De novo autoimmune hepatitis following liver transplantation for primary biliary cirrhosis.

De novo autoimmune hepatitis (AIH) is a rare disorder first described in 1998. It appears in patients with liver transplants for non-AIH etiology. The few cases in the literature make it a little known entity due to mechanisms that are unclear. We present the case of a woman with a liver transplant whose graft developed de novo AIH. Diagnosed with primary biliary cirrhosis, the patient underwent liver transplantation for refractory pruritus. Sixteen months after transplantation, we detected alterations in the hepatic profile with hypertransaminasemia and elevated alkaline phosphatase, together with elevated antinuclear antibodies. IgG levels were normal. Histological findings indicated AIH. The patient responded rapidly to treatment with prednisolone and azathioprine. De novo AIH in patients transplanted for PBC may cause graft dysfunction. This report also analyzes the case with respect to the other four reported cases, discussing etiologic hypotheses.

Liquen plano vulvar en la infancia / Childhood vuvar lichen planus in children

El liquen plano (LP) es una dermatosis inflamatoria, no infecciosa, que puede afectar a piel, mucosas y anejos. Es infrecuente en niños, sobre todo en las formas de afectación de mucosas. Mientras que la historia natural del liquen plano cutáneo tiende a la remisión espontánea, las lesiones orogenitales y, en particular, la forma erosiva, suelen ser persistentes. La clínica del liquen plano vulvar puede comprender desde casos asintomáticos, hasta pacientes con prurito, gran dolor y quemazón vulvar. Puede autorresolverse, responder al tratamiento o bien progresar hacia una alteración de la estructura genital externa, con formación de sinequias e incluso estenosis vaginal. El tratamiento clásico consiste en la aplicación de corticoides tópicos de elevada potencia, requiriendo los casos complicados tratamientos más agresivos. Recientemente, se ha utilizado el tacrolimus como terapéutica del liquen plano y se han obtenido buenos resultados. Se presenta el caso de una niña prepuberal con liquen plano vulvar

Lichen planus is an noninfectious, inflammatory dermatosis that usually affects the skin, mucosa, hair and nails. It is uncommon in children, in whom mucosal involvement is especially rare. While cutaneous lichen planus eventually resolves spontaneously, the oral and genital lesions, in particular in the erosive form, are usually persistent. Vulvar lichen planus can be asymptomatic or can be accompanied by symptoms ranging from itching to intense pain and burning sensation of the vulvar region. It can resolve without any treatment or respond well to treatment, or its progression may lead to changes in the structure of the external genitalia, with synechia that may cause vaginal narrowing or closure. Highly potent topical corticosteroids are very useful. although complicated cases may require more aggressive treatments. Tacrolimus has recently been used with very good results

Traqueobronquitis necrotizante: descripción de dos casos / Necrotizing tracheobronchitis: a report of two cases

Se presentan dos niños diagnosticados de traqueobronquitis necrotizante. En ambos casos la enfermedad constituye una complicación grave de la ventilación mecánica y la presentación clínica se caracteriza por hipercapnia, pérdida de movimientos torácicos y episodios de obstrucción grave de la vía aérea que producen la muerte. La valoración histológica post mortem manifiesta una necrosis característica de la mucosa traqueal, compatible con el diagnóstico de traqueobronquitis necrotizante. Es necesario el reconocimiento de esta complicación yatrogénica en un niño ventilado cuando las condiciones respiratorias empeoran de forma drástica (AU)

Síndrome febril, poliadenopatías y hepatoesplenomegalia / Low-fever syndrome, multiple lymph node enlargement, and liver and spleen enlargement

No disponible

Paciente con hipercalcemia y tumor de células de los islotes pancreáticos (paratirinoma) / Patient with hypercalcemia and islet cell carcinoma of the pancreas (parathyrimoma)

No disponible

[Malformations associated with esophageal atresia]. / Malformaciones asociadas a la atresia de esófago: su influencia sobre la mortalidad. Estudio de 277 casos operados (1965-1981).

Associated malformations present in 277 newborn patients operated during the last sixteen years for esophageal atresia and tracheoesophageal fistula are described. Incidence on concomitant anomalies of each organ system is compared to that published by various authors during this period of time. Mortality, as related to each organ system and to the patient classification according to Waterston risk-groups, is studied during two different periods (1965-1975 and 1976-1981) considering in the second one a significant improvement of pre and postoperative care.