Falla hepática aguda sobre crónica (ACLF), síndrome subdiagnosticado con alta mortalidad / Acute-on-chronic liver failure (ACLF), an underdiagnosed syndrome with high mortality

Acute on chronic liver failure is an increasingly recognized syndrome characterized by acute decompensation of chronic liver disease associated with organ failure and high short-term mortality. ACLF is frequent, affecting between 24 and 40% of patients admitted for complications of cirrhosis. Sepsis, active alcoholism, and relapse of chronic viral hepatitis are the most frequent precipitating factors. However, in up to 40%­50% of the cases of ACLF have no identifiable trigger. The stage of severity of Acute on chronic liver failure is very important because it allows us to stratify patients according to their prognosis, evaluate therapeutic response, determine transplant urgency, deciding intensive care unit admission, and also have a basis on which to decide therapeutic futility. (AU)

Enfermedad hepática por alcohol / Alcoholic liver disease

Excessive alcohol consumption is an important cause of preventable morbidity and mortality. We have to bealert to chronic alcohol usage or abuse. Simple tests (AUDIT, CAGE) can be applied quickly on outpatientcare. We highlight advances in understanding the immune and molecular mechanisms; there is disruptionof the intestinal barrier with bacterial translocation, as well as endotoxins which activate the liver's innateimmunity, causing apoptosis, necrosis, inflammation and fibrosis. Alcoholic hepatitis is most common inpatients between 40 and 60 years of age, preferably male with jaundice, fever, ascites, hepatomegaly. Thediagnosis is confirmed with a history of alcoholic consumption, mild to moderate AST and ALT values,a AST/ALT ratio > 2, hyperbilirrubinemia and prolonged prothrombin time. There are scores to evaluatethe severity and the need of corticoid therapy, such as modified Maddrey discriminating function andMELD score. Lille score assesses the response to treatment in the seventh day. The risks and benefits ofliver biopsy should be evaluated individually. The cornerstone of treatment remains alcohol abstinence.Nutritional management must be carefully monitored. Proteins requirements are standardized based onweight. The use of corticoids with 40 mg of prednisolone each day is the most widely accepted therapy,indicated on patients with MMDF higher than 32 or MELD score higher than 21. If Lille score is higherthan 0.45 at the seven day under corticoid therapy, treatment must be interrupted. The use of pentoxifyllinewould only be effective for prevention of hepatorenal syndrome...

El consumo excesivo de alcohol es una causa importante de morbimortalidad prevenible. Debemos estaratentos en detectar a pacientes con dependencia o abuso crónico de alcohol. Test sencillos (AUDIT, CAGE)pueden aplicarse rápidamente en consulta ambulatoria. Destacamos avances en el conocimiento moleculare inmunológico, existe disrupción de la barrera intestinal con translocación bacteriana y endotoxinas conactivación del sistema inmune innato del hígado, produciendo apoptosis celular, necrosis e inflamación yfibrosis. La hepatitis alcohólica se presenta principalmente en pacientes entre 40 y 60 años, preferentementeen varones con ictericia, fiebre, ascitis, hepatomegalia. El diagnóstico se confirma con antecedentes deingesta alcohólica, GOT y GPT elevadas en forma leve o moderada, relación GOT/GPT mayor de 2, hiperbilirrubinemiay tiempo de protrombina prolongado. Existen scores para evaluar la gravedad y necesidad demanejo con corticoides como función discriminante de Maddrey modificada y MELD. El puntaje de Lilleevalúa respuesta del tratamiento al séptimo día. El riesgo/beneficio de la biopsia hepática se evalúa caso acaso. La piedra angular del tratamiento sigue siendo la abstinencia. Manejo nutricional debe ser riguroso.Requerimientos proteicos están estandarizados por peso. La terapia con corticoides (prednisolona 40 mg/día) es la más ampliamente aceptada, con indicación en pacientes con FDMm mayor a 32 o MELD mayora 21. Si el puntaje de Lille es mayor de 0,45 a los 7 días con corticoides, deben suspenderse. Pentoxifilinasólo tendría efecto en prevenir el desarrollo de síndrome hepatorrenal (SHR). Hay nuevas terapias enevaluación, como el uso de G-CSF...

Ligadura endoscópica, una nueva opción terapéutica para la ectasia vascular antral: presentación de dos casos clínicos / Endoscopic band ligation, for gastric antral vascular ectasia: Report of two cases

Gastric antral vascular ectasia is an uncommon cause of chronic anemia, occasionally associated with cirrhosis. The most accepted therapy is argon plasma coagulation (APC), however there are refractory cases. We report two females with cirrhosis, aged 60 and 72 years, in whom management with APC was insufficient and in whom the need for hospital admissions and transfusions were reduced using the technique of endoscopic band ligation.

Espectro clínico de la hepatotoxicidad por nitrofurantoína en serie clínica de 12 pacientes / Clinical spectrum of nitrofurantoin hepatotoxicity in a series of 12 patients

Nitrofurantoin, commonly used for prolonged periods, can produce different patterns of liver damage. Patients: 12 women, mean age 55 years (range 17-72), with recurrent urinary infections, treated with nitrofurantoin for long periods of time (2 months to 15 years), who presented with secondary liver disease. Results: 7 had acute hepatitis (3 fulminant), 3 chronic hepatitis, and 2 cirrhosis. All acute cases had consistent liver biopsies, and 2 were treated with steroids and azathioprine for 2 and 7 months, with liver tests normalization. Two fulminant cases were transplanted (submassive hepatic necrosis on explanted livers) and 1 was successfully treated with steroids and mycofenolate. The 3 cases of chronic hepatitis also had confirmatory biopsies and 1 received steroids and azathioprine, with full recovery. The other 2 responded to the drug withdrawal and the 2 cirrhotic patients had only symptomatic treatment. All patients were negative for hepatitis virus, 7 (58 percent had positive anti-nuclear and/or anti-smooth muscle antibodies and 4 (33 percent) had elevated IgG levels. Conclusions: Nitrofurantoin may cause severe acute liver disease, even requiring liver transplantation. Nitrofurantoin can also cause chronic liver disease, have markers of autoimmunity and respond to immunosuppressive therapy. These data confirmed that nitrofurantoin can induce liver diseases, probably due to immunological mechanisms.

La nitrofurantoína, comúnmente utilizada por períodos prolongados, puede producir daño hepático, con diferentes formas de presentación y evolución. Pacientes: 12 mujeres, edad promedio 55 años (rango 17 a 72), con infecciones urinarias recurrentes, usuarias de nitrofurantoína por períodos prolongados (2 meses a 15 años), que presentaron daño hepático asociado a la droga. Resultados: 7 casos de hepatitis aguda (3 fulminantes), 3 casos de hepatitis crónica y 2 casos de cirrosis. Todos los casos de hepatitis agudas tenían biopsia hepática compatible y 2 fueron tratadas con corticoides y azatioprina por 2 y 7 meses, con normalización de los exámenes. De las 3 pacientes con hepatitis fulminante, 2 fueron trasplantadas (necrosis submasiva en el hígado explantado) y 1 fue tratada con corticoides y micofenolato, con buena respuesta. Los 3 casos de hepatitis crónica tenían confirmación histológica y 1 se trató con corticoides y azatioprina, con excelente evolución. Las otras pacientes respondieron favorablemente sólo a la suspensión del fármaco. Los 2 casos con cirrosis han recibido tratamiento sintomático. Todas las pacientes fueron negativas para los virus hepatitis, 7/12 (58 por ciento) tenían anticuerpos antinucleares y/o antimúsculo liso positivos y 4/12 (33 por ciento) IgG elevada. Conclusión: La nitrofurantoína puede provocar una severa enfermedad hepática aguda, requiriendo incluso trasplante hepático. Además, puede producir hepatitis crónica y cirrosis, tener marcadores de autoinmunidad y buena respuesta a la terapia inmunosupresora habitual. Lo anterior confirma su capacidad de inducir un daño hepático, probablemente por mecanismos inmunológicos.

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia / Primary biliary cirrhosis: a thirteen years experience

Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

Hepatitis crónica por virus C: factores asociados a la severidad del daño histológico / Factors associated with the severity of liver damage in chronic hepatitis C

Background: Twenty percent of patients with chronic hepatitis C evolve to cirrhosis in 10 to 20 years. The degree of steatosis and hepatic iron stores in liver biopsy increase the risk. Age, high body mass index, diabetes mellitus and alcohol consumption are factors associated to the severity of liver damage. Aim: To study the association of steatosis and increased iron stores in the liver biopsy and age, overweight, alcohol consumption and diabetes with the severity of liver damage in patients with hepatitis C virus infection. Patients and methods: Retrospective study of 84 liver biopsies of patients with chronic infection with hepatitis C virus were studied. The pathological appearance was classified as stage I when chronic hepatitis with mild activity without fibrosis was observed; as stage II when moderate chronic hepatitis with mild fibrosis was observed and as stage III when there was a moderate chronic hepatitis with fibrosis or cirrhosis. The amount of steatosis and iron deposition in the biopsy were also assessed. Results: Forty one percent of patients were in stage I, 32% in stage II and 27% in stage III. Patients in stage I were younger than those in stages II and III (40.7 and 52.2 years respectively, p <0,001). No association between the severity of liver damage and the degree of steatosis, hemosiderosis, body mass index or alcohol intake, was observed. The frecuency of diabetes mellitus increased along with pathological staging (3, 15 and 30% in stages I, II and III, respectively, p <0,05). Conclusions: This study confirms that severity of chronic hepatitis C is associated with age and the presence of diabetes mellitus.

Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia / Primary sclerosing cholangitis: A twelve-year experience

Background:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of patient with PSC seen in two reference centers. Patients and methods: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. Results: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. Conclusions: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseases.

Trombosis venosa mesentérica: manifestaciones clínicas, terapia y evolución / Mesenteric vein thrombosis: clinical manifestations, treatment and outcome

Background: Mesenteric vein thrombosis (MVT) is uncommon and accounts for 5-10percent of all mesenteric ischemic events. In 80percent of cases, an etiologic factor is found. The clinical presentation varies and the diagnosis is made based on imaging studies. The treatment involves anticoagulation alone or in combination with surgery. Aim: To describe the clinical characteristics of patients with MVT. Patients and methods: Retrospective and prospective review of all cases with MVT, treated between 1995-2001. The clinical presentation, imaging studies, treatment and outcome were evaluated. Results: 29 cases of MVT were reviewed (14 females, age 56 ± 15 years). Twenty two patients (76percent) had recognizable risk factors. The main symptoms were abdomianl pain (86percent) and vomiting (55percent). The diagnosis of acute mesenteric ischemia was suspected on admission only in 6 patients (21percent). Thirteen patients underwent transabdominal color Doppler ultrasonography and the diagnosis was confirmed for 11 of these (85percent). Twenty out of 24 patients (85percent) studied with computed tomography, had positive signs of MVT. Twenty one patients (72percent) recieved anticoagulation, 10 of whom also underwent surgery. Four patients (14percent) received surgical treatment alone. Four patients were not treated. Seven patients (24percent) died. Conclusions: MVT is difficult to identify. It is necessary to have a high degree of suspicion in patients who have risk factors. The diagnosis is made with imaging studies. The treatment consists of early anticoagulation and surgical intervention when indicated.

Nuevos virus de hepatitis / New hepatitis viruses

Desde 1990 se han descrito nuevos virus responsables de hepatitis que hasta ese momento eran catalogadas como No A- No B. El virus de la hepatitis E fue descrito como virus transmisión entérica responsable de brotes epidémicos en Asia y México, documentados retrospectivamente. Es un virus RNA, de la familia Caliciviridae, que produce un cuadro similar al virus de la hepatitis A y no produce infección ni partación crónica. Posterior a 1995 se han identificado por técnicas de genéticas molecular nuevos virus, teóricamente responsables de hepatitis post-transfuncional como el virus de la hepatitis G y el virus TT, pero aún no existe certeza de su verdadero rol patogénico