RESUMO
MYH9 disease is a rare genetic disorder in which there is a mutation in the gene for the non-muscle myosin heavy chain IIA. It initially causes macrothrombocytopenia followed by other clinical manifestations. When the patient reaches adulthood, he can develop chronic kidney failure. Thus, the risk of suffering a hemorrhage, difficulty in repairing and, infections increases in individuals with this disease. In addition, the use of drugs in these patients should be carefully evaluated. An adult patient sought dental care with a complaint associated with a tooth with advanced dental caries. He had severe thrombocytopenia (7000 platelets/mm3 ), hearing loss, and chronic kidney failure. The diagnosis of MYH9 disease was confirmed through genotyping. After clinical examination, extraction was planned. Local and systemic procedures were used to prevent hemorrhage, especially postoperatively. Although the patient had an infection at the surgical wound site and no episode of postoperative bleeding, the repair process occurred normally. The purpose of this article is to report the surgical management of a patient with MYH9 disease.
Assuntos
Cárie Dentária , Perda Auditiva Neurossensorial , Falência Renal Crônica , Trombocitopenia , Adulto , Masculino , Humanos , Cadeias Pesadas de Miosina/genética , Proteínas Motores Moleculares/genética , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/complicações , Trombocitopenia/complicações , Trombocitopenia/genética , Mutação , Falência Renal Crônica/complicaçõesAssuntos
Doenças Ósseas Metabólicas , Doenças Ósseas , Distúrbio Mineral e Ósseo na Doença Renal Crônica , Insuficiência Renal Crônica , Biópsia , Doenças Ósseas/diagnóstico por imagem , Doenças Ósseas/etiologia , Doenças Ósseas Metabólicas/diagnóstico por imagem , Doenças Ósseas Metabólicas/etiologia , Osso e Ossos , Distúrbio Mineral e Ósseo na Doença Renal Crônica/complicações , Humanos , Insuficiência Renal Crônica/complicaçõesRESUMO
Abstract Introduction: Chronic kidney disease - mineral and bone disorders (CKD-MBD) are common in dialysis patients. Definition of targets for calcium (Ca), phosphorus (P), parathormone (iPTH), and alkaline phosphatase (ALP) and their treatment recommendations, are provided by international guidelines. There are few studies analyzing CKD-MBD in peritoneal dialysis (PD) patients and the impact of guidelines on mineral metabolism control. The aim of our study was to describe the prevalence of biomarkers for CKD-MBD in a large cohort of PD patients in Brazil. Methods: Data from the nation-wide prospective observational cohort BRAZPD II was used. Incident patients were followed between December 2004 and January 2011. According to KDOQI recommendations, reference ranges for total Ca were 8.4 to 9.5 mg/dL, for P, 3.5 to 5.5 mg/dL, for iPTH, 150-300 pg/mL, and for ALP, 120 U/L. Results: Mean age was 59.8 ± 16 years, 48% were male, and 43% had diabetes. In the beginning, Ca was 8.9 ± 0.9 mg/dL, and 48.3% were on the KODQI target. After 1 year, Ca increased to 9.1 ± 0.9 mg/dL and 50.4% were in the KDOQI preferred range. P at baseline was 5.2 ± 1.6 mg/dL, with 52.8% on target, declining to 4.9 ± 1.5 mg/dL after one year, when 54.7% were on target. Median iPTH at baseline was 238 (P25% 110 - P75% 426 pg/mL) and it remained stable throughout the first year; patients within target ranged from 26 to 28.5%. At the end of the study, 80% was in 3.5 meq/L Ca dialysate concentration, 66.9% of patients was taking any phosphate binder, and 25% was taking activated vitamin D. Conclusions: We observed a significant prevalence of biochemical disorders related to CKD-MBD in this dialysis population.
Resumo Introdução: Os distúrbios minerais e ósseos da doença renal crônica (DMO-DRC) são comuns em pacientes em diálise. A definição de metas para cálcio (Ca), fósforo (P), paratormônio (PTHi) e fosfatase alcalina (FA) e suas recomendações de tratamento são fornecidas por diretrizes internacionais. Há poucos estudos analisando o DMO-DRC em pacientes em diálise peritoneal (DP) e o impacto das diretrizes no controle do metabolismo mineral. O objetivo do nosso estudo foi descrever a prevalência de alterações nos marcadores para DMO-DRC em uma grande coorte de pacientes em DP no Brasil. Métodos: Foram utilizados dados da coorte observacional prospectiva nacional BRAZPD II. Pacientes incidentes foram acompanhados entre Dezembro de 2004 e Janeiro de 2011. De acordo com as recomendações do KDOQI, os intervalos de referência para Ca total foram de 8,4 a 9,5 mg/dL, para P, 3,5 a 5,5 mg/dL, para PTHi, 150-300 pg/mL, e para FA, 120 U/L. Resultados: A idade média foi de 59,8 ± 16 anos, 48% eram homens e 43% tinham diabetes. No início, o Ca era de 8,9 ± 0,9 mg/dL, e 48,3% estavam na meta do KODQI. Após 1 ano, o Ca aumentou para 9,1 ± 0,9 mg/dL e 50,4% estavam na faixa preferida do KDOQI. P basal era 5,2 ± 1,6 mg/dL, com 52,8% na meta, diminuindo para 4,9 ± 1,5 mg/dL após um ano, quando 54,7% estavam na meta. O PTHi basal mediano foi de 238 (P25% 110 - P75% 426 pg/mL) e permaneceu estável durante o primeiro ano; os pacientes dentro da meta variaram de 26 a 28,5%. No final do estudo, 80% estavam na concentração de 3,5 meq/L de Ca dialisato, 66,9% dos pacientes estavam tomando qualquer quelante de fosfato, e 25% estavam tomando vitamina D ativada. Conclusões: Observamos uma prevalência significativa de distúrbios bioquímicos relacionados ao DMO-DRC nesta população em diálise.
Assuntos
Humanos , Masculino , Feminino , Adulto , Idoso , Distúrbio Mineral e Ósseo na Doença Renal Crônica/etiologia , Distúrbio Mineral e Ósseo na Doença Renal Crônica/epidemiologia , Diálise Peritoneal , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/terapia , Hormônio Paratireóideo , Cálcio , Prevalência , Diálise Renal , Objetivos , Pessoa de Meia-Idade , MineraisRESUMO
INTRODUCTION: Chronic kidney disease - mineral and bone disorders (CKD-MBD) are common in dialysis patients. Definition of targets for calcium (Ca), phosphorus (P), parathormone (iPTH), and alkaline phosphatase (ALP) and their treatment recommendations, are provided by international guidelines. There are few studies analyzing CKD-MBD in peritoneal dialysis (PD) patients and the impact of guidelines on mineral metabolism control. The aim of our study was to describe the prevalence of biomarkers for CKD-MBD in a large cohort of PD patients in Brazil. METHODS: Data from the nation-wide prospective observational cohort BRAZPD II was used. Incident patients were followed between December 2004 and January 2011. According to KDOQI recommendations, reference ranges for total Ca were 8.4 to 9.5 mg/dL, for P, 3.5 to 5.5 mg/dL, for iPTH, 150-300 pg/mL, and for ALP, 120 U/L. RESULTS: Mean age was 59.8 ± 16 years, 48% were male, and 43% had diabetes. In the beginning, Ca was 8.9 ± 0.9 mg/dL, and 48.3% were on the KODQI target. After 1 year, Ca increased to 9.1 ± 0.9 mg/dL and 50.4% were in the KDOQI preferred range. P at baseline was 5.2 ± 1.6 mg/dL, with 52.8% on target, declining to 4.9 ± 1.5 mg/dL after one year, when 54.7% were on target. Median iPTH at baseline was 238 (P25% 110 - P75% 426 pg/mL) and it remained stable throughout the first year; patients within target ranged from 26 to 28.5%. At the end of the study, 80% was in 3.5 meq/L Ca dialysate concentration, 66.9% of patients was taking any phosphate binder, and 25% was taking activated vitamin D. CONCLUSIONS: We observed a significant prevalence of biochemical disorders related to CKD-MBD in this dialysis population.
Assuntos
Distúrbio Mineral e Ósseo na Doença Renal Crônica , Diálise Peritoneal , Insuficiência Renal Crônica , Adulto , Idoso , Cálcio , Distúrbio Mineral e Ósseo na Doença Renal Crônica/epidemiologia , Distúrbio Mineral e Ósseo na Doença Renal Crônica/etiologia , Feminino , Objetivos , Humanos , Masculino , Pessoa de Meia-Idade , Minerais , Hormônio Paratireóideo , Prevalência , Diálise Renal , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/terapiaRESUMO
OBJECTIVES: This study aimed to evaluate the potential anti-inflammatory effects of vitamin D supplementation under uremic conditions, both in vivo and in vitro, and its effects on the parameters of mineral metabolism. METHODS: Thirty-two hemodialysis patients were randomly assigned to receive placebo (N=14) or cholecalciferol (N=18) for six months. Serum levels of calcium, phosphate, total alkaline phosphatase, intact parathyroid hormone (iPTH), and vitamin D were measured at baseline and after three and six months. The levels of fibroblast growth factor-23 (FGF-23), interleukin-1ß (IL-1ß), and high-sensitivity C-reactive protein (hs-CRP) were also measured at baseline and at six months. Human monocytes were used for in vitro experiments and treated with cholecalciferol (150 nM) and uremic serum. Cell viability, reactive oxygen species (ROS) production, and cathelicidin (CAMP) expression were evaluated using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay, dichloro-dihydro-fluorescein diacetate assay, and real time-quantitative polymerase chain reaction, respectively. RESULTS: Both patient groups were clinically and biochemically similar at baseline. After six months, the levels of vitamin D and iPTH were higher and lower, respectively, in the cholecalciferol group than in the placebo group (p<0.05). There was no significant difference between the parameters of mineral metabolism, such as IL-1ß and hs-CRP levels, in both groups. Treatment with uremic serum lowered the monocyte viability (p<0.0001) and increased ROS production (p<0.01) and CAMP expression (p<0.05); these effects were counterbalanced by cholecalciferol treatment (p<0.05). CONCLUSIONS: Thus, cholecalciferol supplementation is an efficient strategy to ameliorate hypovitaminosis D in hemodialysis patients, but its beneficial effects on the control of secondary hyperparathyroidism are relatively unclear. Even though cholecalciferol exhibited anti-inflammatory effects in vitro, its short-term supplementation was not effective in improving the inflammatory profile of patients on hemodialysis, as indicated by the IL-1ß and hs-CRP levels.
Assuntos
Colecalciferol , Deficiência de Vitamina D , Anti-Inflamatórios/uso terapêutico , Colecalciferol/uso terapêutico , Suplementos Nutricionais , Fator de Crescimento de Fibroblastos 23 , Humanos , Hormônio Paratireóideo/uso terapêutico , Diálise Renal , Vitamina DAssuntos
Humanos , Distúrbio Mineral e Ósseo na Doença Renal Crônica/complicações , Doenças Ósseas/etiologia , Doenças Ósseas/diagnóstico por imagem , Doenças Ósseas Metabólicas/etiologia , Doenças Ósseas Metabólicas/diagnóstico por imagem , Insuficiência Renal Crônica/complicações , Biópsia , Osso e OssosRESUMO
OBJECTIVES: This study aimed to evaluate the potential anti-inflammatory effects of vitamin D supplementation under uremic conditions, both in vivo and in vitro, and its effects on the parameters of mineral metabolism. METHODS: Thirty-two hemodialysis patients were randomly assigned to receive placebo (N=14) or cholecalciferol (N=18) for six months. Serum levels of calcium, phosphate, total alkaline phosphatase, intact parathyroid hormone (iPTH), and vitamin D were measured at baseline and after three and six months. The levels of fibroblast growth factor-23 (FGF-23), interleukin-1β (IL-1β), and high-sensitivity C-reactive protein (hs-CRP) were also measured at baseline and at six months. Human monocytes were used for in vitro experiments and treated with cholecalciferol (150 nM) and uremic serum. Cell viability, reactive oxygen species (ROS) production, and cathelicidin (CAMP) expression were evaluated using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay, dichloro-dihydro-fluorescein diacetate assay, and real time-quantitative polymerase chain reaction, respectively. RESULTS: Both patient groups were clinically and biochemically similar at baseline. After six months, the levels of vitamin D and iPTH were higher and lower, respectively, in the cholecalciferol group than in the placebo group (p<0.05). There was no significant difference between the parameters of mineral metabolism, such as IL-1β and hs-CRP levels, in both groups. Treatment with uremic serum lowered the monocyte viability (p<0.0001) and increased ROS production (p<0.01) and CAMP expression (p<0.05); these effects were counterbalanced by cholecalciferol treatment (p<0.05). CONCLUSIONS: Thus, cholecalciferol supplementation is an efficient strategy to ameliorate hypovitaminosis D in hemodialysis patients, but its beneficial effects on the control of secondary hyperparathyroidism are relatively unclear. Even though cholecalciferol exhibited anti-inflammatory effects in vitro, its short-term supplementation was not effective in improving the inflammatory profile of patients on hemodialysis, as indicated by the IL-1β and hs-CRP levels.
Assuntos
Humanos , Deficiência de Vitamina D , Colecalciferol/uso terapêutico , Hormônio Paratireóideo/uso terapêutico , Vitamina D , Diálise Renal , Suplementos Nutricionais , Anti-InflamatóriosRESUMO
Considering the new coronavirus epidemic (Covid-19), the Brazilian Society of Nephrology, represented by the Peritoneal Steering Committee, in agreement with the and the Dialysis Department, developed a series of recommendations for good clinical practices for peritoneal dialysis (PD) clinics, to be considered during the period of the Covid-19 epidemic. We aim to minimize the disease spread, protecting patients and staff, and ensuring the quality of the treatment provided and adequate follow-up for PD patients. The recommendations suggested at this moment must be adapted to each clinic's reality and the conditions of the structural and human resources, dependent on the adequate financial provision of the public health system for its full implementation.
Assuntos
Betacoronavirus , Infecções por Coronavirus/prevenção & controle , Falência Renal Crônica/terapia , Pandemias/prevenção & controle , Diálise Peritoneal/normas , Pneumonia Viral/prevenção & controle , Brasil , COVID-19 , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Desinfecção/métodos , Desinfecção/normas , Humanos , Falência Renal Crônica/complicações , Máscaras , Nefrologia/normas , Doenças Profissionais/prevenção & controle , Equipe de Assistência ao Paciente , Educação de Pacientes como Assunto , Isolamento de Pacientes/métodos , Isolamento de Pacientes/normas , Diálise Peritoneal/instrumentação , Diálise Peritoneal/métodos , Equipamento de Proteção Individual , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , SARS-CoV-2 , Sociedades Médicas , Telemedicina/legislação & jurisprudência , Telemedicina/métodos , Telemedicina/normas , Unidade Hospitalar de Urologia/organização & administração , Unidade Hospitalar de Urologia/normasRESUMO
ABSTRACT Considering the new coronavirus epidemic (Covid-19), the Brazilian Society of Nephrology, represented by the Peritoneal Steering Committee, in agreement with the and the Dialysis Department, developed a series of recommendations for good clinical practices for peritoneal dialysis (PD) clinics, to be considered during the period of the Covid-19 epidemic. We aim to minimize the disease spread, protecting patients and staff, and ensuring the quality of the treatment provided and adequate follow-up for PD patients. The recommendations suggested at this moment must be adapted to each clinic's reality and the conditions of the structural and human resources, dependent on the adequate financial provision of the public health system for its full implementation.
RESUMO Considerando a nova epidemia de coronavírus (Covid-19), a Sociedade Brasileira de Nefrologia, representada pelo Comitê de Diálise Peritoneal, em concordância com a diretoria e o Departamento de Diálise, desenvolveu uma série de recomendações de boas práticas clínicas para os serviços de diálise peritoneal a serem consideradas durante o período da epidemia de Covid-19, com o objetivo de minimizar a disseminação da doença, proteger pacientes e funcionários e garantir a qualidade do tratamento prestado e acompanhamento adequado para os pacientes em DP. As recomendações aqui sugeridas devem ser adaptadas a cada realidade de serviço e às condições estruturais e de recursos humanos e dependem da provisão financeira adequada do sistema público de saúde para sua plena implementação.
Assuntos
Humanos , Pneumonia Viral/prevenção & controle , Diálise Peritoneal/normas , Infecções por Coronavirus/prevenção & controle , Pandemias/prevenção & controle , Betacoronavirus , Falência Renal Crônica/terapia , Brasil , Desinfecção/métodos , Unidade Hospitalar de Urologia/normas , Telemedicina/normas , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Equipamento de Proteção Individual , SARS-CoV-2 , COVID-19RESUMO
Abstract Introduction: Treating secondary hyperparathyroidism (SHPT), a common condition associated with death in patients with chronic kidney disease, is a challenge for nephrologists. Calcimimetics have allowed the introduction of drug therapies no longer based on phosphate binders and active vitamin D. This study aimed to assess the safety and effectiveness of cinacalcet in managing chronic dialysis patients with severe SHPT. Methods: This retrospective study included 26 patients [age: 52 ± 12 years; 55% females; time on dialysis: 54 (4-236) months] on hemodialysis (N = 18) or peritoneal dialysis (N = 8) with severe SHPT (intact parathyroid hormone (iPTH) level > 600 pg/mL) and hyperphosphatemia and/or persistent hypercalcemia treated with cinacalcet. The patients were followed for 12 months. Their serum calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), and iPTH levels were measured at baseline and on days 30, 60, 90, 180, and 365. Results: Patients with hyperphosphatemia (57.7%), hypercalcemia (23%), or both (19.3%) with iPTH > 600 pg/mL were prescribed cinacalcet. At the end of the study, decreases were observed in iPTH (1348 ± 422 vs. 440 ± 210 pg/mL; p < 0.001), Ca (9.5 ± 1.0 vs. 9.1 ± 0.6 mg/dl; p = 0.004), P (6.0 ± 1.3 vs. 4.9 ± 1.1 mg/dl; p < 0.001), and ALP (202 ± 135 vs. 155 ± 109 IU/L; p = 0.006) levels. Adverse events included hypocalcemia (26%) and digestive problems (23%). At the end of the study, 73% of the patients were on active vitamin D and cinacalcet. Three (11.5%) patients on peritoneal dialysis did not respond to therapy with cinacalcet, and their iPTH levels were never below 800 pg/mL. Conclusion: Cinacalcet combined with traditional therapy proved safe and effective and helped manage the mineral metabolism of patients with severe SHPT.
Resumo Introdução: O tratamento do hiperparatireoidismo secundário (HPTs), patologia comum e associada à mortalidade na doença renal crônica, é um desafio para o nefrologista. Advento dos calcimiméticos propiciou terapêutica medicamentosa diferente da usual, baseada em quelantes de fósforo e vitamina D ativa. O objetivo deste estudo foi avaliar segurança e efetividade de cinacalcete no controle do HPTs grave de pacientes em diálise crônica. Métodos: Estudo retrospectivo 26 pacientes [idade: 52 ± 12 anos; 55% mulheres; tempo em diálise: 54 (4-236) meses], em hemodiálise (N = 18) ou diálise peritoneal (N = 8), com HPTs grave (nível de paratormônio intacto (PTHi) > 600 pg/mL), com hiperfosfatemia e/ou hipercalcemia persistentes, em tratamento com cinacalcete. Período de seguimento de 12 meses. Avaliados níveis séricos de cálcio (Ca), fósforo (P), fosfatase alcalina (FA) e PTHi no início do seguimento, 30, 60, 90, 180 e 365 dias. Resultados: Indicações para início do cinacalcete: hiperfosfatemia (57,7%), hipercalcemia (23%), ou ambos (19,3%) com PTH > 600 pg/mL. Ao final do seguimento, observada redução dos níveis PTHi (1348 ± 422 vs. 440 ± 210 pg/mL; p < 0,001), Ca (9,5 ± 1,0 vs. 9,1 ± 0,6 mg/dl; p = 0,004), P (6,0 ± 1,3 vs. 4,9 ± 1,1 mg/dl; p < 0,001) e FA (202 ± 135 vs. 155 ± 109 UI/L; p = 0,006). Eventos adversos: hipocalcemia (26%) e queixas digestivas (23%). No fim do estudo, 73% pacientes utilizavam vitamina D ativada associada ao cinacalcete. Três (11,5%) pacientes, todos em DP, não responderam ao cinacalcete, mantendo níveis PTHi > 800 pg/mL. Conclusão: Utilização de cinacalcete, associado à terapia tradicional, em pacientes com HPTs grave foi segura, eficiente e associada a melhor controle do metabolismo mineral.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Diálise Renal , Calcimiméticos/uso terapêutico , Cinacalcete/uso terapêutico , Hiperparatireoidismo Secundário/tratamento farmacológico , Hiperparatireoidismo Secundário/sangue , Hormônio Paratireóideo/sangue , Fósforo/sangue , Vitamina D/uso terapêutico , Cálcio/sangue , Estudos Retrospectivos , Seguimentos , Resultado do Tratamento , Fosfatase Alcalina/sangue , Hiperfosfatemia/tratamento farmacológico , Calcimiméticos/efeitos adversos , Cinacalcete/efeitos adversos , Hipercalcemia/tratamento farmacológico , Hipocalcemia/etiologia , Falência Renal Crônica/terapiaRESUMO
Abstract Hypertension (blood pressure > 140/90 mm Hg) is very common in patients undergoing regular dialysis, with a prevalence of 70-80%, and only the minority has adequate blood pressure (BP) control. In contrast to the unclear association of predialytic BP recordings with cardiovascular mortality, prospective studies showed that interdialytic BP, recorded as home BP or by ambulatory blood pressure monitoring in hemodialysis patients, associates more closely with mortality and cardiovascular events. Although BP is measured frequently in the dialysis treatment environment, aspects related to the measurement technique traditionally employed may be unsatisfactory. Several other tools are now available and being used in clinical trials and in clinical practice to evaluate and treat elevated BP in chronic kidney disease (CKD) patients. While we wait for the ongoing review of the CKD Blood Pressure KIDGO guidelines, there is no guideline for the dialysis population addressing this important issue. Thus, the objective of this review is to provide a critical analysis of the information available on the epidemiology, pathogenic mechanisms, and the main pillars involved in the management of blood pressure in stage 5-D CKD, based on current knowledge.
Resumo A hipertensão (pressão arterial > 140/90 mmHg) é muito comum em pacientes submetidos à diálise regular, com uma prevalência de 70-80%, e apenas a minoria tem controle adequado da pressão arterial (PA). Em contraste com a associação incerta entre de PA pré-dialítica com mortalidade cardiovascular, estudos prospectivos mostraram que a PA interdialítica, registrada como PA domiciliar ou pela monitorização ambulatorial da pressão arterial em pacientes em hemodiálise, está mais relacionada à mortalidade e eventos cardiovasculares. Embora a PA seja medida com frequência no ambiente de tratamento de diálise, aspectos relacionados à técnica de medição tradicionalmente empregada podem ser insatisfatórios. Várias outras ferramentas estão agora disponíveis, e estão sendo usadas em ensaios clínicos e na prática clínica para avaliar e tratar a PA elevada em pacientes com doença renal crônica (DRC). Enquanto esperamos pela revisão das diretrizes do KIDGO para a pressão sanguíneana DRC, não há nenhuma diretriz para a população em diálise abordando essa importante questão. Assim, o objetivo desta revisão é fornecer uma análise crítica das informações disponíveis sobre a epidemiologia, os mecanismos patogênicos e os principais pilares sustentadores do manejo da pressão arterial no estágio 5-D da DRC, com base no conhecimento atual.
Assuntos
Humanos , Diálise Peritoneal , Hipertensão/terapia , Hipertensão/epidemiologia , Prevalência , Fatores de Risco , Monitorização Ambulatorial da Pressão Arterial , Dieta Hipossódica , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/terapia , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Anti-Hipertensivos/uso terapêuticoRESUMO
INTRODUCTION: Treating secondary hyperparathyroidism (SHPT), a common condition associated with death in patients with chronic kidney disease, is a challenge for nephrologists. Calcimimetics have allowed the introduction of drug therapies no longer based on phosphate binders and active vitamin D. This study aimed to assess the safety and effectiveness of cinacalcet in managing chronic dialysis patients with severe SHPT. METHODS: This retrospective study included 26 patients [age: 52 ± 12 years; 55% females; time on dialysis: 54 (4-236) months] on hemodialysis (N = 18) or peritoneal dialysis (N = 8) with severe SHPT (intact parathyroid hormone (iPTH) level > 600 pg/mL) and hyperphosphatemia and/or persistent hypercalcemia treated with cinacalcet. The patients were followed for 12 months. Their serum calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), and iPTH levels were measured at baseline and on days 30, 60, 90, 180, and 365. RESULTS: Patients with hyperphosphatemia (57.7%), hypercalcemia (23%), or both (19.3%) with iPTH > 600 pg/mL were prescribed cinacalcet. At the end of the study, decreases were observed in iPTH (1348 ± 422 vs. 440 ± 210 pg/mL; p < 0.001), Ca (9.5 ± 1.0 vs. 9.1 ± 0.6 mg/dl; p = 0.004), P (6.0 ± 1.3 vs. 4.9 ± 1.1 mg/dl; p < 0.001), and ALP (202 ± 135 vs. 155 ± 109 IU/L; p = 0.006) levels. Adverse events included hypocalcemia (26%) and digestive problems (23%). At the end of the study, 73% of the patients were on active vitamin D and cinacalcet. Three (11.5%) patients on peritoneal dialysis did not respond to therapy with cinacalcet, and their iPTH levels were never below 800 pg/mL. CONCLUSION: Cinacalcet combined with traditional therapy proved safe and effective and helped manage the mineral metabolism of patients with severe SHPT.
Assuntos
Calcimiméticos/uso terapêutico , Cinacalcete/uso terapêutico , Hiperparatireoidismo Secundário/sangue , Hiperparatireoidismo Secundário/tratamento farmacológico , Diálise Renal , Adulto , Idoso , Fosfatase Alcalina/sangue , Calcimiméticos/efeitos adversos , Cálcio/sangue , Cinacalcete/efeitos adversos , Feminino , Seguimentos , Humanos , Hipercalcemia/tratamento farmacológico , Hiperfosfatemia/tratamento farmacológico , Hipocalcemia/etiologia , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Hormônio Paratireóideo/sangue , Fósforo/sangue , Estudos Retrospectivos , Resultado do Tratamento , Vitamina D/uso terapêuticoRESUMO
ABSTRACT Introduction: Anemia is a frequent multifactorial complication of CKD seen in patients on dialysis derived mainly from impaired erythropoietin (EPO) production. A less common cause of anemia in individuals with CKD is pure red cell aplasia (PRCA) secondary to the production of anti-EPO antibodies. Objective: This paper aimed two describe two cases of PRCA secondary to the production of anti-EPO antibodies including choice of treatment, patient progression, and a literature review. Materials: This study included the cases of two patients with CKD on hemodialysis with severe anemia in need of specific investigation and management. Results: Patient 1 with CKD secondary to hypertension treated with EPO for 7 months showed persistent decreases in hemoglobin (Hb) levels despite the subcutaneous administration of increasing doses of EPO; the patient required recurring blood transfusions. Workup and imaging tests were negative for the main causes of anemia in individuals with CKD on dialysis. Patient 2 with CKD secondary to adult polycystic kidney disease had been taking EPO for 2 years. The patient developed severe abrupt anemia the month he was started on HD, and required recurring transfusions to treat the symptoms of anemia. Workup and imaging findings were inconclusive. Specific laboratory tests confirmed the patients had anti-EPO antibodies. After six months of immunosuppressant therapy (corticosteroids + cyclosporine) the patients were stable with Hb > 9.0 g/dl. Conclusion: PRCA is a rare condition among patients on dialysis treated with rhEPO and should be considered as a possible cause of refractory anemia. Treating patients with PRCA may be challenging, since the specific management and diagnostic procedures needed in this condition are not always readily available.
RESUMO Introdução: Anemia é complicação frequente da Doença Renal Crônica (DRC) em pacientes dialíticos. Apresenta caráter multifatorial principalmente pela insuficiente produção de eritropoietina (EPO). Situação rara causadora de anemia na DRC é Aplasia Pura de Células Vermelhas (APCV), em decorrência da produção de anticorpos anti-EPO. Objetivo: Descrever 2 casos de APCV com formação de anticorpos anti-EPO, sua abordagem clínica, evolução e revisão de literatura. Métodos: Dois pacientes em hemodiálise que desenvolveram anemia grave, necessitando investigação e manejo específico. Resultados: Paciente nº 1: feminina, 75 anos, DRC secundária à hipertensão arterial. Após 7 meses com EPO desenvolveu queda persistente em valores de hemoglobina (Hb) mesmo com incremento em doses EPO SC, necessitando transfusões de sangue recorrentes. Extensa investigação laboratorial e de imagem resultou negativa para principais causas de anemia. Paciente nº 2: masculino, 66 anos, DRC secundária à DRPA, há 2 anos em uso de EPO. No mês de entrada em HD desenvolveu anemia severa, também exigindo transfusões recorrentes para tratamento da anemia sintomática. Extensa investigação laboratorial e por imagem, sem chegar a uma conclusão definitiva. Em ambos os casos a presença de anticorpos anti-EPO foi confirmada por exames laboratoriais específicos. Terapia imunossupressora resultou em estabilização do quadro e Hb > 9,0 g/dl em ambos os pacientes, 6 meses após início do tratamento. Conclusão: APCV é condição rara entre pacientes dialíticos que recebem EPOHuR e deve ser lembrada como causa de anemia refratária. Seu manejo específico e diagnóstico laboratorial nem sempre acessível, tornando desafiadora a condução dos casos para o nefrologista.
Assuntos
Humanos , Masculino , Feminino , Idoso , Proteínas Recombinantes/uso terapêutico , Eritropoetina/imunologia , Eritropoetina/uso terapêutico , Diálise Renal/efeitos adversos , Aplasia Pura de Série Vermelha/etiologia , Anticorpos Neutralizantes/sangue , Falência Renal Crônica/tratamento farmacológico , Proteínas Recombinantes/efeitos adversos , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Eritropoetina/biossíntese , Eritropoetina/efeitos adversos , Transplante de Rim , Resultado do Tratamento , Ciclosporina/administração & dosagem , Ciclosporina/uso terapêutico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/uso terapêuticoRESUMO
INTRODUCTION: Anemia is a frequent multifactorial complication of CKD seen in patients on dialysis derived mainly from impaired erythropoietin (EPO) production. A less common cause of anemia in individuals with CKD is pure red cell aplasia (PRCA) secondary to the production of anti-EPO antibodies. OBJECTIVE: This paper aimed two describe two cases of PRCA secondary to the production of anti-EPO antibodies including choice of treatment, patient progression, and a literature review. MATERIALS: This study included the cases of two patients with CKD on hemodialysis with severe anemia in need of specific investigation and management. RESULTS: Patient 1 with CKD secondary to hypertension treated with EPO for 7 months showed persistent decreases in hemoglobin (Hb) levels despite the subcutaneous administration of increasing doses of EPO; the patient required recurring blood transfusions. Workup and imaging tests were negative for the main causes of anemia in individuals with CKD on dialysis. Patient 2 with CKD secondary to adult polycystic kidney disease had been taking EPO for 2 years. The patient developed severe abrupt anemia the month he was started on HD, and required recurring transfusions to treat the symptoms of anemia. Workup and imaging findings were inconclusive. Specific laboratory tests confirmed the patients had anti-EPO antibodies. After six months of immunosuppressant therapy (corticosteroids + cyclosporine) the patients were stable with Hb > 9.0 g/dl. CONCLUSION: PRCA is a rare condition among patients on dialysis treated with rhEPO and should be considered as a possible cause of refractory anemia. Treating patients with PRCA may be challenging, since the specific management and diagnostic procedures needed in this condition are not always readily available.
Assuntos
Anticorpos Neutralizantes/sangue , Eritropoetina/imunologia , Eritropoetina/uso terapêutico , Falência Renal Crônica/tratamento farmacológico , Proteínas Recombinantes/uso terapêutico , Aplasia Pura de Série Vermelha/etiologia , Diálise Renal/efeitos adversos , Idoso , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/uso terapêutico , Ciclosporina/administração & dosagem , Ciclosporina/uso terapêutico , Eritropoetina/efeitos adversos , Eritropoetina/síntese química , Feminino , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Transplante de Rim , Masculino , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Proteínas Recombinantes/efeitos adversos , Aplasia Pura de Série Vermelha/tratamento farmacológico , Resultado do TratamentoRESUMO
Hypertension (blood pressure > 140/90 mm Hg) is very common in patients undergoing regular dialysis, with a prevalence of 70-80%, and only the minority has adequate blood pressure (BP) control. In contrast to the unclear association of predialytic BP recordings with cardiovascular mortality, prospective studies showed that interdialytic BP, recorded as home BP or by ambulatory blood pressure monitoring in hemodialysis patients, associates more closely with mortality and cardiovascular events. Although BP is measured frequently in the dialysis treatment environment, aspects related to the measurement technique traditionally employed may be unsatisfactory. Several other tools are now available and being used in clinical trials and in clinical practice to evaluate and treat elevated BP in chronic kidney disease (CKD) patients. While we wait for the ongoing review of the CKD Blood Pressure KIDGO guidelines, there is no guideline for the dialysis population addressing this important issue. Thus, the objective of this review is to provide a critical analysis of the information available on the epidemiology, pathogenic mechanisms, and the main pillars involved in the management of blood pressure in stage 5-D CKD, based on current knowledge.