Uso de corticosteroides no tratamento da lesão hepática aguda grave decorrente de anabolizante estanazolol / Use of corticosteroids in the treatment of stanazolol-induce severe acute liver injury

A busca pelo corpo perfeito pode gerar graves consequências para a população que faz uso indiscriminado de substâncias visando a resultados rápidos. O caso relatado se refere a um pa- ciente de 21 anos, do sexo masculino, na cidade de São Paulo (SP), que apresentou quadro de síndrome colestática 15 dias após uso do anabolizante estanazolol para fins estéticos na ativi- dade física, evoluindo com hepatite medicamentosa grave, com aumento de transaminases, hiperrubilinemia às custas de bilirrubina direta e fatores de coagulação, sem resposta satis- fatória ao tratamento de suporte convencional, com melhora significativa após introdução de corticoterapia.

Searching for the perfect body image can cause severe conse- quences to the population using substances indiscriminately to reach results fast. The case reported refers to a male patient, 21 years old, from the city of São Paulo (SP), who developed choles- tatic syndrome 15 days after the use of the steroid Stanazol for aesthetic purposes during physical activity, progressing with se- vere drug-induced hepatitis, transaminases, bilirubin, and coagu- lation factors increase with no satisfactory response to the con- ventional support treatment, and significant improvement after the introduction of corticotherapy.

Ursodeoxycholic acid in the prevention of gallstones in patients subjected to Roux-en-Y gastric bypass

Abstract Purpose: To evaluate the contribution of ursodeoxycholic acid (UDCA) in the first 12 months after Roux-en-Y gastric bypass in the prevention of gallstone formation. Methods: A community-based clinical trial was conducted. A total of 137 patients were included in the study; 69 were treated with UDCA, starting 30 days after the surgery, at a dose of 150 mg twice daily (300 mg/day) over a period of 5 consecutive months (GROUP A), and 68 were control patients (GROUP B). The patients were followed-up, and ultrasonography was performed to determine the presence of gallstones at various times during follow-up. Demographic, anthropometric and comorbid indicators were obtained. The data were subjected to normality tests and evaluated using appropriate tests. Results: Patients did not differ in their baseline characteristics. Of the 69 patients who used UDCA, only one patient developed cholelithiasis (1%), whereas 18 controls (26%) formed gallstones (OR = 24.4, p <0.001). Also, other factors were found not to influence the formation of calculi, such as pre-operative or postoperative hepatic steatosis or diabetes (p = 0.759, 0.468, 0.956). Conclusion: The results demonstrated that patients who did not use UDCA showed a 24.4-fold greater probability of developing cholelithiasis.

Comparison on the Efficacy and Safety of Biphenyl Dimethyl Dicarboxylate and Ursodeoxycholic Acid in Patients with Abnormal Alanine Aminotransferase: Multicenter, Double-blinded, Randomized, Active-controlled Clinical Trial / 대한소화기학회지

BACKGROUND/AIMS: Chronic hepatocellular damage is closely associated with hepatic fibrosis and fatal complication in most liver diseases. The aim of this study is to compare the efficacy and safety of biphenyl dimethyl dicarboxylate (DDB) and ursodeoxycholic acid (UDCA) in patients with abnormal ALT. METHODS: One-hundred thirty-five patients with elevated ALT were randomized to receive either 750 mg/day of DDB or 300 mg/day of UDCA for 24 weeks in 4 referral hospitals. Ninety-three (69%) patients had non-alcoholic steatohepatitits, 27 (20%) had alcoholic hepatitis, and 15 (11%) had chronic hepatitis. The primary end point was the rate of ALT normalization at week 24. The secondary endpoints were changes in AST, liver stiffness, and the incidence of adverse events. RESULTS: A total of 101 patients completed 24 weeks of therapy. ALT normalization at week 24 was observed in 44 (80.0%) patients in DDB group and 16 (34.8%) in UDCA group (p<0.001). Higher mean reduction of ALT levels from baseline to 24 weeks was seen in DDB group compared with UDCA group (-70.0% vs. -35.9%, p<0.001). Normalization of AST level (p=0.53) and change in the liver stiffness (p=0.703) were not significantly different between the two groups. Severe adverse drug reaction occurred in 1 patient in DDB group but the subject continued therapy during the study period. CONCLUSIONS: DDB was not inferior to UDCA for normalizing ALT level. Furthermore it was safe and well tolerated by patients with abnormal ALT.

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

No abstract available.

Biliary Cast Syndrome in Non-Liver Surgery Patients / 대한소화기학회지

Biliary cast describes the presence of casts within the biliary tree. It is resultant sequel of cholangitis and hepatocyte damage secondary to bile stasis and bile duct injury. Biliary cast syndrome was first reported in patient undergone liver transplantation. The pathogenesis of biliary cast is not clearly identified, but proposed etiologic factors include post-transplant bile duct damage, ischemia, biliary infection, or post-operative biliary drainage tube. Although biliary casts are uncommon, most of biliary cast syndrome are reported in the liver transplant or hepatic surgery patients. A few reports have been published about non-transplant or non-liver surgery biliary cast. We report two cases of biliary cast syndrome in non-liver surgery patients.

The diagnosis and treatment of primary biliary cirrhosis / 대한간학회지

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is various from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibody and histologic findings of nonsuppurative destructive cholangitis. Although the diagnosis is straightforward, it could be underdiagnosed because of its asymptomatic presentation, or underrecognition of the disease. UDCA in a dose of 13-15 mg/kg is the widely approved therapy which can improve the prognosis of patients with PBC. However, one-third of patients does not respond to UDCA therapy and may require liver transplantation. Every effort to diagnose PBC in earlier stage and to develop new therapeutic drugs and clinical trials should be made.

Colestasia intrahepática familiar progresiva tipo 3: presentación de casos clínicos y actualización de tema / Type 3 familial intrahepatic cholestasis: clinical cases and update

Abstract: Familial Intrahepatic Cholestasis (FIC) includes a heterogeneous group of recessive autosomic alterations characterized by hepatocellular cholestasis secondary to the interruption of the normal process of synthesis of bilis. Objective: A description of FIC in 3 of 5 children of an index family. Clinical case: a 5 y.o. child with hepatosplenomegaly increased serum hepatic enzymes and biliary acids. Abdominal echography showed alterations compatible with hepatic fibrosis. Biopsy showed bridge fibrosis, duct proliferation, minimal chronic cholestasis. These findings were compatible with a phenotype FIC-3 with elevate levels of Gamma-glutamyl transferase. A mutation of MDR3 gene is responsible for the absence of biliary phospholipids, allowing a detergent effect of biliary acids upon the duct epithelium, developing cholangitis, fibrosis and later cirrhosis. Among four brothers, the mutation was found in two twin sisters. Three affected brothers were treated with ursodeoxicolic acid, 30 mg/Kg. Excellent results were obtained in the twin girls not in the index boy. The clinical expression of this illness is variable, and an elevation of aminotransferase must call attention to this possibility. Early diagnostic and treatment could avoid the development of hepatic damage and cirrhosis.

La Colestasia Intrahepática Familiar Progresiva (CIFP) comprende un grupo heterogéneo de alteraciones autosómicas recesiva caracterizadas por una colestasia hepatocelular secundaria a una interrupción del proceso normal de síntesis de la bilis. Objetivo: Describir la presentación de CIFP en 3 de 5 hijos de una familia estudiada. Caso clínico: Paciente de 5 a±os de edad (caso 1), que presenta una hepatoesplenomegalia, aumento de enzimas hepáticas y de ácidos biliares en suero. La ecotomografía abdominal describe alteraciones compatibles con fibrosis hepática. La biopsia reveló fibrosis en puente, proliferación ductular y colestasia crónica mínima. Estos hallazgos fueron compatibles con el fenotipo de una CIFP-3 con niveles elevados de Gamaglutamiltransferasa (GGT). Una mutación del gen MDR3 es responsable de la ausencia de fosfolípidos en la bilis, lo que permite la acción detergente de los ácidos biliares sobre el epitelio de los conductos desencadenando una colangitis, fibrosis y luego cirrosis. De los cuatro hermanos del caso 1 se detectó la enfermedad en 2 hermanas gemelas (casos 2 y 3). Estos tres niños afectados fueron tratados con ácido ursodeoxicólico 30 mg/kilo/peso, logrando excelentes resultados en las gemelas pero no en el caso 1. Conclusión: Se presenta a 3 hermanos con el fenotipo de CIFP. La expresión clínica de esta enfermedad puede ser variable y de manifestación tardía, la elevación de las aminotransferasas debe considerar esta patología en el diagnóstico diferencial de las numerosas causas que dan origen a un aumento de estas enzimas. Sólo el diagnóstico y tratamiento precoz puede evitar la evolución a un daño hepático irreversible como es la cirrosis.

¿Mejora el uso de ácido ursodeoxicólico el pronóstico perinatal en mujeres embarazadas con colestasia intrahepática del embarazo? / Ursodeoxycholic acid in the treatment of intrahepatic cholestasis of pregnancy

La colestasia intrahepática del embarazo (CIÉ) es un cuadro clínico caracterizado por prurito palmo plantar de predominio nocturno y elevación de ácidos biliares conjugados séricos en el tercer trimestre del embarazo. Esta patología puede asociarse a ictericia y complicaciones como la muerte fetal. Aunque el manejo obstétrico de la CIÉ es eminentemente clínico, el ácido ursodeoxicólico (UDCA) ha sido utilizado efectivamente en el tratamiento sintomático de esta patología y en la corrección de los marcadores bioquímicos de la enfermedad. Además se ha sugerido que su uso estaría asociado a una disminución de las complicaciones fetales. Esta revisión tiene por objeto verificar la validez del uso de UDCA para mejorar el pronóstico fetal. Para esto se realizó una búsqueda detallada de la bibliografía médica en diferentes bases de datos. Aplicando distintos criterios se seleccionaron finalmente 4 artículos que constituyen la base de esta revisión. Fundamentados en la evidencia, se concluye que no existen datos suficientes en la literatura que apoyen el uso de UDCA para mejorar en forma efectiva los resultados perinatales. A pesar de que algunos estudios evaluados proponen un mejor desenlace fetal en pacientes tratados con UDCA, éstos no cuentan con la validez y el poder estadístico necesario para modificar la conducta actual frente a esta patología.

Intrahepatic cholestasis of pregnancy (ICP) is a disease of the third trimester of pregnancy involving pruritus and elevated bile acid levels. Once thought to be benign for both mother and fetus, ICP has been associated with maternal jaundice and increased rates of fetal morbidity and mortality. However ursodeoxycholic acid (UDCA) has proved to be effective and safe in patients with ICP, attenuating pruritus and correcting some biochemical abnormalities in the mothers. The fetal outcome has also been suggested to improve in patients receiving UDCA. This review intends to verify the validity of the UDCA uses to reduce fetal morbidity and mortality. For this a detailed search of the medical bibliography was done in different data bases. Applying different criteria, 4 articles were finally selected and this constitutes the base of our review. Based on the research, one concludes that there is not enough evidence in the literature to support the use of UDCA to improve perinatal outcome. Although some studies propose an improved fetal outcome in patient using UDCA, these do not count with the validity and the statistical power to modify the present management of ICP.

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.

Primary Biliary Cirrhosis / 대한간학회지

Primary biliary cirrhosis (PBC) is a chronic cholestatic autoimmune liver disease that predominantly affects middle-aged women. It is characterized by slowly progressive destruction of the small intrahepatic bile ducts together with portal inflammation, and this initially leads to fibrosis and later to cirrhosis. It is currently accepted that the pathogenesis of PBC is multifactorial with genetic and environmental factors interplaying to determine the disease onset and progression. In addition to antimitochondrial antibody (AMA), which is the hallmark of PBC and is detected in at least 90% of the patients, other autoantibodies (antinuclear antibody, anti-smooth muscle antibody and rheumatoid factor, etc.) may also be found in the patients. There is no correlation between the titer of AMAs and the disease severity. Most patients are diagnosed either during the asymptomatic phase of PBC or after presenting with non-specific symptoms. Pruritus and fatigue are the most common symptoms of PBC. The prognosis of PBC has improved significantly during the last few decades. Patients are now diagnosed earlier in its clinical course, they are more likely to be asymptomatic at diagnosis and they are more likely to receive medical treatment. A wide variety of drugs have been assessed for the treatment of this condition: such immunosuppressive agents as corticosteroids, cyclosporine and azathioprine have a weak effect on the disease's natural history. Ursodeoxycholic acid (UDCA) is the only currently approved medical treatment. For PBC patients with end-stage liver disease or an unacceptable quality of life, liver transplantation is the only accepted therapeutic option. Early diagnosis and treatment of PBC are important because effective treatment with UDCA has been shown to delay disease progression and improve rate survival in the early stage.

Deflazacort for Type-1 Autoimmune Hepatitis in a Korean Girl

Prednisone or prednisolone are the mainstay drug treatments for autoimmune hepatitis in children. However, long-term use of corticosteroid is associated with the risk of steroid-induced toxicities, and this situation requires newer immuno-suppressive agents for the treatment of autoimmune hepatitis, especially in growing children. An 11-yr-old Korean girl with type-1 autoimmune hepatitis discontinued prednisolone due to toxicities, i.e., hirsutism, buffalo hump, and skin striae, and remained clinical and biochemical remission under replacement of deflazacort and ursodeoxycholic acid combination therapy. A follow-up liver biopsy after 19 months of deflazacort and ursodeoxycholic acid treatment showed histologic remission.

Treatment of cholestasis and cholestatic disorders.

The future of therapy of chronic cholestatic disorders is bright given the large number of new drugs that are in the initial phases of study. UDCA is currently the mainstay of therapy.

Tratamento näo cirúrgico da litíase biliar / Non-surgical treatment of the biliary lithiasis

Revendo a literatura, procurou-se atualizar o tratamento näo cirúrgico da colelitiase e da coledocolitíase, frente as drogas existentes, tais como ácidos queno e ursodeoxicólicos, compostos terapênicos (Rowachol), metil-butil-éter terceário (MTBE), litotripsia extracorpórea, no primeiro caso e, nas coledocolitíases o uso de fármacos por via oral, esfincterotomia endoscópica com ou sem dissoluçäo e/ou extraçäo de cálculos, a disoluçäo com entubaçäo transhepática percutânea, a infusäo de solventes por sonda nasobiliar, a infusäo de solventes pelo dreno em T e a litotripsia extracorpórea ou endoscópica, respectivamente

El síndrome prostato-hemorroidal / The prostato-hemorrhoidal syndrome

Se presenta un replanteamiento del papel patogénico de las hemorroides en algunas enfermedades diagnosticadas primariamente como prostáticas. Se efectúa una revisión histórica, se presentan las bases anátomo-clínicas y los signos y síntomas de presentación como así también las sugerencias terapéuticas para resolver el síndrome próstato-hemorroidal.