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ABSTRACT Background: Urinary tract infection (UTI) is a common disease in childhood and one of the most common causes of consultation in the pediatric emergency department. Its incidence ranges between 5% and 10% in children under 2 years old. Objective: To generate recommendations on the diagnosis, treatment, and follow-up of UTI in pediatric patients in Colombia. Methods: 15 questions of clinical interest in the diagnosis, treatment, and follow-up of UTI in pediatric patients were formulated. A systematic review of the literature was carried out to identify the clinical practice guidelines (CPG) available in UTI, in order to use this evidence to answer the proposed questions and articulate the recommendations. Local and international tools were used to select and evaluate the CPGs. Information was retrieved from the selected guidelines, preliminary recommendations were compiled, and final recommendations were approved by expert consensus. Finally, the strength and direction of each recommendation was assessed. Results: A total of 4 CPGs were selected: American Academy of Pediatrics, Spanish Ministry of Health, McTaggart, and National Institute for Health and Care Excellence. Final recommendations for the diagnosis, treatment, and follow-up of UTI are presented in this paper. Conclusions: These recommendations will guide the teams to make clinical decisions regarding health care of pediatric patients with UTI in Colombia. This will help to improve health care and to generate policies for timely diagnosis, treatment, and follow-up in these patients.
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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.
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HumanosRESUMO
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).
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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.
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Humanos , Adulto , Púrpura Trombocitopênica Trombótica , Transplante de Rim , Escherichia coli Shiga Toxigênica , Síndrome Hemolítico-Urêmica Atípica , Anemia HemolíticaRESUMO
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.
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Humanos , Criança , Púrpura Trombocitopênica Trombótica , Síndrome Hemolítico-Urêmica Atípica/complicações , Anemia Hemolítica , Síndrome Nefrótica/complicações , Proteínas do Sistema ComplementoRESUMO
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.
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Anemia Hemolítica , Síndrome Hemolítico-Urêmica Atípica , Síndrome Nefrótica , Púrpura Trombocitopênica Trombótica , Síndrome Hemolítico-Urêmica Atípica/complicações , Criança , Proteínas do Sistema Complemento , Humanos , Síndrome Nefrótica/complicaçõesRESUMO
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.
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Anemia Hemolítica , Síndrome Hemolítico-Urêmica Atípica , Transplante de Rim , Púrpura Trombocitopênica Trombótica , Escherichia coli Shiga Toxigênica , Adulto , HumanosRESUMO
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.
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Humanos , Tuberculose Urogenital , Falência Renal CrônicaRESUMO
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.
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Humanos , Linfócitos T Citotóxicos , Transplante de Rim , Linfo-Histiocitose HemofagocíticaRESUMO
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.
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Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Vasculite/tratamento farmacológico , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Diálise Peritoneal/métodos , Antagonistas do Ácido Fólico/efeitos adversos , Antagonistas do Ácido Fólico/uso terapêutico , Falência Renal Crônica/terapia , Pancitopenia/etiologia , Pancitopenia/terapia , Choque Séptico/etiologia , Choque Séptico/tratamento farmacológico , Metotrexato/sangue , Resultado do Tratamento , Mucosite/etiologia , Mucosite/tratamento farmacológico , Antagonistas do Ácido Fólico/sangue , Antibacterianos/uso terapêuticoRESUMO
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.
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Antagonistas do Ácido Fólico/efeitos adversos , Antagonistas do Ácido Fólico/uso terapêutico , Falência Renal Crônica/terapia , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Diálise Peritoneal/métodos , Vasculite/tratamento farmacológico , Adulto , Idoso , Antibacterianos/uso terapêutico , Feminino , Antagonistas do Ácido Fólico/sangue , Humanos , Masculino , Metotrexato/sangue , Pessoa de Meia-Idade , Mucosite/tratamento farmacológico , Mucosite/etiologia , Pancitopenia/etiologia , Pancitopenia/terapia , Choque Séptico/tratamento farmacológico , Choque Séptico/etiologia , Resultado do TratamentoRESUMO
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.
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Humanos , Idoso de 80 Anos ou mais , Sistema Nervoso Central , Poliangiite MicroscópicaRESUMO
Dengue infection has been associated with multiple renal complications, including glomerulonephritis, acute tubular necrosis, tubulointerstitial nephritis, and thrombotic microangiopathy (TMA), this last one being a rare complication of dengue, with only a few reported cases. TMA associated with dengue can be explained by an alteration in the activity of the enzyme ADAMTS13, leading to thrombotic thrombocytopenic purpura; or it can be secondary to direct or indirect endothelial injury by the virus, which leads to hemolytic uremic syndrome. Here, we present a case of severe TMA, not related to ADAMTS13, which was clearly associated with dengue infection.
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Dengue/complicações , Troca Plasmática/métodos , Microangiopatias Trombóticas/terapia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/terapia , Injúria Renal Aguda/virologia , Adolescente , Adulto , Criança , Dengue/sangue , Feminino , Síndrome Hemolítico-Urêmica/etiologia , Síndrome Hemolítico-Urêmica/terapia , Síndrome Hemolítico-Urêmica/virologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Abstract Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.
Resumo A hiperoxalúria primária (HP) é um distúrbio genético muito raro, caracterizado por deficiência total ou parcial das enzimas relacionadas ao metabolismo do glioxilato, superprodução de oxalato de cálcio que se deposita em vários órgãos (principalmente os rins) resultando em litíase recorrente, nefrocalcinose e doença renal terminal (DRT). Nos pacientes com DRT que recebem transplante renal, a doença apresenta recidiva em 100% dos casos, com perda do enxerto nos primeiros cinco anos após o transplante num elevado percentual de pacientes. Três distúrbios moleculares foram descritos na HP: mutação dos genes da alanina-glioxilato aminotransferase (AGXT), glioxilato redutase/hidroxipiruvato redutase (GRHPR) e 4-OH-2-oxoglutarato aldolase (HOGA1). Apresentamos dois casos de pacientes com histórico de litíase renal diagnosticados com hiperoxalúria primária no período pós-transplante, manifestada na forma de perda precoce do enxerto com evidências de cristais de oxalato de cálcio na biópsia renal, hiperoxalúria, hiperoxalemia e testes genéticos compatíveis. Os pacientes foram tratados com abordagem nutricional, líquidos orais em abundância, piridoxina, hidroclorotiazida e citrato de potássio. Contudo, os pacientes apresentaram deterioração lenta e gradual da função do enxerto e evoluíram para doença renal terminal.
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Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico , Hiperoxalúria Primária/diagnóstico , Transplante de RimRESUMO
Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.