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RATIONALE & OBJECTIVE: Hemodialysis (HD) is the most common form of kidney replacement therapy. This study aimed to examine the use, availability, accessibility, affordability, and quality of HD care worldwide. STUDY DESIGN: A cross-sectional survey. SETTING & PARTICIPANTS: Stakeholders (clinicians, policy makers, and consumer representatives) in 182 countries were convened by the International Society of Nephrology from July to September 2018. OUTCOMES: Use, availability, accessibility, affordability, and quality of HD care. ANALYTICAL APPROACH: Descriptive statistics. RESULTS: Overall, representatives from 160 (88%) countries participated. Median country-specific use of maintenance HD was 298.4 (IQR, 80.5-599.4) per million population (pmp). Global median HD use among incident patients with kidney failure was 98.0 (IQR, 81.5-140.8) pmp and median number of HD centers was 4.5 (IQR, 1.2-9.9) pmp. Adequate HD services (3-4 hours 3 times weekly) were generally available in 27% of low-income countries. Home HD was generally available in 36% of high-income countries. 32% of countries performed monitoring of patient-reported outcomes; 61%, monitoring of small-solute clearance; 60%, monitoring of bone mineral markers; 51%, monitoring of technique survival; and 60%, monitoring of patient survival. At initiation of maintenance dialysis, only 5% of countries used an arteriovenous access in almost all patients. Vascular access education was suboptimal, funding for vascular access procedures was not uniform, and copayments were greater in countries with lower levels of income. Patients in 23% of the low-income countries had to pay >75% of HD costs compared with patients in only 4% of high-income countries. LIMITATIONS: A cross-sectional survey with possibility of response bias, social desirability bias, and limited data collection preventing in-depth analysis. CONCLUSIONS: In summary, findings reveal substantial variations in global HD use, availability, accessibility, quality, and affordability worldwide, with the lowest use evident in low- and lower-middle-income countries.
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Internacionalidade , Falência Renal Crônica/terapia , Padrões de Prática Médica , Diálise Renal , Derivação Arteriovenosa Cirúrgica , Custo Compartilhado de Seguro , Custos e Análise de Custo , Estudos Transversais , Países Desenvolvidos , Países em Desenvolvimento , Gastos em Saúde , Acessibilidade aos Serviços de Saúde , Humanos , Nefrologia , Medidas de Resultados Relatados pelo Paciente , Qualidade da Assistência à Saúde , Inquéritos e Questionários , Transporte de PacientesRESUMO
RATIONALE & OBJECTIVE: Approximately 11% of people with kidney failure worldwide are treated with peritoneal dialysis (PD). This study examined PD use and practice patterns across the globe. STUDY DESIGN: A cross-sectional survey. SETTING & PARTICIPANTS: Stakeholders including clinicians, policy makers, and patient representatives in 182 countries convened by the International Society of Nephrology between July and September 2018. OUTCOMES: PD use, availability, accessibility, affordability, delivery, and reporting of quality outcome measures. ANALYTICAL APPROACH: Descriptive statistics. RESULTS: Responses were received from 88% (n=160) of countries and there were 313 participants (257 nephrologists [82%], 22 non-nephrologist physicians [7%], 6 other health professionals [2%], 17 administrators/policy makers/civil servants [5%], and 11 others [4%]). 85% (n=156) of countries responded to questions about PD. Median PD use was 38.1 per million population. PD was not available in 30 of the 156 (19%) countries responding to PD-related questions, particularly in countries in Africa (20/41) and low-income countries (15/22). In 69% of countries, PD was the initial dialysis modality for≤10% of patients with newly diagnosed kidney failure. Patients receiving PD were expected to pay 1% to 25% of treatment costs, and higher (>75%) copayments (out-of-pocket expenses incurred by patients) were more common in South Asia and low-income countries. Average exchange volumes were adequate (defined as 3-4 exchanges per day or the equivalent for automated PD) in 72% of countries. PD quality outcome monitoring and reporting were variable. Most countries did not measure patient-reported PD outcomes. LIMITATIONS: Low responses from policy makers; limited ability to provide more in-depth explanations underpinning outcomes from each country due to lack of granular data; lack of objective data. CONCLUSIONS: Large inter- and intraregional disparities exist in PD availability, accessibility, affordability, delivery, and reporting of quality outcome measures around the world, with the greatest gaps observed in Africa and South Asia.
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Acessibilidade aos Serviços de Saúde , Internacionalidade , Falência Renal Crônica/terapia , Diálise Peritoneal , Padrões de Prática Médica , Pessoal Administrativo , Custo Compartilhado de Seguro , Custos e Análise de Custo , Estudos Transversais , Atenção à Saúde , Países Desenvolvidos , Países em Desenvolvimento , Gastos em Saúde , Política de Saúde , Humanos , Nefrologistas , Nefrologia , Avaliação de Resultados em Cuidados de Saúde , Medidas de Resultados Relatados pelo Paciente , Médicos , Qualidade da Assistência à Saúde , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Health information systems (HIS) are fundamental tools for the surveillance of health services, estimation of disease burden and prioritization of health resources. Several gaps in the availability of HIS for kidney disease were highlighted by the first iteration of the Global Kidney Health Atlas. METHODS: As part of its second iteration, the International Society of Nephrology conducted a cross-sectional global survey between July and October 2018 to explore the coverage and scope of HIS for kidney disease, with a focus on kidney replacement therapy (KRT). RESULTS: Out of a total of 182 invited countries, 154 countries responded to questions on HIS (85% response rate). KRT registries were available in almost all high-income countries, but few low-income countries, while registries for non-dialysis chronic kidney disease (CKD) or acute kidney injury (AKI) were rare. Registries in high-income countries tended to be national, in contrast to registries in low-income countries, which often operated at local or regional levels. Although cause of end-stage kidney disease, modality of KRT and source of kidney transplant donors were frequently reported, few countries collected data on patient-reported outcome measures and only half of low-income countries recorded process-based measures. Almost no countries had programs to detect AKI and practices to identify CKD-targeted individuals with diabetes, hypertension and cardiovascular disease, rather than members of high-risk ethnic groups. CONCLUSIONS: These findings confirm significant heterogeneity in the global availability of HIS for kidney disease and highlight important gaps in their coverage and scope, especially in low-income countries and across the domains of AKI, non-dialysis CKD, patient-reported outcomes, process-based measures and quality indicators for KRT service delivery.
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Sistemas de Informação em Saúde , Insuficiência Renal Crônica , Estudos Transversais , Países em Desenvolvimento , Humanos , Rim , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/terapiaRESUMO
BACKGROUND: As hypophosphatemia is a common multifactorial problem of kidney transplantation (Tx), this research aimed at studying the frequency of posttransparent hypophosphatemia in the early postkidney Tx period and investigating the risk components associated with the situation. MATERIALS AND METHODS: In this study, 50 renal transplant recipients on the day before (-1) and on days 10 (+10) and 30 (+30) days after kidney Tx were examined for the levels of serum phosphate (Pi). Levels of serum creatinine (Cr), Pi, 25-hydroxyvitamin D (25[OH] D), intact parathyroid hormone (iPTH) and fibroblast growth factor 23 (FGF-23), the 24 h urinary excretion of Pi and Cr, estimated glomerular filtration rate (eGFR), and the ratio of transport maximum of Pi (TMP) to eGFR (TMP/GFR) were evaluated on the same days. RESULTS: Hypophosphatemia (serum Pi <2.5 mg/dl) was seen in 0%, 40%, and 42% of the patients on days -1, +10, and +30, respectively. The levels of 25(OH)D and iPTH were not significantly different in patients with and without hypophosphatemia on days +10 and +30. Compared to those with normophosphatemia, pre-Tx FGF-23 level was significantly higher in patients with hypophosphatemia on days +10 and +30, respectively. The regression coefficient of TMP/GFR and Cr was positive on days -1, +10, and +30. The coefficient of pre-Tx FGF-23 on post-Tx serum Pi was negative on days +10 (P < 0.03) and +30 (P < 0.003), and the coefficient of post-Tx FGF-23 was negative just on day +10 with serum Pi (P < 0.008). CONCLUSION: The main causes of post-Tx hypophosphatemia in the multivariate linear analysis were pre-Tx FGF-23 and post-Tx FGF-23 levels on days +10, post-Tx Cr, and TMP/GFR.
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BACKGROUND: Anemia is one of the most prevalent complications in patients with chronic kidney disease, which is believed to be caused by the insufficient synthesis of erythropoietin by the kidney. This phase III study aimed to compare the efficacy and safety of CinnaPoietin® (epoetin beta, CinnaGen) with Eprex® (epoetin alfa, Janssen Cilag) in the treatment of anemia in ESRD hemodialysis patients. METHODS: In this randomized, active-controlled, double-blind, parallel, and non-inferiority trial, patients were randomized to receive either CinnaPoietin® or Eprex® for a 26-week period. The primary endpoints of this study were to assess the mean hemoglobin (Hb) change during the last 4 weeks of treatment from baseline along with the evaluation of the mean weekly epoetin dosage per kilogram of body weight that was necessary to maintain the Hb level within 10-12 g/dL during the last 4 weeks of treatment. As the secondary objective, safety was assessed along with other efficacy endpoints. RESULTS: A total of 156 patients were included in this clinical trial. There was no statistically significant difference between treatment groups regarding the mean Hb change (p = 0.21). In addition, the mean weekly epoetin dosage per kg of body weight for maintaining the Hb level within 10-12 g/dL showed no statistically significant difference between treatment arms (p = 0.63). Moreover, both products had comparable safety profiles. However, the incidence of Hb levels above 13 g/dL was significantly lower in the CinnaPoietin® group. CONCLUSION: CinnaPoietin® was proved to be non-inferior to Eprex® in the treatment of anemia in ESRD hemodialysis patients. The trial was registered in Clinicaltrials.gov (NCT03408639).
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Anemia/tratamento farmacológico , Epoetina alfa/administração & dosagem , Eritropoetina/administração & dosagem , Hematínicos/administração & dosagem , Falência Renal Crônica/complicações , Adulto , Idoso , Anemia/sangue , Anemia/etiologia , Epoetina alfa/efeitos adversos , Eritropoetina/efeitos adversos , Feminino , Hematínicos/efeitos adversos , Hemoglobinas/análise , Humanos , Injeções Subcutâneas , Falência Renal Crônica/sangue , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Proteínas Recombinantes/administração & dosagem , Proteínas Recombinantes/efeitos adversos , Diálise Renal , Resultado do TratamentoRESUMO
INTRODUCTION AND AIM: Occult hepatitis C infection (OHCI) is the presence of HCV-RNA in the liver or peripheral blood mononuclear cells (PBMC) accompanying with negative serologic results. The aim of this study was to evaluate the prevalence of OHCI among Iranian chronic hemodialysis (HD) patients. MATERIAL AND METHODS: In this cross sectional study 200 chronic HD patients with negative HCV antibody enrolled the study. Blood sample of patients were obtained, followed by Polymerase Chain reaction (PCR) testing for detection of HCV RNA. Patients with positive serum HCV RNA were considered as manifest hepatitis C infection (MHCI). However, patients with negative serum HCV RNA underwent further tests on PBMCs for detection of OHCI. RESULTS: Serum HCV RNA was positive in 2 (1%) patients whom considered as MHCI, and 6 (3.03%) patients had positive PBMC HCV RNA. CONCLUSION: In conclusion, chronic HD patients have been considered as a high risk group for hepatitis C infection. The results of this study suggest that these patients are also at risk for OHCI. Furthermore, evaluating PBMCs to detect HCV RNA would be a sensitive diagnostic method to find OHCI patients.
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Hepatite C/epidemiologia , Falência Renal Crônica/terapia , Diálise Renal/efeitos adversos , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Estudos Transversais , Feminino , Hepacivirus/genética , Hepatite C/sangue , Hepatite C/diagnóstico , Hepatite C/virologia , Humanos , Irã (Geográfico)/epidemiologia , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/epidemiologia , Leucócitos Mononucleares/virologia , Masculino , Pessoa de Meia-Idade , Prevalência , RNA Viral/sangue , Medição de Risco , Fatores de Risco , Carga Viral , Adulto JovemRESUMO
IgG4-related disease (IgG4-RD) is a chronic systemic inflammatory disease, characterized by tissue infiltration of lymphocytes and IgG4-secreting plasma cells, presenting by fibrosis of different tissues, which is usually responsive only to oral steroids therapy. Kidneys are the most commonly involved organs, exhibiting renal insufficiency, tubulointerstitial nephritis, and glomerulonephritis. Here, we describe a patient with acute renal insufficiency who was presented with edema, weakness, anemia and multiple lymphadenopathies. Kidney and lymph node biopsy showed crescentic glomerulonephritis in kidneys and lymphoplasmacytic infiltration in lymph nodes. After a course of treatment with an intravenous pulse of corticosteroid and cyclophosphamide, the patient's symptoms subsided, and kidney function improved. DOI: 10.52547/ijkd.7788.
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Ciclofosfamida , Glomerulonefrite , Doença Relacionada a Imunoglobulina G4 , Humanos , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Glomerulonefrite/imunologia , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/diagnóstico , Glomerulonefrite/patologia , Ciclofosfamida/uso terapêutico , Masculino , Linfonodos/patologia , Imunossupressores/uso terapêutico , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/imunologia , Rim/patologia , Biópsia , Imunoglobulina G/sangue , Glucocorticoides/uso terapêutico , Pessoa de Meia-Idade , Resultado do Tratamento , Linfadenopatia/etiologia , Plasmócitos/imunologia , Plasmócitos/patologiaRESUMO
INTRODUCTION: Recurrence of glomerulonephritis (GN) after kidney transplant (Tx) may be associated with allograft loss. This study aimed to evaluate the frequency and prognosis of de novo or recurrent post-Tx GN. METHODS: We reviewed 1305 kidney Tx biopsy samples obtained between 2006 and 2020. The biopsy specimens were divided into post-Tx GN (recurrent or de novo) and control groups (i.e., no detectable GN in biopsy). Demographic and baseline characteristics of the patients and kidney survival rates were analyzed. RESULTS: From 1305 kidney transplanted biopsies, 350 repeated biopsies for transplant rejection were excluded. Among 955 analyzed biopsies, (mean age: 40.4 ± 13.48 years, mean transplantation duration: 4.54 ± 3.98 years, 74.6% males), the frequency of GN was 10.78%. The most common recurrent post-Tx GN was IgA nephropathy (22.3%), followed by secondary focal segmental glomerulonephritis (FSGS, 19.4%), primary FSGS (19.4%), and membranous glomerulonephritis (17.5%). In the post-Tx GN group, the mean serum creatinine and proteinuria were 3.28 ± 1.97 mg/dL and 2730 ± 1244 mg/d at the biopsy time and 4.14 ± 1.86 mg/dL and 2020 ± 1048 mg/d, at the end of the study. There was a significant relationship between baseline serum creatinine and graft loss (P < .001). One-, five-, and ten-year graft survival rates were 97%, 81%, and 63% in the postTx GN, and 100%, 92%, and 59% in the control group. The median time to graft loss after biopsy, (graft survival after biopsy), was significantly lower in the post-Tx GN group (P < .000). The other accompanying factors had no significant impact on graft survival. CONCLUSION: The median time to graft loss after biopsy was significantly lower in post-Tx GN. Baseline serum creatinine had a significant association with graft loss. Optimal management of recurrent or de novo GN should be a main focus of post-transplant care. DOI: 10.52547/ijkd.7205.
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Glomerulonefrite por IGA , Glomerulonefrite , Glomerulosclerose Segmentar e Focal , Transplante de Rim , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Biópsia , Creatinina , Glomerulonefrite/complicações , Glomerulonefrite por IGA/complicações , Glomerulosclerose Segmentar e Focal/complicações , Sobrevivência de Enxerto , Transplante de Rim/efeitos adversos , Prevalência , Prognóstico , Estudos RetrospectivosRESUMO
INTRODUCTIONS: Malnutrition-inflammation-atherosclerosis is an independent risk factor and the most significant cause of death in dialysis patients, accounting for about 50% of deaths in the population. Moreover, the high incidence of cardiovascular-induced mortality in patients with end-stage kidney disease cannot be fully attributed to cardiovascular (CVD) risk factors only. Studies suggest that risk factors such as oxidative stress, inflammation, bone disorders, vascular stiffness, and energy protein loss are closely related to CVD and its associated mortality in these patients. Moreover, dietary fat is a crucial factor in CVD. This study focused on determining the relationship between malnutrition-inflammation and fat quality indicators among CKD patients. METHODS: This study was conducted on 121 hemodialysis patients aged 20 to 80 years in a teaching hospital affiliated to Hashminejad kidney center in Tehran, Iran during 2020 to 2021. Data on general characteristics and anthropometric indices were collected. The malnutrition-inflammation score was assessed by using MIS and DMS questionnaires and dietary intake was measured by a 24-hour recall questionnaire. RESULTS: Out of 121 hemodialysis patients participating in the study, 57.3% were male and 42.7% were female. Anthropometric demographic characteristics showed no significant difference among diverse groups with heart disease (P > .05). There was no significant relationship between malnutrition-inflammation and heart disease indices in hemodialysis patients (P > .05). Furthermore, there was no correlation between the dietary fat quality index and heart disease (P > .05). CONCLUSION: In this study, there was no significant relationship between the malnutrition-inflammation index and the dietary fat quality index with cardiac disease in hemodialysis patients. Further studies are needed to have a tangible conclusion. DOI: 10.52547/ijkd.7280.
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Doenças Cardiovasculares , Cardiopatias , Falência Renal Crônica , Desnutrição , Humanos , Masculino , Feminino , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/complicações , Estado Nutricional , Gorduras na Dieta/efeitos adversos , Irã (Geográfico)/epidemiologia , Desnutrição/etiologia , Desnutrição/complicações , Inflamação/etiologia , Diálise Renal/efeitos adversos , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Cardiopatias/complicaçõesRESUMO
INTRODUCTION: Membranous nephropathy (MN) has variable clinical outcomes, ranging from spontaneous remission to slow progression to kidney failure. Since the clinical outcomes of MN have not been studied in a large sample size in Iran, this study was designed to evaluate the outcome of patients diagnosed with MN at Hasheminejad Kidney Center (HKC), Tehran. METHODS: In this retrospective cohort study, 1086 patients with a diagnosis of MN who were biopsied between 1998 and 2018 in HKC were evaluated through a review of medical records for baseline clinical and laboratory characteristics at the time of biopsy and through a review of follow-up charts and phone calls for the evaluation of clinical outcomes. Of these patients, 551 could be followed for clinical outcomes. The composite outcome included kidney loss (hemodialysis, transplantation, or death). The effect of demographic, clinical, laboratory, and pathological variables on kidney survival was determined by the Cox-regression model using SPSS-16 software at a significance level of .05. RESULTS: Sex (P < .05), higher weight (P < .05), older age (P < .001), hypertension (P < .001), higher baseline proteinuria and lower glomerular filtration rate (GFR) at the onset of the disease were associated with kidney failure (P < .001). A higher percentage of interstitial fibrosis, tubular atrophy, global sclerosis, and a higher pathological class of membranous nephropathy were significantly associated with disease outcome in the univariate Cox-regression analysis (P < .001). Kidney survival rates at 5, 10, and 15 years were 86%, 74%, and 56%; respectively. CONCLUSION: Our study suggests that baseline demographic, clinical and laboratory factors affect kidney outcomes. Patients who are considered high-risk based on the criteria listed above may need to be candidates for more aggressive therapy. DOI: 10.52547/ijkd.7373.
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Glomerulonefrite Membranosa , Insuficiência Renal , Humanos , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/patologia , Estudos Retrospectivos , Irã (Geográfico)/epidemiologia , Progressão da Doença , Rim , Taxa de Filtração GlomerularRESUMO
National strategies for addressing chronic kidney disease (CKD) are crucial to improving kidney health. We sought to describe country-level variations in non-communicable disease (NCD) strategies and CKD-specific policies across different regions and income levels worldwide. The International Society of Nephrology Global Kidney Health Atlas (GKHA) was a multinational cross-sectional survey conducted between July and October 2018. Responses from key opinion leaders in each country regarding national NCD strategies, the presence and scope of CKD-specific policies, and government recognition of CKD as a health priority were described overall and according to region and income level. 160 countries participated in the GKHA survey, comprising 97.8% of the world's population. Seventy-four (47%) countries had an established national NCD strategy, and 53 (34%) countries reported the existence of CKD-specific policies, with substantial variation across regions and income levels. Where CKD-specific policies existed, non-dialysis CKD care was variably addressed. 79 (51%) countries identified government recognition of CKD as a health priority. Low- and low-middle income countries were less likely to have strategies and policies for addressing CKD and have governments which recognise it as a health priority. The existence of CKD-specific policies, and a national NCD strategy more broadly, varied substantially across different regions around the world but was overall suboptimal, with major discrepancies between the burden of CKD in many countries and governmental recognition of CKD as a health priority. Greater recognition of CKD within national health policy is critical to improving kidney healthcare globally.
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INTRODUCTION: Crescents (C) have been recently added to the Oxford classification of IgA nephropathy (IgAN) consisting of mesangial hypercellularity (M), endocapillary hypercellularity (E), segmental sclerosis (S) and tubular atrophy/ interstitial fibrosis (T) (MEST). The aim of the study was to assess the added impact of crescents, on development of end-stage kidney disease (ESKD) in IgAN patients Methods. On-hundred fifteen IgAN patients (76% male, mean age: 37 ± 13 years, mean serum creatinine: 4.0 ± 4.3 mg/dL, mean proteinuria: 3.4 ± 2.5 g/d) were followed for 43 ± 29 months. MEST score was defined according to Oxford classification (M0/M1, E0/ E1, S0/S1). To increase the power, T was defined as T0 ≤ 25% and T1 > 25%. Crescents were defined as C0, "absence" and C1 "at least one" crescent. In sensitivity analysis, the risk of ESKD was estimated at different cut-off levels of at least 10, 20, and 30% crescents. RESULTS: Forty patients (35%) developed ESKD. Among those 14% with at least one crescent, 21 patients (46%) developed ESKD. In 11 patients with C ≥ 30%, 66% and among 57 patients with T1, 60% and in 27 patients with T1 + C1 74% developed ESKD. In adjusted model, only C ≥ 30% (HR = 3.15, 95% CI: 1.15 to 11.00; P = 0.027) and the presence of T1+ C1 (HR = 7.18, 95% CI: 1.90 to 27.10, P = 0.004) were associated with increased risk of ESKD. The median kidney survival was 78.0 months (95% CI: 70.5 to 85.6 months), in patients with T0 + C0 and 32.3 months (95% CI: 19.3 to 45.3 months) in patients with T1 + C1. CONCLUSION: In this study T ≥ 25%, and the presence of crescents ≥ 30%, were independently associated with increased risk of ESKD. This risk was strongly increased in the combined presence of at least one crescent and T1 ≥ 25%, that predicted a high ESKD rate. DOI: 10.52547/ijkd.6685.
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Glomerulonefrite por IGA , Falência Renal Crônica , Adulto , Feminino , Glomerulonefrite por IGA/complicações , Humanos , Rim , Falência Renal Crônica/complicações , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Proteinúria/etiologia , Medição de Risco , Adulto JovemRESUMO
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD), one of the common inherited disorders in humans, is characterized by the development and enlargement of renal cysts, often leading to end-stage renal disease (ESRD). In this study, Iranian ADPKD families were subjected to high-throughput DNA sequencing to find potential causative variants facilitating the way toward risk assessment and targeted therapy. METHODS: Our protocol was based on the targeted next generation sequencing (NGS) panel previously developed in our center comprising 12 genes involved in PKD. This panel has been applied to investigate the genetic causes of 32 patients with a clinical suspicion of ADPKD. RESULTS: We identified a total of 31 variants for 32 individuals, two of which were each detected in two individuals. Twenty-seven out of 31 detected variants were interpreted as pathogenic/likely pathogenic and the remaining 4 of uncertain significance with a molecular diagnostic success rate of 87.5%. Among these variants, 25 PKD1/2 pathogenic/likely pathogenic variants were detected in 32 index patients (78.1%), and variants of uncertain significance in four individuals (12.5% in PKD1/2). The majority of variants was identified in PKD1 (74.2%). Autosomal recessive PKD was identified in one patient, indicating the similarities between recessive and dominant PKD. In concordance with earlier studies, this biallelic PKD1 variant, p.Arg3277Cys, leads to rapidly progressive and severe disease with very early-onset ADPKD. CONCLUSION: Our findings suggest that targeted gene panel sequencing is expected to be the method of choice to improve diagnostic and prognostic accuracy in PKD patients with heterogeneity in genetic background.
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Rim Policístico Autossômico Dominante , Humanos , Análise Mutacional de DNA/métodos , Sequenciamento de Nucleotídeos em Larga Escala , Irã (Geográfico) , Mutação , Rim Policístico Autossômico Dominante/genética , Rim Policístico Autossômico Dominante/diagnóstico , Canais de Cátion TRPP/genéticaRESUMO
BACKGROUND: Kidney transplantation (KT) is the optimal treatment for kidney failure and is associated with better quality of life and survival relative to dialysis. However, knowledge of the current capacity of countries to deliver KT is limited. This study reports on findings from the 2018 International Society of Nephrology Global Kidney Health Atlas survey, specifically addressing the availability, accessibility, and quality of KT across countries and regions. METHODS: Data were collected from published online sources, and a survey was administered online to key stakeholders. All country-level data were analyzed by International Society of Nephrology region and World Bank income classification. RESULTS: Data were collected via a survey in 182 countries, of which 155 answered questions pertaining to KT. Of these, 74% stated that KT was available, with a median incidence of 14 per million population (range: 0.04-70) and median prevalence of 255 per million population (range: 3-693). Accessibility of KT varied widely; even within high-income countries, it was disproportionately lower for ethnic minorities. Universal health coverage of all KT treatment costs was available in 31%, and 57% had a KT registry. CONCLUSIONS: There are substantial variations in KT incidence, prevalence, availability, accessibility, and quality worldwide, with the lowest rates evident in low- and lower-middle income countries. Understanding these disparities will inform efforts to increase awareness and the adoption of practices that will ensure high-quality KT care is provided around the world.
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Falência Renal Crônica , Transplante de Rim , Países em Desenvolvimento , Acessibilidade aos Serviços de Saúde , Humanos , Falência Renal Crônica/epidemiologia , Transplante de Rim/efeitos adversos , Qualidade de VidaRESUMO
INTRODUCTION: Dysregulated vitamin D metabolism is one of the most important issues in chronic kidney disease- mineral and bone disorder (CKD-MBD). Patients with end-stage kidney disease (ESKD) receive large amounts of calcitriol, i.e., 1,25 -dihydroxy vitamin D [1-25(OH)2D], for suppression of parathyroid hormone (PTH). The aim of this study was to evaluate the 1-25(OH)2D status in maintenance hemodialysis patients and its correlation with 25(OH) D level and calcitriol consumption and to determine whether the usual practice of administrating large amounts of calcitriol for suppression of PTH may lead to toxic serum levels. METHODS: One hundred and fifty-six maintenance hemodialysis patients were enrolled. Demographic data, comorbid conditions and history of medication use (cumulative and current doses) were retrieved from Hemodialysis Data Processor Software previously designed for our center. Predialysis serum samples were measured for serum levels of 25(OH)D and 1-25(OH)2D accompanying by markers of mineral bone metabolism and inflammation. RESULTS: Of 156 patients, 66% were male and the mean age was 56.5 ± 16.3 years. There was no significant correlation between serum level of 25(OH)D and 1,25(OH)2D (r = 0.12, P > .05). Only current ingestion of vitamin D was correlated with both 25(OH) D (r = 0.324, P < .001) and 1,25(OH)2D serum levels (r = 0.334, P < .001). There was no significant relationship between current or cumulative calcitriol consumption and 1,25(OH)2D serum level. 1,25(OH)2D/25(OH)D ratio which, represents the degree of vitamin D hydroxylation efficiency was 0.9 pg/ng (expected value in no CKD > 2.2 pg/ng). CONCLUSION: Calcitriol consumption was not correlated with increased serum 1,25(OH)2D level and the practice of hyperparathyroidism treatment with calcitriol may be safely continued, though we are not yet aware of the 1,25(OH)2D status at the cellular levels.
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Diálise Renal , Vitamina D , Adulto , Idoso , Calcitriol , Humanos , Masculino , Pessoa de Meia-Idade , Hormônio Paratireóideo , Vitamina D/análogos & derivadosRESUMO
INTRODUCTION: Despite significant improvement in End Stage Kidney Disease (ESKD) patient's management, and better availability of dialysis for caregivers, mortality among these patients is unacceptably high. METHODS: We collected the data of 751 incident hemodialysis patients from March 2004 to November 2018. Survival curves was created by using the Kaplan-Meier method. Comorbidities, as well as time-dependent values of laboratory findings, were examined as independent factors by three models of Cox regression analysis. RESULTS: The median follow-up period was 31.7 months (1.08 to 169.28). Patient survival rates were 88%, 77%, 56%, 32%, 26% ,16% and 12%, at 1, 2, 4,6, 8, 10, 12 and 14 years of follow-up, respectively. The most common cause of mortality was cardiovascular disease. We observed lower survival rates in patients ≥ 65 years (HR = 2.684, 95% CI: 1.133 to 3.377; P < .001), diabetes mellitus (HR = 1.729, 95% CI: 1.484 to 2.014; P < .001) and walking disability (HR = 2.505; 95% CI: 2.104 to 2.983; P < .001). Low hemoglobin level (HR = 1.496; 95% CI: 1.257 to 1.779; P < .001), hyperphosphatemia (HR = 1.305, 95% CI: 1.104 to 1.542; P = 0.002) and high low-density lipoprotein cholesterol level (HR = 1.933; 95% CI: 1.431 to 2.611; P < .001) were predictors of mortality. A single pool Kt/V > 1.2 (HR = 0.743, 95% CI: 0.635 to 0.870; P < .001) and high serum creatinine level (HR = 0.842, 95% CI: 0.811 to 0.874; P < .001) showed protective effects. CONCLUSION: Our study showed a high survival rate in a single center cohort of hemodialysis patients in Iran. Traditional risk factors of mortality in general population, as well as indices of dialysis efficacy and general health status were the main predictors of mortality. Nationwide registries are necessary to investigate the dialysis survival rates and their predictors in our country. DOI: 10.52547/ijkd.6435.
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Falência Renal Crônica , Diálise Renal , Estudos de Coortes , Humanos , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Modelos de Riscos Proporcionais , Fatores de RiscoRESUMO
INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) is one of the important causes of end stage kidney disease (ESKD). We evaluated the progression risk factors of primary FSGS to chronic kidney disease (CKD) or ESKD with a predictive model including clinical and histological predictors. METHODS: 201 patients with primary FSGS (59% male, mean age: 38 ± 15 years), were studied. Time-dependent Cox model and C statistics were used for the predictive model. Interaction and correlation between independent variables were estimated. RESULTS: During 55 ± 27 months of follow-up, 82 patients (41%) developed CKD (46) or ESKD (36) patients. In adjusted model, 1 unit of higher serum creatinine (SCr) at baseline (HR = 1.39, 95% CI: 1.15 to 1.70) and 1% increase in glomeruli with segmental glomerulosclerosis (SGS) (HR = 1.03, 95% CI: 1.02 to 1.04) or interstitial fibrosis/tubular atrophy (IF/TA) (HR = 1.03, 95% CI: 1.01 to 1.05) increased the risk of CKD/ESKD. In adjusted model, higher baseline proteinuria and collapsing variant were not associated with risk of CKD/ESKD. By adding SGS and IF/TA scores to baseline SCr in the model, discrimination by C statistics was 0.83 (95% CI: 0.77 to 0.90). Median renal survival was 3.1 years (95% CI: 2.2 to 4.1 years) in patients with highest risk score (baseline eGFR < 25 mL/min/1.73m2 + IF/TA/SGS > 50%), and 8.1 years (95% CI: 7.7 to 8.6 years).in those with lowest score (baseline eGFR > 75 mL/ min/1.73m2 + IF/TA/SGS < 5%). CONCLUSION: In primary FSGS, higher baseline SCr, increased SGS and IF/TA, but not baseline proteinuria and collapsing pathology, were the predictors for CKD/ESKD. These findings indicated the importance of timely detection and referral in prognosis of primary FSGS. DOI: 10.52547/ijkd.6442.
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Glomerulosclerose Segmentar e Focal , Falência Renal Crônica , Insuficiência Renal Crônica , Adulto , Feminino , Glomerulosclerose Segmentar e Focal/diagnóstico , Humanos , Rim , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Proteinúria/etiologia , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Adulto JovemRESUMO
Kidney failure is the permanent impairment of kidney function associated with increased morbidity, hospitalization, and requirement for kidney replacement therapy. A total of 11 countries in the Middle East region (84.6%) responded to the survey. The prevalence of chronic kidney disease in the region ranged from 5.2% to 10.6%, whereas prevalence of treated kidney failure ranged from 152 to 826 per million population. Overall, the incidence of kidney transplantation was highest in Iran (30.9 per million population) and lowest in Oman and the United Arab Emirates (2.2 and 3.0 per million population, respectively). Long-term hemodialysis services were available in all countries, long-term peritoneal dialysis services were available in 9 (69.2%) countries, and transplantation services were available in most countries of the region. Public funding covered the costs of nondialysis chronic kidney disease care in two-thirds of countries, and kidney replacement therapy in nearly all countries. More than half of the countries had dialysis registries; however, national noncommunicable disease strategies were lacking in most countries. The Middle East is a region with high burden of kidney disease and needs cost-effective measures through effective health care funding to be available to improve kidney care in the region. Furthermore, well-designed and sustainable health information systems are needed in the region to address current gaps in kidney care in the region.
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OBJECTIVES: The Global Kidney Health Atlas (GKHA) is a multinational, cross-sectional survey designed to assess the current capacity for kidney care across all world regions. The 2017 GKHA involved 125 countries and identified significant gaps in oversight, funding and infrastructure to support care for patients with kidney disease, especially in lower-middle-income countries. Here, we report results from the survey for the second iteration of the GKHA conducted in 2018, which included specific questions about health financing and oversight of end-stage kidney disease (ESKD) care worldwide. SETTING: A cross-sectional global survey. PARTICIPANTS: Key stakeholders from 182 countries were invited to participate. Of those, stakeholders from 160 countries participated and were included. PRIMARY OUTCOMES: Primary outcomes included cost of kidney replacement therapy (KRT), funding for dialysis and transplantation, funding for conservative kidney management, extent of universal health coverage, out-of-pocket costs for KRT, within-country variability in ESKD care delivery and oversight systems for ESKD care. Outcomes were determined from a combination of desk research and input from key stakeholders in participating countries. RESULTS: 160 countries (covering 98% of the world's population) responded to the survey. Economic factors were identified as the top barrier to optimal ESKD care in 99 countries (64%). Full public funding for KRT was more common than for conservative kidney management (43% vs 28%). Among countries that provided at least some public coverage for KRT, 75% covered all citizens. Within-country variation in ESKD care delivery was reported in 40% of countries. Oversight of ESKD care was present in all high-income countries but was absent in 13% of low-income, 3% of lower-middle-income, and 10% of upper-middle-income countries. CONCLUSION: Significant gaps and variability exist in the public funding and oversight of ESKD care in many countries, particularly for those in low-income and lower-middle-income countries.
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Falência Renal Crônica , Diálise Renal , Estudos Transversais , Países em Desenvolvimento , Acessibilidade aos Serviços de Saúde , Humanos , Falência Renal Crônica/terapiaRESUMO
INTRODUCTION: Atherosclerosis is associated with increased intima- media thickness (IMT) and vascular calcification (VC) in maintenance hemodialysis (MHD) patients. Fetuin-A is a serum protein, which inhibits vascular calcification. The aim of this study was to investigate the association between fetuin-A and VC, in a group of MHD patients. METHODS: One hundred and forty-three MHD patients were included and followed for 3 years. Blood samples were studied for calcification and inflammation markers and fetuin-A was checked 3 times at the start, middle and the end of the study. We used common carotid doppler sonography for assessment of indices of VC, which were performed at baseline and at the end of the study. Vascular calcification was defined as a common carotid intima media thickness ≥ 0.8 mm on either side or the existence of any plaque or stenosis ≥ 50% on either side. RESULTS: From 143 patients (mean age 57.5 ± 15.9, 60.1% male), 104 patients (75.4%) had VC at baseline. The mean age and the prevalence of DM were significantly higher in patients with VC (P < .001 for both). There was no significant difference in the levels of Pi, PTH, and fetuin-A between the two groups. In a multiple logistic regression model at baseline only age (OR = 1.09, P < .001), and diabetes mellitus (OR = 4.59, P < .05) were associated with VC and dialysis vintage had a marginal association (OR = 1.20, P = .09). At the end of the study only age (OR = 1.12, P < .001), and CRP (OR = 1.14, P < .05) were associated with VC. The mean survival of patients with VC was significantly lower than the patients without VC (31.87 ± 0.95 vs. 33.73 ± 1.29, P < .05), however the mortality was not affected by fetuin-A level. CONCLUSION: Survival rate of patients without VC was higher than the patients with VC. We didn't find any correlation between the level of fetuin-A and VC. It seems that the traditional risk factors of VC, including age and diabetes mellitus are the main predictors of VC in MHD patients.