• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.67-72
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• 2003

Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.23-28
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• 2016

Purpose: We investigated whether serum levels of insulin growth factor-1 (IGF-1) and insulin growth factor binding protein-3 (IGFBP-3) are valuable in predicting clinical outcomes or are correlated with other laboratory findings in children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: We examined 27 children who were consecutively admitted to our hospital with HSP between January 2011 and February 2012. Blood tests (C-reactive protein, white blood cell count, platelet count, erythrocyte sedimentation rate, albumin, immunoglobulin A, complement C3, antineutrophil cytoplasmic antibody, IGF-1, IGFBP-3) and urine tests were performed upon admission. IGF-1 and IGFBP-3 were resampled in the recovery phase. Controls included 473 children whose IGF-1 and IGFBP-3 were sampled for evaluating their growth, at the outpatient department of pediatric endocrinology in our hospital. IGF-1 and IGFBP-3 were compared between the HSP children and controls, and between the acute and recovery phases in HSP children. The ability of these values to predict clinical outcomes including renal involvement was analyzed using bivariate logistic regression analysis (BLRA). Results: IGF-1 and IGFBP-3 were not different between the HSP children and controls ($148.7{\pm}117.6$ vs. $69.2{\pm}96.9$, P=0.290: $3465.9{\pm}1290.9$ vs. $3597.2{\pm}1,127.6$, P=0.560, respectively). There was no significant difference in IGF-1 or IGFBP-3 between acute and recovery phases. Based on the BLRA, no variable, including IGF-1 and IGFBP-3, could predict clinical outcomes including the presence of nephritis Conclusion: We concluded that IGF-1 and IGFBP-3 do not predict clinical outcomes of HSP, including renal involvement, in this study.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.171-175
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• 2015

Nephrotic syndrome (NS) is a common chronic disease in children; in 90 percent of cases, the condition is primary (idiopathic). Toxic nephropathy can be induced by herbal medicines, and is mainly manifested as tubulointerstitial nephritis and rarely, as glomerulopathy. Here in, we describe two cases of steroid-sensitive NS, which developed after the patients received herbal medicines. A 5-year-old boy and an 8-year-old girl were separately admitted within a short time period with acute onset of generalized edema, proteinuria, hypoalbuminemia, and hypercholesterolemia. Each patient had previously taken herbal medicine, which had been prescribed by different oriental medical clinics for different conditions. The patients were diagnosed with herbal medicine-induced NS and were treated empirically by a standard steroid therapy, with subsequent resolution of their NS. One patient relapsed, but her NS again responded to steroid therapy. We described two unusual cases of prototypical pediatric, steroid-sensitive NS, which was presumed to be minimal-change disease that developed after the administration of herbal medicines. We also reviewed the literature.

• The Journal of Korean Medicine
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• v.16 no.1 s.29
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• pp.51-70
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• 1995

To find the cause, pathology, oriental presciptions for SLE(systemic lupus erythematous) in the field of oriental medicine, we studied the clinical reports. The results were obtained as follows: 1. SLE can' be thought to be a category of chronic fatigue, numbness, yang poison' & erythema. 2. The chief cause of SLE could be a febrile poison and the lack of physiological fluid and the mechanism was that febrile poison attacted area of circulation and blood and injuried the physiological fluid to arise edema and clotted blood. 3. The therapy of SLE was chiefly clarifying heat and toxiciding in acute stage while reinforcing kidney and liver and supplementing Yin & clarifying in chronic stage, sometimes supplementing Qi, eliminating clotted blood, excuding wind and draining water could be applied. 4. The therapy for SLE nephritis was shown to be increasing yin and clarifying heat with toxiciding, reinforcing spleen and kidney, draining water, supplementing yin & Qi etc. The chief prescriptions were Seogakjihwangtang(犀角地黃湯), Wookakjihwangtang(牛角地黃湯), yeechihwan(二至丸), Daepowonjeon(大補元煎), Daepoeumhwan(大補陰煎), Kikukjihwangtang(杞菊地黃湯), Yookmijihwangtang(六味地黃湯) which indicates Rhemanniae radix added prescrition could be used chiefly for the treatment of SLE.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.30-37
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. Methods : The clinical features, initial laboratory and pathologic findings, and clinical out-come were evaluated in 60 children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. Results : The ratio of male to female patients was 1.2:1. The interval between the onset of $Henoch-Sch\"{o}nlein$ purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome In 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. Conclusion : Renal manifestation and pathologic findings correlated with the clinical out-come. It is necessary to evaluate the correlation between pathologic findings and treatment.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.170-177
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• 2008

Purpose : The aim of this study was to evaluate whether the incidence of relapse or nephritis might be influenced by the duration of corticosteroid therapy in children with Henoch-Schonlein purpura(HSP). Methods : We retrospectively analyzed 186 children with a diagnosis of HSP in two major hospitals in Ilsan, Korea from the years 2000 to 2003. To evaluate whether renal involvement or relapse might be influenced by the duration of corticosteroid therapy in children with HSP, one pediatric nephrologist from hospital A, maintained corticosteroid therapy for at least 2 weeks(Group A, n=94). The other from hospital B used only during the symptomatic period(Group B, n=92). Results : There were no significant differences in age, sex, body weight, white blood cell count, hemoglobin, hematocrit, platelet count, serum protein and albumin levels between the two groups. The incidence of abdominal pain or arthralgia also did not differ between two groups. However, the duration of steroid therapy was significantly longer in Group A than in Group B and the cumulative dose of prednisolone was also higher in Group A than in Group B. The development of nephritis was more frequent in Group A. Conclusion : The longer duration of steroid use was not associated with the decreased rate of nephritis. Therefore, corticosteroids should be used carefully in a selected group of HSP children, and be tapered rapidly after control of the acute symptoms.

• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.126-130
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• 2004

Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

• The Korean Journal of Nuclear Medicine
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• v.19 no.1
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• pp.127-136
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• 1985

To evaluate change of serum $beta_2-microglobulin$ concentration$(s\beta_2-MG)$ and the usefulness of $s\beta_2-MG$ and $s\beta_2-MG/serum$ creatinine concentration(sCr) ratio in various renal diseases, $s\beta_2-MG$ and sCr were measured in 25 normal controls and 90 patients of various renal diseases(16 cases of glomerulonephritis, 12 cases of acute renal failure, 8 cases of chronic renal failure, 24 cases of nephrotic syndrome, 15 cases of tubulointerstitial diseases and 15 cases of lupus nephritis) using $Phadebas^\circledR$ $Beta_2-Micro$ Test kits. The results were as follows; 1) In normal control, the mean value of $s\beta_2-MG$ was $1.65{\pm}0.41mg/l$ and the mean value of $s\beta_2-MG/sCr$ ratio was $0.14{\pm}0.05$. 2) In various renal diseases, the mean value of $s\beta_2-MG$ was $6.74{\pm}5.47mg/l$. The mean value of $s\beta_2-MG/sCr$ ratio was $0.24{\pm}0.11$ and significantly elevated than that of normal control. (p<0.05) 3) The correlation between $s\beta_2-MG$ and sCr in glomerular and tubulointerstitial disease was log $s\beta_2-MG-0.90$ log sCr-0.48 and its correlation coefficient was 0.78(p<0.05). 4) In glomerular disease, the correlation between $s\beta_2-MG$ and sCr was log $s\beta_2-MG-0.89$ log sCr-0.46(r - 0.76) and in tubulointerstitial disease, it was log, $s\beta2-MG-0.95$ log sCr-0.59 (r-0.87). There was no significant difference between the two groups(p<0.05). 5) Among 32 cases of glomerular and tubulointerstitial disease patients, whose sCr was within normal range, 17 cases showed elevated $s\beta_2-MG$. The mean values of $s\beta_2-MG/sCr$ ratio in these patients was $0.30{\pm}0.14$ and significantly elevated than that of normal control(p<0.05). 6) In 15 cases of lupus nephritis, 12 cases showed elevated $s\beta_2-MG$ with normal sCr and 12 cases showed elevated $s\beta_2-MG/sCr$ ratio. With above results, it was found that the $s\beta_2MG$ can be used as an index of glomerular filtration rate as in the case of sCr and that $s\beta_2-MG/sCr$ ratio can be used as a tool in early detection of slightly decreased glomerular filtration rate and in detection of the renal disease of increased $\beta_2-MG$ production.

• 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.