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1.
Iatreia ; 35(4)dic. 2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1534608

ABSTRACT

Antecedente: el trasplante renal es el tratamiento de elección en la enfermedad crónica terminal. Un adecuado seguimiento en el postrasplante mejora la supervivencia del injerto y del paciente a largo plazo. Objetivo: comparar los desenlaces clínicos de la población trasplantada renal que vive en el área metropolitana de Medellín con los que residen por fuera de este lugar, con el fin de plantear un modelo de atención para el seguimiento por medio de la telemedicina. Métodos: estudio descriptivo, retrospectivo y de un único centro. Se determinó la tasa de supervivencia mediante las curvas de Kaplan-Meier. Resultados: durante el período 2005-2015 se realizaron 754 trasplantes, el 42 % vivía por fuera del área metropolitana. Al agrupar esta cohorte según el lugar de residencia, se observó que la supervivencia de los pacientes residentes en el área metropolitana a 1, 3 y 5 años fue del 96,8 %, 93,7 % y 91,8 %, respectivamente, en contraste con el 94,4 %, 90,3 % y 85,2 % de los del área rural. Esta diferencia fue estadísticamente significativa a favor de los que viven en Medellín (log-rank test p = 0,048; Hazard ratio = 1,68; IC 95 % 0,99-2,84, p = 0,052). Conclusión: la supervivencia fue inferior en los pacientes trasplantados renales que viven por fuera del área Metropolitana. Lo anterior motiva el desarrollo de un modelo de atención para estos pacientes mediado por la telemedicina para facilitar el acceso al seguimiento postrasplante.


Background: Kidney transplantation is the treatment of choice for end-stage renal disease. An adequate post-transplant follow-up improves the graft and patient's long-term survival. Objective: The aim of this study was to compare the outcomes of kidney transplant patients who live in the Medellin metropolitan area with those who live outside this area, to propose a model for follow-up care through telemedicine. Methods: Descriptive, retrospective and one-center study. Kaplan-Meier method was used to determine the survival rate. Results: Between 2005 and 2015, 742 patients were transplanted, 42% of whom lived outside the metropolitan area. The survival rates after 1, 3 and 5 years of treatment in patients in the metropolitan area of Medellín compared to those outside were 96.8%, 93.7% y 91.8% and 94.4%, 90.3% y 85.2% respectively, with statistically significant differences (Log-rank test p=0.048, Hazard ratio 1.68, IC 95% 0.99-2.84, p=0.052). Conclusion: The survival rate was lower in kidney transplant patients living outside the urban area. These findings motivate the development of a telemedicine project to facilitate the follow-up of these patients after a kidney transplantation.

2.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1536020

ABSTRACT

Introducción la amiloidosis es una enfermedad rara, producto del plegamiento y depósito normal de proteínas en tejidos y órganos. Esta enfermedad puede tener un compromiso renal que se manifiesta con síndrome nefrótico y deterioro de la función renal y su etiología puede estar asociada a amiloidosis con compromiso sistémico, siendo la amiloidosis AL y la amiloidosis AA las más frecuentes, esta última está asociada a inflamación crónica grave de origen infecciosa o autoinmune. Para el diagnóstico es fundamental el estudio sistémico multidisciplinario (hematológico, cardiaco, autoinmune, infeccioso y neoplásico), y cuando hay compromiso renal: la biopsia con estudio completo de microscopía de luz, tinciones especiales incluyendo rojo congo, inmunofluorescencia y microscopía electrónica. Cuando no se logra establecer la causa, la espectrometría de masas es una ayuda crucial para el diagnóstico específico. Objetivo se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico. Presentación del caso se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico Discusión y conclusiones se considera que la espectrometría de masas es un estudio diagnóstico muy importante para establecer el diagnóstico etiológico de la amiloidosis cuando otros métodos no han logrado establecerlo.


Introduction Amyloidosis is a rare disease, resulting from the accumulation and deposition of insoluble proteins in tissues or organs. This disease may involve the kidney, resulting in nephrotic syndrome and renal failure. The amyloidosis has been associated with systemic involvement, with AL amyloidosis and AA amyloidosis being the most common. The last is associated with various inflammatory disorders as chronic infections and autoimmune diseases. A multidisciplinary approach is required to the diagnosis (hematologic, cardiac, autoimmune, infectious, neoplastic) and in cases of renal involvement, a kidney biopsy with complete study of light microscopy, special stains including congo red, immunofluorescence, electron microscopy is essential for diagnosis. In cases where the cause cannot be stablished, mass spectrometry is practical tool to the identification of the correct type of amyloidosis. Purpose Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis. Case presentation Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis Discussion and conclusion Mass spectrometry is considered a useful diagnostic test to confirm the etiology of amyloidosis, especially if other methods are insufficient to establish it.

3.
Acta méd. colomb ; 47(2): 35-38, Apr.-June 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1419922

ABSTRACT

Abstract The advanced stages of chronic kidney disease are associated with mineral and bone metabolism disorders, which increase the risk of serious complications such as uremic calciphylaxis. Below we present the case of a 65-year-old male patient with multiple comorbidities, including stage 5 chronic kidney disease with long-term hemodialysis treatment, who presented refractory secondary hyperparathyroidism complicated by penile necrosis secondary to uremic calciphylaxis. We believe this case may be useful in sensitizing the medical community on the seriousness of uremic calciphylaxis, emphasizing the importance of prevention and early diagnosis before complications such as necrosis occur. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).


Resumen Los estadios avanzados de la enfermedad renal crónica se asocian a alteraciones en el metabolismo mineral óseo, lo cual aumenta el riesgo de complicaciones graves como la calcifilaxis urémica. A continuación se presenta el caso de un paciente masculino de 65 años, con múltiples comorbilidades, entre ellas enfermedad renal crónica estadio 5 en terapia de hemodiálisis durante largo tiempo, quien presentó hiperparatiroidismo secundario de difícil manejo y se complicó con necrosis peneana secundaria a calcifilaxis urémica. Se considera que este caso puede ser útil para sensibilizar a la comunidad médica sobre la gravedad de la calcifilaxis urémica por lo cual es muy importante la prevención y realización de un diagnóstico temprano antes de que se produzcan complicaciones como la necrosis. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2224).

4.
Acta méd. colomb ; 47(2): 39-42, Apr.-June 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1419923

ABSTRACT

Abstract Thrombotic microangiopathies (TMAs) are characterized by microvascular occlusion secondary to diffuse endothelial damage which produces inflammation, platelet aggregation and red blood cell destruction, causing ischemic injury to the affected organ. They are clinically characterized by Coombs-negative microangiopathic hemolytic anemia, and multiple organ damage (mainly of the kidneys, central nervous system, cardiovascular apparatus and gastrointestinal tract). They may occur systemically or locally, and they have multiple etiologies. In patients with cancer, determining the cause of thrombotic microangiopathy is a great diagnostic challenge, with the most frequent etiologies being active malignant neoplasms, disseminated intravascular coagulation, infections and antineoplastic drugs. We present the clinical case of a patient with unresectable pancreatic adenocarcinoma on chronic gemcitabine treatment, and highlight the importance of suspecting and distinguishing chemotherapy-induced TMAs from neoplasm-induced TMAs, as their prognosis and treatment are very different. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).


Resumen Las microangiopatías trombóticas (MAT) se caracterizan por la oclusión microvascular como consecuencia de una lesión endotelial difusa que produce inflamación, agregación plaquetaria y destrucción de glóbulos rojos, causando daño isquémico del órgano afectado. Se caracterizan clínicamente por anemia hemolítica microangiopática, Coombs negativo, daño multiorgánico (principalmente de riñones, sistema nervioso central, aparato cardiovascular y tracto gastrointestinal). Su presentación puede ser sistémica o localizada y sus etiologías son múltiples. En los pacientes con cáncer es un gran reto diagnóstico establecer la causa de la microangiopatía trombótica, siendo las etiologías más frecuentes la neoplasia maligna activa, la coagulación intravascular diseminada, infecciones y medicamentos antineoplásicos. Se presenta el caso clínico de una paciente con adenocarcinoma cáncer de páncreas irresecable, en manejo crónico con gemcitabina y se resalta la importancia de sospechar y distinguir la MAT inducida por quimioterapia, de la causada por la neoplasia ya que el pronóstico y tratamiento son muy diferentes. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).

5.
Iatreia ; 35(2): 117-130, abr.-jun. 2022. tab
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1421626

ABSTRACT

Resumen El trasplante renal es el tratamiento de elección en la enfermedad renal crónica terminal porque mejora la calidad de vida y la supervivencia de los pacientes al compararlo con la diálisis. Sin embargo, para mantener un injerto funcional y evitar el rechazo es necesario el uso de inmunosupresión potente durante toda la vida del injerto, lo cual puede tener como complicaciones una mayor susceptibilidad a presentar infecciones, desarrollo de cáncer, alteraciones metabólicas y problemas cardiovasculares. Los pacientes infectados con el virus de la inmunodeficiencia humana tienen alto riesgo de desarrollar enfermedad renal crónica terminal por múltiples causas. En el siglo pasado, el trasplante renal se consideraba contraindicado para estos pacientes. No obstante, hoy en día el trasplante renal se considera una opción terapéutica para pacientes adecuadamente seleccionados y con protocolos de manejo bien establecidos. Reportándose supervivencia reportadas del injerto y del paciente a tres años de 88,2 % y 82,6 % respectivamente. Este artículo de revisión tiene como objetivo revisar la experiencia mundial existente en el manejo de los pacientes trasplantados renal con infección por VIH.


Summary Kidney transplantation is the recommended treatment for end-stage chronic kidney disease, improving patients' quality of life and survival compared to dialysis. Nevertheless, to keep a functional graft and avoid rejection, strong immunosuppression is required during the graft's lifetime, which can lead to complications such as increased susceptibility to infections, development of cancer, metabolic changes and cardiovascular problems. Patients infected with the human immunodeficiency virus are at high risk of developing end-stage renal disease. Previous this century, kidney transplantation was considered contraindicated for these patients group. However nowadays, kidney transplantation is a therapeutic option for well-selected patients and with well-established treatment protocols. Several studies reported a three-year graft survival rate of 88,2% and patient survival of 82,6%. In this article, we present an overview of the worldwide experience with the treatment of kidney transplant patients with HIV infection.

6.
Iatreia ; 35(2): 131-140, abr.-jun. 2022. tab, graf
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1421627

ABSTRACT

Resumen La enfermedad renal crónica (ERC) se define como la alteración funcional o estructural progresiva de los riñones que persiste por 3 meses o más. Esta enfermedad afecta el 10 al 15 % de la población mundial, siendo la diabetes mellitus, la hipertensión arterial, las glomerulopatías primarias y las enfermedades genéticas las etiologías más frecuentemente asociadas. Sin embargo, en los países pobres se reportan otras enfermedades causantes de la ERC; entre ellas la nefropatía mesoamericana (NM). La NM se presenta principalmente en la población masculina joven, lo cual lleva a pérdida de la vida laboral productiva. En Colombia hay una alta prevalencia de ERC con etiología no estudiada (mal llamada desconocida) y hay pocas investigaciones de la ERC en las comunidades agrícolas. Teniendo en cuenta que la población campesina de Colombia se expone a noxas climáticas y laborales similares a Mesoamérica, es posible que esta nefropatía también sea una causa de ERC en nuestra población. El presente artículo hace una revisión de la ERC de las comunidades agrícolas, con el fin de sensibilizar el personal de salud en la importancia de la búsqueda de esta enfermedad en la población vulnerable, lo cual podría impactar de una forma positiva en la salud de los agricultores y campesinos.


Summary Chronic kidney disease (CKD) is defined as an abnormality of the kidney structure or function for ≥ 3 months. This disease affects 10% to 15% of the world's population, with diabetes, arterial hypertension, primary glomerulopathies and genetic disorders being the most common etiologies associated with this disease worldwide. Nevertheless, in low-income countries, other diseases causing CKD are also reported; among them, Mesoamerican nephropathy, which is a common cause of CKD in Mesoamerica, especially in the young male population, leading to loss of productive working capacity. In Colombia, the prevalence of CKD with unknown etiology is high and there are few studies on chronic kidney disease in agricultural communities, given that the agricultural population in Colombia is exposed to harmful climatic and occupational agents similar to those in Mesoamerica, it is possible that this nephropathy is also a cause of CKD in our population. This article provides an overview of CKD in agricultural communities to sensitize health workers to the importance of screening for this disease in vulnerable populations, which could have a positive impact on the health of farmers.

7.
Acta méd. colomb ; 47(1): 44-48, ene.-mar. 2022. graf
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1374103

ABSTRACT

Resumen La espondiloartropatía destructiva es una patología osteoarticular presente en algunos pacientes con enfermedad crónica que puede afectar varios niveles de la columna vertebral y puede ser asintomática, generar dolor o causar complicaciones que ponen en peligro la integridad de la médula espinal y/o la vida. Presentamos el caso de un hombre de 70 años con enfermedad renal crónica terminal en hemodiálisis quien consultó por dolor dorsal y paraplejia, en quien se diagnosticó espondiloartropatía destructiva no infecciosa por imágenes y estudio histopatológico. Este caso nos muestra la importancia de pensar en esta patología y la necesidad de un enfoque multidisciplinario en el diagnóstico y manejo. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2193).


Abstract Destructive spondyloarthropathy is a bone and joint disease which presents in some patients with chronic illnesses and may affect various levels of the spinal column. It may be asymptomatic, cause pain, or produce spinal cord and/or life-endangering complications. We present the case of a 70-year-old man with end-stage renal disease on hemodialysis who consulted due to back pain and paraplegia. He was diagnosed with destructive noninfectious spondyloarthropathy through imaging and histopathological studies. This case shows us the importance of considering this disease and the need for a multidisciplinary approach in its diagnosis and management. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2193).

8.
Acta méd. colomb ; 46(4): 43-45, Oct.-Dec. 2021.
Article in English | LILACS-Express | LILACS | ID: biblio-1374087

ABSTRACT

Abstract Chronic kidney disease is a condition with high morbidity, mortality and healthcare costs which affects all population groups, having a significant impact on their quality of life. Its classification has been modified over time and there is still no universal consensus to differentiate a physiological change in kidney clearance from a pathological change. Below, we will discuss the importance of reconsidering the definition and classification in the general population according to age, including children and adults. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.2080).

9.
J. bras. nefrol ; 43(4): 591-596, Dec. 2021. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1350916

ABSTRACT

Abstract Thrombotic microangiopathies are disorders characterized by nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-systemic failure. They are classified as thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome, and typical hemolytic uremic syndrome. The latter is associated with intestinal infections by Shiga toxin-producing bacteria. Typical hemolytic uremic syndrome in adults is an extremely rare condition, characterized by high morbidity and mortality. It has been seldom described in solid organ transplant recipients. Here is presented the case of a kidney transplant recipient who had typical hemolytic uremic syndrome with multisystem commitment, refractory to management and with a fatal outcome.


Resumo Microangiopatias trombóticas são distúrbios caracterizados por anemia hemolítica microangiopática não imune, trombocitopenia e insuficiência multissistêmica. Elas são classificadas como púrpura trombocitopênica trombótica, síndrome hemolítico-urêmica atípica e síndrome urêmica hemolítica típica. Essa última está associada a infecções intestinais por bactérias produtoras da toxina Shiga. A síndrome hemolítica urêmica típica em adultos é uma condição extremamente rara, caracterizada por alta morbimortalidade. Esta é raramente descrita em receptores de transplantes de órgãos sólidos. Apresentamos aqui o caso de um receptor de transplante renal que apresentava síndrome hemolítico-urêmica típica com comprometimento multissistêmico, refratário ao tratamento, e com desfecho fatal.


Subject(s)
Humans , Adult , Purpura, Thrombotic Thrombocytopenic , Kidney Transplantation , Shiga-Toxigenic Escherichia coli , Atypical Hemolytic Uremic Syndrome , Anemia, Hemolytic
10.
Rev. colomb. nefrol. (En línea) ; 8(2): e203, jul.-dic. 2021. tab, graf
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1423853

ABSTRACT

Resumen Introducción: el trasplante renal es el tratamiento de elección para los pacientes con enfermedad renal crónica terminal; sin embargo, un trasplante renal anticipado no siempre es posible y muchos pacientes requieren algún tipo de terapia de reemplazo renal previa al trasplante. Objetivo: este estudio tiene como objetivo evaluar los desenlaces a corto y largo plazo, post-trasplante renal, de acuerdo con la modalidad de diálisis previa al trasplante. Métodos: estudio de tipo cohorte retrospectiva. Se incluyeron pacientes mayores de 18 años que recibieron un trasplante renal durante 2005-2018. Inicialmente se realizó estadística descriptiva y posteriormente se realizó análisis multivariado mediante un modelo de riesgos proporcionales de Cox, con el objetivo de evaluar la relación entre la supervivencia del paciente y del injerto y la necesidad de diálisis previa al trasplante. Se evaluó, además, la tasa de filtración glomerular durante los primeros dos años del trasplante renal. Resultados: durante 2005-2018 se realizaron 925 trasplantes renales, 289 estaban en diálisis peritoneal, 439 en hemodiálisis y 197 no estaban en diálisis. La supervivencia del paciente a 6, 12, 24, 48 y 60 meses posterior al trasplante renal fue de 97,5 %, 96,7 %, 96,0 %, 93,7 % y 92,3 %, respectivamente, y del injerto fue de 94,5 %, 92,9 %, 90,5 %, 84,8 %, 81,1 %, también respectivamente, sin encontrar diferencia estadísticamente significativa entre los que tuvieron diálisis peritoneal vs. hemodiálisis (p = 0,402, p = 0,180), tampoco se encontraron diferencias significativas en el análisis multivariado. El 8,1 % de los pacientes presentó rechazo agudo en el primer año post trasplante y el 13,2 % durante todo el seguimiento, sin encontrarse diferencias significativas entre los que habían tenido diálisis peritoneal vs. hemodiálisis. No se encontraron diferencias en la TFG. Conclusiones: no se encontraron diferencias estadísticamente significativas en los desenlaces duros a corto y largo plazo, según la modalidad de diálisis pretrasplante.


Abstract Introduction: kidney transplantation is the standard treatment option for patients with end-stage chronic kidney disease. However, early kidney transplantation is not always possible, and many patients require renal replacement therapy. This study aims to evaluate the short and long-term outcomes after kidney transplantation in patients undergoing dialysis therapy before transplantation. Methods: a retrospective cohort of renal transplant patients older than 18 years of age were evaluated during the years 2005-2018. A descriptive analysis was performed. A proportional Cox-Hazard model was used to evaluate the relationship between patient and transplant survival and pre-transplant dialysis. An analysis of variance was used to compare the glomerular filtration rate during the first two years after transplantation. Results: between 2005-2018, 925 kidney transplants were performed, of which 289 required peritoneal dialysis, 439 haemodialysis and 197 did not require dialysis. Patient and transplant survival rates at 6, 12, 24, 48, 60 months after kidney transplantation were 97.5%, 96.7%, 96.0%, 93.7%, 92.3%, 94.5%, 92.9%, 90.5%, 84.8%, 81.1%. No statistically significant difference was found between peritoneal dialysis patients and hemodialysis patients (p=0.402, p=0.180). Acute rejection occurred in 8.1% of patients in the first year after transplantation and in 13.2% during the entire follow-up. Glomerular filtration rate values were similar in patients with and those without pre-transplant dialysis Conclusions: there were no significant statistical differences in short and long-term outcomes among patients undergoing pre-transplant dialysis vs. no.

11.
J. bras. nefrol ; 43(3): 434-439, July-Sept. 2021. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1550478

ABSTRACT

Abstract BK virus nephropathy in kidney transplantation is widely recognized as an important cause of graft dysfunction and loss. In the case of transplants of organs other than kidney, BK virus nephropathy in native kidneys has been recognized as a cause of chronic kidney disease, which is related with immunosuppression; however, the diagnosis is usually late because the renal dysfunction is attributed to other causes, such as toxicity by anticalcineurinic drugs, interstitial nephritis due to medications, hemodynamic changes, diabetes, hypertension, etc. We report a case of BK virus nephropathy in a patient who underwent heart transplantation due to peripartum cardiomyopathy. The kidney biopsy reported active chronic tubulointerstitial nephritis associated with late stage polyomavirus nephritis and the blood viral load for BK virus was positive (logarithm 4.5). The immunosuppressive treatment was reduced, and after two years of follow-up, the patient had stable renal function with a serum creatinine of 2.5 mg/dL (GFR of 23.4 mL/min/1.73m2). We recommend that the BK virus be considered as a cause of renal dysfunction in heart transplant recipients, with the aim of detecting its replication in time to reduce immunosuppressive therapy before irreversible compromise of renal function may manifest.


Resumo A nefropatia pelo vírus BK no transplante renal é amplamente reconhecida como uma importante causa de disfunção e perda do enxerto. No caso de transplantes de órgãos que não sejam rins, a nefropatia pelo vírus BK em rins nativos tem sido reconhecida como uma causa de doença renal crônica, que está relacionada com imunossupressão; entretanto, o diagnóstico é geralmente tardio porque a disfunção renal é atribuída a outras causas, tais como toxicidade por drogas anticalcineurínicas, nefrite intersticial devido a medicamentos, alterações hemodinâmicas, diabetes, hipertensão, etc. Relatamos um caso de nefropatia pelo vírus BK em um paciente que foi submetido a transplante cardíaco devido à cardiomiopatia periparto. A biópsia renal relatou nefrite túbulo-intersticial crônica ativa associada à nefrite por poliomavírus em estágio avançado e a carga viral sanguínea para o vírus BK foi positiva (logaritmo 4,5). O tratamento imunossupressor foi reduzido, e após dois anos de acompanhamento, o paciente apresentava função renal estável com creatinina sérica de 2,5 mg/dL (TFG de 23,4 mL/min/1,73m2). Recomendamos que o vírus BK seja considerado como uma causa de disfunção renal em receptores de transplante cardíaco, com o objetivo de detectar sua replicação a tempo de reduzir a terapia imunossupressora antes que um comprometimento irreversível da função renal possa se manifestar.

12.
J. bras. nefrol ; 43(3): 440-444, July-Sept. 2021. graf
Article in English, Portuguese | LILACS | ID: biblio-1340119

ABSTRACT

Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.


Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.


Subject(s)
Humans , Child , Purpura, Thrombotic Thrombocytopenic , Atypical Hemolytic Uremic Syndrome/complications , Anemia, Hemolytic , Nephrotic Syndrome/complications , Complement System Proteins
13.
Med. lab ; 25(3): 569-580, 2021. ilus
Article in Spanish | LILACS | ID: biblio-1343146

ABSTRACT

La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad


Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options


Subject(s)
Glomerulonephritis , Purpura , IgA Vasculitis , Steroids , Biopsy , ISCOMs , Glomerulonephritis, IGA , Kidney Failure, Chronic
14.
Iatreia ; 33(4): 360-369, oct.-dic. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1143088

ABSTRACT

RESUMEN La tuberculosis es una enfermedad infecciosa y frecuente en países en vía de desarrollo. Esta puede causar una amplia variedad de complicaciones y presentaciones atípicas con alta morbimortalidad. De la forma genitourinaria se sospechada muy poco, razón por la cual su diagnóstico se hace, usualmente, de forma tardía o no se realiza. Esto conlleva a consecuencias muy graves en los pacientes, por ejemplo, la enfermedad renal crónica terminal. A continuación, se presenta un reporte de caso de una paciente con la anterior enfermedad, secundaria a una tuberculosis renal bilateral diagnosticada tardíamente y se realiza una revisión de la literatura sobre este tema.


SUMMARY Tuberculosis is a common infectious disease in developing countries, which can cause a variety of complications and atypical manifestations with high morbidity and mortality. The urogenital form is rarely suspected, resulting in delayed diagnosis or even no diagnosis, which can have serious consequences for the patients, such as chronic end-stage renal disease. We report on a patient with chronic end-stage renal failure caused by a delayed diagnosis of bilateral renal tuberculosis and a literature review on this topic.


Subject(s)
Humans , Tuberculosis, Urogenital , Kidney Failure, Chronic
15.
Acta méd. colomb ; 45(1): 37-39, Jan.-Mar. 2020. tab
Article in English | LILACS, COLNAL | ID: biblio-1124068

ABSTRACT

Abstract Renal crisis is a complication with significant morbidity and mortality in scleroderma patients and a rare entity in kidney transplantation. It may present with highly variable clinical manifestations, mainly arterial hypertension, acute kidney failure and multisystemic involvement. Following is the report of a patient with late reoccurrence of scleroderma crisis in kidney transplantation, who was given successful treatment with angiotensin-converting enzyme inhibitors.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1215).


Resumen La crisis renal es una complicación con importante morbilidad y mortalidad en pacientes con esclerodermia y una rara entidad en trasplante renal, que se puede presentar con manifestaciones clínicas muy variables, principalmente hipertensión arterial, falla renal aguda y compromiso multisistémico. A continuación se reporta el caso de una paciente con recurrencia tardía de la crisis esclerodérmica en el trasplante renal, que recibió tratamiento exitoso con inhibidores de la enzima convertidora de angiotensina.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1215).


Subject(s)
Humans , Female , Middle Aged , Acute Kidney Injury , Recurrence , Angiotensin-Converting Enzyme Inhibitors , Morbidity , Kidney Transplantation
16.
Iatreia ; 33(2): 155-166, 20200000. tab, graf
Article in Spanish | LILACS | ID: biblio-1114787

ABSTRACT

RESUMEN La nefritis tubulointersticial aguda hace referencia a un tipo de daño renal que afecta principalmente el intersticio y ocasiona la lesión renal aguda, potencialmente reversible. Su curso puede ser subclínico, con deterioro progresivo hasta evolucionar hacia la insuficiencia renal crónica. La nefritis tubulointersticial aguda tiene múltiples etiologías, las más frecuentes son los medicamentos, productos herbales, las infecciones y las enfermedades autoinmunes. Las principales manifestaciones clínicas son la poliuria, polaquiuria, nocturia, dolor lumbar, microhematuria y leucocituria, aunque puede ser totalmente asintomática. El tratamiento depende de la causa de base y los esteroides juegan un papel importante cuando la condición es de origen medicamentoso o autoinmune. El pronóstico generalmente es bueno, si el problema se identifica de forma oportuna y se trata adecuadamente.


SUMMARY Acute tubulointerstitial nephritis is a kidney lesion that mainly affects the interstitium and can lead to a reversible acute kidney injury. It can progress subclinically, with progressive development towards chronic renal failure. Acute tubulointerstitial nephritis has several causes, being the most common medications, herbal products, infections and autoimmune diseases. The main clinical manifestations are polyuria, polaquiuria, nycturia, lumbar pain, microhematuria, leukocyturia, although the patients also can be completely asymptomatic. Treatment is determined by the underlying cause. Steroids play an important role when tubulointerstitial nephritis is caused by medication or autoimmune diseases. The prognosis is usually good if the problem is detected early and treated properly.


Subject(s)
Humans , Nephritis, Interstitial , Renal Insufficiency , Acute Kidney Injury
17.
Iatreia ; 32(4): 311-320, oct.-dic. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1056311

ABSTRACT

RESUMEN La linfohistiocitosis hemofagocítica (LHH) posterior al trasplante renal hace referencia a un estado hiperinflamatorio grave, asociado a la activación no controlada de los linfocitos T citotóxicos y macrófagos por causa infecciosas y/o secundaria al tratamiento inmunosupresor. Las causas más prevalentes dentro de las infecciones son la histoplasmosis, la tuberculosis y las infecciones por virus herpes. Se caracteriza por fiebre, organomegalias, citopenias, hiperferritinemia, hipertrigliceridemia y/o hipofibrinogenemia; puede acompañarse con hemofagocitosis documentada en la médula ósea, el hígado u otros órganos. Su curso puede ser fulminante con progresión a falla multisistémica y la muerte. El tratamiento va enfocado a controlar tempranamente la causa desencadenante, reducir la inmunosupresión y controlar la inflamación. En pocos casos es necesario el uso de otros inmunosupresores, quimioterapia o, en situaciones muy seleccionadas, se puede requerir el trasplante de médula ósea.


SUMMARY Hemophagocytic lymphohistiocytosis (HLH) in renal transplant recipients is a life-threatening hyper-inflammatory syndrome; associated with uncontrolled activation of cytotoxic T-lymphocytes and macrophages due to infections or immunosuppressive therapy. Histoplasmosis, tuberculosis and herpes virus infection are among the leading infectious causes. It is characterized by fever, organomegaly, cytopenia, hyperferritinemia, hypertrigiceridemia and/or hypofibrinogenemia; which may be accompanied by hemophagocytosis in bone marrow, liver or other organs. HLH can follow a rapidly fatal course, with progression to multisystemic failure and death. The treatment is based on early control of the triggering cause, reducing immunosuppression and stop the inflammatory process. In some cases, is necessary to use other immunosuppressant, chemotherapy and in a very few cases, a bone marrow transplant may be required.


Subject(s)
Humans , T-Lymphocytes, Cytotoxic , Kidney Transplantation , Lymphohistiocytosis, Hemophagocytic
18.
J. bras. nefrol ; 41(3): 427-432, July-Sept. 2019. tab
Article in English | LILACS | ID: biblio-1040255

ABSTRACT

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.


Resumo Apesar de sua toxicidade, o metotrexato é um medicamento eficaz no controle de várias doenças. A mielossupressão, um de seus principais efeitos adversos, aumenta em gravidade e frequência nos pacientes com insuficiência renal. Apresentamos o caso de um homem de 68 anos de idade com doença renal terminal relacionada à vasculite associada ao ANCA em diálise peritoneal, que recebeu a medicação em dose baixa em função da atividade da doença e que teve como complicação pancitopenia grave com mucosite, tratada com medidas de suporte e diálise peritoneal com múltiplas trocas. Revisamos 20 casos publicados até o presente momento sobre pancitopenia associada a metotrexato em pacientes em diálise. Foi identificada alta morbidade e mortalidade, razão pela qual seu uso nesse tipo de paciente não é recomendado. No entanto, quando esta complicação ocorre, uma opção terapêutica pode ser o uso de diálise peritoneal com múltiplas trocas, além da terapia de suporte para toxicidade medicamentosa. Maiores estudos são necessários para demonstrar o papel da diálise peritoneal com múltiplas trocas na remoção desse medicamento.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Vasculitis/drug therapy , Methotrexate/adverse effects , Methotrexate/therapeutic use , Peritoneal Dialysis/methods , Folic Acid Antagonists/adverse effects , Folic Acid Antagonists/therapeutic use , Kidney Failure, Chronic/therapy , Pancytopenia/etiology , Pancytopenia/therapy , Shock, Septic/etiology , Shock, Septic/drug therapy , Methotrexate/blood , Treatment Outcome , Mucositis/etiology , Mucositis/drug therapy , Folic Acid Antagonists/blood , Anti-Bacterial Agents/therapeutic use
19.
Biomédica (Bogotá) ; 39(supl.2): 20-25, ago. 2019. graf
Article in Spanish | LILACS | ID: biblio-1038824

ABSTRACT

Resumen Las feohifomicosis cerebrales son infecciones graves causadas por mohos dematiáceos, entre los cuales Cladophialophora bantiana es una de las especies más comúnmente aislada. Esta tiene tropismo por el sistema nervioso central y frecuentemente produce abscesos cerebrales en pacientes inmunocompetentes; además, en los inmunocomprometidos también puede ocasionar infección diseminada. Pese a la disponibilidad de medicamentos antifúngicos de amplio espectro, a menudo se requiere también la intervención quirúrgica; de todas maneras, la mortalidad es elevada. El diagnóstico debe hacerse interviniendo para tomar la muestra y hacer el cultivo y las pruebas de sensibilidad. Se presenta aquí el caso de un paciente con trasplante renal que presentó un absceso cerebral por C. bantiana, el cual se extrajo mediante resección quirúrgica. El paciente recibió tratamiento con voriconazol, con adecuada respuesta, mejoría y sin secuelas neurológicas.


Abstract Cerebral feohifomycosis are severe infections caused by dematiaceous fungi. Cladophialophora bantiana is one of the most commonly isolated species; it has central nervous system tropism and it often manifests as a brain abscess in immunocompetent patients. In immunocompromised patients, it can lead to brain abscesses and disseminated infections. Despite the availability of broad-spectrum antifungal drugs, it is a must to perform surgical management, in addition to drug therapy. However, mortality is high. The diagnostic approach must be invasive to establish a timely diagnosis and direct treatment based on culture and susceptibility tests. We report a case of brain abscess caused by C. bantiana in an immunosuppressed patient who was treated with surgical resection and voriconazole with an adequate response to therapy and without neurological sequels.


Subject(s)
Humans , Male , Middle Aged , Postoperative Complications/microbiology , Brain Abscess/microbiology , Kidney Transplantation , Saccharomycetales/isolation & purification , Cerebral Phaeohyphomycosis/microbiology , Postoperative Complications/surgery , Postoperative Complications/etiology , Postoperative Complications/drug therapy , Recurrence , Hyperoxaluria, Primary/complications , Hyperoxaluria, Primary/diagnosis , Hyperoxaluria, Primary/genetics , Brain Abscess/surgery , Brain Abscess/etiology , Brain Abscess/drug therapy , Amphotericin B/therapeutic use , Renal Dialysis , Immunocompromised Host , Combined Modality Therapy , Craniotomy , Nephrolithiasis/etiology , Cerebral Phaeohyphomycosis/surgery , Cerebral Phaeohyphomycosis/etiology , Cerebral Phaeohyphomycosis/drug therapy , Graft Rejection/drug therapy , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Antifungal Agents/therapeutic use
20.
Iatreia ; 31(3): 300-304, jul.-set. 2018. tab, graf
Article in Spanish | LILACS | ID: biblio-975481

ABSTRACT

RESUMEN El compromiso neurológico del sistema nervioso central (SNC) en las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCAS, del inglés anti-neutrophil cytoplasmic autoantibodies) es raro y potencialmente catastrófico. El estándar de tratamiento ha sido la ciclofosfamida con pulsos de esteroides, sin embargo, este esquema no tiene evidencia fuerte para el compromiso del sistema nervioso central y no está exento de efectos adversos graves sobre todo en la población anciana. En los últimos años, ha aparecido el rituximab como terapia alternativa a la ciclofosfamida para inducir la remisión en este tipo de vasculitis, no obstante, su uso con compromiso neurológico grave también ha sido anecdótico. Se presenta el caso de una paciente de 84 años de edad con poliangeítis microscópica y compromiso neurológico y renal grave, tratada con rituximab evolucionando favorablemente alcanzando la remisión de la enfermedad.


SUMMARY The neurological involvement of the central nervous system (CNS) in vasculitis associated with ANCAS is rare and potentially catastrophic. The standard treatment is cyclophosphamide with pulses of steroids; however, this scheme has no strong evidence for central nervous system involvement and is not free of serious adverse effects especially in the elderly population. In recent year's rituximab has appeared as an alternative therapy to cyclophosphamide to induce remission in this type of vasculitis, however its use with severe neurological involvement has also been anecdotal. We present the case of 84-year-old patient who presented a microscopic polyangiitis with severe neurological and renal involvement, treated with rituximab with a favorable evolution in reaching remission of the disease.


Subject(s)
Humans , Aged, 80 and over , Central Nervous System , Microscopic Polyangiitis
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