만성 대사성 산증에서 요 음이온차를 이용한 요 산성화능의 평가

한진석(Jin Suk Han),주권욱(Kwon Wook Joo),정윤철(Yoon Chul Jung),임춘수(Choon Soo Lim),김연수(Yon Su Kim),안규리(Cu Rie Ahn),김성권(Suhng Gwon Kim),이정상(Jung Sang Lee),김근호(Gheun Ho Kim) 대한내과학회 1993 대한내과학회지 Vol.45 No.4

N/A Objectives : The clinical usefulness of the urine anion gap as an indirect index of theammonium excretion was reported only in patients with hyperchloremic metabolic acidosis. And the significance of the urine anion gap and its relation with other indices of urine acidification are not clearly defined yet. We are aimed to evaluate the clinical usefulness of the urine anion gap and its relations with other indices of urine acidification in normal and high anion gap metabolic acidosis. Methods : We measured the urine acidification indices (ammonium, titratable acid, net acid) and the urine anion gap in 24 patients with chronic renal failure, 7 patients with dista1 renal tubular acidosis, and 8 healthy adults with acid loading (normal controls), whose arterial blood bicarbonate concentrations were 14.0 mmol/ L (7.0 to 20.0 mmol/L)(median;range), 18.1 mmol/L(15.0 to 20.0 mmol/L), 19.5 mmol/L(16.8 to 22.D mmol/L), respectively. Results: The urinary excretion of ammonium in chronic renal failure(4.5 mmol/day; 1.6 to 11.8 mmol/ day) and renal tubular acidosis (19.8 mmol/day; 6.9 to 27.2 mmol/day) were lower than in normal controls (52. 5mmol/day; 37.3 to 69.4mmol/day)(p<0.05). The urinary excretion of titratable acid in chronic renal failure (4.9 mmol/day; 0.1 to 19.7 mmol/day) and renal tubular acidosis (2.8 mmol/day; 0.1 to 20.2 mmol/day) were lower than in normal controls (6.2 mmol/day; 20.6 to 36.9mmol/day)(p<0.05) The urinary excretion of net acid in chronic renal failure(8.8 mmol/day; 0.1 to 28.2 mmol/day) and renal tubular acidosis (12.9 mmol/day; 0. 1 to 33.6 mmol/day) were also lower than in normal controls (77.9mmol/day; 62.7 to 98.9 mmol/day)(p<0. 05). The urine anion gap in chronic renal failure (22.9 mmol/L; 13.0 to 43.2 mmol/L) and renal tubular acidosis (36.0 mmol/L; 7.0 to 82.0 mmol/L) were higher than in normal controls (-14.6 mmol/L; 40.7 to 2.2 mmol/ L) (p<0.05), and had inverse relation with urine ammonium (r=-0.71, p<0.01), titratable acid (r=-0.76, p<0.01), and had inverse relation with urine ammonium (r=-0.83, p <0.01), respectively. The urine anion gap in chronic renal failure and renal tubular acidosis were all above 5.0 mmol/L. Conclusion : We concluded that the urine anion gap in chronic metabolic acidosis would be a good clinical index of the impairment of urine acidification in the distal nephron, and reflect not only urine ammmonium excretion but also urinary excretion of net acid.

고칼륨혈증을 동반한 원위 신세관성 산증의 임상상

강미정 ( Kang Mi Jeong ),곽충환 ( Gwag Chung Hwan ),진규복 ( Jin Gyu Bog ),황은아 ( Hwang Eun A ),한승엽 ( Han Seung Yeob ),박성배 ( Park Seong Bae ),김현철 ( Kim Hyeon Cheol ) 대한신장학회 2004 Kidney Research and Clinical Practice Vol.23 No.2

배 경 : 신세관성 산증은 신세관에서의 산성화 기능장애로 대사성 산증 초래하는 질환이다. 이중 제 4형 신세관성 산증은 원위 신원에서 칼륨과 수소이온의 분비장애로 인해 산혈증과 고칼륨혈증이 생기는 질환이다. 제 4형 신세관성 산증은 신세관성 산증 증에서 가장 빈도가 높으며 흔히 저레닌성 저알도스테론증이 동반됨이 알려져 있다. 그러나 아직도 국내에서는 세 4형 신세관성 산증에 관한 몇몇 증례만 보고되었을 뿐 종합적인 임상 보고는 거의 없다. 방 법 : 저자들은 1984년부터 2003년 3월까지 계명의대 동산병원에서 진단한 고칼륨혈증을 동반한 원위 신세관성 산증 50명을 대상으로 임상소견, 동반질환 및 전해질 소견, 치료 및 경과를 후향적으로 관찰하였다. 결 과 : 전체 50명 가운데 남자 23명, 여자 27명이었으며 평균 연령은 50.8≥19.5세였다. 동반질환으로는 신이식 후 14명(28%), 당뇨병 11명 (22%), 전신성 홍반성 남창 6명 (12%), 고혈압 6명 (12%), 만성 신세관간질성 신질환 6명 (12%), 그 외 윈인미상이 5예 (10%)였다. 임상증상으로는 무증상의 고칼륨혈증이 17명 (34%)으로 가장 많았고, 그 다음은 전신쇄약감 14명 (28 %), 전신부종 8명 (16%), 호흡곤란 5명 (10%), 기타 증상이 6예 (12%) 있었다. 진단시 신기능은 혈청 평균 creatinine 2.1±1.3 ㎎/dL, 평균 creatinine 청소율 25.6±16.4 mL/min 이었으며 혈청 pH는 7.3±0.05, 혈청 HCO₃^(-) 16.3±2.9 mEq/L을 보였다. 혈청 음이온 차는 평균 8.05±2.16, 혈청 Na는 평균 136.9±4.6 mEq/L, 혈청 Cl는 평균 110.9±5.2 mEq/L이었고, 혈청 K는 평균 6.18±0.64 mEq/L로 증가되었다. 요 pH는 평균 5.83±0.87이었고, 요 음이온차는 평균 37.9±30.6의 양의 값을 보였다. 혈장 레닌과 알도스데론치의 평균은 각각 3.82±7.16 ng/mL/hr, 110.2±108.16 ng/mL이었다. 치료는 9-α-fludrocortisone 단독이 19명 (38%), furosemide 단독 7명 (14%), K-교환수지 단독 5명 (10%), furosemide와 K-교환수지 병용 5명 (10%), 식이로 조절한 경우가 10명 (20%)이었다. 결 론 : 제 4형 신세관성 산증은 비교적 흔한 전해질 대사 질환으로 원인 미상의 고칼륨혈증이 동반될 경우 가장 먼저 의심해야할 질환 중의 하나로 조기 진단을 통한 병태생리적 치료가 필요할 것으로 생각된다. Purpose : Renal tubular aicdosis (RTA) is a disorder of renal acidification out of porportion to the reduction in glomerular filtration rate. Type Ⅳ RTA refers to hyperkalemic metabolic acidosis resulting from aldosterone deficiency or resistance. The incidence of each type RTA has not been reported exactly, however reports on type Ⅳ RTA have been recently increasing. Methods : A retrospective clinical analysis was performed in 50 patients with hyperkalemic distal renal tubular acidosis diagnosed between Jan. 1984 and Feb. 2003 at Department of Internal Medicine, Keimyung University, Dongsan Medical Center. Results : From 1984 to 2003, 50 cases of hyperkalemic distal renal tubular acidosis were diagnosed. The mean age was 50.8±19.5 years. The two most common conditions were posttransplantation (28%), and diabetes mellitus (22%), which were followed by hypertension (12%), systemic lupus erythematosus (12%), chronic renal failure (12%), and others (26%). Asymptomatic hyperkalemia (34%), and muscle weakness (28%) were the two most common clinical presentations. All patients demonstrated normal anion gap acidosis with positive urine anion gap. The mean creatinine learance was 25.6±16.4 mL/min. The mean baseline PRA and aldosterone levels were 3.82±7.16 ng/mL/hr and 110.02±108.2 ng/mL, respectively. Hyperkalemia was well responded to 9-α-fludrocortisone, furosemide, K-exchane resin, and combinations of these regimens. Conclusion : Type Ⅳ RTA is the most common type of RTA in children and adults, and can be an important cause of asymptomatic hyperkalemia. Therefore, type Ⅳ RTA should be included in the diffrential diagnosis of unexplained hyperkalemia in various clinical settings. (Korean J Nephrol 2004;23(2):263-269)

증례 : 그레이브스씨병과 동반된 원위부 신세관성 산증

황정원 ( Jung Won Hwang ),한군희 ( Koon Hee Han ),김영돈 ( Young Don Kim ),홍현일 ( Hyun Il Hong ),심명숙 ( Myoung Sook Shim ),김진엽 ( Jin Yub Kim ) 대한내과학회 2005 대한내과학회지 Vol.69 No.-

저자들은 갑상샘과다증 환자에서 원위 신세관 산증을 진단한 1예를 경험하였다. 갑상샘과다증 환자에서 저칼륨증에 의한 근육 마비가 발생한 경우 증상 호전 후에도 저칼륨증이 지속된다면 다른 원인을 감별해야 할 것으로 생각한다. Distal renal tubular acidosis is characterized by a decrease in net hydrogen ion secretion from renal collecting tubules. The most common causes of distal renal tubular acidosis in adults are autoimmune disorders, such as Sjogren syndrome, systemic lupus erythematosus, and rheumatoid arthritis. Compared to other autoimmune disorders, the concurrence of renal tubular acidosis and autoimmune thyroid disease is extremely rare. We report a patient who presented with hypokalemic paralysis associated with Graves` disease and distal renal tubular acidosis. (Korean J Med 69:S915-S918, 2005)

쇼그렌 증후군에 동반된 1형 신세뇨관 산증 및 골연화증 1예

이호준 ( Ho Jun Lee ),이성지 ( Sung Ji Lee ),박성창 ( Seong Chang Park ),박동진 ( Dong Jin Park ),김태종 ( Tae Jong Kim ),이신석 ( Shin Seok Lee ),박용욱 ( Yong Wook Park ) 대한류마티스학회 2012 대한류마티스학회지 Vol.19 No.4

Sjogren`s syndrome is a chronic autoimmune disease characterized by decreased function of the lacrimal and salivary glands. In addition, many other organs can be involved in patients with Sjogren`s syndrome. Overt or latent renal tubular acidosis is an uncommon extraglandular manifestation in Sjogren`s syndrome, and osteomalacia is a rare complication of renal tubular acidosis. It has been rarely reported that osteomalacia is associated with distal renal tubular acidosis in patients with Sjogren`s syndrome. We report a case of a 34-year-old female patient who was initially presented with muscle weakness in both lower extremities. The patient was then diagnosed with Sjogren`s syndrome complicated by osteomalacia and renal tubular acidosis.

원위부 신세뇨관성 산증으로 발현한 Sjo¨gren Syndrome 1예

장상아,한제호,손현식,윤건호,조철수,차봉연,김호연,이광우,손호영,강성구 대한내과학회 1993 대한내과학회지 Vol.44 No.6

저자들은 저칼륨혈증을 주증상으로 내원한 환자에서, 원위부 신세뇨관성 산증으로 발현된 Sjo¨gren's 증후군 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Distal renal tubular acidosis is a condition characterized by an inability of the distal nephron to acidify the urine, causing hyperchloremic metabolic acidosis, hypercalciuria, hypokalemia, nephrocalcinosis, and nephrolithiasis. Both hereditary and aquired forms have been described, the latter commonly associated with immune-mediated disorders. It has recently been noted that diseases characterized by hyperglobulinemia are occasionally complicated by renal tubular acidosis, and this combination of hyperglobulinemia and renal tubular acidosis has been reported in Sjo¨gren's syndrome and other immune-mediated disorders. Recently we experienced a case of Sjo¨gren's syndrome of which manifestations were severe hypokalemia, and other features of distal renal tubular acidosis. We presents a case of Sjo¨gren's syndrome complicated by renal tubular acidosis with literature review.

Incomplete Distal Renal Tubular Acidosis with Nephrocalcinosis

Joon Seok Choi,김창성,Jeong Woo Park,배은희,Seong Kwon Ma,Soo Wan Kim 전남대학교 의과학연구소 2011 전남의대학술지 Vol.47 No.3

We report the case of a female patient with incomplete distal renal tubular acidosis with nephrocalcinosis. She was admitted to the hospital because of acute pyelonephritis. Imaging studies showed dual medullary nephrocalcinosis. Subsequent evaluations revealed hypokalemia, hypocalcemia, hypercalciuria, and hypocitraturia with normal acid-base status. A modified tubular acidification test with NH4Cl confirmed a defect of urine acidification, which is compatible with incomplete distal tubular acidosis. We treated our patient with potassium citrate, which corrects hypokalemia and prevents further deposition of calcium salts.

Sjo¨gren 증후군과 자가 면역 갑상선 질환에 동반된 원위부 신세뇨관 산증 1례

김남원,이윤우,김철수,김예회 인제대학교 1990 仁濟醫學 Vol.11 No.3

Distal renal tubular acidosis is a disorder caused by a failure in maintaining steep hydrogen concentratin gradient between blood and urine, characterized by inappropriately high uring pH even under the state of severe acidosis. Renal tubular acidosis often coexist with autoimmune diseases such as Sjo‥gren's syndrome, hypergammaglobulinemic purpura, systemic lupus erythematosus, autoimmune liver and thyroid diseases. The immunologic injuries have been thought accountable for the renal derangement in these settings. Authors recently experienced a case of 24year old female who presented the manifestations of distal renal tubular acidosis and multi-organ involvement of autoimmune process such as Sjo‥gren's syndrome and hypothyroidism, The patient was treated with potassium, bicarbonate, and thyroxine supplement with marked symtomatic improvement.

그레이브스병에서 발생한 원위 신세뇨관 산증 1예

심은희,신영신,박민규,여창우,강진두,윤성운,이광재 대한노인병학회 2013 Annals of geriatric medicine and research Vol.17 No.3

Renal tubular acidosis (RTA) is a metabolic acidosis caused by impaired excretion of hydrogen ions or reabsorption of bicarbonate. Disorders caused by impairment of bicarbonate reabsorption in the proximal tubule are classified as proximal RTA, whereas those resulting from impairment of hydrogen ion secretion at the distal tubule are called distal RTA. The most common causes of distal renal tubular acidosis in adults are autoimmune disorders including Sjögren syndrome, systemic lupus erythematosis, rheumatoid arthritis, and autoimmune thyroiditis. Of the thyroiditis states, Graves’ disease-associated RTA is a rare disease. We experienced and managed one case of hypokalemic muscle weakness asso- ciated with Graves’ disease and distal renal tubular acidosis.

Adult Idiopathic Renal Fanconi Syndrome: A Case Report

Dae Jin Park,장기석,Gheun-Ho Kim 전해질고혈압연구회 2018 Electrolytes & Blood Pressure Vol.16 No.2

Renal Fanconi syndrome(RFS) is caused by generalized proximal tubular dysfunction and can be divided into hereditary and acquired form. Adult-onset RFS is usually associated with drug toxicity or systemic disorders, and modern molecular genetics may explain the etiology of previous idiopathic cases of RFS. Here, we report the case of a 52-year-old woman with RFS whose etiology could not be identified. She presented with features of phosphaturia, renal glucosuria, aminoaciduria, tubular proteinuria, and proximal renal tubular acidosis. Her family history was unremarkable, and previous medications were nonspecific. Her bone mineral density was compatible with osteoporosis, serum intact parathyroid hormone level was mildly elevated, and 25(OH) vitamin D level was insufficient. Her blood urea nitrogen and serum creatinine levels were 8.4 and 1.19mg/dL, respectively(estimated glomerular filtration rate, 53mL/min/1.73m2). Percutaneous renal biopsy was performed but revealed no specific renal pathology, including mitochondrial morphology. No mutation was detected in EHHADH gene. We propose the possibility of involvement of other genes or molecules in this case of adult RFS.

신이식후 발생한 고칼륨혈증

김현철(Hyun Chul Kim),이강욱(Kang Wook Lee),이원기(Won Kee Lee),박성배(Sung Bae Park),조원현(Won Hyun Cho),박철희(Choal Hee Park),전동석(Dong Seok Jeon),김재룡(Jae Ryong Kim) 대한내과학회 1991 대한내과학회지 Vol.40 No.5

N/A Disorders of renal tubular function following renal transplantation are being recognized with increasing frequency. The incidence of hyperkalemia following renal transplantation has significantly increased in cyclosporine treated patients compared to those treated with azathioprine. The inpatient and outpatient records of 115 consecutive cyclosporine-treated patients, who underwent renal transplantation at Keimyung University Medical Center from December 1984 to December 1989, were reviewed for the development of hyperkalemia during the 3-month period immediately following renal transplantation. Unexplained hyperkalemia developed in 33 out of the 115 (28.7%) cycloporine-treated renal transplant recipients. The average onset of hyperkalemia was 15.4 days (range: 8~35), and the mean duration of hyperkalemia was 17.5 days. Hyperkalemia was mild-to-moderate in most case, but only 3 cases were severe (K>6.5 mEq/L) enough to require emergency treatment. The mean dosage of cyclosporine was 9.0 ±1.8 mg/kg, and whole blood (serum) cyclosporine levels were in the higher range 694±268ng/ml (268±70ng/ml). Hyperkalemia was frequently associated with hyperchloremic acidosis and normal anion gap, which is consistent with type IV renal tubular acidosis. Despite hyperkalemia, plasma aldosterone levels were in the lower normal range, and plasma renin activity levels were normal. Further studies are needed to establish the exact mechanism of hyperkalemia after renal transplantation.