• Childhood Kidney Diseases
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• v.27 no.2
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• pp.64-69
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• 2023

Membranoproliferative glomerulonephritis (MPGN) is a complex group of renal diseases characterized by a specific pattern of glomerular injury that includes thickening of the capillary wall and mesangial expansion, leading to a heterogeneous group of conditions. This review article offers a comprehensive overview of MPGN, its new classification, pathophysiology, diagnostic evaluation, and management options.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.93-99
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• 2019

C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence microscopy. Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are subclasses of C3 glomerulopathy that are distinguishable by electron microscopy. Highly electron-dense transformation of glomerular basement membrane is characteristic of DDD. C3GN should be differentiated from post-infectious glomerulonephritis and other immune complex-mediated glomerulonephritides showing C3 deposits.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.81-84
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• 2005

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis that can affect multiple organs predominantly the skin, joints, gastrointestinal tract and kidney. Although the specific pathogenesis of HSP is not known, there are several hypotheses. Although the importance of the complement activation in glomerular injury in HSP has been suggested, the complement levels and the blood pressure in those patients are usually normal and massive proteinuria is not common. And pathologic renal changes also have been reported to show a large variety of glomerular changes. However, to our knowledge, a membranoproliferative glomerulo-nephritis (MPGN) is a rare renal clinicopathologic manifestation of HSP. We report a 6-year-old boy with HSP who developed MPGN with hypertension, massive proteinuria, and hypo-complementemia revealed activation of the classical complement pathway, although we could not exclude the possibility of other hypocomplementemic glomerulonephritis including post-streptococcal acute glomerulonephritis.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.237-244
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• 2005

Membranoproliferative glomerulonephritis (MPGN) is a progressive primary glomerulonephritis characterized by mesangial proliferation with increased mesangial matrix, subendothelial immune deposits, mesangial interposition and a double contour feature of the glomerular basement membrane. The glomerular involvement in MPGN is usually diffuse, however, cases of focal or segmental MPGN have been reported by several authors. We report a case of focal segmental MPGN with prolonged hypocomplementemia for ,3 years in a 5 years old girl. (J Korean Soc Pediatr Nephrol 2005;9:237-244)

• Journal of Yeungnam Medical Science
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• v.30 no.2
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• pp.136-140
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• 2013

Diabetic nephropathy (DN) is a common complication and the leading cause of end-stage renal disease (ESRD) in diabetic patients. The occurrence of non-diabetic renal disease (NDRD) in diabetic patients has been increasingly recognized in recent years. Generally, renal injuries in DN are deemed difficult to reverse, whereas some NDRDs are often treatable and even remittable. Thus, the diagnosis of NDRD in patients with diabetes mellitus (DM) via a kidney biopsy would be significant for its prognosis and therapeutic strategy. According to recent studies, the most common NDRD is IgA nephropathy in type 2 diabetic patients, and some cases of minimal change disease and membranous glomerulonephritis have been reported in Korea. However, membranoproliferative glomerulonephritis (MPGN) is an uncommon condition in diabetic patients. To our knowledge, there has been no case yet of MPGN, except in a child with type 1 DM. We present an unusual case of a 27-year-old woman who had type 2 DM with MPGN, as confirmed via a kidney biopsy.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.97-101
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• 2005

Renal diseases other than diabetic nephropathy can occur In diabetic patients. Urinary abnormalities or clinical courses inconsistent with the natural progression of diabetic nephropathy are suggestive of non-diabetic renal diseases and should lead to more extensive investigations. Presence of non-diabetic renal diseases in diabetic patients can alter the treatment plan and the prognosis. We report a 9-year-old girl who had type 1 membranoproliferatiye glomerulonephritis as well as type 1 diabetes mellitus.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.325-332
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• 1986

Since Combes' first description of hepatitis B virus associated membranous glomerulonephritis, many reports have shown a strong association of hepatitis B virus infection with various types of nephropathies, especially membranous glomerulonephritis and membranoproliferative glomerulonephritis. Recently, the authors experienced a case of membraoproliferative glomerulonephritis, type I in a 16-year-old male patient with persistent HBs and HBe antigenemia. One year prior to admission the patient was told of hepatitis at routine check, but he remained asymptomatic throughout. He was admitted to our hospital with chief complaints of proteinuria and microscopic hematuria found on routine urinalysis. Liver and kidney biopsy revealed chronic persistent hepatitis and membranoproliferative glomerulonephritis type I, respectively. We report a case of hepatitis B virus associated nephropathy with review of the literatures. Considering the endemic nature of hepatitis B virus infection in Korea, the incidence of hepatitis B viurs associated nephropathy would be proportionally high in comparison with those of other countries. So, the importance of this entity merits special consideration in our country. Further study concerning pathogenesis, epidemiology, and treatment may be needed.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.17-21
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• 2018

Thymic stromal lymphopoietin (TSLP) is an interleukin-7-like cytokine that is an important trigger and initiator of many allergic diseases. TSLP promotes a T-helper type 2 (Th2) cytokine response that can be pathological. A relationship is formed both at the induction phase of the Th2 response through polarization of dendritic cells to drive Th2 cell differentiation and at the effector phase of the response, by promoting the expansion of activated T cells and their secretion of Th2 cytokines and TSLP. In transgenic mice with TSLP overexpression, it has been reported that TSLP leads to the development of mixed cryoglobulinemic membranoproliferative glomerulonephritis. In addition, TSLP can play an important role in the pathogenesis of IgA nephropathy and systemic lupus erythematosus-related nephritis. From our knowledge of the role of TSLP in the kidney, further studies including the discovery of new therapies need to be considered based on the relationship between TSLP and glomerulonephritis.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.45-51
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• 2006

Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.204-210
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• 2003

Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).