• Journal of Yeungnam Medical Science
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• v.14 no.2
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• pp.399-414
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• 1997

There are several factors concerning to anemia in chronic renal failure patients. But when rHuEPO is used, most of these factors can be overcome, and the levels of hemoglobin are increased. However, about 10% of the renal failure patients represent rHuEPO-resistant anemia eventhough high dosage of rHuEPO. For these cases, desferrioxamine can be applied to correct rHuEPO resistnacy, and many mechanism of DFO are arguing. So we are going to know whether DFO can be applied to correct anemia of the such patients, how long its effect can be continued. The seven pateients as experimental group(DFO+EPO) who represent refractoriness to rHuEPO and the other seven patients as control group(EPO) were included. Experimental group had lower than 9 g/dL of hemoglobin levels despite high rHuEPO dosage (more than 4000U/Wk) and showed normocytic normochromic anemia. There were no definitve causes of anemia such as hemorrhage or iron deficiency. Control group patients had similar characteristics in age, mean dialysis duration but showed adequate response to rHuEPO. DFO was administered to experimental group for 8 weeks along with rHuEPO(the rHuEPO individual mean dosage had been determined by mean dosage of the previous 6 months. Total mean dosage; 123.5 U/Kg/Wk). After 8 weeks of DFO administration, the hemoglobin and rHuEPO dosage levels were checked for 15 consecutive months. It should be noted that the patients determined their own rHuEPO dosage levels according to hemoglobin levels and economic status. In conrol group, rHuEPO was administered by the same method used in experimental group without DFO through the same period. Fifteen months of observation period after DFO trial were divided as Time I(7 months after DFO trial) and Time II(8 months after Time I). The results are as follows: Before DFO trial, mean hemoglobin level of experimental group was 7.8 g/dL, which is similar level(p>0.05) to control group(mean Hb; 8.2 g/dL). But in experimental group, significantly(p<0.05) higher dosages of rHuEPO(mean; 123.5 U/Kg/Wk) than control group (mean; 41.6 U/Kg/Wk) had been used. It means resistancy to rHuEPO of experimental group. But after DFO trial, the hemoglobin levels of the experimental group were increased significantly(p<0.05), and these effect were continued to Time II.(Time I; mean 8.6g/dL, Time II; mean 8.6g/dL) The effects of DFO to hemoglobin were continued for 15 months after DFO trial with similar degree through Time I, Time II. Also, rHuEPO dosages used in the experimental group were decreased to similar levels of the control group after DFO trial and these effect were also continued for 15 months(Time I; mean 48.1 U/Kg/Wk. Time II; mean 51.8 U/Kg/Wk). In the same period, hemoglobin levels and rHuEPO dosages used in the control group were not changed significantly. Notibly, hemoglobin increment and rHuEPO usage decrement in experimental group were showed maxilly in the 1st month after DFO trial. That is, after the use of DFO, erythopoiesis was enhanced with a reduced rHuEPO dosage. So we think rHuEPO reisistancy can be overcome by DFO therapy. In conclusion, the DFO can improve the anemia caused by chronic renal failure at least over 1 year, and hence, can reduce the dosage of rHuEPO for anemia correction. Additional studies in order to determine the mechanism of DFO on erythropoiesis and careful attention to potential side effects of DFO will be needed.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.49-54
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• 2008

Background: Aortic diseases tend to involve the entire aorta. Hence, there is the constant possibility of the need for a secondary operation at the remnant aorta. This study analyzed our cases of secondary aortic surgery in order to determine its characteristics and problems. Material and Method: Between April 2003 and June 2007, 12 patients (6 male and 6 female) underwent thoracoabdominal aortic replacement as a secondary aortic operation. Their clinical courses were analyzed. Four of the patients underwent lower thoracobadominal aortic replacement under the normothermic femorofemoral bypass, and the others underwent an entire thoracobdominal aortic replacement under deep hypothermic circulatory arrest. Result: There was no death or paraplegia. As local complications, there were 3 cases of wound infection and 2 cases of an immediate reoperation caused by bleeding and one case of delayed wound. revision for a contaminated perigraft hematoma. As a systemic complication, there was one case of renal insufficiency, which required hemodialysis and one case of respiratory insufficiency that needed prolonged ventilator care. The mean admission period was $30{\pm}21$ days. All the patients were followed up for $626{\pm}542$ days without reoperation or other problems. Conclusion: Using properly selected patients and a careful approach, thoracoabdominal aortic replacement can be performed safely as a secondary aortic surgery.

• KSBB Journal
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• v.17 no.4
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• pp.403-408
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• 2002

For stimulating the in vivo secretion of IGF-1 (insulin-like growth factor-1) which is well known to promote the various physiological actions in human body, the natural herbal extract, YGF251 (young growth factor 251), was developed and evaluated for its effect as IGF-1 secretagogue in this study. The clinical study was peformed as double blind test, and 31 adult female and male volunteers between the age of 40 and 70 were investigated for their changes of concentration of IGF-1 , insulin level, weight, blood pressure, and liver and kidney functions. As the result of paired sample test on the change of the concentration of IGF-1, in YGF251 treated group, it was 245.6 ng/mL before dosing. The concentration of IGF-1 was increased to 269.3 ng/mL after a month and to 275.6 ng/mL after two months, and both were statistically significant (p〈0.05). While in control group, the concentration of IGF-1 was 280.0 ng/mL before dosing, but decreased to 239.2 ng/mL after a month and to 230.2 ng/mL after two months, and both were also statistically significant (p〈0.05). In YGF251 treated group, the concentration of insulin in blood increased about 2 times after a month dosing as an average level, but in control group, it showed a decrease of 36% compared with before dosing. And there were little changes regarding to the measured weight and blood pressure. Various measured data in order to observe the alteration in liver and kidney functions by the administration of YGF251 showed a little change within measuring error range.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.78-86
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• 2001

Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

• Journal of Chest Surgery
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• v.39 no.9 s.266
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• pp.692-698
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• 2006

Background: Owing to the fact that the average life span has increased and the progress in medical science has been made, the number of patients with chronic renal failure (CRF) who have to take hemodialysis (HD) has been going up gradually. Accordingly, it is considered to be as a significant issue to obtain blood vessels which can be used repetitively and supply enough blood flows. Therefore, there have been various kinds of study on an inosculation rate andfactors influencing it following an arteriovenous fistula (AV fistula) and lots of studies are ongoing for the purpose of escalating the inosculation rate. The authors analyzed the effects of short-term result, age, sex, diabetes and hypertension on arteriovenous inosculations in 134 anatomical snuffbox operated subjects among the patients who have taken an AV fistula at this center. Material and Method: Based on 134 patients who underwent an AV fistula at the department of thoracic surgery of this center from July, 2000 to May, 2004, the difference in arteriovenous inosculation rate was compared and analyzed depending or age (discriminated by 65-year-old), sex and the condition of the presence or absence of diabetes and hypertension. Correlation analyses were conducted for each parameter and statistical tests were performed by using SPSS for windows Release 11.0.1, which were determined to be statistically significant if p value was below 0.05. Result: The total number of operations was 169 including 35 of re-operations. The male/female rate was 70 : 64 (52% : 48%). The average age was $56.3{\pm}12.26$ years and there were 33 (24%) old aged patients above 65-year-old; there were 103 (71%) patients with hypertension and 90 (67%) patients with diabetes. Overall arteriovenous inosculation rate was $93{\pm}2.4%,\;91{\pm}2.7%,\;89{\pm}3.0%$ at 6, 12, 24 months, respectively. The arteriovenous inosculation rate of above 65-year-old patient group was $85{\pm}4.8%,\;80{\pm}5.8%,\;80{\pm}5.8%$ and below 64-year-old patient group's was $85{\pm}4.8%,\;80{\pm}5.8%,\;80{\pm}5.8%$ at given time points, respectively, which showed higher inosculation rate in below 64-year-old patient group with a statistical significance (p=0.0034). However, no statistical significance was found between the patients with hypertension and diabetes and the patients with no complication. In addition, there was no statistical significance in inosculation rate between male and female. Conclusion: The arteriovenous inosculation ratewas higher in the treated patient below 64-year-old than in the treated patient above 65-year-old. Thus it is advantageous for increase in long-term inosculation rate to obtain hemodialysis routes at an early age. The conditions of sex and the presence or absence of diabetes and hyper- tension do not make statistically significant effect on the arteriovenous inosculation rate.

• Journal of Chest Surgery
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• v.37 no.5
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• pp.416-422
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• 2004

Acute renal failure (ARF) is a common postoperative complication after the cardiac surgery. Postoperative ARF have various causes, and are combined with other complications rather than being the only a complication. It deteriorates the general condition of the patient, and makes it difficult to manage the combined complications by disturbing the adequate medication and fluid therapy. We have planned this study to evaluate the effects of postoperative ARF after the on-pump coronary artery bypass surgery (CABG) on the recovery of patients and identify the risk factors. Method and Material: We reviewed the medical records of patients who underwent CABG with cardiopulmonary bypass by a single surgeon from Jan. 2000 to Dec. 2002, We checked the preoperative factors; sex, age, history of previous serum creationism over 2.0 mg/㎗, preoperatively last checked serum creatinine, diabetes, hypertension, left ventricular ejection fraction, intraoperative factors; whether the operation is an emergent case or not, cardiopulmonary bypass time, aortic cross clamp time, the number of distal anastomosis, postoperative factors: IABP. Then we have studied the relations of these factors and the cases of postoperative peak serum creatinine over 2.0 mg/㎗. Result: There were 19 cases with postoperative peak serum creatinine over 2.0 mg/㎗ in a total 97 cases. Dialysis were done in 3 cases for ARF with pulmonary edema and severely reduced urine output. There were 8 cases (42.1%) with combined complications among the 19 patients. This finding showed a significant difference from the 5 cases (6,4%) in the patients whose creatinine level have not increased over 2.0 mg/㎗. The mortalities are different as 1.3% to 10.5%. The risk factors that are related with postoperative serum creatinine increment over 2.0 mg/㎗ are diabetes, the history of previous serum creatinine over 2.0 mg/㎗ and left ventricular ejection fraction. Conclusion: Postoperative ARF after the on-pump CABG is related with preoperative diabetes, the history of previous serum creatinine over 2,0 mg/㎗ and left ventricular ejection fraction. Postoperative ARF could De the reason for increased rate of complications and mortality after on-pump CABG. Therefore, in the patients with these risk factors, the efforts to prevent postoperative ARF like off-pump CABG should be considered.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Journal of the Korean Society of Food Science and Nutrition
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• v.31 no.5
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• pp.847-885
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• 2002

The purpose of this study was to develop a web-based internet program for nutritional assessment and diet Prescription by renal diseases. Renal diseases were classified by nephrotic syndrome, renal failure, hemodialysis and peritoneal dialysis. The system consisted of five parts according to their functions and contents. The first part is to assess the general health status such as body weight, obesity index, basal metabolic rate and total energy requirement by the input of age, sex, height, weight and degree of activity. The second part was designed to investigate dietary history of patient, that is, to find out his inappropriate dietary habit and give him some suggestions for appropriate dietary behavior by investigating his dietary history. This part also offers the diet and nutrition management by personal status with renal disease, and the information for food selection, snacks, convenience foods, dine-out, behavioral modification, cooking methods, food exchange lists and terms. The third part is evaluating their energy and nutrients intake by comparing with recommended dietary allowance for Koreans or standardized data for patient with renal disease. In this part, it is also analyzing energy and nutrients of food consumed by food group and meals, and evaluating the status of nutrient intake. The fort]1 one, a major part of the system, is implementing the diet and menu planning by using food exchange lists. This Part Provides the patient with menus lists and I day menu suitable to his weight, activity and the status of renal disease. The fifth part is providing information on energy and nutrients of foods and drinks, and top 20 foods classified by nutrients. These results are finally displayed as tabular forms and graphical forms on the computer screen.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.161-169
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• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.