ABSTRACT C3 glomerulopathies are a rare group of glomerular diseases resulting from excessive activation of the alternative complement pathway. The pathogenesis involves genetic, acquired, or immunologic defects in regulators of the alternative complement pathway. Currently, these diseases have a chronic course with progression to end-stage renal disease in up to 50% of adult patients. We report a case of a young woman who presented with nephrotic syndrome and renal disfunction. During follow-up, the diagnosis of C3 glomerulopathy associated with complement factor I deficiency was established. After relapse of nephrotic proteinuria, immunosuppressive therapy with mycophenolate mofetil and glucocorticoid was added to supportive care. There is no disease-specific therapy for C3 glomerulopathies, and treatment is focused on reducing proteinuria and renal inflammation. Supportive treatment with ACE inhibitors or angiotensin receptor blockers is the first-line therapy. Immunosuppressive therapy with mycophenolate mofetil plus corticosteroids is associated with higher chances of clinical remission and lower risk of kidney failure. Complement targeting therapies, such as eculizumab, avacopan, danicopan, iptacopan, and pegcetacoplan, are being studied as potential new therapeutic options. This article highlights the need for more clinical trials to test new therapeutic agents, as well as to better understand the heterogeneity of the disease and its long-term outcomes.

ABSTRACT This paper aims to map the different theoretical options related to the Precautionary Principle (PP). Great part of the literature on it can be systematized by answering to three different questions: is there a basic structure in the PP? If so, in which interpretation of the PP does this structure express itself? Finally, are its damage or knowledge conditions fixed or adjustable? The first question separates realist from non-realist approaches. The second question allows us to discriminate monist, dualist, or pluralist positions in relation to the three interpretations of the PP: decision rule, procedural requirement, or epistemic rule. Finally, the third question distinguishes rigid from non-rigid formulations of the principle. Based on this mapping, one can not only navigate through the different formulations of the PP present both in official documents and in specialized literature, but also deflect some of its common objections, and understand Hans Jonas’ eventual connection with PP. Notwithstanding, this mapping does not capture other important themes attached to PP, which motivates a final distinction between narrow and broader forms of PP.

RESUMO Este artigo tem como objetivo mapear as diferentes opções teóricas relativas ao Princípio da Precaução (PP). Grande parte da literatura sobre ele pode ser sistematizada ao se responder três questões: há uma estrutura básica no PP? Em caso positivo, em qual interpretação do PP tal estrutura se expressa? Por fim, suas condições de dano ou de conhecimento são fixas ou ajustáveis? A primeira pergunta separa abordagens realistas daquelas não realistas. Por sua vez, a segunda pergunta permite discriminar posições monistas, dualistas ou pluralistas em relação às três interpretações do PP: regra de decisão, requerimento procedimental ou regra epistêmica. Por fim, a terceira pergunta distingue formulações rígidas do princípio daquelas não rígidas. Com base em tal mapeamento, pode-se não apenas navegar pelas distintas formulações do PP presentes tanto em documentos oficiais quanto na literatura especializada, mas também contornar algumas de suas objeções comuns, e entender a eventual conexão de Hans Jonas com PP. Não obstante, esse mapeamento não captura outros temas importantes relacionados ao PP, o que motiva uma última distinção entre formulações estreitas e amplas do PP.

The authors present two cases of tubulointerstitial nephritis and uveitis syndrome (TINU). TINU is a well-known yet rarely reported oculorenal inflammatory clinical entity. A high level of suspicion is needed to identify this entity. The first case is that of a 24-year-old female presenting with bilateral red eye and ocular pain. Laboratory investigation revealed renal insufficiency (serum creatinine 4.9mg/dL) and kidney biopsy presented tubulointerstitial nephritis. The second case is that of a 69-year-old female initially diagnosed with uveitis who later presented with general symptoms and renal insufficiency (serum creatinine 3.96 mg/dL) and whose kidney biopsy showed a tubulointerstitial nephritis pattern. Both patients were treated with steroids and renal function improved. It may be worth screening for uveitis in all patients with tubulointerstitial nephritis and evaluating renal function in those presenting with apparent idiopathic uveitis.

Introduction: Preservation of urine volume and residual renal function in patients on peritoneal dialysis (PD) is a major concern. Some factors have been associated with better prognosis, such as the use of biocompatible solutions, furosemide, or renin-angiotensin-aldosterone system blockers. However, results from previous studies have not been consistent. We thus aimed to study the relation between baseline characteristics of incident patients on PD, treatment characteristics, glomerular filtration rate (GFR) and urine volume (UV) variation. Subjects and methods: We retrospectively analyzed incident patients on PD (first option) in our unit in terms of variation of UV and GFR after 24 months of follow-up. We studied the association between GFR and UV decline and baseline characteristics (age, gender, diabetes mellitus or hypertension diagnoses, body mass index, CKD etiology, and use of beta-blockers, diuretics, renin-angiotensin-aldosterone system blockers) as well as PD treatment characteristics (PD modality, use of icodextrin, dialysis days per week, presence of peritonitis, membrane characteristics such as Ca125 peritoneal level and dialysate-to-plasma creatinine), dwell hours per day and glucose load) using Spearman correlation for numerical variables and differences of means for binomial variables. Results: We analyzed 25 patients. Urine volume decreased on average 0.59 mL after 24 months and glomerular filtration rate declined from 7.9 to 7.03 mL/min/1.73m2. All patients used biocompatible solutions. We did not find any association between glucose-exposure, use of diuretics or renin-angiotensin-aldosterone system blockers and urine volume or glomerular filtration rate decline. There was a significant relation between diuresis and GFR changes. Discussion: Our patients present a slower decline of residual renal function than that described in the literature. Strategies to preserve diuresis, including the use of biocompatible solutions, may explain these results.

ABSTRACT In the article is analyzed the relationship among the academic index as value of the quality in the abilities and knowledge acquired by the students in the pre-university teaching and the average in the entrance test to the superior education. The fundamental objective was directed to determine the influence that exercises the academic index in the results of the students in the entrance exams and in the final average in the career grant for the continuation of university studies in Pinar del Río province, among the years 2016 and 2017. The study had a descriptive character, being used statistical methods that allowed to determine the contradiction among the results obtained by the students in the entrance test and its academic index and the questioning of this last one as a real and reliable value of the academic quality of the students that they opt for the continuity of university studies.

RESUMEN En el artículo se analiza la relación entre el índice académico como valor de la calidad en las habilidades y conocimientos adquiridos por los estudiantes en la enseñanza preuniversitaria y el promedio en los exámenes de ingreso a la educación superior. El objetivo fundamental estuvo dirigido a determinar la influencia que ejerce el índice académico en los resultados de los estudiantes en los exámenes de ingreso y en el promedio final en el otorgamiento de carrera para la continuación de estudios universitarios en la provincia de Pinar del Río entre los años 2016 y 2017. El estudio tuvo un carácter descriptivo, fundamentado en el análisis estadístico de los documentos finales de otorgamiento de carrera universitarias en la provincia de Pinar del Río en los años 2016 y 2017, utilizándose métodos estadísticos de tendencia central, dispersión y correlación de datos que permitieron determinar la contradicción entre los resultados obtenidos por los estudiantes en los exámenes de ingreso y su índice académico y el cuestionamiento de este último como un valor real y fiable de la calidad académica de los estudiantes que optan por la continuidad de estudios universitarios.

Crescentic glomerulonephritis is a severe form of glomerular inflammation. IgA nephropathy (IgAN) is the most common primary glomerulonephritis and rarely presents as crescentic glomerulonephritis with rapidly progressive glomerulonephritis. Treatment of IgAN includes renin-angiotensin system blockade and immunosuppression in select cases with persistent proteinuria and/or renal failure, which are also indications for biopsy. Prognosis markers guide treatment and crescents are recognized as an indication of powerful immunosuppression as they are associated with rapid decline of renal function. We describe a case of crescentic IgA nephropathy with preserved renal function that was associated with several episodes of severe tonsillitis. Renin-angiotensin system blockade and corticosteroids were used and renal function remains stable. To our knowledge, this is the third case described in the literature of a patient with crescents and IgA glomerular deposition but with preserved renal function. A more benign aspect of crescents and association with tonsillitis episodes may explain a more favorable prognosis

Introduction: Complex procedures for anorectal disorders are uncommonly performed as Ambulatory Surgery (AS). The aim of this study was to describe the early results of an AS program that included complex procedures such as advancement rectal flaps for fistula repair. Material and methods: A retrospective descriptive study was performed with 186 patients who were submitted to AS because of benign anorectal disorders. The AS program for anorectal disorders started when a Colorectal Surgery Unit with broad experience in anorectal surgery joined an AS Unit that was already working in the hospital. Hospital admissions and need of early reoperation were analyzed. Results: One hundred and ninety seven procedures were performed between January 2014 and December 2015. One hundred and five (53.3%) fistula repair surgeries were performed, as well as 33 (16.8%) hemorrhoidectomies and 11 (5.6%) anal sphincter repairs. Among the patients who were operated because of an anorectal fistulae, 25 (23.8%) were submitted to advancement rectal flap. One patient (0.5%) could not be discharged after the surgery because the procedure performed was more complex than previously expected. All other patients were discharged. Three patients (1.5%) were addmited during early postoperative course, all of them had to undergo revisional surgery because of hemorrhage, pain and anorectal abscess respectively. Conclusions: Anorectal procedures, including complex procedures such as advancement rectal flap and sphincter repair, can be performed as AS with a low percentage of patients addmited before or after discharge.

Introducción: La realización en régimen de cirugía mayor ambulatoria (CMA) de procedimientos proctológicos complejos es aún infrecuente en nuestro medio. El objetivo del presente trabajo es presentar los resultados iniciales de un programa de cirugía proctológica en régimen de CMA que incluye procedimientos complejos tales como esfinteroplastias o colgajos de avance rectal. Material y métodos: Se realizó un estudio descriptivo retrospectivo en el que se incluyeron 186 pacientes intervenidos de patología proctológica en un programa de CMA. El programa se basó en la unión de una Unidad de Coloproctología con amplia experiencia en cirugía proctológica y una Unidad de CMA ya establecida en el centro. Se analizaron los resultados tanto de ingreso como de reintervención en el postoperatorio inmediato y precoz. Resultados: Entre enero de 2014 y diciembre de 2015 se realizaron un total de 197 intervenciones, destacando 105 (53,3%) cirugías de fístula anal, 33 (16,8%) hemorroidectomías y 11 (5,6%) esfinteroplastias. De los pacientes intervenidos por fístula perianal, en 25 (23,8%) se realizó un colgajo de avance rectal. Una paciente (0,5%) requirió ingreso tras la cirugía por haberse realizado un proceso más complejo del inicialmente programado, todos los demás pacientes pudieron ser dados de alta sin complicaciones. Tres pacientes (1,5%) reingresaron en el postoperatorio precoz y fueron reoperados debido a una hemorragia poshemorroidectomía, un absceso perianal y dolor anal. Conclusiones: La implementación de un programa de patología proctológica en régimen de CMA que incluya procedimientos complejos como el colgajo de avance endorrectal o la esfinteroplastia es factible, con una baja tasa de ingresos en hospitalización y reingresos posteriores.

A 45-year old white female with personal history of psychiatric disorder, severe hypokalaemia, medullary sponge kidney and chronic kidney disease, not on dialysis, was admitted to the Emergency Department after syncope. She was hypotensive and dehydrated. Arterial blood revealed metabolic alkalosis and no hypoxaemia. Laboratory tests revealed altered renal function, hypokalaemia, hyperparathyroidism, normal calcaemia and slightly elevated phosphorus. Exuberant alterations on chest radiography led to performing a chest computed tomography, which was suggestive of pulmonary metastatic calcification. She also had signs of calcification of the kidney. The main diagnoses were chronic kidney disease, medullary sponge kidney, hypokalaemia, dehydration, hypotension and pulmonary metastatic calcification. Tissue calcification can be metastatic or dystrophic. Pulmonary metastatic calcification is most commonly due to chronic kidney disease. Risk factors for tissue calcification in this patient were chronic kidney disease, hyperparathyroidism and elevated phospho-calcium product. The hyperparathyroidism was most probably secondary. Treatment aimed at correcting the water and electrolyte disturbances and admission to the psychiatric ward, with improvement of renal function, normalization of blood pressure and correction of the hypokalemia. To treat hyperparathyroidism, she was referred for parathyroidectomy

A lesão renal aguda associada ao uso de contraste iodado é a 3ª causa mais frequente de lesão renal aguda intra-hospitalar, sendo responsável por 11% de todos os casos. A lesão renal aguda induzida pelo contraste associa-se a pior prognóstico a curto e longo prazo, bem como a maior duração do internamento hospitalar e maiores custos. À medida que o número de angiografias diagnósticas e terapêuticas e tomografias computorizadas aumenta na prática clínica e que maiores doses de contraste são administradas a doentes com idades mais avançadas e maior número de co-morbilidades, a lesão renal aguda induzida pelo contraste é um problema crescente. A lesão renal aguda induzida pelo contraste é uma forma única de lesão renal aguda na medida em que os factores de risco são conhecidos e é previsível no tempo. Assim, torna-se possível a implementação de medidas profilácticas nos doentes em risco. Os autores pesquisaram artigos sobre lesão renal aguda induzida pelo contraste e estratégias profilácticas em diversas revistas nefrológicas (‘‘American Journal of Kidney Disease’’, ‘‘Journal of the American Society of Nephrology’’, ‘‘Clinical Journal of the American Society of Nephrology’’, ‘‘Kidney International’’ and ‘‘Nephrology Dialysis Transplantation’’). Posteriormente, foram efectuadas pesquisas individuais na base de dados MEDLINE® de estudos randomizados controlados e metanálises para cada estratégia profiláctica encontrada. Várias abordagens na prevenção da lesão renal aguda induzida pelo contraste têm sido descritas, das quais a hidratação vigorosa e o uso e contraste iodado não-iónico são as mais importantes. A administração de N-acetilcisteína oral é também uma estratégia popular em virtude do seu favorável perfil de risco/benefício. As estatinas têm vindo a ser descritas como protectoras contra a lesão renal aguda induzida pelo contraste. Os autores fazem uma revisão da patologia e das intervenções profilácticas testadas, propondo uma estratégia preventiva.

Contrast-induced acute kidney injury is the third leading cause of intrahospital acute kidney disease, accounting for 11% of all cases. It is associated with a worse prognosis on the short- and long-term, as well as with extended hospital stay and increase in health-care costs. As the number of diagnostic and interventional angiographies and computerized tomography increases in clinical practice and higher doses of contrast media are administered to sicker and older patients, contrast-induced acute kidney injury is an increasing problem. Contrast-induced acute kidney injury is a unique form of acute kidney injury in that its risk factors are known and its timing predictable. This makes room for the implementation of prophylactic measures in patients at risk. Several articles were searched in nephrology journals (‘‘American Journal of Kidney Disease’’,‘‘Journal of the American Society of Nephrology’’, ‘‘Clinical Journal of the American Society of Nephrology’’, ‘‘Kidney International’’ and ‘‘Nephrology Dialysis Transplantation’’) for a global view on contrast-induced acute kidney injury and prophylactic strategies. Subsequently, individual searches were made on MEDLINE® database for randomized controlled trials and meta-analyses on each prophylactic strategy encountered. Several approaches to contrast-induced acute kidney injury prevention have been reported, of which vigorous hydration and the use of non-ionic contrast media are the most important. The administration of oral N-acetylcysteine is also a popular strategy in virtue of its favorable risk/benefit profile. Statins have also been reported as protective against contrast-induced acute kidney injury. The authors review the disease and studied prophylactic interventions, presenting a practical approach to the prevention of contrast-induced acute kidney injury.

O micofenolato de mofetil (MMF) genérico é uma alternativa mais barata e possivelmente tão eficaz quanto a molécula original na imunossupressão de manutenção em receptores de transplantes de órgãos sólidos, nomeadamente transplante renal. Para ser aprovada, cada nova formulação genérica tem que demonstrar bioequivalência ao fármaco original em voluntários saudáveis. No entanto, a farmacocinética do MMF não é necessariamente igual em voluntários saudáveis e receptores de transplantes. Perante a imposição da conversão dos doentes para MMF genérico os autores avaliaram e compararam exposição ao ácido micofenólico (AMF) com o fármaco original e com o genérico em 14 doentes convertidos ao genérico na sua unidade de transplante renal. A exposição ao AMF, avaliada pela AUC0-12h estimada a partir de 3 determinações séricas em tempos pré-definidos, foi ligeiramente mais elevada com a formulação genérica (40.49 versus 34.96mcg.h/mL, p = 0,041). Não se verificou variação significativa da TFGe, hemoglobina nem contagem de leucócitos após a conversão para o fármaco genérico. Apenas um dos doentes convertidos para MMF genérico desenvolveu efeitos adversos do foro gastrointestinal de novo que resolveram após reintrodução da molécula original. Em conclusão, na nossa amostra não se verificou variação clinicamente significativa na exposição ao AMF, função do enxerto renal nem na incidência de efeitos adversos com a formulação genérica, a curto prazo

Generic mycophenolate mofetil (MMF) is a cheaper and possibly equally effective option as the original molecule for maintenance immunosuppression in solid organ transplant recipients. In order to be approved, each generic formulation has to prove bioequivalence to the reference product in healthy volunteers. However, MMF pharmacokinetics may be different between healthy volunteers and transplant recipients. Faced with the introduction of the generic formulation in the pharmacy of their kidney transplant unit, the authors studied and compared the exposure to mycophenolic acid (MPA) before and after the switch in 14 kidney transplant recipients converted to the generic molecule. The exposure to MPA as evaluated by the AUC0-12h estimated by a limited sample strategy was slightly higher with the generic formulation (40.49 versus 34.96mcg.h/mL, p = 0.041). There was no significant variation in the eGFR, haemoglobin and leucocyte count after the switch. Only one of the patients converted to the generic formulation developed de novo adverse gastrointestinal effects, which resolved after restart of the original formulation. In conclusion, in our cohort there was no clinically significant variation in the exposure to MPA, kidney graft function or incidence of adverse events with the generic formulation on a short follow-up period

INTRODUÇÃO: O hipotiroidismo é amplamente reconhecido por seus efeitos sobre os diferentes sistemas orgânicos, levando ao hipometabolismo. No entanto, o hipotiroidismo subclínico, sua apresentação mais prevalente, tem sido recentemente relacionado ao risco cardiovascular e também com complicações materno-fetais em gestantes. OBJETIVOS: Nestas diretrizes clínicas, vários aspectos do hipotiroidismo foram discutidos com objetivos claros de ajudar os médicos a tratar pacientes com hipotiroidismo e de compartilhar algumas das nossas experiências clínicas na América Latina. MATERIAIS E MÉTODOS: A Sociedade Latino-Americana de Tireoide formou uma Força-Tarefa para desenvolver diretrizes baseadas em evidências clínicas sobre o hipotiroidismo. Foi realizada uma revisão sistemática da literatura existente, com foco em bancos de dados primários do MedLine/PubMed e Lilacs/SciELO. Foram feitas análises para avaliar a qualidade metodológica no sentido de selecionar os melhores estudos. A força de recomendação em uma escala de A-D foi baseada no Centro de Oxford para a Medicina Baseada em Evidência - Níveis de Evidência 2009 - , permitindo uma opinião imparcial, desprovida de pontos de vista subjetivos. As áreas de interesse compreenderam estudos de diagnóstico, triagem, tratamento e uma seção especial de hipotiroidismo na gravidez. RESULTADOS: Foram feitos vários questionamentos relacionados ao diagnóstico, triagem e tratamento do hipotiroidismo na população adulta e, especificamente, em mulheres grávidas. Foram elaboradas vinte e seis recomendações baseadas nas respostas a essas perguntas. Apesar da falta de evidências em algumas áreas como o tratamento do hipotiroidismo, de 279 referências, 73% eram de Grau A e B, 8% de Grau C e 19% de Grau D. CONCLUSÕES: Essas diretrizes baseadas em evidências clínicas sobre o hipotiroidismo poderão fornecer um critério consensual de como tratar o hipotiroidismo na América Latina. Apesar de a maior parte dos estudos referidos ser da experiência internacional em hipotiroidismo, o ponto de vista dos tiroidologistas da América Latina foi contemplado.

INTRODUCTION: Hypothyroidism has long been known for its effects on different organ systems, leading to hypometabolism. However, subclinical hypothyroidism, its most prevalent form, has been recently related to cardiovascular risk and also to maternal-fetal complications in pregnant women. OBJECTIVES: In these clinical practice guidelines, several aspects of this field have been discussed with the clear objectives of helping physicians treat patients with hypothyroidism, and of sharing some of our Latin American-based clinical experience. MATERIALS AND METHODS: The Latin American Thyroid Society commissioned a Task Force on Hypothyroidism to develop evidence-based clinical guidelines on hypothyroidism. A systematic review of the available literature, focused on the primary databases of MedLine/PubMed and Lilacs/SciELO was performed. Filters to assess methodological quality were applied to select the best quality studies. The strength of recommendation on a scale from A-D was based on the Oxford Centre for Evidence--based Medicine, Levels of Evidence 2009, allowing an unbiased opinion devoid of subjective viewpoints. The areas of interest for the studies comprised diagnosis, screening, treatment and a special section for hypothyroidism in pregnancy. RESULTS: Several questions based on diagnosis, screening, treatment of hypothyroidism in adult population and specifically in pregnant women were posed. Twenty six recommendations were created based on the answers to these questions. Despite the fact that evidence in some areas of hypothyroidism, such as therapy, is lacking, out of 279 references, 73% were Grade A and B, 8% Grade C and 19% Grade D. CONCLUSIONS: These evidence-based clinical guidelines on hypothyroidism will provide unified criteria for management of hypothyroidism throughout Latin America. Although most of the studies referred to are from all over the world, the point of view of thyroidologists from Latin America is also given.

The results of a descriptive cross-sectional research carried out at "Dr. Mario Escalona Reguera" Teaching Polyclinic, in Alamar, are showed in this article aimed at dealing with what happens when one of the spouses giving origin to the families included in this study die and, therefore, they live the evolutive stage of dissolution as a group. To this end, the family cards of the family physician's offices corresponding to a Basic Working Group were reviewed. 60 families that fulfilled all the previously established inclusion requirements were investigated. The interview was used as a technique to collect information. It was made with the previous consent of the person that had lost his/her spouse. Among the most important results, it was found that widowhood is faced by a greater number of women and that in general most of the interviewed did not expect their mate's death. The family is stressed as the main network of support at this stage, which is very difficult. The mood disorders related to the loss of the spouse are more referred to by females.

En este articulo se expresan los resultados de una investigación descriptiva de corte transversal, realizada en el Policlínico Docente "Dr. Mario Escalona Reguera", en Alamar, con la intención de abordar lo que sucede cuando muere uno de los cónyuges que dio origen a las familias incluidas en este estudio, y que por tanto, viven la etapa evolutiva de disolución como grupo. Para ello, se revisaron las fichas familiares de los consultorios pertenecientes a un Grupo Básico de Trabajo, conformando nuestro universo de investigación con 60 familias que cumplían Ios requisitos de inclusión establecidos previamente. Se utilizó la entrevista como técnica para la recogida de información, realizada con previo consentimiento del que afrontaba la pérdida del cónyuge en estas familias. Dentro los resultados más importantes se observó que la viudez, en nuestro trabajo, es afrontada por mayor número de mujeres, y que en general, la mayoría de los entrevistados no esperaban la muerte de su campanero(a). Se destaca la familia como la red de apoyo principal en esta etapa, percibida por demás, como muy difícil, y son más referidos los trastornos del estado de ánimo relacionados con la pérdida del cónyuge, en las féminas, que entre los hombres.