Mariza Leitão Valadares Roquete

Clinical and Laboratory Predictors of Esophageal Varices in Children and Adolescents With Portal Hypertension Syndrome

Objectives: To determine the clinical and laboratory parameters that may predict the presence of esophageal varices in children and adolescents with portal hypertension. Patients and Methods: Overall, 111 patients with portal hypertension and no previous history of digestive bleeding underwent esophagogastroduodenoscopy for detection of esophageal varices. A univariate analysis initially was carried out, followed by a logistic regression analysis to identify the independent variables associated with the presence of esophageal varices. Sensitivity and specificity rates, positive predictive value, negative predictive value, and the accuracy of the predictive variables identified among cirrhotic patients were calculated with the esophagogastroduodenoscopy as the reference test. Results: Sixty percent of patients had esophageal varices on the first esophagogastroduodenoscopy. Patients with portal vein thrombosis and congenital hepatic fibrosis were 6.15-fold more likely to have esophageal varices than cirrhotic patients. When we analyzed 85 cirrhotic patients alone, splenomegaly and hypoalbuminemia remained significant indicators of esophageal varices. Only spleen enlargement showed appropriate sensitivity and negative predictive value (97.7% and 91.7%, respectively) to be used as a screening test for esophageal varices among cirrhotic patients. Conclusions: In reference services and research protocols, endoscopic screening should be performed in all patients with portal vein thrombosis and congenital hepatic fibrosis. Among cirrhotic patients, the indication should be conditioned to clinical evidence of splenomegaly or hypoalbuminemia. For clinicians, the recommendation is to emphasize the orientations given to guardians of patients with portal vein thrombosis and congenital hepatic fibrosis as to the risk of digestive bleeding. Cirrhotic patients with hypoalbuminemia and splenomegaly should receive the same orientations.

Effect of treatment of hepatic histopathology in children and adolescents with autoimmune hepatitis.

Objective: To evaluate the effects of treatment on liver histopathology of children and adolescents diagnosed with autoimmune hepatitis. Patients and Methods: Histopathological evaluation of liver biopsies performed at diagnosis or early during treatment, compared with biopsies after immunosuppressive treatment of 20 children and adolescents diagnosed with autoimmune hepatitis who presented clinical and biochemical remission for at least 24 months. Liver histopathology was assessed by 2 pathologists. Agreement between their interpretations was analyzed with κ statistics. Results: The age at diagnosis of the 20 patients varied from 1.7 years to 11.6 years (median = 6.6 years); 18 were females. The mean duration of clinical and biochemical remission, up to performance of liver biopsy, was 4.1 years. The mean interval between the initial and posttreatment biopsies was 4.6 years (range 2–9.7 years). Assessing the necroinflammatory activity, a mean reduction by 6.3 (observer 1) and 7.3 (observer 2) in the grading score was observed (P = 0.00000). When staging was assessed, a mean reduction by 1.8 (observers 1 and 2) in the score was observed after treatment (P = 0.00008), and there was no progression toward cirrhosis. No regression of cirrhosis was observed. Conclusions: On histopathological evaluation, the immunosuppressive treatment improved the fibrosis scores, with an arrest in its progression and no development into cirrhosis. Fibrosis control is mainly associated with regression of necroinflammatory activity, which is the main treatment component in autoimmune hepatitis.

Portal vein thrombosis in children and adolescents.

OBJECTIVE To review the literature on portal vein thrombosis in children and adolescents, focusing on its diagnosis, complications and treatment. SOURCES OF DATA The medical literature of the past 10 years was reviewed using the PubMed and MEDLINE search engines, with major focus on portal vein thrombosis and its clinical outcomes. The following keywords or expressions were used for the web search: portal vein thrombosis, extra-hepatic portal vein obstruction, prognosis, children, portal hypertension, esophagogastric varices. Additionally, we also reviewed the articles cited in the references of the initially selected papers, as well as relevant textbooks. SUMMARY OF THE FINDINGS Portal vein thrombosis is one of the most common causes of portal hypertension among children. The initial clinical manifestation is characterized either by episodes of upper gastrointestinal bleeding or by splenomegaly on routine clinical examination. The major complications include upper gastrointestinal bleeding, hypersplenism secondary to splenomegaly, growth retardation, and portal biliopathy. The diagnosis is made by abdominal Doppler ultrasonography. Treatment is targeted at the complications and includes primary and secondary prophylaxis against upper gastrointestinal bleeding (which results from the rupture of esophageal varices), and portosystemic shunting in selected cases. CONCLUSIONS Portal vein thrombosis is one of the major triggers of upper gastrointestinal bleeding in children. Bleeding episodes have a remarkable effect on the quality of life of affected patients. Thus, appropriate diagnosis and treatment are needed in order to reduce morbidity and mortality.

Trombose de veia porta em crianças e adolescentes

OBJETIVO: Apresentar uma revisao atualizada de trombose de veia porta na infância e adolescencia, enfatizando o diagnostico, suas complicacoes e tratamento. FONTE DOS DADOS: Foi realizada revisao da literatura, dos ultimos 10 anos, atraves de pesquisa bibliografica na Internet nos principais sites de busca medica, como o PubMed e MEDLINE, com enfoque na doenca trombose de veia porta e suas repercussoes clinicas. As principais palavras-chave e expressoes pesquisadas foram: portal vein thrombosis, extra-hepatic portal vein obstruction, prognosis, children, portal hypertension, esophagogastric varices. Alem disso, foram consultados os artigos citados nas referencias dos trabalhos selecionados na pesquisa inicial e dos livros textos. SINTESE DOS DADOS: A trombose de veia porta e uma das causas mais comuns de hipertensao porta na infância. A apresentacao clinica inicial pode ser atraves de episodios de hemorragia digestiva ou da presenca de esplenomegalia em exame clinico de rotina. As principais complicacoes sao a hemorragia digestiva, hiperesplenismo secundario a esplenomegalia, retardo de crescimento e biliopatia portal. O diagnostico e realizado atraves da ultra-sonografia abdominal com Doppler. O tratamento e direcionado para as complicacoes, incluindo profilaxia primaria e secundaria de hemorragia digestiva, consequente a ruptura de varizes esofagicas, e derivacoes porto-sistemicas, em casos selecionados. CONCLUSOES:A trombose de veia porta e uma das causas mais importantes de hemorragia digestiva em criancas. Esses episodios acarretam impacto importante na qualidade de vida dos pacientes acometidos. Dessa forma, uma abordagem diagnostica e terapeutica adequada e desejavel na tentativa de se reduzir a morbimortalidade.

Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia

OBJECTIVES To define the sensitivity, specificity and accuracy of the ultrasound triangular cord sign and hepatic histopathology, in isolation or in combination, for diagnostic differentiation between biliary atresia and intrahepatic cholestasis. METHODS This was a retrospective study carried out between January 1990 and December 2004. Fifty-one cases of biliary atresia and 45 of intrahepatic cholestasis were analyzed. Histopathology was performed blind by a pathologist. The triangular cord sign was identified in ultrasound reports as the only diagnostic sign of biliary atresia. Sensitivity, specificity and accuracy were calculated for the triangular cord sign and histology both in isolation and in combination. The gold standard for diagnosis of biliary atresia was the appearance of the extrahepatic biliary tree via laparotomy. RESULTS The triangular cord sign alone had sensitivity of 49%, specificity of 100% and accuracy of 72.5%. Histopathology compatible with extrahepatic biliary obstruction alone had 90.2% sensitivity, 84.6% specificity and 87.8% accuracy. The triangular cord sign and histopathology in isolation or combination resulted in sensitivity of 93.2%, specificity of 85.7% and accuracy of 90.3%. CONCLUSIONS Finding the triangular cord sign on ultrasound is an indication for laparotomy. If the triangular cord sign is negative, liver biopsy is indicated; if histopathology reveals signs of biliary atresia, explorative laparotomy is indicated. In cases where the triangular cord sign is absent and histopathology indicates neonatal hepatitis or other intrahepatic cholestasis, clinical treatment or observation are recommended in accordance with the diagnosis.

Hepatite auto-imune tipo 1 em crianças e adolescentes: avaliação da suspensão do tratamento imunossupressor

Objective: To assess treatment withdrawal in children and adolescents with autoimmune hepatitis, with clinical and laboratory remission for a minimum period of 24 months, determining the relapse rate after treatment withdrawal. Method: This is a descriptive, retrospective and partially prospective study of 21 children and adolescents with type 1 autoimmune hepatitis treated at the Outpatient Division of Pediatric Hepatology, Teaching

Sickle cell disease: a clinical and histopathologic study of the liver in living children.

Purpose To study hepatic lesions in living children with sickle cell disease. Although such lesions are well known, the possibility of chronic persistent damage to the parenchyma resulting from the disease itself or iron overload is controversial. Patients and Methods Sixteen children with sickle cell disease aged 15 months to 18 years were prospectively investigated out of 741 patients younger than 20 years registered at Hemominas Foundation, Brazil. Inclusion criteria for biopsy were as follows: five had positive anti-hepatitis C virus (HCV) antibody, two had positive hepatitis B virus (HBV) surface antigen and persistently increased levels of aminotransferases, and nine underwent biopsy during abdominal surgery. Results Children with B or C hepatitis were significantly older than the others and also had increased levels of both alanine and aspartate aminotransferases. Lobular architecture was preserved in all nonviral cases; mild to moderate sinusoidal dilatation was present in eight, hepatocellular regenerative activity in six, and focal necrosis and corresponding mild inflammatory infiltrate in three. No fibrosis or bile duct damage was observed. Mild to severe hemosiderosis was present in all children except one. The degree of iron overload was clearly associated with the number of previous blood transfusions. All five children with HCV had chronic hepatitis. One of the children with HBV had incomplete and the other had definite cirrhosis. Conclusions These data indicate that chronic hepatic lesions in children with sickle cell disease may be caused by viral damage. Patients without evidence for viral etiology had reversible vascular lesions. Follow-up studies would be necessary to confirm this hypothesis but are precluded for ethical reasons. Hemosiderosis was not associated with fibrosis in any nonviral cases, but a larger number of patients would be necessary to establish valid conclusions.

[Autoimmune hepatitis in children and adolescents: clinical study, diagnosis and therapeutic response].

Objective: the aim of this study was to evaluate the clinical, laboratory and histopathological characteristics and the response to immunosuppression in children and adolescents with autoimmune hepatitis (AIH). Methods: the present research is a descriptive study consisting of 39 children and adolescents with AIH who receive care at the Department of Pediatric Gastroenterology of Hospital das Clinicas (UFMG) from 1986 to 1998. Results: childrens age ranged from 1.6 to 17 years (mean 8.7 ± 3.49), most of them were females (87.2%). There were three types of clinical presentations: chronic (53.9%), acute (41%), and serious hepatic failure (5.1%). The most relevant laboratory parameters were the aminotransferases and g-globulin increase. Antinuclear antibodies were positive in 66.7% of the patients, while smooth muscle antibodies were positive in 52.8% and anti-LKM1 in 3% of the patients. In the histopathology the most important findings were the piecemeal necrosis (93.7%), moderate to severe portal inflammation (78.1%), definitive or incomplete cirrhosis (76.9%), absence of lesion of biliary ducts (93.7%) and presence of rosettes (90.6%). During the treatment, 77.8% obtained complete resolution, associated to side effects in 27.8% of them. Seven patients died (17.9%). During the treatment there was significant z score reduction (p< 0.05) for height/age. Conclusions: after carrying out this study, we observed that the typical characteristics of AIH were: female sex, several clinical presentations, increased aminotransferase, and hypergammaglobulinemia. Histopathology showed a predominance of incipient and/or definitive cirrhosis associated with moderate to severe portal inflammation and piecemeal necrosis. Treatment using corticosteroids and azathioprine, turned out to be effective. However, the reduction in the height/age z score probably represents an adverse effect of corticoid treatment.

Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar

Objectives: To define the sensitivity, specificity and accuracy of the ultrasound triangular cord sign and hepatic histopathology, in isolation or in combination, for diagnostic differentiation between biliary atresia and intrahepatic cholestasis. Methods: This was a retrospective study carried out between January 1990 and December 2004. Fifty-one cases of biliary atresia and 45 of intrahepatic cholestasis were analyzed. Histopathology was performed blind by a pathologist. The triangular cord sign was identified in ultrasound reports as the only diagnostic sign of biliary atresia. Sensitivity, specificity and accuracy were calculated for the triangular cord sign and histology both in isolation and in combination. The gold standard for diagnosis of biliary atresia was the appearance of the extrahepatic biliary tree via laparotomy. Results: The triangular cord sign alone had sensitivity of 49%,

Coeliac disease in identical twins.

Coeliac disease occurred at the same age in MZ twins. The diagnosis was confirmed by histology of the small intestine, rapid response to a gluten-free diet, and relapse after reintroduction of gluten.