Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Vigilancia de Hepatitis virales y de etiología desconocida / Surveillance of viral hepatitis and unknown etiology

Se presenta brevemente la situación mundial y regional de hepatitis virales y de hepatitis de etiología desconocida, con su correspondiente análisis de los casos residentes en la Ciudad de Buenos Aires durante el período 2020-2021. Describe la cobertura de vacunación en los años 2018-2021.

Comunicado de risco: hepatite aguda grave de etiologia desconhecida em crianças / Risk statement: severe acute hepatitis of unknown etiology in children

A Comunicação de risco tem como objetivo apoiar na divulgação rápida e eficaz de conhecimentos às populações, parceiros e partes intervenientes possibilitando o acesso às informações fidedignas que possam apoiar nos diálogos para tomada de medidas de proteção e controle em situações de emergência em saúde pública. Na presente versão apresentaremos a situação epidemiológica e as medidas a serem adotadas mediante o aumento de casos de hepatite aguda grave de etiologia desconhecida em crianças, em diversos países do mundo

Risk communication aims to support the rapid and effective dissemination of knowledge to populations, partners and stakeholders, enabling access to reliable information that can support dialogues for taking protection and control measures in public health emergency situations. In this version, we will present the epidemiological situation and the measures to be adopted in view of the increase in cases of severe acute hepatitis of unknown etiology in children in several countries around the world

Hepatitis sifilítica: reporte de una serie de casos / Syphilitic hepatitis: report of three cases

Liver involvement occurs in 0.2 to 3% of patients with syphilis. We report three patients with liver involvement in syphilis. A 52-year-old male presenting with erythema and malaise. Laboratory showed a gamma glutamyl transpeptidase (GGT) of 853 u/l, alkaline phosphatases of 1,010 U/L and VDRL was positive. Treatment with penicillin resolved the skin problem and normalized liver enzymes. A HIV positive 30-year-old male in peritoneal dialysis presenting with itching, malaise and markedly elevated GGT and alkaline phosphatases. VDRL was positive. He was treated with penicillin with remission of symptoms and enzyme normalization. A 43-year-old male presenting with erythema, malaise, arthralgias and elevated GGT and alkaline phosphatases. VDRL was positive and treatment with penicillin reverted symptoms and laboratory abnormalities.

Scarlet fever associated with hepatitis in pediatrics: a case report / Hepatitis asociada a escarlatina en pediatría: reporte de caso

INTRODUCCIÓN: La escarlatina es una enfermedad común en Pediatría, causada por Estreptococo beta hemolítico grupo A (SBHGA), la cual generalmente se presenta después de un episodio de faringitis, y con excelente pronóstico general. La hepatitis secundaria a escarlatina es una complicación, descrita muy rara vez en niños. Nuestro objetivo fue reportar la ocurrencia de hepatitis secundaria a escarlati na en un paciente pediátrico. CASO CLÍNICO: Varón de 12 años cursando escarlatina, quien se presentó con una historia de 4 días de ictericia, coluria y disminución del apetito. Los exámenes de laboratorio revelaron elevación de las transaminasas y de los niveles de bilirrubina total y directa, y estudios vira les negativos para Hepatitis A, B y C, Virus de Epstein Barr, Parvovirus B19, Citomegalovirus, Virus Herpes 6 y Herpes simplex 1 y 2. Ecografía abdominal fue normal. DISCUSIÓN: La hepatitis es una complicación inhabitual de la escarlatina, cuya patogénesis aún no está clara. La producción de citoquinas a través del daño celular mediado por la exotoxina pirógena estreptocócica, se ha propuesto como un posible mecanismo de hepatotoxicidad en infecciones por SBHGA. CONCLUSIÓN: La hepati tis asociada a escarlatina continúa siendo una entidad rara, pero de curso benigno, con recuperación plena en semanas a meses.

INTRODUCTION: Scarlet fever is a common illness in pediatrics caused by group A beta-hemolytic streptococcus (GABHS), which usually occurs after an episode of pharyngitis, and has an overall excellent prognosis. Hepatitis secondary to scarlet fever is a rare complication described in adults and even less frequently in children. Our objective was to describe a case of hepatitis secondary to scarlet fever in a pediatric patient. CLINICAL CASE: A 12-year-old male with scarlet fever presented with a 4-day history of jaundice, dark urine, and decreased appetite. Laboratory tests revealed elevated liver enzy mes and total and direct bilirubin levels, and negative studies for hepatitis A, B and C, Epstein Barr virus, parvovirus B19, adenovirus, cytomegalovirus, human herpes virus-6, and herpes simplex virus 1 and 2. Abdominal ultrasound examination was normal. DISCUSSION: The pathogenesis of scarlet fever associated hepatitis remains unclear. Streptococcal pyrogenic exotoxin mediated cellular injury via cytokine production has been proposed as a possible mechanism of hepatotoxicity in GABHS infections. CONCLUSION: Hepatitis secondary to scarlet fever remains a rare but benign entity, with complete recovery expected over weeks to months.

Situación de las hepatitis virales / Present status of virals hepatitis

Informe especial donde se analizaron todos los casos de hepatitis virales notificados a los sistemas oficiales (módulos C2-SNVS y SIVILA-SNVS operativos hasta el 28-04-2018 y SNVS 2.0 para los casos posteriores a dicha fecha) de los residentes de la Ciudad de Buenos Aires y con domicilio desconocido, entre las Semanas Epidemiológicas (SE) 1 y 52 de 2017 y con datos hasta el 27 de mayo de 2018 (SE 21 completa). Para el análisis por cuatrisemanas se incluyen casos hasta el 19/05/2018 (cuatrisemana epidemiológica 5 completa). Cabe aclarar que se presentan casos confirmados en los módulos clínicos en los que no se cuenta con la notificación por laboratorio. Diferentes notificaciones pueden corresponder a un mismo paciente estudiado para más de una patología. Para el análisis de hepatitis B se incluyen además 3 notificaciones provenientes de bancos de sangre. Para la elaboración de tasas se utilizaron los datos de proyecciones de población de la Dirección General de Estadística y Censos de la Ciudad Autónoma de Buenos Aires. (AU)

Espectro morfológico de la biopsia hepática / Morphologic spectrum of liver biopsy

La biopsia hepática constituye un excelente método diagnóstico en el espectro de la patología hepática. Para algunos autores la biopsia hepática no es considerada como el "método más indicado" debido al error de muestreo y las variaciones interobservador, sin embargo, sigue considerándose por muchos el "método de elección". Se recopilaron y analizaron en forma retrospectiva, las preparaciones histológicas y las boletas de solicitud de biopsias de todos los casos de biopsias hepáticas, provenientes de la Sección de Patología Gastrointestinal y Hepática "Dr. Pedro Grases" del Instituto Anatomopatológico "Dr. José A. O’Daly" de la Universidad Central de Venezuela, en el lapso comprendido entre enero de 1996 y diciembre de 2006. Pacientes 50,8% eran mujeres y 46,8% hombres. El grupo etario más afectado (55,4%) fue entre 31 y 60 años. Los hallazgos clínicos más frecuentes fueron ictericia (7,9%) y dolor en hipocondrio derecho (5,3%). Frecuencia de los diagnósticos histopatológicos: esteatosis (15,1%), hepatitis por virus C (12,3%), tumores metastásicos (8,9%), cirrosis (8,1%), esteatohepatitis (6,6%), patologías vasculares (5,4%), tumores primarios hepáticos (4,1%). La biopsia hepática es una excelente herramienta para el diagnóstico y tratamiento, si se realiza una buena correlación clínico patológica

Liver biopsy is an excellent diagnostic method in the spectrum of liver pathology. For some authors liver biopsy is considered the "best method" due to sampling error and interobserver variations, however is still considered by many the "method of choice". We collected and analyzed retrospectively, the histological preparations and request ballots biopsies of all cases of liver biopsies, from the Section of Gastrointestinal and Liver Pathology "Dr. Pedro Grases "Institute of Pathology" Dr. José A. O’Daly "Central University of Venezuela, in the period between January 1996 and December 2006. 50.8% patients were female and 46.8% male. The most affected age group (55.4%) was between 31 and 60 years. The most frequent clinical findings were jaundice (7.9%) and right upper quadrant pain (5.3 %). Frequency of histopathological diagnoses: steatosis (15.1%), hepatitis C virus (12.3%), metastatic tumors (8.9%), cirrhosis (8.1%), steatohepatitis (6.7%), vascular diseases (5.4%), primary liver tumors (4.1%). Liver biopsy is an excellent tool for diagnosis and treatment, if you do a good clinicopathologic correlation

Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Glycogenic hepatopathy (GH) is an uncommon cause of serum transaminase elevation in type I diabetes mellitus (DM). The clinical signs and symptoms of GH are nonspecific, and include abdominal discomfort, mild hepatomegaly, and transaminase elevation. In this report we describe three cases of patients presenting serum transaminase elevation and hepatomegaly with a history of poorly controlled type I DM. All of the cases showed sudden elevation of transaminase to more than 30 times the upper normal range (like in acute hepatitis) followed by sustained fluctuation (like in relapsing hepatitis). However, the patients did not show any symptom or sign of acute hepatitis. We therefore performed a liver biopsy to confirm the cause of liver enzyme elevation, which revealed GH. Clinicians should be aware of GH so as to prevent diagnostic delay and misdiagnosis, and have sufficient insight into GH; this will be aided by the present report of three cases along with a literature review.

Avaliação de manifestações bucais em pacientes pediátricos submetidos ao transplante hepático / Evaluation of oral manifestations in liver transplanted pediatric patients

Introdução. O transplante hepático se tornou a principal opção terapêutica para o tratamento de várias doenças hepáticas. Subsequentemente ao transplante, é necessária a administração de terapia imunossupressora para evitar rejeição ao órgão transplantado. A avaliação odontológica é fundamental para eliminação ou prevenção do surgimento de focos infecciosos. Além disso, faz-se necessário o acompanhamento dos efeitos colaterais em cavidade bucal relacionados ao uso de drogas imunossupressoras. Objetivo. Avaliar as condições odontológicas previamente ao transplante hepático e identificar as alterações bucais apresentadas após o transplante hepático. Pacientes e métodos. Foi realizado estudo retrospectivo de 265 pacientes pediátricos submetidos ao transplante hepático no Hospital A.C. Camargo, São Paulo-SP, entre janeiro de 2002 e dezembro de 2009. As informações clínicas como idade, gênero, diagnóstico da doença hepática, data do transplante, terapia imunossupressora (tipo, dose e duração), tratamento odontológico e a presença de alterações bucais pós-transplante foram coletadas dos prontuários médicos. Análise estatística foi realizada buscando estabelecer informações relevantes quanto aos riscos e possíveis fatores preditivos para o desenvolvimento de manifestações bucais. Resultados. A idade ao transplante hepático variou de 3,5 a 210,7 meses, tendo uma mediana de 15,5 meses. Dos 265 pacientes, 150 pacientes (56,6%) eram do gênero feminino e 165 (62,3%) eram leucodermas. Dentre as doenças de base, a atresia de vias biliares foi a mais frequente, acometendo 170 (64,1%) pacientes.

Um total de 73 pacientes foi avaliado pelo Departamento de Estomatologia previamente ao transplante, e destes, 34 (46,6%) apresentaram cárie. Quanto à presença de pigmentação dentária por bilirrubina, 172 pacientes foram avaliados e destes, 100 (58,1%) apresentaram pigmentação. Em relação à presença de hipoplasia do esmalte dentário, a alteração foi observada em 56 (34,4%) de 163 pacientes. Interessantemente, dos 100 pacientes com pigmentação dentária por bilirrubina, 97 apresentavam doenças colestáticas (p<0,001). Quanto aos casos de hipoplasia do esmalte, 52 (92,9%) pertenciam ao grupo de doenças colestáticas (p<0,001). Diversas alterações em mucosa bucal foram encontradas após o transplante, sendo que 135 pacientes apresentaram alguma complicação. Dos 265 pacientes estudados, o principal problema encontrado foi infecção pelo vírus herpes simples, em 48 pacientes, sendo que 66,7% dos casos ocorreram a partir de 12 meses após o transplante. A segunda doença infecciosa mais comum em cavidade oral foi a candidose, observada em 39 pacientes e 61,5% dos casos ocorreram durante o primeiro semestre pós-transplante. Importantes alterações bucais foram encontradas em pacientes em uso de tacrolimus, como a hipertrofia de papilas linguais, ressecamento labial, edema labial, fissuras labiais, fissuras linguais, queilite angular, mucosa com aspecto de pedra de calçamento e língua despapilada, observadas em 45, 39, 39, 38, 27, 11 e 6 pacientes, respectivamente. A hiperplasia gengival medicamentosa foi observada em 13 pacientes, sendo que 8 estavam em uso de ciclosporina e todos os outros usavam além do tacrolimus, drogas bloqueadoras de canais de cálcio. A complicação que ocorreu mais precocemente nos pacientes após o transplante foi a doença linfoproliferativa, em média 5,2 meses após o transplante. Conclusões. O índice de cáries foi elevado refletindo as precárias condições de saúde bucal desses pacientes. A pigmentação por bilirrubina.

Introduction. Liver transplantation has become the main therapeutic option for the treatment of various liver diseases. Subsequent to transplantation, it is necessary to administer immunosuppressive treatment to avoid rejection of the graft. A dental evaluation is critical to eliminate or prevent the emergence of infectious foci. Moreover, it is necessary to monitor the side effects in the oral cavity related to the use of immunosuppressive drugs. Objectives. To evaluate the dental conditions prior to liver transplantation and to identify oral abnormalities presented after liver transplantation. Pacients and methods. A retrospective study of 265 pediatric patients who underwent liver transplantation at Hospital A.C. Camargo, São Paulo-SP, between January 2002 and December 2009 was performed. Clinical information such as age, gender, diagnosis of liver disease, date of transplantation, immunosuppressive therapy (type, dose and duration), dental treatment and oral changes after transplantation were collected from medical records. Statistical analysis was performed in order to establish relevant information about the risks and possible predictive factors for the development of oral manifestations.

Hepatotoxicidade induzida por sulfassalazina: relato de caso / Hepatotoxicity induced by sulfasalazine: case report

A sulfassalazina é ainda muito utilizada nas doenças inflamatórias intestinais, sobretudo na retocolite ulcerativa leve e moderada. Entretanto, seu uso é relacionado a vários efeitos colaterais, incluindo disfunção hepática grave.Este é um relato do caso de paciente masculino, 21 anos, portador de retocolite ulcerativa moderada, com queixa de inapetência, febre, artralgia e icterícia, há sete dias. Antecedente pessoal de uso de sulfassalazina 4 g/dia há seis semanas. Ao exame físico apresentava-se ictérico, com exantema em membros e edema de membros inferiores. Exames complementares mostravam aumento de bilirrubinas, enzimas hepáticas e canaliculares e da proteína C reativa. Com o diagnóstico de hepatotoxicidade por sulfassalazina, foi suspensa a medicação e introduzido prednisona 20 mg/dia e ciprofloxacino 1 g/dia. Recebeu alta no terceiro dia de internação após melhora clínica e laboratorial. Atualmente encontra-se assintomático e em uso de azatioprina 150 mg/dia.

The sulfasalazine is widely used in inflammatory bowel disease, especially in mild and moderate ulcerative rectocolitis. However, its use is related to several side effects, including severe liver dysfunction. We report the case of male patient, 21 years, with the moderate ulcerative rectocolitis, complaining of inappetence, fever, arthralgia and jaundice for seven days. Personal history includes use of sulfasalazine 4 g/day during six weeks. The physical examination revealed jaundiced, with members in rash and lower extremity edema. Laboratory exams showed an increase in bilirubin, liver enzymes and canalicular and C-reactive protein. With the diagnosis of hepatotoxicity by sulfasalazine, this medication was suspended, and introduced prednisone 20 mg/day and ciprofloxacin 1g/day. He was discharged on the third day of admission after clinical and laboratorial improvement. Currently, he is asymptomatic and in use of azathioprine 150 mg/day.

Q Fever as a Cause of Acute Hepatitis Accompanying Fever / 대한소화기학회지

Q fever is a zoonotic infection caused by Coxiella burnetti, which has been previously regarded as an uncommon infectious disease in Korea but is sporadically reported recently. Common manifestations of acute Q fever usually present as influenza-like illness, pneumonia and occasionally hepatitis. Herein, we report 4 cases of acute Q fever as a cause of acute hepatitis and fever. All patients had fever and non-specific symptoms, and laboratory test showed acute hepatitis. Antibody surveys for many virus infections and bacterial cultures were negative. Finally, they were diagnosed acute Q fever by an indirect microimmunofluorescence test. Liver biopsy in 3 patients revealed granuloma including one with typical fibrin-ring. All patients had complete resolution of symptoms and signs with doxycycline treatment. Q fever should be considered in the differential diagnosis of patients with fever of unknown origin with acute hepatitis in Korea.

Can stereological studies be helpful in differentiating biliary atresia from neonatal hepatitis?

Cholestatic jaundice is a potentially dangerous condition which is often misdiagnosed by paediatricians as physiological or breast milk jaundice. The two most common causes of neonatal cholestasis [NC] are biliary atresia [BA] and neonatal hepatitis [NH]. Early and accurate differentiation of these two entities is very important as early surgery in BA improves the biliary drainage but the delay leads to irreversible hepatocellular damage. There has been much discussion over the value of hepatobiliary radioisotopic scans, liver histopathological features, serum g-glutamyl transpeptidase [GGTP] levels, and other tests which are widely used for differentiation of BA from NH. Stereology provides practical techniques for extracting quantitative information about a threedimensional material from measurements made on two-dimensional planar sections of the tissues. We suggest that the stereological study of different components of liver tissue may be useful in determining the function of liver and differentiating some liver diseases such as biliary atresia and neonatal hepatitis

Cholestatic Hepatitis and Thrombocytosis in a Secondary Syphilis Patient

The incidence of acute hepatitis in syphilis patient is rare. First of all, our patient presented with hepatitis comorbid with thrombocytosis. To our knowledge, this is only the second report of syphilitic hepatitis with thrombocytosis. The 42-yr-old male complained of flulike symptoms and skin eruptions on his palms and soles. Laboratory findings suggested an acute hepatitis and thrombocytosis. Serologic test results were positive for VDRL. He recovered from his symptoms and elevated liver related enzymes with treatment. Because syphilitic hepatitis can present without any typical signs of accompanying syphilis, syphilis should be considered as a possible cause in acute hepatitis patients.

Clinical factors influencing liver stiffness as measured by transient elastography (Fibroscan(R)) in patients with chronic liver disease / 대한간학회지

BACKGROUND/AIMS: Transient elastography as performed using the Fibroscan(R) is a useful noninvasive method for evaluating hepatic fibrosis. However, recent studies have found that liver stiffness measurement (LSM) values are inappropriately elevated in acute hepatitis or in the acute flare state of chronic hepatitis, suggesting that the LSM value obtained by the Fibroscan(R) is not a reliable marker for fibrosis. We retrospectively evaluated the clinical factors influencing the LSM value obtained using transient elastography as performed using the Fibroscan(R) in patients with chronic liver disease. METHODS: A total of 298 patients who were followed in Kungpook National University Hospital from November 2007 to May 2008 due to previously established liver cirrhosis or chronic liver disease were investigated using the Fibroscan(R), laboratory test, ultrasound, and/or abdominal computed tomography. RESULTS: The 298 patients were aged 47.8+/-12.9 years (mean+/-SD). The cut-off value for a diagnosis of liver cirrhosis was 12.5 kPa (as used in previous studies). Thirty-six patients (15%) and 202 patients (85%) with chronic liver disease without clinical manifestation of cirrhosis had LSMs of >12.5 kPa and <12.5 kPa, respectively. Multivariate analysis revealed that LSM values were unusually increased in patients with chronic liver disease who were older (P=0.007) or who had increased gamma gultamyltranspetidase (GGT) (P=0.022), decreased albumin (P=0.015), or increased total bilirubin (P=0.009). CONCLUSIONS: This study reveals that age, GGT, and albumin are clinical factors influencing LSM values. This reinforces the need to interpret LSM values in the context of a defined diagnosis, biochemical data, radiologic examination, and other clinical findings.

Case of Malarial Hepatitis by Plasmodium Vivax / 대한소화기학회지

Malarial infection is one of the most important tropical diseases, but also increasing in the temperate regions. Severe malaria with organ dysfunction is commonly associated with Plasmodium falciparum, but rarely with Plasmodium vivax. Malarial hepatitis is also unusual in P. falciparum and very rare in P. vivax. Only 3 cases of malarial hepatitis caused by P. vivax have been reported in the world. Because the presence of hepatitis in malaria indicates a more severe illness with higher incidence of other complications and poor prognosis, malarial patients should be meticulously monitored for hepatic dysfunction with or without jaundice. We report here a case of malarial hepatitis caused by P. vivax that was presented by fever, general ache, nausea, fatigue, and significant elevation of aminotransferase and bilirubin.