PURPOSE: The long term disease course and prognostic factors were evaluated in childhood Henoch-Schonlein purpura nephritis(HSPN). METHODS: A total of 75 children(44 boys and 31 girls) with HSPN were included in this study. The onset age was 8.0+/-3.1 years(2.3-15.3 years), and the follow-up period was 4.3+/-3.6 years(1.0-17.1 years). Kidney biopsy was done in 24 children(32%). Initial clinical and laboratory findings were evaluated. In addition, polymorphisms of the renin angiotensin system(RAS) genes(insertion/deletion in intron 16 of ACE gene, M235T in AGT gene, and A1166C in AGTR gene) were analysed. The initial and last clinical states were classified into 4 groups as follows:A, normal; B, minor urinary abnormalities; C, active renal disease (nephrotic-range proteinuria and/or hypertension with serum creatinine < or =1.5 mg/dL); D, renal insufficiency. RESULTS: At the onset, the clinical states of the patients were B in 26(35%), C in 46(61%), and D, in 3(4%). The distribution of the RAS gene polymorphism of HSPN were not different from that of 100 healthy control subjects. At the last follow-up, the clinical states of the patients were A in 23(31%), B in 38(50%), C in 9(12%), and D in 5(7%). A multiple logistic regression identified age at the onset and initial urine protein excretion as significant prognostic factors. Analysis of genotypes of the 3 RAS genes as prognostic values revealed no statistical significance. CONCLUSION: Older age at onset and severe proteinuria were identified as poor prognostic factors of childhood HSPN. Implication of the RAS gene polymorphism in HSPN could not be validated in this small-scale retrospective study.
Age of Onset ; Angiotensins ; Biopsy ; Child* ; Creatinine ; Follow-Up Studies ; Genes, ras ; Genotype ; Humans ; Hypertension ; Introns ; Kidney ; Logistic Models ; Nephritis* ; Proteinuria ; Purpura, Schoenlein-Henoch* ; Renal Insufficiency ; Renin ; Retrospective Studies

PURPOSE: Interleukin 1 receptor antagonist(IL-1ra) is an endogenous antiinflammatory agent that binds to IL-1 receptor and thus competitively inhibits the binding of IL-1alpha and IL-1beta. Allele 2 in association with various autoimmune diseases has been reported. In order to evaluate the influence of IL-1ra gene VNTR polymorphism on the susceptibility to HSP and its possible association with disease severity, manifested by severe renal involvement and renal sequelae, we studied the incidence of carriage rate and allele frequency of the 2 repeats of IL-1ra allele 2(IL1RN*2) of the IL-1ra gene in children with HSP with and without renal involvement. METHODS: The IL-1ra gene polymorphisms were determined in children with HSP with(n= 40) or without nephritis(n=34) who had been diagnosed at Busan Paik Hospital and the control groups(n=163). Gene polymorphism was identified by PCR amplification of the genomic DNA. RESULTS: The allelic frequency and carriage rate of IL1RN*1 were found most frequently in patients with HSP and in controls. The allelic frequency of IL1RN*2 was higher in patients with HSP compared to that of controls(4.7% vs. 2.5%, P=0.794). The carriage rate of IL1RN*2 was higher in patients with HSP compared to that of controls(8.1% vs. 6.8%, P= 0.916). The allelic frequency of IL1RN*2 was higher in patients with HSP nephritis compared to that of HSP(6.3% vs.2.9%, P=0.356). The carriage rate of IL1RN*2 was higher in patients with HSP nephritis compared to that of HSP(10.0% vs. 5.9%, P=0.523). Among 13 patients with heavy proteinuria(>1.0 g), 11 had IL1RN*1, 1 had IL1RN*2 and the others had IL1RN*4. At the time of last follow up 4 patients had sustained proteinuria and their genotype was IL1RN*1. CONCLUSION: The allelic frequency and carriage rate of IL1RN*1 were found most frequently in patients with HSP and in controls. Our study suggests that the carriage rate and allele frequency of the 2-repeats of IL-1ra allele 2(IL1RN*2) of the IL-1ra gene may not be associated with susceptibility and severity of renal involvement in children with HSP.
Alleles ; Autoimmune Diseases ; Busan ; Child* ; DNA ; Follow-Up Studies ; Gene Frequency ; Genotype ; Humans ; Incidence ; Interleukin 1 Receptor Antagonist Protein ; Interleukin-1* ; Interleukins* ; Nephritis* ; Polymerase Chain Reaction ; Proteinuria ; Purpura, Schoenlein-Henoch* ; Receptors, Interleukin-1*

PURPOSE: Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. METHODS: The study group consisted of 73 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. RESULTS: The age(mean+/-SD) of patients was 4.53+/-2.53 years old. The male to female ratio was 52:21. In 95%, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables:age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.006). CONCLUSION: The rapidity of response is expected to be one of the predictive factors for relapse in children with SRNS.
Biopsy ; Child* ; Diagnosis ; Female ; Gyeongsangbuk-do ; Hematuria ; Humans ; Male ; Medical Records ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Pediatrics ; Recurrence* ; Retrospective Studies

PURPOSE: Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. METHODS: We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. RESULTS: Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62% in the 4 week treatment group and 52% in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was 1.5+/-1.2 times in the 4 week treatment group and 1.1+/-1.2 times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. CONCLUSION: The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.
Appetite ; Biopsy ; Blood Pressure ; Child ; Cholesterol ; Creatinine ; Daegu ; Humans ; Kidney ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Prednisolone* ; Recurrence ; Retrospective Studies ; Serum Albumin

PURPOSE: To determine the histological findings and treatment outcome in cases of childhood nephrotic syndrome which required renal biopsy. METHODS: We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 169 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). RESULTS: Minimal change disease(MCD) was found in 52.1% of the patients, followed by diffuse mesangial proliferation(33.1%), focal segmental gomerulosclerosis(5.3%), membranoproliferative glomerulonephritis(2.4%), membranous nephropathy(2.4%), and IgA nephropathy(1.8 %). In MCD children, 14.8% had hematuria, 22.7% had hypertension, 5.7% showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2% had hematuria, 21.0% was found to be hypertensive, 7.4% of children showed decreased renal function and only 3(3.7%) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0%) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. CONCLUSION: Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate.
Biopsy ; Child ; Complement System Proteins ; Diagnosis* ; Hematuria ; Humans ; Hypertension ; Immunoglobulin A ; Methylprednisolone ; Nephrotic Syndrome* ; Pediatrics ; Retrospective Studies ; Seoul ; Treatment Outcome

PURPOSE: Hypogammaglobulinemia has been observed in nephrotic syndrome, but its pathophysiology remains unknown. We evaluated serum immunoglobulins, IgG subclasses, and vaccine-induced viral antibodies(anti-hepatitis B surface IgG and anti-measles IgG) in children with minimal change nephrotic syndrome(MCNS). METHODS: Using the stored sera, the levels of immunoglobulin(IgG, IgM, IgA, and IgE) and IgG subclasses(IgG 1, 2, 3, and 4), anti-HBs Ab and anti-measles IgG of 21 children with MCNS were analyzed and compared to those of 25 age-matched healthy children. RESULTS: The mean values of IgG and IgG1 were 390+/-187 mg/dL and 287+/-120 mg/dL in nephrotic children, and 1,025+/-284 mg/dL and 785+/-19 mg/dL in control children, respectively. The values of the total IgG and the 4 IgG subclasses in nephrotic children were all significantly depressed(P<0.001), but the IgM(251+/-183 mg/dL vs. 153+/-55 mg/dL, P=0.02) and IgE values(P=0.01) were elevated, and the IgA values were not changed. The seropositivity of anti-HBs IgG was 42.9%(9 of 21 cases) in the MCNS group and 52%(13/25) in the control group, and that of anti-measles IgG was 76%(16/21) and 92%(23/25), respectively, but there was no statistical difference between the two groups. CONCLUSION: IgG and IgG subclass levels in MCNS children are all depressed without significant seronegativity of the vaccine-induced viral antibodies. Further studies are needed to resolve the cause of hypogammaglobulinemia in MCNS.
Agammaglobulinemia ; Antibodies, Viral* ; Child ; Humans ; Immunoglobulin A ; Immunoglobulin E ; Immunoglobulin G* ; Immunoglobulin M ; Immunoglobulins ; Nephrosis, Lipoid* ; Nephrotic Syndrome

PURPOSE: Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A beta-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. METHODS: Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A beta-hemolytic streptococcal infection were evaluated. RESULTS: Six(12.5%) patients showed elevation of serum creatinine level but there was no patient with persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9%). Hematuria disappeared within 6 months(79%) and proteinuria within 6 months(100%) from the disease onset. CONCLUSION: Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4%) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequela.
Biopsy ; Blood Pressure ; Child* ; Complement System Proteins ; Creatinine ; Glomerulonephritis* ; Hematuria ; Humans ; Hypertension ; Kidney Failure, Chronic ; Prognosis* ; Proteinuria ; Renal Insufficiency ; Streptococcal Infections

PURPOSE: Urine collection using a sterile adhesive bag for urinalysis has been used commonly in infants and young children. However, this method has had some drawbacks. So, this study was performed to evaluate the usage of disposable diapers as a substitute for the routine urine collection method. METHODS: 60 mL of self voided fresh urine was collected from 99 patients. Half of the urine was poured on the disposable diapers which did not contain absorbent gel beads. After 1 hr, we obtained the urine samples(extracted urine) by compressing the wet diapers using a 50 ml syringe. Routine, microscopic and biochemical analyses were performed on the other half of fresh urine and extracted urine. Then we compared each result by correlative analysis. RESULTS: 198 samples from 99 patients were evaluated. The results of routine urinalysis except WBC and biochemical urinalysis showed a significant correlation between the two groups(P<0.05). The relative coefficients of urine SG, pH, glucose, protein, blood and leukocytes between the two groups were 0.964, 0.938, 0.977, 0.956, 0.931 and 0.738, respectively. Those of urinary sodium, potassium, chloride and creatinine were 0.997, 0.998, 0.995 and 0.998, respectively. Microscopic examinations showed lower relative coefficients than the other results, 0.740 for RBC and 0.602 for WBC, but these were still significant(P<0.05). CONCLUSION: The results of the urine analysis with extracted urine from diapers correlates well with that of fresh urine. This new method is very helpful and can be used as an alternative of urine collection, especially for infants and young children.
Adhesives ; Child* ; Creatinine ; Glucose ; Humans ; Hydrogen-Ion Concentration ; Infant* ; Leukocytes ; Potassium ; Sodium ; Syringes ; Urinalysis ; Urine Specimen Collection*

PURPOSE: Podocytes are critical in maintaining the filtration barrier of the glomerulus and are dependent on the integrity of slit diaphragm(SD) proteins including nephrin, P-cadherin, and others. Diabetic proteinuric condition demonstrates defects in SD molecules as well as ultrastructural changes in podocytes. We examined the molecular basis for this alteration of SD molecules especially on P-cadherin as a candidate regulating the modulation of pathogenic changes in the barrier to protein filtration. METHODS: To investigate whether high glucose and AGE induce changes in SD, we cultured rat GEpC under normal(5 mM) or high glucose(30 mM) and AGE- or BSA-added conditions and measured the change of P-cadherin expression by Western blotting and RT- PCR. RESULTS: We found that administration of high glucose decreased the P-cadherin production significantly in the presence or absence of AGE by Western blotting. In RT-PCR high glucose with or without AGE also significantly decreased the expression of P-cadherin mRNA compared to those of controls. Such changes were not seen in the osmotic control. CONCLUSION: We suggest that high glucose with or without AGE suppresses the production of P-cadherin at the transcriptional level and that these changes may explain the functional changes of SD in diabetic conditions.
Animals ; Blotting, Western ; Cadherins* ; Diabetic Nephropathies ; Filtration ; Glucose* ; Glycosylation* ; Podocytes ; Polymerase Chain Reaction ; Rats ; RNA, Messenger

Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.
Constriction, Pathologic ; Ear ; Extremities ; Female ; Forehead ; Humans ; Hydronephrosis ; Hypertelorism ; Hypertrichosis ; Hypospadias ; Infant ; Intellectual Disability ; Klebsiella pneumoniae ; Male ; Nephrocalcinosis ; Nose ; Quadriplegia ; Seizures ; Tongue ; Urinary Tract Infections