Use of the new proliferation signal inhibitor everolimus in renal transplant patients in Spain: preliminary results of the EVERODATA registry.

Everolimus (Eve) has shown good efficacy and safety profiles in clinical trials in combination with low doses of cyclosporine but there is limited experience in other modes, especially with calcineurin inhibitor elimination. We developed a retrospective study to analyze its clinical use after approval in Europe in 2005. Herein we have presented the results of a series of 272 patients followed for the first 6 months after Eve introduction. In 93.8% of cases Eve was introduced after the first month posttransplantation (conversion use), and 6 months after introduction, the CNI had been eliminated in 75% of cases. The main indication for Eve introduction was the diagnosis of a malignant neoplasm (42%), whereas the combined indication of prevention and/or treatment of toxicity, especially nephrotoxicity, accounted for 46.3% of cases. Initial doses were low (1.37 mg/d), but were progressively increased up to 2 mg/d at 6 months. Renal function remained unchanged during the follow-up period, whereas proteinuria moderately increased. Only 5 cases (2%) of acute rejection episodes were observed with excellent patient and graft survivals at 6 months after conversion. Further analysis of this extensive series of patients with a longer follow-up is needed.

Kidney transplantation with grafts from non-heart-beating donors.

We analyzed the function and outcome of 16 kidney transplants performed in our hospital from non-heart-beating donors who were harvested at other hospitals. The cold ischemia times were longer and the delayed graft function rates higher. However, graft function was no different from that of kidneys from heart-beating donors. This experience has encouraged us to use this type of donor to reduce the transplant waiting list.

[Endovascular treatment of massive hemoptysis by bronchial artery embolization: short-term and long-term follow-up over a 15-year period]. / Tratamiento endovascular mediante embolización arterial bronquial en la hemoptisis masiva. Seguimiento a corto y largo plazo durante 15 años.

OBJECTIVE: To present our experience of using arterial embolization for the endovascular treatment of massive hemoptysis along with the results of follow-up over a 15-year period. PATIENTS AND METHODS: A total of 401 patients with hemoptysis were referred to the minimally invasive surgery unit of the Hospital Universitario Lozano Blesa de Zaragoza between April 1989 and September 2004 for diagnosis and possible endovascular treatment. Of those patients, 314 met criteria for massive hemoptysis and treatment was attempted using embolization in 287 (91.4%). The most common cause of hemoptysis was bronchiectasis (n=99, 31.5%), followed by lesions due to tuberculosis (n=57, 18.1%) and chronic bronchitis (n=47, 14.9%). RESULTS: Angiography of the bronchial arteries provided evidence to account for the hemoptysis in 287 patients (91.4%). The affected arteries were satisfactorily embolized in 281 (97.9%). Endovascular treatment was clinically successful in 256 of those patients (91.1%). Embolization had to be repeated during the hospital stay in 19 patients (6.7%) and was effective in 52.6% of those cases. The 6 patients in whom embolization was not satisfactory underwent thoracotomy. The mean follow-up in 201 patients (71.5%) was 2372.5 days (range, 61-5475 days). Eighty patients (28.4%) were lost to follow-up for various reasons and at different points. Recurrence of hemoptysis occurred on 1 or more occasions in 45 patients (22.3%) but only 21 (10.4%) required repeat embolization. Minor complications that did not require treatment were observed in 88 patients (28.0%). CONCLUSIONS: Embolization of bronchial arteries is a nonsurgical treatment that is safe and effective in patients with massive hemoptysis.

[Prolonged acute renal failure and severe polyradiculopathy in ethylene glycol intoxication]. / Fracaso renal agudo prolongado y polirradiculopatía severa en paciente intoxicado con etilenglicol.

Ethylene glycol intoxication involves acute renal failure and severe metabolic acidosis. Prolonged renal insufficiency can occur but terminal chronic renal failure has been reported in very few cases. We describe a patient who after ingestion of 920 ml of ethylene glycol developed prolonged acute renal failure needing hemodialysis for 37 days and then he partly recovered renal function. The patient developed a severe sensitive-motor and autonomic polyradiculopathy.

Renal artery stenosis after kidney transplantation: diagnostic and therapeutic approach.

Transplant renal artery stenosis (TRAS) is a common complication after transplantation and an important cause of graft dysfunction. Many factors have been implicated as possible causes of TRAS, such as damage from trauma and atherosclerosis. We reviewed all 286 patients transplanted in our unit from January 1990 to July 1997 to study the prevalence, clinical features, and diagnostic and therapeutic approach. Thirteen patients with TRAS were identified, and their mean age was 40 +/- 15 years. The detected incidence was 4.5%. They were treated with triple therapy (prednisone, azathioprine and cyclosporine A). The mean age of the donors was 28 +/- 27 years. TRAS was diagnosed within nine months after transplant. All patients were studied with Doppler ultrasound, renography with captopril and angiography. The preferred initial therapy was percutaneous transluminal balloon renal angioplasty. Angioplasty was performed in four patients with good results. Two patients underwent surgery because angioplasty was not possible. Blood pressure control could be achieved with less antihypertensive medication after angioplasty. Transplantectomy was performed in one patient because of surgical complications. In conclusion, most patients with TRAS can be treated successfully with percutaneous transluminal angioplasty as the initial interventional treatment of choice for high-grade renal artery stenosis, and surgical revascularization is indicated when percutaneous transluminal angioplasty cannot be done or is unsuccessful.

Immunoprophylaxis with Simulect (basiliximab) in kidney transplantation: results from routine clinical practice at 18 kidney transplant units.

OBJECTIVE: The objective of this study is to assess a Simulect (basiliximab) regimen in routine clinical practice in the Spanish kidney transplantation units to evaluate efficacy and safety. METHODS: In this prospective, observational study, data on demographics, parameters of efficacy, and safety in patients who under with kidney transplantation treated with Simulect (basiliximab) were collected through an on-line collection system. RESULTS: One hundred sixty three patients at 18 kidney transplant units included 12 months follow-up. The patient mean age was 52 years (DS 13,67) including 96 (58.90%) men and 67 (41.10%) women. Cold ischemia time was 19 hours (DS 6,79). Only 2 patients presented with PRA >50%. For prophylactic immunosuppression, 67.13% of patients received triple therapy with CNI (cyclosporine 49.65% or tacrolimus 17.48%), MMF (66.43%) or AZA (10.49%), and steroids. Incidence of acute rejection (AR) at 12 months was 12.27% (1.84% steroid-resistant). In subgroup analysis, AR was 13.5% in nondiabetics and 4.5% in diabetics, including 3 steroid-resistant episodes (1.84%) in nondiabetics and none in diabetics. In relation to donor age, AR was incidence 10.3% in patients with kidneys from donors aged 50 years or younger and 10.6% when donors were older than 50 years, including 1 (1.73%) and 2 (1.93%) steroid-resistant episodes, respectively. The graft and patient survival rates at 12 months were 90% and 98%, respectively. CONCLUSIONS: Simulect (basiliximab) used in routine clinical practice provided good prophylaxis against acute rejection in several kidney transplant patient populations, similar to that observed in randomized clinical studies with excellent tolerability and safety.

[Lupus nephropathy in childhood and familial lupus. Genetic study of a family]. / Nefropatía lúpica en la infancia y lupus familiar. Estudio genético de una familia.

We report a case of a 14 1/2-year-old boy who was diagnosed of systemic lupus erythematosus in the background of an acute nephritic syndrome, 3 1/2 years after being diagnosed of idiopathic thrombocytopenic purpura. The familial history suggested the presence of other cases of SLE, which were proven with relevant clinical and laboratory studies. A genetic study for disease markers was carried out and a correlation was found with haplotypes HLA A25, B18, BW6, DRX, and DQW; C2 deficiency was ruled out. We conclude that it is of paramount importance to rule out the existence of familial SLE in front of infantile SLE, particularly in boys, and we emphasize the necessity of keeping on further searching for genetic markers of the disease.

[Nephronophthisis: study of 10 cases. Incidence, natural history and associated pathology (author's transl)]. / Nefronoptisis: análisis de 10 casos. Incidencia, historia natural y patología asociada.

Nephronophthisis is a chronic interstitial nephropathy which in childhood may lead to terminal renal failure. Between January 1975 and July 1980, 41 children with terminal renal failure were seen in our service, of which 10 (21.9%) presented with nephronophthisis. Age of the patients ranged from 3.5-18 years, boys were in the majority (8/2). Three cases were isolated and 7 were familial (3 families). Onset was during the first year of life in 8 patients, and polydipsia-polyuria were the first symptoms. Retarded growth and anemia proportionate to the degree of renal failure were present in all patients. When diagnosed, 5 patients (50%) presented terminal renal failure, and the other 5 had renal failure of different degrees. Moderate proteinuria was found in 4 patients, without changes in urine sediment. Sodium depletion in urine was high in 5 cases and maximal urine osmolarity was less than 500 muOsm/l after hydropenia in all cases. Four had associated mental deficiency with cerebellar ataxia associated in two and congenital hepatic fibrosis (confirmed histologically) in one. The diagnosis was confirmed by biopsy in 8 and in two of these on frozen section during nephrectomy prior to kidney transplantation. At present, five of the patients are in maintenance hemodialysis, two died at home due to cardiovascular complications in terminal renal insufficiency and the remaining ones presented different degrees of renal insufficiency. Time elapsed between onset of the symptoms and inclusion in hemodialysis or death ranges form 6 months to 13 years (mean 6.7 years).

[Scintigraphy diagnosis of renovascular hypertension in renal transplanted patients]. / Diagnóstico gammagráfico de hipertensión renovascular en trasplantados renales.

UNLABELLED: Captopril renography (CR) has been shown to be a useful technique in the diagnosis of renovascular hypertension (RVH). This disease is a significant complication of the kidney transplanted patient so that early diagnosis would be extremely useful to preserve renal function and prevent graft loss. This work evaluates the role of CR, together with arteriography and doppler-ultrasound, in the diagnosis of RVH. MATERIALS: A total of 19 transplanted patients with clinical suspicion of RVH underwent an isotopic study, a doppler-ultrasound study and an arteriography. Scintigraphy was performed 1 hour after a 50 mg dose of captopril, with oral hydration and i.v. administration of approximately 111 MBq of 99mTc-MAG3 and 20 mg of furosemide. If abnormal, a subsequent renography in baseline conditions was performed for comparison. RESULTS: Overall, 11 patients had renovascular hypertension. Sensitivity and specificity of CR were 91% and 80% respectively, 100% and 60% for US and 91% and 100% for arteriography. The CR yielded one false positive result and 2 false negatives cases: one with 50% stenosis and one polar artery stenosis. Six patients were successfully dilated or operated. CONCLUSION: CR seems to be a reliable diagnostic tool for the diagnosis of renovascular hypertension in kidney transplanted patients.

Variables asociadas a complicaciones de la punción con aguja fina frente a biopsia con aguja gruesa de las lesiones pulmonares / Variables associated with complications of fine needle puncture against core needle biopsy of pulmonary injuries

Introducción. El cáncer de pulmón es la primera causa de muerte por cáncer contando con terapias específicas que exigen identificar estructuras moleculares; por ello se utiliza más biopsia aguja gruesa, pudiendo aumentar el riesgo de complicaciones. El objetivo es comparar tasa de complicaciones de punción aguja fina (PAAF) frente a biopsia aguja gruesa (BAG) y analizar variables asociadas. Material y método. Análisis retrospectivo observacional de 146 pacientes con lesiones pulmonares periféricas, practicando PAAF o BAG guiada con técnicas de imagen. El periodo de estudio (marzo 2012-septiembre 2013), durante la primera mitad se realizó PAAF y durante la segunda BAG. Se calculó la tasa de complicaciones y se analizaron variables relativas al tamaño y localización lesión, técnica y presencia de enfisema. El estudio estadístico se realizó mediante un análisis univariado seguido de un modelo de regresión logística. Resultados. Se realizó PAAF en 66 pacientes (45,2%) y BAG en 80 (54,8%). Tasa global complicaciones 36,3% (53 casos), siendo neumotórax la más frecuente 43 casos (29,5%). La tasa de complicaciones fue menor en BAG que PAAF (28,8% vs 45,5%) (p= 0,037) en análisis univariado. En análisis multivariado las variables que asociaron mayor probabilidad complicaciones fueron: número pases ≥ 3 (OR 0,57), tamaño lesión < 30 mm (OR 3,45) y distancia lesión-pleura > 15 mm (OR 0,94). Conclusiones. Los factores que asociaron de forma independiente complicaciones fueron tamaño lesión, distancia lesión-pleura y número pases. La menor tasa complicaciones de BAG se explica por el menor número de pases realizados

Introduction. Lung cancer is the leading cause of cancer death, with specific therapies that require identification of molecular structures; therefore, a core needle biopsy is used, which can increase the risk of complications. The objective is to compare the rate of complications of fine needle puncture (FNP) versus core needle biopsy (CNB) and analyze associated variables. Material and method. Retrospective observational analysis of 146 patients with peripheral pulmonary lesions, practicing FNP or CNB guided with imaging techniques. Study period (March 2012-September 2013), during the first half FNP was carried out and during the second CNB. The complication rate was calculated and variables related to size and location of the lesion, technique and presence of emphysema were analyzed. The statistical study was carried out through a univariate analysis followed by a logistic regression model. Results. FNP was performed in 66 patients (45.2%) and CNB in 80 (54.8%). Overall rate complications 36.3% (53 cases) with pneumothorax being the most frequent 43 cases (29.5%). The complication rate was lower in CNB than FNP (28.8% vs 45.5%) (p= 0.037) in univariate analysis. In multivariate analysis, variables that associated the highest probability of complications were: number of passes ≥ 3 (OR 0.57), lesion size < 30 mm (OR 3.45) and lesion-pleura distance > 15 mm (OR 0.94). Conclusions. Factors that independently associated complications were lesion size, lesion-pleura distance and number of passes. The lower complication rate of BAG is explained by lower number of passes made

Use of mycophenolate sodium in stable renal transplant recipients in Spain: preliminary results of the MIDATA study.

INTRODUCTION: The use of mycophenolate mofetil (MMF) is limited by gastrointestinal adverse events (GI-AEs). Enteric-coated mycophenolate sodium (EC-MPS) was developed to avoid these effects. METHODS: This multicenter prospective study sought to analyze the clinical benefit of EC-MPS among 726 stable renal transplant recipients in Spain. The data collection included: doses and trough levels of mycophenolic acid (MPA) and calcineurin inhibitors (CNI), renal function, routine biochemical parameters (3-6 months preconversion, baseline, and 1, 3, 6, and 12 months of EC-MPS initiation), as well as graft and patient survivals and adverse events. RESULTS: The main indication for EC-MPS introduction was GI-AEs associated with MMF (44.1%). Preliminary data showed that before introduction of EC-MPs there was a progressive deterioration of renal function, as demonstrated by a negative slope of the creatinine clearance (P < .005). However, after EC-MPS conversion, the slope became positive (P < .05), suggesting an improvement in renal function. Only in 4.8%, EC-MPS was stopped due to GI-AEs. There was an increase in MPA serum levels (P < .01) and a reduction in CNI doses. Interestingly, 80% of 85 patients without MMF treatment because of severe GI-AEs tolerated EC-MPS, including 43% who could be treated with adequate doses of EC-MPS (>or=720 mg/d). CONCLUSIONS: There was a significant improvement in GI-AEs after conversion from MMF to EC-MPS. The use of lower doses of CNI and the better tolerability of EC-MPS could be the underlying causes of improvement in renal function.

Migraña hemipléjica familiar / Familial hemiplegic migraine

Las cefaleas constituyen una causa frecuente de consulta en Pediatría, siendo la migraña el tipo más frecuente de las cefaleas primarias en la infancia. La asociación entre cefalea y hemiplejía aguda en la infancia pudiera corresponder a múltiples etiologías debiéndose considerar las de carácter hereditario. La migraña hemipléjica familiar se caracteriza por la presencia de crisis migrañosas con trastornos motores deficitarios transitorios, afasia o alteraciones sensitivas o sensoriales. Se describe el caso de una adolescente femenina de 12 años de edad, con antecedentes familiares de migraña, historia de cefalea migrañosa de un año de evolución, que cumple con los criterios establecidos por la Sociedad Internacional de Cefaleas de migraña hemipléjica familiar. El examen neurológico y los paraclínicos complementarios fueron normales. Se realizó tratamiento con flunarizina e ibuprofeno con evolución satisfactoria

Headaches are a frequent cause of consultation in Pediatrics, migraine being the most common type of primary headaches in children. The association between headache and acute hemiplegia in childhood may correspond to multiple etiologies, including those considered as inherited. Familial hemiplegic migraine is characterized by the presence of migraine crisis with transient motor deficit disorders, aphasia and sensitive or sensory disturbances. We describe the case of a 12 year-old girl with a family history of migraine, and migraine headache of a year of evolution, which meets the criteria established by the International Headache Society of Familial Hemiplegic Migraine. Neurological examination and the paraclinical studies were normal. She was treated with flunarizine and ibuprofen with satisfactory outcome