RESUMO
Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over-activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy-associated aHUS, and aHUS in renal transplant patients.
Assuntos
Anticorpos Monoclonais Humanizados/farmacologia , Síndrome Hemolítico-Urêmica Atípica , Via Alternativa do Complemento , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Síndrome Hemolítico-Urêmica Atípica/etiologia , Síndrome Hemolítico-Urêmica Atípica/fisiopatologia , Síndrome Hemolítico-Urêmica Atípica/terapia , Via Alternativa do Complemento/efeitos dos fármacos , Via Alternativa do Complemento/genética , Via Alternativa do Complemento/imunologia , Gerenciamento Clínico , Humanos , Fatores Imunológicos/farmacologiaRESUMO
Patients with acute kidney injury (AKI) in the intensive care unit (ICU) are often suitable for palliative care due to the high symptom burden. The role of palliative medicine in this patient population is not well defined and there is a lack of established guidelines to address this issue. Because of this, patients in the ICU with AKI deprived of the most comprehensive or appropriate care. The reasons for this are multifactorial including lack of palliative care training among nephrologists. However, palliative care in these patients can help alleviate symptoms, improve quality of life, and decrease suffering. Palliative care physicians can determine the appropriateness and model of palliative care. In addition to shared decision-making, advance directives should be established with patients early on, with specific instructions regarding dialysis, and those advance directives should be respected.