Faculty of Medicine
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Item type:Publication, Lymphangiomas of the liver and spleen: rare case presentation(2020); ; Objectives: Lymphatic malformations are benign lesions of vascular origin that show lymphatic differentiation. It is considered the lymphatic equivalent of a hemangiomas of blood vessels. A hepatic lymphangioma is a rare benign neoplasm and is usually associated with lymphangiomas of other viscera. It can occur at any age and most lesions are found inciden- tally. Splenic lymphangiomas are relatively rare benign tumors that correspond to abnormal dilatation of lymphatic chan- nels that can be either congenital or acquired. On imaging, they usually present as lobulated and multiloculated cystic le- sions without solid component or significant enhancement. Here we report a rare case of a hepatic cystic lymphangioma in a 73-year-old man and multiple lymphangiomas (cyst) in spleen. Material and methods: It was discovered on a routine ultrasound examination and the patient had no obvious symptoms. A surgical resection of adenocarcinoma of prostate was performed one year ago. There is no need of hemotherapy after sur- gical treatment. Abdominal ultrasonography and computed tomography (CT) showed “hepatic neoplasm” and multiple cys- tic focal lesions in the spleen. Bone biopsy excluded hematological abnormalities. Laboratory examination with tumor markers and X-chest ray were normal. Screening gastroscopy and colonoscopy was performed, and the patient had reflux esophagitis and pendular polyp in sigmoid colon. After polypectomy of sigmoid polyp, histopathology findings show tubu- lous polyp. After all examinations, spleen biopsy was performed. Results: Histological examination of spleen revealed multiple cystic structures lined with epithelial cells on the inner walls, accompanied by interstitial swelling and necrosis, marked as lymphangiomas. Conclusion: A hepatic lymphangioma can be solitary, cystic or associated with multiple liver lesions and is characterized by cystic dilatation of lymphatic vessels in the hepatic parenchyma. A solitary lymphangioma is unusual. Presentation ranges from asymptomatic incidental finding to a large multicentric, symptomatic mass require surgical intervention. They may occur alone on the spleen or as a part of systemic lymphangiomatosis. In our case patient has been followed up for nearly two years with no worsen and enlarged lesions. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Early diagnostic ultrastructural features of Wilson’s disease(Taylor & Francis Online, 2017-01-02); Wilson’s disease is an autosomal recessive genetic disorder of copper metabolism that is characterized by a defective incorporation of copper into ceruloplasmin. This leads to decreased excretion of copper and accumulation in excess in the liver, brain, and kidneys. Excess copper acts as a prooxidant and promotes generation of free radicals, leading to cell injury. We report a case with early ultrastructural characteristics. A 7-year-old female patient was admitted in the Pediatric Clinic because of abdominal pain and vomiting for a period of 5 days. Clinical examination showed palpatory firm and enlarged liver, 4 cm below the costal margin. Laboratory results showed elevated aminotransferases, low serum ceruloplasmin levels, negative serology for hepatotropic viruses, and absence of ophthalmologic symptoms. After the thorough examination, liver biopsy was done. Biopsy specimens were fixated in glutaraldehyde and embedded in Durcupan resin. Semi-thin sections dyed with Toluidine Blue and ultra-thin sections treated with uranyl acetate and lead citrate were made. Light-microscopic analysis of the semi-thin sections shows hepatic lobules with a moderate portal chronic inflammation and fibrosis. The trabecular architecture is disrupted as a result of fatty and parenchymal hepatocyte degeneration and focal piece-meal necrosis. There are evident apoptotic bodies and intranuclear glycogen inclusion. Electron-microscopic analysis of the ultra-thin sections shows degenerated hepatocytes, where the cytoplasm contains increased numbers of enlarged and relatively pleomorphic mitochondria with dilated cristae and intracristal spaces. Also, there is an increased number of peroxisomes, dilatation of smooth and rough endoplasmic reticulum, and neutral lipid vacuoles. There is discrete, plexiform electron-dense material in the cytosol of some hepatocytes located in the perinuclear and paranuclear regions, as well as in some mitochondria. Apart from the distinguishing ultrastructural features of the later symptomatic stage of Wilson’s disease (increased lipofuscin in pericanalicular regions, multivesiculated electron-dense copper deposits within the lysosomes, and finely granular hemosiderin electron densities), electron-microscopic analysis of an early stage disease can show only subtle and sometimes non-specific features. Nonetheless, transmission electron microscopy is a valuable diagnostic tool for this rare disease. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Acute haemolytic anemia with liver failure as initial clinical manifestations of Wilson's Disease.(Springer, 2015-09); ; ; ; Objective: Wilson's disease is an autosomal recessive disorder of copper transport. It results in accumulation of copper in the liver, brain, and other organs. Severe hemolytic anemia is an unusual clinical presentation. Since effective treatment is available for this disease, early and correct diagnosis is important. We report a case of a previously undiagnosed Wilson's disease revealed by acute intravascular hemolytic anemia and liver failure as an initial manifestation of disease. Method: A previously healthy, 11-year-old boy, presenting with abdominal pain, jaundice, tea-colored urine, and anemia was admitted to the pediatric hospital. Clinical and laboratory investigations for suspected hematological and hepatic disorder were immediately taken. Blood sample for genetic testing for Wilson's disease was performed. The patient was treated with plasmapheresis and other supportive treatment, but unfortunately the condition turned fatal 6 days after hospitalization. Results: Autopsy revealed shock with hemolytic anemia and respiratory distress syndrome superimposed on liver cirrhosis. Positive Orcein-Shikata and Rubeanic acid stain, ultrastructural visualization of electron-dense lysosomal deposits and liver copper content analysis, in conjuction with genetic results, confirmed the diagnosis of Wilson's disease as cause of cirrhosis. Conclusion: Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Synovial sarcoma of the liver - a case report(Scientific Foundation SPIROSKI, 2011-06-15); ; Jovcevski, AlenWe report a case of synovial sarcoma of liver in a 44 year old man, presented as a tumor mass in left hepatic lobe. The patient was admitted at the hospital with clinical symptoms of acute abdomen and severe pain in the right upper quadrant. Imaging examinations showed a tumor mass in the left hepatic lobe and free liquid in the abdominal cavity, due to the rupture of the tumor. A resection of 2 segments of the left hepatic lobe, where the tumor was located, was performed. Morphological, immunohistochemical and FISH studies confirmed the diagnosis of monophasic synovial sarcoma. Additional clinical and imaging examinations, made after the surgery, did not confirm tumor mass in any other localization. The patient refused any therapy other than surgery, at that time. A relapsing tumor mass was found 6 months later and another surgical intervention was done. The patient received five monotherapy cycles of Doxorubicin, 75 mg/m2, after the second surgical intervention. He is still alive 11 months after the first operation receiving the same therapy and having second relapsing inoperable tumor mass filling the retroperitoneal space and a great fraction of the abdominal cavity.