Drugs, Chinese Herbal ; therapeutic use ; Glomerulosclerosis, Focal Segmental ; drug therapy ; Humans ; Sclerosis

Type IV collagen is a component of the extracellular matrix in the basement membrane. Abnormal secretion or assembly of type IV collagen may lead to kidney lesions resulting in numerous nephropathy symptoms, e.g., Alport syndrome, thin basement membrane nephropathy, and focal segmental glomerulosclerosis. Treatment for type IV collagen-related nephropathy includes drugs, kidney transplantation, gene and cell therapy. However, drugs are not always effective, and kidney transplantation is hindered by the shortage of donors. Moreover, basement membrane nephritis often occurs after kidney transplantation. Therefore, gene and cell therapy probably is the most promising treatment for type IV collagen related nephropathies.
Cell- and Tissue-Based Therapy ; Collagen Type IV ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Nephritis, Hereditary

Adult ; Cyclosporine ; Glomerulosclerosis, Focal Segmental/drug therapy* ; Humans ; Leflunomide/therapeutic use* ; Nephrosis, Lipoid/drug therapy* ; Nephrotic Syndrome ; Steroids

The aim of this article was to investigate the mechanisms of emodin in antagonizing against organ fibrosis, and to illustrate that emodin can be an effective Chinese herbal preparation for treatment of organ fibrosis.
Animals ; Emodin ; therapeutic use ; Fibrosis ; drug therapy ; Glomerulosclerosis, Focal Segmental ; drug therapy ; etiology ; Humans ; Kidney ; metabolism ; pathology ; Liver Cirrhosis ; drug therapy ; Phytotherapy ; Pulmonary Fibrosis ; drug therapy

PURPOSE: Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. METHODS: Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87 patients were selected for this study. They showed poor response to 4-week daily steroid therapy either initially (initial nonresponder) or subsequently in the disease course (subsequent nonresponder). The medical records including renal pathologic findings were analyzed retrospectively. RESULTS: The mean age at the onset of nephrotic syndrome was 7.3+/-4.1 years and male to female ratio was 62:25. Pathologically, 28 (32%) had minimal change lesion (MCL), 47 (54%) had focal segmental glomerulosclerosis (FSGS) and 12 (14%) had others. There were 15 (54%) initial nonresponders and 13 (46%) subsequent nonresponders in the MCL group, and there were 26 (55%) and 21 (45%), respectively, in the FSGS group. The incidence of hematuria was less frequent in the MCL group. The frequencies of hypertension and azotemia were not significantly different between in the MCL and the FSGS group. Among 10 patients with MCL in whom the steroid therapy were extended to 6 weeks, 3 patients responded subsequently. And 1 of 3 patients among the FSGS group responded to 8-week daily steroid therapy. The 2nd line drug therapy such as oral cyclophosphamide, intravenous pulsed methylprednisolone, enalapril, dipyridamole, etc. was tried in 26 patients with MCL and all 47 patients with FSGS. In the MCL group, 7 of 13 initial nonresponders and 9 of 13 subsequent nonresponders responded to these 2nd line drug therapies. In the FSGS group, 10 of 26 initial nonresponders and 11 of 21 subsequent nonresponders responded to these therapies. While only 1 subsequent nonresponder in the MCL group progressed to chronic renal failure, 9 initial and 4 subsequent nonresponders progressed in the FSGS group. CONCLUSIONS: The FSGS group formed about a half and the MCL group formed about a third of steroid-resistant nephrotic syndrome in children. Although the response to 2nd line drug therapies was not different between 2 groups, the incidence of progression to chronic renal failure was significantly higher in the FSGS group.
Azotemia ; Child ; Cyclophosphamide ; Dipyridamole ; Drug Therapy ; Enalapril ; Female ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Male ; Medical Records ; Methylprednisolone ; Nephrotic Syndrome* ; Retrospective Studies ; Seoul

Fabry disease is an X-linked recessive lysosomal storage disease that is caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. This deficiency results in progressive lysosomal accumulation of glycosphingolipid with particular globotriaosylceramide which accumulates in the heart, kidneys, and the nervous system. The classic Fabry diease affects males, who typically experience an early onset of neuropathic pain, angiokeratoma, and anhydrosis or hypohydrosis. The introduction of enzyme replacement therapy necessitates early awareness of Fabry disease and knowledge of disease- related complications. We experienced a man presenting with acroparesthesia, anhydrosis and proteinuria, who had no residual alpha-galactosidase A activity on leukocytes and mutation analysis demonstrated thiamine deletion at position 1077, exon 7 of GLA gene. He was initially diagnosed as focal segmental glomerulosclerosis without electron microscopic examination three years ago. Now he is being treated with recombinant alpha-galactosidase A via intravenous administration for 1 month.
Administration, Intravenous ; alpha-Galactosidase ; Angiokeratoma ; Enzyme Replacement Therapy ; Exons ; Fabry Disease* ; Glomerulosclerosis, Focal Segmental* ; Heart ; Humans ; Kidney ; Leukocytes ; Lysosomal Storage Diseases ; Male ; Nervous System ; Neuralgia ; Proteinuria ; Thiamine

Collapsing glomerulopathy (CG) has become an important cause of end-stage renal disease (ESRD). First delineated from other proteinuric glomerular lesions in the 1980s, CG is now recognized as a common, distinct pattern of proliferative parenchymal injury that portends a rapid loss of renal function and poor responses to empirical therapy. The first cases in the literature trace back to human-immunodeficiency-virus (HIV)-negative patients who underwent biopsy in 1979. A 45-year-old male patient complained of hematuria and proteinuria eight years ago. He showed an abrupt serum creatinine increase from 1.75 to 2.65mg/dL in the last preceding months. Afterwards, his serum creatinine progressively increased up to 6.82mg/dL. Moreover, his 24 h urine protein level was determined to have reached 6,171 mg/day, as opposed to 670 mg/day a year earlier. Consequently, renal biopsy was performed, and its result showed collapsing glomerulopathy, compatible with the diagnosis. He has undergone continuous ambulatory peritoneal dialysis as renal replacement therapy. Thus, it is reported herein that a patient clinically diagnosed with chronic kidney disease eight years ago showed a sudden renal-function decrease and was clinicopathologically diagnosed with collapsing glomerulopathy based on the results of his renal biopsy.
Biopsy ; Creatinine ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Kidney Failure, Chronic ; Male ; Peritoneal Dialysis, Continuous Ambulatory ; Proteinuria ; Renal Insufficiency, Chronic ; Renal Replacement Therapy

OBJECTIVE: To determine the factors associated with the eff ect of steroid on adult primary nephrotic syndrome. METHODS: The general information, laboratory examination and renal pathological type of 425 patients with primary nephrotic syndrome were retrospectively analyzed. RESULTS: Th ere were significant differences in the response to steroid among the pathological types of minimal change disease, focal segmental glomerulosclerosis and IgA nephropathy. Th e patients in the age of 14-24 years old showed the strongest response to steroid (P<0.05). Th e IgA level in the steroid resistance group was lower than that in the non-steroid resistance group (P<0.05). There was no significant difference in urine protein in 24 hour quantitation in the steroid resistance group between pre- and post-treatment (P>0.05), while there was significant difference in urine protein in 24 hour quantitation in the non-steroid resistance group between pre- and post-treatment (P<0.05). CONCLUSION Pathological types and ages of the patients are related to the steroid curative effect. The decrease in IgA probably affects the effect of steroid on primary nephrotic syndrome.
Adolescent ; Adult ; Glomerulonephritis, IGA ; drug therapy ; Glomerulosclerosis, Focal Segmental ; drug therapy ; Humans ; Kidney ; physiopathology ; Nephrosis, Lipoid ; drug therapy ; Nephrotic Syndrome ; drug therapy ; Proteinuria ; Retrospective Studies ; Steroids ; therapeutic use ; Urinalysis ; Young Adult

No abstract available.
Adolescent ; Biopsy, Fine-Needle ; Fluorodeoxyglucose F18/diagnostic use ; Glomerulosclerosis, Focal Segmental/therapy ; Graft Rejection/drug therapy ; Humans ; Immunosuppressive Agents/therapeutic use ; *Kidney Transplantation ; Liver/radiography/ultrasonography ; Lymphoproliferative Disorders/pathology/*radiography/radionuclide imaging ; Male ; Positron-Emission Tomography ; Tomography, X-Ray Computed

OBJECTIVETo investigate the effects of Tongluo Recipe on the expression of collagen IV (Col IV), fibronectin (FN), laminin (LN), transforming growth factor-beta1 (TGF-beta1) in rat renal tissues and explore the mechanism underlying these effects in rats with glomerular sclerosis.

METHODSThe pathological changes in the renal tissues of rats with glomerular sclerosis were observed microscopically, and the expressions of Col IV, FN, LN, and TGF-beta1 were detected using immunohistochemical staining and image analysis system.

RESULTSTongluo Recipe significantly decreased the expressions of Col IV, FN, LN and TGF-beta1 in the renal tissue of rats with glomerular sclerosis (P<0.05 or P<0.01) and obviously alleviated the renal pathologies (P<0.01).

CONCLUSIONThe therapeutic effects of Tongluo Recipe are probably mediated by lowered expressions of Col IV, FN, LN and TGF-beta1.

Animals ; Collagen Type IV ; biosynthesis ; Drugs, Chinese Herbal ; therapeutic use ; Fibronectins ; biosynthesis ; Glomerulosclerosis, Focal Segmental ; drug therapy ; metabolism ; pathology ; Kidney ; drug effects ; metabolism ; pathology ; Male ; Phytotherapy ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Transforming Growth Factor beta1 ; biosynthesis ; Treatment Outcome