RESUMO
Refractory celiac disease is an uncommon condition which might be associated to poor prognosis. It is often treated with immunosuppressive medications, with poor results. It is divided in type 1 and type 2, the latter carrying a high risk for lymphoma and mortality. A case of a 41 year old female patient with refractory celiac disease type 2 is reported. She was treated with oral budesonide for six months, achieving histological remission.
Assuntos
Doença Celíaca , Adulto , Budesonida , Doença Celíaca/diagnóstico , Feminino , HumanosRESUMO
Neuroparacoccidiodimycosis (NPDM) is an uncommon granulomatous disease, which more frequently affects immunocompromised male patients over 30 years of age in the course of chronic lung disease. Paracoccidioides brasiliensis (PB) is an endemic fungus in Brazil, and grows as thick-walled yeast (with round to oval bodies) measuring 10 µm to 60 µm in diameter. Neuroparacoccidiodimycosi may develop many years after transmission and/or primary lung involvement. The authors describe a case of NPDM affecting a male patient, 52 years of age, farmer, heavy smoker, with clinical complaint of headache, asthenia, seizures, and prostration in the previous nine months. Upon physical examination, the patient presented regular general condition, without other relevant physical alterations. Computed tomography (CT) showed multiple bilateral pulmonary nodules associated to enlargement of the mediastinal lymph node. Magnetic resonance imaging (MRI) and CTscans of the central nervous system showed six heterogeneous nodular lesions compromising the frontal and parietal lobes, the largest one measuring 3.8 3.2 3.2 cm. The hypothesis of a neoplastic process compromising the lung and brain was considered. A biopsy of the mediastinal lymph node showed epithelioid granulomas, which exhibited round, thin-walled fungal structures in Grocott silver stain. The stereotactic biopsy of the frontal lesion was constituted by necrotic tissue admixed with some round to oval, thin-walled fungi measuring 10 µm to 60 µm, compatible with PB (identified on Grocott silver stain/confirmed in culture). The diagnosis of NPDM was then established. The employed therapeutic regimen was intravenous amphotericin B, itraconazole, and sulfamethoxazole-trimetropin. After ninety days of clinical follow-up, no episodes of seizures/neurological deficits were identified, and a marked decrease in the number and size of the lung and brain lesions were found.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Paracoccidioidomicose/terapia , Hospedeiro Imunocomprometido , Infecções Fúngicas do Sistema Nervoso Central/cirurgia , Antifúngicos/uso terapêutico , Paracoccidioides , Paracoccidioidomicose/diagnóstico por imagem , Infecções Fúngicas do Sistema Nervoso Central/diagnóstico por imagemRESUMO
SUMMARY Refractory celiac disease is an uncommon condition which might be associated to poor prognosis. It is often treated with immunosuppressive medications, with poor results. It is divided in type 1 and type 2, the latter carrying a high risk for lymphoma and mortality. A case of a 41 year old female patient with refractory celiac disease type 2 is reported. She was treated with oral budesonide for six months, achieving histological remission.
Assuntos
Humanos , Masculino , Feminino , Doença Celíaca/diagnóstico , BudesonidaRESUMO
Introduction Angiosarcoma (AG) is a malignant mesenchymal neoplasm that predominantly affects the soft tissues and, to variable degrees, expresses themorphological and functional characteristics of the endothelium. The incidence of sarcomas of the central nervous system(CNS) is low (0.5% to 2.7%), and AGs involving the brain are even rarer. Case Description A 45-year-old male patient presented with complaints of headache, nausea, and vomiting. An examination showed bilateral papilledema and a right lung pleurotomy. The patient's previous history included drug addiction, pulmonary tuberculosis, lung abscess, pleural empyema, and pulmonary artery embolization for severe hemoptysis. Computed tomography/magnetic resonance imaging scans revealed a large intra-axial lesion extending into the right parietal and temporal lobes, with hemorrhagic zones. The patient underwent surgical resection of the lesion. Microscopy showed a poorly-differentiated, high-grade malignant tumor composed of plump/epithelioid cells forming small vascular spaces and solid nests, compatible with AG.In the postoperative period, the patient developed recurrent hemoptysis. A biopsy of the tissues adjacent to the pleurotomy determined the diagnosis of pulmonary AG. At 30 days after the resection, the patient died from hemoptysis, hemothorax, lung atelectasis, and intracranial hypertension related to the recurrence of the brain tumor. Conclusion Angiosarcoma is a rare neoplasia related to short survival due to the high proliferative index, which must be considered in patients presenting hemorrhagic tumors. No specific genetic abnormalities have been described for this neoplasia.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Tuberculose Pulmonar/etiologia , Anemia , Hemangiossarcoma/cirurgia , Hemangiossarcoma/complicações , Prognóstico , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias do Sistema Nervoso Central/diagnóstico , Hemangiossarcoma/fisiopatologia , Hemangiossarcoma/diagnóstico por imagem , Metástase NeoplásicaRESUMO
BACKGROUND: BK polyomavirus-associated nephropathy (PyVAN) is an important complication after kidney transplantation. Prevalence ranges from 1% to 10%, and graft loss occurs in approximately 50% of the cases. There is no effective treatment, so early viral detection with immunosuppression tapering is the current strategy to prevent PyVAN. AIMS: To verify the frequency of PyVAN in a single center and evaluate the response to immunosuppressive adjustments through graft survival analysis. METHODS: Retrospective evaluation of a cohort of kidney transplant recipients with biopsy-proven PyVAN, compared with no-PyVAN patients regarding clinical aspects, immunosuppression, and graft survival over at least 2 years. RESULTS: There were 1404 kidney transplants analyzed in the study period, 58 with biopsy-proven PyVAN. Cumulative incidence was 4.1%. Median time from transplantation to PyVAN diagnosis was 6 (1-41) months. PyVAN was associated with recipient male gender (P = .041) and deceased donation (P = .005). Graft survival was inferior for PyVAN compared to no-PyVAN patients, 81.8% vs 75.2%, P = .019. Thirteen (22.4%) PyVAN patients lost their grafts, nine (15.5%) losses attributed to BKPyV infection. Three patients with BKPyV-associated graft losses were submitted to a successful second kidney transplant, with no evidence of viral replication during follow-up. CONCLUSION: PyVAN still is an important cause of kidney graft failure. Even though implementing active vigilance and immunosuppressive adjustment, this real-life single-center study demonstrated inferior graft survival in PyVAN patients compared to non-PyVAN.
Assuntos
Rejeição de Enxerto/virologia , Nefropatias/virologia , Transplante de Rim/efeitos adversos , Infecções por Polyomavirus/etiologia , Infecções Tumorais por Vírus/etiologia , Adulto , Vírus BK/patogenicidade , Feminino , Sobrevivência de Enxerto , Humanos , Terapia de Imunossupressão/efeitos adversos , Rim/patologia , Rim/virologia , Nefropatias/prevenção & controle , Masculino , Pessoa de Meia-Idade , Infecções por Polyomavirus/complicações , Estudos Retrospectivos , Fatores Sexuais , Transplante Homólogo/efeitos adversos , Infecções Tumorais por Vírus/complicações , Viremia , Adulto JovemRESUMO
Abstract Lipoprotein glomerulopathy (LPG) is an uncommon cause of nephrotic syndrome and/or kidney failure. At microscopy, LPG is characterized by the presence of lipoprotein thrombi in dilated glomerular capillaries due to different ApoE mutations. ApoE gene is located on chromosome 19q13.2, and can be identified in almost all serum lipoproteins. ApoE works as a protective factor in atherosclerosis due its interaction with receptor-mediated lipoprotein clearance and cholesterol receptor. Most common polymorphisms include ApoE2/2, ApoE3/2, ApoE3/3, ApoE4/2, ApoE4/3, and ApoE4/4. All age-groups can be affected by LPG, with a discrete male predominance. Compromised patients typically reveal dyslipidemia, type III hyperlipoproteinemia, and proteinuria. LPG treatment includes fenofibrate, antilipidemic drugs, steroids, LDL aphaeresis, plasma exchange, antiplatelet drugs, anticoagulants, urokinase, and renal transplantation. Recurrence in kidney graft suggests a pathogenic component(s) of extraglomerular humoral complex resulting from abnormal lipoprotein metabolism and presumably associated to ApoE.
Resumo A glomerulopatia por lipoproteínas (GLP) é uma patologia rara que causa síndrome nefrótica e/ou insuficiência renal. Na microscopia, a GLP é caracterizada pela presença de trombos de lipoproteínas em capilares glomerulares dilatados devido a diferentes mutações no gene da ApoE. O gene da ApoE está localizado no cromossomo 19q13.2 e pode ser identificado em quase todas as lipoproteínas séricas. A ApoE age como fator de proteção na arterioesclerose por conta de sua interação com a depuração de lipoproteínas mediada por receptores e com o receptor de colesterol. Dentre os polimorfismos mais comuns destacam-se ApoE2/2, ApoE3/2, ApoE3/3, ApoE4/2, ApoE4/3 e ApoE4/4. A GLP pode acometer indivíduos de todas as faixas etárias, com discreta predominância do sexo masculino. Pacientes afetados tipicamente apresentam dislipidemia, hiperlipoproteinemia tipo III e proteinúria. O tratamento da GLP é conduzido com fenofibrato, antilipêmicos, corticosteroides, LDL-aferese, troca de plasma, antiplaquetários, anticoagulantes, uroquinase e transplante renal. Recidiva no enxerto renal indica a existência de componentes patogênicos do complexo humoral extraglomerular resultante de metabolismo lipoproteico anômalo, possivelmente associado a ApoE.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Adulto , Pessoa de Meia-Idade , Nefropatias/patologia , Nefropatias/terapia , Apolipoproteínas E/genética , Fatores Sexuais , Transplante de Rim , Resultado do Tratamento , Nefropatias/complicações , Nefropatias/genética , Falência Renal Crônica/cirurgia , Falência Renal Crônica/etiologia , Mutação , Hipolipemiantes/uso terapêuticoRESUMO
Lipoprotein glomerulopathy (LPG) is an uncommon cause of nephrotic syndrome and/or kidney failure. At microscopy, LPG is characterized by the presence of lipoprotein thrombi in dilated glomerular capillaries due to different ApoE mutations. ApoE gene is located on chromosome 19q13.2, and can be identified in almost all serum lipoproteins. ApoE works as a protective factor in atherosclerosis due its interaction with receptor-mediated lipoprotein clearance and cholesterol receptor. Most common polymorphisms include ApoE2/2, ApoE3/2, ApoE3/3, ApoE4/2, ApoE4/3, and ApoE4/4. All age-groups can be affected by LPG, with a discrete male predominance. Compromised patients typically reveal dyslipidemia, type III hyperlipoproteinemia, and proteinuria. LPG treatment includes fenofibrate, antilipidemic drugs, steroids, LDL aphaeresis, plasma exchange, antiplatelet drugs, anticoagulants, urokinase, and renal transplantation. Recurrence in kidney graft suggests a pathogenic component(s) of extraglomerular humoral complex resulting from abnormal lipoprotein metabolism and presumably associated to ApoE.
Assuntos
Nefropatias/patologia , Nefropatias/terapia , Adulto , Apolipoproteínas E/genética , Pré-Escolar , Feminino , Humanos , Hipolipemiantes/uso terapêutico , Nefropatias/complicações , Nefropatias/genética , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Masculino , Pessoa de Meia-Idade , Mutação , Fatores Sexuais , Resultado do TratamentoRESUMO
ABSTRACT Despite the advances in knowledge of the neoplasm behavior, the exact understanding of the biological processes that involve the malignant tumors is not yet fully elucidated. Mechanisms such as subversion of phagocytosis, cytokines and chemokines expression, blocking of immunity and apoptosis regulation are recognized ways of tumor survival. Several of these mechanisms are also used as survival strategies of microorganisms. This article makes an analogy and compares the biological behavior of bacteria, viruses and neoplasms, showing several common points in their mechanisms of growth and survival, questioning cancer as a distinct living organism and introducing the hypothesis of synergy between viruses and bacteria in the development of neoplasms. Up to now, there are no experimental studies that effectively investigate the association between more than one microorganism as an etiologic factor of cancer.
RESUMO Apesar dos avanços no conhecimento do comportamento das neoplasias, o entendimento exato dos processos biológicos que envolvem os tumores malignos ainda não está totalmente elucidado. Mecanismos como subversão da fagocitose, expressão de citoquinas e quimiocinas, bloqueio da imunidade e regulação da apoptose são vias conhecidas de sobrevivência dos cânceres. Vários desses mecanismos também são utilizados como estratégias de sobrevivência de microrganismos. Este artigo faz uma analogia e compara o comportamento biológico das bactérias, dos vírus e das neoplasias, mostrando vários pontos em comum nos seus mecanismos de crescimento e sobrevivência, questionando o câncer como um organismo vivo distinto e introduzindo a hipótese de sinergia entre vírus e bactérias no desenvolvimento das neoplasias. Não há, até o momento, estudos experimentais que investiguem efetivamente a associação entre mais de um microrganismo como fator etiológico do câncer.