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1.
Nephron ; 144(11): 537-549, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32950988

RESUMO

Atypical hemolytic uremic syndrome (aHUS) is a rare cause of thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and multisystem end organ involvement, most commonly affecting the kidney. Diagnosis is clinical, after exclusion of other TMA causes. Primary aHUS arises from genetic abnormalities, resulting in uncontrolled complement activity, while a variety of clinical scenarios cause secondary aHUS, including infection, pregnancy, malignancy, autoimmune disease, and medications. They can also induce a temporary complement deregulation with an overlap between both scenarios, which can make differential diagnosis difficult. Primary aHUS can be sporadic or familial and is associated with a high rate of progression to ESRD. Many aHUS patients relapse in the native or transplanted kidneys, leading to kidney failure. The introduction of eculizumab has changed the prognosis of aHUS, by inducing hematologic remission, improving or stabilizing kidney functions, and preventing graft failure. The early institution of appropriate therapy can prevent multiorgan damage, so is essential to recognize and differentiate the TMA syndromes. Eculizumab is considered now the first-line treatment, and it is recommended lifelong therapy. However, the high cost of therapy has led to make efforts to develop precise complement functional and genetic studies that help physicians to determine the appropriate duration of eculizumab therapy. Nowadays, more studies are needed to select candidates to adjustment of therapy.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/tratamento farmacológico , Inativadores do Complemento/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/genética , Síndrome Hemolítico-Urêmica Atípica/fisiopatologia , Síndrome Hemolítico-Urêmica Atípica/cirurgia , Humanos , Transplante de Rim , Prognóstico
2.
Cent European J Urol ; 70(4): 439-444, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29410900

RESUMO

INTRODUCTION: Perigraft fluid collection (PFC) is a common complication after kidney transplant. Its etiology is not clear and not all the causes have been identified. The influence of the type of donor has never been evaluated. Our aim was to compare the incidence, severity and management of PFC in recipients of grafts from uncontrolled donors after circulatory death (DCD) with normothermic extracorporeal membrane oxygenation (NECMO) versus recipients of grafts from donors after brain death (DBD). MATERIAL AND METHODS: We conducted a retrospective cohort study of 300 kidney transplants performed in our center between 2007 and 2012. Patients were divided in two groups: 150 recipients of Maastricht II DCD graft and 150 recipients of the DBD graft. Incidence, severity according to Clavien scale and management were analyzed in both groups, and comparison was carried out using Chi-square. RESULTS: Of the 300 kidney recipients analyzed, 93 (31.4%) suffered PFC, showing no difference between DBD (32.0%) and DCD (30.8%) groups (p = 0.9). Complicated PFC rate (defined as a PFC generating vascular compression, fever or urinary tract obstruction) was 22.9% in the DBD group versus 22.2% in the DCD group (p = 1); most complicated PFC were due to urinary tract obstruction (81%), with no difference between the groups (p = 1). Concerning Clavien scale, 78.5% of the PFC in our series were Clavien I, 19.4% Clavien IIIa and 2.2% Clavien IIIb, with no difference between both groups (p = 1). CONCLUSIONS: PFC is a frequent complication that appears in a third of our patients, showing no difference in the incidence or severity between DBD and uncontrolled DCD graft recipients.

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