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2.
J Thromb Haemost ; 16(10): 1953-1963, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30063819

RESUMEN

Essentials Mortality due to bleeding vs. arterial thrombosis in dialysis patients is unknown. We compared death causes of 201 918 dialysis patients with the general population. Dialysis was associated with increased mortality risks of bleeding and arterial thrombosis. Clinicians should be aware of the increased bleeding and thrombosis risks. SUMMARY: Background Dialysis has been associated with both bleeding and thrombotic events. However, there is limited information on bleeding as a cause of death versus arterial thrombosis as a cause of death. Objectives To investigate the occurrence of bleeding, myocardial infarction and stroke as causes of death in the dialysis population as compared with the general population. Methods We included 201 918 patients from 11 countries providing data to the ERA-EDTA Registry who started dialysis treatment between 1994 and 2011, and followed them for 3 years. Age-standardized and sex-standardized mortality rate ratios for bleeding, myocardial infarction and stroke as causes of death were calculated in dialysis patients as compared with the European general population. Associations between potential risk factors and these causes of death in dialysis patients were investigated by calculating hazard ratios (HRs) with 95% confidence intervals (CIs) by the use of Cox proportional-hazards regression. Results As compared with the general population, the age-standardized and sex-standardized mortality rate ratios in dialysis patients were 12.8 (95% CI 11.9-13.7) for bleeding as a cause of death (6.2 per 1000 person-years among dialysis patients versus 0.3 per 1000 person-years in the general population), 13.4 (95% CI 13.0-13.9) for myocardial infarction (22.5 versus 0.9 per 1000 person-years), and 12.4 (95% CI 11.9-12.9) for stroke (14.3 versus 0.7 per 1000 person-years). Conclusion Dialysis patients have highly increased risks of death caused by bleeding and arterial thrombosis as compared with the general population. Clinicians should be aware of the increased mortality risks caused by these conditions.


Asunto(s)
Hemorragia/mortalidad , Enfermedades Renales/terapia , Infarto del Miocardio/mortalidad , Diálisis Renal/efectos adversos , Accidente Cerebrovascular/mortalidad , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Causas de Muerte , Europa (Continente)/epidemiología , Femenino , Humanos , Enfermedades Renales/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Sistema de Registros , Medición de Riesgo , Factores de Riesgo , Distribución por Sexo , Factores de Tiempo
4.
Acta Anaesthesiol Scand ; 60(9): 1230-40, 2016 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-27378715

RESUMEN

BACKGROUND: Acute kidney injury (AKI) is a relatively common complication following CABG and is associated with adverse outcomes. Nonetheless, we hypothesized that the majority of patients make a good long-term recovery of their renal function. We studied the incidence and risk factors of AKI together with renal recovery and long-term survival in patients who developed AKI following CABG. METHODS: This nationwide study examined AKI among 1754 consecutive patients undergoing CABG in 2001-2013. AKI was defined according to the KDIGO criteria. RESULTS: Postoperatively 184 (11%) patients developed AKI; 121 (7%), 27 (2%), and 36 (2%) at stages 1, 2, and 3, respectively. AKI was an independent risk factor for chronic kidney disease (CKD) and AKI patients had worse post-operative outcomes. Lower pre-operative glomerular filtration rate, higher EuroSCORE and BMI, diabetes, reoperation, and units of red blood cells transfused were independent risk factors of AKI. At post-operative day 10, renal recovery rates, defined as serum creatinine ratio <1.25 of baseline, were 96 (95% CI 91-99%), 78 (95% CI 53-90%), and 94% (95% CI 77-98%) for AKI stages 1, 2, and 3, respectively. Long-term survival was predicted by AKI with 10-year survival of patients without AKI being 76% and those with AKI stages 1, 2, and 3 being 63%, 56%, and 49%, respectively (P < 0.001). CONCLUSION: Depending on the severity of the initial AKI, 78-97% of patients made good recovery of their kidney function. However, AKI was significantly linked to progression to CKD and long-term survival remained markedly affected by the severity of the initial kidney injury.


Asunto(s)
Lesión Renal Aguda/mortalidad , Puente de Arteria Coronaria/efectos adversos , Riñón/fisiopatología , Complicaciones Posoperatorias/mortalidad , Lesión Renal Aguda/epidemiología , Lesión Renal Aguda/fisiopatología , Anciano , Femenino , Tasa de Filtración Glomerular , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Recuperación de la Función , Estudios Retrospectivos , Factores de Riesgo
5.
J Nutr Sci ; 5: e10, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27066255

RESUMEN

Low circulating levels of total 25-hydroxyvitamin D (25(OH)D) have been associated with an increased risk of adverse effects after cardiac surgery. The metabolites, 25(OH)D2 and 25(OH)D3, provide a good index of vitamin D status. In this study, we examined the association between preoperative plasma levels of total 25(OH)D, 25(OH)D2 and 25(OH)D3 and the risk of postoperative atrial fibrillation (POAF) following open heart surgery. The levels of plasma 25(OH)D2 and 25(OH)D3 in 118 patients, who underwent coronary artery bypass grafting and/or valvular surgery, were measured immediately prior to surgery and on postoperative day 3 by liquid chromatography-tandem mass spectrometry. Patients who developed POAF had higher median plasma levels of 25(OH)D2 than those who remained in sinus rhythm (SR) (P = 0·003), but no significant difference was noted in levels of 25(OH)D3 or total 25(OH)D between the two groups (P > 0·05). By univariate analysis, patients with total 25(OH)D and 25(OH)D2 levels above the median had higher frequency of POAF (P < 0·05) and the incidence of POAF increased significantly with each higher quartile of preoperative plasma levels of 25(OH)D2 (P = 0·001), an association that was independent of confounding factors. In both the SR and POAF groups, the median plasma levels of 25(OH)D2, 25(OH)D3 and total 25(OH)D were lower (P < 0·05) on the third postoperative day compared with preoperatively. Our findings demonstrate that higher plasma levels of 25(OH)D2 are associated with increased risk of POAF, while this is not the case for 25(OH)D3 or total 25(OH)D. The reason for these discrepant results is not clear but warrants further study.

6.
Am J Transplant ; 14(11): 2623-32, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25307253

RESUMEN

Adenine phosphoribosyltransferase (APRT) deficiency is a rare autosomal recessive enzyme defect of purine metabolism that usually manifests as 2,8-dihydroxyadenine (2,8-DHA) nephrolithiasis and more rarely chronic kidney disease. The disease is most often misdiagnosed and can recur in the renal allograft. We analyzed nine patients with recurrent 2,8-DHA crystalline nephropathy, in all of whom the diagnosis had been missed prior to renal transplantation. The diagnosis was established at a median of 5 (range 1.5-312) weeks following the transplant procedure. Patients had delayed graft function (n=2), acute-on-chronic (n=5) or acute (n=1) allograft dysfunction, whereas one patient had normal graft function at the time of diagnosis. Analysis of allograft biopsies showed birefringent 2,8-DHA crystals in renal tubular lumens, within tubular epithelial cells and interstitium. Fourier transformed infrared microscopy confirmed the diagnosis in all cases, which was further supported by 2,8-DHA crystalluria, undetectable erythrocyte APRT enzyme activity, and genetic testing. With allopurinol therapy, the allograft function improved (n=7), remained stable (n=1) or worsened (n=1). At last follow-up, two patients had experienced allograft loss and five had persistent chronic allograft dysfunction. 2,8-DHA nephropathy is a rare but underdiagnosed and preventable disorder that can recur in the renal allograft and may lead to allograft loss.


Asunto(s)
Adenina Fosforribosiltransferasa/deficiencia , Rechazo de Injerto , Trasplante de Riñón , Errores Innatos del Metabolismo/etiología , Urolitiasis/etiología , Adulto , Anciano , Femenino , Humanos , Masculino , Errores Innatos del Metabolismo/fisiopatología , Persona de Mediana Edad , Recurrencia , Urolitiasis/fisiopatología
7.
Eur J Clin Nutr ; 68(1): 114-8, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-24169465

RESUMEN

BACKGROUND/OBJECTIVES: Randomised controlled trials (RCTs) evaluating the effect of fish oil supplementation on postoperative atrial fibrillation (POAF) following cardiac surgery have produced mixed results. In this study, we examined relationships between levels of red blood cell (RBC) n-3 long-chain polyunsaturated fatty acids (LC-PUFAs) and the incidence of POAF. SUBJECTS/METHODS: We used combined data (n=355) from RCTs conducted in Australia and Iceland. The primary end point was defined as POAF lasting >10 min in the first 6 days following surgery. The odds ratios (ORs) for POAF were compared between quintiles of preoperative RBC n-3 LC-PUFA levels by multivariable logistic regression. RESULTS: Subjects with RBC docosahexaenoic acid (DHA) in the fourth quintile, comprising a RBC DHA range of 7.0-7.9%, had the lowest incidence of POAF. Subjects in the lowest and highest quintiles had significantly higher risk of developing POAF compared with those in the fourth quintile (OR=2.36: 95% CI; 1.07-5.24 and OR=2.45: 95% CI; 1.16-5.17, respectively). There was no association between RBC eicosapentaenoic acid levels and POAF incidence. CONCLUSIONS: The results suggest a 'U-shaped' relationship between RBC DHA levels and POAF incidence. The possibility of increased risk of POAF at high levels of DHA suggests an upper limit for n-3 LC-PUFAs in certain conditions.


Asunto(s)
Fibrilación Atrial/sangre , Fibrilación Atrial/epidemiología , Procedimientos Quirúrgicos Cardíacos , Ácidos Docosahexaenoicos/efectos adversos , Ácidos Docosahexaenoicos/sangre , Adolescente , Adulto , Australia/epidemiología , Suplementos Dietéticos , Ácidos Docosahexaenoicos/administración & dosificación , Ácido Eicosapentaenoico/administración & dosificación , Ácido Eicosapentaenoico/efectos adversos , Ácido Eicosapentaenoico/sangre , Femenino , Aceites de Pescado/administración & dosificación , Humanos , Islandia/epidemiología , Incidencia , Modelos Logísticos , Masculino , Análisis Multivariante , Oportunidad Relativa , Cuidados Posoperatorios , Ensayos Clínicos Controlados Aleatorios como Asunto , Adulto Joven
8.
Artículo en Inglés | MEDLINE | ID: mdl-23999253

RESUMEN

BACKGROUND: Open heart surgery is associated with a systemic inflammatory response. The n-3 long-chain polyunsaturated fatty acids (LC-PUFA), eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA), and the n-6 LC-PUFA arachidonic acid (AA) may contribute to modulation of the inflammatory response. OBJECTIVE: We investigated whether the preoperative levels of EPA, DHA and AA in plasma phospholipids (PL) and red blood cell (RBC) membrane lipids in patients (n=168) undergoing open heart surgery were associated with changes in the plasma concentration of selected inflammatory mediators in the immediate postoperative period. RESULTS AND CONCLUSIONS: The postoperative concentration of TNF-ß was lower (P<0.05) and those of hs-CRP, IL-6, IL-8, IL-18 and IL-10 higher (P<0.05) than the respective preoperative concentrations. We observed that the preoperative levels of EPA and AA in plasma PL and RBC membrane lipids were associated with changes in the concentration of pro-inflammatory and anti-inflammatory mediators, suggesting a complex role in the postoperative inflammatory process.


Asunto(s)
Ácido Araquidónico/sangre , Membrana Celular/metabolismo , Quimiocinas/sangre , Ácidos Docosahexaenoicos/sangre , Ácido Eicosapentaenoico/sangre , Eritrocitos/metabolismo , Adulto , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/prevención & control , Procedimientos Quirúrgicos Cardíacos , Método Doble Ciego , Ácidos Grasos Omega-3/administración & dosificación , Femenino , Cardiopatías/sangre , Cardiopatías/inmunología , Cardiopatías/cirugía , Humanos , Masculino , Persona de Mediana Edad , Fosfolípidos/sangre , Complicaciones Posoperatorias/prevención & control , Periodo Posoperatorio , Periodo Preoperatorio , Ensayos Clínicos Controlados Aleatorios como Asunto , Resultado del Tratamiento
9.
J Thromb Haemost ; 10(12): 2484-93, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-22970891

RESUMEN

BACKGROUND: It is has been suggested that dialysis patients have lower mortality rates for pulmonary embolism than the general population, because of platelet dysfunction and bleeding tendency. However, there is limited information whether dialysis is indeed associated with a decreased mortality risk from pulmonary embolism. OBJECTIVE: The aim of our study was to evaluate whether mortality rate ratios for pulmonary embolism were lower than for myocardial infarction and stroke in dialysis patients compared with the general population. METHODS: Cardiovascular causes of death for 130,439 incident dialysis patients registered in the ERA-EDTA Registry were compared with the cardiovascular causes of death for the European general population. RESULTS: The age- and sex-standardized mortality rate (SMR) from pulmonary embolism was 12.2 (95% CI 10.2-14.6) times higher in dialysis patients than in the general population. The SMRs in dialysis patients compared with the general population were 11.0 (95% CI 10.6-11.4) for myocardial infarction, 8.4 (95% CI 8.0-8.8) for stroke, and 8.3 (95% CI 8.0-8.5) for other cardiovascular diseases. In dialysis patients, primary kidney disease due to diabetes was associated with an increased mortality risk due to pulmonary embolism (HR 1.9; 95% CI 1.0-3.8), myocardial infarction (HR 4.1; 95% CI 3.4-4.9), stroke (HR 3.5; 95% CI 2.8-4.4), and other cardiovascular causes of death (HR 3.4; 95% CI 2.9-3.9) compared with patients with polycystic kidney disease. CONCLUSIONS: Dialysis patients were found to have an unexpected highly increased mortality rate for pulmonary embolism and increased mortality rates for myocardial infarction and stroke.


Asunto(s)
Infarto del Miocardio/mortalidad , Embolia Pulmonar/mortalidad , Diálisis Renal , Accidente Cerebrovascular/mortalidad , Estudios de Cohortes , Femenino , Humanos , Masculino
10.
Aging Cell ; 10(2): 233-8, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21108732

RESUMEN

The most frequently used model to describe the exponential increase in mortality rate over age is the Gompertz equation. Logarithmically transformed, the equation conforms to a straight line, of which the slope has been interpreted as the rate of senescence. Earlier, we proposed the derivative function of the Gompertz equation as a superior descriptor of senescence rate. Here, we tested both measures of the rate of senescence in a population of patients with end-stage renal disease. It is clinical dogma that patients on dialysis experience accelerated senescence, whereas those with a functional kidney transplant have mortality rates comparable to the general population. Therefore, we calculated the age-specific mortality rates for European patients on dialysis (n=274 221; follow-up=594 767 person-years), for European patients with a functioning kidney transplant (n=61 286; follow-up=345 024 person-years), and for the general European population. We found higher mortality rates, but a smaller slope of logarithmic mortality curve for patients on dialysis compared with both patients with a functioning kidney transplant and the general population (P<0.001). A classical interpretation of the Gompertz model would imply that the rate of senescence in patients on dialysis is lower than in patients with a functioning transplant and lower than in the general population. In contrast, the derivative function of the Gompertz equation yielded the highest senescence rates for patients on dialysis, whereas the rate was similar in patients with a functioning transplant and the general population. We conclude that the rate of senescence is better described by the derivative function of the Gompertz equation.


Asunto(s)
Envejecimiento/fisiología , Fallo Renal Crónico/mortalidad , Modelos Teóricos , Mortalidad , Adulto , Anciano , Anciano de 80 o más Años , Europa (Continente) , Humanos , Fallo Renal Crónico/fisiopatología , Fallo Renal Crónico/terapia , Persona de Mediana Edad , Sistema de Registros , Adulto Joven
11.
Eur J Intern Med ; 21(2): e1-6, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20206862

RESUMEN

The European Board of Internal Medicine is working towards enhancing the training in Internal Medicine in Europe. One of the most important tasks is to ensure that training programmes reach an acceptable level of quality. The Board does not accredit training centres as this is the responsibility of national authorities. The purpose of this paper is to provide guidance for the accreditation process. The content of the paper has been developed from a publication on medical education produced by the World Federation for Medical Education. Basic standards which should be met by all training centres are outlined. Quality development describes standards which centres should aim for although progress will to some extent be influenced by resources, stage of development and local circumstances.


Asunto(s)
Medicina Interna/educación , Acreditación/organización & administración , Acreditación/normas , Educación de Postgrado en Medicina/organización & administración , Educación de Postgrado en Medicina/normas , Evaluación Educacional/normas , Europa (Continente) , Consejo Directivo , Medicina Interna/normas
12.
Clin Nephrol ; 65(1): 34-42, 2006 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-16429840

RESUMEN

BACKGROUND: Several types of replacement fluid and methods of anticoagulation have been employed for continuous renal replacement therapy, but there is no consensus on a preferred approach. We evaluated the indications for the selection of replacement fluid and anticoagulant among critically ill patients receiving continuous venovenous hemofiltration (CVVH) and assessed the effect of the selection on the efficacy of anticoagulation and complications. METHODS: We retrospectively studied 29 consecutive patients who received CVVH in the Medical Intensive Care Unit at Massachusetts General Hospital. There were 3 types of replacement solution available, an isotonic citrate solution which was also used for regional anticoagulation of the extracorporeal circuit, and bicarbonate and lactate solutions which were used with low-dose heparin or no anticoagulant. Blood flow rate was set at 120 ml/min when citrate replacement fluid was used and at 200 ml/min with bicarbonate or lactate. The replacement fluid was administered proximal to the hemofilter at a constant rate of 1,600 ml/h. RESULTS: There were 22 patients who received citrate replacement fluid which was mainly chosen for the purpose of anticoagulation in the setting of contraindications to heparin. 12 patients received bicarbonate, predominantly when citrate was considered contraindicated due to liver failure or high-anion gap metabolic acidosis, and 2 received lactate; 8 of these 14 patients were anticoagulated with heparin and 6 were managed without anticoagulation. There were 44 filters used in the patients receiving citrate with a median filter life of 42.0 (interquartile range 22.2 - 70.7) hours. Only 8 of the 44 filters were lost due to clotting. Heparin was used for anticoagulation of 17 filters and no anticoagulation was used in the case of 15 filters, resulting in a median filter life of 43.0 (13.5 - 75.0) and 12.0 (4.0 - 33.0) hours, respectively. Clinically significant bleeding occurred in 2 patients, 1 receiving citrate and another receiving heparin. No patient had evidence for citrate toxicity, metabolic alkalosis or hypernatremia. 14 (48.3%) patients survived. CONCLUSIONS: The use of regional citrate anticoagulation of the CVVH circuit appears advantageous in patients with increased risk of bleeding and bicarbonate-based replacement fluid seems desirable in patients with lactic acidosis due to shock and/or severe liver failure. Tailoring the type of replacement fluid and method of anticoagulation to the individual patient leads to long filter lives, excellent metabolic control and minimal complications.


Asunto(s)
Anticoagulantes , Ácido Cítrico , Hemofiltración , Lactatos , Bicarbonatos , Femenino , Humanos , Soluciones Isotónicas , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Soluciones
13.
Diabet Med ; 22(2): 182-7, 2005 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-15660736

RESUMEN

AIMS: Diabetic nephropathy is an uncommon cause of end-stage renal disease in Iceland in contrast to most industrialized countries. The aim of this study was to examine the incidence of diabetic nephropathy in Iceland. METHODS: All patients diagnosed with Type 1 diabetes in Iceland before 1992 were studied retrospectively. Patients diagnosed before age 30, who were insulin dependent from the onset, were defined as having Type 1 diabetes. Diabetic nephropathy was defined as persistent proteinuria measured with a dipstick test (Albustix) on three consecutive clinic visits at least 2 months apart. Patients were followed to the end of year 1998, to their last recorded outpatient visit, or until death. The cumulative incidence of diabetic nephropathy was calculated with the Kaplan-Meier method and presented according to the duration of diabetes divided into 5-year intervals. RESULTS: A total of 343 patients with Type 1 diabetes were identified. The mean follow-up period was 20.2 +/- 11.4 (mean +/- sd) years. Only 9.3% of patients were lost to follow-up. Sixty-five patients developed diabetic nephropathy. The cumulative incidence was 22.6% at 20 years and levelled off at 40.3% after approximately 35 years of diabetes duration. No significant changes in cumulative incidence were observed over time. Mean glycated haemoglobin was 8.4% in patients with proteinuria and 7.8% in a group of patients without proteinuria that was matched for age, gender and duration of diabetes (P = 0.04). CONCLUSIONS: The cumulative incidence of diabetic nephropathy in Iceland is comparable with previously reported cumulative incidence rates and has remained unchanged. Glycaemic control was significantly better in patients without proteinuria.


Asunto(s)
Diabetes Mellitus Tipo 1/epidemiología , Nefropatías Diabéticas/epidemiología , Fallo Renal Crónico/epidemiología , Adolescente , Adulto , Edad de Inicio , Anciano , Niño , Femenino , Humanos , Islandia/epidemiología , Incidencia , Masculino , Persona de Mediana Edad , Proteinuria/epidemiología
15.
Am J Kidney Dis ; 38(3): 473-80, 2001 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-11532677

RESUMEN

The purpose of this study was to characterize the clinical, diagnostic, and prognostic features of adenine phosphoribosyltransferase (APRT) deficiency in Icelandic patients, as well as determine their genotype. Medical records of all known patients in Iceland were reviewed. Urinalysis and polymerase chain reaction-based DNA mutation analysis were performed in all patients, siblings, and living parents of index cases. Twenty-three individuals homozygous for type I APRT deficiency were identified in 16 families from 1983 to 1998. There were 12 males and 11 females, and the median age at diagnosis was 37 years (range, 0.5 to 62 years). Seventeen patients were index cases and 6 patients were diagnosed during screening of first-degree relatives. Eighteen patients had symptomatic disease, 15 of whom experienced nephrolithiasis; 4 patients had mild to moderate renal insufficiency, 1 patient had advanced renal failure, and 1 patient died of uremic complications. Six patients experienced recurrent urinary tract infections and 3 infants had a history of reddish-brown diaper stains. Five patients were asymptomatic; 3 of these patients were diagnosed during routine urinalysis and 2 patients were identified during family screening. Urinary 2,8-dihydroxyadenine crystals were detected in all cases, except for the patient who died of end-stage renal failure. All 23 patients were homozygous for the same mutation (D65V) in the APRT gene. Allopurinol therapy successfully prevented further stone formation and significantly improved renal function in most patients with renal insufficiency. Our results suggest that APRT deficiency may be more common than previously recognized and can lead to severe renal failure if left untreated.


Asunto(s)
Adenina Fosforribosiltransferasa/deficiencia , Adenina Fosforribosiltransferasa/genética , Adenina/análogos & derivados , Adenina/orina , Cálculos Renales/etiología , Lesión Renal Aguda/etiología , Lesión Renal Aguda/patología , Adolescente , Adulto , Biopsia , Niño , Preescolar , Análisis Mutacional de ADN , Femenino , Genotipo , Humanos , Islandia , Lactante , Riñón/patología , Fallo Renal Crónico/etiología , Fallo Renal Crónico/patología , Masculino , Persona de Mediana Edad
16.
Eur J Gastroenterol Hepatol ; 13(4): 433-6, 2001 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-11338076

RESUMEN

Wilson's disease, an autosomal recessive disorder of copper transport, usually presents with symptoms from the liver or central nervous system. Rarely, the initial manifestation is fulminant hepatic failure. The abnormal gene (ATP7B) is located on chromosome 13q and encodes a copper-transporting ATPase. A large number of mutations have been reported. We describe a previously healthy 16-year-old girl who presented with fulminant hepatic failure. The girl died within 24 h of admission to a hospital from refractory shock. Autopsy revealed cirrhosis and widespread necrosis of the liver. The copper content of the liver was markedly increased (975 micrograms/g dry weight), strongly suggesting a diagnosis of Wilson's disease. Genetic studies revealed that the girl was homozygous for the mutation 2007 del7, which is the mutation found in all Wilson's disease patients previously identified in Iceland. This is the first known case of fulminant hepatic failure due to Wilson's disease in Iceland. Despite the same mutation, the clinical picture is vastly different from other Icelandic patients with Wilson's disease, who all presented with relatively late-onset neurological disease. This suggests that factors other than the specific mutation have significant impact on the phenotype of the disease.


Asunto(s)
Deleción Cromosómica , Mutación del Sistema de Lectura , Degeneración Hepatolenticular/genética , Adolescente , Resultado Fatal , Femenino , Genotipo , Humanos , Islandia , Fenotipo
17.
Laeknabladid ; 86(9): 557-65, 2000 Sep.
Artículo en Islandés | MEDLINE | ID: mdl-17018945

RESUMEN

During the past 40 years, solid-organ transplantation has evolved into a routine clinical procedure for the management of end-stage heart, kidney, liver and lung disease as well as diabetes mellitus. This has mainly been accomplished through advances in understanding the molecular mechanisms involved in the rejection of allografts which has led to major improvements in immunosuppressive therapy. The discovery of the immunosuppressive drug cyclosporine which came into clinical use in the early eighties, revolutionized the field of transplantation. The short-term survival of allografts is now excellent but relentless loss of grafts over time due to chronic rejection remains a major problem. A number of complications can affect transplant recipients, most of which result from intensive immunosuppressive treatment. Among those are life-threatening infections and malignancies. The key issues concerning long-term management of transplant recipients are discussed.

18.
Laeknabladid ; 86(9): 571-6, 2000 Sep.
Artículo en Islandés | MEDLINE | ID: mdl-17018947

RESUMEN

Renal transplantation is the treatment of choice for most patients with end-stage renal disease. The improved success of this treatment modality over the past four decades can large part be attributed to advances in immunosuppressive therapy. However, while the demand for renal transplantation has been steadily growing due to the rising incidence of end-stage renal disease, shortage of organ donors is a major limitation. The shortage of kidneys for transplantation has been met with an increase in the use of living donors. Renal allograft survival has improved over the years, although late graft loss is still a significant problem. One and five-year survival of living donor grafts is approximately 94% and 72%, and 88% and 60% for cadaveric donor grafts, respectively. The main causes of late graft loss are death of the patient and chronic allograft nephropathy. Risk factors for chronic allograft nephropathy are complex and include both immunlogic and non-immunologic mechanisms. Finally, the results of renal transplantation in Icelandic recipients are discussed.

19.
Kidney Int ; 55(5): 1991-7, 1999 May.
Artículo en Inglés | MEDLINE | ID: mdl-10231464

RESUMEN

BACKGROUND: Systemic heparinization is associated with a high rate of bleeding when used to maintain patency of the extracorporeal circuit during continuous renal replacement therapy (CRRT) in critically ill patients. Regional anticoagulation can be achieved with citrate, but previously described techniques are cumbersome and associated with metabolic complications. METHODS: We designed a simplified system for delivering regional citrate anticoagulation during continuous venovenous hemofiltration (CVVH). We evaluated filter life and hemorrhagic complications in the first 17 consecutive patients who received this therapy at our institution. Blood flow rate was set at 180 ml/min. Ultrafiltration rate was maintained at 2.0 liters/hr and citrate-based replacement fluid (trisodium citrate 13.3 mM, sodium chloride 100 mM, magnesium chloride 0.75 mM, dextrose 0.2%) was infused proximal to the filter to maintain the desired fluid balance. Calcium gluconate was infused through a separate line to maintain a serum-ionized calcium level of 1.0 to 1.1 mM. RESULTS: All patients were critically ill and required mechanical ventilation and vasopressor therapy. Systemic heparin anticoagulation was judged to be contraindicated in all of the patients. A total of 85 filters were used, of which 64 were lost because of clotting, with a mean life span of 29.5 +/- 17.9 hours. The remaining 21 filters were discontinued for other reasons. Control of fluid and electrolyte balance and azotemia was excellent (mean serum creatinine after 48 to 72 hr of treatment was 2.4 +/- 1.2 mg/dl). No bleeding episodes occurred. Two patients, one with septic shock and the other with fulminant hepatic failure, developed evidence for citrate toxicity without a significant alteration in clinical status. Nine patients survived (52.9%). CONCLUSION: Our simplified technique of regional anticoagulation with citrate is an effective and safe form of anticoagulation for CVVH in critically ill patients with a high risk of bleeding.


Asunto(s)
Anticoagulantes/administración & dosificación , Ácido Cítrico/administración & dosificación , Hemofiltración/métodos , Hemorragia/prevención & control , Fallo Renal Crónico/tratamiento farmacológico , Adulto , Anciano , Femenino , Hemorragia/inducido químicamente , Humanos , Fallo Renal Crónico/mortalidad , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del Tratamiento
20.
Laeknabladid ; 85(1): 9-24, 1999 Jan.
Artículo en Islandés | MEDLINE | ID: mdl-19321912

RESUMEN

OBJECTIVE: Renal replacement therapy for end-stage renal disease (ESRD) jas been provided in Iceland since 1968 when hemodialysis was begun. Kidney transplantation in Iceland patients has benn performed abroad since 1970 mainly in Copenhagen, Gothenburg and Boston. The purpose of theis retrospective study was to determine the changes in incidencs, prevalence, and outcome of ESRD treatment during the period 1968-1997 and compare the results with other ESRD programs, mainly in the Nordic countries. MATERIAL AND METHODS: Included in this study were all patients who began renal replacement therapy for ESRD during the study period and remained on therapy for at least six weeks. Data were obtained from the registry of ESRD, compiled by the Dialysis Service of the National University Hospital. The data were used to determine the annual incidence and prevalence of treated ESRD. Changes in parameters, such as age at the beginning of renal replacement therapy, gender distribution, causes of ESRD, treatment modalities, and survival were evaluated. Annual mortality rate was calculated as deaths per 100 life-years. Comparison of means was done by the twö sample t-test, survival was estimated by the Kaplan-Meier method and survival differences weere determined with the Mantel-Cox test. RESULTS: A total of 201 patients began therapy for ESRD during this 30 year period. The number of patients beginning renal replacement therapy in each of the three consecutive decades was 27, 59 and 115, respectively, which corresponds to 12.8, 25.1 and 44 per million population per year. The mean age rose throughout hte study period nad was 54.8 in the final decade. The prevalence per million population was 72 in 1977, 182 in 1987 nad 356 in 1997. Diabetic nephropathy was not observed as a cause of ESRD until the last decade when it accounted for 12% of new patiens. Hemodialysis was the sole dialysis modality undtil 1985. Peritoneal dialysis has since provided approximately one third of the dialysis treatment. The number of renal transplants was 13, 30 and 58 for each decade, respectively. At the end of 1997 htere were 59 functioning allografts and of these 45 were from living donors. Patients with a functioning allograft were 70% of all ESRD patients at the end of 1997. Allografts came predominantly from cadveric donors during the first two decades but living donors were 65% in the final decade. The five year survival of transplanted patients (81%) was markedly superior to that of dialyzed patients (16%). The annual mortality rate declined for the whole period, during the last decade it was 10.7 per 100 life-years for all patients, 27.9 for hemodialysis patients, 15.3 for peritonial dialysis patients and 2.1 for transplanted patients. Death was mainly from cardiovascular causes and infections. CONCLUSIONS: There has been marked increase in the incidence and prevalence of treated ESRD in Iceland during the last 30 years. However, the incidence is low compared to the other Nordic countries, mainly as a rresult of low incidence of ESRD due to glomerulonephritis and diabetic nephropathy. Nearly half the ESRD population has recieved a renal transplant. Only Norway has a higher prevalence of transplanted patients among the ESRD pool. The percentage of living donor grafts among the transplanted patients is the highest the auhtors are aware of. Five year patient survival and renal allograft survival in Iceland were comparable to other countries.

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