Respuesta terapéutica al ácido ursodesoxicólico en pacientes cubanos con colangitis biliar primaria / Therapeutic response to ursodeoxycholic acid in cuban patients with primary biliary cholangitis

Introducción: La colangitis biliar primaria es una enfermedad hepática, crónica y progresiva. El tratamiento con ácido ursodesoxicólico ha ampliado la esperanza de vida de estos pacientes. Objetivo: Describir la respuesta terapéutica al ácido ursodesoxicólico en pacientes con colangitis biliar primaria. Métodos: Estudio descriptivo, longitudinal y ambispectivo en pacientes atendidos en el Instituto de Gastroenterología entre septiembre de 2003 y enero de 2020. Se evaluaron variables clínicas, de laboratorio, histológicas y terapéuticas. El análisis de los resultados se realizó con el paquete SPSS. Resultados: Se incluyeron 45 pacientes. Hubo un predominio del sexo femenino (95,6 %) y una mediana de edad de 54 años. Los niveles bajos de aspartato amino transferasa (p=0,009 HR=0,98) y fosfatasa alcalina (p=0,005, HR=0,99), así como la presencia del síndrome de superposición (p=0,046 HR=3,08) se relacionaron con una buena respuesta al ácido ursodesoxicólico. La mayoría de los que no respondieron al tratamiento tenían cirrosis hepática (68 %). No se observaron diferencias en la supervivencia de los pacientes de acuerdo con su respuesta al tratamiento (p =0,585). Conclusiones: La respuesta terapéutica fue efectiva en menos de la mitad de los tratados con ácido ursodesoxicólico. La cirrosis hepática, el síndrome de superposición y los niveles elevados de aspartato amino transferasa y fosfatasa alcalina se asociaron a la mala respuesta terapéutica.

Introduction: Primary biliary cholangitis is a chronic and progressive liver disease. Treatment with ursodeoxycholic acid has extended the life expectancy of these patients. Objective: To describe the therapeutic response to ursodeoxycholic acid in patients with primary biliary cholangitis. Methods: Descriptive, longitudinal and ambispective study in patients treated at the Institute of Gastroenterology between September 2003 and January 2020. Clinical, laboratory, histological and therapeutic variables were evaluated. The analysis of the results was performed with the SPSS package. Results: Forty-five patients were included, with a predominance of female gender (95.6%) and a average age of 54 years. Low levels of aspartate amino transferase (p=0.009 HR=0.98) and alkaline phosphatase (p=0.005, HR=0.99), as well as the presence of overlap syndrome (p=0.046 HR=3.08) were associated with a better response to ursodeoxycholic acid. Less than half of the patients responded to conventional treatment with UDCA (47.7 %), most of the non-responders suffer from liver cirrhosis (68 %). No differences were observed in patient survival according to their response to treatment (p =0.585). Conclusions: Therapeutic response was effective in less than half of those treated with ursodeoxycholic acid. Liver cirrhosis, overlap syndrome, and elevated aspartate amino transferase and alkaline phosphatase levels were associated with poor therapeutic response.

Revision and update on clinical practice guideline for liver cirrhosis / 대한간학회지

No abstract available.

Afecciones hepatobiliares autoinmunes / Auto-immune hepatobiliary diseases

Las afecciones hepato-biliares autoinmunes son relativamente infrecuentes en la práctica clínica y es posible que una cierta proporción de ellas pasen inadvertida o se incluyan en el grupo de las hepatopatías criptogénicas. Histológicamente se caracterizan por una inflamación crónica, persistente y progresiva de las células y conductos intrahepáticos que es característica sólo en las etapas iniciales ya que en las avanzadas son ocultadas por la fibrosis propia de la cirrosis. Clínicamente se destacan las formas colestásicas con prurito, ictericia, compromiso del estado general. En las formas avanzadas aparecen las manifestaciones de insuficiencia hepática; la hipertensión portal y las neoplasias (hepatocarcinoma y colangiocarcinomas) que son menos frecuentes en estas hepatologías autoinmunes que en las por virus C. El tratamiento médico se basa en el empleo de corticoides y/o azatioprina en el caso de las hepatitis autoinmunes y de colestiramina o ácido ursodeoxicólico en la cirrosis biliar primaria y en la colangitis esclerosante.

Autoimmune hepatitis: a study of 50 patients.

INTRODUCTION: Autoimmune hepatitis (AIH) is a well-defined entity in the West but there are sparse Indian data on this disease. AIM: To study the clinical profile and response to treatment of Indian patients with AIH. METHODS: This is a part retrospective and part prospective study of 50 patients (median age 48 years, range 11-82; 43 women) seen between 1995 to 2001, diagnosed to have AIH as per the revised scoring system. Clinical and laboratory profile, response to treatment, and complications of treatment were analyzed. RESULTS: AIH accounted for 6% of all patients with liver disease seen during the period. The presenting symptoms were gastrointestinal in 43 and non-gastrointestinal in 7, with median symptom duration of 6 months (range 2 weeks to 40 years). Forty patients (80%) had chronic liver disease. Associated illnesses were present in 28 patients. Twenty-six patients were classified as definite and the rest as probable AIH. Forty-nine patients had Type 1 AIH. Five patients had overlap syndrome. Forty-five patients (90%) received immunosuppressive therapy. Twelve of 18 patients receiving only prednisolone and 21 of 27 patients receiving prednisolone and azathioprine combination responded. Thirteen (26%) patients had therapy-related complications (infectious 5, non infectious 8) with two treatment-related deaths. CONCLUSION: Type 1 AIH was the predominant type of AIH. The majority of patients with AIH presented with chronic liver disease. There was good response to immunosuppressive therapy. Therapy-related complications occurred in one-fourth of patients.

Uso de quercetina a longo prazo em ratos cirróticos / Long term use of quercetin in cirrhotic rats

OBJETIVO: Avaliar o uso a longo prazo do flavonóide quercetina em ratos cirróticos por ligadura de ducto biliar comum (LDB). MÉTODOS: Foram utilizados 32 ratos machos Wistar, sendo submetidos à LDB ou simulação, e distribuídos em 4 grupos: 1) controle, 2) cirróticos, 3) cirróticos tratados com quercetina 50mg/kg, intraperitonealmente, desde o segundo dia após o procedimento cirúrgico; e 4) cirróticos tratados após o décimo quarto dia do procedimento cirúrgico. Analisou-se a função hepática por meio de testes bioquímicos (BT e BD) e atividade enzimática (ALT, AST, FA e GGT). Na análise anatomopatológica, utilizou-se a coloração de Hematoxilina & Eosina (H&E) e de Picrosírius para fibrose. A análise estatística para avaliação de sobrevivência foi realizada pelo teste Kaplan-Meier. RESULTADOS: Os resultados de sobrevivência dos oito animais de cada grupo foram: Grupo 1 = 200 dias de sobrevivência; Grupo 2 = 46 dias; Grupo 3 = 71 dias; e o Grupo 4 = 90 dias. Nos animais com ligadura de ducto biliar comum houve aumento das provas de função hepática e enzimáticas que se reduziu hipoteticamente com o tratamento com quercetina. Foram identificadas cirrose, congestão vascular porta e centrolobular na análise histopatológica por H&E e Picrosírius. CONCLUSÃO: O uso da quercetina diminuiu de maneira significante as alterações bioquímicas provocadas pela cirrose, aumentando o tempo de sobrevivência dos animais com cirrose biliar secundária à LDB, como verificado pelo teste de análise de sobrevivência.

Mecanismos de acción y aplicaciones terapéuticas del ácido ursodeoxicólico en las enfermedades hepáticas colestásicas / Potential mechanisms of action and therapeutic applications of the ursodeoxycholic acid in cholestasis

El ácido ursodeoxicólico es un ácido biliar hidrofílico, que representa una pequeña fracción del pool de ácidos biliares. En las dos últimas décadas, numerosas enfermedades hepáticas colestásicas crónicas (cirrosis biliar primaria, colangitis esclerosante primaria, colestasia intrahepática del embarazo) han sido tratadas con AUDC. Sin embargo, hasta hoy su eficacia sólo ha sido demostrada en la cirrosis biliar primaria, siendo necesario realizar estudios que permitan definir claramente sus indicaciones. Su efecto es mediado por una disminución del daño de los ácidos biliares tóxicos retenidos sobre la membrana celular de los hepatocitos, a través de una estimulación de la secreción biliar, mejoría del flujo biliar y disminución del daño hepático mediado por el sistema inmune

Methotrexate therapy in primary biliary cirrhosis

OBJECTIVE: We investigated the effect of low-dose oral MTX in a group of patients with PBC. MATERIAL AND METHODS: Sixteen female with a mean age of 51.16 yrs were included in the present study. Criteria for entry into the trial were elevated serum alkaline phosphatase activity, liver biopsy findings diagnostic of or compatible with PBC, and a positive result for serum mitochondrial antibodies with a title > 1/80. Pts. In cirrhotic stage or jaundice were excluded. Ten pts were symptomatic. Pruritus and fatigue were present in 10 pts. At. Inclusion, 7 (43.75 per cent) presented with histological stage 1.6 (37.5 per cent) stage II and 3 (18.75 per cent) stage III (Scheuer). Control liver biopsy was performed on admission and were repeated at 2 years. All pts were treated with MTX 15 mg/wk given orally in three divided doses over 24 hours during 2 years. For statistical the Kruskal Wallis test was used. RESULTS: Fourteen pts completed 2 years of therapy. Two withdraw from treatment, 1 did so at month 2 due to the worsening of hepatocellular function and development of intense fatigue associated with an elevation of ALT to 30 times over the normal levels and another at month 4 because of interstitial pneumonitis. Pruritus resolved in 5 of the 10 symptomatic patients. All asymptomatic patients remained so during the trial. At the end of the study period, a decline in the following laboratory parameters were observed: Alkaline phosphatase (ALP), from 123 +/- 66 IU/L to 63 +/- 42 IU/L (p = 0.0064); gammaglutamyltransferase (GGT), from 197.3 +/- 121.5 IU/L to 137.2 +/- 56.9 IU/L (p = 0.3352); AST from 47.4 +/- 33 IU/L to 19 +/- 12.4 IU/L (p = 0.0899) and ALT from 54.6 +/- 35 IU/L to 21.12 +/- 5.5 IU/L (p = 0.0771). Liver histology status at the end of MTX therapy improved from baseline in only 3 (18.7 per cent) patients and progression was observed in 8 (50 per cent), to cirrhosis in 4, 3 of them with stage I and another with stage II at the begin of the trial. CONCLUSION: Our results do not support the introduction of MTX as monotherapy in the treatment of PBC. The rapid advance off the disease in some patients during the treatment observed in this cohort of pts merits a word of caution

Cirrose biliar primária: relato de um caso tratado com ácido ursodesoxicólico / Primary biliar cirrhosis: a case report of a treatment with ursodesoxicholic acid

A cirrose biliar primária é de incidência rara e pouco registrada no Brasil. O prurido persistente numa mulher de meia-idade, associado a colestase detectada em laboratório, levou-nos a hipótese da doença, confirmada histologicamente. O uso do ácido ursodesoxicólico teve resposta favorável, tanto no quadro clínico como laboratorial

Cirrose biliar primária / Primary biliary cirrhosis

O autor apresenta um caso de cirrose biliar primária seguido por quatro anos e meio. Houve neste tempo melhora e piora do quadro clínico. Há referência à boa resposta terapêutica a ciclofosfamida e azatioprina

Síndrome hepatocutáneo / Hepato-cutaneous syndrome

El síndrome hepato-cutáneo comprende la asociación de hepatopatías autoinmunes, cirrosis biliar primaria o hepatitis crónica activa y lesiones cutáneas de tipo prurigoide debidas a vasculitis alérgicas. La coexistencia con otras entidades de idéntica patogenica le haría formar parte de una enfermedad autoinmune multisistémica