Cirrhotic Cardiomyopathy

Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiologic disturbances and diastolic and/or systolic dysfunction in patients with chronic liver disease, especially those with ascites and portal hypertension. This disorder is a well-defined entity in adults, but pediatric data are limited. Clinical and laboratory findings are generally latent. The diagnostic criteria are prolonged QT on electrocardiography due to metabolic and extrahepatic causes, in addition to some abnormal echocardiography findings. If echocardiographic findings are normal and only specific prolonged QT is present, this disorder is named as “latent CCMP”; otherwise, it is “manifest CCMP.” This disorder is important because it may lead to problems such as cardiac failure and dysrhythmia before or after liver transplantation. Moreover, it may worsen the prognosis

Retrospective Comparison of Moderate and Severe Diaphragmatic Eventration in Children: Efficiency of Radiological Classification

WOS: 000443041100007Aim: Diaphragmatic eventration (DE) is a congenital or acquired elevation of the hemi-diaphragm. The indications for surgery may be challenging because clinical symptoms do not always correlate with radiological severity. We aim to identify the factors for the necessity and the efficiency of thoracoscopic diaphragmatic plication (TDP) in children with DE. Materials and Methods: A retrospective cross-sectional analysis of patients treated for DE (April 2006-August 2017) was performed. Demographics, type of DE, laterality, associated malformations and clinical symptoms were evaluated. Patients were grouped in two groups (moderate and severe) according to their diaphragmatic elevation levels on X-ray at admission. The severe DE group (SDE, n=14) had a DE of more than 2 vertebral bodies whereas the DE was 2 vertebral bodies or less in the moderate DE group (MDE, n=16). The groups were then compared regarding the necessity of TDP. The efficiency of TDP was analyzed by comparison of the outcome of patients who underwent TDP with that of conservative management. Results: There were 30 DE cases with a median age of 13.75 months. DE was acquired in 5 patients. The right side was the dominant side (21/30). The most common clinical symptoms were pneumonia (21) and respiratory distress (7) while 6 cases were asymptomatic. Acquired DE and respiratory distress were significantly higher in the SDE group. Four patients (25%) in the MDE group and 13 patients (92.9%) in the SDE group required TDP (p=0.000). The total number of cases of pneumonia was significantly higher in the conservatively treated patients in the follow-up (p=0.023). Conclusion: Two vertebral bodies may be an efficient cut-off level to discriminate between MDE and SDE. Absolute indications for TDP are SDE, acquired DE and respiratory distress at admission. Patients that are conservatively treated are more prone to pneumonia

Valproic Acid-Associated Vanishing Bile Duct Syndrome

Hepatotoxicity as a result of valproic acid therapy is well documented. Elevation in aminotransferase activities is rarely associated with symptoms. It sometimes manifests as acute liver failure. Here, we report a 8-year-old girl who was referred for unresolving jaundice and itching for 3 months. Past history revealed afebrile convulsion 5 months previously and beginning of valproic acid treatment. Valproic acid was discontinued after the development of jaundice. Physical examination revealed ichterus, xanthomas on extensor surfaces of extremities, and hepatomegaly without any sign of chronic liver disease. Total and direct bilirubin levels were 20.2 and 12.9 mg/dL, respectively. Enzyme activities indicating cholestasis were increased together with blood cholesterol. Tests for infectious and autoimmune, metabolic, and genetic disorders were not informative. Liver biopsy revealed portal inflammation, severe bile duct loss, and cholestasis. The patient was considered to have valproic acid-associated vanishing bile duct syndrome, which has not been reported previously

The role of the non-invasive serum marker FibroTest-ActiTest in the prediction of histological stage of fibrosis and activity in children with naive chronic hepatitis B infection

The aim of this study was to investigate whether the non-invasive serum marker FibroTest-ActiTest (FT-AT) reliably predicts the histological stage of fibrosis and/or activity, and decreases the need for a liver biopsy. Twenty-five children with naive chronic hepatitis B were analyzed for haptoglobin, alpha 2-macroglobulin, apolipoprotein A1, bilirubin, gamma-glutamyl transferase, and alanine aminotransferase activity, and the FT-AT scores were computed. FT-AT scores were compared with histological data. FT predicted insignificant fibrosis in 14/23 (61%) patients at a cut-off level of 0.31. Nine patients (36%) had significant histological fibrosis, but none were predicted by FT. There was no correlation between FT scores and histological stage of fibrosis (r: -0.221, p = 0.228). All 4 patients with significant histological activity had corresponding significant activity in AT (100%). Fifteen out of the 19 patients (78.9%) with significant activity in AT had insignificant histological activity. At the cut-off level of 0.36, AT predicted insignificant activity in all 6 patients (100%). There was no correlation between AT scores and histological activity (r: 0.245, p = 0.237). According to histological data, 12 patients were candidates for treatment, but FT-AT did not predict 3 of them (25%). FT-AT does not appear ready for use in detecting either the stage of fibrosis or activity in children with chronic hepatitis B

Portal Gastropathy and Duodenopathy in Children With Extrahepatic and Intrahepatic Portal Hypertension: Endoscopic Diagnosis and Histologic Scoring

Objectives: The aim of the study was to determine the frequency of portal gastropathy (PG) and duodenopathy (PD) in children, to document the correlation of various clinical and laboratory parameters associated with portal hypertensive gastroduodenal lesions, to compare the endoscopic portal hypertensive lesions with different histologic findings, and to evaluate the use of a possible histologic scoring system