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台 灣 腎 臟 醫 學 會 南 部 地 方 會 【第 213 次腎臟學學術討論會】 節目表及摘要 第 213 次腎臟學學術討論會 時 間:民國 99 年 3 月 14 日上午 8:30 – 12:00 地 點:高雄榮民總醫院 第二會議室 第六十四次透析人員繼續教育課程 【南部場次】 時 間:民國 99 年 3 月 14 日下午 2:00—5:00 地 點:高雄榮民總醫院 第一、二會議室 【北部場次】 時 間:民國 99 年 3 月 14 日上午 9:00—12:00 地 點:國防醫學院 致德堂 台 灣 腎 臟 醫 學 會 南 部 地 方 會 時間: 民國 99 年 3 月 14 日(星期日) 地點: 高雄榮民總醫院 第二會議室 ♣ 第 213 次腎臟學學術討論會 ♣ 【病例討論 I】 主持人: 李建德 李柏蒼 08:30 - 08:45 1. A Rare Case of Gravida with Transient Polyuria and Polydipsia after Sixth Month of Pregnancy 謝秉閶 周康茹 方華章 李柏蒼 陳建良 張子爰 許智揚 黃偉傑 鍾孝民 高雄榮民總醫院腎臟科 08:45 - 09:00 2. MALT Lymphoma-associated Waldenström Macroglobulinemia Presenting with Solitary Kidney Tumor: A Case Repor 紀伯叡1 裴松南2 黃棟樑3 黃純真4 黃惠勇1 李建德1 高雄長庚紀念醫院 腎臟科1 血液腫瘤科2 放射診斷科3 病理科4 09:00 - 09:15 3. Association of Light and Heavy Chain Amyloidosis with Monoclonal Immunoglobulin Deposition Disease: A Case Report 李俊儒1 李宜哲1 洪士元1 王志生2 蔡明凱3 陳逸鴻3 項正川3 義大醫院 內科部腎臟科 1 高雄榮民總醫院 病理部 2 國軍高雄總醫院 內科部腎臟科 3 09:15 - 09:30 4. Successful Treatment of Calcific Uremic Arteriolopathy: A Case Report and Literature Review 葉慧宗1 黃英哲1 陳建銘2 嘉義市宏醫診所1 長庚紀念醫院嘉義分院整型外科2 09:30 - 09:45 5. Chronic Hypothyroidism Presented with Chronic Kidney Disease, Severe Rhabdomyolysis and Dyslipidemia 蘇信元 王明誠 宋俊明 曾進忠 程盟夫 張育誌 吳安邦 國立成功大學醫學院附設醫院 內科部腎臟科 09:45 - 10:00 Break 10:00 - 10:50 外賓演講 主持人:陳鴻鈞 主任 The Experience of Rapid Growth in a Peritoneal Dialysis Center In Southern China-Guangzhou Xueqing Yu, MD, PhD. Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, PR China. 1 【病例討論II】 主持人: 王明誠 郭美娟 10:50 - 11:05 6. Thinking Approach for a Patient of Proteinuria with Discrepancy between Urine Dipstick and Urine Protein-to-Creatinine Ratio 黃俊祺 黃尚志 陳鴻鈞 高雄醫學大學附設醫院 内科部 腎臟內科 11:05 - 11:20 7. An Unrecognized Spontaneous Ruptured Bladder Due To Tuberculosis and Concurrent Urinary Tract Infection 李岳庭 李隆志 蔡孟憲 1 楊文洲 2 林景坤 李建德 許國泰 高雄長庚紀念醫院 內科部 腎臟科 感染科 1 泌尿科 2 11:20 - 11:35 8. Tubulointerstitial Nephritis and Uveitis Syndrome (TINU Syndrome) with Fanconi Syndrome 姚彥宏 1,林崇棋 1,張由美 2,楊安航 3,黎思源 1,林志慶 1,林堯彬 1, 楊五常 1,楊智宇 1,* 1 台北榮民總醫院內科部腎臟科, 2 台北榮民總醫院眼科部, 3 台北榮民總醫院病理部 11:35 - 11:50 9. Renal Amyloidosis: A Patient with Ankylosing Spondylitis Presenting with Chronic Diarrhea and Progressive Renal Insufficiency 趙若雁, 王明誠, 宋俊明, 曾進忠, 程盟夫, 吳安邦, 張育誌 國立成功大學醫學院附設醫院 腎臟科 11:50 - 12:05 10. A Case with Glucocorticoid Deficiency Associated Hyponatremia Developed to Diabetes Inspidus after Glucocorticoid Replacement – A Case Report and Literature Review 潘怡如 周康茹 方華章 李柏蒼 陳建良 張子爰 許智揚 黃偉傑 鍾孝民 高雄榮民總醫院腎臟科 2 一個罕見的孕婦懷孕第六月後表現暫時性多尿和劇渴— 病例報告及文獻回顧 A Rare Case of Gravida with Transient Polyuria and Polydipsia after Sixth Month of Pregnancy 謝秉閶 周康茹 方華章 李柏蒼 陳建良 張子爰 許智揚 黃偉傑 鍾孝民 Ping-Chang Hsieh, MD; Kang-Ju Chou, MD; Hua-Chang Fang, MD; Po-Tsang Lee, MD; Chien-Liang Chen, MD; Tsu-Yuan Chang, MD; Chih-Yang Hsu, MD; Wei-Chieh Hunag, MD; Hsiao-Min Chung, MD 高雄榮民總醫院腎臟科 Division of Nephrology, Kaohsiung Veterans General Hospital Polyuria during pregnancy is rare, and it is a challenging diagnostic and therapeutic problem in clinical practice. The differential diagnosis of polyuria in pregnancy includes primary polydipsia, head trauma, ingestion of drugs, osmotic diuresis, and diabetes insipidus. Diabetes insipidus in pregnancy can be categorized into three groups depending on the response to arginine vasopressin (AVP) and 1-deamino-8-D-arginine vasopressin (dDAVP): AVP and dDAVP sensitive (central), AVP and dDAVP resistant (nephrogenic), and AVP-resistant and dDAVP sensitive (gestational). Here, we report a case of gestational diabetes insipidus. A healthy 31-year-old woman, gravida 2 para 1, whose first gestation had been normal, noted polyuria and polydipsia after her sixth month of pregnancy. She denied any medication and these symptoms continued. At 37 weeks and 2 days of gestation, she underwent a Cesarean section owing to breech presentation and delivered a healthy baby boy. Due to severe polyuria (7200ml urine in half of a day) and hypernatremia, she was transferred to our hospital. At the emergent department, she had a plasma sodium 169mmol/L, potassium 3.2mmol/L, calcium 9.6mg/dl, creatinine 1.1 mg/dl, and normal liver function test. Her urine specific gravity was <1.005 and there was no glycosuria. Water deprivation test revealed responsiveness to dDVAP (urine osmolality from 141 mOsmol/kg to 528 mOsmol/kg). Her plasma AVP level was 11.61 pg/ml, and other pituitary functions were normal. Brain MRI scan demonstrated absence of normal posterior pituitary lobe high signal intensity on T1WI. Intranasal dDAVP was given, and polyuria and polydipsia subsided. After 51 days, her polyuria and polydipsia improved, and her urine osmolality reached 486 mOsmol/kg after water restriction. 關鍵字:多尿,紝娠尿崩症,高鈉血症,懷孕 Key word:polyuria, gestational diabetes insipidus, hypernatremia, pregnancy 3 以單一腎臟腫瘤表現之 MALT 淋巴瘤併發 Waldenström Macroglobulinemia Waldenström Macroglobulinemia Associated MALT Lymphoma Presenting with Solitary Kidney Tumor: A Case Report 紀伯叡 1 裴松南 2 黃棟樑 3 黃純真 4 黃惠勇 1 李建德 1 Po-Jui Chi, Sung-Nan Pei, Tung-Liang Huang, Shun-Chen Huang, Yeong-Ng Hwee, Chien-Te Lee 高雄長庚紀念醫院 內科部 腎臟科 1 血液腫瘤科 2 放射診斷科 3 病理科 4 A 72-year-old lady with medical history of chronic kidney disease and gouty arthritis for five years had incidentally found a 5.8 x 5.4 cm mix-echoic mass over right middle kidney on renal echogram without relevant symptoms. Physical examination and laboratory test was with normal range except elevated serum creatinine (Cr) (Cr, 1.94 mg/dL, white blood cell count 5800 cells/uL, hemoglobin 12.2g/dL, calcium 9.4 mg/dL, uric acid 8.2 mg/dL, lactate dehydrogenase 187 u/L). Urine examination was unremarkable. Abdominal computed tomography (CT) disclosed an ill-defined homogenous soft tissue mass with mild enhancement measuring 6.4 x 5.6 cm over right kidney. No definite enlargement of retroperitoneal lymph node or pelvic side wall invasion was found. Renal biopsy was thus performed and histologic examination showed diffusely infiltration of small mature lymphoid cells, which is typical of mucosa-associated lymphoid tissue (MALT) lymphoma. Immunohistochemical stain revealed positive for CD20, and negative for CD5, CD10, CD15, CD30, CD23. Serum protein electrophoresis and immunofixation studies found the prescence of monoclonal IgM kappa paraproteinemia. Bone marrow study revealed positive staining with CD-20 lymphoid cell. She then received leukeran and prednisolone for eight months with regression of renal mass as evidenced by CT scan. Surgical removal was not performed during treatment course. MALT lymphoma can present with solitary mass in extra-medullary tissue including the kidney. Waldenström macroglobulinemia (WM) is one of the complications associated with lymphoproliferative disorders. Literature review did not find any report of renal MALT lymphoma with accompanying WM. Herein for the first time, we report a patient presented with renal mass, proved to be MALT lymphoma and complicated with WM. Her treatment response is uneventful and the renal mass has regressive change after chemotherapy. 關鍵字: MALT 淋巴瘤, 腎臟腫瘤, Waldenström 巨球蛋白血症 Keywords: MALT lymphoma, kidney tumor, Waldenström macroglobulinemia 4 類澱粉沉積症合併單株免疫球蛋白沉積症:病例報告 Association of Light and Heavy Chain Amyloidosis with Monoclonal Immunoglobulin Deposition Disease: A Case Report 李俊儒 1 李宜哲 1 洪士元 1 王志生 2 蔡明凱 3 陳逸鴻 3 項正川 3 Chun-Ju, Li M.D.1, Yi-Jer,Lee M.D.1 , Shih-Yuan, Hung M.D.1, Jyh-Seng, Wang M.D.2, Ming-Kai Tsai, M.D.3, I-Hung Chen, MD3, Jeng-Chuan Shiang, M.D3 義大醫院 內科部腎臟科 1 高雄榮民總醫院 病理部 2 國軍高雄總醫院 內科部腎臟科 3 1 Division of Nephrology, Department of Internal Medicine, E-DA Hospital. 2Department of pathology, Kaohsiung Veterans General Hospital. 3Division of Nephrology, Department of Internal Medicine, Kaohsuing Armed Forced General Hospital Among the varied and biochemically diverse group of protein folding disorders that are collectively known as the amyloidosis. AL-amyloidosis, where deposits are derived from the immunoglobulin light chain fragments, is one of the most prevalent forms of amyloidosis. In contrast, AH-amyloidosis, resulting from the deposition of immunoglobulin heavy chains, is a rare disease with very few cases thus far reported. The kidney light microscopic examination shows Congo red stained and electron microscopy shows non-branching fibrils with a diameter of 8-10 nm. In the contrast, monoclonal immunoglobulin deposition diseases (MIDD) are non Congo red stained and its most characteristic ultra structural feature is the presence of non-fibrillar, electron-dense deposits. Both amyloidosis and MIDD primarily affect older individuals and are always associated with some form of plasma cell dyscrasias. The cases with simultaneous presentation of both AL-amyloidosis and MIDD are rare. There was so far no report of association of AL/AH amyloidosis with MIDD. Here we present a case of coincident AL/AH amyloidosis and MIDD with the presentation of end-stage renal disease. This 55y/o man with hypertension history suffered from legs edema and dyspnea for 2 months. Physical examination showed bilateral chest basal rales, legs severe edema and splenomegaly. Lab data showed WBC:11.7×10 3/ul, Hb:12.4 g/dl , PLT:142×10 3/ul, BUN:89 mg/dl, Crea:9.1 mg/dl, Na:131 mEq/L, K:3.8 mEq/L, Albumin:2.9 g/dl, Urine analysis: WBC 0-1/HPF, RBC 0-1/HPF, Protein 3+, daily urine protein 1.8g. The IgG, IgA, IgM, C3, C4 levels were all within normal range, ANA, RPR/VDRL, HBsAg, Anti-HCV all showed negative. Renal echo showed renal parachymal disease with right renal size 8.86x4.09 cm and left renal size 9.88x4.44 cm. Serum immune electrophoresis showed IgA lambda gammopathy. Bone marrow biopsy showed normocellular bone marrow with no definite evidence of neoplasm present. Renal biopsy showed monoclonal immunoglobulin deposition disease, consistent with lambda light chain (AL) and IgA heavy chain (AH) amyloidosis. Unfortunately, his renal function deteriorated rapidly and started to receiving hemodialysis therapy about one month later. 關鍵字: 漿細胞惡質, 類澱粉沉積症, 單株免疫球蛋白沉積症,腎衰竭 Key words: Amyloidosis, Monoclonal immunoglobulin deposition disease, Plasma cell dyscrasias, Renal failure. 5 鈣化性尿毒性小動脈病變成功治療經驗:病例報告與文獻回顧 Successful Treatment of Calcific Uremic Arteriolopathy:A Case Report and Literature Review 葉慧宗 1 黃英哲 1 陳建銘 2 Hui-Tsung Yeh1 Ing-Jer Huang1 Chien-Ming Chen2 1 2 嘉義市宏醫診所 長庚紀念醫院嘉義分院整型外科 Hong Yi clinic, Chiayi, Taiwan1 Chang Gung Memorial Hospital, Plastic and Reconstructive Surgery, Chiayi department, Chiayi, Taiwan2 Calcific uremic arteriolopathy (CUA; calciphylaxis) is an uncommon but serious life-threatening complication of end-stage renal disease. It is characterized by calcifications in the media of small to medium-size blood vessels of the dermis and subcutaneous fat. Its clinical manifestations include violaceous or mottled skin lesions, plaques, and subcutaneous nodules, which can develop into painful ischemic ulcers with necrosis. Clinical suspicion is the single most important feature of the diagnosis. The routine use of biopsy as a diagnostic procedure is controversial, as it may produce a nonhealing ulcer. Current treatment focuses on early recognition and prevention of hyperparathyroidism, hypercalcemia, hyperphosphatemia, and elevated calcium-phoshate product, scrupulous and gentle surgical care and general supportive measures. Successful treatments of CUA with pharmacotherapeutic interventions such as sodium thiosulfate, bisphosphonates and cinacalcet have been published, but further research is necessary to fully describe the optimal use of these agents. A 71-year-old lady with end-stage renal failure secondary to Ig A nephropathy had undergone thrice weekly hemodialysis since 2001. Hyperparathyroidism and hyperphosphatemia had existed when she was transferred to our hemodialysis center in June 2002. Two painful skin lesions occurred spontaneously in medial aspect of left posterior calf in January 2009. Two weeks later these lesions developed into dusky ulcerations with eschar formation. An even smaller plaque-like cutaneous defect was noted in lateral aspect of left posterior calf just behind lateral malleolus. The diagnosis of CUA was highly suspected. All these lesions were excruciatingly painful and slowly progressed to large necrotic wounds. A series of X-ray examinations revealed significant calcification of aorta, vessels in pelvis and lower extremities. In nearby area of knee joint calcified vessels as small as 0.5 mm in diameter could be found. In addition to aggressive wound care and supportive treatment we had also tried sodium thiosulfate administration and a short course of oral Fosamax. However the necrotic wounds worsened and she needed surgical debridement at hospital. The procedure was performed perfectly on May 11, 2009. Successful percutaneous transluminal angioplasty was also carried out for peripheral artery occlusive disease of left lower extremity. She was discharged after one month of hospitalization and subsequently medicated with Cinacalcet 30 mg per day. Hyperparathyroidism, hypercalcemia and hyperphosphatemia got well control thereafter. The trouble lesions improved step by step and healed totally on August 5, 2009. 關鍵字: 鈣化性尿毒性小動脈病變,硫代硫酸鈉,雙磷酸鹽,cinacalcet Key words: calcific uremic arteriolopathy, sodium thiosulfate, bisphosphonate cinacalcet 6 慢性甲狀腺功能低下以慢性腎臟病, 嚴重橫紋肌溶解症與血脂異常為 表現 Chronic Hypothyroidism Presented with Chronic Kidney Disease, Severe Rhabdomyolysis and Dyslipidemia 蘇信元 王明誠 宋俊明 曾進忠 程盟夫 張育誌 吳安邦 Shin-Yuan Su, Ming-Cheng Wang, Junne-Ming Sung, Chin-Chung Tseng, Meng-Fu Cheng, Yu-Tzu Chang, An-Bang Wu 國立成功大學醫學院附設醫院 內科部腎臟科 Division of Nephrology, Department of Internal Medicine, National Cheng-Kung Univesity Hospital, Tainan, Taiwan Hypothyroidism may present with tiredness, weakness, dry skin, cold intolerance, weight gain with poor appetite, constipation and difficulty concentrating. However, dyslipidemia and mild to moderate elevated creatine kinase(CK) level are also noted. Reversible renal impairment associated with hypothyroidism was ever reported. We report a case with chronic hypothyroidism presented with chronic kidney disease, severe rhabdomyolysis and dyslipidemia. After thyroxine treatment, normalization of renal function, lipid profile and CK level is noted. A 35-year-old man with history of chronic kidney disease, stage II (baseline serum creatinine 1.5-1.8 mg/dL), chronic hepatitis B and dyslipidemia on Fluvastatin treatment presented with generalized weakness and malaise for two weeks. Severe rhabdomyolysis with peak CK level 11573 U/L was noted. Tracing back his history, he had stopped taking statin by himself for one month before the episode. So statin was not the cause for the severe rhabdomyolysis. After a series study, primary hypothyroidism was diagnosed (TSH 91 μIU/mL, total T4 0.5 μg/dL and total T3 < 19 ng/dL), and CK level declined gradually after thyroxine use. Interesting, normalization of renal function and lipid profile occurred in subsequent course. We should remind that rhabdomyolysis, reversible renal impairement and dyslipidemia may also be the presentation of hypothyroidism. If we can make the correct diagnosis early, some unnecessary medication can be avoided. 中文關鍵字: 甲狀腺功能低下, 慢性腎臟病, 橫紋肌溶解症, 血脂異常 Key word: hypothyroidism, chronic kidney disease, rhabdomyolysis, dyslipidemia 7 外賓演講The Experience of Rapid Growth in a Peritoneal Dialysis Center In Southern China-Guangzhou Xueqing Yu Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, PR China. Peritoneal dialysis (PD) is acknowledged worldwide as a well-accepted form of renal replacement therapy (RRT) for end-stage renal disease (ESRD). Its use throughout China has grown rapidly in recent years and it has been established as one of the main renal replacement therapies. Now there are nearly 20000 PD patients in China. Department of Nephrology The First Affiliated Hospital, Sun Yat-sen University is one of the National Key Departments in China. Of special relevance is the PD center, which has been in operation since 1964. During the period of 1964 to 1978, intermittent peritoneal dialysis (IPD) was used for the treatment of acute renal failure. In the late of 1970s, the first continuous ambulatory peritoneal dialysis (CAPD) protocol was initiated for the treatment of chronic renal failure in China. In 2006, a standard PD program was implemented for PD patient training, education, treatment and follow-up. With the establishment of a standard PD program in 2006, the number of PD patients has grown rapidly. By the end of 2009, the number of PD patients increased to 695. The yearly incident PD patients were 155, 195, 269 and 271, respectively. A total of 334 incident CAPD patients who began PD in our program from Jan 1, 2006, to Dec 31, 2007 were analyzed, of which 189 were male (56.6%) and mean age at the start of CAPD was 48.5 ± 16.2 years (15-82yr). Mean follow-up period was 25.8±12.1 (3.0 - 47.9) months. At the end of 1, 2, and 3 years, the patient survival rates were 91%, 82%, and 78%, respectively. Upon multivariate Cox proportional hazards analysis, higher age, diabetes, cardiovascular disease (CVD) and lower albumin at initiation were independent predictors of overall mortality. Cumulative technique survival (death-censored) at the end of 1, 2 and 3 years was 97%, 94%, and 92%, respectively. A total 130 episodes of peritonitis were observed in 83 patients. The overall peritonitis rate was 1 per 66.3 patient months. By the end of the observation period, the estimated median peritonitis-free time was 37.2 months(95% CI 35.2 -39.1 months). Our study revealed excellent short-term patient and technique survival and a low rate of peritonitis in our rapidly developing center. However, the long-term outcome on PD in our center needs to be investigated further. 8 C u m p a tie n t s u rviva l 1.0 Age <65ys 0.8 p=0.000 0.6 0.4 Age >65ys 0.2 0.0 0.00 10.00 20.00 30.00 40.00 50.00 Tim e o f fo llo w-u p in m o n th n 12m 24m 36m Age< 64ys 271 95% 87% 86% Age≥65 ys 63 75% 59% 45% Total 334 91% 82% 78% (318) (254.5) (148) Number exposed to risk Fig.1. Kaplan–Meier plot for cum patient survival according to age categories. 9 C u m te c h n iq u e s u rviva l 1.0 Age <65ys p=0.000 0.8 Age >65ys 0.6 0.4 0.2 0.0 0.00 10.00 20.00 30.00 40.00 50.00 Tim e o f fo llo w-u p in m o n th n 12m 24m 36m Age< 64ys 271 99% 95% 94% Age≥65 ys 63 89% 84% 74% Total 334 97% 93% 91% Number exposed to risk (308) (246.5) (146.5) Fig.2. Kaplan–Meier plot for cum technique survival according to age categories. 10 一蛋白尿病患尿液試紙和尿液蛋白/肌酸酐比值不相符的思考分析 Thinking Approach for a Patient of Proteinuria with Discrepancy between Urine Dipstick and Urine Protein-to-Creatinine Ratio 黃俊祺 黃尚志 陳鴻鈞 Jiun-Chi Huang, Shang-Jyh Hwang, Hung-Chun Chen 高雄醫學大學附設醫院 内科部 腎臟內科 Division of Nephrology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan Urine dipstick is widely used for screening patients with kidney disease and proteinuria. It mainly detects albumin in the urine. The drawback should be compensated by sulfosalicylic acid test, which detects all protein in the urine. However, sulfosalicylic acid test was gradually omitted and replaced by spot urine protein-to-creatinine ratio, as its convenience and reliability to measure daily protein loss in the urine, compared to collecting 24-hour urine. We reported a case with heavy proteinuria, and found mismatch between the above two modalities. A 62-year-old male with chronic kidney disease and hypertension, complaining of foamy urine for 3 months, presented to our nephrology out patient department. His urine dipstick for protein was only “+”, but nephrotic range of proteinuria (8.28 mg/mg) was noted by measuring spot urine protein-to-creatinine ratio. He was admitted for advanced study of nephrotic syndrome. Serum IgG, IgA and IgM levels were depressed. HbsAg, IgG Anti-HCV and cryoglobulin were all negative. However, serum albumin level was 4.46 g/dL, which is unexpected in a patient with nephrotic syndrome. Serum protein electrophoresis was carried out, but it showed no abnormal peak. Immunofixation electrophoresis demonstrated monoclonal lambda light chain in both serum and urine. Renal biopsy was performed as well as bone marrow aspiration. Immature plasma cells were more than 25% in bone marrow study. Skull x-ray showed punched-out osteolytic lesions. Therefore, this patient was diagnosed of multiple myeloma with lambda light chain monoclonal gammopathy. It reminds us that when discrepancy between urine dipstick and spot urine creatinine-to-protein ration in a patient with proteinuria, we should notice the probability of Bence Jones protein and search for evidence of multiple myeloma. Key words:Proteinuria, Urine dipstick, Chronic kidney disease 關鍵字:蛋白尿, 尿液試紙, 慢性腎衰竭 11 泌尿道結核合併尿道感染導致自發性膀胱破裂:病例報告 An Unrecognized Spontaneous Ruptured Bladder Due To Tuberculosis and Concurrent Urinary Tract Infection 李岳庭 李隆志 蔡孟憲 1 楊文洲 2 林景坤 李建德 許國泰 Yueh-Ting Lee, Lung-Chih Lee, Moan-Shane Tsai1, Wen-Chou Yang2, King-Kwan Lam, Chien-Te Lee, Kuo-Tai Hsu 高雄長庚紀念醫院 內科部 腎臟科 感染科 1 泌尿科 2 Division of Nephrology, Division of Infectious Diseases1, Department of Internal Medicine, Department of Urology2, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Kaohsiung, Taiwan Spontaneous bladder rupture (SBR) is an uncommon medical emergency with non-specific clinical features. SBR secondary to infection has rarely been reported. Herein, we encountered a 78-year-old man developed acute lower abdominal pain without trauma history. His hemodynamic status was stable initially and laboratory evaluation showed leukocytosis (WBC:27,800 cells/uL), acute renal failure (Cr:1.79 mg/dl) and marked pyuria. Abdominal computed tomography (CT) revealed moderate bilateral hydronephrosis and extraperitoneal bladder rupture. Urine culture yielded ESBL-E.coli. Conservative therapy with intravenous antibiotic (Flumarin 1g Q8h initially and subsequent Meropenam 1g Q8h according to culture sensitivity) and bladder catheter drainage were performed. Abdominal pain improved gradually. Cystoscopy later did not disclose evidence of SBR. He was discharged with bladder catheter drainage after 3 weeks’ hospitalization. But, patient was readmitted one week later for recurrent lower abdominal pain. Repeated abdominal CT showed progression of inflammatory stranding in lower abdominal areas. No remarkable perivesicular fluid was documented. Urine culture yielded Mycobacterium tuberculosis several weeks later. Anti-tuberculosis regimen (Pyrazinamide1250mg QD, Isoniazid 400mg QD, Rifampicin 600mg QD, and Ethambutol 800mg QD) were then prescribed. He was discharged on the 46th admission day with continuous anti-tuberculosis regimen for 9 months. Follow-up studies including CT scan and gallium scan revealed resolution of previous inflammatory process and urine culture did not yield any microorganism. His renal function remains rather stable. In conclusion, a high index of suspicion of SBR should be considered in patients presented with acute lower abdominal pain and signs of urinary tract infection. Conservative treatment only with bladder catheter drainage in the initial stage and anti-tuberculosis therapy has been successful in the treatment of genitourinary tract tuberculosis complicated with SBR. 關鍵字:自發性膀胱破裂、泌尿生殖結核病、泌尿道感染 Keywords: Spontaneous bladder rupture, genitourinary tuberculosis, urinary tract infection 12 腎小管間質炎與葡萄膜炎症候群合併范可尼氏症 Tubulointerstitial Nephritis and Uveitis Syndrome (TINU Syndrome) with Fanconi Syndrome 姚彥宏 1,林崇棋 1,張由美 2,楊安航 3,黎思源 1,林志慶 1,林堯彬 1,楊五常 1,楊智宇 1,* Yen-Hung Yao1, Chung-Chi Lin1, Yu-Mei Chung2, An-Hang Yang3, Szu-Yuan Li1, Chih-Ching Lin1, Yao-Ping Lin1, Wu-Chang Yang1, Chih-Yu Yang1,* 1 台北榮民總醫院內科部腎臟科, 2 台北榮民總醫院眼科部, 3 台北榮民總醫院病理部 1 Division of Nephrology, Department of Medicine, and 2 Department of Ophthalmology, and 3 Department of Pathology, Taipei Veterans General Hospital, Taipei, Taiwan We report a 57-year-old woman with concurrent tubulointerstitial nephritis and uveitis syndrome (TINU syndrome) and Fanconi syndrome. She presented with sudden onset of bilateral ocular pain, blurred vision, acute renal failure, glucosuria, and proteinuria. Slit lamp examination revealed acute bilateral anterior uveitis. Tubulointerstitial nephritis was confirmed by kidney biopsy. Laboratory examination revealed normoglycemic glucosuria, proteinuria, normal anion-gap metabolic acidosis, phosphaturia, urinary uric acid wasting, and kaliuresis leading to hypokalemia. Her vision and renal function improved gradually after systemic steroid therapy. There have been only five reports of TINU syndrome which had features of Fanconi syndrome; four of them are Japanese. Our patient is the first reported non-Japanese Asian with concurrent TINU syndrome and Fanconi syndrome. It is still not clear if there is any racial or gender factors. The prevalence of TINU syndrome must be underestimated, and its association with Fanconi syndrome requires further investigation. Key words: Tubulointerstitial nephritis, Uveitis, TINU syndrome, Fanconi syndrome 13 僵直性脊椎炎,續發全身性類澱粉沉積症併腎臟侵犯之病例報告 Renal Amyloidosis: A Patient with Ankylosing Spondylitis Presenting with Chronic Diarrhea and Progressive Renal Insufficiency 趙若雁, 王明誠, 宋俊明, 曾進忠, 程盟夫, 吳安邦, 張育誌 Jo-Yen Chao, Ming-Cheng Wang, Junne-Ming Sung, Chin-Chung Tseng, Meng-Fu Cheng, An-Bang Wu, Yu-Tzu Chang 國立成功大學醫學院附設醫院 腎臟科 Division of Nephrology, Department of Internal Medicine, National Cheng-Kung University Hospital, Tainan, Taiwan Systemic secondary (AA) amyloidosis is an uncommon but progressive complication following chronic inflammatory disease. Serum amyloid A, an acute phase reactant, deposits in various tissue leading to organ dysfunction. Ankylosing spondylitis, left untreated, may lead to secondary amyloidosis. We report a patient with ankylosing spondylitis and secondary renal amyloidosis which progressed to end-stage renal disease. A 41-year-old man presented with diffuse abdominal pain and chronic diarrhea for one month. He just underwent cholecystectomy for cholecystitis and the pathology of gall bladder showed amyloidosis. Gastric ulcer bleeding and colonic ulcer with amyloid deposition were found later. Ankylosing spondylitis was diagnosed subsequently with typical sacroiliitis present on radiograph and symptoms of chronic back pain, though not typically inflammatory. Afterward he had regular visits in rheumatology OPD. The level of erythrocyte sedimentation rate and C-reactive protein were persistently high despite treatment. One year later he admitted for renal insufficiency and newly-found proteinuria. Other symptoms included poor appetite, postprandial vomiting and diarrhea. Renal biopsy was done for rapidly deteriorated renal function. Amorphous eosinophilic material was found over the glomeruli with positive Congo-red stains. Renal amyloidosis was diagnosed, suspicious secondary to ankylosing spondylitis. Thus TNF-alpha blocker, Adalimumab was prescribed. However his renal function didn’t improve and renal replacement therapy was initiated later. He was free of gastrointestinal symptoms two months after the initiation of Adalimumab and urine output increased gradually. He is now still on maintenance hemodialysis. No severe infection ever occurred. TNF-alpha blockade provides a promising treatment strategy for systemic, including renal, AA amyloidosis if given early and timely. Renal function and proteinuria may stabilize or even improve once the systemic inflammation is under control. 中文關鍵字:類澱粉沉積症、僵直性脊椎炎、腎衰竭、腫瘤壞死因子阻斷劑 Keywords: Renal amyloidosis, ankylosing spondylitis, TNF-alpha blocker 14 一個低血鈉以類固醇治療後顯現尿崩症的病患—病例報告及文獻回顧 A Case with Glucocorticoid Deficiency Associated Hyponatremia Developed to Diabetes Inspidus after Glucocorticoid Replacement – A Case Report and Literature Review 潘怡如 周康茹 方華章 李柏蒼 陳建良 張子爰 許智揚 黃偉傑 鍾孝民 Yi-Ju Pan, MD; Kang-Ju Chou, MD; Hua-Chang Fang, MD; Po-Tsang Lee, MD; Chien-Liang Chen, MD; Tsu-Yuan Chang, MD; Chih-Yang Hsu, MD; Wei-Chieh Hunag, MD ; Hsiao-Min Chung, MD 高雄榮民總醫院腎臟科 Division of Nephrology, Kaohsiung Veterans General Hospital Glucocorticoid deficiency might cause hyponatremia that usually responds to glucocorticoid replacement. However, in patients with hypothalamic lesion with / without sella turcica involvement, latent diabetes insipidus could be unmasked after glucocorticoid replacement. We described a 69-year-old female who presented with central diabetes inspidus after glucocorticoid replacement for glucocorticoid deficiency related hyponatremia. She had history of right nasal cavity diffuse large B cell lymphoma and received chemotherapy + radiotherapy. She visited ED due to general malaise and vomiting, and euvolemic hyponatremia was noted. Hormonal studies disclosed panhypopituitarism with low ACTH, TSH, FSH /LH level. Brain MRI revealed a 1.5 cm soft tissue lesion over optic chiasm with marked post GD-DTPA enhancement. After glucocorticoid + thyroxine replacement, hypernatremia in the presence of dilute urine was found. Central diabetes inspidus (DI) was confirmed later by a significant increase in urine osmolality after vasopressin administration. This correlates with the endocrine concept that clinical manifestation of diabetes insipidus depends upon the functional integrity of the anterior lobe, in particular median eminence-pituitary-adrenal axis. In conclusion, central diabetes insipidus might develop after glucocorticoid replacement for glucocorticoid deficiency related hyponatremia. 關鍵詞:低血鈉,腎上腺醣皮質激素缺乏,尿崩症 Key word:hyponatremia, glucocorticoid deficiency, diabetes inspidus 15 台灣腎臟醫學會 第六十四透析人員繼續教育課程 【南部場次】 時 間:民國 99 年 3 月 14 日下午 2:00—5:00 地 點:高雄榮民總醫院 第 1.2 會議室 主 題:透析病患鈣、磷異常與心血管鈣化 主持人:張哲銘 醫師 14:00-14:40 1、高血磷症的治療 黃偉傑 醫師 高雄榮民總醫院 腎臟科 14:40-15:20 2、透析病患之骨骼病變 程盟夫 醫師 成大醫院 腎臟科 15:20-15:40 休 息 15:40-16:20 3、續發性副甲狀腺功能亢進與治療 張哲銘 醫師 高雄市立小港醫院 腎臟科 16:20-17:00 4、透析病患之心血管鈣化 蔡育哲 醫師 高雄長庚醫院 腎臟科 16 台灣腎臟醫學會 第六十四次透析人員繼續教育課程 【北部場次】 時 間:民國 99 年 3 月 14 日上午 9:00—12:00 地 點:國防醫學院 致德堂 主 題:透析病患鈣、磷異常與心血管鈣化 主持人:姜文智 醫師 09:00-09:40 1、高血磷症的治療 姜文智 醫師 台大醫院 腎臟科 09:40-10:20 2、透析病患之骨骼病變 許育瑞 醫師 三軍總醫院 腎臟科 10:20-10:40 休 息 主持人:林堯彬 醫師 10:40-11:20 3、續發性副甲狀腺功能亢進與治療 張明揚 醫師 林口長庚醫院 腎臟科 11:20-12:00 4、透析病患之心血管鈣化 林堯彬 醫師 台北榮民總醫院 腎臟科 17