Clinical Characteristics and Outcomes of Acute Hepatitis A in Korea: A Nationwide Multicenter Study

The aim of this study was to investigate the clinical characteristics of acute hepatitis A during a recent outbreak in Korea. Data of patients diagnosed with acute hepatitis A from 2007 to 2009 were collected from 21 tertiary hospitals retrospectively. Their demographic, clinical, and serological characteristics and their clinical outcomes were analyzed. A total of 4,218 patients (mean age 33.3 yr) were included. The median duration of admission was 9 days. The mean of the highest ALT level was 2,963 IU/L, total bilirubin was 7.3 mg/dL, prothrombin time INR was 1.3. HBsAg was positive in 3.7%, and anti-HCV positive in 0.7%. Renal insufficiency occurred in 2.7%, hepatic failure in 0.9%, relapsing hepatitis in 0.7%, and cholestatic hepatitis in 1.9% of the patients. Nineteen patients (0.45%) died or were transplanted. Complications of renal failure or prolonged cholestasis were more frequent in patients older than 30 yr. In conclusion, most patients with acute hepatitis A recover uneventfully, however, complication rates are higher in patients older than 30 yr than younger patients. Preventive strategies including universal vaccination in infants and active immunization of hepatitis A to adult population should be considered for prevention of community-wide outbreaks of hepatitis A in Korea.

Síndrome de Alagille experiencia clínica de catorce casos en Medellín, Colombia / Alagille syndrome, clinical experience of fourteen cases in Medellin, Colombia

Introducción: El Síndrome de Alagille corresponde a una alteración autosómica dominante con expresión variable. Se caracteriza por colestasis crónica con escasez de los conductos biliares interlobulares asociada a alteraciones cardiovasculares, oftálmicas, sistema esquelético, riñones y facies característica. Su distribución es mundial con frecuencia de 1 por cada 100000 nacidos vivos. Objetivo: Describir las características clínicas, la evolución y la sobrevida de catorce pacientes, con diagnóstico de Síndrome de Alagille atendidos en un período de 16 años en Medellín, Colombia. Materiales y métodos: Es un trabajo observacional descriptivo de reporte de casos de los hallazgos morfológicos y la evolución de los pacientes con diagnóstico de Síndrome de Alagille. Resultados: Grupo compuesto por ocho niños y seis niñas, con edades entre los dos meses y los diez años al momento de diagnóstico. El síndrome completo se presentó en 28%. Los hallazgos más frecuentes, estenosis valvular de la arteria pulmonar y la alteración vertebral se presentaron en el 79%. Tres pacientes 21%, fallecieron, uno de ellos después de recibir trasplante hepático. De los once sobrevivientes dos niñas fueron sometidas a trasplante y se encuentran en buenas condiciones. Los nueve restantes padecen hepatopatía colestásica crónica y reciben tratamiento médico. Conclusión: El Síndrome de Alagille se debe tener en cuenta en el diagnóstico de colestasis crónica infantil. Para establecer la distribución y frecuencia de esta enfermedad en nuestro país es necesario desarrollar investigaciones que idealmente incluyan el estudio de la mutación genética en los pacientes y su familia cercana.

Introduction: The Alagille Syndrome is an autosomic dominant disorder with variable expression. Chronic cholestasis, characteristic facial appearance and abnormalities heart, skeleton, eye, kydnes with hypoplasia of the biliary ducts. Initial description in France, with mundial distribution her frecuence is 1/100000. Objective: To describe the clinical characteristic and evolutions of fourteen patients with diagnosis Alagille Syndrome in Medellín Colombia. Materials and methods: Descriptive retrospective study with variables obtained from clinical records of patients with diagnosis Alagille Syndrome. Results: Eight boys and six girls. The age at diagnosis varied two months at nine years. Complete syndrome was present in 28%. The most frecuent alterations were valvular stenosis pulmonary artery and failure of anterior vertebral arch fusion (butterfly vertebrae) 79%. The clinical evolution was variable, death occurred in three patients 21%, one girl post liver transplantation. Nine children had chronic hepatopathy controlled with medical treatment and two girls had liver transplantation with satisfactory evolution. Conclusions: In Colombia, the poblational incidence is not defined it is necessary to know the distribution of syndrome at future study.

Diagnóstico diferencial de colestase neonatal: parâmetros clínicos e laboratoriais / Differential diagnosis of neonatal cholestasis: clinical and laboratory parameters

Objetivo: Avaliar se os parâmetros clínicos e laboratoriais poderiam auxiliar no diagnóstico diferencial da colestase neonatal (CN) intra- e extra-hepática. Métodos: Estudo retrospectivo de pacientes com CN hospitalizados na Clínica de Hepatologia Pediátrica do Hospital de Clínicas da Universidade Estadual de Campinas (UNICAMP), Campinas (SP), entre dezembro de 1980 e março de 2005. A abordagem para o diagnóstico da CN foi padronizada. De acordo com o diagnóstico, os pacientes foram classificados em dois grupos: I (colestase neo natal intra-hepática) e II (colestase neonatal extrahepática). Para verificar se havia associação com a variável categórica, os testes de qui-quadrado e Mann-Whitney foram utilizados com correções para idade para a análise de covariância (ANCOVA). A determinação da precisão das variáveis clínicas e laboratoriais para a diferenciação dos grupos foi realizada através da análise da curva ROC. Resultados: Cento e sessenta e oito pacientes foram avaliados (grupo I = 54,8 por cento e grupo II = 45,2 por cento). Nos pacientes com menos de 60 dias de vida, houve predominância de causas intra-hepáticas, enquanto que naqueles com mais de 60 dias, houve predominância de etiologia extrahepática (p < 0,001). A mediana de peso ao nascer foi mais baixa no grupo I (p = 0,003), assim como o comprimento ao nascer (p = 0,007). Os valores da mediana de bilirrubina direta foram mais altos no grupo II (p = 0,006). Os valores de gama glutamil transferase (GGT) (10 vezes mais altos do que o limite de normalidade) apresentaram sensibilidade de 56,3 por cento, especificidade de 91,5 por cento e acurácia de 75,7 por cento para o diagnóstico de colestase extra-hepática. Conclusão: No presente estudo, a CN extra-hepática apresentou maior peso e comprimento ao nascer, hipocolia/acolia fecal, colúria, hepatomegalia, aumento de GGT (10,8 vezes mais alto do que o limite de normalidade) e um atraso no encaminhamento para a investigação no hospital terciário.

Objective: To evaluate if clinical and laboratory parameters could assist in the differential diagnosis of intra and extra-hepatic neonatal cholestasis (NC). Methods: Retrospective study of NC patients admitted at the Pediatric Hepatology Outpatient Clinic of the teaching hospital of Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil, between December 1980 and March 2005. The approach to the diagnosis of NC was standardized. According to diagnosis, patients were classified into two groups: I (intra-hepatic neonatal cholestasis) and II (extra-hepatic neonatal cholestasis). In order to verify if there was association with the categorical variable, the chi-square and Mann-Whitney tests were used, with corrections for age for the covariance analysis (ANCOVA). The determination of accuracy of the clinical and laboratory variables for differentiation of the groups was made using the analysis of the ROC curve. Results: One hundred and sixty-eight patients were evaluated (group I = 54.8 percent and group II = 45.2 percent). In the patients with less than 60 days of life there was predominance of intra-hepatic causes, whereas, in those older than 60 days, there was predominance of extra-hepatic etiology (p < 0.001). Median birth weight was lower in group I (p = 0.003), as well as length at birth (p = 0.007). Median values of direct bilirubin were higher in group II (p = 0.006). Values of gamma-glutamyltransferase (GGT) (10 times higher than the limit of normality) presented sensitivity of 56.3 percent, specificity of 91.5 percent, and accuracy of 75.7 percent for the diagnosis of extra-hepatic cholestasis. Conclusion: In the present study, extra-hepatic NC presented greater weight and length at birth, fecal hypocholia/acholia, choluria, hepatomegaly, increase in GGT (10.8 times higher than the limit of normality), and a delay for investigation in the tertiary center.

Colestasis: enfoque actual / Cholestasis: current approach

La colestasis, tiene incidencia de 1:60-375 ictéricos a las 2 semanas de edad, es potencialmente grave, presentan riesgos inmediatos como coagulopatías por hemorragia severa ante el déficit de la absorción de la vitamina K, y con su diagnóstico precoz, se identifican patologías que tienen tratamiento, incluso, trasplante hepático. Como consecuencia de la colestasis, hay retención de sales biliares, daño celular hepático, y descenso de la bilis a nivel intestinal, que ocasionan mala digestión de grasas y proteínas, con defectos en las vitaminas liposolubles. Existen hepatopatías primariassecundarias a una serie de entidades genéticas y metabólicas, y colestasis secundarias a otros problemas, que en el adulto generan enfermedad hepática. Es necesario establecer protocolos deidentificación del niño con ictericia.

Cholestasis has 1:60-375 incidence of jaundice at 2 weeks of age, are potentially serious, immediateand present risk of severe bleeding coagulopathy to the shortfall in the absorption of vitamin K, andearly diagnosis, identify diseases that are treatable, even liver transplantation.As a result of cholestasis, there is retention of bile salts, liver cell damage and decrease of bile in the intestine, causing poor digestion of fats and protein, with defects in soluble vitamins. There are primaryliver disease secondary to a variety of metabolic and genetic entities, and cholestasis secondary toother problems, generated in the adult liver disease. It is necessary to establish protocols for the identification of children with jaundice.

Colestase da gravidez: revisão de literatura / Obstetric cholestasis: review of the literature

Colestase da gravidez (CG) é uma doença hepática específica da gravidez que tipicamente ocorre a partir do final do segundo trimestre. É uma doença de etiologia heterogênea (multifatorial) com contribuição de fatores genéticos e hormonais, caracterizada por prurido generalizado intenso e alterações das provas de função hepática, estando associada ao aumento das taxas de morbidade e mortalidade fetal. A revisão de literatura realizada refere-se à epidemiologia, etiologia, manifestações clínicas, achados laboratoriais e o manejo da CG na qual se conclui que o conhecimento por parte dos profissionais sobre a doença é fundamental para que seja realizado um manejo adequado das gestantes, visando principalmente prevenir complicações fetais. Apesar do número significativo de estudos relacionados com a CG, vários aspectos da sua etiologia e patogênese não foram elucidados. O diagnóstico é feito por meio de achados clínicos e das alterações das provas de função hepática e aumento dos níveis de ácidos biliares. O ácido ursodesoxicólico é a droga atualmente utilizada na terapêutica da CG com eficácia no controle do prurido e no restabelecimento de níveis normais dos ácidos biliares. Porém, há necessidade da condução de pesquisas e ensaios clínicos para a melhor condução desta doença.

Obstetric cholestasis (OC) is a specific hepatic pathology of the pregnancy that typically happens in the end of the second trimester. It is a heterogeneous etiology (multifactorial) disease with contribution from genetic and hormonal factors, characterized by intense itch and abnormal liver function tests, being associated to the increase of perinatal morbidity and mortality. The revision of accomplished literature refers to the epidemiology, etiology, clinical manifestations, laboratory findings and of which management OC concludes that knowledge by professionals about the disease is the key to an appropriate management, undertaken by pregnant women, seeking mainly to prevent fetal complications. In spite of the significant number of studies related with OC, several aspects of its etiology and pathogenesis were not elucidated. The diagnosis is made through the clinical discoveries and abnormal liver function tests and increase of the levels of bile acids. The ursodeoxycholic acid is now the drug used in the therapeutics of OC with effectiveness in the control of the itch and in the re-establishment of normal levels of the bile acids. However, new research and clinical trials are required for best conduction of this pathology

Neonatal cholestasis in Thai infants.

The objective of this study was to study etiologies and outcome of neonatal cholestasis in Thai infants. The medical records of infants aged less than 3 months with the diagnosis of neonatal cholestasis in Department of Pediatrics, Siriraj Hospital from 1993 to 2004 were retrospectively reviewed. The etiologies were diagnosed by history, physical examination, and proper investigations. There were 252 infants, including 135 males (53.6%) and 117 females (46.4%). The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. TPN-related cholestasis was increasingly found due to advance management of critically ill premature infants. Inborn error of metabolism were suspected in 8 patients (3.21%). Seventeen cases (6.7%) developed cholestasis during the first week of life due to hemolytic anemia, intrauterine infection, hypoxia and others. During the 3 month follow-up period, 6 cases died of progressive dysfunction of liver and one case with idiopathic neonatal hepatitis died from intracranial bleeding from vitamin K deficiency. In conclusion, INH and EHBA are the most common causes of neonatal cholestasis. Due to advance management and nutritional support in critically ill premature infants, TPN-related cholestasis is found more often. Inborn error of metabolism related to neonatal cholestases is uncommon in Thai infants. Overall short-term prognosis of neonatal cholestases is good.

Significación clínico-epidemiológica de la hepatotoxicidad por fármacos en la consulta hepatológica / Clinic-epidemiological significance of the hepatotoxicity by pharmaceutical preparations in the liver consultation

Se analizaron los datos epidemiológicos y clínico-evolutivos de la hepatotoxicidad por fármacos en una experiencia de 10 años (1988-1998) de nuestra Unidad de Hígado, que incluye 10342 historias clínicas de registro prospectivo. La prevalencia en este material fué de 5,6 por ciento, con ligero predominio femenino (1.4:1) y en mayores de 40 años; más del 50 por ciento ingirieron 2 o más fármacos. Predominaron las formas agudas (91.4 por ciento) e ictéricas (60.4 por ciento) con manifestaciones sistémicas de hipersensibilidad en 29.3 por ciento, el 4.5 por ciento de las formas agudas se presentaron como fallo hepático agudo severo, con necesidad de transplante hepático en un caso. los 4 casos de hepatitis crónica presentaron evolución a la cirrosis en un caso, y un caso de colestasis con ductopenia (CBP-simil) evolucionó favorablemente en 19 semanas, recibiendo ácido ursode-soxicólico 10 mg/k/día. Seis fármacos representaron el 53.4 por ciento de los casos: anticonceptivos orales, isoniacida, sulfamidas, clorpropamida, carbamacepina y amiodarona.

Neonatal cholestasis syndrome: Indian scene.

Neonatal cholestasis syndrome (NCS) in India has largely remained ignored. Three questions need to be addressed: (a) What is known about NCS in India (b) Where do we stand and (c) What needs to be done? Current data on etiology of NCS indicates that biliary atresia contributes to about 40% of all NCS cases. There is considerable delay in the referral of patients to appropriate centres for management. A delay of 120.8 +/- 60.5 days in biliary atresia and 65.9 +/- 39.2 days in neonatal hepatitis were documented. Biliary atresia cases need to be diagnosed and operated by eight weeks of age so as to have the best results. Delayed referral after 3 months of age, not only bring down the success rate considerably but also adversely affect the management with regard to surgical procedures, nutritional support, control of ascites and finally the cost. Cirrhosis rapidly develops in children with biliary atresia. At this stage the only option left is liver transplantation. An important obstacle in the care of infants with NCS is the misconception of jaundice in newborns. This needs to be handled at a professional level in the training of undergraduates and postgraduates and the lay public. Public awareness campaigns like "Yellow Alert" may be useful in this direction.

Síndrome colestática do lactente: análise retrospectiva de 177 casos / Infantile cholestatic syndrome: analyse of 177 cases

O objetivo deste trabalho foi verificar a ocorrência das diferentes causas de colestase do lactente em nosso meio e reiterar a importância da definiçäo diagnóstica precoce. Foram analisados retrospectivamente os prontários de 177 pacientes com colestase do lactente, atendidos consecutivamente na Disciplina de Gastroenterologia Pediátrica da Escola Paulista de Medicina nos últimos 17 anos. Atresia de vias biliares extra-hepática (A.V.B) foi diagnosticada em 42 (23,7 pôr cento) pacientes, hepatite neonatal idiopática em 81 (45,8 pôr cento) e hepatite neonatal com causa definidaem 48 (27,1 pôr cento). Nessa casuística, 6 pacientes, cujo primeiro diagnóstico de hepatite crônica confirmado em biópsias subsequentes realizadas durante o acompanhamento ambulatorial. E fundamental que os lactentes com síndrome colestática sejam rapidamente encaminhados para centros especializados pois, apenas 24 (pôr cento) dos pacientes com A.V.B. chegaram ao nosso seerviço antes da oitava semana de vida, a tempo de se submeterem à correçäo cirúrgica, com melhores chances de sobrevida

Insuficiência renal aguda pós-operatória: estudo comparativo em cirurgia abdominal / Postoperative acute kidney failure: comparative study in abdominal surgery

A insuficiência renal aguda (IRA) foi estudada comparativamente em 582 pacientes portadores de câncer gástrico, hipertensäo portal equistossomótica, peritonite e icterícia obstrutiva, tratados no Hospital das Clínicas da UGMG, no período de 1977 a 1987. A incidência de IRA pós-operatória nesse grupo foi de 4,9% (29 casos). A incidência de IRA pós-operatória foi mais elevada no grupo de pacientes com icterícia obstrutiva (12,5%) e naqueles com peritonite. A mortalidade global dos pacientes com IRA foi de 79,3%, sendo mais elevada em pacientes com hipertensäo portal e mais baixa em pacientes com câncer gástrico. A infecçäo mostrou-se significativa associada à IRA nos pacientes com hipertensäo portal e perionite. No câncer gástrico e icterícia obstrutiva, essa associaçäo näo foi significativa. Os resultados obtidos mostram que a IRA pós-operatória na icterícia obstrutiva é importante fator de morbidade e mortalidade

Coledocoduodenoanastomosis; complicaciones alejadas en patología biliar benigna / Choledochoduodenostomy; long term complication in benign biliary pathology

El objetivo de este trabajo es determinar y caracterizar la incidencia de complicaciones alejadas de la coledocoduodenoanastomosis en patología biliar benigna. Se estudiaron las historias clínicas de 360 pacientes en los que se efectuó el procedimiento entre 1972 y 1988, identificándose 23 pacientes con complicaciones mayores (6,4%). Se dividieron de acuerdo a las características clínicas: 1) colangitis leves (n= 7, 1,94%), con cuadros clínicos con escasa sintomatología; 2) colangitis graves (n= 8, 2,22%), con internación por compromiso general. Presentaron colangitis a repetición el 37,5% de los pacientes, con una mortalidad del 12,5%; 3) abscesos hepáticos (n= 6, 1,66%), con cuadros clínicos más graves y mortalidad elevada (33%) que requirieron terapéutica invasiva; 4) ictericias obstructivas (n= 2, 0,55%), caracterizados por oclusión de la fístula biliar y posterior resolución quirúrgica. La coledocoduodenoanastomosis sería responsable de complicaciones alejadas, de las cuales las más frecuente son las colangitis.

Colestasis intrahepática del embarazo / Intrahepatic cholestasis of pregnancy

Se revisaron 29 trabajos científicos publicados en diversas partes del mundo, encontrándose una incidencia de la colestasis intrahepática del embarazo diez veces mayor en Suecia, Finlandia, Polonia y Chile. El rol etiopatogénico principal recae en los estrógenos. Se inicia en el tercer trimestre del embarazo y el purito es el rasgo más predominante y perturbador de la enfermedad. Se han usado diversos tratamientos farmacológicos como el fenobarbital, la colestiramina, el Secholex, silimarina, y el 5 adenosil 1 metionina, con buenos resultados sobre todo con el uso del último