• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.206-209
• /
• 2001

Clq nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial Clq deposits in immunofluorescence microscopy and electron dense deposits on electron microscopy. It was described as a distinct disease entity in 1985 by Jennette and Hipp. Thirty four cases were reported in the literature but there has been no pediatric case reported in Korea yet. It commonly presents with steroid- resistent nephrotic syndrome in older children and young adults, and occasionally nephritic-nephrotic syndrome or rapidly progressive glomerulonephritis We report a case of Clq nephropathy in a 23-month-old girl with steroid-dependent nephrotic syndrome. (J. Korean Soc Pediatr Nephrol 2001;5 : 206-9)

• Childhood Kidney Diseases
• /
• v.26 no.2
• /
• pp.101-106
• /
• 2022

Nephrotic syndrome (NS) is a hypercoagulable state in which children are at risk of venous thromboembolism. A higher risk has been reported in children with steroid-resistant NS than in those with steroid-sensitive NS. The mortality rate of cerebral venous sinus thrombosis (CVST) is approximately 10% and generally results from cerebral herniation in the acute phase and an underlying disorder in the chronic phase. Our patient initially manifested as a child with massive proteinuria and generalized edema. He was treated with albumin replacement and diuretics, angiotensin-converting enzyme inhibitor, and deflazacort. Non-contrast computed tomography showed areas of hyperattenuation in the superior sagittal sinus when he complained of severe headache and vomiting. Subsequent magnetic resonance imaging revealed empty delta signs in the superior sagittal, lateral transverse, and sigmoid sinuses, suggesting acute CVST. Immediate anticoagulation therapy was started with unfractionated heparin, antithrombin III replacement, and continuous antiproteinuric treatment. The current report describes a life-threatening CVST in a child with steroid-dependent NS, initially diagnosed by contrast non-enhanced computed tomography and subsequently confirmed by contrast-enhanced magnetic resonance imaging, followed by magnetic resonance venography for recanalization, addressing successful treatment.

• Clinical and Experimental Pediatrics
• /
• v.58 no.8
• /
• pp.275-282
• /
• 2015

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to be as effective, and short-term daily low-dose steroids during upper respiratory infections reduced relapses. Steroid resistance or congenital NS are indications for genetic study and renal biopsy, since the list of genes involved in NS is lengthening.

• Childhood Kidney Diseases
• /
• v.19 no.2
• /
• pp.105-111
• /
• 2015

Purpose: Steroid dependent nephrotic syndrome (SDNS) is a chronic illness in childhood hard to treat. Steroid sparing drugs are often used, because long-term steroid therapy can cause severe side effects. We studied to compare efficacy between MMF and other drugs including cyclosporine and levamisole. Methods: This study was performed retrospectively on patients with SDNS, who were treated at Pusan National University Children's hospital. MMF group included 11 patients who were treated with MMF for at least six months between June 2012 and July 2014. As control groups, cyclosporine group (n=15) and levamisole group (n=18) included patients treated between January 2008 and July 2014. Number of relapse was analyzed in patients treated more than six months, and relapse free for one year was analyzed in patients treated more than one year. Results: In MMF group, ten were boys and mean age at onset was 5.8 years. Mean age at starting of MMF was 8.6 years. Number of relapse in MMF group was reduced significantly after treatment from 3.4 /year to 0.2 /year (P=0.003). There was no significant difference in number of relapse among groups (MMF: 0.2 /year, cyclosporine: 0.5 /year, levamisole: 0.5 /year). Comparing the early relapse within six months after treatment levamisole group was significantly higher than the other two groups (P=0.04). Conclusions: MMF which is used in SDNS significantly reduced the relapse and side effects were rare. In addition, MMF did not show any significant difference in comparison with the other two groups in number of relapse and relapse free for one year.

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

• Childhood Kidney Diseases
• /
• v.20 no.2
• /
• pp.45-49
• /
• 2016

Purpose: This study was performed to evaluate the effects of cyclosporine-A (CsA) on linear growth in pediatric patients with steroid-dependent (SDNS) or resistant nephrotic syndrome (SRNS). Methods: Thirty-five pediatric patients with SDNS or SRNS undergoing glucocorticoid (GC) and/or CsA treatment were retrospectively reviewed. Seventeen patients were treated with GC alone and 18 were treated with GC and CsA. The cumulative doses of GC and CsA were quantified (mg/kg/day). Linear growth during the follow-up period was defined as the difference in Z-score between the initial and final height according to the follow-up period (${\Delta}$ height Z score/year). The associations between linear growth and clinical parameters were analyzed. Results: The linear growth of patients in the two groups was not significantly different (P=0.262). The ${\Delta}$ height Z score/year did not show a significant correlation with the cumulative doses of CsA, but was negatively correlated with the cumulative dose of GC and positively correlated with the Z score for height at the time of diagnosis. Conclusion: In children with SDNS or SRNS undergoing GC therapy, added CsA treatment may not have harmful effects on linear growth.

• Clinical and Experimental Pediatrics
• /
• v.59 no.sup1
• /
• pp.72-75
• /
• 2016

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age. Tests ruled out allergic and parasitic disease etiologies. Gastroduodenoscopy with biopsy revealed marked eosinophilic infiltration in the duodenum. Renal biopsy findings indicated minimal change disease spectrum without eosinophilic infiltration. The oral deflazacort dosage was increased, and the patient was discharged after abdominal pain resolved. To our knowledge, this is the first report of eosinophilic gastroenteritis in a patient with minimal change disease.

• Childhood Kidney Diseases
• /
• v.22 no.1
• /
• pp.1-6
• /
• 2018

Rituximab (RTX) is a chimeric monoclonal antibody that inhibits CD20-mediated B-cell proliferation and differentiation. Several studies have examined its use in intractable nephrotic syndrome (NS) with some positive results. However, those studies examined such effects for a short-term period of 1 year, and some patients continued to relapse after a lapse in RTX treatment. Our use of RTX as a maintenance therapy (RTX injection when the CD19 cell count exceeded $100-200/{\mu}L$ before relapse) showed some noticeable efficacy. We used RTX in 19 patients with steroid-dependent NS (SDNS). In 12 patients treated with RTX maintenance therapy, only one relapse occurred. The mean treatment period was $23.4{\pm}12.7months$, and the mean number of RTX administrations was $3.9{\pm}1.6$. The relapse rates were decreased (from 2.68/year to 0.04/year), and the drug-free period also increased (from 22.5 days/year to 357.1 days/year) during maintenance therapy. The other seven patients were treated with one cycle of RTX or additional cycles in case of relapse (non-maintenance therapy). Relapse rates were significantly decreased after RTX treatment (from 1.76/year to 0.96/year, P=0.017). The relapse-free period was $15.55{\pm}7.38$ (range, 5.3-30.7) months. No severe side effects of RTX were found except for a hypersensitivity reaction such as fever and chills during its infusion. In conclusion, RTX is considered an effective and safe option to reduce the relapse rate by a single- or maintenance-interval therapy in SDNS.

• Kidney Research and Clinical Practice
• /
• v.36 no.3
• /
• pp.257-263
• /
• 2017

Background Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Methods Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. Results The patients were followed for $4.7{\pm}1.9years$ and received $5.2{\pm}2.3cycles$ of RTX over $2.8{\pm}1.1years$. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from $3.4{\pm}2.0per$ year initially to $0.4{\pm}0.8per$ year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from $2.8{\pm}1.5per$ year before RTX treatment to $1.3{\pm}0.8per$ year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. Conclusion This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

• Childhood Kidney Diseases
• /
• v.3 no.1
• /
• pp.11-19
• /
• 1999

Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. MCNS most commonly appears between the ages of 2 and 10 yr. But the incidence and prognosis in adolescent MCNS are different from those found in young children; the prognosis and the response to therapy is unfavorable with increasing ages. So we compared the prevalence and the clinical manifestations of adolescent MCNS with that of childhood MCNS for management of adolescent MCNS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of the 216 cases with MCNS which were divided into children group and adolescent group by their age of onset; under 12 years(childhood) and between 12-18 years(adolescent). Results: 1) The number of childhood idiopathic nephrotic syndrome was 245 cases, and that of adolescent idiopathic nephrotic syndrome was 55 cases. 188 cases($77\%$) showed MCNS, 30 cases($12\%$) FSGS, 4 cases($1.6\%$) MSPCN in childhood idiopathic nephrotic syndrome; 28 cases($51\%$) showed MCNS, 12 cases($22\%$) FSGS in adolescent idiopathic nephrotic syndrome. 2) The mean onset age was $7.53{\pm}5.5$ years, and the male to female ratio was 3.8:1 in childhood onset and 2.5:1 in adolescent onset with male predominance. 3) Hematuria was associated with $17\%$ of childhood onset and $39.3\%$ of adolescent onset disease(P=0.005). Hypertension appeared in $0.5\%\;and\;7\%$ in each group without significant difference between the groups. 4) 24 hour urine protein, SPI, albumin, BUN, cholesterol level showed no significant difference. 5) The response of childhood onset and adolescent onset MCNS to steroid therapy showed complete remission in $11.7\%\;&\;14.7\%$, infrequent relapsing in $29.2\%\;&\;28.5\%$, frequent relapsing in $23.9\%\;&\;14.7\%$, steroid dependent in $21.8\%\;&\;28.6\%$ each. Steroid resistant showed $13.3\%\;&\;14.7\%$ with no significance. 6) Immunosuppresant therapy was performed $57\%$ in childhood onset and $65\%$ in adolescent onset. 7) Mean number of relapse and duration from onset to first relapse showed no significance between two groups. Conclusion : Our results indicate that the incidence of hematuria, the rate of steroid dependent and frequent relapsing, and the recurrence rate were higher in adolescent MCNS; showed poorer steroid responsiveness and prognosis. Our data also point to the need for a more aggressive therapy to treat and make recommendations for the adolescent population as a whole.