• Childhood Kidney Diseases
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• v.4 no.1
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• pp.33-39
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• 2000

Purpose: Our study was designed to investigate the association of MHC Class II (DR, DQ) allele with IgA nephropathy and its significance as a prognostic factor for progression to ESRD Material and Methods: 69 children with IgA nephropathy with normal renal function(serum creatinine $\leq$ 1.5mg/dL) was classified as group A and 70 patients who received renal transplantation due to IgA nephropathy were selected as group B. The HLA-DQB1 and HLA-DRB1 alleles were studied by polymerase chain reaction using sequence specific primers. We have compared the difference in alleles between these two groups and with normal control and also examined any possible effect of the MHC class II genes on the histopathological severity and prognosis of IgAN. Results: Mean age was $8.8{\pm}2.9$ years in group A and $35.0{\pm}15.5$ years in group B. Male to female ratio was 2.8:1 in group A and 2.5:1 in group B. There was a significantly higher frequency of HLA-$DQB1^*03\;and\;DQB1^*05$ in Group B. The frequency of HLA-$DQB1^*0302\;and\;^*05031$ allele had increasing tendency in Group B(P<0.05). HLA-$DRB1^*03\;and\;^*05$ were more common in Group B(P<0.05). HLA-$DRB1^*04$ allele was the most common DR alleles in both group, but there was no statistical significance. There were no significant correlation with MHC class 13 genes on the hjstopathological severity in Group A. Conclusion: In conclusion, $HLA-DQB1^*0302\;and\;HLA-DQB1^*05031 $ allele seemed to be more common in transplanted patients compared to group with normal renal function suggesting that this allele is associated with poor prognosis in IgAN. However larger studies and follow up are required to confirm this due to uncharacterized heterogeneity in etiopathogenesis of IgA nephropathy and possibly one or more than one gene may exert influence in determining susceptibility to the diseases.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.17-24
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• 2000

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.77-83
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• 2000

Purpose: Pregnancy in transplanted mother is considered as a high-risk pregnancy, and significant incidences of prematurity and low-birthweight(LBW) infants have been reported. The objective of this study is to examine the outcome of pregnancy in transplanted mothers and to evaluate the current growth status in their children. Patients and Methods: We retrospectively reviewed 54 pregnancies in 40 kidney recipients until June 1999. Outcomes of pregnancy were reviewed and assessment of the current growth status in children was performed. Results: 54 pregnancies in 40 recipients were identified; 22 ended in termination of pregnancy because of unwanted pregnancy or therapeutic purposes. And of the other 32, 29 livebirths resulted in 28 recipients. The mean age of conception was $30.3{\pm}3.8$ years, with a mean interval from transplantation to conception of $35.9{\pm}23.2$ months. All patients were maintained on immunosuppressive regimens. Incidence of drug-treated hypertension(HTN) prior to pregancy was $52\%$, HTN during pregnancy, $48\%$; preeclampsia, $41\%$; urinary tract infection, $48\%$; oligohydramnios $4\%$; and no rejection during pregnancy and up to 3month post delivery. Of the 29 liveborn infants, prematurity(<37wk) occurred in $52\%$, LBW(<2500g) in $62\%$, VLBW(<1500g) in $7\%$ and $48\%$ born intrauterine growth retardation(IUGR). Mean gestational age was $36.3{\pm}3.0\;wk$; a mean birthweight, $2.23{\pm}0.6\;kg$; a mean birth-height, $45.1{\pm}3.6cm$. Current mean height standard deviation score (height SDS) was $0.29{\pm}0.91$ and mean weight SDS was $0.62{\pm}1.34$. Only one child($4\%$) under 1 year of age was below 10 percentile in height. Most of children had no medical problems except for 4 children; cleft palate(1), tuberous sclerosis(1), essential hematuria(1), and one child expired due to sepsis. Conclusion: This study showed similar incidence of premaure birth($57\%$) and low birth weight infants($62\%$), but lower incidence of spontaneous abortion($5.6\%$) was observed and compared to other studies. Postnatal growth in majority of children($96\%$) achieved catch-up growth before 1 year. Present study supports a more optimistic view of pregnancy in renal transplant mother and normal growth in their children.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 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
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• v.11 no.1
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• pp.41-50
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• 2007

Purpose : Dialysis in children with chronic renal failure presents with many difficulies. The purpose of this study is to find an improved method in chronic dialysis in infants and children less than 2 years of age by analyzing the experience with 10 cases. Methods : A retrospective review of the medical records of 10 patients(6 boys and 4 girls) was conducted. The patients had chronic renal failure and underwent chronic dialysis at Samsung medical center from March 1999 to February 2007. Results : At Initiation of dialysis, the median age was 3 months old(22 days-20 months), the median body weight was 3.75 kg(2.2-10.3 kg), and the median serum creatinine level was 4.3 mg/dL(2.0-11.4 mg/dL). The median duration of dialysis was 29.5 months(3-62 months). Dysplastic kidney disease was the most common underlying renal disease. Two patients were treated with hemodialysis, 4 patients with peritoneal dialysis, and 4 patients eventually switched dialysis modality. Nine of the 10 patients took erythropoietin and anti-hypertensive drugs. At the end of the follow up period, 1 patient received kidney transplantation, 2 patients died due to sepsis, and 5 patients were treated with peritoneal dialysis. Two patients were lost to follow up. The most common complication of dialysis was infection. Achieving vascular access and maintaining proper catheter function were the most important factors in treating patients with hemodialysis. The growth status of patients was aggravated after 6 month of dialysis but improved after 1 year of dialysis. Patients showed better growth on peritoneal dialysis than hemodialysis. Conclusion : Chronic dialysis can be performed successfully in infants and children under 2 years of age. Vascular access was the main limitation of hemodialysis, and infection was the common problem in both hemodialysis and peritoneal dialysis. To improve the patients survival rate and quality of life, major efforts should be directed toward the prevention of infection and preservation of catheter function. (J Korean Soc Pediatr Nephrol 2007;11:41-50)

• Journal of Embryo Transfer
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• v.2 no.1
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• pp.36-46
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• 1987

The present work was designed to understand the mechanism of superovuiation and the cause of early embryo loss and Implantation failure in the superovulating immature female rats which were elaborated by a pituitary gland transplantation. A pituitary gland obtained from the orchidectomized rats was transplanted under the right kidney capsule of 28 day old female rats (PGT group) on the starting day of experiment which was designated as Day 2. The grafted pituitary glands were removed at 6h (RPGT 6h group), 12h (RPGT 12hgroup) and 24h (RPGT 24h group) after the transplantation. Control rats were treated with 41U PMSG on Day 2 (PMSG group). The estrous cycle and the levels of plasma progesterone and estradiol-17$\beta$ were observed on Day 0, Day 5, respectively. The implantation sites, the weights of ovary and uterus, and the number of corpora lutea were examined in all group on Day 8. The resuft obtained were summarized as follows: 1. The percentages of the number of the rats in proestrus and estrus were 93.0%, 82.6%, 0%, 90.7% and 89.5% in PMSG, PGT, RPGT6h, RPGT12h and RPGT24h group, respectively. The synchronization of estrus cycle was dchieved in all groups. 2. The mating rates of each group were 80.2, 75.0, 0, 56.4, 57.8% in PMSG, PGT, RPGT6h, RPGT12h, RPGT24h group, respectively. 3. The numbers of copora lutea on Day 8 were 47.1 i 4.9, 18.1 $\pm$0.5, 14.1 i 0.3 and 8.9 $\pm$ 0.3 in PGT, RPGT24h, RPGTl2h and PMSG group, respectively. There were signIficantly difference between all groups (P<0.05). 4. The numbers of implantation sites (18.1 +- 4.0) in PGT group on Day 8 were higher than those of PMSG (8.5 $\pm$ 2.5), RPGT 12h (9.8 i 0.2) and RPGT 24h group (10.8 i 0.2) (P<0.05). 5. The ovarlan weights in PGT (95.2 $\pm$ 14.3mgIlOOg BW), RPGT 12h (51.7 $\pm$ 0.6mgIlOOg BW), and RPGT24h (57.9 $\pm$ 0.9mg/l00g 8W) groups were significantly higher than those of PMSG group (30.4 $\pm$7.4mg/l00g BW) (P<0.05). 6. The uterine weights in PMSG (672.4 $\pm$ 4.7mg/l00g 8W), and PGT (660.7 $\pm$7.8mg/l00g BW) groupswere greater than those of RPGT 12h (403.0 $\pm$ 1.lmg/lOOg BW) and RPGT 24h (490.1 $\pm$ 0.9mg/l00g BW) group (P<0.05). 7. The plasma progesterone levels in PGT groups (15 lng/ml) on Day 5 were higher than those of PMSG (83ng/ml), RPGT 12h (S7ng/ml), RPGT24h (8lng/ml) group (P<0.05). 8. The plasma estradiol-17$\beta$ levels in PMSG group (lBSpg/ml) on Day S were higher than those of RPGT 24h (l3pg/ml) group (P<0.05). But estradiol-17$\beta$ levels in PGT and RPGT 12h group were too low to discuss.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• The Journal of the Korea Contents Association
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• v.20 no.4
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• pp.464-474
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• 2020

This study provides basic data for nursing intervention to increase self management of hemodialysis patients by identifying the relation among their illness perception, physiological indicators, and self management. The participants were 134 patients receiving hemodialysis at a general hospital in Seoul. Data were collected by using a structured questionnaire and medical records. The collected data were analyzed by using descriptive statistics, independent t-test, one-way ANOVA, Pearson correlation coefficient and multiple regression analysis with the SPSS/WIN 23.0 program. The significant factors influencing self-management of hemodialysis patients have been identified with 8 variables. The first one is registration for kidney transplantation (β=-.20, p=.034). Among sub items of illness perception are consequence (β=-.20, p=.031), treatment control (β=.19, p=.040), and illness coherence (β=-.18, p=.049). In addition, among physiological indicators are hematocrit (β=.38, p<.001), hemoglobin (β=.29, p=.005), BUN (β=-.25, p=.010), and phosphorous (β=.22, p=.033). These variables explained 26.3% for self-management of hemodialysis patients. In order for hemodialysis patients to improve their self-management, a concrete nursing intervention improving the treatment control among illness perception as well as improving the understanding of physical indicators needs to be provided.

• Korean Journal of Microbiology
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• v.47 no.3
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• pp.249-254
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• 2011

Mycophenolic acid blocking the synthesis of xanthosine monophosphate is a nonnucleoside inhibitor of inosine monophosphate dehydrogenase. Therefore mycopholoic acid is a drug currently used as immunosuppressive agent in transplantation of heart, kidney and liver. Mycophenolic acid has been industrially produced through fermentation process by fungus Penicillium brevi-compactum. In this study, the profile of mycophenolic acid fermentation was observed in 5L-jar fermentor to investigate the utilization of carbon and nitrogen sources and the production of mycophenolic acid. It was investigated that what kind of carbon sources was better to cell growth and mycophenolic acid production. Fructose was the best carbon source for mycophenolic acid fermentation, but it is the most expensive one. Thereafter molasses containing sucrose as the supply source of fructose was confirmed to be the best carbon source for the industrial production. Use of molasses increased the fermentation yield of mycophenolic acid more than two times higher than glucose. It was confirmed that urea was the best inorganic nitrogen source, which did not give rise to sudden drop of culture pH. Addition of urea increased the fermentation yield of mycophenolic acid about 3.6 times higher than addition of ammonium nitrate as control. Casein, peptone and casamino acid originated from milk protein increased the fermentation yield of mycophenolic acid about 3.4 times higher than control. Peptone and casamino acid, which are casein hydrolysates, increased cell growth considerably as well.

• Journal of Clinical Nutrition
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• v.10 no.2
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• pp.45-50
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• 2018

Purpose: Intense multidisciplinary team effort is required for the intestinal rehabilitation of patients afflicted with the short bowel syndrome (SBS). These include enteral and parenteral nutrition (PN) support, monitoring of complications related to treatment, and considering further medical or surgical options for intestinal adaptation. Methods: In the Intestinal Rehabilitation Team (IRT) at the Samsung Medical Center, we have experienced 20 cases of adult SBS requiring multidisciplinary intestinal rehabilitation. This study is a retrospective review of the collected medical records. Results: Of the 20 subjects treated, 12 patients were male and 8 patients were female. At the time of referral to the IRT, the mean age was 51.5 years, and the mean body weight was 50.1 kg, which was 90% of the usual body weight. The diseases or operative managements preceding massive bowel resection were malignancy in 11 cases, cardiac surgery in 2 cases, trauma in 2 cases and one case, each of tuberculosis, corrosive esophagitis, atrial fibrillation, simultaneous pancreas and kidney transplantation, and perforated appendicitis. Of these, there were 14 survivals and 6 mortalities. The fatalities were attributed to progression of disease, intestinal failure-associated liver disease, and sepsis (unrelated to intestinal failure) (2 cases each). Among the 14 surviving patients, 8 patients have been weaned off PN, whereas 6 are still dependent on PN (mean PN dependence 36%). Conclusion: This paper reports the results of multidisciplinary intestinal rehabilitation of adult short bowel patients treated at the Samsung Medical Center. Further studies are required to improve survival and enteral tolerance of these patients.