Síndrome Urémico Hemolítico

Descripción General

Definición

El síndrome urémico hemolítico es una enfermedad de los LOS Neisseria capilares (microangiopatía) que provoca la formación de coágulos de sangre, anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types causada por la destrucción de los LOS Neisseria eritrocitos en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum estos capilares coagulados ( anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica), lesión renal aguda y disminución de las plaquetas (trombocitopenia).

Epidemiología

• La mayoría de los LOS Neisseria casos se observan en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum niños < 5 años de edad
• 2–3 casos por cada 100 000 niños en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum Estados Unidos
• Principal causa de lesión renal aguda en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum niños (> 6 meses a 5 años de edad)
• El 90% de los LOS Neisseria casos están asociados a diarreas causadas por Escherichia coli Escherichia coli The gram-negative bacterium Escherichia coli is a key component of the human gut microbiota. Most strains of E. coli are avirulent, but occasionally they escape the GI tract, infecting the urinary tract and other sites. Less common strains of E. coli are able to cause disease within the GI tract, most commonly presenting as abdominal pain and diarrhea. Escherichia coli productora de toxina Shiga (STEC, por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
• Más comúnmente visto en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el verano y en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum poblaciones rurales

Etiología

La etiología se clasifica como adquirida (infecciosa o no infecciosa) o hereditaria.

• Síndrome urémico hemolítico adquirido
  • Infecciosa
    • Escherichia coli Escherichia coli The gram-negative bacterium Escherichia coli is a key component of the human gut microbiota. Most strains of E. coli are avirulent, but occasionally they escape the GI tract, infecting the urinary tract and other sites. Less common strains of E. coli are able to cause disease within the GI tract, most commonly presenting as abdominal pain and diarrhea. Escherichia coli productora de toxina tipo Shiga: la más común
    • Especies de Shigella Shigella Shigella is a genus of gram-negative, non-lactose-fermenting facultative intracellular bacilli. Infection spreads most commonly via person-to-person contact or through contaminated food and water. Humans are the only known reservoir. Shigella
    • Streptococcus Streptococcus Streptococcus is one of the two medically important genera of gram-positive cocci, the other being Staphylococcus. Streptococci are identified as different species on blood agar on the basis of their hemolytic pattern and sensitivity to optochin and bacitracin. There are many pathogenic species of streptococci, including S. pyogenes, S. agalactiae, S. pneumoniae, and the viridans streptococci. Streptococcus pneumoniae
    • VIH
  • Autoanticuerpos contra los LOS Neisseria factores del complemento
  • Toxicidad de los LOS Neisseria medicamentos
    • Medicamenos asociados a malignidad o a los LOS Neisseria receptores de trasplantes de órganos (ciclosporina y tacrolimus Tacrolimus A macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro. Immunosuppressants)
    • Drogas de abuso (cocaína)
  • Otros:
    • Lupus eritematoso sistémico
    • Síndrome antifosfolipídico
• Síndrome urémico hemolítico hereditario
  • Mutaciones del gen del complemento (C3 y CD46)
  • Errores congénitos del metabolismo (metabolismo de la cobalamina C)

Fisiopatología

La fisiopatología del síndrome urémico hemolítico secundario a la toxina Shiga ha HA Hemolytic anemia (HA) is the term given to a large group of anemias that are caused by the premature destruction/hemolysis of circulating red blood cells (RBCs). Hemolysis can occur within (intravascular hemolysis) or outside the blood vessels (extravascular hemolysis). Hemolytic Anemia sido bien descrita.

1. Las toxinas bacterianas son absorbidas por el intestino y entran en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la circulación sistémica.
2. Se unen a las células endoteliales causando una lesión endotelial directa a través de:
   • Producción de óxido nítrico (NO, por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
   • Secreción de interleucina-1 (IL-1) y factor de necrosis Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply. Ischemic Cell Damage tumoral ( TNF TNF Tumor necrosis factor (TNF) is a major cytokine, released primarily by macrophages in response to stimuli. The presence of microbial products and dead cells and injury are among the stimulating factors. This protein belongs to the TNF superfamily, a group of ligands and receptors performing functions in inflammatory response, morphogenesis, and cell proliferation. Tumor Necrosis Factor (TNF), por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
   • Activación de los LOS Neisseria macrófagos
3. Ocasiona la agregación plaquetaria y la trombosis localizada en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum capilares y arteriolas:
   • Los LOS Neisseria vasos de los LOS Neisseria glomérulos están especialmente afectados → disminución de la tasa de filtración glomerular (TFG)
   • Disminución del flujo sanguíneo renal → aumento de la renina
4. Los LOS Neisseria eritrocitos se lesionan mecánicamente al AL Amyloidosis atravesar la microvasculatura trombótica: lo que causa anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica microangiopática

Otras formas de síndrome urémico hemolítico:

• Mecanismos patológicos variados
• Todos tienen en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum común el daño endotelial.
• Conducen a la formación de trombos y a la anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica

Presentación Clínica

Antecedentes

• Enfermedad prodrómica con dolor Dolor Inflammation abdominal, vómitos y diarrea sanguinolenta
• Otras pistas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la historia clínica pueden ser:
  • Brote de conocido concurrente/miembro de la familia con síndrome urémico hemolítico concurrente
  • Miembro de la familia con antecedentes de síndrome urémico hemolítico (puede sugerir una causa genética)
  • Episodio previo de síndrome urémico hemolítico (puede sugerir una causa mediada por el complemento)

Examen físico

Los LOS Neisseria síntomas suelen aparecer entre 5–7 días posterior a la diarrea y pueden incluir:

• Letargo o irritabilidad aguda
• Palidez
• Síndrome nefrítico:
  • Oliguria Oliguria Decreased urine output that is below the normal range. Oliguria can be defined as urine output of less than or equal to 0. 5 or 1 ml/kg/hr depending on the age. Renal Potassium Regulation, sobrecarga de volumen o deshidratación
• Progresión de los LOS Neisseria síntomas neurológicos:
  • Alteración del estado mental
  • Convulsiones

Diagnóstico

El diagnóstico es clínico, basado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la tríada clásica de:

1. Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica microangiopática
   • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types normocítica con nivel de hemoglobina < 8 g/dL
   • Prueba de Coombs directa negativa
   • Frotis de sangre periférica → esquistocitos y células falciformes
2. Trombocitopenia:
   • Recuento de plaquetas < 140 000/mm³ (normalmente alrededor de 40 000/mm³)
3. Lesión renal aguda:
   • Elevación de la creatinina sérica y del nitrógeno de urea Urea A compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids. Urea Cycle (BUN) en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum sangre
   • Puede manifestarse como hematuria Hematuria Presence of blood in the urine. Renal Cell Carcinoma, proteinuria Proteinuria The presence of proteins in the urine, an indicator of kidney diseases. Nephrotic Syndrome in Children u oliguria Oliguria Decreased urine output that is below the normal range. Oliguria can be defined as urine output of less than or equal to 0. 5 or 1 ml/kg/hr depending on the age. Renal Potassium Regulation

Tratamiento

El tratamiento del síndrome urémico hemolítico se realiza principalmente mediante terapias de soporte.

• Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types: Transfusiones de glóbulos rojos cuando estén indicadas (hemoglobina < 6 o 7 g/dL)
• Trombocitopenia: transfusiones de plaquetas solo en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum caso de hemorragia clínica significativa
• Lesión renal aguda:
  • Líquidos intravenosos
  • Control de electrolitos
  • Suspensión de cualquier medicamento nefrotóxico
  • Diálisis si es grave
• Hipertensión: antagonistas del calcio
• Nutrición: ingesta de calorías según las necesidades

Hay que tener en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum cuenta que el tratamiento con antibióticos durante las enfermedades diarreicas sanguinolentas causadas por bacterias productoras de toxina Shiga se asocia con un mayor riesgo de desarrollar síndrome urémico hemolítico.

Pronóstico

• El diagnóstico y el tratamiento temprano tienen un resultado favorable: mortalidad: < 5%
• Sin embargo, la mayoría recupera completamente la función renal:
  • El 30% queda con algún grado de insuficiencia renal.
  • El 5% queda dependiente de la diálisis.
• Cuando el síndrome urémico hemolítico no está asociado a diarrea, el pronóstico es más grave:
  • Síndrome urémico hemolítico relacionado con los LOS Neisseria neumococos:
    • 20% de mortalidad
    • El 80% de los LOS Neisseria pacientes requieren diálisis

Diagnóstico Diferencial

• Coagulación intravascular diseminada (CID): asociada a niños con enfermedades graves, como el shock Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock séptico. A diferencia del síndrome urémico hemolítico, la CID se presenta con estudios de coagulación anormales, incluyendo un tiempo de protrombina (TP) y un tiempo de tromboplastina parcial ( TTP TTP Thrombotic thrombocytopenic purpura (TTP) is a life-threatening condition due to either a congenital or an acquired deficiency of adamts-13, a metalloproteinase that cleaves multimers of von Willebrand factor (vWF). The large multimers then aggregate excessive platelets resulting in microvascular thrombosis and an increase in consumption of platelets. Thrombotic Thrombocytopenic Purpura) prolongados, una disminución del fibrinógeno y un aumento del dímero D.
• Púrpura trombocitopénica trombótica (PTT): se produce debido a la inhibición o deficiencia de ADAMTS13, que provoca una disminución de la degradación de los LOS Neisseria multímeros del factor von Willebrand, y un aumento de la agregación plaquetaria y la trombosis. Los LOS Neisseria análisis de laboratorio muestran esquistocitos, aumento del lactato deshidrogenasa ( LDH LDH Osteosarcoma) y parámetros de coagulación normales. Los LOS Neisseria síntomas incluyen una pentada clásica de síntomas neurológicos y renales, fiebre, trombocitopenia y anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types hemolítica microangiopática.
• Vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus sistémica: Los LOS Neisseria pacientes con vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus sistémica no presentarán un pródromo de diarrea asociado a la enfermedad. También es probable que presenten síntomas sistémicos, como artralgias y/o erupciones.