Search

Gestational alloimmune liver disease, previously known as neonatal hemochromatosis, is characterized by severe liver disease in neonatal period, associated with intra and extrahepatic iron accumulation. It is postulated an alloimmune origin, which has opened new opportunities in the treatment and prevention during risk pregnancies, changing the prognosis of this pathology. We report the case of a newborn that presents early liver failure, with clinical and analytical features compatible with gestational alloimmune liver disease. Exchange transfusion was made and gamma globulins were given, with good clinical evolution.

Therapeutic hypothermia is the current standard treatment in newborns with moderate to severe hypoxic-ischemic encephalopathy, changing the outcome of these children. It is considered a safe technique with almost no side effects. A possible adverse side event is subcutaneous fat necrosis, which is an acute self-limiting panniculitis that develops during the first weeks of life. We report a case of a newborn at term suffering hypoxic-ischemic encephalopathy with a generalized multiform erythematous rash and firm and indurated plaques over the back, buttocks and extremities on his 12th day of life after being treated with therapeutic hypothermia. Histopathological study after skin punchbiopsy confirmed the suspicion of subcutaneous fat necrosis. The infant developed asymptomatic moderate hypercalcaemia within the first month of life, which was treated with intravenous fluids and diuretics. Serum calcium levels decreased and normalized in 3 months, with progressive disappearance of skin lesions.

Gestational alloimmune liver disease, previously known as neonatal hemochromatosis, is characterized by severe liver disease in neonatal period, associated with intra and extrahepatic iron accumulation. It is postulated an alloimmune origin, which has opened new opportunities in the treatment and prevention during risk pregnancies, changing the prognosis of this pathology. We report the case of a newborn that presents early liver failure, with clinical and analytical features compatible with gestational alloimmune liver disease. Exchange transfusion was made and gamma globulins were given, with good clinical evolution.La enfermedad hepática gestacional aloinmune, previamente conocida como hemocromatosis neonatal, se caracteriza por enfermedad hepática grave que se inicia en el período neonatal, asociada al acúmulo intra- y extrahepático de hierro. Se postula un potencial origen aloinmune, lo que ha abierto posibilidades en el tratamiento y en la prevención durante los embarazos de riesgo y ha cambiado el pronóstico de esta patología. Exponemos el caso de una recién nacida que presentó falla hepática precoz, con características clínicas y analíticas compatibles con enfermedad hepática gestacional aloinmune. Se realizó una exanguinotransfusión y se administró tratamiento con gammaglobulinas, con buena evolución posterior de la paciente.

Therapeutic hypothermia is the current standard treatment in newborns with moderate to severe hypoxic-ischemic encephalopathy, changing the outcome of these children. It is considered a safe technique with almost no side effects. A possible adverse side event is subcutaneous fat necrosis, which is an acute self-limiting panniculitis that develops during the first weeks of life. We report a case of a newborn at term suffering hypoxic-ischemic encephalopathy with a generalized multiform erythematous rash and firm and indurated plaques over the back, buttocks and extremities on his 12th day of life after being treated with therapeutic hypothermia. Histopathological study after skin punchbiopsy confirmed the suspicion of subcutaneous fat necrosis. The infant developed asymptomatic moderate hypercalcaemia within the first month of life, which was treated with intravenous fluids and diuretics. Serum calcium levels decreased and normalized in 3 months, with progressive disappearance of skin lesions.La hipotermia terapéutica constituye el tratamiento de elección para los recién nacidos con encefalopatía hipóxico-isquémica moderada-grave, que cambia el pronóstico de estos niños y se acepta como una técnica segura y con escasas complicaciones secundarias considerando su beneficio. Un posible efecto adverso es la necrosis grasa subcutánea, una paniculitis transitoria aguda de presentación durante las primeras semanas de vida. Se presenta un caso de un recién nacido a término afecto de encefalopatía hipóxico-isquémica con lesiones cutáneas en forma de eritema multiforme generalizado y placas eritematosas firmes e induradas en la espalda, los glúteos y las extremidades a los 12 días de vida, tras el tratamiento con hipotermia. El estudio histopatológico, tras la biopsia cutánea, confirmó la sospecha de necrosis grasa subcutánea. Al mes de vida, presentó cifras elevadas de calcio sérico, que precisaron tratamiento con hidratación intravenosa y diuréticos. La calcemia descendió hasta normalizarse a los 3 meses, con desaparición progresiva de las lesiones cutáneas.