Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I.

Patients with primary hyperoxaluria type I (PH I) are prone to develop early kidney failure. Systemic deposition of calcium-oxalate (CaOx) crystals starts, when renal function declines and plasma oxalate increases. All tissue, but especially bone, heart and eyes are affected. However, liver involvement, as CaOx deposition or chronic hepatitis/fibrosis has never been reported. We examined liver specimen from 19 PH I patients (aged 1.5 to 52 years at sample collection), obtained by diagnostic biopsy (1), at autopsy (1), or transplantation (17). With polarization microscopy, birefringent CaOx crystals located in small arteries, but not within hepatocytes were found in 3/19 patients. Cirrhosis was seen in one, fibrosis in 10/19 patients, with porto-portal and nodular fibrosis (n = 1), with limitation to the portal field in 8 and/or to central areas in 5 patients. Unspecific hepatitis features were observed in 7 patients. Fiber proliferations were detectable in 10 cases and in one sample transformed Ito-cells (myofibroblasts) were found. Iron deposition, but also megakaryocytes as sign of extramedullary erythropoiesis were found in 9, or 3 patients, respectively. Overall, liver involvement in patients with PH I was more pronounced, as previously described. However, CaOx deposition was negligible in liver, although the oxalate concentration there must be highest.

Monitoreo de parámetros cardiorespiratorios durante la endoscopia digestiva superior / Superior digestive endoscopy during cardiospiratory parameter

La endoscopia Digestiva Superior (EDS), actualmente con la técnica de video-color incorporada, es un procedimiento de uso rutinario para la visualización y diagnóstico de patología del tracto digestivo superior. Este procedimiento se realiza a pacientes de todas las edades, incluyendo a pacientes de edad avanzada. Sin embargo no se ha enfatizado mucho en el monitoreo de algunos importantes parámetros hemodínámicos de rutina, en estos pacientes ancianos, con o sin enfermedad cardiorespiratoria. Nosotros estudiamos a 47 pacientes de diferente sexo y grupo etareo, a quienes se practicó la endoscopia digestiva superior sin uso de drogas sedantes. Se monitorizó la saturación de oxígeno arterial (HbO2Sat), la tensión arterial (TA) y la frecuencia cardíaca (FC), antes, durante y después del procedimiento. No se presentaron alteraciones de significación con la HbO2-Sat. Algunos pacientes presentaron elevación de la TA y FC y otros tuvieron un comportamiento contrario. Estos cambios observados son de mayor significación en pacientes ancianos con enfermedad cardiovascular previa. Nosotros sugerimos el monitoreo cardiorespiratorio en pacientes de edad avanzada, quienes van a ser sometidos a un procedimiento de EDS