ABSTRACT
Atypical uremic haemolytic syndrome is a variant of thrombotic micro-andiopathy characterized by non-autoimmune hemolytic anemia, thrombocytopenia and acute renal failure as a result of excessive activation of the complement. Up to 60% of patients have mutations in the genes that encode the complement system. A disensing factor is required for its manifestation, including gestation. It is an entity with a high morbidity, which can decrease drastically if an early diagnosis is made and appropriate treatment is initiated. Administration of ecuilizumab has demonstrated rapid process disruption, reducing the need for extrarenal purification therapies and improving renal function and patient prognosis.
Subject(s)
Acute Kidney Injury , Anemia, Hemolytic , Atypical Hemolytic Uremic Syndrome , Acute Kidney Injury/etiology , Acute Kidney Injury/therapy , Atypical Hemolytic Uremic Syndrome/diagnosis , Atypical Hemolytic Uremic Syndrome/therapy , Female , Humans , Pregnancy , PrognosisABSTRACT
El síndrome hemolítico urémico atípico es una variante de la microangiopatía trombótica caracterizada por presentar anemia hemolítica no autoinmune, trombocitopenia y fallo renal agudo como consecuencia de una excesiva activación del Sistema del complemento. Hasta en un 60% de los pacientes presentan mutaciones en los genes que codifican el sistema del complemento. Se requiere un factor desencadenante para su manifestación, entre los que se encuentra la gestación. Es una entidad que presenta una elevada morbimortalidad, que puede disminuir de forma relevante si se realiza un diagnóstico precoz y se inicia tratamiento adecuado. La administración de eculizumab ha demostrado la interrupción rápida del proceso, con reducción de la necesidad de terapias de depuración extrarrenal y mejoría de la función renal y pronóstico de las pacientes.(AU)
Atypical uremic haemolytic syndrome is a variant of thrombotic micro-andiopathy characterized by non-autoimmune hemolytic anemia, thrombocytopenia and acute renal failure as a result of excessive activation of the complement. Up to 60% of patients have mutations in the genes that encode the complement system. A disensing factor is required for its manifestation, including gestation. It is an entity with a high morbidity, which can decrease drastically if an early diagnosis is made and appropriate treatment is initiated. Administration of ecuilizumab has demonstrated rapid process disruption, reducing the need for extrarenal purification therapies and improving renal function and patient prognosis.(AU)
Subject(s)
Humans , Female , Young Adult , Hemolytic-Uremic Syndrome , Pregnant Women , Thrombocytopenia , Thrombotic Microangiopathies , Anemia, Hemolytic , Indicators of Morbidity and MortalityABSTRACT
Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).
Subject(s)
Anemia, Hemolytic , Atypical Hemolytic Uremic Syndrome , Purpura, Thrombotic Thrombocytopenic , Thrombotic Microangiopathies , Algorithms , Atypical Hemolytic Uremic Syndrome/therapy , Female , Humans , Male , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Thrombotic Microangiopathies/diagnosis , Thrombotic Microangiopathies/etiology , Thrombotic Microangiopathies/therapyABSTRACT
Las microangiopatías trombóticas (MAT) son un conjunto de síndromes clínicos que asocian anemia hemolítica, trombocitopenia y disfunción orgánica, principalmente renal o neurológica. Están asociados a una morbimortalidad significativa, por lo que su diagnóstico y tratamiento precoz son esenciales. En este artículo detallamos 2 casos de MAT; una paciente con una púrpura trombocitopénica trombótica (PTT) y otra paciente con un síndrome hemolítico urémico atípico (SHUa).(AU)
Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).(AU)
Subject(s)
Humans , Female , Adult , Middle Aged , Thrombotic Microangiopathies/congenital , Thrombotic Microangiopathies/drug therapy , Thrombotic Microangiopathies/therapy , Anemia, Hemolytic , Thrombocytopenia , Atypical Hemolytic Uremic Syndrome , Purpura, Thrombotic Thrombocytopenic , ADAMTS13 Protein , Anesthesiology , Cardiopulmonary Resuscitation , Indicators of Morbidity and MortalityABSTRACT
Thrombotic microangiopathies (TMA) are a group of clinical syndromes associated with haemolytic anaemia, thrombocytopenia and organ dysfunction, mainly renal or neurological. They are associated with significant morbidity and mortality, so early diagnosis and treatment are essential. In this article we report two cases of TMA; a patient with thrombotic thrombocytopenic purpura (TTP) and a patient with atypical haemolytic uraemic syndrome (aHUS).
ABSTRACT
Atypical uremic haemolytic syndrome is a variant of thrombotic micro-andiopathy characterized by non-autoimmune hemolytic anemia, thrombocytopenia and acute renal failure as a result of excessive activation of the complement. Up to 60% of patients have mutations in the genes that encode the complement system. A disensing factor is required for its manifestation, including gestation. It is an entity with a high morbidity, which can decrease drastically if an early diagnosis is made and appropriate treatment is initiated. Administration of ecuilizumab has demonstrated rapid process disruption, reducing the need for extrarenal purification therapies and improving renal function and patient prognosis.