• Title/Summary/Keyword: nephrotic syndrome

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A Study Burden, Social Support and Quality of Life in Mothers of a Child with Nephrotic Syndrome (신증후군 환아 어머니의 부담감, 사회적 지지 및 삶의 질)

  • 성미혜
    • Journal of Korean Academy of Nursing
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    • v.30 no.3
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    • pp.670-681
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    • 2000
  • The purpose of this study was to identity the level of burden, social support and quality of life of the subjects. The subjects of this study were 68 mothers of nephrotic syndrome patients whose children were hospitalized in one pediatric ward of the University Hospital in Seoul. The data was collected using questionnaires, and the period of the data collection was from Nov. 15 to Dec. 31, 1999. The instruments used for this study were the Burden Measurement Instrument developed by Montgomery et. al(1985), social support measurement instrument designed Brandt an Weinert(1978) and Quality of life scale designed by Ro,Yoo JA(1988). The data analysis was done by SPSS, t-test, ANOVA and the Pearson correlation coefficient. The results of were as follows. 1. The level of burden showed a mean score of 54.47, the level of social support, a mean score of 86.00 and the level quality of life, a mean score of 140.20. 2. The level of burden differed according to mother's religion, patient's purpose for admission and perceived patient's condition by mothers. 3. The level of social support and the level of quality of life differed according to perceived patient's condition by mothers. 4. There was a negative correlation between burden and social support(r=-.348, p<.001). Also, burden was negatively related with quality of life(r=-3.97, p<.001). Social support was positively related with quality of life(r=.064, p<.001).

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Treatment of Henoch-Sch$\ddot{o}$nlein Purpura with Intravenous Immunoglobulin

  • Chung, Hyo-Seok;Kim, Won-Duck;Lee, Eun-Sil;Choi, Kwang-Hae;Park, Yong-Hoon;Kim, Yong-Jin
    • Journal of Yeungnam Medical Science
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    • v.18 no.2
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    • pp.246-252
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    • 2001
  • We report the result of a high-dose intravenous immunoglobulin therapy in a Henoch-Schnlein purpura patient with severe abdominal pain and nephrotic syndrome who did not respond to methylprednisolone pulse therapy. Kidney biopsy showed diffuse mesangial cell proliferative glomerulonephritis with fibrocellular crescent formation in approximately 50% of glomeruli. Mesangium of all glomeruli were strong positive for IgA and C3 antibodies. High-dose intravenous immunoglobulin treatment was introduced and dramatic improvement of gastrointestinal symptom and proteinuria as well as hematuria was noted. Immunoglobulin administration should be considered in Henoch-Schnlein purpura patients with steroid-resistant intractable gastrointestinal manifestation and renal involvement.

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Primary Pneumococcal Peritonitis in a Healthy Child (건강한 소아에서 발생한 원발성 폐렴구균성 복막염 1례)

  • Yang, Jeong-Soo;Lee, Min-Hae;Choi, Myoung-Bum;Park, Chan-Hoo;Woo, Hyang-Ok;Youn, Hee-Shang
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.5 no.1
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    • pp.83-87
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    • 2002
  • Primary peritonitis usually refers to a bacterial infection of the peritoneal cavity without a demonstrable intra-abdominal source. Most cases occur in children with ascites resulting from nephrotic syndrome or cirrhosis. Rarely, it may occur in previously healthy children less than 7years of age, usually a girl. Distinguishing primary peritonitis from appendicitis may be impossible in patients without a history of nephrotic syndrome or cirrhosis. Accordingly, the diagnosis of primary peritonitis is made only at laparotomy. We report one case of primary pneumococcal peritonitis in a 27-month-old female who underwent explorative laparotomy to discover the cause of suspicious intestinal perforation and mechanical ileus. Later, pneumococci were cultured in blood and gram-positive diplococci were isolated from the pus of peritoneal cavity.

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Acute aortoiliac thrombosis in minimal change disease

  • Soyoung Lee;Hwarim Kang;Jongho Shin;Kyeong Min Kim
    • Journal of Medicine and Life Science
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    • v.19 no.3
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    • pp.125-129
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    • 2022
  • Patients with nephrotic syndrome (NS) are generally known to be at greater risk for thrombosis, with arterial thrombosis-related complications being relatively rare compared to venous thrombosis-related complications. This report describes a 46-year-old male with historically proven minimal change disease (MCD) complicated by acute aortoiliac thrombosis. He had been diagnosed with MCD 8 months previously and was treated successfully with steroids. He was prescribed a second course of high-dose steroids (prednisolone 1 mg/kg/day) due to a relapse of MCD at the outpatient clinic 8 days before the emergency department visit. The patient presented with severe pain in both lower limbs and was diagnosed with aortoiliac thrombosis that developed during high-dose steroid treatment. He subsequently underwent surgical thromboembolectomy. Hypoalbuminemia has the strongest association with the risk of thromboembolism. According to international clinical practice guidelines, anticoagulant therapy is recommended when serum albumin is ≤2-2.5 g/dL. However, as serum albumin levels may be relatively high in the early phase of NS, as in this case report, an individualized anticoagulation strategy for each patient should be considered, regardless of serum albumin levels.

The Levels of IgG Subclasses and Vaccine-induced Viral Antibodies in Childhood Minimal Change Nephrotic Syndrome (소아 신증후군에서 IgG 아군 및 바이러스 항체 값)

  • Song Yong-Hoon;Lee Kyung-Yil;Kim Dong-Un;Koh Dae-Kyun;Lee Byung-Churl
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.143-148
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    • 2005
  • Purpose : Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). Methods : Using the stored sera, the levels of immunoglobulin(IgC, IgM, IgA, and IgC) and IgG subclasses(IgG 1, 2, ,3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. Results : The mean values of IgG and IgG1 were $390{\pm}187\;mg/dL$ and $287{\pm}120\;mg/dL$ in nephrotic children, and $1,025{\pm}284\;mg/dL$ and $785{\pm}19\;mg/dL$ in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM($251{\pm}183\;mg/dL\;vs. 153{\pm}55\;mg/dL$, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9$\%$(9 of 21 cases) in the MCNS group and 52$\%$(13/25) in the control group, and that of anti-measles IgG was 75$\%$(16/21) and 92$\%$(23/25), respectively, but there was no statistical difference between the two groups. Conclusion : IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS. (J Korean Soc Pediatr Nephrol 2005;9:143-148)

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A case of Nutcracker Syndrome Associated with Proteinuria (단백뇨를 동반한 Nutcracker 증후군 1례)

  • Son Jin-Tae;Rho Kwang-Sik;Kim Pyung-Kil;Kim Mung-Jun
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.166-169
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    • 1997
  • The nutcracker syndorme refers to compression of the left renal vein between the aorta and the superior mesentric artery which results in renal vein, left gonadal vein varices, hematuria and left sided flank pain. We report this experience of 11yr-11mon of girl has typical Nutcracker syndrome with persistent proteinuria and without typical hematuria. According to the renal biopsy for persistent proteinuria, biopsy shows pathologic findings similar to minimal change nephrotic syndrome. All symtpoms relieved without any specific treatments but she had no response to steroid treatment for persistent proteinuria. Now she was followed up through OPD base without symptom and consideration of surgical intervention.

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Initial Prednisolone Treatment for Primary Nephrotic Syndrome in Children-4 Weeks versus 6 Weeks (일차성 신증후군 환아의 첫 관해를 위한 4주와 6주 스테로이드 치료 비교)

  • Choi Jung Youn;Park Mi Young;Kim Hye Suk;Lee Kyung Hoon;Kim Jun Sik;Park Yong Hoon
    • Childhood Kidney Diseases
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    • v.9 no.2
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    • pp.159-166
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    • 2005
  • Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

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Therapeutic Effect of Cyclosporine A on Severe Henoch-$Sch{\ddot{o}}nlein$ Purpura Nephritis (중증 Henoch-$Sch{\ddot{o}}nlein$ Purpura Nephritis 환아에서 Cyclosporine A의 치료효과)

  • Chin Hyun Jong;Kim Ji Hong;Kim Pyung Kil;Jeong Hyeon Joo
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.110-117
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    • 1998
  • Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

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Clinicopathological evaluation of Childhood Henoch-$Sch{\ddot{o}}nlein$ Purpura(HSP) Nephritis with Nephrotic Syndrome (신증후군을 동반한 Henoch-$Sch{\ddot{o}}nlein$ 신염 환아의 임상병리 적 고찰)

  • Kang Hyeon-Ho;Yoon Kyung-Leem;Cho Byoung-Soo
    • Childhood Kidney Diseases
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    • v.4 no.2
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    • pp.111-119
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    • 2000
  • Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

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Plasminogen Activator Inhibitor Type 1 Gene Polymorphism in Patients with Minimal Change Nephrotic Syndrome (소아 신증후군 환자에서 Plasminogen Activator Inhibitor Type 1 유전자 다형성)

  • Kim Young-Min;Hong Hyun-Kee;Kim Sung-Do;Cho Byoung-Soo
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.26-32
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    • 2004
  • Purpose : Hypercoagulability is present in patients with nephrotic syndrome. Plasminogen activator inhibitor type 1(PAI-1) is a major inhibitor of plasminogen activators. PAI-1 inactivates both tissue plasminogen activator(tPA) and urokinase plasminogen activator(uPA) by rapid formation of inactive 1:1 stoichiometric complexes. Recently some studies showed that the enhanced PAI-1 expression may be involved in the intraglomerular fibrinogen/fibrinrelated antigen deposition seen in nephrotic syndrome. Methods : PAI-1 gene promoter -844(G/A) polymorphism was evaluated in 146 children with minimal change nephrotic syndrome(MCNS) and 230 control subjects. The patients with MCNS were subdivided into 85 infrequent-relapser(IR) group and 61 frequent relapser(FR) group. PCR of PAI-1 gene promoter region including -844(G/A) and RFLP using the restriction enzyme Xhol were performed for each DNA samples extracted from the groups. Results : The distribution of PAI-1 genotype in the control group was G/G 81(32.5%), A/A 42(16.9%), and G/A 126(50.6%). The distribution of PAI-1 genotypes in the IR group of MCNS was G/G 29(34.1%), A/A 15(17.7%), and G/A 41(48.2%). The distribution of PAI-1 genotype in the FR group of MCNS was G/G 17(27.9%), A/A 18(29.5%), and G/A 26(42.6%). There was a significantly increased frequency of A/A genotype(P=0.0251) in the FR group of MCNS. Conclusion : Our results indicate that the PAI-1 gene promoter A/A genotype may be associated with the FR in MCNS.

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