Hepatic encephalopathy: nomenclature, pathogenesis and treatment.

Hepatic encephalopathy (HE) is a neuropsychiatric syndrome in patients with liver failure and/or a portal-systemic bypass. Since 2002 a new nomenclature of HE exists, that classifies HE in encephalopathy type A (associated with acute liver failure), type B (associated with portal-systemic bypass), and type C (associated with liver cirrhosis). HE type A is characterized by a rapid development to coma, cerebral edema, and a poor short-term prognosis. Therefore, these patients should be referred to a liver transplantation center. Standard treatment of HE consists of non absorbable disaccharides, non absorbable antibiotics, and a diet with an appropriate amount of proteins. In addition, the possibility of performing a liver transplantation should be evaluated. In patients with intractable HE other alternative treatments adjunct to standard treatment, like zinc, sodium benzoate, ornithine aspartate, branched chain amino acids, flumazenil, and bromocriptine should be considered.

La encefalopatía hepática: terminología, etiología y tratamiento / Hepatic encephalopathy: nomenclature, pathogenesis and treatment

La encefalopatía hepática (EH) es un síndrome neuropsiquiátrico que aparece como complicación en pacientes con una insuficiencia hepática y/o con una derivación portosistémica. A partir de 2002 existe una nueva terminología de la EH, que la organiza en encefalopatía de tipo A (asociada con una insuficiencia hepática aguda), de tipo B (asociada con una derivación portosistémica), y de tipo C (asociada con una cirrosis hepática). La encefalopatía de tipo A se caracteriza por una rápida evolución a coma, edema cerebral y un mal pronóstico a corto plazo, por lo que estos pacientes deben ser referidos a un centro de trasplante hepático. El tratamiento estándar de la EH consiste en disacaridasas no absorbibles, antibióticos no absorbibles, una dieta con una cantidad adecuada en proteínas, y también se deben evaluar la posibilidad de un trasplante hepático. En pacientes con una EH refractaria se deben considerar otros tratamientos alternativos, junto con el tratamiento estándar, como el zinc, el benzoato de sodio, la ornitina aspartato, los aminoácidos de cadena ramificada, el flumazenilo, y la bromocriptina (AU)

Is it a medical error if we do not screen cirrhotic patients for minimal hepatic encephalopathy?

Minimal hepatic encephalopathy (MHE) refers to subtle neurocognitive and neurophysiological defects in patients with liver cirrhosis without clinical signs of hepatic encephalopathy. Using appropriate diagnostic methods the prevalence of MHE is approximately 25-30%. MHE has clinical significance as it results in a diminished daily functioning, precedes overt hepatic encephalopathy and is associated with a poor prognosis. Treatment with non-absorbable disaccharides can reverse the neurocognitive and neurophysiological defects found in MHE. The failure to diagnose MHE in apparently normal cirrhotic patients could, therefore, be considered a medical error. However, whether treatment also improves patients' quality of life and prognosis remains to be determined.

[Hyperammonemia in hydronephrosis]. / Hyperammoniëmie bij hydronefrose.

A man aged 81 with bilateral hydronephrosis presented with lethargy deepening into coma caused by hyperammonaemia. The hyperammonaemia was most likely caused by excessive absorption of ammonia produced by Proteus mirabilis in the obstructed and dilated urinary tract. The patient died within a few hours.