ABSTRACT
A 39-year-old man with a history of hypertension for 10 years and 10 pack-years smoking, was admitted with dyspnea and generalized edema. On admission, renal insufficiency accompanied with nephrotic syndrome was diagnosed. Even on careful examination including history, blood chemistry test, and fundoscopic examination, no clinical evidence of diabetes was found. Renal biopsy findings, which strongly resembled that of diabetic nodular glomerulosclerosis in microscopic features, showed glomerular hypertrophy and nodular mesangeal sclerosis. Additional immunohistochemistry and ultrastructural investigations excluded other possible diseases that should be differentiated; membranoproliferative glomerulonephritis, thrombotic microangiopathy, amyloidosis, monoclonal immunoglobulin deposition disease, fibrillary glomerulonephritis, and immunotactoid glomerulopathy. Idiopathic nodular glomerulosclerosis is histopathologically similar to nodular diabetic glomerulosclerosis but is unusually developed in persons with hypertension and smoking history. Though there were three reports about cases of nodular glomerulosclerosis in the Korean literature, the cases were related to hepatitis B virus or diabetic retinopathy without overt diabetes. We report a rare case of idiopathic nodular glomerulosclerosis that was related to hypertension and smoking without other medical history.
Subject(s)
Adult , Humans , Amyloidosis , Biopsy , Chemistry , Diabetic Nephropathies , Diabetic Retinopathy , Dyspnea , Edema , Glomerulonephritis , Glomerulonephritis, Membranoproliferative , Hepatitis B virus , Hypertension , Hypertrophy , Immunoglobulins , Immunohistochemistry , Nephrotic Syndrome , Renal Insufficiency , Sclerosis , Smoke , Smoking , Thrombotic MicroangiopathiesABSTRACT
Idiopathic nodular glomerulosclerosis is an unusual entity with histopathologic features resembling nodular diabetic glomerulosclerosis but occurs in non diabetic patients and predominantly in older males with smoking and long standing hypertension. It is characterized histologically by nodular mesangial sclerosis, glomerular basement membrane thickening, and arteriolosclerosis in the absence of immune-type deposit, and clinical presentations typically include renal insufficiency and severe proteinuria with relatively poor prognosis. We experienced a rare case of idiopathic nodular glomerulosclerosis presenting rapid progressive renal failure in a 42-year-old man without clinical evidence of diabetes. He was admitted with peripheral edema and renal insufficiency. He had a history of hypertension for 2 years and chronic hepatitis B for 5 years. Funduscopy and renal ultrasound were normal. HbA1c and oral glucose tolerance test were normal and on serum and urine electrophoresis, M-spike was not found. Light microscopic examination demonstrated the characteristic features of lobular glomerulonephritis showing glomerular hypertrophy and nodular mesangial sclerosis. Immunofluorescence was negative for immune-type deposits. Electron microscopy showed marked increase of mesangial matrix, mesangial cell proliferation, moderate effacement of foot processes, and glomerular basement thickening without electron-dense deposits or other specific fibrils. Idiopathic nodular glomerulosclerosis was diagnosed. After discharge, he continued smoking and his blood pressure was not controlled. Three months after first biopsy, second biopsy was performed because of increasement of serum creatinine and specimens showed progression of tubulointerstitial change and nodular mesangial sclerosis. Subsequent serum creatinine was progressively increased and hemodialysis was started 13 months after diagnosis.
Subject(s)
Adult , Humans , Male , Arteriolosclerosis , Biopsy , Blood Pressure , Creatinine , Diabetic Nephropathies , Diagnosis , Edema , Electrophoresis , Fluorescent Antibody Technique , Foot , Glomerular Basement Membrane , Glomerulonephritis , Glucose Tolerance Test , Hepatitis B, Chronic , Hypertension , Hypertrophy , Mesangial Cells , Microscopy, Electron , Prognosis , Proteinuria , Renal Dialysis , Renal Insufficiency , Sclerosis , Smoke , Smoking , UltrasonographyABSTRACT
A 45-year-old woman was admitted with proteinuria, hematuria and intermittent generalized edema. On admission, hypertension, chronic B-viral hepatitis were evident together with a decreased renal function. Light and electron microscopy revealed diffuse and nodular glomerulosclerosis indistinguishable from diabetic nodular glomerulosclerosis. Diabetes mellitus, however, had been excluded both by extensive clinical and by laboratory investigation. The differential diagnosis also included primary and secondary membranoproliferative glomerulonephritis, thrombotic microangiopathy, amyloidosis, monoclonal immunoglobulin deposition disease, fibrillary glomerolonephritis, immunotactoid glomerulopathy, light chain deposition disease. However, these entities could be excluded by immunohistochemistry and ultrastructural investigations. We report a case of idiopathic nodular glomerulosclerosis, which has not been previously reported in the Korean literature.
Subject(s)
Female , Humans , Middle Aged , Amyloidosis , Diabetes Mellitus , Diabetic Nephropathies , Diagnosis, Differential , Edema , Glomerulonephritis, Membranoproliferative , Hematuria , Hepatitis , Hypertension , Immunoglobulins , Immunohistochemistry , Microscopy, Electron , Proteinuria , Thrombotic MicroangiopathiesABSTRACT
Nodular glomerulosclerosis was first described by Kimmelstiel and Wilson in 1936. Diabetic retinopathy and nephropath y are manifestation of the microangiopathy associated with diabetes. The severity of diabetic nephropathy and the occurrence of retinopathy correlate with the duration of clinical diabetes. However, there have been few reports of patients presents presenting with the classic lesions of diabetic microangiopathy in the absence of a known history of diabetes. These reports raise questions regarding the relationship and significance of carbohydrate intolerance to these pathologic abnormalities. A 34-year-old male patient clinically characterized by massive proteinuria and hypertension without evidence of systemic disease is reported. Renal biopsy showed the nodular glomerulosclerosis (Kimmelstiel-Wilson lesion) characteristic of diabetes. Direct opthalmoscopy and fluorescein angiography demonstrated a picture of advanced proliferative diabetic retinopathy. The patient had no history of diabetes mellitus and upon testing had normal glucose values in response to an oral glucose tolerance test. It is concluded that the nodular glomerulosclerosis lesions and proliferative retinopathy, thought to be specific for diabetes mellitus, may present in the absence of either overt clinical diabetes or impaired glucose tolerance.
Subject(s)
Adult , Humans , Male , Biopsy , Diabetes Mellitus , Diabetic Angiopathies , Diabetic Nephropathies , Diabetic Retinopathy , Fluorescein Angiography , Glucose , Glucose Tolerance Test , Hypertension , ProteinuriaABSTRACT
Objective To retrospectively investigate and compare the clinical features in type 2 diabetic patients with various lesions of diabetic nephropathy.Methods One hundred and fifty patients of type 2 diabetes mellitus were registered from December 1990 to April 2004,among them 73 cases of diffuse glomerulosclerosis (DIF)and 77 nodular glomerulosclerosis(NOD)were all proven by renal biopsy.Data such as the durations of diabetes mellitus and hypertension,body mass index(BMI),diabetic retinopathy,HbA_1c,plasma albumin, proteinuria,urine N-acetyl-?,-glucosaminidase,urine osmolarity,ereatinine clearance rate(Ccr)were collected and compared.Results(1)Compared with the patients with DIF,the patients with NOD had longer duration of diabetes mellitus[(122.0?8.1 vs 56.0?7.8)months,P