• The Journal of Korean Medicine
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• v.21 no.1
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• pp.20-28
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• 2000

The purpose of this research is to investigate the synergistic effect of herb medicines with hydrocortisone and the regulation effect on the immune system of Onbitang and Dangguijakyaksan at the supernatant of PHA-P stimulated PBMC in the patients with minimal change nephrotic syndrome(MCNS). From the measurement of the concentration rate of IL-4, sCD23 and IL-13, the experiment yielded the following results : The Onbitang group showed a greater tendency to suppress IL-4 and IL-13 levels in MCNS group with no statistical significance. It showed very strong suppression in soluble CD23 compared with control group in MCNS group. The Dangguijakyaksan group, though not statistically significant, was inclined to suppress IL-4 level in MCNS group. It shows stronger suppression in sCD23 and IL-13 levels than these of control group in MCNS group. As for the synergistic effect, the group of hydrocortisone with herb medicines(Onbitang or Danguijakyaksan) produced more suppressive effect to IL-13 level in MCNS group than that of hydrocortisone-only group. They also tended to suppress sCD23 and IL-4 levels, though no statistical significance can be given. As to the suppressive effect of 1L-13 level, the group of Onbitang with hydrocortisone showed an increase of 22.6%, while the group of Dangguijakyaksan with hydrocortisone showed 14.7%. So Onbitang is more effective than Dangguijakyaksan. From the above results, a combinative treatment(herb medicines with hydrocortisone) can be an alternative method to substitute for steroid therapy. It can be a more effective therapy than steroid-only therapy because it is expected to reduce side effects and it shows more special effect to suppress IL-13 level. Based on the present results, further investigation concerning the serum IgE elevation is needed.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.1-6
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• 2019

Nephrotic syndrome (NS) is the most common glomerular disorder in childhood, and a vast majority of cases are idiopathic. The precise cause of this common childhood disease is not fully elucidated despite significant advancements in our understanding of podocyte biology. Idiopathic NS has been considered "a disorder of T-cell function" mediated by a circulating factor that alters podocyte function resulting in massive proteinuria since the last four decades. Several circulatory factors released from T-cells are considered to be involved in pathophysiology of NS; however, a single presumptive factor has not been defined yet. Extended evidence obtained by advances in the pathobiology of podocytes has implicated podocytes as critical regulator of glomerular protein filtration and podocytopathy. The candidate molecules as pathological mediators of steroid-dependent NS are CD80 (also known as B7-1), hemopexin, and angiopoietin-like 4. The "two-hit" hypothesis proposes that the expression of CD80 on podocytes and ineffective inhibition of podocyte CD80 due to regulatory T-cell dysfunction or impaired autoregulation by podocytes results in NS. Recent studies suggest that not only T cells but also other immune cells and podocytes are involved in the pathogenesis of MCNS.

• 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.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.25-28
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• 2016

Nephrotic syndrome is associated with a hypercoagulable state, which results in thromboembolism as one of its main complications. Various pathogenetic factors that cause the hypercoagulable state in nephrotic syndrome have been recognized. We report on a 19-year-old female with a minimal-change disease who developed pulmonary thromboembolism combined with intracardiac thrombus while on tapering steroid. Our patient showed hypoalbuminemia with an episode of shock, and was successfully treated with thrombolysis and anticoagulation therapy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.206-211
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• 2002

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.22-27
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• 2018

Purpose: Podocytes are important architectures that maintain the crucial roles of glomerular filtration barrier functions. Despite this structural importance, however, the mechanisms of the changes in podocytes that can be an important pathogenesis of minimal change nephrotic syndrome (MCNS) are not clear yet. The aim of this study was to investigate whether apoptosis is induced by interleukin (IL)-13 in cultured human podocytes. Methods: Human podocytes were treated with different IL-13 doses and apoptotic cells were analyzed using terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL assay) and fluorescence-activated cell sorting (FACS). Results: The IL-13 increased the number of TUNEL-positive cells in a dose-dependent manner at 6 and 18 hours (P<0.05 and P<0.05, respectively). The apoptosis rate was appeared to be increased slightly in the IL-13-stimulated podocytes (8.63%, 13.02%, and 14.46%; 3, 10 and 30 ng/mL, respectively) than in the control cells (7.66%) at 12 hours by FACS assay. Conclusion: Our study revealed that IL-13 expression may increase podocyte apoptosis. Blocking the IL-13 signal pathway can potentially play an important role in regulating the apoptosis of podocytes.

• 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.

• 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.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.16-22
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• 2003

Purpose : Minimal Change Disease(MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis factor-${\alpha}$ ($TNF-{\alpha}$) are involved in the pathogenesis of MCD. This study was done to see the changes of plasma and urinary $TNF-{\alpha}$, and their effects on the permeability of glomerular basement membrane. Methods : Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary $TNF-{\alpha}$ were measured. Employing the Millicell system, $TNF-{\alpha}$ were screened for the permeability factors. Results : Urinary $TNF-{\alpha}$ during relapse was significantly increased(P<0.01). No significant change was seen in the plasma $TNF-{\alpha}$ during relapse when compared to those in remission and the healthy controls. Furthermore, in the in vitro Millicell system, $TNF-{\alpha}$ did not produce a significant change in albumin permeability. Conclusion : Therefore, it seems that $TNF-{\alpha}$ may not play a disease-specific role in the pathogenesis of MCD.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.16-23
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• 2007

Purpose : Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated the relationship between the serum IgG and at bumin levels for children with minimal change nephrotic syndrome(MCNS). Methods : The levels of immunoglobulin G(IgG), albumin and total cholesterol of a total of 46 children with MCNS(proteinuria $>40mg/m^2/h$, and serum albumin level <2.5g/dL were analyzed. Results : The mean values of albumin, IgG and total cholesterol in MCNS children were $1.7{\pm}0.3g/dL,\;368{\pm}143mg/dL\;and\;431{\pm}78mg/dL$, respectively. There was an inverse correlation between the albumin values and the total cholesterol values(r=0.68, P=0.0001), whereas there was a direct-proportional correlation between albumin values and the IgG values(r=0.4, P=0.01). Conclusion : The IgG level is associated with albumin level, and it may reflect the severity of urinary protein loss in MCNS. Further studies are needed to evaluate this phenomenon.