RESUMEN
Budd-Chiari syndrome can be defined as an interruption or diminution of the normal blood flow out of the liver. Patients with Budd-Chiari syndrome present with varying degrees of symptomatology that can be divided into the following categories: fulminant, acute, subacute and chronic. The subacute form is the most common presentation. A majority of patients with Budd-Chiari syndrome have an underlying hypercoagulability state. We present the case of a young woman with Crohn s disease on oral contraceptives who developed bilateral pulmonary thromboembolism and Budd-Chiari syndrome.
Asunto(s)
Síndrome de Budd-Chiari/complicaciones , Anticonceptivos Orales/efectos adversos , Enfermedad de Crohn/complicaciones , Etinilestradiol/efectos adversos , Embolia Pulmonar/inducido químicamente , Adulto , Síndrome de Budd-Chiari/diagnóstico por imagen , Colonoscopía , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/diagnóstico por imagen , Femenino , Heparina de Bajo-Peso-Molecular/uso terapéutico , Humanos , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/tratamiento farmacológico , Radiografía Abdominal , Radiografía Torácica , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
OBJECTIVE: To analyze the clinical characteristics, treatment, and follow-up of a cohort of 29 patients with Wilson's disease (WD) within the region of Murcia. PATIENTS AND METHOD: We reviewed the medical records of 29 cases of WD (mean age, 20.3 +/- 13.4 years) diagnosed during the last 16 years. RESULTS: The most frequent reason for consultation was upon discovering a high transaminase level in almost half the patients, followed by tremors or dystonia in 17% of patients, respectively. A Kayser-Fleischer ring was observed in 17/29 (58.6%) of patients (100% of patients with pure neurological involvement and 35% of patients with pure clinical hepatic disease; p < 0.001). Blood copper levels not associated with ceruloplasmin as well as cupruria were notably superior in patients with neurological symptoms and in those with liver cirrhosis at the time of diagnosis. Patient clinical symptoms remained stable with D-penicillamine or trientine, or improved during the observation period, for 18 out of 29 patients (62%), while 11 out of 29 patients (38%) got worse. CONCLUSIONS: In our region patients with WD are diagnosed at a younger age, and in most cases for hepatic disease. Patients with neurological disease or liver cirrhosis had a high level of free copper not associated to ceruloplasmin and cupruria. The disease had a favorable evolution in all patients but those diagnosed with hepatic disease or advanced neurological disease.