Demographic of short gut syndrome: Increasing demand is not followed by referral of potential candidates for small bowel transplantation

Background. Despite improvements in small bowel transplantation (SBTx), early referral of patients with irreversible intestinal failure (IF) remains a major obstacle. In this study we evaluated the demand for SBTx among seven surgical pediatric centers located at least 200 km from our center.Methods. From 1997 to 2001, 640 patients have been treated for neonatal diseases, including 248 who underwent a minor or major intestinal resection. Twenty-four patients with major resections presented with short gut syndrome, requiring total parenteral nutrition (TPN). The greatest demand was in postsurgical neonates with necrotizing enterocolitis, gastroschiesis, onphalocoeles, or midgut volvulus, and in three adults with postradiotherapy arteritis (n = 2) and mesenteric vein thromboses (n = 1). The median length of residual bowel after resection was 20 to 30 cm, without an ileocecal valve. Four patients were referred for SBTx evaluation; three died while awaiting a donor; 20 were not referred, among whom 14 died of TPN complications.Results. Approximately 62 children per year require nutritional support for IF, most of whom develop complications related to TPN. Because many patients who are TPN-dependent develop complications, we believe that early referral would reduce mortality.Conclusions. Greater medical awareness about the feasibility of SBTx procedures and earlier referral may improve results and quality of life after transplant.

Ocorrência de anticorpos par o vírus da artrite equina em cavalos criados nas mesorregiões macro metropolitana paulista e Campinas

Pós-graduação em Medicina Veterinária - FMVZ

Pesquisa da fração do sistema complemento C4d, capilarite e infiltração por macrófagos em biópsias de transplante renal: papel na rejeição aguda mediada por anticorpos e na sobrevida do enxerto

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Developing a provisional definition of flare in patients with established gout

Objective Various nonvalidated criteria for disease flare have been used in studies of gout. Our objective was to develop empirical definitions for a gout flare from patient-reported features. Methods Possible elements for flare criteria were previously reported. Data were collected from 210 gout patients at 8 international sites to evaluate potential gout flare criteria against the gold standard of an expert rheumatologist definition. Flare definitions based on the presence of the number of criteria independently associated with the flare and classification and regression tree approaches were developed. Results The mean +/- SD age of the study participants was 56.2 +/- 15 years, 207 of them (98%) were men, and 54 of them (26%) had flares of gout. The presence of any patient-reported warm joint, any patient-reported swollen joint, patient-reported pain at rest score of >3 (010 scale), and patient-reported flare were independently associated with the study gold standard. The greatest discriminating power was noted for the presence of 3 or more of the above 4 criteria (sensitivity 91% and specificity 82%). Requiring all 4 criteria provided the highest specificity (96%) and positive predictive value (85%). A classification tree identified pain at rest with a score of >3, followed by patient self-reported flare, as the rule associated with the gold standard (sensitivity 83% and specificity 90%). Conclusion We propose definitions for a disease flare based on self-reported items in patients previously diagnosed as having gout. Patient-reported flare, joint pain at rest, warm joints, and swollen joints were most strongly associated with presence of a gout flare. These provisional definitions will next be validated in clinical trials.

Arterite actínica de artéria subclávia: relato de caso e revisão de literatura

Diversas são as complicações possíveis da radioterapia na adjuvância do tratamento de neoplasias. Lesões actínicas de artéria subclávia em pacientes submetidos a este tipo de tratamento para neoplasia de mama são complicações conhecidas, porém com poucos relatos de casos publicados. No presente relato, descrevemos um caso de oclusão de artéria subclávia direita em paciente submetida à radioterapia para tratamento de neoplasia de mama, tratada com a revascularização convencional, com interposição de enxerto de politetrafluoretileno (PTFE). Na revisão da literatura realizada, foram encontrados doze casos descritos que evidenciaram diferentes opções terapêuticas. Concluímos que a arterite actínica de artéria subclávia é uma doença incomum, entretanto sua hipótese deve ser aventada em todos os pacientes com isquemia de membro superior já submetidos a tratamento de radioterapia.

Impatto della glomerulopatia cronica da trapianto sulla disfunzione tardiva del rene trapiantato

INTRODUCTION. Late chronic allograft disfunction (CAD) is one of the more concerning issues in the management of patients (pts) with renal transplant (tx). Humoral immune response seems to play an important role in CAD pathogenesis. AIM OF THE STUDY. To identify the causes of late chronic allograft disfunction. METHODS. This study (march 2004-august 2011) enrolled pts who underwent renal biopsy (BR) because of CAD (increase of creatininemia (s-Cr) >30% and/or proteinuria >1g/day at least one year after tx). BR were classified according to 1997/2005 Banff classification. Histological evaluation of C4d (positive if >25%), glomerulitis, tubulitis, intimal arteritis, atrophy/fibrosis and arteriolar-hyalinosis were performed. Ab anti-HLA research at BR was an inclusion criteria. Pts were divided into two groups: with or without transplant glomerulopathy (CTG). RESULTS. Evaluated BR: 93/109. BR indication: impaired s-Cr (52/93), proteinuria (23/93), both (18/93). Time Tx-BR: 7.4±6.3 yrs; s-Cr at BR: 2.7±1.4 mg/dl. CTG group(n=49) not-CTG group(n=44) p Time tx-BR (yrs) 9.3±6.7 5.3±5.2 0.002 Follow-up post-BR (yrs) 2.7±1.8 4.1±1.4 0.0001 s-Cr at BR (mg/dl) 2.9±1.3 2.4±1.5 NS Rate (%) of pts: Proteinuria at BR 61% 25% 0.0004 C4d+ 84% 25% <0.0001 Ab anti-HLA+ 71% 30% 0.0001 C4d+ and/or Ab antiHLA 92% 43% 0.0001 Glomerulitis 76% 16% <0.0001 Tubulitis 6% 32% 0.0014 Intimal arteritis 18% 0% 0.002 Arteriolar hyalinosis 65% 50% NS Atrophy/fibrosis 80% 77% NS Graft survival 45% 86% 0.00005 Histological Diagnosis: CTG group (n=49:Chronic rejection 94%;IgA recurrence + humoral activity 4%;IIA acute rejection + humoral activity 2%. Not-CTG group (n=44: GN recurrence 27%;IF/TA 23%; acute rejection 23%;BKV nephritis 9%; mild not specific alterations 18%. CONCLUSIONS: CTG is the morphological lesion mainly related to CAD. In the 92% of the cases it is associated with markers of immunological activity. It causes graft failure within five years after diagnosis in 55% of pts.

Rapid induction of remission in large vessel vasculitis by IL-6 blockade. A case series

OBJECTIVE: To evaluate the effect of IL-6 blockade using tocilizumab in inducing remission of arterial large vessel vasculitides (LVV). METHODS: Five consecutive patients with giant-cell arteritis (GCA) and two with Takayasu’s arteritis (TA) were treated by tocilizumab infusions (8 mg/kg). Tocilizumab was given every other week for the first month and once monthly thereafter. Clinical symptoms of disease activity, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level and glucocorticoid (GC) dosage necessary to maintain remission were prospectively assessed. MR angiography was performed to monitor local inflammation. RESULTS: Of the seven patients three were newly diagnosed and four showed GC resistance, i.e. GC could not be lowered to less than 7.5 mg/day. The mean follow-up time was 4.3 months (range 3–7 months). All patients achieved a rapid and complete clinical response and normalisation of the acute phase proteins. Remarkably, prednisone dosage could be reduced within 12 weeks to a mean of 2.5 mg/day (range 0–10 mg/day). No relapse and no drug-related side effects were noted. CONCLUSION: Collectively the data suggest that IL-6 blockade using tocilizumab qualifies as a therapeutic option to induce rapid remission in large vessel vasculitides.

Amaurosis fugax mit erhöhten Akutphasenproteinen

Case report of a 66-year-old woman with episodes of amaurosis fugax and hemicranic headache with otherwise normal ophthalmologic and neurological examinations and normal imaging. While ESR was in the normal range for patient's age, acute phase proteins (C-reactive protein and fibrinogen) were elevated. Giant cell arteritis was proved by temporal artery biopsy. Giant cell arteritis should be considered as an important differential diagnosis of amaurosis fugax even in patients with normal ESR. Acute phase protein testing can give relevant diagnostic information.

A zinc finger-containing papain-like protease couples subgenomic mRNA synthesis to genome translation in a positive-stranded RNA virus

The genome expression of positive-stranded RNA viruses starts with translation rather than transcription. For some viruses, the genome is the only viral mRNA and expression is regulated primarily at the translational level and by limited proteolysis of polyproteins. Other virus groups also generate subgenomic mRNAs later in the reproductive cycle. For nidoviruses, subgenomic mRNA synthesis (transcription) is discontinuous and yields a 5′ and 3′ coterminal nested set of mRNAs. Nidovirus transcription is not essential for genome replication, which relies on the autoprocessing products of two replicase polyproteins that are translated from the genome. We now show that the N-terminal replicase subunit, nonstructural protein 1 (nsp1), of the nidovirus equine arteritis virus is in fact dispensable for replication but crucial for transcription, thereby coupling replicase expression and subgenomic mRNA synthesis in an unprecedented manner. Nsp1 is composed of two papain-like protease domains and a predicted N-terminal zinc finger, which was implicated in transcription by site-directed mutagenesis. The structural integrity of nsp1 is essential, suggesting that the protease domains form a platform for the zinc finger to operate in transcription.

Hepatic expression of mature transforming growth factor beta 1 in transgenic mice results in multiple tissue lesions.

Aberrant expression of transforming growth factor beta 1 (TGF-beta 1) has been implicated in a number of disease processes, particularly those involving fibrotic and inflammatory lesions. To determine the in vivo effects of overexpression of TGF-beta 1 on the function and structure of hepatic as well as extrahepatic tissues, transgenic mice were generated containing a fusion gene (Alb/TGF-beta 1) consisting of modified porcine TGF-beta 1 cDNA under the control of the regulatory elements of the mouse albumin gene. Five transgenic lines were developed, all of which expressed the Alb/TGF-beta 1 transgene selectively in hepatocytes. The transgenic line 25 expressing the highest level of the transgene in the liver also had high (> 10-fold over control) plasma levels of TGF-beta 1. Hepatic fibrosis and apoptotic death of hepatocytes developed in all the transgenic lines but was more pronounced in line 25. The fibrotic process was characterized by deposition of collagen around individual hepatocytes and within the space of Disse in a radiating linear pattern. Several extrahepatic lesions developed in line 25, including glomerulonephritis and renal failure, arteritis and myocarditis, as well as atrophic changes in pancreas and testis. The results from this transgenic model strongly support the proposed etiological role for TGF-beta 1 in a variety of fibrotic and inflammatory disorders. The transgenic model may also provide an appropriate paradigm for testing therapeutic interventions aimed at neutralizing the detrimental effects of this important cytokine.

Elaboración de folletos informativos sobre enfermedades infecciosas de los equinos para transferir a centros reproductivos equinos

La elaboración de esta propuesta surge a raíz de la aparición de casos de equinos afectados y muertos por el Virus del Oeste del Nilo (VON) en la Provincia de Córdoba, y del re-surgimiento de un brote de Arteritis viral equina (AVE) en la provincia de Buenos Aires y de Córdoba en el corriente año 2010. Estas dos enfermedades consideradas exóticas históricamente para la provincia de Córdoba (y la Argentina) fueron diagnosticadas en el territorio provincial recientemente; el VON en 2010 y la AVE en 2000/1 y de nuevo en 2010. Está claro que existe una gran desinformación a nivel de productores, trabajadores y profesionales de la industria equina respecto a la existencia y forma de enfrentarse a éstas y otras enfermedades infecciosas que afectan a los equinos y en algunos casos también a los humanos. Es más, hemos podido comprobar cómo las autoridades sanitarias oficiales toman a veces medidas desacertadas frente al control de estas nuevas enfermedades, suponemos por falta de informacion. Además como docente de la asignatura "enfermedades transmisibles y toxicas de los equinos" por más de 15 años puedo asegurar que existe una carencia marcada de material didáctico y bibliográfico en español. En Córdoba existen importantes centros de reproducción equina, que concentran varios centenares de equinos de alto valor genético y de distintos orígenes geográficos como así también numerosos productores de equinos de distintas razas. A través de las consultas recibidas en algunos casos percibimos el grado de desinformación y sentimos la necesidad de difundir información necesaria respecto a las muchas enfermedades infecciosas que pueden afectar a los equinos. Los cambios climáticos, la globalización del mercado de semen, embriones y reproductores equinos, el tránsito de equinos a los distintos eventos deportivos o de recreación representan un riesgo significativo para la transmisión de enfermedades. Dos importantes centros equinos reproductivos ubicados en el sur de la provincia nos han manifestado su necesidad de conocer y estar actualizados sobre las enfermedades infecciosas que pueden y de hecho impactan sobre sus poblaciones equinas, Estos centros manejan poblaciones de yeguas donantes de embriones que representan una elit genetica de equinos de Salto y Polo principalmente, ademas a grandes poblaciones de yeguas receptoras de embriones que se utilizan como medio para la produccion de potrillos de alto valor. En centros de este tipo, la prevencion y control de enfermedades infecciosas especialmente aquellas potencialmente letales es critica, ya que su incidencia podria tener un gran impacto negativo y con las consecuentes pérdidas económicas para no solo estos centros sino tambien para sus clientes y para la provincia. Asimismo, como resultado de mi asistencia a la mesa provincial equina y el dialogo con personal de la Secretaría de Ganadería del Ministerio de Agricultura ganaderia y Alimentos de la Pcia, surgió la idea de elaborar folletos informativos para cada una de las principales enfermedades infecciosas para su distribucion en forma impresa en los distintos eventos hípicos de la provincia y en forma digital desde la pagina web de la Secretaría.

Isquemia Crítica dos Membros Superiores. Manifestação Inicial de Arterite de Células Gigantes - Caso Clínico

Introdução: A arterite de células gigantes (ACG), de etiologia desconhecida, é a vasculite sistémica mais comum nos adultos e pode ter uma ampla variedade de apresentações clínicas. Atinge mais frequentemente os ramos extracranianos da artéria carótida mas, em 10-15% dos casos, pode ocorrer o envolvimento das artérias subclávia, axilar e braquial. Caso clínico: Tratava-se de uma doente do sexo feminino, de 80 anos, com antecedentes de HTA e doença cerebrovascular. Foi observada no serviço de urgência por arrefecimento e dor em repouso nos membros superiores, com evidências de cianose digital distal bilateral. As queixas tinham tido início 2 meses antes e agravamento progressivo desde então. Realizou um angio-TC que mostrou a existência de oclusão de ambas as artérias axilares/braquiais proximais e imagens sugestivas de vasculite ao nível de ambas as artérias subclávias, aorta e artérias femorais comuns. Foi medicada com corticoterapia; contudo, por não apresentar melhoria significativa após 5 dias, optou-se por realizar um bypass carotídeo-umeral bilateral. Após a cirurgia, ocorreu resolução completa das queixas e a doente apresentava pulso radial palpável bilateralmente. Seis meses após a cirurgia, a doente encontrava-se assintomática e os bypasses permeáveis. Conclusão: O presente trabalho pretende expor o caso de uma doente com o diagnóstico inaugural e ACG,que se apresentou com isquemia crítica bilateral e simultânea. Este quadro clínico exigiu a realização de um procedimento de revascularização raro.

Caracterización y prevalencia del compromiso ocular en pacientes con enfermedad autoinmune y autoinflamatoria en un centro de referencia reumatológica en Colombia entre los años 2000 a 2015

RESUMEN Objetivo: Estimar la prevalencia de las diferentes enfermedades oftalmológicas que aparecen en el contexto de una enfermedad autoinmune (EAI) en pacientes de un centro de referencia reumatológica en Colombia, según características clínicas y sociodemográficas durante un período de 15 años, comprendido entre los años 2000 a 2015. Métodos: Se realizó un estudio descriptivo, observacional de prevalencia. El tipo de muestreo fue aleatorio estratificado con asignación proporcional en el programa Epidat 3.4. Los datos se analizaron en el programa SPSS v22.0 y se realizó análisis univariado de las variables categóricas, para las variables cuantitativas se realizaron medidas de tendencia central. Resultados: De 1640 historias clínicas revisadas, se encontraron 634 pacientes (38,65%) con compromiso ocular. Si excluimos los pacientes con SS, que por definición presentan ojo seco, 222 pacientes (13,53%) presentaron compromiso oftalmológico. Del total de pacientes, el 83,3% fueron mujeres. La AR fue la enfermedad autoinmune con mayor compromiso oftalmológico con 138 pacientes (62,2%), y en último lugar la sarcoidosis con 1 solo paciente afectado. La QCS fue la manifestación más común en todos los grupos diagnósticos de EAI, con 146 pacientes (63,5%). De 414 pacientes con Síndrome de Sjögren (SS) y QCS 8 presentaron compromiso ocular adicional, siendo la uveítis la segunda patología ocular asociada en pacientes con SS y la primera causa en las espondiloartropatias (71,4 %). Los pacientes con catarata (4,1%) presentaron la mayor prevalencia de uso de corticoide (88.8%). De 222 pacientes, 28 (12,6%) presentaron uveítis. Del total de pacientes, 16 (7,2%) presentaron maculopatía por antimalaráricos y 6 (18,75%) de los pacientes con LES. Los ANAS se presentaron en el 100% los pacientes con trastorno vascular de la retina. Los pacientes con epiescleritis presentaron la mayor proporción de positivización de anticuerpos anti-DNA. La EAI que más presentó epiescleritis fue LES con 4 pacientes (12,5%) El 22% de paciente con anticuerpos anti-RNP presentaron escleritis y 32,1% de los pacientes con uveítis presentaron HLA-B27 positivo. Las manifestaciones oftalmológicas precedieron a las sistémicas entre un 11,1% y un 33,3% de los pacientes. Conclusión: Las enfermedades oculares se presentan con frecuencia en los pacientes colombianos con EAI (38.65%), siendo la AR la enfermedad con mayor compromiso ocular (62,2%) y la QCS la enfermedad ocular con mayor prevalencia en todas las EAI (63,5%). La uveítis se presentó en 28 pacientes (12,6%). Las manifestaciones oftalmológicas pueden preceder a las sistémicas. El examen oftalmológico debe ser incluido en los pacientes con EAI, por ser la enfermedad ocular una comorbilidad frecuente. Adicionalmente, los efectos oftalmológicos de las medicaciones sistémicas utilizadas en EAI deben ser estrechamente monitorizados, durante el curso del tratamiento.