Esophageal Artery Pseudoaneurysm and Takayasu Arteritis in a Patient with Autosomal Dominant Polycystic Kidney Disease

김현숙,Yeonsil Yu,Kwang Eon Shim,Jin Eop Kim,Junga Koh,Jong-Woo Yoon,안규리,오윤규 전해질고혈압연구회 2018 Electrolytes & Blood Pressure Vol.16 No.1

A 47-year-old female previously diagnosed with ADPKD visited the hospital due to sudden pain in her upper abdomen and back. Esophagogastroduodenoscopy, contrast-enhanced abdominal computed tomography (CT), and CT angiography identified an esophageal artery pseudoaneurysm and hematoma in the esophagus. Urgent angiography and embolization were performed. After the procedure, CT angiography and positron emission tomography were performed due to differences in blood pressure between the arms. The patient was also found to have Takayasu arteritis and subsequently received outpatient follow-up care. The possible mechanisms that cause vascular abnormalities in ADPKD patients include damaged vascular integrity due to abnormal polycystin expression caused by PKD mutations and connective tissue abnormalities. Further research is needed to confirm these mechanisms, and ADPKD patients should be assessed for vascular abnormalities.

A Case of Post-radiotherapy Urethral Stricture with Spontaneous Bladder Rupture, Mimicking Obstructive Uropathy due to Cancer Metastasis

신준영,윤상민,최혁재,이시내,김해봉,주우철,송준호,김문재,이승우 전해질고혈압연구회 2014 Electrolytes & Blood Pressure Vol.12 No.1

Non-traumatic, spontaneous urinary bladder rupture is a rare complication of urethral stricture. Furthermore, its symptoms are often nonspecific, and misdiagnosis is common. The authors experienced a case of urethral stricture with spontaneous bladder rupture and bilateral hydronephrosis, mimicking obstructive uropathy attributed to cancer metastasis. A 55-year-old woman was admitted with abdominal pain and distension, oliguria, and an elevated serum creatinine level. She had undergone radical hysterectomy for uterine cervicalcancer and received post-operative concurrent chemoradiation therapy 13 years previously. Non-contrast enhanced computed tomography showed massive ascites and bilateral hydronephrosis. The initial diagnosis was acute kidney injury due to obstructive uropathy caused by malignant disease. After improvement of her renal function by bilateral percutaneous nephrostomy catheterization, contrast-enhanced computed tomography and a cytologic examination of ascites showed no evidence of malignancy. However, during retrograde pyelography, a severe urethral stricture was found, and subsequent cystography showed leakage of contrast into the peritoneal cavity and cystoscopy revealed a defect of the posterior bladder wall. After urethral dilatation and primary closure of the bladder wall, acute kidney injury and ascites were resolved.

Kidney and Phosphate Metabolism

최낙원 전해질고혈압연구회 2008 Electrolytes & Blood Pressure Vol.6 No.2

The serum phosphorus level is maintained through a complex interplay between intestinal absorption, exchange intracellular and bone storage pools, and renal tubular reabsorption. The kidney plays a major role in regulation of phosphorus homeostasis by renal tubular reabsorption. Type IIa and type IIc Na/Pi transporters are important renal Na‐dependent inorganic phosphate (Pi) transporters, which are expressed in the brush border membrane of proximal tubular cells. Both are regulated by dietary Pi intake, vitamine D, fibroblast growth factor 23 (FGF23) and parathyroid hormone. The expression of type IIa Na/Pi transporter result from hypophosphatemia quickly. However, type IIc appears to act more slowly. Physiological and pathophysiological alteration in renal Pi reabsorption are related to altered brush‐border membrane expression/content of the type II Na/Pi cotransporter. Many studies of genetic and acquired renal phosphate wasting disorders have led to the identification of novel genes. Two novel Pi regulating genes, PHEX and FGF23, play a role in the pathophysiology of genetic and acquired renal phosphate wasting disorders and studies are underway to define their mechanism on renal Pi regulation. In recent studies, sodium–hydrogen exchanger regulatory factor 1 (NHERF1) is reported as another new regulator for Pi reabsorption mechanism.

Pharmacologic Management of the Cardio-renal Syndrome

김창성 전해질고혈압연구회 2013 Electrolytes & Blood Pressure Vol.11 No.1

Cardio-renal syndromes are disorders of the heart and kidney wherein acute or long-term dysfunction in one organ may induce acute or long-term dysfunction of the other. Because of this complex organ interaction, management of cardiorenal syndrome must be tailored to the underlying pathophysiology. Clinical guidelines exist for the treatment of heart failure or renal failure as separate conditions. Thus far, however, there has been no consensus about managing patients with cardio-renal and reno-cardiac syndromes. Pharmacologic treatment remains a controversial subject. Standard cardiac drugs such as diuretics and inotropes may have limited effect because resistance often develops after long- term use. Recent studies of patients with acute cardio-renal syndromes have focused onnewer therapies, including phosphodiesterase inhibitors, vasopressin antagonists, adenosine A1 receptor antagonists, and renal protective dopamine. Initial clinical trials of these agents have shown encouraging results in some patients with heart failure, but have failed to demonstrate a clear superiority over more conventional treatments. Similarly, the benefits of diuretics, aspirin, erythropoietin agents, and iron supplements for management of chronic cardiorenal syndromes are unknown.

Thiazide-Induced Hyponatremia

황규식,김근호 전해질고혈압연구회 2012 Electrolytes & Blood Pressure Vol.10 No.1

The importance of thiazide-induced hyponatremia (TIH) is reemerging because thiazide diuretic prescription seems to be increasing after the guidelines recommending thiazides as first-line treatment of essential hypertension have been introduced. Thiazide diuretics act by inhibiting reabsorption of Na+ and Cl¯ from the distal convoluted tubule by blocking the thiazide-sensitive Na+/Clcotransporter. Thus, they inhibit electrolyte transport in the diluting segment and may impair urinary dilution in some vulnerable groups. Risk factors predisposing to TIH are old age, women, reduced body masses, and concurrent use of other medications that impair water excretion. While taking thiazides, the elderly may have a greater defect in water excretion after a water load compared with young subjects. Hyponatremia is usually induced within 2 weeks of starting the thiazide diuretic, but it can occur any time during thiazide therapy when subsequent contributory factors are complicated, such as reduction of renal function with aging, ingestion of other drugs that affect free water clearance, or changes in water or sodium intake. While some patients are volume depleted on presentation, most appear euvolemic. Notably serum levels of uric acid, creatinine and urea nitrogen are usually normal or low, suggestive of syndrome of inappropriate secretion of antidiuretic hormone. Despite numerous studies, the pathophysiological mechanisms underlying TIH are unclear. Although the traditional view is that diuretic-induced sodium or volume loss results in vasopressin-induced water retention, the following 3 main factors are implicated in TIH: stimulation of vasopressin secretion, reduced free-water clearance, and increased water intake. These factors will be discussed in this review. Key Words: hyponatremia; thiazides; water; diuretics; vasopressins

Delta Neutrophil Index is Useful to Predict Poor Outcomes in Male Patients with Alcoholic Ketoacidosis

Min Keun Kim,Han Wul Shin,You Jin Kim,Jae Won Yang,Jae Seok Kim,Byoung Geun Han,Seung Ok Choi,이준영 전해질고혈압연구회 2019 Electrolytes & Blood Pressure Vol.17 No.1

Background: Alcoholic ketoacidosis(AKA) is known as a benign disease, but the related mortality reported in Korea is high. Acidosis and alcohol change the immunity profile, and these changes can be identified early using the delta neutrophil index(DNI). We aimed to evaluate the use of DNI and other standard laboratory parameters as predictors of prognosis in AKA patients. Methods: One hundred eighteen males with AKA were evaluated at the Wonju Severance Christian hospital between 2009 and 2014. We performed a retrospective analysis of demographic, clinical, and laboratory parameters data. Receiver operating characteristic curves(ROC) and multivariate Cox regression was used to identify renal survival and mortality. Results: Survival patients had lower initial DNI levels than non-survival patients(4.8±6.4 vs 11.4±12.5, p<0.001). In multivariate-adjusted Cox regre- ssion analysis, higher initial increased DNI(HR 1.044, 95% CI 1.003-1.086, p=0.035), and lower initial pH(HR 0.044, 95% CI 0.004-0.452, p=0.008) were risk factors for dialysis during hospitalization. Further, higher initial DNI level(HR 1.037; 95% CI 1.006-1.069; p=0.018), lower initial pH(HR 0.049; 95% CI 0.008-0.312; p=0.001) and lower initial glomerular filtration rate(GFR)(HR 0.981; 95% CI 0.964-0.999; p=0.033) were predictors of mortality. A DNI value of 4.5% was selected as the cut-off value for poor prognosis and Kaplan-Meier plots showed that AKA patients with an initial level DNI ≥4.5% had lower cumulative survival rates than AKA patients with an initial DNI <4.5%. Conclusion: Increased initial serum DNI levels may help to predict renal sur- vival and prognosis in male AKA patients.

Transient Blindness in a Patient with Severe Metformin-Associated Lactic Acidosis(MALA)

Jae Wan Jeon,Wonjung Choi,Hae Ri Kim,Young Rok Ham,Dae Eun Choi,Ki Ryang Na,Kang Wook Lee,Soo Ya Bae,Seong Hoon Kim 전해질고혈압연구회 2019 Electrolytes & Blood Pressure Vol.17 No.1

A 68-year-old man presented at the emergency room with sudden blindness. The day before, he had eaten sashimi and eel and drank alcohol for dinner. He experienced nausea, vomiting, and dizziness afterward. His medical his- tory included hypertension and diabetes, and the latter was treated with met- formin. Initial laboratory tests revealed severe metabolic acidosis(lactic aci- dosis). Massive hydration and intravenous sodium bicarbonate replacement therapies were initiated, but severe metabolic acidosis(lactic acidosis) did not resolve, in turn, leading to hemodialysis, which decreased metabolic aci- dosis. The patient’s blindness improved, and his vision gradually recovered. As it is not easy to distinguish between blindness related to metformin-asso- ciated lactic acidosis(MALA) and blindness related to other causes, rapid correction of metabolic acidosis through hemodialysis might be helpful in differentiating this from of blindness from blindness related to other causes.

Pharmacologic Treatment of Chronic Hyperkalemia in Patients with Chronic Kidney Disease

Gheun-Ho Kim 전해질고혈압연구회 2019 Electrolytes & Blood Pressure Vol.17 No.1

Hyperkalemia is frequently complicated in patients with advanced chronic kidney disease(CKD) because kidney is the major route of potassium excretion. Urinary potassium excretion is reduced according to the decline in glomerular filtration rate, and the risk of hyperkalemia is increased in patients with high potassium intake, advanced age, diabetes mellitus, congestive heart failure, and medica- tions such as renin-angiotensin-aldosterone system(RAAS) blockades. On the other hand, the benefits of RAAS blockades and a high-potassium diet should be considered in CKD patients. To overcome these contradictory treatment stra- tegies, potassium binders have emerged as new options to enhance fecal pota- ssium excretion. In different regions of the world, four types of potassium binders are preferentially used. Whereas sodium polystyrene sulfonate(SPS) exchanges sodium for potassium, calcium polystyrene sulfonate(CPS) has the advantage of avoiding hypervolemia because it exchanges calcium for potassium. SPS was first introduced in the 1950s and used for a long time in western countries, and CPS is currently prescribed in Asia including South Korea. In contrast with the paucity of clinical studies using SPS or CPS, the recent ran- domized, controlled trials reported that two newer potassium binders, patiromer and sodium zirconium cyclosilicate(ZS-9), effectively and safely reduce serum potassium levels in CKD patients taking RAAS blockades. Our experiences showed that the long-term administration of a small dose of CPS was also effec- tive and safe in treatment of chronic hyperkalemia. Further comparative trials among patiromer, ZS-9, and CPS are required to provide guides to cost-effective management of hyperkalemia in CKD patients.

Uric Acid Puzzle: Dual Role as Anti-oxidantand Pro-oxidant

강덕희,하성규 전해질고혈압연구회 2014 Electrolytes & Blood Pressure Vol.12 No.1

Hyperuricemia is known to be associated with the presence of cardiovascular and metabolic syndrome and with the development of incipient kidney disease and an accelerated renal progression. However, an elevated uric acid level was not generally regarded as a true etiology or mediator, but an indicator of these diseases. Uric acid has recently regained the clinical interest and popularity based on emerging data suggesting the causative role of hyperuricemia in cardiovascular and renal disease. Experimental data demonstrates oxidative stress is one of the earliest phenomena observed in vascular, renal, liver cells and adipocytes exposed to uric acid. Since uric acid is one of the major antioxidantsof plasma acting as a free radical scavenger and a chelator of transitional metal ion, uric acid-induced oxidative stress seems paradoxical. Data regarding the clinical implication of hyperuricemia is even more confusing, which defines hyperuricemia as a useless parameter to be eliminated from routine follow-up or a major risk factor to be therapeutic target. With a review of experimental and epidemiologic data, the presence of molecular switch to regulate the role of uric acid as anti- or pro-oxidant in different compartment of our body is suggested, which may shed light on understanding the paradoxical role of uric acidand solving the “uric acid debate”.

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.