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MED-13. Idiopathic retroperitoneal fibrosis: case-report.

A 38-year-old previously healthy African American male presented to ED with a dull lower abdominal and flanks pain for the past 5 weeks. No precipitating or aggravation factors were identified. He had been feeling nauseous. He had no complaints pertaining to urinary system. Physical examination was benign except for mild periorbital puffiness and mild vague tenderness over suprapubic region. Laboratory data revealed low hemoglobin (9.3 gm/dL), high blood urea nitrogen (29 mg/dL), high serum creatinine (4.8 mg/dL), low calcium (8.1 mg/dL) and high phosphorous (5.6 mg/dL). Urine examination showed occult blood. Ultrasound of the kidneys revealed mild to moderate bilateral hydronephrosis. CT scan of abdomen showed a retroperitoneal soft tissue density contributing to bilateral hydronephrosis and hydroureters. The open biopsy of mass showed fibrosis and chronic lymphoplasmacytic inflammatory infiltrate consistent with idiopathic retroperitoneal fibrosis. A diagnosis of obstructive uropathy secondary to idiopathic retroperitoneal fibrosis was made. Patient underwent bilateral percutaneous nephrostomies and was referred to tertiary care center for further management. Idiopathic retroperitoneal fibrosis in an uncommon connective tissue disorder. More common secondary causes of retroperitoneal fibrosis should always be excluded. Idiopathic retroperitoneal fibrosis usually occurs in individuals between 40-60 years of age. Our patient was slightly younger than the age group involved. The symptoms are insidious in onset and occur due to impingement of ureters and less commonly that of inferior vena cava or arterial system. During early stage patients usually experience a dull, non-colicky pain in girdle distribution. In the late stages symptoms due to ureteral obstruction predominate. Diagnosis is made on the basis of radiographic findings and biopsy. Treatment of idiopathic retroperitoneal fibrosis has been traditionally surgical. However, medical therapy with glucocorticoids and immunosuppressive agents has shown good results. Therefore, this relatively uncommon cause of obstructive uropathy should always be kept in mind.

M. Gautam, J. George, N. Shaikh, M. Nashat, and M. Rather. Mercy Catholic Medical Center, Drexel University College of Medicine, Darby PA.
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Title Annotation:Section on Internal Medicine
Author:Rather, M.
Publication:Southern Medical Journal
Date:Oct 1, 2004
Words:327
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