Portal Hypertension in Children: A Tertiary Center Experience in Turkey.

Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. Results: The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Conclusion: Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.

The evaluation of the use of 2D shear-wave ultrasound elastography in differentiation of clinically insignificant and significant liver fibrosis in pediatric age group.

OBJECTIVE: To determine the accuracy of 2D shear-wave elastography (2D-SWE) in pediatric age group patients in differentiating clinically insignificant and significant liver fibrosis using METAVIR fibrosis scoring system as the gold standard. INTRODUCTION: Liver biopsy has long been the gold standard in liver fibrosis diagnosis. However, due to probable complications and sampling variabilities, the need for more accurate and non-invasive techniques has increased. 2D-SWE is a non-invasive technique used in the evaluation of liver stiffness and utilized more and more in routine clinical practice with recent advances and researches. MATERIALS AND METHODS: In this retrospective single-center study, we included 46 pediatric age group patients who had a liver parenchymal biopsy and 2D-SWE evaluation regardless of etiology. For 2D-SWE, the LOGIQ E9 system (GE Medical Systems, Wisconsin, USA) and, for histopathological evaluation, METAVIR fibrosis scoring system were utilized. Patients were further subgrouped as clinically insignificant (METAVIR Score F0-1) and significant (METAVIR Score F2-4). The Kolmogorov-Smirnov and Mann-Whitney U tests were employed for statistical analysis. The diagnostic accuracy of 2D-SWE was assessed, and cutoff values were set by ROC curve analysis. RESULTS: kPa values were statistically different between clinically significant and insignificant fibrosis patient groups (p < 0.001). kPa value of 8.92 was designated as the best cutoff value according to the Youden Index. CONCLUSION: 2D-SWE is one of the non-invasive techniques in the evaluation of liver fibrosis. Our findings suggest that 2D-SWE accurately differentiate clinically insignificant and significant liver fibrosis.

Portal Hypertensive Biliopathy as a Cause of Severe Cholestasis in Children With Congenital Hepatic Fibrosis.

Portal hypertensive biliopathy may occur in patients with noncirrhotic hepatic fibrosis. Portal hypertensive biliopathy treatment should be focused on management of portal hypertension and relief of biliary obstruction. In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option.

Differential diagnosis in ulcerative colitis in an adolescent: Chronic granulomatous disease needs extra attention.

Chronic granulomatous disease (CGD) is a primary immune deficiency that is commonly diagnosed under the age of 5 years (95%) and is rarely seen in adulthood. CGD may manifest as inflammatory bowel disease (IBD) in childhood. Without proper diagnosis, these patients may be monitored for years as IBD; some may even be regarded as steroid-resistant ulcerative colitis (UC) and end up having a colectomy. In this case report, we described a patient who had been followed-up for years as UC and subsequently underwent colectomy, but was finally diagnosed in adulthood as primary immune deficiency.