ABSTRACT
ABSTRACT Hepatopulmonary hydatidosis (HPH) is a very rare condition in children with a prevalence of 11%. An 8-year-old girl with advanced HPH was successfully treated in our institution without complications. The coexistence of large numbers of high hydatid cyst makes this case very unusual and interesting.
Subject(s)
Humans , Female , Adolescent , Appendicitis/etiology , Barium Sulfate/adverse effects , Contrast Media/adverse effects , SuppurationABSTRACT
Epidermolysis bullosa (EB) is an inherited, autosomal recessive, bullous disease, characterized by blisters followed with skin and mucosal erosions. We present a case of a male infant with pyloric atresia associated with junctional EB (Carmi syndrome). The patient underwent urgent laparotomy after prompt stabilization. Postoperative course was uneventful. Nine months later, the patient died in the paediatric intensive care unit from respiratory distress syndrome. Prognosis is usually very poor. Death usually occurs during the first year of life, as a result of septic complications.
La epidermólisis bullosa (EB) es una enfermedad hereditaria, autosómica recesiva, y bullar, caracterizada por ampollas acompañadas de erosiones de las mucosas y la piel. Presentamos el caso de un niño con atresia pilórica asociada con EB juntural (síndrome de Carmi). El paciente fue sometido a laparotomía urgente después de una rápida estabilización. Curso postoperatorio transcurrió sin incidentes. Nueve meses más tarde, el paciente murió en la unidad de cuidados intensivos pediátricos de síndrome de dificultad respiratoria (SDR). El pronóstico es generalmente muy pobre. La muerte ocurre generalmente durante el primer año de vida, como consecuencia de las complicaciones sépticas.