• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.120-124
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• 2001

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.

• Clinical and Molecular Hepatology
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• v.24 no.4
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• pp.409-416
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• 2018

Background/Aims: Left ventricular diastolic dysfunction (LVDD) is an early manifestation of cardiac dysfunction in patients with liver cirrhosis (LC). However, the effect of LVDD on survival has not been clarified, especially in decompensated LC. Methods: We prospectively enrolled 70 patients with decompensated LC, including ascites or variceal bleeding, at Daejeon St. Mary's Hospital from April 2013 to April 2015. The cardiac function of these patients was evaluated using 2D echocardiography with tissue Doppler imaging. The diagnosis of LVDD was based on the American Society of Echocardiography guidelines. The primary endpoint was overall survival. Results: Forty-four patients (62.9%) had LVDD. During follow-up (22.3 months), 18 patients died (16 with LVDD and 2 without LVDD). The survival rate was significantly lower in patients with LVDD than in those without LVDD (31.1 months vs. 42.6 months, P=0.01). In a multivariate analysis, the Child-Pugh score and LVDD were independent predictors of survival. Moreover, patients with a ratio of early filling velocity to early diastolic mitral annular velocity (E/e') ${\geq}10$ (LVDD grade 2) had lower survival than patients with E/e' ratio < 10. Conclusions: The presence of LVDD is associated with poor survival in patients with decompensated LC. Therefore, it may be important to monitor and closely follow LVDD patients.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.3
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• pp.307-312
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• 2023

Hepatic arterioportal fistulae are abnormal communications between the hepatic artery and portal vein. They are reported to be congenital or acquired secondary to trauma, iatrogenic procedures, hepatic cirrhosis, and hepatocellular carcinoma, but less likely to occur spontaneously. Extrahepatic portal venous obstruction (EHPVO) can lead to pre-hepatic portal hypertension. A spontaneous superimposed hepatic arterioportal fistula can lead to pre-sinusoidal portal hypertension, further exacerbating its physiology. This report describes a young woman with long-standing EHPVO presenting with repeated upper gastrointestinal variceal bleeding and symptomatic hypersplenism. Computed tomography scan demonstrated a cavernous transformation of the portal vein and a macroscopic hepatic arterioportal fistula between the left hepatic artery and portal vein collateral in the central liver. The hepatic arterioportal fistula was associated with a flow-related left hepatic artery aneurysm and a portal venous collateral aneurysm proximal and distal to the fistula, respectively. Endovascular coiling was performed for the hepatic arterioportal fistula, followed by proximal splenorenal shunt procedure. This case illustrates an uncommon association of a spontaneous hepatic arterioportal fistula with EHPVO and the utility of a combined endovascular and surgical approach for managing multifactorial non-cirrhotic portal hypertension in such patients.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.350-365
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• 2023

Backgrounds/Aims: Extra hepatic portal venous obstruction (EHPVO) is the most common cause of portal hypertension in Indian children. While endoscopy is the primary modality of management, a subset of patients require surgery. This study aims to report the short- and long-term outcomes of EHPVO patients managed surgically. Methods: All the patients with EHPVO who underwent surgery between August 2007 and December 2021 were retrospectively reviewed. Postoperative complications were classified after Clavien-Dindo. Binary logistic regression in Wald methodology was used to determine the predictive factors responsible for unfavourable outcome. Results: Total of 202 patients with EHPVO were operated. Mean age of patients was 20.30 ± 9.96 years, and duration of illness, 90.05 ± 75.13 months. Most common indication for surgery was portal biliopathy (n = 59, 29.2%), followed by bleeding (n = 50, 24.8%). Total of 166 patients (82.2%) had shunt procedure. Splenectomy with esophagogastric devascularization was the second most common surgery (n = 20, 9.9%). Nine major postoperative complications (Clavien-Dindo > 3) were observed in 8 patients (4.0%), including 1 (0.5%) operative death. After a median follow-up of 56 months (15-156 months), 166 patients (82.2%) had favourable outcome. In multivariate analysis, associated splenic artery aneurysm (p = 0.007), isolated gastric varices (p = 0.004), preoperative endoscopic retrograde cholangiography and stenting (p = 0.015), and shunt occlusion (p < 0.001) were independent predictors of unfavourable long-term outcome. Conclusions: Surgery in EHPVO is safe, affords excellent short- and long-term outcome in patients with symptomatic EHPVO, and may be considered for secondary prophylaxis.

• Tuberculosis and Respiratory Diseases
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• v.41 no.5
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• pp.504-512
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• 1994

Background: It is well known that severe hypoxemia is often associated with liver cirrhosis without preexisting cardiac or pulmonary diseases. Pulmonary vascular impairments, more specifically, intrapulmonary shunting have been considered as a major mechanism. Intrapulmonary shunting arises from pulmonary vascular dilatation at the precapillary level or direct arteriovenous communication and has relationship with the characteristic skin findings of spider angioma. However, these results are mainly from Western countries where alcoholic and primary biliary cirrhosis are dominant cuases of cirrhosis. It is uncertain that the same is true in viral hepatitiss associated liver cirrhosis, which is dominant causes of liver cirrhosis in Korea. We investigated the incidences of hypoxemia and orthodeoxia in Korean cirrhotic patients dominantly composed of postnecrotic cirrhosis and the significance of intrapulmonary shunting as the suggested mechanism of hypoxemia, Method: We performed the arterial blood gas analysis separately both at the supine and errect position in 48 stable cirrhotic patients without the evidences of severe complications such as ascites, variceal bleeding, and hepatic coma. According to the results of arterial blood gas analysis, all patients were divided into hypoxemic and normoxemic group. In each group, pulmonary function test and Tc-99m-MAA whole body scan were performed. The shunting fraction was calculated based on the fact that the sum of cerebral and bilateral renal blood flow is 32% of the systemic blood flow. Results: The hypoxemia of $PaO_2$ less than 80 mmHg was observed in 9 patients(18.8%) and Orthodeoxia more than 10 mmHg was observed in 8 patients(16.7%). But there was no patient with significant hypoxemia of $PaO_2$ less than 60 mmHg. $PaO_2$ was significantly decreased in the patients with spider angioma than the pathients without spider angioma and showed no correlation with the serologic type and severities of liver function test findings. Any parameters of pulmonary function test did not demonstrate the difference between normoxemic and hypoxemic group. But hypoxemic group showed significantly increased shunt fraction of $11.4{\pm}4.1%$ than normoxemic group of $4.1{\pm}2.0%$ (p<0.05). Conclusions: Hypoxemia is not infrequently observed complication in liver cirrhosis and intrapulmonary shunting is suggested to p1ay a major ro1e in the development of hypxemia. But there was no great likelihood of clinically significant hypoxemia in our domestic cirrhotic patients predominantly composed of postnecrotic type.