• Journal of Chest Surgery
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• v.47 no.2
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• pp.160-162
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• 2014

Cardiac involvement in hydatid disease is more seldom than the involvement of the liver and the lungs. Cardiac cyst hydatid disease is diagnosed incidentally or by means of symptoms such as dyspnea and angina pectoris. Here, we present the case of a 45-year-old male patient who underwent open heart surgery for a randomly detected cardiac cyst hydatid during investigations carried out in a healthcare institution after accidentally falling from height. On the other hand, this patient did not have any complaints associated with hydatid disease before this event.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.1
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• pp.70-78
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• 2022

Purpose: Cholestasis resulting from cytomegalovirus (CMV)-induced hepatitis manifests in 40% of patients with a CMV infection. Ganciclovir treatment in children with CMV infections has proven to be highly effective. Until now, there are very few studies have identified predictive factors for liver biochemistry improvement after ganciclovir therapy. This study aimed to identify the predictors of liver biochemistry improvement in patients with CMV cholestasis after ganciclovir treatment. Methods: A retrospective cohort study was conducted using medical records from Dr. Sardjito General Hospital Yogyakarta, Indonesia from 2013 to 2018. CMV cholestasis was confirmed based on serum CMV IgG and IgM positivity and/or blood and urine CMV antigenemia positivity. Incomplete medical records and other etiologies for cholestasis, such as biliary atresia, choledochal cyst, metabolic diseases, and Alagille syndrome, were excluded. Patient age at cholestasis diagnosis and ganciclovir treatment, duration of CMV cholestasis, history of prematurity, central nervous system involvement, and nutritional status were analyzed and presented as an odds ratio (OR) with a 95% confidence interval (95% CI). Results: CMV cholestasis with ganciclovir therapy was found in 41 of 54 patients. Multivariate analysis showed that a shorter duration of CMV cholestasis (OR: 4.6, 95% CI: 1.00-21.07, p=0.04) was statistically significant for liver biochemistry improvement after 1 month of ganciclovir treatment. The remaining factors that were analyzed were not significant predictors of liver biochemistry improvement in patients with CMV cholestasis after ganciclovir treatment. Conclusion: A shorter duration of CMV cholestasis is the predictor of liver biochemistry improvement after 1 month gancyclovir treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.146-153
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• 2020

Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD. Methods: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25). Results: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term. Conclusion: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.

• Tuberculosis and Respiratory Diseases
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• v.44 no.6
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• pp.1396-1402
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• 1997

Epithelioid Hemangioendothelioma(EH) is a rare vascular tumor, originating from endothelial cells. The principal locations are lung, soft tissue, bone and liver. This tumor is of borderline malignancy, relatively benign course. In the lung, the tumor is often multifocal, bilateral and frequently lead to the mistaken diagnosis of metastatic carcinoma. Although EH of the lung is relatively slow growing tumor, extensive pulmonary involvement, systemic metastasis, mainly to the liver have been documented. A 26-year-old man with EH involving the lung and liver was reported. Chest X-ray and chest CT showed multiple nodules in both lung fields and Abdominal CT multiple round low densities in liver. Transbronchial lung biopsy was performed. The patient diagnosed as EH by light microscopic finding and immunohistochemical study for Factor VIII-related antigen.

• Biomolecules & Therapeutics
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• v.32 no.3
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• pp.341-348
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• 2024

Endoplasmic reticulum (ER) stress plays a crucial role in liver diseases, affecting various types of hepatic cells. While studies have focused on the link between ER stress and hepatocytes as well as hepatic stellate cells (HSCs), the precise involvement of hepatic macrophages in ER stress-induced liver injury remains poorly understood. Here, we examined the effects of ER stress on hepatic macrophages and their role in liver injury. Acute ER stress led to the accumulation and activation of hepatic macrophages, which preceded hepatocyte apoptosis. Notably, macrophage depletion mitigated liver injury induced by ER stress, underscoring their detrimental role. Mechanistic studies revealed that ER stress stimulates macrophages predominantly via the PERK signaling pathway, regardless of its canonical substrate ATF4. hnRNPA1 has been identified as a crucial mediator of PERK-driven macrophage activation, as the overexpression of hnRNPA1 effectively reduced ER stress and suppressed pro-inflammatory activation. We observed that hnRNPA1 interacts with mRNAs that encode UPR-related proteins, indicating its role in the regulation of ER stress response in macrophages. These findings illuminate the cell type-specific responses to ER stress and the significance of hepatic macrophages in ER stress-induced liver injury. Collectively, the PERK-hnRNPA1 axis has been discovered as a molecular mechanism for macrophage activation, presenting prospective therapeutic targets for inflammatory hepatic diseases such as acute liver injury.

• Preventive Nutrition and Food Science
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• v.3 no.1
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• pp.56-61
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• 1998

This study was conducted to investigate the effect of dietary supplementation of vitamin A on the membrane property and ultrastructure in ethanol-administered rat livers. Male Sprague-Dawley rats weighing of 130 ~150g were fed with experimental diets for 7 weeks. The diets contained different types of vitamin A which were $\beta$-carotene, retinyl acetate and retinoic acid. After feeding theexperimental diets for 7 weeks, a dose of 3.0g ethanol (30%, W/V)/kg B.W was injected to rats intraperitoneally. Control rats received 0.9% saline containing isocaloric sucrose instead of ethanol. Plasma membrane fluidity of liver decreased in rats fed with vitamin a -Deficient diet with ethanol as compared to that of control rats. Fluidity change of liver plasma membrane that ethanol had induced was influenced by dietary supplementation of vitamin A, but not influenced by the type of supplemented vitamin. A . The ultrastructural changed of hepatic mitrochondria were observed in some rats such as vitamin A-deficient rats with ethanol. Inadequate consumptionof vitamin A contributed to ultrastructural changes such as swelled mitochondria occurred by ethanol-induced hepatotoxicity. Although accurate mechanism involved in the plasma membrane-stabilizing effect of vitamin A is still unclear, dietary supplementation of vitamin A such as retinyl acetate is neede to modulate this change. The direct involvement of membrane property on the cell damage caused by ethanol treatment remains to be established.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.109-113
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• 2024

Cholangiocarcinoma is a heterogeneous group of aggressive tumors that correspond to the second most common primary liver tumor. They can be classified according to their anatomical position concerning the biliary tree, and each subtype demonstrates different behavior and treatment. A 38-year-old male patient presenting solely right lumbar pain was diagnosed with a 7 cm hepatic tumor involving segments I, Iva, and VIII associated with involvement of the hepatic veins. He underwent a bloc resection of hepatic segments I, II, III, IV, partial V, partial VII, and VIII; right, middle, and left hepatic veins; and inferior vena cava segment, with perfusion of the remaining liver in situ with a preservation solution. As the patient had a large accessory inferior right hepatic vein draining the remaining liver, no reimplantation of hepatic veins was necessary. He remained clinically stable in outpatient follow-up, with excellent performance status-current survival of 2 years 6 months after surgical treatment.

• Proceedings of the Korean Society of Applied Pharmacology
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• 1993.04a
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• pp.71-71
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• 1993

Carboxylesterase is widely distributed in the tissues of vertebrates, insects, plants and mycobacteria. Among various tissues of animals and humans, the highest esterase activity with various substrates is found in the liver. Kidney has moderate carboxylesterase activity in the proximal tubules. Considerable esterase activity is also found in the small intestine epithet elial cells and serum of mammals. Besides these tissues, carboxylesterase has been found in the lung, testis, adipose tissue, nasal mucosa and even in the central nervous system. Hepatic microsomal carboxylesterase catalyzes the hydrolysis of a wide variety of endogenous and exogenous compounds such as carboxylester, thioester and aromatic amide. Since carboxylesterases are important for metabolic activation of prodrugs and detoxification of xenobiotics, differences in substrate specificity and immunological properties of this enzyme are important in connection with choosing a suitable laboratory animal for the evaluation of biotransformation and toxicity of drugs. On the other hand, liver, kidney, intestine and serum were found to contain multiple forms of carboxylesterases in animal species and humans. In fact, we have purified more than fifteen isoforms of carboxylesterases from microsomes of liver, kidney and intestinal mucosa of nine animal species and humans. and characteristics of these isoforms were compared each other in terms of their physical and immunochemical properties. On the other hand, we have reported that hepatic microsomal carboxylesterases are induced by many exogenous compounds such as phenobarbital, polycyclic aromatic hydrocarbons, Aroclor 1254, aminopyrine and clofibrate. Later, we showed that some isoforms of hepatic carboxylesterase were induced by glucocorticoids such as dexamethasone and 16 ${\alpha}$-carbonitrile, but other isoforms were rather inhibited by these compounds. These findings indicate that involvement of carboxylesterases in the metabolism and toxicity of drugs should be explained by the isoforms involved. Since 1991, we have carried out detailed research investigating the types of carboxylesterases involved in the metabolic activation of CPT-11, a derivative of camptothecin, to the active metabolite, SN-38. The results obtained strongly suggest that some isoforms of carboxylesterase of liver microsomes and intestinal mucosal membrane are exclusively involved in CPT-11 metabolism. In this symposium, the properties of carboxylesterase isoforms purified from liver, kidney and intestine of animal species and humans are outlined. In addition, metabolism of CPT-11, a novel antitumor agent, by carboxylesterases in relation to the effectiveness will also be discussed.

• Clinical and Experimental Pediatrics
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• v.58 no.6
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• pp.211-217
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• 2015

Purpose: Mycoplasma pneumoniae (MP) infection is a major cause of respiratory infection in school-aged children. Extrapulmonary manifestations of MP infection are common, but liver involvement has been rarely reported. The aim of this study was to determine the clinical characteristics of MP-associated hepatitis. Methods: This prospective study included 1,044 pediatric patients with MP infection diagnosed serologically with MP IgM at one medical center from January 2006 to December 2012. Eighty of these patients had elevated levels of serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT), each greater than 50 IU/L, without any other specific liver disorder and were compared with the 964 children without liver disorders. Results: In total, 7.7% of patients with MP infection had a diagnosis of hepatitis, especially in fall and winter. The ratio of male to female patients was 1.7:1, and the mean age of the patients was 5 years and 5 months. The most common symptoms were cough, fever, and sputum. Anorexia was the most common gastrointestinal symptom, followed by nausea/vomiting, diarrhea, and abdominal pain. Mean levels of AST and ALT were 100.65 IU/L and 118.73 IU/L, respectively. Serum AST/ALT level was normalized within 7.5 days on average without complications. The mean duration of hospitalization (11.3 days) was longer for children with hepatitis than for those without hepatitis (P=0.034). Conclusion: MP-associated hepatitis is not uncommon and has a relatively good prognosis. Therefore, clinicians should be concerned about liver involvement in MP infection but avoid further unnecessary evaluation of hepatitis associated with MP.