No abstract available.
Glomerular Basement Membrane*

Thin basement membrane disease is defined as diffuse thinning of the glomerular basement membrane, and is clinically characterized by persistent hematuria, minimal proteinuria, normal renal function, and a benign course. It can occur together with other glomerular diseases. We experienced a case of thin basement membrane disease concurrent with minimal-change disease. Treatment with corticosteroid resulted in improved proteinuria and peripheral edema during the follow-up period.
Basement Membrane ; Edema ; Follow-Up Studies ; Glomerular Basement Membrane ; Hematuria ; Nephrosis, Lipoid ; Proteinuria

Normal human glomerular basement membrane (GBM) and mesangial matrix (MM) contain several different basement membrane components in varying degrees. The characteristic morphological and ultrastructural changes in patients with diabetic nephropathy are the thickening of the GBM and the expansion of the MM. In order to investigate the changes of extracellular matrix components in diabetes, the immunohistochemical localization was performed in 17 cases with different degrees using antisera to human collagen types I, III, IV, VI, fibronectin, and laminin. The following results were obtained: 1. The reactivity for collagen IV was increased in expanded MM in the diffuse glomerulosclerosis (GS). With the progression to the nodule formation, collagen IV was prominently decreased in the peripheral area of the nodules. 2. Collagen VI was increased in GBM and MM in the diffuse GS, it was especially prominent in the expanded MM. With the progression to nodule formation, collagen VI was prominently increased in the periphery of the nodules. 3. Interstitial collagen I and III were not stained in many of the cases with the diffuse GS. With the progression to nodule formation, these were slightly expressed. A lamellar pattern of positive reaction was noted at the periphery of the late nodular lesions. 4. Fibronectin was increased in GBM & MM in the diffuse GS, it was especially intense in the MM. With the progression to the nodule formation, the reactivity of antibody to the fibronectin was decreased. 5. Laminin was weakly stained along the GBM & trace in the MM, but was not changed in the nodular GS. In summary, the expanded mesangial matrix in the diffuse GS showed a markedly increased staining for collagen IV, fibronectin and collagen VI. Less intense linear staining for collagen VI, fibronectin, laminin, collagen IV and collagen III was noted along the GBM. In the nodular GS, the composition of the early nodules resembled that of the diffuse GS. However, the late nodular lesion of the nodular GS revealed decreased reactivity for collagen IV and fibronectin at the periphery of the nodule, where collagen VI and interstitial collagen I and III were increased in laminated pattern.
Basement Membrane ; Collagen ; Diabetic Nephropathies* ; Extracellular Matrix* ; Fibronectins ; Glomerular Basement Membrane ; Humans ; Immune Sera ; Laminin

The thickness of the glomerular basement membrane may vary not only in glomerular disease, but also in normal persons according to age and sex. But there has been no data on the normal thickness of the basement membrane in Korea. This study was designed to determine the glomerular basement membrane thickness as a reference value according to age and sex, in 50 cases of minimal change disease obtained from patients aged 2~67 years. Measurement of glomerular basement membrane was made on electron micrograph using an image analyzer. The thickness of each case was estimated by the arithmetic and harmonic mean methods. The mean thickness of the glomerular basement membrane was 291.9 47.9 nm by harmonic mean method and 284.2 43.7 nm by arithmetic mean method. And the harmonic mean thickness of the glomerular basement membrane according to age was 249.1 32.5 nm (1~5 years), 256.6 45.3 nm (6~10 years), 279.2 57.9 nm (11~15 years), 303.2 43.8 nm (16~20 years), 335.3 37.5 nm (21~30 years), and 291.1 22.5 nm (over 30 years), respectively. There was a trend that the thickness of glomerular basement membranes increased with the age till 30 years of age. There was no significant sex-related difference. In conclusion, the mean glomerular basement membrane thickness is comparable to the data from western people and shows a trend of increasing thickness according to the age.
Basement Membrane ; Glomerular Basement Membrane* ; Humans ; Kidney ; Korea ; Nephrosis, Lipoid* ; Reference Values

=Abstract= 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.
Child, Preschool* ; Female* ; Glomerular Basement Membrane ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative* ; Humans

No abstract available.
Glomerular Basement Membrane* ; Heparan Sulfate Proteoglycans* ; Heparitin Sulfate*

IgA nephropathy and thin basement membrane disease are common glomerular diseases in persistent microscopic hematuria with or without proteinuria. However, these two conditions cannot be easily distinguished on the biochemical or urinary findings alone. Therefore, renal biopsy is required for correct identification of the two conditions in most cases. Recently, it has been reported that thinning of glomerular basement membrane is accompanied with precipitation of electron dense deposits in some patients with IgA nephropathy. We report a case of IgA nephropathy associated with thin basement membrane disease in a 19-year-old male with microscopic hematuria and mild proteinuria. After 2 years' treatment with angiotensin II receptor blocker, the patient exhibited persistent microscopic hematuria but decreased proteinuria. Our finding concurs with the previous reports indicating that patients with both IgA nephropathy and thin basement membrane disease do not have different clinical features compared to those with IgA nephropathy alone. In addition, clinical outcome does not appear to be affected by thin basement membrane disease when these two conditions are combined.
Basement Membrane ; Biopsy ; Electrons ; Glomerular Basement Membrane ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Immunoglobulin A ; Male ; Proteinuria ; Receptors, Angiotensin ; Young Adult

Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/or pulmonary hemorrhage. The presence of severe crescentic glomerular inflammation with linear deposition of immunoglobulin G along the glomerular basement membrane is pathognomonic. Because renal function is rapidly and often irretrievably destroyed, many patients require hemodialysis all through their lifetime. We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary hemorrhage. The patient was treated with pulse methylprednisolone and plasmapheresis followed by oral corticosteroid and cyclophosphamide. His renal function was successfully recovered with early diagnosis and aggressive treatment.
Adrenal Cortex Hormones ; Anti-Glomerular Basement Membrane Disease ; Autoimmune Diseases ; Cyclophosphamide ; Early Diagnosis ; Glomerular Basement Membrane ; Hemorrhage ; Humans ; Immunoglobulin G ; Immunosuppression ; Inflammation ; Methylprednisolone ; Plasmapheresis ; Renal Dialysis ; Renal Insufficiency

The Psammomys obesus lives in natural desert habitat on low energy (LE) diet, however when maintained in laboratory conditions with high energy (HE) diet it exhibits pathological metabolic changes resembling those of type 2 diabetes. We have evaluated and correlated the histopathology, metabolic and functional renal alterations occurring in the diabetic Psammomys. Renal function determined by measuring glomerular filtration rate (GFR), protein excretion, protein/creatinine ratio and morpho-immunocytochemical evaluations were performed on HE diet diabetic animals and compared to LE diet control animals. The diabetic animals present a 54% increase in GFR after one month of hyperglycemic condition and a decrease of 47% from baseline values after 4 months. Protein excretion in diabetic animals was 5 folds increased after 4 months. Light microscopy showed an increase in glomeruli size in the diabetic Psammomys, and electron microscopy and immunocytochemical quantitative evaluations revealed accumulation of basement membrane material as well as frequent splitting of the glomerular basement membrane. In addition, glycogen-filled Armanni-Ebstein clear cells were found in the distal tubules including the thick ascending limbs of the diabetic animals. These renal complications in the Psammomys, including changes in GFR with massive proteinuria sustained by physiological and histopathological changes, are very similar to the diabetic nephropathy in human. The Psamommys obesus represents therefore a reliable animal model of diabetic nephropathy.
Animals ; Basement Membrane ; Diabetic Nephropathies ; Diet ; Ecosystem ; Evaluation Studies as Topic ; Extremities ; Gerbillinae ; Glomerular Basement Membrane ; Glomerular Filtration Rate ; Humans ; Light ; Microscopy ; Microscopy, Electron ; Models, Animal ; Proteinuria

A 29-year-old male patient was admitted due to his general weakness and poor oral intake for several months. He was diagnosed as having Crohn disease 16 years ago and total colectomy was performed 10 years ago. On the 3rd day after admission, gross hematuria and sudden hemoptysis combined with diffuse infiltration were noted on chest X-ray. His symptoms and the diffusely increased lung opacities improved with administering high-dose steroid therapy. Later, anti-GBM antibody was found to be positive on the laboratory findings. We report here on a rare case of Goodpsture syndrome combined with prolonged Crohn disease along with a review of literature.
Adult ; Anti-Glomerular Basement Membrane Disease* ; Colectomy ; Crohn Disease* ; Hematuria ; Hemoptysis ; Humans ; Lung ; Male ; Thorax