Hiperbilirrubinemia Neonatal

Descripción General

Definición

La hiperbilirrubinemia neonatal se define como una coloración amarilla de la piel y la esclerótica del recién nacido, generalmente debida al AL Amyloidosis depósito tisular de bilirrubina no conjugada, el producto final del catabolismo de las hemoproteínas.

Epidemiología

• El 60% de los LOS Neisseria niños a término y el 80% de los LOS Neisseria bebés pretérmino tienen ictericia visible después del nacimiento.
• Más común 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 los LOS Neisseria asiáticos orientales y los LOS Neisseria nativos americanos

Etiología

Aumento de la bilirrubina indirecta (no conjugada)

Prueba de Coombs positiva: isoinmunización

• Incompatibilidad ABO
• Incompatibilidad del Rh
• Otras aloinmunizaciones

Prueba de Coombs negativa

• Policitemia:
  • Transfusión de gemelo a gemelo
  • Transfusión materno-fetal
  • Retraso 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 pinzamiento del cordón umbilical
  • Pequeño para la edad gestacional
• Hemoglobina baja/normal:
  • Aumento del recuento de reticulocitos con morfología anormal:
    • Deficiencia de glucosa-6-fosfato deshidrogenasa ( G6PD G6PD Pentose Phosphate Pathway)
    • Deficiencia de piruvato quinasa
    • Otra deficiencia enzimática
    • Coagulación intravascular diseminada
    • Esferocitosis
    • Eliptocitosis
    • Estomatocitosis
    • Picnocitosis
  • Recuento de reticulocitos elevado/normal con morfología normal:
    • Hemorragia cerrada (cefalohematoma)
    • Aumento de la circulación enterohepática (retraso 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 las evacuaciones, obstrucción intestinal)
    • Ingesta calórica inadecuada
    • Asfixia
    • Hipotiroidismo
    • Lactancia materna
    • Síndrome de Gilbert
    • Síndrome de Crigler-Najjar

Aumento de la bilirrubina directa (conjugada)

• Sepsis Sepsis Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by hypotension despite adequate fluid infusion, it is called septic shock. Sepsis and Septic Shock
• Infección intrauterina
• Trastornos de las vías biliares
• Obstrucción biliar
• Enfermedad hemolítica grave
• Quiste del colédoco
• Fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans quística
• Galactosemia Galactosemia Galactosemia is a disorder caused by defects in galactose metabolism. Galactosemia is an inherited, autosomal-recessive condition, which results in inadequate galactose processing and high blood levels of monosaccharide. The rare disorder often presents in infants with symptoms of lethargy, nausea, vomiting, diarrhea, and jaundice. Galactosemia
• Deficiencia de antitripsina alfa-1
• Tirosinemia
• Síndrome de Alagille
• Hepatitis

Factores de Riesgo

Principales factores de riesgo

• Características clínicas de alto riesgo antes del alta o niveles de bilirrubina sérica basados 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 nomograma de Bhutani
• Ictericia 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 las primeras 24 horas de vida
• Incompatibilidad del grupo sanguíneo materno-fetal y prueba de antiglobulina positiva
• Edad gestacional 35–36 semanas
• Fototerapia previa 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 hermanos
• Cefalohematoma o hematomas importantes
• Lactancia materna exclusiva, especialmente si la lactancia no marcha bien y la pérdida de peso es excesiva
• Trastorno hemolítico conocido 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 niño
• Origen étnico de Asia ASIA Spinal Cord Injuries oriental (deficiencia de G6PD G6PD Pentose Phosphate Pathway)

Factores de riesgo menores

• Ubicarse 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 zona de riesgo intermedio-alto 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 última lectura de bilirrubina antes del alta, según el nomograma de Bhutani
• Edad gestacional 37–38 semanas
• Ictericia observada antes del alta
• Hermano con ictericia previa
• Macrosomía inducida por la diabetes Diabetes Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus
• Edad materna ≥ 25 años
• Sexo masculino

Factores de riesgo adicionales

• Deshidratación
• Infección
• Retraso 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 paso del meconio
• Medicamentos y sustancias: oxitocina ( 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 madre), detergentes fenólicos ( 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 guardería)
• Policitemia
• Sepsis Sepsis Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by hypotension despite adequate fluid infusion, it is called septic shock. Sepsis and Septic Shock
• Acidosis Acidosis A pathologic condition of acid accumulation or depletion of base in the body. The two main types are respiratory acidosis and metabolic acidosis, due to metabolic acid build up. Respiratory Acidosis
• Baja albúmina sérica

Factores que disminuyen el riesgo

• Niveles de bilirrubina clínica o sérica de bajo riesgo antes del alta
• Edad gestacional ≥ 41 semanas
• Alimentación exclusiva con biberón
• Afroamericano
• Alta hospitalaria después de 72 horas

Clasificación

Hiperbilirrubinemia indirecta

• → no conjugada
• Fisiológica (siempre no conjugada o indirecta): debida a la mayor producción de bilirrubina causada por la descomposición de los LOS Neisseria eritrocitos del feto y la limitación transitoria del hígado para conjugar la bilirrubina
  • Se espera que aparezca después de las 1ras 24 horas y que alcance su pico para el día 2–4 con 5–6 mg/dL y que luego caiga a < 2 mg/dL al AL Amyloidosis final de la 1ra semana
  • Los LOS Neisseria niveles normales de bilirrubina a los LOS Neisseria 10–14 días son < 1 mg/dL
• Ictericia fisiológica exagerada (siempre no conjugada o indirecta):
  • Puede alcanzar niveles elevados de bilirrubina sérica total (> 15 mg/dL) 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 presencia de factores de riesgo
• Aumento de la circulación enterohepática de la bilirrubina
  • Ictericia por leche materna: etiología poco clara, observada 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 bebés alimentados exclusivamente con leche materna
• Ictericia por lactancia (fracaso de la lactancia): secundaria a una nutrición e hidratación inadecuadas
• Causas genéticas que provocan un aumento de la hemólisis de los LOS Neisseria eritrocitos
  • Estructural: esferocitosis hereditaria y eliptocitosis
  • Enzimática: deficiencia de G6PD G6PD Pentose Phosphate Pathway, deficiencia de piruvato quinasa
• Causas genéticas que conducen a una disminución del aclaramiento
  • Síndrome de Crigler-Najjar
  • Síndrome de Gilbert
• Sepsis Sepsis Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by hypotension despite adequate fluid infusion, it is called septic shock. Sepsis and Septic Shock

Hiperbilirrubinemia directa

• → conjugada
• Causas obstructivas
  • Atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) biliar
  • Quiste biliar
  • Colangitis esclerosante neonatal
• Causas infecciosas
  • Infecciones congénitas (patógenos TORCH)
  • Infecciones perinatales adquiridas
• Causas genéticas
  • Síndrome de Alagille
  • Deficiencia de antitripsina alfa-1
• Causas metabólicas
  • Galactosemia Galactosemia Galactosemia is a disorder caused by defects in galactose metabolism. Galactosemia is an inherited, autosomal-recessive condition, which results in inadequate galactose processing and high blood levels of monosaccharide. The rare disorder often presents in infants with symptoms of lethargy, nausea, vomiting, diarrhea, and jaundice. Galactosemia
  • Tirosinemia

Diagnóstico

Antecedentes

• Antecedentes familiares
  • Hermano con ictericia, esplenectomía
  • Cálculos biliares 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 familia sospechosa de enfermedad de células falciformes
• Antecedentes gestacionales/perinatales
  • Infecciones maternas
  • Uso de drogas ilícitas o de remedios herbales durante el embarazo
  • Antecedentes de traumatismos al AL Amyloidosis bebé durante el parto
• Antedecentes postnatales
  • Heces acólicas
  • Pérdida de peso anormal, especialmente cuando se alimenta con lactancia materna exclusiva
  • Administración de RhoGAM^®
  • Signos de infección

Examen físico

• General
  • Evaluar el tono y el nivel de actividad del neonato
  • Los LOS Neisseria bebés con hiperbilirrubinemia suelen estar somnolientos.
• Examen de la piel
  • La evaluación de la ictericia puede ser dificultosa bajo luz fluorescente.
  • Debe 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 la pigmentación natural del niño
  • La ictericia neonatal progresa cefalocaudalmente:
    • Regla general:
      • Ictericia 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 cara (aproximadamente 5 mg/dL)
      • Ictericia hasta la mitad del abdomen (aproximadamente 15 mg/dL)
      • Ictericia hasta las plantas (aproximadamente 20 mg/dL)
  • Evaluar también:
    • Cefalohematoma
    • Petequias
• Examen neurológico
  • Signos que requieren fototerapia inmediata:
    • Disminución del tono muscular
    • Convulsiones
    • Llanto alterado
• Otros hallazgos importantes: hepatoesplenomegalia, microcefalia, malformaciones congénitas, signos de deshidratación, infección, fiebre

Indicaciones para las pruebas de laboratorio

• Ictericia 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 las primeras 24 horas o ictericia que parece excesiva para la edad del bebé: comprobar la bilirrubina sérica total
• Considerar la evaluación del riesgo basada 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 nomograma de Bhutani
• Si la bilirrubina sérica total aumentan rápidamente o el bebé requiere fototerapia, compruebe:
  • Hemograma y frotis
  • Grupo sanguíneo y prueba de Coombs
  • Bilirrubina directa y repetir la bilirrubina sérica total 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 24 horas
  • Recuento de reticulocitos, G6PD G6PD Pentose Phosphate Pathway, albúmina, monóxido de carbono 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 espiración final (si está disponible), gases sanguíneos ( acidosis Acidosis A pathologic condition of acid accumulation or depletion of base in the body. The two main types are respiratory acidosis and metabolic acidosis, due to metabolic acid build up. Respiratory Acidosis)
• Bilirrubina directa elevada: realizar análisis de orina, urocultivo y estudio de sepsis Sepsis Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by hypotension despite adequate fluid infusion, it is called septic shock. Sepsis and Septic Shock
• Ictericia presente > 3 semanas: comprobar hipotiroidismo, tamizar la galactosemia Galactosemia Galactosemia is a disorder caused by defects in galactose metabolism. Galactosemia is an inherited, autosomal-recessive condition, which results in inadequate galactose processing and high blood levels of monosaccharide. The rare disorder often presents in infants with symptoms of lethargy, nausea, vomiting, diarrhea, and jaundice. Galactosemia y comprobar la bilirrubina directa

Tratamiento

• El objetivo del tratamiento de la hiperbilirrubinemia neonatal no conjugada es evitar la neurotoxicidad y prevenir el kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn.
• Fototerapia:
  • Tratamiento de primera línea para la hiperbilirrubinemia no conjugada
  • Se considera cuando los LOS Neisseria niveles de bilirrubina indirecta alcanzan el 50–70% del máximo
  • Provoca cambios conformacionales que convierten la bilirrubina 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 forma soluble lumirrubina, que se excreta 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 orina y la bilis
  • 420–470 nm son las frecuencias de luz que producen los LOS Neisseria mejores resultados.
  • Las complicaciones incluyen:
    • Deshidratación
    • Cambios térmicos ambientales → hipertermia (más común) o hipotermia (por exposición).
    • Síndrome del bebé bronceado ( 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 presencia de hiperbilirrubinemia directa)
    • Daños 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 córnea (si los LOS Neisseria ojos no están debidamente cubiertos)
• Inmunoglobulina intravenosa: 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 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 isoinmune que no responde a la fototerapia y que alcanza niveles máximos de bilirrubina indirecta
• Exanguinotransfusión:
  • Cuando la fototerapia no logra controlar la hiperbilirrubinemia
  • Cuando hay signos de kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn independientemente de los LOS Neisseria niveles de bilirrubina
  • 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 los LOS Neisseria niveles de bilirrubina que se acercan al AL Amyloidosis máximo para ese bebé, especialmente durante las 1ras 48 horas
  • 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 casos de hiperbilirrubinemia grave, 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 grave e hidropesía (se utiliza cuando la fototerapia y la inmunoglobulina intravenosa fallan)
• Tratamiento complementario: se fomenta la alimentación enteral, la reanimación con líquidos 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 los LOS Neisseria bebés deshidratados

Complicaciones

Disfunción neurológica inducida por la bilirrubina:

• Se produce cuando la bilirrubina atraviesa la barrera hematoencefálica, causando daños 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 los LOS Neisseria tejidos
• Efectos sutiles a largo plazo que causan trastornos 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:
  • Visión
  • Audición
  • Habla
• Encefalopatía aguda por bilirrubina:
  • 1ras 48 horas: letargo, mala alimentación, pérdida del reflejo de Moro, aspecto cada vez más enfermo, dificultad respiratoria
  • Mitad de la 1ra semana: hipertonía extensora, opistótonos y fiebre
  • Después de la 1ra semana: hipertonía
• Encefalopatía bilirrubínica crónica ( kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn):
  • 1er año: hipotonía, aumento de los LOS Neisseria reflejos tendinosos profundos, retraso 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 motricidad
  • Después del 1er año: coreoatetosis, balismo, temblor, mirada hacia arriba, pérdida de audición neurosensorial