• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.40-43
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• 2016

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

• The Korean Journal of Medicine
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• v.93 no.6
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• pp.575-581
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• 2018

Retroperitoneal fibrosis (RPF) is a rare disease characterized by marked fibro-inflammatory tissue in the retroperitoneum. Approximately 70% of cases of RPF are idiopathic, while the rest can be secondary to several other causes. The diagnosis is mainly obtained by imaging modalities such as computed tomography (CT). However, histological examination should be considered when the clinical manifestations and imaging studies suggest malignancy. In particular, in malignant diseases with retroperitoneal metastases, abnormal collagen plaques are formed from an exuberant desmoplastic response, which may not be distinguishable from RPF on CT scans. Therefore, even if CT suggests RPF, biopsy is essential to identify malignant disease because it typically results in a fatal prognosis. Here, we report a case of metastatic adenocarcinoma of unknown primary site that developed only in the retroperitoneum and was initially diagnosed as RPF based on CT findings.

• Tuberculosis and Respiratory Diseases
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• v.44 no.4
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• pp.935-941
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• 1997

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retroperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic sclerosing mediastinitis with orbital fibrous dysplasia of unknowm cause, which was confirmed by open lung biopsy, so reported it with a review of literature.

• Nutrition Research and Practice
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• v.7 no.5
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• pp.366-372
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• 2013

The application of polyphenols has attracted great interest in the field of functional foods and nutraceuticals due to their potential health benefits in humans. However, the effectiveness of polyphenols depends on their bioactivity and bioavailability. In the present study, the bioactive component from green tea extract (GTE) was administrated orally (50 mg/kg body weight/day) as free or in a microencapsulated form with maltodextrin in rats fed a high fructose diet. High fructose diet induced features of metabolic syndrome including hypertriglyceridemia, hyperuricemia, increased serum total cholesterol, and retroperitoneal obesity. In addition, myocardial fibrosis was increased. In rats receiving high fructose diet, the lowering of blood triglycerides, total cholesterol, non esterified fatty acid (NEFA) and uric acid, as well as the reduction in final body weight and retroperitoneal fat weight associated with the administration of GTE, led to a reversal of the features of metabolic syndrome (P < 0.05). In particular, the administration of microencapsulated GTE decreased myocardial fibrosis and increased liver catalase activity consistent with a further alleviation of serum NEFA, and hyperuricemia compared to administration of GTE. Taken together, our results suggest that microencapsulation of the bioactive components of GTE might have a protective effect on cardiovasucular system by attenuating the adverse features of myocardial fibrosis, decreasing uric acid levels and increasing hepatic catalase activity effectively by protecting their bioactivities.

• Journal of Chest Surgery
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• v.52 no.4
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• pp.239-242
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• 2019

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1106-1110
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• 2006

Chylous ascites is a rare condition caused by various diseases and conditions that interfere with the abdominal or retroperitoneal lymphatics, and uncommonly it can manifest as a post-operative complication after abdominal, retroperitoneal or mediastinal surgery. Chylous ascites can be diagnosed by a high triglyceride content in ascites. The authors experienced a 5-year-old girl with congenital hepatic fibrosis who presented with chylous ascites after a splenorenal shunt operation, who was successfully managed by fasting and total parenteral nutrition, followed by a lipid-free diet with medium chain triglyceride supplementation. Here, the authors report this case of post-operative chylous ascites after a splenorenal shunt (Warren shunt) operation with a review of the pertinent literature.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.359-363
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• 2009

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.