Pediatric Acute Liver Failure as Presentation of Autoimmune Hepatitis: Learning from a Fatal Case.

Severe acute liver failure (SALF) is a rare condition in children. Up to 50% of the cryptogenic causes of SALF are associated with autoimmune hepatitis (AIH). This report presents a 5-year-old girl with progressive jaundice for 10 days. Her 1999 AIH diagnostic score totaled 11 points, compatible with probable AIH. She fulfilled the SALF criteria and the King's College criteria for liver transplantation, despite treatment with corticosteroids, and underwent the transplant, but died in the immediate postoperative period due to massive bleeding. Subsequently, the liver-kidney microsome type 1 result was 1:80, increasing the AIH score to 13 points. The final diagnosis was probable AIH type 2, associated with SALF. The biopsy of the explanted liver was compatible with fulminant hepatitis. This report highlights the difficult diagnosis of AIH in SALF, limitations of the diagnostic criteria for SALF in indications for emergency transplantation, and the uncertain therapeutic response produced by corticosteroids.

Non-invasive predictors of esophageous varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction.

OBJECTIVE: To identify non-invasive predictors of esophageal varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction (EHPVO). METHODS: 53 patients younger than 20 years with chronic liver disease or EHPVO and no history of bleeding or prophylactic treatment of esophageal varices (EV) were assessed. They were divided into 2 groups: group I (35 with chronic liver disease) and group II (18 with EHPVO). Their blood count, international normalized ratio (INR), albumin, bilirubin, abdominal ultrasonography and upper endoscopy results were taken. A splenic index was determined by dividing the patients' spleen dimension by its uppermost limit according to their age. The variables were compared to EV presence or not. Univariate (chi-square test, Fischer's exact test and Wilcoxon exact test) and multivariate (logistic regression) analyses were performed. A receiver operating characteristic (ROC) curve was constructed and the area under the ROC curve was calculated. RESULTS: EV were observed in 48.5% of group I patients and in 83.3% of group II patients. Low platelet count (p = 0.0015), splenomegaly (p = 0.0003) and splenic index (p = 0.0007) were statistically significant predictors of EV among group I patients. The multivariate analysis showed low platelet count (odds = 21.7) as an independent predictor of EV in patients with chronic liver disease. CONCLUSION: Platelet count, splenic index and platelet-splenic index ratio were predictors of EV in children and adolescents with chronic liver disease. There were no EV predictors among group II patients.

Preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta / Non-invasive predictors of esophageous varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction

OBJETIVO: Identificar preditores não invasivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta. PACIENTES E MÉTODOS: Estudo transversal que incluiu 53 crianças e adolescentes com hepatopatia crônica ou obstrução extra-hepática da veia porta, sem antecedente de hemorragia digestiva ou tratamento de varizes esofágicas, com até 20 anos de idade. Dois grupos foram formados: grupo I (35 pacientes com hepatopatia crônica) e grupo II (18 com obstrução extra-hepática da veia porta). Foram realizados hemograma, razão normalizada internacional, albumina, bilirrubina total, ultrassonografia de abdome e endoscopia digestiva alta. O índice esplênico foi determinado dividindo a dimensão esplênica pelo valor do limite superior da normalidade. As variáveis foram comparadas quanto à presença ou não de varizes esofágicas através de análise univariada (testes qui-quadrado, exato de Fischer e de Wilcoxon) e multivariada (regressão logística). A acurácia foi determinada a partir da área sob a curva ROC. RESULTADOS: As varizes esofágicas foram observadas em 48,5% dos pacientes do grupo I e em 83,3% do grupo II. Plaquetopenia (p = 0,0015), esplenomegalia (p = 0,0003) e a razão plaquetas/índice esplênico (p = 0,0007) se mostraram indicadores preditivos de varizes esofágicas entre os pacientes do grupo I. Após análise multivariada, a plaquetopenia (odds ratio = 21,7) se manteve como um indicador independente da presença de varizes esofágicas entre os pacientes com hepatopatia crônica. CONCLUSÃO: O número de plaquetas, o índice esplênico e a razão plaquetas/índice esplênico se mostraram preditivos de varizes esofágicas em crianças e adolescentes com hepatopatia crônica. Não foram encontrados preditores de varizes esofágicas entre os pacientes com obstrução extra-hepática da veia porta.

OBJECTIVE: To identify non-invasive predictors of esophageal varices in children and adolescents with chronic liver disease or extrahepatic portal venous obstruction (EHPVO). METHODS: 53 patients younger than 20 years with chronic liver disease or EHPVO and no history of bleeding or prophylactic treatment of esophageal varices (EV) were assessed. They were divided into 2 groups: group I (35 with chronic liver disease) and group II (18 with EHPVO). Their blood count, international normalized ratio (INR), albumin, bilirubin, abdominal ultrasonography and upper endoscopy results were taken. A splenic index was determined by dividing the patients' spleen dimension by its uppermost limit according to their age. The variables were compared to EV presence or not. Univariate (chi-square test, Fischer's exact test and Wilcoxon exact test) and multivariate (logistic regression) analyses were performed. A receiver operating characteristic (ROC) curve was constructed and the area under the ROC curve was calculated. RESULTS: EV were observed in 48.5% of group I patients and in 83.3% of group II patients. Low platelet count (p = 0.0015), splenomegaly (p = 0.0003) and splenic index (p = 0.0007) were statistically significant predictors of EV among group I patients. The multivariate analysis showed low platelet count (odds = 21.7) as an independent predictor of EV in patients with chronic liver disease. CONCLUSION: Platelet count, splenic index and platelet-splenic index ratio were predictors of EV in children and adolescents with chronic liver disease. There were no EV predictors among group II patients.

Cytomegalovirus frequency in neonatal intrahepatic cholestasis determined by serology, histology, immunohistochemistry and PCR.

AIM: To determine cytomegalovirus (CMV) frequency in neonatal intrahepatic cholestasis by serology, histological revision (searching for cytomegalic cells), immunohistochemistry, and polymerase chain reaction (PCR), and to verify the relationships among these methods. METHODS: The study comprised 101 non-consecutive infants submitted for hepatic biopsy between March 1982 and December 2005. Serological results were obtained from the patient's files and the other methods were performed on paraffin-embedded liver samples from hepatic biopsies. The following statistical measures were calculated: frequency, sensibility, specific positive predictive value, negative predictive value, and accuracy. RESULTS: The frequencies of positive results were as follows: serology, 7/64 (11%); histological revision, 0/84; immunohistochemistry, 1/44 (2%), and PCR, 6/77 (8%). Only one patient had positive immunohistochemical findings and a positive PCR. The following statistical measures were calculated between PCR and serology: sensitivity, 33.3%; specificity, 88.89%; positive predictive value, 28.57%; negative predictive value, 90.91%; and accuracy, 82.35%. CONCLUSION: The frequency of positive CMV varied among the tests. Serology presented the highest positive frequency. When compared to PCR, the sensitivity and positive predictive value of serology were low.

High frequency of human cytomegalovirus DNA in the liver of infants with extrahepatic neonatal cholestasis.

BACKGROUND: Biliary atresia (BA) is the most severe hepatic disorder in newborns and its etiopathogenesis remains unknown. Viral involvement has been proposed, including the human cytomegalovirus (HCMV). The aims of the study were to use the polymerase chain reaction (PCR) to screen the liver tissue of infants with extrahepatic cholestasis for HCMV and to correlate the results with serological antibodies against HCMV and histological findings. METHODS: A retrospective study in a tertiary care setting included 35 patients (31 BA, 1 BA associated with a choledochal cyst, 2 congenital stenosis of the distal common bile duct and 1 hepatic cyst). HCMV serology was determined by ELISA. Liver and porta hepatis were examined histologically. Liver samples from infants and a control group were screened for HCMV DNA. RESULTS: Twelve patients had HCMV negative serology, 9 were positive for IgG antibodies and 14 were positive for IgG and IgM. Nine liver and seven porta hepatis samples were positive for HCMV DNA but none of the control group were positive (general frequency of positivity was 34.3%-12/35). There was no correlation between HCMV positivity by PCR and the histological findings. The accuracy of serology for detecting HCMV antibodies was low. CONCLUSION: These results indicate an elevated frequency of HCMV in pediatric patients with extrahepatic neonatal cholestasis. They also show the low accuracy of serological tests for detecting active HCMV infection and the lack of correlation between HCMV positivity by PCR and the histopathological changes.

Steroid-induced cataracts in patients with autoimmune hepatitis.

BACKGROUND: Posterior subcapsular cataract (PSC) has been reported in patients on corticosteroids therapy for various pathologies. Autoimmune hepatitis (AIH) is a progressive inflammatory hepatic disease for which corticosteroids are recommended but PSC has not yet been reported in AIH patients on corticosteroids therapy. METHODS: The AIH patients at our service underwent an ophthalmological assessment and their files were analyzed establish the dosage and duration of medication, the presence of visual complaints and the type of AIH, in order to correlate these with the ophthalmological findings. Patients were examined with a slit lamp following pupil dilation. RESULTS: 17 AIH patients evaluated, only one patient had PSC. CONCLUSION: Although the frequency of PSC was low (1/17) in AIH patients on costicosteroid therapy, these individuals should undergo occasional ophthalmological assessment for the presence of cataracts since their visual acuity and quality of life may be adversely affected.

Steroid-induced cataracts in patients with autoimmune hepatitis.

BACKGROUND: Posterior subcapsular cataract (PSC) has been reported in patients on corticosteroids therapy for various pathologies. Autoimmune hepatitis (AIH) is a progressive inflammatory hepatic disease for which corticosteroids are recommended but PSC has not yet been reported in AIH patients on corticosteroids therapy. METHODS: The AIH patients at our service underwent an ophthalmological assessment and their files were analyzed establish the dosage and duration of medication, the presence of visual complaints and the type of AIH, in order to correlate these with the ophthalmological findings. Patients were examined with a slit lamp following pupil dilation. RESULTS: 17 AIH patients evaluated, only one patient had PSC. CONCLUSION: Although the frequency of PSC was low (1/17) in AIH patients on costicosteroid therapy, these individuals should undergo occasional ophthalmological assessment for the presence of cataracts since their visual acuity and quality of life may be adversely affected.