processes. Renal mRNA expression of IL-6 and TNF was decreased in ASC-treated animals, whereas IL-4. IL-10, and HO-1 expression increased despite a lack of ASCs in the kidneys as determined by SRY analysis. As expected, untreated kidneys shrank at 6 weeks, whereas the kidneys of ASC-treated animals remained normal in size, showed less collagen deposition, and decreased staining for FSP-1, type I collagen, and Hypoxyprobe. the renal protection seen in ASC-treated animals was followed by reduced serum levels of TNF-alpha, KC, RANTES, and IL-1 alpha. Surprisingly, treatment with ASCs at 6 weeks, when animals already showed installed fibrosis, demonstrated amelioration of functional parameters, with less tissue fibrosis observed and reduced mRNA expression of type I collagen and vimentin. ASC therapy can improve functional parameters and reduce progression of renal fibrosis at early and later times after injury, mostly due to early modulation of the inflammatory response and to less hypoxia, thereby reducing the epithelial-mesenchymal transition.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)DECIT/Ministerio do SaudeComplex Fluids INCTUniversidade Federal de São Paulo, Div Nephrol, Escola Paulista Med, Expt & Clin Immunol Lab, BR-04023900 São Paulo, BrazilUniv São Paulo, Dept Pathol, São Paulo, BrazilUniv Fed Triangulo Mineiro, Div Pathol, Uberaba, MG, BrazilUniv São Paulo, Dept Immunol, Lab Transplantat Immunobiol, São Paulo, BrazilUniversidade Federal de São Paulo, Div Nephrol, Escola Paulista Med, Expt & Clin Immunol Lab, BR-04023900 São Paulo, BrazilFAPESP: 09/13251-6FAPESP: 07/07139-3CNPq: 473844/2009-5Web of Scienc

Toxoplasmose disseminada sepse símile em dois pacientes com AIDS

O presente relato descreve dois pacientes que apresentaram toxoplasmose aguda, disseminada e grave como primeira manifestação oportunista da síndrome da imunodeficiência adquirida. Os achados clínicos e laboratoriais foram similares aos de sepse ou choque séptico e, em ambos os casos houve evolução rápida para óbito. À necropsia, foi observada reação inflamatória e presença de taquizoítos e cistos de Toxoplasma gondii na maioria dos órgãos examinados.This report describes two patients who presented acute disseminated and severe toxoplasmosis as the first opportunistic disease related to acquired immunodeficiency syndrome. At admission, clinical and laboratory findings were similar to sepsis or septic shock and a fast evolutive course to death occurred in both cases. At necropsy, an inflammatory reaction and presence of a great number of Toxoplasma gondii cysts and tachyzoites were observed in most organs examined

A novel single amino acid deletion impairs fibronectin function and causes familial glomerulopathy with fibronectin deposits: case report of a family

Abstract Background Glomerulopathy with fibronectin deposits is an autosomal dominant disease associated with proteinuria, hematuria, hypertension and renal function decline. Forty percent of the cases are caused by mutations in FN1, the gene that encodes fibronectin. Case presentation This report describes two cases of Glomerulopathy with fibronectin deposits, involving a 47-year-old father and a 14-year-old son. The renal biopsies showed glomeruli with endocapillary hypercellularity and large amounts of mesangial and subendothelial eosinophilic deposits. Immunohistochemistry for fibronectin was markedly positive. Whole exome sequencing identified a novel FN1 mutation that leads to an amino-acid deletion in both patients (Ile1988del), a variant that required primary amino-acid sequence analysis for assessment of pathogenicity. Our primary sequence analyses revealed that Ile1988 is very highly conserved among relative sequences and is positioned in a C-terminal FN3 domain containing heparin- and fibulin-1-binding sites. This mutation was predicted as deleterious and molecular mechanics simulations support that it can change the tertiary structure and affect the complex folding and its molecular functionality. Conclusion The current report not only documents the occurrence of two GFND cases in an affected family and deeply characterizes its anatomopathological features but also identifies a novel pathogenic mutation in FN1, analyzes its structural and functional implications, and supports its pathogenicity.https://deepblue.lib.umich.edu/bitstream/2027.42/152212/1/12882_2019_Article_1507.pd