Chronic graft-versus-host disease of the kidney in patients with allogenic hematopoietic stem cell transplant.

INTRODUCTION: Allogenic hematopoietic stem cell transplant (allo-HSCT) is the treatment of choice for several hematological diseases. Although rare, patients could present nephrotic syndrome as a clinical feature of chronic graft-versus-host disease (cGVHD). The objective of our study is to screen patients with allo-HSCT to determine who developed a glomerular pathology in the context of cGVHD. PATIENTS AND METHODS: We studied patients who underwent allo-HSCT treatment in our center between October 1995 and October 2012 and who developed glomerular pathology. cGVHD was defined as a pathology when it appeared after 100 d post-allo-HSCT. RESULTS: Five hundred eighty-three allo-HSCT were performed. The prevalence of cGVHD of the kidney was 1.03%. All patients with cGVHD of the kidney were hosts who received peripheral blood from an identical HLA match donor. GVHD prophylaxis with calcineurin inhibitors plus methotrexate was administered in five cases, and prophylaxis with sirolimus was used in another case. cGVHD of the kidney was seen to appear after the removal of the prophylaxis for GVHD, within 33 ± 11.54 months intervals after allo-HSCT in five patients and in another patient, it appeared despite immunosuppressive therapy being administered. All patients had proteinuria, within 11.82 ± 9.03 g/d ranges. The kidney biopsies revealed membranous glomerulonephritis (four patients), focal segmental glomerulonephritis (one patient) and lupus nephropathy class III (one patient). It seems, immunosuppressive therapy achieved complete remission, within the first year of treatment in four patients. Although in three of them, the proteinuria recurred when we tried to remove the therapy; two patients have recently started treatment, being in partial remission now. CONCLUSIONS: cGVHD of the kidney is a rare complication after allo-HSCT, related with the removal of the immunosuppression. Monitoring proteinuria in these patients may be useful. In our patients, a complete remission was achieved; although the removal of the immunosuppression may lead to the appearance of outbreaks. We must reconsider the treatment of glomerular pathology secondary to cGVHD.

Perioperative ischemic injury and fibrotic degeneration of muscle in a forearm allograft: functional follow-up at 32 months post transplantation.

Allografts of the forearm are still uncommon in the field of composite tissue allograft transplantation. In November 2007, a right-hand allograft and a left-hand/full-length forearm allograft were transplanted in a 30-year-old man who lost both hands and the vision in his left eye due to an explosion. The patient underwent alemtuzumab and steroid induction therapy. Tacrolimus, mycophenolate mofetil, and low doses of steroids were given to prevent rejection. The allografts were rejected 3 times, but these episodes were controlled successfully. The immunosuppressive regimen was switched from tacrolimus to sirolimus because of increased serum creatinine. The left allograft showed a flexion contracture due to muscle fibrosis that was conjectured to be associated with a perioperative ischemic injury and permitted only a Moberg-type key pinch. In contrast, an excellent grade of function was observed in the right allograft. The Disabilities of the Shoulder, Arm, and Hand score improved from 70.83 to 36.6 and intrinsic musculature returned to both allografts. The patient was able to work 2 years after transplantation. This is the first report of an ischemic injury related to the successful allotransplantation of a composite tissue.

Hypotension, as consequence of the interaction between tacrolimus and mirtazapine, in a patient with renal transplant.

The prevalence of psychiatric disorders in dialyzed patients is estimated around 5-20% of the cases. This explains the high use of antidepressant drugs in these patients. We present the case of a 68-year-old woman with a history of renal failure, with chronic hemodialysis and a depressive syndrome in treatment with Mirtazapine. In November 2008, the patient received a renal graft. An immunosuppressant treatment was started with Basiliximab, Tacrolimus, Mycophenolate Mofetil, and corticosteroids. The patient did not present renal immediate renal function. Four days after the transplant, the treatment with Mirtazapine was re-applied, with an asymptomatic hypotension after 2 hours, and without surgical complications. Tacrolimus blood levels were higher than 15 ng/ml. In our opinion, hypotension was a consequence of the interaction Mirtazapine-Tacrolimus in a patient without immediate renal function. This situation has not been described in the literature before, and hypotension could have had negative consequences in the evolution of the graft.

Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant.

Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimens. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+) who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

A new mutation in the COL4A3 gene responsible for autosomal dominant Alport syndrome, which only generates hearing loss in some carriers.

Bilateral sensorineural hearing loss is a characteristic feature of Alport syndrome, which is always linked to renal manifestations so they have a parallel evolution and prognosis, and deafness helps to identify the renal disease. We report a family that suffers an autosomal dominant Alport syndrome caused by a previously undescribed mutation in the COL4A3 gene, in which several members have hearing impairment as the only clinical manifestation, suggesting that in this family deafness can occur independent of renal disease. This mutation is also present in a patient with anterior lenticonus, an observation only found in families with recessive and sex-linked Alport disease.

Natural progression of renal function in the elderly: analysis of poor prognosis factors associated with chronic kidney disease.

In the last few years a debate has emerged on the range of normal renal function and the rate at which renal disease progresses in the elderly. In this review we analysed, on the basis of the results of the study Ancianos con enfermedad renal crónica del Hospital General de Segovia (Elderly people with chronic kidney disease of the Hospital General de Segovia), the poor prognosis factors associated with this disease: proteinuria, episodes of acute renal failure and heart failure, and the role of uric acid. Elderly people with chronic kidney disease who present these poor prognosis factors may benefit from follow-up by Nephrology.

Encephalopathy caused by lanthanum carbonate.

Lanthanum carbonate is a nonaluminum, noncalcium phosphate-binding agent, which is widely used in patients with end-stage chronic kidney disease. Until now, no significant side-effects have been described for the clinical use of lanthanum carbonate, and there are no available clinical data regarding its tissue stores. Here we report the case of a 59-year-old patient who was admitted with confusional syndrome. The patient received 3750 mg of lanthanum carbonate daily. Examinations were carried out, and the etiology of the encephalopathy of the patient could not be singled out. The lanthanum carbonate levels in serum and cerebrospinal fluid were high, and the syndrome eased after the drug was removed. The results of our study confirm that, in our case, the lanthanum carbonate did cross the blood-brain barrier (BBB). Although lanthanum carbonate seems a safe drug with minimal absorption, this work reveals the problem derived from the increase of serum levels of lanthanum carbonate, and the possibility that it may cross the BBB. Further research is required on the possible pathologies that increase serum levels of lanthanum carbonate, as well as the risks and side-effects derived from its absorption.

C1q nephropathy in a patient with Gitelman syndrome.

Bartter syndrome can manifest in three different forms and is rarely concomitant with glomerular nephropathies. However, this association is more frequently observed in children. We report the case of a 50-year-old woman with Gitelman syndrome for the past 30 years who also had a nephrotic syndrome of recent appearance. Her renal biopsy revealed hyperplasia of the juxtaglomerular apparatus and mesangial deposits of C1q, with no clinical or serological evidence of systemic erythematous lupus. We have not found any reports of instances of association of Gitelman syndrome and nephrotic syndrome arising from C1q nephropathy in adult patients. Our case suggests the possible existence of an association between hypokalaemic tubular nephropathies and glomerular nephropathies that may cause nephrotic syndrome.

Recidiva de vasculitis asociada a anticuerpos anticitoplasma de neutrófilos en un paciente con trasplante renal / Recurrence of ANCA-associated vasculitis in a patient with kidney transplant

La afectación renal de las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) puede conducir a enfermedad renal crónica con necesidad de tratamiento renal sustitutivo. En estos enfermos el trasplante renal ofrece excelentes tasas de supervivencia del injerto y del receptor a largo plazo, por lo que pueden ser trasplantados cuando la enfermedad está en remisión. Sin embargo, la amenaza de recidivas de la enfermedad en el injerto se mantiene, aunque, con las modernas pautas de inmunosupresión, su incidencia es menor. Presentamos el caso de un varón diagnosticado de glomerulonefritis extracapilar tipo III C-ANCA (+) que desarrolló una recidiva de la enfermedad en el injerto renal 8 años después de ser trasplantado. La intensificación de la inmunosupresión con plasmaféresis consiguió controlar la enfermedad (AU)

Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimens. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+) who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease (AU)

Poliangeítis microscópica en paciente con cirrosis biliar primaria. Complicaciones del tratamiento / Microscopic polyangiitis in a patient with primary biliary cirrhosis: Treatment complications

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The Spanish experience with hand, forearm, and arm transplantation.

This article summarizes the findings from 3 recipients of hand allografts, including a description of the preparatory surgery and the transplant and secondary procedures to enhance the function of the hand, forearm, and arm allografts. The study focuses on the complications and disability reported by each patient, with a minimum follow-up of 2 years. The few complications were controlled successfully with medical treatment. Hand transplantation is a major reconstructive procedure that requires careful medical follow-up. The authors provide the first report of a significant improvement in disabilities of the upper limb as a result of hand allotransplantation.

Hipocalcemia, hiperfosforemia y elevación de la paratohormona, ¿un arduo diagnóstico diferencial? / Hypocalcaemia, hyperphosphataemia and elevated parathyroid hormone, a difficult differential diagnosis?

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Evolución natural de la función renal en el anciano: análisis de factores de mal pronóstico asociados a la enfermedad renal crónica / Natural progression of renal function in the elderly: analysis of poor prognosis factors associated with chronic kidney disease

En los últimos años se ha generado un debate sobre el rango de función renal normal y el ritmo de progresión de la enfermedad renal en el anciano. En esta revisión analizamos, basándonos en los resultados del estudio Ancianos con enfermedad renal crónica del Hospital General de Segovia, los factores de mal pronóstico asociados a esta enfermedad: proteinuria, episodios de fracaso renal agudo y de insuficiencia cardíaca, y el papel del ácido úrico. Los ancianos con enfermedad renal crónica que presenten estos factores de mal pronóstico serían los que se podrían beneficiar del seguimiento por Nefrología (AU)

In the last few years a debate has emerged on the range of normal renal function and the rate at which renal disease progresses in the elderly. In this review we analysed, on the basis of the results of the study Ancianos con enfermedad renal crónica del Hospital General de Segovia (Elderly people with chronic kidney disease of the Hospital General de Segovia), the poor prognosis factors associated with this disease: proteinuria, episodes of acute renal failure and heart failure, and the role of uric acid. Elderly people with chronic kidney disease who present these poor prognosis factors may benefit from follow-up by Nephrology (AU)

Enfermedad renal crónica en el anciano: influencia del género / Chronic kidney disease in the elderly: the impact of patients' sex

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