Guilherme Rocha

End-stage renal disease and dialysis in hereditary amyloidosis TTR V30M: presentation, survival and prognostic factors.

Classical familial amyloid polyneuropathy may have a course with progressive renal impairment. We studied 62 patients (24 males, 38 females) with FAP, transthyretin variant V30M, and end-stage renal disease (ESRD) treated with hemodialysis, all referred to a single center over a period of 11 years. Clinical course, morbidity and survival after dialysis were analyzed. Patients mean age at first dialysis was 51.5 ± 10.7 years, and mean duration of neuropathy was 10.2 ± 3.8 years. The most frequent form of presentation of FAP nephropathy was nephrotic proteinuria with renal dysfunction. In the year prior to dialysis, renal function declined rapidly, and fluid overload was the main indication to initiate treatment. The presence of decubitus ulcers, significant disability, venous catheter for definitive vascular access for long-term treatment, and permanent bladder catheter, were related to death during the first year of dialysis. The mean duration of renal replacement therapy was 21 months, with a 54.5% one year, and 38.4% two year treatment survival. However, when the duration of neurological symptoms at first dialysis exceeded 10 years, survival was significantly lower. Infections, (41% were decubitus ulcers with sepsis) were the cause of early, as well as late mortality. Early creation of vascular access for hemodialysis, surveillance of skin wounds, and intervention on neurogenic bladder are essential to improve the prognosis of ESRD in FAP.

Chylous Ascites in a Renal Transplant Recipient Under Sirolimus (Rapamycin) Treatment

Ascites is a rare complication of renal transplantation. Ascites has been reported after kidney transplantation due to rejection, decapsulation of the graft, urinary or vascular leak, lymphocele, transudation, or infection. While technical complications of the procedure are the most frequent cause, portal hypertension and graft rejection are other causes. Ascites can occur after renal transplantation independent of kidney function. Usually, a time relation can be made between the surgical procedure and ascites development. Chylous ascites is still more uncommon; it is usually related to traumatic lymphatic injury. Drugs are rarely associated with the genesis of ascites. Sirolimus has been associated with a high rate of lymphoceles, lymphedema, and pulmonary alveolar proteinosis. The exact mechanisms remain unknown. The risk for lymphocele formation with sirolimus is 12% to 15%. Ascites is an adverse effect with an incidence between 3% and 20%, but no relation between sirolimus and chylous ascites was previously established. We present a clinical report of chylous ascites in a renal transplant patient under sirolimus therapy; our investigation pointed to sirolimus as the cause.

New-Onset Lupus Nephritis in a Kidney Transplant Recipient With Cystinosis—Differential Diagnosis With Cysteamine-Induced Lupus: Case Report

A case of lupus nephritis in an adult female kidney transplant recipient with cystinosis under cysteamine therapy is reported. Previous reports of new-onset lupus in cystinotic patients have focused in a possible relationship of lupus with cysteamine therapy, but no obvious pathophysiological association has been disclosed. The authors present a case of a 19-year-old female kidney transplant recipient with cystinosis admitted for acute allograft dysfunction, with clinical and immunologic manifestations of lupus nephritis. Cysteamine was considered as a potential cause of drug-induced lupus, and we temporarily interrupted this drug. The clinical picture, the negativity of antihistone antibodies, the nondisappearance of antinuclear antibodies after discontinuation of the drug, and the clinical stability after resuming cysteamine therapy suggested that the underlying mechanism of lupus was unrelated to the drug. This may be the first report of new-onset lupus in a kidney transplant recipient with cystinosis. Clinicians should be aware of the association of autoimmune abnormalities in patients with cystinosis.

C1q nephropathy and malignancy

Dear Editor, C1q nephropathy (C1qN) is an idiopathic glomerular disease characterized by extensive mesangial deposition of C1q with associated mesangial immune complexes, in the absence of evidence of systemic lupus erythematosus. Por tanto, aunque la información de que disponemos para el manejo de dichas pacientes es aún escasa, los últimos resultados publicados deben llevarnos a ser optimistas en el tratamiento de estas situaciones.