Analysis of Kidney Donation and Its Relationship With Graft Failure of the Recipient at 1 Year.

INTRODUCTION: Currently, the shortage of organs available for kidney transplantation and a change in donors' and recipients' profiles (elderly, with cardiovascular risk, donors after cardiac death), it is becoming necessary to assess grafts from expanded-criteria donors (ECD) in order to have methods that allow us to predict viability and graft survival. OBJECTIVE: The aim of this study was to analyze the different methods of renal donor assessment (estimated glomerular filtration rate [eGFR], preimplantation biopsy, and Kidney Donor Profile Index [KDPI] score) as predictors of graft survival and renal function of our recipient at 1 year. METHODS: We performed a descriptive and retrospective study of 183 deceased donor kidney transplantations performed at our center between 2011 and 2015. We calculated the KDPI scores, donor eGFR was estimated using the Chronic Kidney Disease Epidemiology Collaboration Formula equation, and biopsies were evaluated using Banff classification. RESULTS: ECDs comprised 59.60%, 93% of donors had an eGFR ≥ 60 mL/min/1.73 m2, and 41% presented with a KDPI score ≥ 90%. The most frequent range in the biopsy score was 0-3. The 1-year graft survival rate was 86.90%. Factors that negatively influenced graft survival were donor/recipient age, ECD, KDPI, and cold ischemia time (CIT). CONCLUSION: Prolonged CIT and KDPI ≥ 90% were donor variables that were related to graft failure at 1 year in our center.

Anti-HLA Antibodies After Precocious Transplantectomy by Vascular Thrombosis.

BACKGROUND: Our objective in this study was to determine the effects of early renal transplantectomy on patients and the production of anti-human leukocyte antigen (anti-HLA) antibodies. METHODS: Between January 2003 and May 2017, we analyzed a group of patients for the presence of specific HLA class I and/or II donor-specific antibodies (DSA), their panel-reactive antibodies (PRA), and the time period in which the antibodies were still detectable after transplantectomy. RESULTS: Anti-HLA antibodies were detected in 60.8% of patients, 60.8% and 52.2% of those patients had anti-class I and anti-class II antibodies, respectively. DSA were detected in 91.7% of the anti-HLA class I patients. Class II DSA were detected all of the patients with anti-HLA class II antibodies. The average (mean ± SD) PRA levels in our patients after transplantectomy was 60 ± 34% in class I and 63 ± 36% in class II. CONCLUSION: Anti-HLA antibodies can be detected well after transplantectomy. Even if the kidney allograft had been transplanted for only a short time, when the intensity of immunosuppression was the highest, many patients developed anti-HLA antibodies. The patients who continued with immunosuppression after transplantectomy did not develop anti-HLA antibodies.

Neuropatía óptica tóxica inducida por Linezolid / Linezolid induced toxic optic neuropathy

El Linezolid es un antimicrobiano sintético que pertenece a un nuevo grupo, elde las oxazolidinonas, aprobado por la Foods and DrugsAdministration (FDA) para su uso en infecciones por microorganismos gram positivos, staphylococcusspp y enterococcusspp, resistentes a la oxacilina y a la vancomicina.Dentro de sus usos principales están enfermedades como neumonías nosocomiales, infecciones de piel y tejidos blandos. Se describe el caso de una paciente con neuropatía óptica asociada al linezolid. La mayoría de estos casos han sido documentados en pacientes que recibieron una terapia superior a los 28 días recomendados.Los médicos debemos estar atentos a diferentes efectos adversos asociados con el linezolid como la neuropatía óptica, aunque es mayor el riesgo de leucopenia, trombocitopenia y neuropatía periférica, asociados a este medicamento (AU)

Linezolid is a new class of synthetic antimicrobial, belonging to the family of oxazolidinones approved by the Food and Drugs Administration for use in gram positive infections by staphylococcus spp and enterococcus spp, resistant tooxacillin and vancomycin. Among its mains uses are diseases such as nosocomial pneumonia, kin infection and soft tissue We describe the case of a patient with optic neuropathy associated with linezolid, most of these cases have been documented in patients who were in therapy at 28 days than recommended, physicians shouldbe alert to various adverse effects associated with linezolid such as optic neuropathy, but there is anincreased risk of leucopenia, thrombocytopenia and peripheral neuropathy associated with this medicine (AU)

Efficacy of dialysis in peritoneal dialysis: utility of bioimpedance to calculate Kt/V and the search for a target Kt.

BACKGROUND: To calculate Kt/V, volume (V) is usually obtained by Watson formula, but bioimpedance spectroscopy (BIS) is a simple and applicable technique to determinate V, along with other hydration and nutrition parameters, in peritoneal dialysis (PD) patients. Dialysis efficacy can also be measured with Kt, but no experience exists in PD, so there is no reference/target value for Kt that must be achieved in these patients to be considered adequately dialyzed. We evaluated the efficacy of PD with Kt/V using Watson formula and BIS for V calculation, assessed hydration status in a PD unit by data obtained by BIS, and attempted to find a reference Kt from the Kt/V previously obtained by BIS. METHODS: In this observational prospective study of 78 PD patients, we measured V using BIS (V bis) and Watson formula (V w) and calculated weekly Kt/V using both volumes (Kt/V bis/V bis and Kt/V w). With the BIS technique, we obtained and subsequently analyzed other hydration status parameters. We achieved a reference Kt, extrapolating the value desired (weekly Kt/V 1.7) to the target Kt using the simple linear regression statistical technique, basing it on the results of the previously calculated Pearson's linear correlation coefficient. RESULTS: Volume was 1.8 l higher by Watson formula than with BIS (p < 0.001). Weekly Kt/V bis was 2.33 ± 0.68, and mean weekly Kt/V w was 2.20 ± 0.63 (p < 0.0001); 60.25 % of patients presented overhydration according to the BIS study (OH >1.1 l). The target value of Kt for the reference weekly Kt/V bis (1.7) was 64.87 l. CONCLUSIONS: BIS is a simple, applicable technique for calculating V in dialysis that can be especially useful in PD patients compared with the anthropometric formulas, by the abnormally distributed body water in these patients. Other parameters obtained by BIS will serve to assess both the distribution of body volume and nutritional status in the clinical setting. The target Kt value obtained from Kt/V bis allowed us to measure the efficacy of PD in a practical way, omitting V measurement.

Impact of peritonitis on long-term survival of peritoneal dialysis patients.

UNLABELLED: The impact of each episode of peritonitis on long-term survival of peritoneal dialysis (PD) patients has yet to be defined. OBJECTIVES: To determine the risk that each episode of peritonitis poses for patient survival and for the PD technique. PATIENTS: 1515 patients included in the Levante registry from 1 January 1993 to 31 December 2005. METHODS: Retrospective analysis of a multicentre registry using Cox regression for time-dependent variables. RESULTS: We analysed 1609 episodes of peritonitis in 716 patients (47.2%). In the univariate analysis, each case of peritonitis treated in the outpatient unit was associated with an increase in mortality (hazard ratio [HR] 1.99, P<.001), which was greater for episodes that required hospitalisation (HR 3.62, P<.001). Mortality increased with each successive episode in the same patient. Multivariate analysis confirmed the association of each case of peritonitis with lower long-term survival (HR 2.01, P<.001), with a different risk for episodes due to gram-positive and gram-negative bacteria and fungi (HR 1.73, 2.43 and 5.71, respectively; P<.001). Other variables associated with mortality were age, low residual renal function, absence of vascular access and comorbidity. Peritonitis was the only independent variable associated with technique failure (HR 1.29, P<.001), with a different risk for episodes due to gram-positive and gram-negative bacteria and fungi (HR 1.73, 2.43 and 5.71, respectively; P<.001). CONCLUSIONS: Episodes of peritonitis negatively influence long-term survival of patients on PD.

[Short cold ischaemia time optimises transplant results for kidneys from expanded criteria donors]. / El tiempo de isquemia fría corto optimiza los resultados de los trasplantes renales efectuados con donantes con criterios expandidos.

INTRODUCTION: Outcome of renal transplant from expanded criteria donors (ECD) is usually inferior than those from standard criteria donors (SCD) and may be improved decreasing cold ischemia time (CIT) and minimizing preservation injury. We compare the results obtained with CIT <15 hours in kidney transplants from ECD vs SCD. SUBJECTS AND METHODS: Prospective, single center study of kidney transplants performed since June 2003 to December 2007. Minimum follow-up period was 12 months. Data of donors, receptors and transplant outcome from ECD and SCD are compared. RESULTS: CIT (mean +/- SD) was 9.3+/-2.5 hours in transplants from ECD (n=24) and 8.3+/-3.3 hours in those from SCD (N=50), p=0.18. We did not find significant differences among recipients of grafts from ECD and those from SCD regarding: primary non-function (4.2% vs 2%, respectively), delayed graft function (16.7% vs 10%), surgical complications (25% vs 16%) or acute rejection episodes (8.3% vs 2%). Glomerular filtration rate at one year follow-up was 65.8+/-14.9 ml/min in ECD recipients and 49.4+/-12.5 ml/min (p<0.0001). One year graft survival was 95.8% in ECD recipients and 94% in SCD recipients (p=0.75). CONCLUSIONS: Short CIT in kidney transplant from ECD leads to similar outcome than that obtained from SCD, although renal function is inferior in ECD grafts.

El tiempo de isquemia fría corto optimiza los resultados de los trasplantes renales efectuados con donantes con criterios expandidos / No disponible

Introducción: Los resultados de los trasplantes efectuados condonantes con criterios expandidos (DCE) son inferiores a los obtenidos con donantes con criterios estándar (DCS). Para optimizar su evolución, se podría reducir su tiempo de isquemiafría (TIF) reduciendo su daño de preservación. Comparamoslos resultados obtenidos al aplicar TIF <15 horas tanto a DCE como a DCS. Material y métodos: Realizamos un estudio unicéntrico, de cohortes, prospectivo, de casos incidentes de trasplante renal de cadáver entre junio de 2003 y diciembre de2007. El tiempo mínimo de seguimiento fue de 12 meses. Comparamos los datos de los donantes, de los receptores y de la evolución de los trasplantes efectuados con DCE frente a los de los DCS. Resultados: El TIF para los DCE (N = 24) y para los DCS (N = 50) fue, respectivamente, de 9,3 ± 2,5 y 8,3± 3,3 horas (p = 0,18). No encontramos diferencias significativas entre los receptores de DCE y DCS en cuanto a: no función primaria del injerto 4,2 vs. 4%, retardo en la función del injerto 16,7 vs. 10%, complicaciones quirúrgicas 25 vs. 16% y rechazos agudos 8,3 vs. 2%. El filtrado glomerular estimado al año para los DCS fue de 65,8 ± 14,9 ml/min y para los DCE de 49,4 ± 12,5 ml/min (p <0,0001). La supervivencia renal al año fue del 95,8% para los receptores de DCE y del 94% para los DCS (p = 0,75). Conclusiones: La aplicación de TIF cortos a los DCE permite conseguir una evolución similar a la de los DCS, aunque su función renal sea en todo momento inferior (AU)

Introduction: Outcome of renal transplant from expanded criteria donors (ECD) is usually inferior than those from standard criteria donors (SCD) and may be improved decreasing cold ischemia time (CIT) and minimizing preservation injury. We compare the results obtained with CIT <15 hours in kidney transplants from ECD vs. SCD. Subjects and Methods: Prospective, single center study of kidney transplants performed since June 2003 to December 2007. Minimum follow-up period was 12months. Data of donors, receptors and transplant outcome from ECD and SCD are compared. Results: CIT (mean ± SD)was 9.3 ± 2.5 hours in transplants from ECD (n = 24) and8.3 ± 3.3 hours in those from SCD (N = 50), p = 0.18. We did not find significant differences among recipients of grafts from ECD and those from SCD regarding: primary non-function (4.2% vs. 2%, respectively), delayed graft function (16.7% vs. 10%), surgical complications (25% vs.16%) or acute rejection episodes (8.3% vs. 2%).Glomerular filtration rate at one year follow-up was 65.8± 14.9 ml/min in ECD recipients and 49.4 ± 12.5 ml/min (p<0.0001). One year graft survival was 95.8% in ECD recipients and 94% in SCD recipients (p = 0.75).Conclusions: Short CIT in kidney transplant from ECD leads to similar outcome than that obtained from SCD, although renal function is inferior in ECD grafts (AU)

Conversion to tacrolimus extended-release formulation: short-term clinical results.

OBJECTIVE: To determine the short-term clinical results of conversion of treatment from tacrolimus twice daily (BID TAC) to the extended-release formulation (OD TAC), milligram for milligram, and whether such conversion is safe in stable kidney transplant recipients. PATIENTS AND METHODS: The study included 38 kidney transplant recipients (median [SD] age, 54.3 [14.4] years) with stable renal function (mean [SD] serum creatinine concentration 1.29 [0.38] mg/dL). Posttransplantation follow-up was 3.4 (3.1) years (range, 4-168 months). All patients had been receiving BID TAC (2.45 [1.52] mg/d) when treatment was converted to OD TAC, milligram for milligram. Follow-up including clinical evaluation and laboratory tests was at 7, 21, and 90 days postconversion. RESULTS: No significant differences were observed during follow-up in serum creatinine concentration, blood glucose level, hemoglobin level, or proteinuria. There were no episodes of acute rejection. No de novo posttransplantation diabetes mellitus was diagnosed; patients with diabetes required similar dosage of hypoglycemia treatment. Arterial pressure remained stable without changes in antihypertension treatment. Tacrolimus doses were not modified (2.45 [1.52] mg/d at baseline vs 2.45 [1.67] mg/d at 3 months postconversion; however, tacrolimus concentration decreased significantly (7.6 [1.8] ng/mL at baseline vs 6.42 [1.13] ng/mL at 3 months postconversion. Reduction in tacrolimus concentration was more remarkable in patients receiving a dose of less than 0.025 mg/kg/d. CONCLUSIONS: Conversion from BID TAC to OD TAC, milligram for milligram, is clinically safe; however, monitoring of tacrolimus concentration in patients receiving low dosage is mandatory to prevent subtherapeutic levels.

[Giant adrenal myelolipoma: hypertension, renal failure and spontaneous rupture]. / Mielolipoma suprarrenal gigante: hipertensión, insuficiencia renal y rotura espontánea.

Adrenal myelolipoma is a rare, benign, slow-growing tumor composed of adipose tissue and hematopoietic elements. It is usually diagnosed incidentally, although there are reports of patients with symptoms and descriptions of retroperitoneal hemorrhage due to rupture of large tumors. The condition has been associated with obesity, high blood pressure and adrenal dysfunction. We present a patient with retroperitoneal hemorrhage due to spontaneous rupture of a myelolipoma, hypertension, and renal failure secondary to nephroangiosclerosis.

Mielolipoma suprarrenal gigante: hipertensión, insuficiencia renal y rotura espontánea / Giant adrenal myelipoma: hypertension, renal failure and spontaneous rupture

El mielolipoma suprarrenal es un tumor raro, benigno, de lento crecimiento,compuesto por tejido graso y elementos hematopoyéticos. Suele diagnosticarse demanera incidental, aunque se han descrito casos de pacientes sintomáticos, e inclusohemorragias retroperitoneales por rotura en los de mayor tamaño. Se ha descritosu asociación con obesidad, HTA y disfunción suprarrenal. Presentamos unpaciente diagnosticado de mielolipoma, con hemorragia retroperitoneal espontáneapor rotura del mismo, e HTA, con insuficiencia renal secundaria a nefroangioesclerosis

Adrenal myelolipoma is a rare, benign, slow-growing tumor composed of adiposetissue and hematopoietic elements. It is usually diagnosed incidentally, althoughthere are reports of patients with symptoms and descriptions of retroperitonealhemorrhage due to rupture of large tumors. The condition has beenassociated with obesity, high blood pressure and adrenal dysfunction. We presenta patient with retroperitoneal hemorrhage due to spontaneous rupture of a myelolipoma,hypertension, and renal failure secondary to nephroangiosclerosis

[Meningoencephalitis by Listeria in the lupus disease]. / Meningoencefalitis por Listeria en el lupus.

We present a patient with lupus nephropathy of 20 years of evolution in treatment with oral steroids who developed a meningoencephalitis associated to bacteraemia by Listeria monocytogenes. The patient was treated successfully with gentamicin and ampicillin for 6 weeks. Infection by Listeria monocytogenes occurs more frequently in individuals with some form of immunodeficiency like lupus disease, with a mortality around 30%.

[Embolization of non-functioning renal allograft: efficacy and control of systemic inflammation]. / Embolización del injerto renal no funcionante: eficacia y control del estado inflamatorio crónico.

BACKGROUND: The graft intolerance syndrome (fever, pain, haematuria) may lead to a chronic inflammatory disease, with cardiovascular repercussion. Nephrectomy is considered the classical treatment of these cases but nowadays renal vascular embolization has been suggested as a possible alternative treatment. The present study concerns seven cases trated with renal vascular embolization in our hospital summarizing data of graft intolerance syndrome and the chronic inflammatory disease. MATERIAL AND METHODS: Between january 2000 and december 2003 seven renal vascular embolization were performed in nonfunctioning renal allograft. The procedure was made with calibrated particles of 300-500micron. Data about complications related to the technique the same as analitic inflammatory parameters before and after treatment (CRP, ferritin, serum albumin, hemoglobin, erythropoietin) were registered. RESULTS: The patients were two men and five women, with median age of 39,7 +/- 8,8 years. The period beween the dialysis and the embolization was of 10,0 +/-8,2 months. Comparing the perion of admission in the hospital due to nephrectomy which was of 17,86+/-4,41 days, the period because of embolization was shorter, being 8,14 +/- 4,53 days. All analitic parameters studied, clearly improved after embolization with decrease of ferritin and CRP, increase of albumin and better hemoglobin level with lower erythropoietin dose. In five of the seven patients there was no renal captation with CT or gammagraphy. Four patients presented a postembolization syndrome, but no other important complication was registered. Neither reembolization nor renal nephrectomy was neccesary in any of the seven cases. CONCLUSION: Percutaneous renal embolization is a simple, easy, safety and effective technique that must be considered as an alternative treatment to nephrectomy, resolving the chronic inflammatory disease secondary to the graft intolerance syndrome.

Meningoencefalitis por Listeria en el lupus / Meningoencephalitis by Listeria in the lupus disease

Presentamos un caso de una paciente con nefropatía lúpica de 20 años de evolución en tratamiento con esteroides que desarrolló una meningoencefalitis asociada a bacteriemia por Listeria monocytogenes. La paciente recibió tratamiento antibiótico con ampicilina y gentamicina durante 6 semanas con excelentes resultados. La infección por Listeria monocytogenes afecta predominantemente a pacientes con cierto grado de inmunosupresión, como pacientes con lupus eritematoso sistémico, con una mortalidad alrededor del 30%

We present a patient with lupus nephropathy of 20 years of evolution in treatment with oral steroids who developed a meningoencephalitis associated to bacteraemia by Listeria monocytogenes. The patient was treated successfully with gentamicin and ampicillin for 6 weeks. Infection by Listeria monocytogenes occurs more frequently in individuals with some form of immunodeficiency like lupus disease , with a mortality around 30%

Embolización del injerto renal no funcionante: eficacia y control del estado inflamatorio crónico / Embolization of non-functioning renal allograft: efficacy and control of systemic inflammation

Introducción: El síndrome de intolerancia al injerto (fiebre, dolor, hematuria), conlleva el desarrollo de un estado inflamatorio crónico, con consecuencias a nivel cardiovascular. La embolización percutánea del injerto renal no funcionante ha sido descrita como alternativa a la nefrectomía. Presentamos los resultados de la embolización de injertos renales no funcionantes en nuestro centro, respecto al síndrome de intolerancia al injerto y al estado inflamatorio. Material y métodos: Desde el 1-1-2000 hasta el 31-12-2003 hemos realizado siete embolizaciones de injertos renales no funcionantes. El procedimiento se realizó con partículas calibradas (embosferas) de 300-500 µ. Se analizaron datos referentes a las complicaciones de la técnica y los parámetros analíticos relacionados con el estado inflamatorio: PCR, ferritina, albúmina, hemoglobina, y dosis de eritropoyetina, antes y después de la embolización. Resultados: Los pacientes, dos varones y cinco mujeres, tenían una edad media de 39,7 ± 8,8 años. Desde el reinicio de la diálisis hasta la embolización transcurrieron 10,0 ± 8,2 meses. La duración del ingreso por embolización fue de 8,14 ± 4,53 días y por nefrectomía de 17,86 ± 4,41 días. No hubo complicaciones importantes, pero cuatro pacientes presentaron un síndrome postembolización. En cinco pacientes se confirmó la ausencia de captación renal por TC o gammagrafía. No ha sido necesaria la reembolización ni la trasplantectomía hasta el momento. Hemos observado una mejoría en todos los parámetros analíticos después de la embolización, con disminución de la PCR y de la ferritina, aumento de la albúmina, y clara mejoría de las cifras de hemoglobina para dosis de eritropoyetina inferiores. Conclusión: La embolización renal percutánea constituye una técnica sencilla, segura y efectiva, que puede indicarse como alternativa a la nefrectomía quirúrgica, y que resuelve el estado inflamatorio crónico secundario a intolerancia al injerto renal no funcionante

Background: The graft intolerance syndrome (fever, pain, haematuria) may lead to a chronic inflammatory disease, with cardiovascular repercussion. Nephrectomy is considered the classical treatment of these cases but nowadays renal vascular embolization has been suggested as a possible alternative treatment. The present study concerns seven cases trated with renal vascular embolization in our hospital summarizing data of graft intolerance syndrome and the chronic inflammatory disease. Material and methods: Between january 2000 and december 2003 seven renal vascular embolization were performed in nonfunctioning renal allograft. The procedure was made with calibrated particles of 300-500µ. Data about complications related to the technique the same as analitic inflammatory parameters before and after treatment (CRP, ferritin, serum albumin, hemoglobin, erythropoietin) were registered. Results: The patients were two men and five women, with median age of 39,7 ± 8,8 years. The period beween the dialysis and the embolization was of 10,0 ± 8,2 months. Comparing the perion of admission in the hospital due to nephrectomy which was of 17,86 ± 4,41 days, the period because of embolization was shorter, being 8,14 ± 4,53 days. All analitic parameters studied, clearly improved after embolization with decrease of ferritin and CRP, increase of albumin and better hemoglobin level with lower erythropoietin dose. In five of the seven patients there was no renal captation with CT or gammagraphy. Four patients presented a postembolization syndrome, but no other important complication was registered. Neither reembolization nor renal nephrectomy was neccesary in any of the seven cases. Conclusion: Percutaneous renal embolization is a simple, easy, safety and effective technique that must be considered as an alternative treatment to nephrectomy, resolving the chronic inflammatory disease secondary to the graft intolerance syndrome

[Nephropathia caused by hantavirus puumala: a case report]. / Nefropatía por hantavirus puumala: a propósito de un caso.

We present a patient from Germany with Hantavirus infection, admitted in the Emergency room of our hospital, with fever, thrombocytopenia, acute renal failure, oliguria, mild proteinuria and hematuria. Percutaneous renal biopsy revealed an acute interstitial nephritis without medulla haemorrhages. The virus infection confirmation was made by detection of IgM against Hantavirus Puumala. This infection should be considered in patients with thrombocytopenia, fever and acute renal failure, over all if they are from North and Central Europe.

Nefropatía por hantavirus puumala: a propósito de un caso / Nephropathia caused by hantavirus puumala: a case report

Presentamos un caso de un paciente alemán con infección por Hantavirus, queacudió al Servicio de Urgencias de nuestro hospital con fiebre, trombocitopenia,fracaso renal agudo oligúrico, proteinuria y hematuria. La biopsia renal percutaneamostró una nefritis intersticial aguda sin hemorragias medulares. El diagnósticose realizó por la positividad de IgM frente al virus Puumala. La infección porHantavirus debe tenerse en cuenta en pacientes con fracaso renal agudo y fiebrecon trombocitopenia, sobre todo si proceden del Centro y Norte de Europa

We present a patient from Germany with Hantavirus infection, admitted in theEmergency room of our hospital, with fever, thrombocytopenia, acute renal failure,oliguria, mild proteinuria and hematuria. Percutaneous renal biopsy revealedan acute interstitial nephritis without medulla haemorrhages. The virus infectionconfirmation was made by detection of lgM against Hantavirus Puumala. This infectionshould be considered in patients with thrombocytopenia, fever and acuterenal failure, over all if they are from North and Central Europe

Amiloidosis primaria (AL) asociada a déficit severo de factor X / Primary amyloidosis associated to severe factor x deficiency

La amiloidosis es un trastorno sistémico, que se caracteriza por el depósito generalizado de material fibrilar proteico con estructura terciaria Beta-plegada, insoluble y resistente a acción proteolítica, en diferentes tejidos y órganos, principalmente en riñón, hígado y corazón, con importante repercusión clínica. La amiloidosis primaria o AL es el subtipo más común de amiloidosis, y es debida al depósito de fragmentos de cadenas ligeras monoclonales, que se confirma con la demostración de depósito de amiloide en biopsias de grasa abdominal, recto, riñón o hígado, si es necesario. Se ha descrito la asociación de esta enfermedad con el déficit de factor X (factor de Stuart) de la coagulación, atribuida a la adsorción de dicho factor a las fibrillas de amiloide. El pronóstico en la mayoría de pacientes es malo, a pesar de tratamiento quimioterápico, siendo la supervivencia de varios meses desde el diagnóstico. Presentamos un caso de amiloidosis primaria con síndrome nefrótico, déficit severo de factor X (sin complicaciones hemorrágicas relevantes), posible afectación cardíaca y buena respuesta a tratamiento quimioterápico a corto plazo (AU)

[Primary amyloidosis associated to severe factor X deficiency]. / Amiloidosis primaria (AL) asociada a déficit severo de factor X.

Amyloidosis is a systemic disease characterized by generalized deposition of beta-organized proteic fibrillar material with green birefringence under polarized light, in different tissues and organs, the most frequent kidney, liver and heart, with important clinical repercussion. Primary or AL amyloidosis is the most common subtype of amyloidosis (1), confirmed by biopsy-proved amyloid deposition in abdominal fat pad, rectum, kidney or liver, if necessary, in which fragments of monoclonal light chains are deposited. Cases with factor X (Stuart factor) of coagulation deficiency associated are described, due to adsorption of this factor to amyloid fibrills. Normally, evolution is fatal, with only few months of survival. We report a case of primary amyloidosis with nephrotic syndrome, severe factor X deficiency (without bleeding complications), possible heart affection and short-term good response to chemotherapic treatment.

[Influence of early or late referral to nephrologist over morbidity and mortality in hemodialysis]. / Referencia precoz y tardía al nefrólogo, su influencia en la morbi-mortalidad en hemodiálisis.

OBJECTIVE: We studied the influence of early vs late referral to nephrologist of patients with chronic renal failure over clinical situation at the onset of hemodialysis and outcome. SUBJECTS AND METHODS: From january 1994 to december 1998, 139 patients started hemodialysis for end-stage renal disease at the Hospital General de Albacete, all of them included in the study and clinical follow-up concluded in december 2001. Patients with rapidly progressive glomerulonephritis were excluded. Early (ER) and late referral (LR) were defined by the time of first nephrology encounter greater than or less than 6 months respectively, before iniciation of hemodialysis. RESULTS: 106 patients (76.25%) were referred early; mean follow-up time 6.3 +/- 4.5 years. 33 patients (23.74%) had late referral, follow-up time was less then six months, 18 patients were followed during less than 4 weeks. There were no differences in demographic data and comorbid conditions between LR and ER patients (age, cardiac and vascular disease, diabetes, neoplasia...). Mean plasma concentration of creatinine and urea was significantly greater, whereas hematocrit and albumin were less in the LRA than the ER group. Emergency dialysis through central vein catheterisation was more frequent in the LR group. Number of admissions and duration of hospital stay were higher in the LR group. No significant differences in nutrition, dialysis doses or anemia were found between the two groups after 6 and 12 months of hemodialysis. Long term outcome was similar in both groups: no significant differences were found in percentage of patients transplanted or deceased after 3 years of treatment. Survival analysis failed to show a difference between ER and LR groups (mean survival time was 73.6 +/- 4.3 months and 73.0 +/- 6 months respectively). CONCLUSION: Late referral to the nephrologist is associated with increased early morbidity vs early referral, although long term outcome is not worse if predialysis comorbid conditions are comparable and dialysis care achieve equal results in dialysis doses, nutrition and anemia in both groups in the first months of treatment. Improvement of outcome of patients referred early to the nephrologist will depend on the adoption of preventive measures over comorbidity factors that should be applied in early stages of renal disease.