• Childhood Kidney Diseases
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• v.5 no.1
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• pp.59-63
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• 2001

There are a considerable number of reports suggesting a common pathogenesis of IgA nephritis(IgANn) Henoch-$Sch{\ddot{o}}nlein$ Purpura(HSP). In previous reports, a patient develops IgAN after kidney transplantation for HSP nephritis, one of Identical twin boys, developed IgAN and the other HSP, and a boy with IgAN later developed HSP. We report two cases, one with IgAN who later developed HSP and the other with HSP who later developed IgAN, suggesting that IgAN and HSP have a common pathogenesis. (J. Korean Soc Pediatr Nephrol 5 : 59- 63, 2001)

• Clinical and Experimental Pediatrics
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• v.58 no.10
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• pp.402-405
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• 2015

IgA nephropathy usually presents as asymptomatic microscopic hematuria or proteinuria or episodic gross hematuria after upper respiratory infection. It is an uncommon cause of end-stage renal failure in childhood. Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression. Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process. We present the case of a 14-year-old patient with IgA nephropathy who had already progressed to end-stage renal failure in spite of immunosuppression and presented with pulmonary hemorrhage during oral prednisone treatment. His lung disease was comparable to diffuse alveolar hemorrhage and was successfully treated with plasmapheresis followed by oral prednisone. This case suggests that pulmonary hemorrhage may develop independently of renal manifestation, and that plasmapheresis should be considered as adjunctive therapy to immunosuppressive medication for treating IgA nephropathy with pulmonary hemorrhage.

• The Journal of Pediatrics of Korean Medicine
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• v.36 no.3
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• pp.35-48
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• 2022

Objectives The purpose of this study is to review relevant clinical studies to investigate the effectiveness of herbal medicines for IgA (Immunoglobulin A) nephropathy in children. Methods Studies on herbal treatment for IgA nephropathy were searched and analyzed through electronic databases such as PubMed, EMBASE, China National Knowledge Infrastructure, Wanfang, CiNii, J-STAGE, Oriental Medicine Advanced Searching Integrated System and Science ON. Studies included Randomized controlled trials and case series. Results We selected nine studies and analyzed the findings. In most studies, improvement of IgA nephropathy was observed after treatment, and was evaluated using factors as 24 hour urine protein, hematuria, Scr (serum creatinine) and BUN (blood urea nitrogen). The most commonly used herbs for IgA nephropathy were Poria cocos (茯苓), Astragalus membranaceus (黃芪), and Rehmannia glutinosa (生地黃). Conclusions Based on the results of the clinical studies, we identified that herbal medicine is an effective treatment for IgA nephropathy. However, additional systematic clinical studies are necessary to prove its effect and safety.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.153-160
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• 2009

Purpose : The intestinal mucosal defect has been known as one of the pathogenicmechanisms of IgA nephropathy. Oral antigens usually induce the activation of Th2 cells and mast cells. These cells secrete cytokines IL-4, IL-5 and TGF-$\beta$, which increase IgA production. Although ketotifen (benzocycloheptathiophene) is an H1 antagonist and a mast cell membrane stabilizer, it could protect the gastrointestinal membrane through inhibiting the production of IL-4, IL-5, PGE2, and LTB4, and decreasing the activity of nitric oxide synthease. Therefore, we have investigated if ketotifen may protect the development of IgA nephropathy with an oral antigen. Methods : ICR mice were used as an animal model orally with Poliovax only [ketotifen (-)], the other group was given oral ketotifen [ketotifen (+)] in addition to Poliovax. Results : Mesangial IgA deposition developed in 11 out of the 18 mice in the ketotifen (-) group, while in three out of the nine mice in ketotifen (+) group. The mesangial change developed in 16 out of the 18 mice in the ketotifen (-) group, while in five out of the nine mice in the ketotifen (+) group. Serum IL-4 and IL-5 levels were not significantly lower in the latter group than in the former. Conclusion : According to the statistical results from the above, ketotifen therapy would be beneficial to reducing mesangial changes in IgA nephropathy.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.133-138
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• 2023

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

• Journal of Life Science
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• v.17 no.5 s.85
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• pp.719-722
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• 2007

IgA nephropathy(IgAN) is considered to be a multifactorial disease with genetic and environmental factors contributing to its pathogenesis. The genes involved in susceptibility and progression of the disease have not yet been clearly elucidated. Megsin is an important candidate gene, predominantly expressed in glomerular mesangium and upregulated in IgAN. To understand biological function of megsin, in this work we have produced transgenic D. melanogaster fly over-expressing human megsin(actin-gal4>UAS-Megsin fly). Introduced human megsin was confirmed by RT-PCR and Western blotting, respectively. Its phenotype is melanin deficiency-abdomen and the megsin gene is stably transferred to the next generations.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.233-238
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• 2008

It has been suspected that various infections, including cytomegalovirus(CMV) infection, are associated with IgA nephropathy. In case of CMV infection, ganciclovir is known to be a treatment of choice for severe CMV infection in general. But ganciclovir has a lot of severe toxicity, so children with normal immunity are seldom treated by ganciclovir when CMV infection is suspected. On the other hand, intravenous immunoglobulin can also be used to treat CMV infection. We report a case of CMV-associated IgA nephrophaty, who was treated with deflazacort and Intravenous immunoglobulin therapy. An 11 years old boy suffered from gross hematuria for 3 days. He had proteinuria, thrombocytopenia(104,000/$mm^3$), antiplatelet antibody(＋), impaired renal function and low serum albumin. His CMV serology was CMV-IgM/IgG(＋/-) and urine CMV-PCR was positive. The renal histological findings revealed IgA nephropathy, WHO class II. His proteinuria persisted despite of deflazacort therapy(2.5 mg/kg/day). Later, intravenous immunoglobulin(1 g/kg) was administered twice. In two years, he showed no gross and microscopic hematuria, and his laboratory findings were also normalized.

• Childhood Kidney Diseases
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• v.26 no.2
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• pp.91-96
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• 2022

The messenger RNA-based vaccine for the coronavirus disease 2019 (COVID-19) may induce glomerulonephritis, including immunoglobulin A nephropathy (IgAN). New-onset IgAN triggered by vaccination against COVID-19 has been reported rarely, especially in children. Herein, we report a pediatric case of newly diagnosed IgAN after administration of the Pfizer vaccine for COVID-19. A 12-year-old girl was referred to our hospital for evaluation of gross hematuria after inoculation with the second dose of Pfizer's COVID-19 vaccine; she had no adverse effects after the first dose. At the time of admission, she showed heavy proteinuria and persistent hematuria. Kidney biopsy revealed an IgAN, and she was treated with an oral steroid and an angiotensin-converting enzyme inhibitor. Four months after discharge, the proteinuria and hematuria resolved completely.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.51-61
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• 2010

Purpose : Previous studies have suggested that Chemokine (C-C motif) ligand-2 (CCL-2; also known as MCP-1) and CCL-5 (also known as RANTES) are possibly associated with the pathogenesis of various inflammatory and non-inflammatory renal diseases. The present study was conducted to investigate association of polymorphisms of CCL-2 and CCL-5 genes with childhood IgA nephropathy (IgAN). Methods : The authors analyzed six single nucleotide polymorphisms (SNPs) of CCL-2 and CCL-5 in 196 pediatric IgAN patients and in 285 healthy controls. We compared variations in SNPs between two several sets of IgAN subgroups, allocated by presence of proteinuria (>4 mg/$m^2$/hour), podocyte foot process effacement, and pathologically advanced disease markers, such as interstitial fibrosis, tubular atrophy, or global sclerosis. Results : Genotypic data of IgAN patients and controls showed no significant SNP frequency difference in both of of CCL-2 and CCL-5. Even though two linkage disequilibrium blocks were formed, there was no significance in the haplotype analysis. In the patient subgroup analysis, no SNP of CCL-2 and CCL-5 was found to be associated with the presence of proteinuria, podocyte foot process effacement, and pathologically advanced disease markers. Conclusion : Our data indicate that no association exists between CCL-2 and CCL-5 SNPs and childhood IgAN susceptibility, and presence of proteinuria, podocyte foot process effacement, and pathologic progression of IgAN.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.147-155
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• 2001

Purpose : IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis tile correlation between urinary abnormalities and GBM thickness and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. Methods : This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. Results : Males accounted for 29/51($56.9\%$) patients with IgAN and 8/26($30.8\%$) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: $43.1\%\;vs\;3.8\%$, p=0.001), the incidence of co-appearance of proteinuria with hematuria ($41.2\%\;vs\;3.8\%$, p=0.001), total amount of protein in 24 hours collected urine ($808{\pm}\;mg\;vs\;251{\pm}200.7\;mg$, p=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine ($23.5\%\;vs\;3.8\%$, p=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was $293.0{\pm}79.2\;nm$(139.7-461.9 nm) and $180.9{\pm}35.8\;nm$(110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was $37.4{\pm}34.4\%$ in IgAN and $93.0{\pm}7.0\%$ in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. Conclusion : The thinning of GBM would be one of the common pathological findings in IgAN Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN, However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play all important role as progressive prognostic indicators in patients with IgAN. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)