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전압 장애성 원위부 신세뇨관성 산증과 신성 요붕증으로 발현한 Sjogren 증후군 1예
김태숙,김문재,송준호,이경주,이승우,최원식,문계혁 대한내과학회 2001 대한내과학회지 Vol.60 No.5
Distal renal tubular acidosis is a condition characterized by an inability of the distal nephron to acidify urine, causing hyperchloremic metabolic acidosis. Distal renal tubular acidosis is classified as proton secretory defect, permeability defect and voltage defect based on its pathophysiology. In the former two, serum level of potassium decreases due to increased excretion of potassium. But in the latter (voltage defect), hyperkalemia is characteristic by impaired the generation of an optimal electrical gradient for hydrogen ion and potassium secretion. We experienced a case of Sjogren`s syndrome associated with both voltage defect distal renal tubular acidosis and nephrogenic diabetes insipidus. The patient was a 58- year-old woman who complained of general weakness, nausea and xerostomia. Laboratory analysis showed metabolic acidosis with alkaline urine and hyperkalemia. Anti-nuclear antibody and anti-ds DNA antibody were positive. She presented with polyuria, low urine osmolarity and inadequate response to DDAVP. The response to Shirmer test was decreased. Salivary scintigraphy showed decrease of uptake in the parotid and submandibular salivary glands. We believe this is the first case report in which Sjogren`s syndrome is associated with both voltage defect distal renal tubular acidosis and nephrogenic diabetes insipidus.(Korean J Med 60:485-489, 2001)
정태연,손석만,이창화,이원재,백승덕,최우혁,정희철,정소연,김남일,서정일,양창헌,이창우,정기훈,김정란 東國大學校醫學硏究所 2002 東國醫學 Vol.9 No.2
위장관은 악성 림프종이 가장 흔히 침범하는 림프절 외 장기이지만, 원발성 소장 악성림프종이 비교적 드물게 발생한다. 장중첩증은 장 림프종의 드문 징후이며, 다양한 영상 검사에 의하여 진단된다. 저자들은 회장 장중첩증에 의해 우연히 발견된 림프종의 예를 보고하는 바이다. 조직학적 소견은 미만성 대 B 세포의 악성 림프종이였고, 중증도의 미만성 대비분열성세포이였다. 환자는 수술적인 절제술과 항암요법을 시행하였고 15개월간 재발이 없는 상태로 추적 관찰 중이다. Although the gastrointestinal tract is one of the most frequent sites of extranodal malignant lymphoma, the occurrence of primary small intestinal lymphoma is relatively uncommon. The intussusception is a rare presenting sign of intestinal lymphoma, and diagnosed by various imaging studies. We report on a case of the lymphoma on ileum manifested by ileo-ileal intussusception. Pathologic type was malignant lymphoma with diffuse large B cell (REAL classification), intermediate grade, diffuse large noncleaved cell (Working formulation). The patient received surgical resection and combined chemotherapy and doing well for 15 months.