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논문분류	춘계학술대회 초록집
제목	Think Renal, Think Wise
저자	Dina Sallam
출판정보	2024; 2024(1):
키워드
초록	IgG4 related diseases (IgG4 RD) are autoimmune disease that is characterized by lymphoplasmacytic infiltrate, fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells, with an occasional elevated serum level of IgG4. Retroperitoneal fibrosis (RPF) is commonly occurred in IgG4 RD, with subsequent obstructive uropathy & acute kidney injury (AKI). Hereby we report a female patient who was had AKI that proved to be due to obstructive uropathy & RPF, which proved too be due to IgG4 RD. A female patient, 10 years old presented with recurrent attacks of epigastric pain, where investigations revealed AKI. Abdominal U/S showed right sided hydronephrosis, with a poorly marginated, periaortic mass, where CT PA with contrast revealed a confluent mass encasing the anterior and lateral sides of the aorta and compressing the IVC & mid-ureter with right sided hydronephrosis, dilated upper ureter with medial deviation of the proximal ureter and mid-ureter at the level of the obstruction. Surgical removal of the mass with decompression of ureters & IVC, with insertion of indwelling ureteral stent was done. Histopathological examination of the mass showed infiltrates with macrophages, plasma cells, B and T lymphocytes, where most plasma cells were positive for immunoglobulin G4 (IgG4). Serum level of igG4 was positive, where the diagnosis of igG4 RD was confirmed. Therapy was stated with steroid & Mycophenolate Mofetil (MMF) was started that improved the disease activity, with no flare ups for 3 years follow up. IgG4 RD should be considered in any patient with RPF, where early diagnosis & managment are necessary for prevention of morbidity & mortality.
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