• Electrolytes & blood pressure
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• v.16 no.2
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• pp.19-22
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• 2018

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 1.19 mg/dL, respectively (estimated glomerular filtration rate, $53mL/min/1.73m^2$). 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.

• The Journal of Korean Medicine
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• v.21 no.4
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• pp.276-285
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• 2000

Paraquat is a very potent herbicide which causes fatal toxicity when ingested, and there is no specific antidote against it. Human ingestion induces acute renal failure, hepatic dysfunction and progressive respiratory failure with high mortality rate. Clinical investigation and medical treatment were done on two cases of acute renal failure caused by paraquat poisoning admitted to the Department of Internal Medicine, Wonkwang University Oriental Chonju Medical Hospital. We report two cases of patients who survived after acute paraquat intoxication, by means of oriental medicine such as Gamdutang, a typical antidote of toxins, chinese ink as an absorbent and burned powder of Rhei Radix et Rhizoma for laxative and so on, western medicine such as gastric lavage, diuretics and fluid therapy. We suggest more experiments and studies related to such treatment for paraquat poisoning be conducted.

• Journal of Korean Clinical Nursing Research
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• v.24 no.3
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• pp.356-362
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• 2018

Purpose: This study was a retrospective cohort study to evaluate the effect of evidence-based guidelines for catheter dysfunction among hemodialysis patients, Success rate and bleeding complications in catheterization were examined. Methods: We performed a retrospective cohort study, including 94 patients with catheter dysfunction who were receiving hemodialysis at a university hospital; 55 in the control group and 39 in the protocol group. This protocol was composed of the catheter dysfunction assessment, conservative management of catheter dysfunction, drug management of catheter dysfunction, catheter function test and maintenance management. Data were analyzed with a Chi-square test and t-test using SPSS/WIN 23.0 program. Results: The patency rate of the protocol group was significantly higher than that of the control group. The rate of bleeding complications was not statistically significant for either the control group or the protocol group before or after application. Conclusion: The application of the guidelines effectively increased the patency rate of the catheter without bleeding complications. It is hoped that this guideline can be disseminated to nurses nationwide to improve the efficiency of catheter function for hemodialysis patients.

• Korean Journal of Clinical Laboratory Science
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• v.49 no.3
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• pp.203-213
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• 2017

Serum creatinine-based eGFR and serum cystatin C-based eGFR are the most popular methods for measuring renal function. Thyroid hormone is known to affect serum creatinine-based eGFR and serum cystatin C-based eGFR; however, the clinical significance of thyroid dysfunctional patients of renal function evaluation has not been fully elucidated to date. This study examined the effect of thyroid hormone on serum creatinine-based eGFR and serum cystatin C-based eGFR. Moreover, we also evaluated the correlation analysis between serum creatinine-based eGFR and serum cystatin C-based eGFR in patients with thyroid dysfunction. A total of 442 patients with hypothyroidism and 284 patients with hyperthyroidism were investigated. A correlation analysis between thyroid hormone and serum creatinine- (and cystatin C-) based eGFR was performed. A correlation analysis between thyroid hormone and serum cystatin-C based eGFR indicated that serum cystatin-C based eGFR is more of an independent biomarker than serum creatinine-based eGFR in thyroid dysfunction patients. Therefore, serum cystatin C-based eGFR more accurately reflects renal function than serum creatinine-based eGFR in thyroid dysfunction patients.

• Annals of Clinical Neurophysiology
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• v.3 no.2
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• pp.136-142
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• 2001

Background : Nervous system dysfunction is a major complication of end stage renal disease. Although severe neurologic symptoms are partially or completely reversed by adequate hemodialysis, even optimally dialyzed patients will usually not return to normal neurocognitive function. To investigate the influence of chronic renal failure and hemodialysis on higher cognitive function electrophysiologically, we studied auditory P300 event-related potentials in 14 hemodialysis patients and 14 age- and sex-matched normal healthy controls. Methods : The subjects consisted of 14 patients(M: 6, F: 8) with chronic renal failure(CRF) for 1 to 10 years and 14 age- and sex-matched healthy controls(M: 5, F: 9). For the reliability of study, patients with diabetes mellitus, abnormal brain CT findings, or low mini-mental state score(below 20) were excluded. Event related potentials(ERPs) for hemodialysis patients were performed at pre- and post-hemodialysis. To obtain ERPs, subjects underwent 2-tone auditory discrimination test(oddball paradigm). Results : Although the age(control: $48.79{\pm}10.31years$, CRF: $51.21{\pm}7.61years$) and mini-mental state score(control: $27.00{\pm}1.71points$, predialysis CRF: $25.07{\pm}3.58points$) were not different in normal control and CRF groups significantly(P>0.05), P300 latencies at Cz(control: $288.11{\pm}17.36msec$, predialysis CRF: $332.35{\pm}42.34msec$) were significantly delayed(P<0.05)and the duration of Trail making test A was significantly prolonged(control: $64.2{\pm}24.2sec$, CRF: $118.9{\pm}101sec$) in CRF group. P300 latencies between pre- and post-hemodialysis CRF patients(predialysis CRF: $332.35{\pm}42.34msec$, postdialysis CRF: $325.82{\pm}38.69msec$) were not significantly different. The P300 latency was not related with the duration of CRF(Spearman's correlation test, r=0.25, P>0.05) and the frequency of hemodialysis(Spearman's correlation test, r=0.28, P>0.05). Conclusions : From these results, we suggest that P300 latency is valuable in evaluating cognitive brain dysfunction in patients with CRF and hemodialysis does not have a significant effect on cognitive brain dysfunction in patients with CRF.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.121-126
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• 2023

The administration of angiotensin type 2 receptor blockers (ARBs) during pregnancy is known to cause ARB fetopathy, including renal insufficiency. We aimed to analyze the outcomes of two patients who survived ARB fetopathy and perform an accompanying literature review. Case 1 was exposed antenatally from a gestational age of 30 weeks to valsartan because of maternal pregnancy-induced hypertension. The patient presented with oliguria immediately after birth, and renal replacement therapy was administered for 24 days. Seven years after birth, renal function was indicative of stage 2 chronic kidney disease (CKD) with impaired urinary concentration. Case 2 had a maternal history of hypertension and transient ischemic attack and was treated with olmesartan until 30 weeks of pregnancy. Renal replacement therapy was performed for 4 days since birth. After 8 years, the patient is with CKD stage 2, with intact tubular function. Recent reports suggest that ARB fetopathy might manifest as renal tubular dysgenesis and nephrogenic diabetes insipidus, in contrast to mild alterations of glomerular filtration. Tubular dysfunction may induce CKD progression and growth retardation. Patients with ARB fetopathy should be monitored until adulthood. The ARB exposure period might be a critical factor in determining the severity and manifestations of fetopathy.

• Journal of Korean Society of Occupational and Environmental Hygiene
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• v.11 no.2
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• pp.153-160
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• 2001

Objectives : The authors evaluated the effects on renal function of workers chronically exposed to low-level organic solvent mixtures. Methods : The authors measured the level of urine ${\beta}2$-microglobulin(${\beta}2$-MG) and microalbumin as biochemical markers of renal function and damage in 29 male workers exposed to organic solvents for more than five years and compared their results with those of 30 male office clerks as a reference group. Results : 1. The mean values of hemoglobin, hematocrit, SGOT, SGPT, ${\gamma}$-GTP were all within normal limits and there was no significant difference, except for hemoglobin(p<0.01), between exposed and reference group. 2. The values of BUN and serum creatinine were within reference limits and there was no significant difference between exposed and reference group. 3. The difference of mean values of urine microalbumin corrected by urine creatinine were statistically significant (p<0.01), but those of urine ${\beta}2$-MG was not. 4. There were no correlation of urine hippuric acids with BUN, serum creatinine, urine microalbumin and ${\beta}2$-MG. 5. There were no significant difference of BUN, serum creatinine, urine microalbumin and ${\beta}2$-MG upon work duration. Conclusions: It is assumed that chronic low-level organic solvent exposure in these workers shows early renal dysfunction, glomerular changes. The result corresponds to previous studies showing the relationship between hydrocarbon exposure and glomerulonephritis. For evaluation of impairment on kidney tubules, we need further study using more precise markers and long-term follow-up.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.26-30
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• 2017

Newborn infants with huge and highly vascular sacrococcygeal teratoma (SCT) are frequently subjected to renal hypoperfusion secondary to high-output cardiac failure. Any underlying renal dysfunction is a significant risk factor for the development of contrast-induced nephropathy (CIN). However, reports on CIN in infants are rare. I report here a case of a premature infant born at 28 weeks and 3 days of gestation with a huge SCT who survived preoperative embolization and surgical resection but presented with persistent non-oliguric renal failure that was suggestive of CIN. During radiological intervention, a contrast medium had been administered at about 10 times the manufacturer-recommended dose for pediatric patients. Despite hemodynamic stabilization and normalization of urine output immediately following surgery, the patient's serum creatinine and cystatin-C levels did not return to baseline until 4 months after birth. No signs of reflux nephropathy were observed in follow-up imaging studies. Dosing guidelines for the use of a contrast medium in radiological interventions should be provided for infants or young patients.

• Journal of Genetic Medicine
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• v.10 no.1
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• pp.7-12
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• 2013

Familial Juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disorder, characterized by early onset of hyperuricemia, gout and progressive kidney disease. Hyperuricemia prior to renal impairment and decreased fractional excretion of uric acid are hallmarks of FJHN. Renal dysfunction gradually appears early in life and results in end-stage renal disease usually between the ages of 20 and 70 years. FJHN is mostly caused by mutations in the uromodulin gene located at 16p12. The course of FJHN is highly variable. Treatment includes management for hyperuricemia, gout and progressive kidney disease. Individuals with gout have been usually treated with allopurinol. But controversy exists as to whether uric acid lowering therapy prevents the progression of chronic kidney disease.