Purpose : We evaluated children with Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) in terms of epidemiology and clinical characteristics. Methods : A total of 424 medical records of children with HSP admitted to The Catholic University of Korea, Daejeon St. Mary's Hospital, from 1987 to 2003 were retrospectively analyzed. Results : The mean annual number of cases was $25.1{\pm}7.9$ and no one year showed an outbreak. There was a steady number of patients throughout the year with a decrease during the summer season. The male-to-female ratio was 1.3 : 1 with the median age of the patients being 6-years-old. The age distribution showed a peak at age 6 in a bell-shaped distribution curve. Purpura was noted in 100 percent of the patients, gastrointestinal involvement in 53.8 percent, joint involvement in 40.8 percent, and renal involvement in 18.9 percent. Nephrotic syndrome occurred in 1 percent of all patients. Conclusion : The epidemiologic and clinical features of HSP were similar to those of other regions in Korea and foreign nations, irrespective of time.
Objective : Membranous nephropathy (MN) is the most common cause of adult nephrotic syndrome worldwide. MN has been defined as granular subepithelial deposition of IgG immune complexes along the glomerular basement membrane (GBM). We aimed to identify the effects of Chungyeolmaksungbang (CYMSB) treatment on cBSA-induced in MN mouse model. Methods : The effect of Chungyeolmaksungbang treatment was studied on the morphology and protein excretion in the cationized bovine serum albumin (cBSA)induced mouse chronic serum sickness nephritis model. One group of mice was given intra-peritoneal (i.p.) immunizing doses of cBSA and complete Freund's adjuvant. One week later, these animals began a single i.p. injection of cBSA for 4 weeks. A second group followed the same injection protocol, but was given CYMSB p.o. Results : Proteinuria significantly was decreased and serum albumin was increased in the group treated with cBSA and CYMSB extract compared with the control. Serum BUN was significantly decreased on CYMSB compared with control. CD3e+/CD19 cells ratio of peripheral blood was decreased and CD4+/CD8 cells was increased. Level of $IL-1{\beta}$ was significantly decreased, and $IFN-{\gamma}$ was significantly increased. Concentration of IgG and IgM was significantly decreased compared with control. Thickness of GBM was decreased on histological analysis of kidney. Deposition of CD4 and CD8 was decreased on immunohistochemical staining of kidney. Conclusions : We conclude that CYMSB treatment may could be a useful remedy agents for treating the MN with cBSA.
Objective : Membranous nephropathy(MN) is one of the most common causes of nephrotic syndrome in adults. MN has been defined as granular subepithelial deposition of IgG immune complexes along the glomerular basement membrane(GBM). However, there is not a satisfactory treatment for MN. We aimed to identify the effect of Impatiens balsamina Linne(IBL) treatment on cationic bovine serum albumin(cBSA)-induced MN in a mouse model. Methods : Mice were divided into 4 groups. The normal group was injected with saline. The Control group was treated with cBSA(50 mg/kg i.p) only. The third group IBL-100, was treated with cBSA(50 mg/kg, i.p) and IBL(100 mg/kg, p.o). The fourth group IBL-400, was treated with cBSA(50 mg/kg, i.p) and IBL (400 mg/kg, p.o). After cBSA and IBL treatment for 6 weeks, we measured change of body weight, proteinuria, serum albumin, total cholesterol, triglyceride, BUN, creatinine, IgA, IgM, IgG, TNF-${\alpha}$, IL-6 and IL-$1{\beta}$ levels. The morphologic changes of renal glomeruli were also observed with a light microscope. Results : The level of proteinuria significantly decreased and serum albumin increased in groups treated with cBSA and IBL extract compared with the control. The levels of serum triglyceride, BUN, IgG, TNF-${\alpha}$, IL-$1{\beta}$ significantly decreased in both IBL groups. In histological findings of kidney tissue, thickening of GBM decreased in both IBL groups. Conclusions : This study shows that IBL might be effective for treatment of acute stage MN. More clinical data and studies are to be done for efficient application.
Cho Hee-Yeon;Lee Bum-Hee;Kang Ju-Hyung;Ha Il-Soo;Cheong Hae-Il;Choi Yong
Childhood Kidney Diseases
/
v.9
no.1
/
pp.83-90
/
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.
Generalized edema and hypoalbuminemia are relatively common presenting manifestations in many clinical situations. The differential diagnosis of hypoalbuminemia include: Kwashiorkor, synthetic dysfunction of the liver, and excessive protein loss as in nephrotic syndrome. In systemic lupus erythematosus (SLE), hypoalbuminemia and generalized edema are most commonly due to protein loss associated with lupus nephritis; gastrointestinal involvement is uncommon, and therefore protein loss through the gastrointestinal tract is quite rare. We report a case of a protein losing enteropathy (PLE) associated with SLE. The patient was referred to our hospital for generalized edema, arthralgia and facial rash. After clinical evaluation, the patient met the criteria for the SLE diagnosis; hypoalbuminemia with general edema was consistent with a protein losing enteropathy. After two weeks of therapy with parenteral high dose glucocorticoid, the patients was improved in laboratory findings as well as clinical symptoms.
Objectives Edema occurs in various disorders, such as heart failure, nephrotic syndrome, renal failure, liver cirrhosis, and cancer. The excessive accumulation of interstitial fluid in limb tissue will often manifest with ankle and knee swelling and lowers patient's quality of life. But the conventional treatment includes restricting dietary sodium and using diuretics. The purpose of this study is about significant improvement of a patient with edema who had suffered from dependent gait accompanied by mild pain, after treatment by herbal medicine based on SCM(Sasang constitutional medicine). Methods The pattern identification of the patient was diagnosed with a superficial disease of Taeeumin. So after the admission, Gunyuljejo-tang was used, almost two times per day during 2 weeks. Also, we applied acupuncture routinely every day and western medicine as needed. We measured the circumferences of Lt. thigh and followed up the bioelectrical impedance analysis and checked the appearance of lower limb. Results and Conclusions The therapy was effective, and the circumferences, which was 59.69cm at onset time, decreased to 52.07cm almost same to the healthy side of her thigh. There was no more edematous sign on the body, either. This case showed that management of Taeeumin's healthy energy(呼散之氣, the energy of exhalation) could be effective in treating edema.
Objective: Adriamycin (ADR) is an important anti-cancer drug which can cause renal toxicity. Given the known anti-inflammatory and antioxidant effects of Plantago major (P. major), the aim of this study was to determine the effects of hydroalcoholic extract of P. major on ADR- induced nephropathy in rats. Methods: Fifty male Wistar albino rats were randomly divided into 5 groups including: control, ADR (5 mg/kg), ADR + P. major (600 and 1200 mg/kg) and P. major (1200 mg/kg). The animals were treated with P. major extract for 5 consecutive weeks and ADR was intravenously injected on the 7th day of the study. Urine and serum samples were collected on days 0, 14, 21, 28, and 35 for the measurement of serum cholesterol and albumin levels and urine protein excretion rate. At the end of the study, the left kidneys were removed for apoptosis assessment. Results: Administration of ADR significantly decreased serum albumin level and increased serum cholesterol and urine protein excretion rate as well as, apoptotic cell numbers compared to the control group (P < 0.001) while had no effect on glomerular filtration rate (P > 0.05). Treatment with P. major, in both 600 and 1200 mg/kg doses, increased serum albumin level and decreased serum cholesterol concentration, urine protein excretion rate and as well as the number of apoptotic cell compared to the ADR group (P < 0.001). Conclusion: Our results showed that the P. major extract effectively protects against ADR- induced nephropathy by reducing kidney apoptosis and improving renal functioning in rats.
Purpose : One of the most important adverse effects of long-term cyclosporine therapy is nephrotoxicity, the morphologic changes of which include interstitial fibrosis and arteriolar hyalinization. Recently, several authors have shown that osteopontin plays an important role in the development of interstitial fibrosis by acting as a macrophage chemoattractant and stimulating the production of $TGF-{\beta}$ in experimental cyclosporine nephrotoxicity. However, the relationship between osteopontin and $TGF-{\beta}$ in humans has not been clearly documented so far. We studied the expression of osteopontin and $TGF-{\beta}$ in children with minimal change nephrotic syndrome treated with cyclosporine to demonstrate whether there is a relationship between cyclosporine toxicity and osteopontin expression as previously shown in animal models. Materials and methods : Nineteen children (15 males and 4 females) were the subject of this study. Renal biopsies had been performed before and after the cyclosporine therapy (mean duration: 15.9 months). In 5 patients, additional biopsies were performed after completing the cyclosporine treatment (mean; 26 months). The expressions of osteopontin and $TGF-{\beta}$ were evaluated by immunohistochemistry in the glomeruli and tubulointerstitium. Results : Osteopontin expression was significantly increased in the glomerular mesangium and tubules after cyclosporine treatment. But there was no statistically significant increase of $TGF-{\beta}$ in the interstitium. There was no significant increase in tubular osteopontin and interstitial $TGF-{\beta}$ expression in those cases developing interstitial fibrosis after cyclosporine treatment compared with cases those not developing interstitial fibrosis. No significant changes in osteopontin or $TGF-{\beta}$ expression were observed in subsequent 5 biopsy samples after discontinuation of cyclosporine compared with the first follow up biopsies. Conclusion : These results suggest that osteopontin is a nonspecific marker of renal injury rather than a mediator of interstitial fibrosis in cyclosporine nephrotoxicity of human.
Purpose : Urinary N-acetyl-beta-D-glucosaminidase(NAG) and beta 2-microglobulin(B2M) is considered to be a marker of tubulointerstitial injury. The aim of this study was to examine the urinary levels of NAG and B2M in children with various renal diseases. Methods : We studied 21 children(8.9$\pm$4.5 years, Male:Female=14:7) and they were divided into three groups: group I(steroid-sensitive nephrotic syndrome-4 patients), group II(various kinds of glomerulonephritis-4 patients), and group III(normal urinalysis or non-glomerular renal diseases-13 patients). Results : Urinary NAG levels in groups I and II were significantly higher than those in group III(19.4$\pm$11.5 and 30.0$\pm$30.1 vs. 4.7$\pm$3.9, P=0.01), while urinary B2M levels did not differ among the 3 groups, although urinary NAG levels were positively correlated with urinary B2M levels(r=0.49, P=0.03). Urinary NAG and B2M levels were all correlated with proteinuria(r=0.79, P<0.001 and r=0.68, respectively, P=0.001) serum albumin(r=-0.72, P<0.001 and r=-0.57, respectively, P=0.01) and cholesterol(r=0.58, P=0.006 and r=0.56, respectively, P=0.013) levels. Conclusions : Urinary excretions of NAG and B2M are increased in children with steroidsensitive nephrotic syndrome and various kinds of glomerulonephritis, suggesting tubular dysfunction might be present in these diseases.
Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.
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