• Childhood Kidney Diseases
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• v.1 no.1
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• pp.82-85
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• 1997

The incidence of nephritis associated with Henoch-Sch nlein purpura varies, but glomerulonephritis consistently accounts for most of the associated morbidity and mortality. A very small number of Henoch-Sch nlein purpura develop rapidly progressive glomerulonephritis. A three-year old male patient who showed acute nephritic nephrotic syndrome developed abdominal pain, arthralgia and multiple purpurae on lower extremities later. Peritoneal dialysis was done at the 6th hospital day and continued for 7 months. Renal biopsy disclosed crescentic glomerulonephritis (with 81% crescent formation) and methylprednisolone pulse therapy was done. These days, his general condition is good, but serum creatinine levels are 1.2-1.3 mg/dL. This case was reviewed briefly with the literatures.

• Pediatric Infection and Vaccine
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• v.22 no.3
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• pp.206-209
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• 2015

We report the case of a 7-year-old boy who showed treatment-nonresponsive hypotension (59/29 mmHg) and decreased left ventricular systolic function (fractional shortening 22%) in the acute stage of Kawasaki disease (KD). The present case serves to highlight that methylprednisolone pulse therapy should be considered in patients with intravenous immunoglobulin nonresponsive symptomatic myocarditis during the acute stage of KD.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.40-43
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• 2016

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

• 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.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.10-21
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• 2010

The overall prognosis of Henoch-Schoenlein purpura (HSP) is favorable, but severe nephritis has a high risk of progression to end stage renal failure. Recent studies emphasize the importance of early treatment in children with severe HSP nephritis, but the treatment of severe HSP nephritis still remains controversial due to the rarity of randomized controlled studies in this field. Nevertheless, several intensive therapies, such as intravenous high-dose methylprednisolone pulse, immunosuppressive/cytotoxic drugs, fibrinolytic therapy, anticoagulants, antiplatelet agent and plasma exchange, have been used in children with severe HSP nephritis. In this review, we focus on the treatment of severe HSP nephritis in children.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.83-90
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• 2005

Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of Idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin h(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehyIPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained In the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.987-990
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• 2006

Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis $factor-{\alpha}$ levels are elevated in the acute phase of the disease, especially in patients who develop CAA. We report a 10-month-old male with KD who failed to respond to multiple doses of IVIG and methylprednisolone and who then was treated with infliximab (5 mg/kg single dose). After infliximab treatment, he became afebrile with normalization of inflammatory markers and no further progression of CAA.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.162-173
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• 2006

Purpose : To report the decreasing indicence of HBV(Hepatitis B virus)-associated membranous nephropathy in children after HBV vaccination and to elucidate the clinical course and treatment strategies of IMN(Idiopathic membranous nephropathy). Methods : We retrospectively reviewed the clinico-pathological findings of HBV-MN and IMN patients who underwent a renal biopsy from 1986 to 2005. We compared the HBV-MN and the IMN groups and the remission and the non-remission groups of patients with IMN. Results : Among 24 cases of MN patients, HBV-MN comprised 6 cases(25%) and IMN 18 cases(75%). Clinical masnifestations were nephrotic syndrome(3 cases, 50%), nephritic syndrome(1 case, 16.7%), asymptomatic(2 cases, 33.4%) in the HBV-MN group, asymptomatic(10 cases, 55.5%), nephrotic syndrome(5 cases, 27.8%), and gross hematuria(3 cases, 16.7%) in the IMN groups. From 1996 to 2000, there were 2 cases(28%) of HBV-MN and 5 cases(72%) of IMN. After 2001 all 10 cases were IMN. In the HBV-MN group, 4 cases(66.7%) received interferon and 1 cases received methylprednisolone pulse therapy. In the IMN group, 16 cases(88.9%) received methylprednisolone, 8 cases(44.4%) were in complete remission, 2 cases(11.1%) were in partial remission, 2 cases(11.1%) were in chronic renal failure, and 5 cases(27.8%) were lost to follow-up with sustained proteinuria, 1 case(5.6%) continued to have frequent relapse of nephrotic syndrome without renal insufficiency. In the comparison between remission and non-remission groups, nephrotic range proteinuria and hypertension were more significantly common in the non-remission group(P<0.05). Conclusion : With HBV vaccination, HBV-MN has decreased markedly. IMN is a rare glomerular disease in children. Because the prognosis for patients with nephrotic range proteinuria is poor this group needs more aggressive treatment.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.227-232
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• 2008

A 16-year-old girl presented with proteinuria and microscopic hematuria detected through mass urinary screening and was diagnosed as having suspected postinfectious glomerulonephritis by renal biopsy. However, heavy proteinuria did not respond to angiotensin converting enzyme inhibitor therapy. After 6 months, cervical lymphadenitis developed and a neck node biopsy showed subacute necrotizing lymphadenitis. After an additional 2 months, she developed facial erythema and thrombocytopenia. A repeat renal biopsy demonstrated lupus nephritis class IV. She was treated with pulse methylprednisolone(500 mg/day intravenously for 3 consecutive days) followed by oral deflazacort and monthly intravenous cyclophosphamide pulse(1 g/$m^2$) for 6 months. We report a case diagnosed as systemic lupus erythematosus(SLE) during medical follow-up after urinary screening.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.213-218
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• 2001

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)