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1.
Retina ; 42(2): 274-282, 2022 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-34483311

RESUMO

PURPOSE: To characterize the spectrum of internal limiting membrane (ILM) disease in Alport syndrome using multimodal imaging, including widefield (WF) and ultra-widefield (UWF) modalities, and to report their relative prevalence according to the genetic pattern of inheritance. METHODS: Cross-sectional clinical study of patients diagnosed with Alport syndrome. All patients underwent UWF color photography and autofluorescence, WF-optical coherence tomography angiography and spectral-domain optical coherence tomography. Demographics, past medical and ophthalmic history, and genetic mutation history were collected. RESULTS: Forty-two eyes of 21 patients (11 men; age 36.6 ± 12.9 years) were included. Macular spectral-domain optical coherence tomography revealed ILM granularity, more frequent in X-linked Alport syndrome and corresponding to dot maculopathy on color fundus. Mid-peripheral spectral-domain optical coherence tomography scans revealed multilamellated ILM in eight eyes (19%), presumably progressive, which corresponded to a cavitary pattern on en-face OCT. En-face OCT revealed multiple areas of retinal nerve fiber layer dehiscence in the macula, overlapping with vascular lacunae on optical coherence tomography angiography, and a coarse arrangement of retinal nerve fiber layer above and below the temporal raphe in 20 eyes (52%). CONCLUSION: Multimodal imaging allowed for the detection/characterization of retinal findings (ILM granularity, progressive ILM lamellation, retinal nerve fiber layer dehiscence, vascular lacunae, and coarse arrangement of retinal nerve fiber layer toward the disc) as multifaceted manifestations of ILM disease in Alport syndrome.


Assuntos
Membrana Basal/diagnóstico por imagem , Nefrite Hereditária/complicações , Fibras Nervosas/patologia , Doenças Retinianas/diagnóstico por imagem , Células Ganglionares da Retina/patologia , Adolescente , Adulto , Membrana Basal/patologia , Angiografia por Tomografia Computadorizada , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Tomografia de Coerência Óptica , Acuidade Visual/fisiologia , Adulto Jovem
2.
Am J Kidney Dis ; 75(3): 417-425, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31585683

RESUMO

Infection is a major cause of morbidity and mortality in patients with chronic kidney disease (CKD), including those receiving maintenance dialysis or with a kidney transplant. Although responses to vaccines are impaired in these populations, immunizations remain an important component of preventative care due to their favorable safety profiles and the high rate of infection in these patients. Most guidelines for patients with CKD focus on the importance of the hepatitis B, influenza, and pneumococcal vaccines in addition to age-appropriate immunizations. More data are needed to determine the clinical efficacy of these immunizations and others in this population and define optimal dosing and timing for administration. Studies have suggested that there may be a benefit to immunization before the onset of dialysis or transplantation because patients with early-stage CKD generally have higher rates of seroconversion. Because nephrologists often serve as primary care physicians for patients with CKD, it is important to understand the role of vaccinations in the preventive care of this patient population.


Assuntos
Infecções Bacterianas/prevenção & controle , Transplante de Rim , Guias de Prática Clínica como Assunto , Diálise Renal , Insuficiência Renal Crônica/terapia , Vacinação/normas , Viroses/prevenção & controle , Humanos
3.
BMC Nephrol ; 21(1): 8, 2020 01 13.
Artigo em Inglês | MEDLINE | ID: mdl-31928529

RESUMO

BACKGROUND: Transfer from a pediatric to an adult medical setting is associated with many barriers. Additionally, there are little data on patients' assessment of the transition process itself. 3 years ago at Lurie Children's Hospital of Chicago, we established a kidney transition program with the help of an adult nephrologist, physician assistant (PA) and social worker (LCSW). After 18 months, we evaluated the patients' perception of the program as part of a continuous quality initiative process. METHODS: Patients who had transitioned from pediatric care and were seen at least once in the adult nephrology clinic were anonymized and asked to take an established 5-point Likert scale survey. Survey questions addressed readiness to transition, the transition process itself, and the perception of adult care. Surveys were followed with semi-structured interviews. 3 readers rated each response as either "negative," "neutral," or "positive." Average, standard deviation and reader reliability were calculated. The readers also selected a word that best depicted each response and those most-common words were counted by question and overall. RESULTS: 17 out of 42 patients (40%) completed the survey. Average age at transition (mean + SD) was 20 + 2 years; the majority of patients (82%) felt ready to transfer to adult care but only 59% felt they were consulted on the timing. 88% of patients felt having a transition appointment and meeting the adult care providers in the pediatric setting to be valuable. Although 94% of patients ultimately felt comfortable in the adult care environment, 18% experienced noticeable differences in treatment recommendations. 13 semi-structured interviews were conducted. Overall, the patients responded positively (3 + 0, 100% reader reliability) to the transition. But, when asked what could have improved the transition, the word the patients used most was, "earlier." CONCLUSION: Young adults (YA) transitioning to adult care often feel ready to transition earlier than their transfer of care date. They subjectively benefit from a transition program that outlines the process of transferring their care. Many YA patients would benefit from a transition program that bolsters patient independence during early adult care visits.


Assuntos
Medicina do Adolescente , Atitude Frente a Saúde , Transição para Assistência do Adulto , Adolescente , Adulto , Chicago , Hospitais Pediátricos , Humanos , Inquéritos e Questionários , Adulto Jovem
4.
J Infect Chemother ; 23(5): 326-332, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28109741

RESUMO

Vancomycin is one of the most commonly utilized antibiotics in US hospitals. It remains the drug of choice for the treatment of serious infections caused by methicillin-resistant Staphylococcus aureus. For many of these deep-seated infections, guidelines recommend achieving troughs of 15-20 mg/L for treatment efficacy. At our institution we observed a number of cases of presumed vancomycin-induced acute tubular necrosis clinically diagnosed by the nephrology service. We report eight cases of presumed vancomycin-induced acute tubular necrosis, three of which required hemodialysis before resolution of nephrotoxicity. Only three of the eight patients received nephrotoxins prior to development of nephrotoxicity. All eight patients ultimately recovered renal function following discontinuation.


Assuntos
Injúria Renal Aguda/induzido quimicamente , Antibacterianos/efeitos adversos , Vancomicina/efeitos adversos , Adulto , Antibacterianos/uso terapêutico , Relação Dose-Resposta a Droga , Feminino , Humanos , Masculino , Staphylococcus aureus Resistente à Meticilina/efeitos dos fármacos , Pessoa de Meia-Idade , Infecções Estafilocócicas/tratamento farmacológico , Vancomicina/uso terapêutico , Adulto Jovem
5.
Curr Rheumatol Rep ; 18(1): 5, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26711696

RESUMO

Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.


Assuntos
Injúria Renal Aguda/etiologia , Escleroderma Sistêmico/complicações , Injúria Renal Aguda/patologia , Injúria Renal Aguda/fisiopatologia , Injúria Renal Aguda/terapia , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Humanos , Transplante de Rim , Prognóstico , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/fisiopatologia
6.
Kidney Int Rep ; 9(2): 214-224, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38344731

RESUMO

Cystinosis is a rare, inherited, lysosomal storage disorder characterized by the progressive accumulation of intralysosomal cystine and subsequent organ and tissue damage. The kidneys are the first and most severely impacted organ. Although cystinosis was once considered a fatal pediatric disease, patients with cystinosis are living well into adulthood with advances in medical care, including kidney transplant and early and continuous use of cysteamine therapy. This increase in life expectancy has revealed an extrarenal phenotype of cystinosis that emerges in adolescence and adulthood, affecting nearly all body systems, including the endocrine and reproductive systems. As individuals with cystinosis are planning for the future, reproductive health and fertility have become areas of increased focus. This narrative review aims to summarize the current understanding of reproductive health and fertility in patients with cystinosis and discuss practical considerations for monitoring and managing these complications.

7.
Antimicrob Agents Chemother ; 57(12): 5854-9, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24018264

RESUMO

Ceftazidime is a broad-spectrum cephalosporin with high-level activity against a variety of Gram-negative pathogens, including Pseudomonas aeruginosa. Improved outcomes are associated with cumulative percentages of a 24-h period that the drug concentration exceeds the MIC under steady-state pharmacokinetic conditions (%TMIC) of >45 to 70% of the dosing interval. Optimal dosing to achieve a 90% probability of target attainment (PTA) in patients receiving high-flux hemodialysis (HFHD) is unknown. We used existing data from six anephric adults receiving hemodialysis to construct a population model with the Pmetrics package for R. From the final model's joint probability density, we simulated the PTA for various ceftazidime dosing regimens, HFHD schedules, and organism MICs. For HFHD every 48 h and 1 g of ceftazidime given posthemodialysis, the PTA exceeds 90% for all isolates with MICs of ≤8 µg/ml, assuming a goal of 70%TMIC. For 72-h dialysis intervals, postdialysis dosing of 1 g is adequate for achievement of the 70%TMIC goal only for organisms with MICs of ≤4 µg/ml, while 2 g is adequate for organisms with MICs of ≤8 µg/ml. A dose of 500 mg once daily, regardless of HFHD schedule, has a 90% PTA for organisms with MICs of ≤16 µg/ml, while 1 g once daily may achieve 100% PTA even for resistant organisms with a MIC of 32 µg/ml. Therefore, to ensure maximal ceftazidime activity, once-daily dosing of 500 mg to 1 g ceftazidime in patients receiving HFHD may be preferable for critically ill patients when MIC data are unavailable and for more resistant organisms with ceftazidime MICs of 16 to 32 µg/ml.


Assuntos
Antibacterianos/farmacocinética , Ceftazidima/farmacocinética , Modelos Estatísticos , Pseudomonas aeruginosa/efeitos dos fármacos , Diálise Renal/métodos , Antibacterianos/farmacologia , Ceftazidima/farmacologia , Contagem de Colônia Microbiana , Simulação por Computador , Esquema de Medicação , Cálculos da Dosagem de Medicamento , Humanos , Infusões Intravenosas , Testes de Sensibilidade Microbiana , Infecções por Pseudomonas/tratamento farmacológico , Infecções por Pseudomonas/microbiologia , Pseudomonas aeruginosa/crescimento & desenvolvimento , Insuficiência Renal Crônica/microbiologia , Insuficiência Renal Crônica/terapia
8.
Transplant Proc ; 55(10): 2410-2413, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37923572

RESUMO

BACKGROUND: Scleroderma renal crisis (SRC) is a devastating complication of diffuse cutaneous systemic sclerosis (dcSSc) that occurs in 5% to 20% of patients in this population. End-stage kidney disease develops in 25% to 40% of SRC, and mortality occurs in 50% at 5 years. Kidney transplantation (KT) is a viable option, but little data exist on outcomes. METHODS: We performed a retrospective study of all patients with dcSSc who underwent KT at Northwestern Hospital between 2000 and 2020. The objective of this study was to determine graft and patient survival at years 5 and 10 post-transplant. RESULTS: Both patient and graft survival were 78% and 100% at 5 and 10 years, respectively. Kidney transplantation is associated with favorable outcomes in patients and graft survival at 5 and 10 years in patients with dcSSc.


Assuntos
Injúria Renal Aguda , Transplante de Rim , Escleroderma Sistêmico , Humanos , Transplante de Rim/efeitos adversos , Estudos Retrospectivos , Chicago , Escleroderma Sistêmico/complicações , Injúria Renal Aguda/etiologia , Illinois
9.
Transl Vis Sci Technol ; 11(3): 23, 2022 03 02.
Artigo em Inglês | MEDLINE | ID: mdl-35311929

RESUMO

Purpose: To analyze the characteristics of the choriocapillaris and the choroid in patients with Alport syndrome (AS) and investigate their clinical and demographic associations. Methods: Multicenter, cross-sectional study. Forty-two eyes with AS were consecutively enrolled. A cohort of 33 healthy eyes was included as controls. Demographics and medical history were collected for each participant. Each eye underwent 3 × 3 swept-source optical coherence tomography angiography (PLEX Elite 9000 2.0; Carl Zeiss Meditec, Dublin, CA, USA) and spectral-domain OCT (Spectralis HRA2; Heidelberg Engineering, Heidelberg, Germany). Choriocapillaris flow deficit (FD) number, mean FD size, total FD area, FD density, subfoveal choroidal thickness (CT), total CT, and choroidal vascularity index (CVI) were compared between AS and control eyes. Factors associated with the FD density and the CVI in AS were explored with multivariable linear mixed models. Results: There was high intragroup variability in choriocapillaris and choroidal measurements in patients with AS. Choriocapillaris FD in patients with AS were more numerous compared to controls (P = 0.02). FD density in eyes with AS increased with older age (estimate = 0.31% for each year; 95% confidence interval [CI], 0.06-0.57; P = 0.02) and was higher in patients with a history of kidney transplant (estimate = 9.66% in case of positive history; 95% CI, 3.52-15.8; P = 0.006). The CVI was lower in eyes with dot maculopathy (estimate = -3.30% if present; 95% CI, -6.38 to -0.21; P = 0.04) and anterior lenticonus (estimate = -6.50% if present; 95% CI, -10.99 to -2.00; P = 0.006). Conclusions: Patients with AS with kidney involvement requiring transplant may present with more severe choriocapillaris impairment. Lower choroidal vascularity was found in the presence of other ocular structural abnormalities. Translational Relevance: An increased load of choriocapillaris flow deficits on optical coherence tomography angiography was found in patients with Alport syndrome who also had severe kidney disease requiring transplant.


Assuntos
Degeneração Macular , Nefrite Hereditária , Corioide/diagnóstico por imagem , Estudos Transversais , Feminino , Humanos , Masculino , Nefrite Hereditária/complicações , Tomografia de Coerência Óptica/métodos
10.
Ann Pharmacother ; 45(12): e64, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22116989

RESUMO

OBJECTIVE: To report a sieving coefficient for peramivir in a patient receiving continuous venovenous hemofiltration (CVVH). CASE SUMMARY: An 18-year-old male presented with chills, myalgias, and dyspnea and was hospitalized. Nasal secretions were positive for influenza by rapid antigen test at an outside facility and oseltamivir was commenced. Oral absorption was predicted to be unreliable, and intravenous peramivir was accessed as an emergency investigational new drug applicaiton (eIND). CVVH was initiated after the development of acute renal failure, with blood samples collected to determine peramivir concentrations. DISCUSSION: Peramivir, an intravenous investigational neuraminidase inhibitor with activity against influenza viruses, has limited data for dosing in the setting of CVVH. A single patient received 600 mg of peramivir intravenously and had blood and ultrafiltrate concentrations measured serially. A sieving coefficient of approximately 0.9 was identified. CONCLUSIONS: Peramivir is well cleared by CVVH, and drug exposure is potentially predictable based on flow rates. Further study is necessary.


Assuntos
Injúria Renal Aguda/metabolismo , Injúria Renal Aguda/terapia , Ciclopentanos/farmacocinética , Guanidinas/farmacocinética , Hemofiltração , Influenza Humana/tratamento farmacológico , Influenza Humana/metabolismo , Ácidos Carbocíclicos , Injúria Renal Aguda/virologia , Adolescente , Ciclopentanos/uso terapêutico , Guanidinas/uso terapêutico , Humanos , Vírus da Influenza A Subtipo H1N1/isolamento & purificação , Influenza Humana/virologia , Masculino , Oseltamivir/uso terapêutico
11.
Presse Med ; 50(1): 104063, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33548376

RESUMO

Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed.


Assuntos
Injúria Renal Aguda/etiologia , Hipertensão Maligna/etiologia , Escleroderma Sistêmico/complicações , Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/patologia , Injúria Renal Aguda/terapia , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Hipertensão Maligna/mortalidade , Hipertensão Maligna/patologia , Hipertensão Maligna/terapia , Gravidez , Complicações na Gravidez/etiologia , Diálise Renal
12.
Urology ; 148: 306-313, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33242556

RESUMO

OBJECTIVE: To examine the variability of estimated glomerular filtration rate (eGFR) in emerging adults with spina bifida (SB) by comparing multiple equations across the transitional age period, hypothesizing that creatinine (Cr)-based equations show greater variability than cystatin-C (CysC)- or combination-based equations. METHODS: A retrospective cohort study was performed from 2012 to 2017 at a multidisciplinary SB clinic. Emerging adults were defined as patients ages 18-28 years old. Four pediatric, 3 adult, and 3 averaged eGFR equations were considered. Cross-sectional variability in eGFR data was assessed using coefficients of variation, chronic kidney disease (CKD) stage classification, and pairwise percent relative difference in eGFR between analogous pediatric and adult equations based on included lab values. Longitudinal changes in eGFR over time were compared across equations using a covariance pattern model accounting for repeated measures. RESULTS: Seventy-five emerging adults with SB (median age 21.8 years; 55% female; 83% with myelomeningocele) were included in cross-sectional analyses. Adult equations gave higher median eGFRs by 22%-27% and generally milder CKD stage classification than analogous pediatric equations. In longitudinal analyses (median follow-up of 22 months), all equations conferred negative eGFR changes over time (range -1.9 to -4.3 mL/min/1.73m2 per year) that were not significantly different. CONCLUSION: In emerging adults with SB, adult equations demonstrated higher median eGFRs by 22%-27% compared to analogous pediatric equations, even with Cystatin-C, and generally downstaged CKD stage classification. The same eGFR equation should be used for serial kidney function monitoring in emerging adults with SB who transition care from pediatric to adult services.


Assuntos
Taxa de Filtração Glomerular , Rim/fisiopatologia , Disrafismo Espinal/fisiopatologia , Transição para Assistência do Adulto , Adolescente , Adulto , Estudos de Coortes , Creatinina/sangue , Estudos Transversais , Cistatina C/sangue , Feminino , Humanos , Masculino , Estudos Retrospectivos , Disrafismo Espinal/sangue , Adulto Jovem
13.
Prog Transplant ; 20(3): 216-20, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20929105

RESUMO

BACKGROUND: Despite the known benefits of preemptive kidney transplantation, its rate of use remains low. OBJECTIVE: To determine whether focused, comprehensive education provided at a clinic for patients with chronic kidney disease would improve the rate of preemptive transplantation and transplant wait times. METHODS: A retrospective cohort study design was used. The rate of preemptive transplantation and transplant wait times were compared between patients with end-stage renal disease who had been followed in a chronic kidney disease clinic for more than 3 months and patients with end-stage kidney disease who had not been followed for chronic kidney disease care during the same period. RESULTS: More African Americans than others had initiated dialysis without having had previous care for chronic kidney disease. The rate of preemptive transplantation was 24% for patients followed in the clinic. For those patients without living donor options, mean transplant referral time was significantly different between patients followed at the clinic and patients who were not: 234 (SD, 392) days before dialysis was started versus 161 (SD, 525) days after dialysis was started (P = .01). CONCLUSION: A chronic kidney disease clinic can influence rates of preemptive kidney transplantation and transplantation referral times.


Assuntos
Assistência Ambulatorial/organização & administração , Falência Renal Crônica/terapia , Transplante de Rim/estatística & dados numéricos , Educação de Pacientes como Assunto/organização & administração , Listas de Espera , Negro ou Afro-Americano/estatística & dados numéricos , Análise de Variância , Distribuição de Qui-Quadrado , Estudos de Coortes , Currículo , Feminino , Humanos , Illinois/epidemiologia , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Equipe de Assistência ao Paciente/organização & administração , Avaliação de Programas e Projetos de Saúde , Encaminhamento e Consulta/organização & administração , Estudos Retrospectivos , Gestão da Qualidade Total/organização & administração
14.
Arthritis Rheumatol ; 71(6): 964-971, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30614663

RESUMO

OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.


Assuntos
Injúria Renal Aguda/classificação , Hipertensão Maligna/classificação , Rim/patologia , Escleroderma Sistêmico/complicações , Injúria Renal Aguda/etiologia , Anemia Hemolítica/classificação , Anemia Hemolítica/etiologia , Pressão Sanguínea , Técnica Delphi , Humanos , Hipertensão/classificação , Hipertensão/etiologia , Hipertensão Maligna/etiologia , Proteinúria/classificação , Proteinúria/etiologia , Índice de Gravidade de Doença
15.
Am J Kidney Dis ; 49(5): 656-63, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17472848

RESUMO

BACKGROUND: Patients with end-stage renal disease (ESRD) have a high incidence of sudden cardiac death and may benefit from implantable cardioverter defibrillators (ICDs). However, ESRD also may predispose patients to device-related complications, potentially offsetting some of the benefits of prophylactic ICD placement. The purpose of this study is to compare the incidence of complications after cardiac rhythm management device (CRMD) implantation in patients with and without ESRD. METHODS: An observational study was performed on 41 patients with ESRD and 123 controls without ESRD who had a CRMD (permanent pacemaker or ICD) implanted at a single institution from 1998 to 2005. Controls were matched for age, sex, type of device, and calendar year of device implantation. Primary and secondary end points were any complication and complications requiring intervention, respectively. RESULTS: 23 complications occurred in 16 of 41 patients with ESRD (39%) versus 13 complications in 13 of 123 matched controls (11%; P < 0.001). Major complications occurred in 29% of patients with ESRD versus 5% of controls (P < 0.001), whereas minor complications occurred in 17% and 6%, respectively (P < 0.03). Hematoma, thrombosis, and device-related complications, including elevated defibrillation thresholds, were more common in patients with ESRD (P < 0.05 for all), and there also was a nonsignificant trend toward greater infection risk (P = 0.1). There were no fatal complications in either group. CONCLUSIONS: Patients with ESRD had greater complication rates after CRMD implantation compared with matched controls, but these complications did not result in death. These results should be considered when evaluating patients with ESRD for prophylactic CRMD implantation, but do not support withholding such therapy.


Assuntos
Desfibriladores Implantáveis/efeitos adversos , Frequência Cardíaca , Falência Renal Crônica/terapia , Idoso , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Gerenciamento Clínico , Falha de Equipamento , Feminino , Frequência Cardíaca/fisiologia , Humanos , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
16.
Adv Chronic Kidney Dis ; 14(1): 105-12, 2007 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17200050

RESUMO

This study examines whether stabilization of the glomerular filtration rate (GFR) is possible in patients with advanced chronic kidney disease (CKD), managed in a CKD clinic. A cohort of 82 patients with stages 4 and 5 CKD was followed for a period of 2 years after initiation of erythropoietin for anemia to determine the GFR and the frequency of primary outcomes (dialysis, transplantation, or death). GFR, calculated by the abbreviated Modification of Diet in Renal Disease formula, was determined every 3 months. After 24 months, 35 subjects (43%) developed a primary outcome. Controlled for other risk factors, the risk of having a primary outcome increased 19.7% for every unit that the GFR decreased (95% confidence interval [CI], 11.9%-26.8%, P < .001) and decreased 21.7% for every unit that the hemoglobin increased (95% CI, 0.5%-38.4%, P < .001). Blacks had a 3.1 times higher risk (95% CI, 1.4-6.9, P = .006) of developing a primary outcome than other ethnicities. In subjects who did not develop primary outcomes (n = 47 or 57%), GFR remained unchanged (19.5 +/- 9.1 at the end of the study v 20.8 +/- 5.3 mL/min/1.73 m(2) at baseline, P = .16). The standardized mortality rate was 4.75 and 9.77 per 100 person-year for stages 4 and 5, respectively. We conclude that stabilization of GFR over a 2-year period can be achieved in many patients with advanced CKD treated with erythropoietin in a CKD clinic. Although the precise reason for the stabilization of GFR cannot be elucidated from this study, our data are "proof of concept" that CKD outcomes can be improved in a CKD clinic setting.


Assuntos
Taxa de Filtração Glomerular , Nefropatias/terapia , Diálise Renal , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Estudos de Coortes , Feminino , Humanos , Nefropatias/fisiopatologia , Falência Renal Crônica/patologia , Falência Renal Crônica/terapia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
18.
Mol Immunol ; 77: 89-96, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27490940

RESUMO

C3 glomerulopathy (C3G) is an ultra-rare complement-mediated renal disease characterized histologically by the predominance of C3 deposition within in the glomerulus. Familial cases of C3G are extremely uncommon and offer unique insight into the genetic drivers of complement dysregulation. In this report, we describe a patient who presented with C3G. Because a relative carried the same diagnosis, we sought an underlying genetic commonality to explain the phenotype. As part of a comprehension genetic screen, we completed multiplex ligation-dependent probe amplification across the complement factor H related region and identified amplification alterations consistent with a genomic rearrangement. Using comparative genomic hybridization, we narrowed and then cloned the rearrangement breakpoints thereby defining a novel fusion gene that is translated into a serum protein comprised of factor H related-5 (short consensus repeats 1 and 2) and factor H-related-2 (short consensus repeats 1-4). These data highlight the role of factor H related proteins in the control of complement activity and illustrate how perturbation of that control leads to C3G.


Assuntos
Proteínas Inativadoras do Complemento C3b/genética , Proteínas do Sistema Complemento/genética , Glomerulonefrite Membranoproliferativa/genética , Adulto , Western Blotting , Hibridização Genômica Comparativa , Feminino , Humanos , Masculino , Reação em Cadeia da Polimerase Multiplex , Linhagem
19.
J Ren Nutr ; 15(3): 345-55, 2005 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-16007564

RESUMO

BACKGROUND: Malnutrition-inflammation complex syndrome and anorexia, common conditions in maintenance dialysis patients, are strongly associated with higher mortality and hospitalization and lower quality of life (QoL) in this population. Megestrol acetate, 800 mg/day, has been shown to increase appetite and food intake and to mitigate inflammation in cachectic AIDS and cancer patients, leading to weight gain, but it is also associated with side effects at this dose. METHODS: We evaluated the efficacy of the oral solution of megestrol acetate in half of its conventional dose in improving the nutritional state and inflammation in 10 hypoalbuminemic dialysis patients (albumin < 3.7 g/dL). Six women and 4 men, ages 60.2 years, took 400 mg of megestrol acetate solution daily for 16 weeks. Anthropometry, dual energy x-ray absorptiometry, 24-hour diet recalls, and biochemical measurements of nutrition and inflammation, including serum C-reactive protein and leptin, were performed. RESULTS: At the end of the 16 weeks of intervention, weight and body mass index increased by 9%, body fat proportion by 31%, and triceps skinfold by 40% (P < .01). Serum albumin increased from 3.0 to 3.3 g/dL and continued to increase significantly to 3.6 g/dL after 3 months postintervention (P = .03). Serum leptin increased from 5.2 to 10.7 ng/mL (P = .09). Daily protein and energy intake increased progressively up to 27% to 42% by the end of the trial (P < or = .01). In 8 patients without acute infection, serum C-reactive protein declined from 1.24 to 0.78 mg/L (P = .06). QoL and appetite were reported to be improved. No major side effects were observed, and all 10 patients completed the 16 weeks of daily intake of megestrol acetate without interruption. CONCLUSIONS: Megestrol acetate oral solution in half of its conventional dose is safe and improves the nutritional state, inflammation, and anorexia in maintenance dialysis patients. Larger-scale placebo-controlled randomized studies are needed to confirm the beneficial effects of 400 mg/day of megestrol acetate in dialysis patients.


Assuntos
Inflamação/tratamento farmacológico , Acetato de Megestrol/administração & dosagem , Desnutrição Proteico-Calórica/tratamento farmacológico , Diálise Renal , Adulto , Idoso , Idoso de 80 Anos ou mais , Anorexia/complicações , Anorexia/tratamento farmacológico , Antropometria , Composição Corporal , Índice de Massa Corporal , Peso Corporal , Proteína C-Reativa/análise , Proteínas Alimentares/administração & dosagem , Ingestão de Energia , Feminino , Humanos , Hipoalbuminemia/complicações , Inflamação/complicações , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Leptina/sangue , Masculino , Acetato de Megestrol/efeitos adversos , Pessoa de Meia-Idade , Estado Nutricional , Estudos Prospectivos , Desnutrição Proteico-Calórica/complicações , Albumina Sérica/análise , Síndrome , Resultado do Tratamento
20.
Semin Nephrol ; 22(6): 526-32, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12430097

RESUMO

In this article, we maintain that the management of patients with chronic kidney disease (CKD) is best provided in a clinic setting that integrates nephrologic expertise, patient education, and comprehensive supportive services. Our experience with a CKD clinic in an urban academic setting is described. As a way to assess and quantify the impact of our clinic on clinical outcomes, we have analyzed our results in terms of 2 variables: presence of permanent access at the time of dialysis initiation and impact on renal function as assessed by calculated glomerular filtration rate (GFR). The number of clinic visits was taken as an index of comprehensive renal care before dialysis initiation. Individuals who started dialysis with a functioning permanent access had been seen in our clinic more frequently than those seen less frequently (20 +/- 3.5 and 4.4 +/- 2.1 visits, respectively, P <.005). The impact on renal function was analyzed in a group of 80 unselected patients stratified into 3 stages based on the recently published National Kidney Foundation Disease Outcomes Quality Initiative (K/DOQI) guidelines: stage III (mean GFR 39 +/- 1.5 mL/min, n = 21), stage IV (mean GFR 21 +/- 0.6 mL/min, n = 46), and stage V (mean GFR 12 +/-.76 mL/min, n = 13). Provision of comprehensive renal care in conjunction with anemia management using weekly injections of erythropoietin subcutaneously resulted in stabilization of GFR in patients with stages IV and V over a period of 15 months of follow-up evaluation. In patients with stage III CKD, GFR decreased over the initial period of follow-up evaluation (first few months), and to a lesser extent by the end of follow-up evaluation (15 mo). Further studies are underway to discern the factor(s) underlying the overall clinic effect versus a beneficial effect of anemia correction on GFR. Our data suggests that stabilization of GFR is a goal that can be accomplished with comprehensive renal care provided in an organized clinic setting.


Assuntos
Instituições de Assistência Ambulatorial/normas , Falência Renal Crônica/terapia , Garantia da Qualidade dos Cuidados de Saúde , Diálise Renal , Anemia/etiologia , Anemia/terapia , Chicago , Assistência Integral à Saúde , Taxa de Filtração Glomerular , Humanos , Falência Renal Crônica/complicações , Apoio Nutricional , Guias de Prática Clínica como Assunto , Encaminhamento e Consulta
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