• Journal of Korean Academy of Fundamentals of Nursing
• /
• v.6 no.1
• /
• pp.78-95
• /
• 1999

The grafting of a kidney has been found to be the best medical treatment for patients who have renal insufficiency failure, but the patients still have experienced much trouble and apprehension. This study was done to further nursing theory developing for patients who have has a kidney graft from another person. The research method followed grounded theory methodology of Strauss and Corbin. The subjects were three female and four male patients. This study done befween Oct. 1997 and Mar. 1998. All of the subjects were interviewed by the author. Interview were done by the long interview technique and observation. In the process of data analysis, 'heart-boiling' was found to be the core phenomenon. The results were composed to 101 concepts. These concepts were grouped into nineteen categories, and then to twelve categories. There were 12 super-class categories as follows ; 'pain', 'heart-boiling', 'experience of dialysis', 'term of admission to a hospital', 'support of other person', 'dependence on God', 'direction', 'negative reaction', 'positive reaction', 'comfortable', 'lacking', 'acceptance'. In this process, 14 hypotheses were derived from the categories as follows ; (1) The more experience with dialysis that the patients have, the stronger the heart-boiling will tend to be. (2) The less experience with dialysis the patients have, the weaker the heart-boiling will tend to be. (3) The longer admission to hospital the patients have, the stronger the heart-boiling will be. (4) The shorter the admission to hospital the patients have, the weaker the heart-boiling will be. (5) The weaker the intense-grief is, the more positive the reaction to heart-boiling the patients wll have. (6) The stronger the intense-grief is, the more negative the reaction to heart-boiling the patients will have. (7) The stronger the support of other persons that the patients have, the more positive the reaction to heart-boiling the patients will have. (8) The weaker the support of other person that the patients have, the more negative the reaction to heart-boiling the patients will have. (9) The stronger the dependence on God that the patients have, the mure positive reaction to heart-boiling the patients will have. (10) The weaker the dependence on God that the patients have, the more negative reaction to heart-boiling the patients will have. (11) The more positive thoughts that the patients have, the more positive reaction to heart-boiling the patinets will have. (12) The more negative thoughts that the patients have, the more negative reaction to heart-boiling the patients will have. (13) The more positive reaction the patients have, the more free from heart-boiling the patients tend to be. (14) The more negative reaction the patients have, the less free from heart-boiling the patients tend to be. From the analysis of observed data and comparing each class, I concluded that there are four formula relation types between reaction of patients and heart-boiling. (1) If patients have the experience of dialysis, have a long term admission to hospital, are strong in heart-boiling, depend on God, have positive thoughts and another's strong support, they experience release by positive reaction to the intense-grief. (2) If patients have the experience of dialysis, have a short term admission to hospital, are weak in heart-boiling, do not depend on God, have negative thoughts, and have few supports from others, they experience attachment to heart-boiling though a negative reaction. (3) If patients have the experience of dialysis, have a long term admission to hospital, are strong in heart-boiling, do not depend on God, and have negative thoughts, they experience attachment to heart-boiling through negative reaction in spite of support from another. (4) If patients have the experience of dialysis, have a long term admission into hospital, are strong in heart-boiling and satisfaction is low, but they have positive thoughts, then they experience acceptance and harmony through the positive reaction to heart-boiling. The results of this study are expected to help the way nurses care for patients who have had a kidney graft from another.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.92-95
• /
• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Childhood Kidney Diseases
• /
• v.4 no.2
• /
• pp.92-101
• /
• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.

• Childhood Kidney Diseases
• /
• v.12 no.1
• /
• pp.38-46
• /
• 2008

Purpose: Growth failure is a common problem in chronic renal failure(CRF). We studied the effect of growth hormone(GH) treatment and the factors influencing growth on chronic peritoneal dialysis patients. Methods: Seventeen patients who were treated with peritoneal dialysis and GH for more than one year were enrolled. Factors influencing growth such as age, height at start of GH treatment, total Kt/Vurea, residual renal Kt/Vurea, hemoglobin, albumin, BUN, creatinine, total $CO_2$, calcium, phosphate and iPTH during GH treatment were compared between the growth group (increase in height-standard deviation score(Ht-SDS) after one year of GH treatment, n=l1) and poor growth group(no increase in Ht-SDS after one year of GH treatment, n=6). Results: The mean age at the start of dialysis was 7.7${\pm}$5.2 years and the mean age at the start of GH treatment was 8.5${\pm}$4.8 years. In the growth group, Ht-SDS at start of GH treatment was smaller(-1.72${\pm}$1.00 vs. -0.77${\pm}$0.88, P=0.048) and residual renal Kt/Vurea was better (1.54${\pm}$0.51 vs. 0.15${\pm}$0.26, P=0.02) than the poor growth group. After three years of GH treatment, Ht-SDS of the growth group was better than the poor growth group. Conclusion: GH treatment in children with peritoneal dialysis was more effective on patients who had more severe growth retardation. The reservation of residual renal function was important for improvement of effect of GH treatment. And the growth response during the first year of GH treatment may be predicted as the indicator for long-term response.

• Childhood Kidney Diseases
• /
• v.10 no.2
• /
• pp.152-161
• /
• 2006

Purpose : In Korea, the school urine screening program is a useful tool for screening urine abnormalities. It is particularly useful in early detection of membranoproliferative glomerulonephritis(MPGN) I, which frequently progresses to chronic renal failure. In this study, we studied the medical history, laboratory findings, and histologic findings of MPGN to gain helpful information on early detection and treatment. Methods : The subjects were 19 children, who were diagnosed with MPGN from kidney biopsies that were performed in ten nationwide university hospitals because of abnormal urine findings from school urine screening programs conducted from July 1999 to April 2004. We divided the patients into 2 groups, a nephrotic range proteinuria group(n=8) and a non-nephrotic proteinuria group(n=11), and retrospectively analyzed the clinical features, laboratory findings, histologic findings, treatment, and clinical course. Results : The mean age at the first abnormal urinalysis was $10.6{\pm}2.2$ years in the nephrotic proteinuria group and $9.6{\pm}3.2$ years in the non-nephrotic proteinuria group. The mean age at the time of kidney biopsy was $11.3{\pm}2.3$ years in the nephrotic range proteinuria group and $10.4{\pm}3.2$ years in the non-nephrotic proteinuria group respectively. There was no significant difference in the mean age and sex between the two groups. In the nephrotic proteinuria group, 6 children had a low plasma C3 level and in the non-nephrotic proteinuria group, 8 children had a low plasma C3 level, but there was no significant difference between the 2 groups. There was no significant difference in the laboratory test results(including WBC count, RBC count, platelet count and other serologic tests) between the 2 groups except for 24 hour urine protein secretion. There was no difference between the 2 groups with regard to the acute and chronic changes in the glomerulus on light microscopic findings, IgG, IgA, Ig M, C1q, C3, C4, fibrogen deposition on immunofluoroscence findings, and mesangial deposits, subendothelial deposits, and subepithelial deposits on electron microscopic findings. The children were treated with corticosteroids, ACE(angiotensin-converting enzyme) inhibitors, dipyridamole and other immunosuppressive agents. During the course of treatment, there were no children whose clinical condition worsened. Among 19 children, 3 children went into remission(2 in the nephrotic proteinuria group, 1 in the non-nephrotic proteinuria group) and 9 children went into a partial remission(4 in the nephrotic proteinuria group, 5 in the non-nephrotic proteinuria group) on urinalysis. There was no significant difference in the treatment results between the two groups. Conclusion : The 73.7% of children who were incidentally diagnosed with MPGN by the school urine screening program had reduced C3. 42.1% of the children had nephrotic range proteinuria. There were no significant differences in clinical features, laboratory test results, light microscopic, immunofluorescence microscopic, and electron microscopic findings between the nephrotic proteinuria group and the non-nephrotic proteinuria group except for the 24 hour urine protein secretion. Therefore, for early detection of MPGN during the school urine screening program, we strongly recommend a kidney biopsy if children have abnormal urine findings such as persistent proteinuria and persistent hematuria, or if the serum C3 is reduced.

• Childhood Kidney Diseases
• /
• v.1 no.1
• /
• pp.53-59
• /
• 1997

There have been many recent reports that unilateral renal agenesis and multicystic dysplastic kidneys are accompanied by contralateral vesicoureteral reflux leading to its injury. We grouped the children with unilateral renal agenesis, renal hypoplasia, multicystic dysplastic kidney into abnormal unilateral renal development and investigated whether it was accompanied with contralateral vesicoureteral reflux. We retrospectively reviewed 96 pediatric cases of unilateral renal agenesis, hypoplasia, multicystic dysplastic kidney diagnosed at Shinchon Severance Hospital, Yongdong Severance Hospital from 1987 to 1996 and Ajou University Hospital from 1994 to 1996. Diagnosis was based on radiological findings, renal hypoplasia being defined as small renal size with no apparent renal scarring and no irregularity of the calyceopelvic system on abdominal sonography or intravenous pyelography. Among the 96 cases,48 cases carried out voiding cystourethrography. 58 cases were male(60%) and 38 cases were female(40%). The cases of abnormal unilateral development on the left side were 45(47%) and that on the right side were 51(53%). Although there were diverse reasons leading to diagnosis, the major ones included were prenatal sonography, urinary tract infection, and other congenital anomalies. In cases of unilateral renal agenesis & hypoplasia the leading factors were urinary tract infection & other congenital anomalies and in cases of multicystic dysplastic kidney that was prenatal sonography. There was a chronological gap between the mean age of diagnosis(1.8 year) and voiding cystouerthrography(2.5 year, P < 0.01). 9 of the 18 unilateral renal agenesis cases, 5 of the 11 unilateral renal hypoplasia cases, and 3 of the 19 unilateral multicystic dysplastic kidney cases showed contralateral vesicoureteral reflux. Average reflux grade was above G III.Among the 17 children who had contralateral vesicoureteral reflux, 3 children had chronic renal failure and ureteroneocystostomy was carried out in 6 children. From the above results we conclude that screening voiding cystourethrography should be performed in children with abnormal unilateral renal development for early detection of vesicoureteral reflux in the contralateral kidney.

• Childhood Kidney Diseases
• /
• v.3 no.2
• /
• pp.170-179
• /
• 1999

Purpose: To evaluate whether or not sodium restriction had its own beneficial effect and increased the efficiency of the anti-hypertensive drugs on the progression of renal failure. Methods: We studied using the excision remnant kidney model. Treatment groups were as follows: 5/6 nephrectomy and a 0.49% (normal-high) sodium diet (NN); 5/6 nephrectomy and a 0.25% (normal-low) sodium diet (LN); 5/6 nephrectomy, a 0.49% sodium diet and enalapril (NNE); 5/6 nephrectomy, a 0.49% sodium diet and nicardipine (NNN); 5/6 nephrectomy, a 0.25% sodium diet and enalapril (LNE); 5/6 nephrectomy, a 0.25% sodium diet and nicardipine (LNN). Both diets were isocaloric and had the same content of protein, phosphorus and calcium. Proteinuria, remnant kidney weight, mesangial expansion scores, and glomerular volume were assessed. Results: Blood pressure tended to be lower in LN compared to NN (P<0.05). NN developed progressive hypertension. LNE, LU, NNE, and NNN reduced blood pressure. LNE, LNN, NNE, NNN, and LN had significantly less proteinuria than NN at 16 weeks (P<0.05). At 24 weeks, LN developed proteinuria (82 mg/day), which were lessened in LNE (54 mg/day) and not lessened in LNN (76 mg/day). Mesangial expansion scores were significantly less in LN rats compared to those in NN rats. Glomerular volumes at 24 weeks in LN rats were significantly less compared to those at 16 weeks in NN rats. Mesangial expansion scores and glomerular volumes at 4, weeks, 12 weeks, and 24 weeks were not different among LN, LNE, and LNN groups. Conclusion: Dietary salt restriction lessens renal damage, at least in part, by inhibiting compensatory renal growth and reducing blood pressure. Enalapril was particularly successful in reducing proteinuria and glomerular injury when combined with dietary salt restriction.

• Journal of Cardiovascular Imaging
• /
• v.26 no.4
• /
• pp.217-225
• /
• 2018

BACKGROUND: In patients with acute heart failure (AHF), diastolic dysfunction, especially pseudonormal (PN) or restrictive filling pattern (RFP) of left ventricle (LV), is considered to be implicated in a poor prognosis. However, prognostic significance of diastolic dysfunction in patients with ischemic heart disease (IHD) has been rarely investigated in Korea. METHODS: We enrolled 138 patients with IHD presenting as AHF and sinus rhythm during echocardiographic study. Diastolic dysfunction of LV was graded as ${\geq}2$ (group 1) or 1 (group 2) according to usual algorithm using E/A ratio and deceleration time of mitral inflow, E'/A' ratio of tissue Doppler echocardiography and left atrial size. RESULTS: Patients in group 1 showed higher 2-year mortality rate ($36.2%{\pm}6.7%$) than those in group 2 ($13.6%{\pm}4.5%$; p = 0.008). Two-year mortality rate of patient with LV ejection fraction (LVEF) < 40% ($26.8%{\pm}6.0%$) was not different from those with LVEF 40%-49% ($28.0%{\pm}8.0%$) or ${\geq}50%$ ($13.7%{\pm}7.4%$; p = 0.442). On univariate analysis, PN or RFP of LV, higher stage of chronic kidney disease (CKD) and higher New York Heart Association (NYHA) functional class were poor prognostic factors, but LVEF or older age ${\geq}75$ years did not predict 2-year mortality. On multivariate analysis, PN or RFP of LV (hazard ratio [HR], 2.52; 95% confidence interval [CI], 1.09-5.84; p = 0.031), higher stage of CKD (HR, 1.57; 95% CI, 1.14-2.17; p = 0.006) and higher NYHA functional class (HR, 1.81; 95% CI, 1.11-2.94; p = 0.017) were still significant prognostic factors for 2-year mortality. CONCLUSIONS: PN or RFP of LV was a more useful prognostic factor for long-term mortality than LVEF in patients with IHD presenting as AHF.

• Childhood Kidney Diseases
• /
• v.4 no.2
• /
• pp.144-153
• /
• 2000

Purpose : Effects to predict tile progression of chronic renal failure (CRF) in children, using mathematical models based on transformations of serum creatinine (Scr) concentration, have failed. Error may be introduced by age-related variations in creatinine production rate. Height (Ht) is a reliable reference for creatinine production in children. Thus, Scr, factored for Ht, could provide a more accurate predictive model. We examined this hypothesis. Methods : The progression of of was detected in 63 children who proceeded to end-stage renal disease. Derivatives of Scr, including 1/Scr, log Scr & Ht/Scr, were defined fir the period Scr was between 2 and 5 mg/dl. Regression equation were used to predict the time, in months, to Scr > 10 mg/dl. The prediction error (PE) was defined as the predicted time minus actual time for each Scr transformation. Result : The PE for Ht/Scr was lower than the PE for either 1/Scr or log Scr (median: -0.01, -2.0 & +10.6 mos respectively; P<0.0001). For children with congenital renal diseases, the PE for Ht/Scr was also lower than for the other two transformations (median: -1.2, -3.2 & +8.2 mos respectively; P<0.0001). However, the PEs for children with glomerular diseases was not as clearly different (median: +0.9, +0.5 & +9.9 respectively). In children < 13 yrs, PE for Ht/Scr was tile lowest, while in older children, 1/Scr provided the lowest PE but not significantly different from that for Ht/Scr. The logarithmic transformation tended to predict a slower progression of CRF than actually occurred. Conclusion : Scr, floored for Ht, appears to be a useful model to predict the rate of progression of CRF, particularly in the prepubertal child with congenital renal disease.

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