ABSTRACT Rituximab, a monoclonal antibody with the ability to bind itself to CD20, leads to a rapid depletion of B-cells (in around 24-72 hours), limiting antibody formation. The marker CD20 does not exist in any other cell type, which makes the action of rituximab specific. This antibody has been used in autoimmune entities such as rheumatoid arthritis and in hematological malignancies, but recently it has gained popularity as an important immunosuppressor in selected kidney diseases. In nephrology, this anti-CD20 antibody had its first indication in anti-neutrophil cytoplasmic antibody-associated vasculitis, but can now be used in primary membranous nephropathy, minimal change disease, lupus nephritis and others. There is also some data that indicates rituximab might have a promising role in other glomerular diseases in the future, but to date evidence is still lacking to recommend its widespread use. The aim of this document is to compile the most recent scientific evidence as to when rituximab should be used in the treatment of various glomerular diseases, and which other may come to benefit from it in the future.

Endogenous endophthalmitis is an intraocular infection secondary to the hematogenous spread of microorganisms. It is an ophthalmological emergency associated with serious complications if not promptly diagnosed and treated. Despite the susceptibility of hemodialysis patients to episodes of bacteremia associated with vascular access, it is considered a rare disease. In fact, very few cases are described worldwide. We present 3 cases of endogenous endophthalmitis, secondary to bacteremia associated to vascular access in patients under hemodialysis treatment, aiming to raise awareness of this not so rare metastatic infection, possibly underdiagnosed in this population

Immunoglobulin G4-related disease (IgG4-RD) is a rare, poorly understood immune mediated disorder. It is characterized by a wide clinical spectrum depending on the organs affected. Serum IgG4 may be elevated, but this is not mandatory. Imaging abnormalities are usually detected in the affected organs, which typically show enlarged dimensions. Definitive diagnosis is made upon tissue biopsy demonstrating lymphoplasmacytic infiltration with predominance of polyclonal IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The most common renal manifestations [(IgG4-related kidney disease (IgG4#8209;RKD)] include tubulointerstitial nephritis, membranous glomerulonephritis and pyelitis. There is, usually, good therapeutic response to corticosteroids, but rituximab may be needed in cases of relapsing or resistant disease. A diagnostic challenge, and because it diagnosis needs specific immunohistochemical staining techniques, IgG4-RKD should be contemplated in the differential diagnosis of obscure kidney disease in order not to be missed.

Novel oral anticoagulants (NOAC) are a group of drugs recently approved for the treatment of patients at high risk of arterial or venous thrombosis. Unlike vitamin K antagonists, NOAC have a faster onset of action, less drug interactions and do not need frequent monitoring, making their prescription more convenient for physicians and patients. However, both classes of drugs have the potential for nephrotoxicity. Anticoagulation-related nephropathy is a form of acute kidney injury caused by excessive anticoagulation, resulting in glomerular haemorrhage and tubular obstruction by red blood casts. It was first described with warfarin, but new cases have been reported with acenocumarol and dabigatran. We report a case of a 82-year-old woman suffering from atrial fibrillation under rivaroxaban treatment with previous normal renal function, admitted to our unit with gross haematuria and acute kidney injury. A renal biopsy revealed typical features of anticoagulant-related nephropathy superimposed on chronic interstitial nephritis and hypertensive nephroangiosclerosis. There was no recovery of renal function despite withdrawal of rivaroxaban and the patient started on chronic haemodialysis three months after initial episode. To the best of our knowledge, this is the first case of a patient with a biopsy-proven anticoagulant nephropathy related to rivaroxaban. As novel oral anticoagulants are increasingly being prescribed and available data on their safety concerning renal toxicity are scarce, this report highlights the importance of a cautious prescription of these drugs and regular clinical and laboratory evaluation to avoid poor clinical outcomes.

Tuberous sclerosis complex is an autosomal dominant disorder characterized by the development of multiple tumours in distinct organs, although the ones most frequently affected are the skin, central nervous system, kidney, lung and liver. The kidney is the third most frequently affected organ, and angiomyolipomas are the most common lesions. Two-thirds of patients have sporadic mutations of the genes responsible for the disease, called tuberous sclerosis complex 1 and 2, encoding hamartin and tuberin, respectively. Hamartin and tuberin are tumoural suppressor proteins that are engaged in the control of cell proliferation and differentiation. When the tuberous sclerosis complex 1-2 suffers mutation, the mammalian target of rapamycin complex 1 pathway is constitutively activated, leading to neoplastic growth. Everolimus is a drug that inhibits mammalian target of rapamycin pathway and it is being used successfully in the treatment of renal angiomyolipomas associated with tuberous sclerosis complex. The authors report a case of a 34-year-old woman with tuberous sclerosis and giant renal angiomyolipoma, who received everolimus 10 mg daily for 6 months, but this was not associated with a reduction in the angiomyolipoma volume. This case describes the use of a systemic therapy in a rare genetic disorder. Although the treatment with everolimus did not reduce the patient’s renal angiomyolipoma volume and, thus, was apparently ineffective, no new lesions, bleeding episodes or deterioration of the kidney function were observed, suggesting that everolimus may have prevented disease progression

Consolidação por Constrição Radial (CCR) é uma técnica de metalurgia do pó que possibilita a obtenção de peças metálicas próximas da forma final, com alta densidade e de formas complexas, com custos de produção mais baixos que as tecnologias convencionais. CCR assemelha-se, em alguns aspectos, ao processo de compactação isostática a quente, sendo que as principais diferenças referem-se ao fato de que o processo CCR utiliza moldes cerâmicos fabricados a partir do processo de microfusão e usa um meio compressor secundário na forma de pó, ambos confinados em um container metálico. Nesse trabalho, desenvolveu-se um sistema experimental de CCR, visando à produção de pré-formas de ferro de alta densidade. Como meio compressor secundário, foi utilizado pó de argila calcinada. As pré-formas produzidas foram caracterizadas quanto à densidade, microestrutura e dureza. Observou-se que a configuração do container metálico/meio compressor secundário/molde cerâmico tem grande influência nas características de densidade e dimensionais das pré-formas. As pré-formas obtidas apresentaram consolidação satisfatória do pó como indicado pela microestrutura apresentada, alta densidade, elevada dureza, pouca formação de óxido e uma correlação direta entre os valores de dureza e densidade.

The Radial Constriction Consolidation (RCC) process can be considered a powder metallurgical technique that allows for the fabrication of components with complex geometries, near-net-shape and full density at low production costs when compared to the traditional process. In some aspects, the RCC process is similar to the hot isostatic pressing process, but it uses a ceramic mold confectioned by investment casting and a powder as a secondary pressing media, all of which are confined in a metallic container. In this paper, a RCC experimental system was developed aiming to produce iron billets with the characteristics of near-net-shape and high density. Clay powder was used as a secondary pressing media. The obtained billets were characterized in terms of density, microstructure and hardness. It was observed that the configuration of the metallic container/ secondary pressing media/ceramic mold has an important influence on the characteristic of density and near-netshape of the billets. The billets presented satisfactory powder consolidation as shown in their microstructure, high density, elevated hardness, little oxide formation and a direct correlation between the values of density and hardness.

OBJETIVO: Meningiomas intraventriculares são lesões raras, mais ainda as que acometem o terceiro ventrículo. Na infância, somente 16 casos foram relatados na literatura. Acrescentamos este relato de meningioma da parte anterior do terceiro ventrículo em uma menina, juntamente com breve revisão da literatura. APRESENTAÇÃO E INTERVENÇÃO: Criança previamente hígida, de 5 anos, foi internada com história de vômitos e cefaléa de 2 meses de duração. Ao exame clínico e neurológico, foi notado somente papiledema. Foi realizada tomografia computadorizada de crânio, que mostrou volumosa lesão na linha média supratentorial, ocupando toda a região do terceiro ventrículo, captante, com dilatação ventricular suprajacente. A paciente foi submetida a craniotomia frontal direita, e a lesão totalmente removida através da via transcalosa interfornical. O pós-operatório foi sem intercorrências, e não foi necessária derivação ventricular permanente. Hoje, dois anos e meio após a alta, a criança está em acompanhamento ambulatorial, sem déficits neurológicos, com bom desempenho escolar e sem evidência de recidiva nas tomografias de controle. CONCLUSÃO: Meningiomas da parte anterior do terceiro ventrículo são lesões raras na infância. O tratamento cirúrgico dos meningiomas intraventriculares no passado era associado a altas taxas de morbidade e mortalidade. Com o advento das modernas técnicas neuroanestésicas e neurocirúgicas, e do suporte intensivo pós-operatório, atualmente estas lesões podem ser removidas na sua totalidade, com morbidade e mortalidade praticamente nulas.

Meningiomas in childhood are rare, and those located in the third ventricle are even rarer, with only 16 cases reported. A five-year-old girl was seen at our hospital with headache and bilateral papilledema. CT scan showed a large third ventricular mass and associated hydrocephalus. Transcallosal interfornical approach was performed with total removal of the tumor, that was attached to the choroid plexus of the anterior third ventricle. Histologically the lesion proved to be a transitional meningioma. The recovery was uneventful, a permanent shunt was not necessary. The diagnosis of these tumors can be done using CT scan or MRI, that usually are sufficient for surgical planning. Angiography usually is not required, unless a vascular lesion is concerned, or embolization is necessary. The treatment of these tumors is surgical, aiming the total removal of the lesion, usually curative once it is acomplished. We report this case, and add a brief review of the literature on third ventricle meningiomas.

Registro de dois casos da síndrome de medula presa nos quais o estabelecimento correto do diagnóstico permitiu adotar conduta terapêutica adequada. Esta consiste na ressecção cirúrgica do filum terminale. São comentados aspectos embriológicos e fisiopatogênicos de interesse à síndrome, bem como são analisadas suas manifestações clínicas principais, os exames complementares que possibilitam o diagnóstico, particularmente a mielografia, e aspectos da terapêutica cirúrgica.

The tethered spinal cord syndrome is a disease that ir part of the group of spinal dysraphisms, that was recently recognized as an individualized nosological entity, yet not frequently diagnosed among us. It is characterized by shortenning and thickening of the filum terminale which prevents the ascent of the spinal cord into spinal canal, the conus medularis abnormaly remainning in a low place. It is associated in all cases with spina bifida. The diagnosis is simple, once the disease is suspected. It is manifested by progressive motor or sensory deficit in the legs, urinary incontinence, scoliosis and leg or back pain, specially in young children. The plain lumbosacral RX always shows spina bifida. Myelography makes the diagnostic. It shows, basically, the negative image of the thickened filum terminale and the low placed conus medularis. Other exams which can help are the computadorized tomography and the ultra-sound of the spinal cord. The surgical treatment is very simple and heals without sequels if done in due time. It consists in a sectioning of the filum terminale through laminectomy Two cases diagnosed and treated at Hospital da Baleia, from Fundação Benjamim Guimarães, Belo Horizonte, are reported in this paper.

É relatado um caso de paciente apresentando a forma crônica da doença de Chagas, no qual foi observada a presença de encefalite chagásica aguda, com demonstração de leishmanias no sistema nervoso central. O caso é único na literatura, sendo interpretado como devido a agravamento da forma crônica da doença de Chagas pela presença de leucemia linfática crônica associada. A existência de leucemia linfática pode levar à extinção de clones linfocitários responsáveis por fenômenos imunitários, criando, assim, condições para o agravamento e agudização da forma crônica da doença de Chagas.

A case of chronic Chagas disease, associated with chronic lymphocitic leukemia is reported. Acute manifestations of Chagas disease, characterized by acute encephalitis, with demonstration of Leishmaniae in the central nervous system were observed. This aspect is unique in the literature, since Chagas encephalitis appears only as a manifestation of the acute form of the disease, and not as a complication of the chronic form. The fact is interpreted, in this case, as secondary to alteration of the immune state, due to the presence of chronic lymphocitic leukemia.