Clinical Characteristics and Prognostic Factors of Hemolytic Uremic Syndrome in Korean Children / 대한신장학회잡지

PURPOSE: Hemolytic uremic syndrome is one of the most frequent cause of acute renal failure in children and can lead to progressive deterioration of renal function. Present nationwide study was undertaken to determine the clinical characteristcs and prognostic factors of hemolytic uremic syndrome in korean children during past 10 years(1990-1999). METHODS: Questionnaires(including clinical data, prodromal illness, lab data, treatment modality and prognosis) were mailed to all teaching hospitals in korea and 27 hospitals responded. During past 10 years, total 149 cases of HUS were diagnosed. Statistical analysis was done by chi-square test, using p<0.05 being "statistically significant". RESULTS: Sex distribution showed slight female preponderance(female 84 vs male 65 cases) and "under 5 years of age" comprised 71.8%(107 cases). Yearly distribution showed increasing number of HUS cases during past 3-4 years and the majority of cases occurred during summer months. Diarrhea was the most common prodromal illness comprising 75.2 % followed by URI 18.2% and in 3.5% of cases no prodromal illness was noted. Lab data (mean+/-SD) showed Hb 7.3+/-2.1g/dL, platelet 49+/-32 X 103cells/mm3, BUN 74+/-36mg/dL, and creatinine 3.7+/-2.8mg/dL. Hypertension was seen in 32.9%, convulsion in 16.7%, mental change in 15.4% and renal replacement therapy(PD or HD) was done in 49.7% of cases. Clinical outcome showed complete recovery in 75.5%, persisting abnormal urinalysis without renal failure in 11.1%, chronic renal failure(including ESRD) in 6.7% and death in 6.7%(9 cases). Poor prognosis was associated with older patients age, higher serum creatinine level, existence of mental change and longer duration of oligoanuria. Out of these, duration of oligoanuria was the most closely associated factor leading to poor outcome. Out of 119 cases with "oligoanuria under 2 weeks", CRF and death were seen in 2 and 7 cases respectively. Compared to this, out of 12 cases with "oligoanuria over 2 weeks", CRF and death were seen in 4 and 2 cases respectively(p<0.0005). CONCLUSION: The incidence of HUS is increasing recently in Korean children. And out of various prognostic factors(older age, higher serum creatinine, existence of mental change and duration of oligoanuria), duration of oligoanuria was the most significantly associated factor leading to poor outcome.

Clinical Characteristics and Prognostic Factors of Hemolytic Uremic Syndrome in Korean Children / 대한신장학회잡지

PURPOSE: Hemolytic uremic syndrome is one of the most frequent cause of acute renal failure in children and can lead to progressive deterioration of renal function. Present nationwide study was undertaken to determine the clinical characteristcs and prognostic factors of hemolytic uremic syndrome in korean children during past 10 years(1990-1999). METHODS: Questionnaires(including clinical data, prodromal illness, lab data, treatment modality and prognosis) were mailed to all teaching hospitals in korea and 27 hospitals responded. During past 10 years, total 149 cases of HUS were diagnosed. Statistical analysis was done by chi-square test, using p<0.05 being "statistically significant". RESULTS: Sex distribution showed slight female preponderance(female 84 vs male 65 cases) and "under 5 years of age" comprised 71.8%(107 cases). Yearly distribution showed increasing number of HUS cases during past 3-4 years and the majority of cases occurred during summer months. Diarrhea was the most common prodromal illness comprising 75.2 % followed by URI 18.2% and in 3.5% of cases no prodromal illness was noted. Lab data (mean+/-SD) showed Hb 7.3+/-2.1g/dL, platelet 49+/-32 X 103cells/mm3, BUN 74+/-36mg/dL, and creatinine 3.7+/-2.8mg/dL. Hypertension was seen in 32.9%, convulsion in 16.7%, mental change in 15.4% and renal replacement therapy(PD or HD) was done in 49.7% of cases. Clinical outcome showed complete recovery in 75.5%, persisting abnormal urinalysis without renal failure in 11.1%, chronic renal failure(including ESRD) in 6.7% and death in 6.7%(9 cases). Poor prognosis was associated with older patients age, higher serum creatinine level, existence of mental change and longer duration of oligoanuria. Out of these, duration of oligoanuria was the most closely associated factor leading to poor outcome. Out of 119 cases with "oligoanuria under 2 weeks", CRF and death were seen in 2 and 7 cases respectively. Compared to this, out of 12 cases with "oligoanuria over 2 weeks", CRF and death were seen in 4 and 2 cases respectively(p<0.0005). CONCLUSION: The incidence of HUS is increasing recently in Korean children. And out of various prognostic factors(older age, higher serum creatinine, existence of mental change and duration of oligoanuria), duration of oligoanuria was the most significantly associated factor leading to poor outcome.

Glomerular Hypertrophy and Sclerosis in Rats with Chronic Puromycin Aminonucleoside Nephropathy / 대한신장학회잡지

PURPOSE: This experimental study was conducted to determine serial morphological changes of rat's kidney with chronic puromycin aminonucleoside (PAN) nephropathy. Special emphasis was given to the occurrence of glomerular hypertrophy and its relationship to the subsequent development of focal segmental glomerulosclerosis(FSGS). METHODS: Sprague-Dawley rats weighing 200-230g were used and divided into control(n=9) and experimental group(n=15). Rats were given subcutaneous injections of PAN at a dose of of 2mg/100g body weight, or an equivalent volume of normal saline and six injection were given over a period of 9 weeks, at weeks 0, 1, 3, 5, 7 and 9. At weeks 4, 8 and 11, rats were sacrificed and kidney weight, kidney weight/body weight(%) and various laboratory tests including serum protein and albumin were determined. Renal tissues were prepared with Histochoice(R) fixative and paraffin embedding for morphologic study. RESULTS: Kidney weight and kidney weight/body weight(%) were increased significantly in experimental group compared to controls at 4, 8 and 11 weeks. Heavy proteinuria along with lowering of serum protein and albumin and elevation of serum cholesterol was seen in experimental group at week 4 and this change became more marked on weeks 8 and 11. The frequency of FSGS in experimental animal, at week 4, 8 and 11 were 0.6%, 10.6% and 26.2% respectively(p<0.05) and the development of FSGS was more marked in juxtamedullary glomeruli compared to cortical glomeruli. Glomerular surface area showed significant increase in experimental animals compared to controls(p<0.01), the percentage of increase being 12.0, 14.7 and 12.3% at week 4, 8 and 11. And the surface areas of juxtamedullary glomeruli were larger than those of cortical glomeruli throughout the study period. CONCLUSION: In summary, present study indicates that glomerular hypertrophy occurs and precedes the development of FSGS in rats with chronic PAN nephropathy and juxtamedullary glomeruli are more susceptible to developing FSGS compared to cortical glomeuli.

Henoch-Schonlein Nephritis Children

PURPOSE: This retrospective study has been undertaken to find out the clinical outcome of children with HS nephritis and its relationship with initial clinical presentation and/or renal pathologic finding. PATIENTS AND METHODS: Study population consisted of 59 children with HS nephritis who have been admitted to the Pediatric department of KyungPook University Hospital from 1987 to 1999, and biopsy was done with indications of heavy proteinuria ( > 1 g/m2/day ) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 year. Patients were divided clinically into 3 groups ; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings were graded from I-V according to International Study of Kidney Disease in Children (ISKDC). RESULTS: Mean age of presentation was 8.1+/-3.0 years and slight male proponderance was noted ( 33 boys and 26 girls ). Histopathologic grading showed Grade I ; 2, Grade II ; 44, and Grade III ; 13 cases. Clinical outcome at the follow-up period of 1-2 years (49 cases) and 3-4 years (30 cases) showed normal urinalysis in 15 ( 30.6% ) and 18 cases ( 60.0%), persistent isolated hematuria in 20 ( 40.8% ) and 2 cases ( 6.7 % ), hematuria with proteinuria in 11 ( 22.5% ) and 8 cases ( 26.6% ), and persistent heavy proteinuria in 3 ( 6.1% ) and 2 cases ( 6.7% ) respectively. Clinical outcome according to histopathologic grading showed the frequency of normalization of urinalysis being lower in Grade III compared to grade I or II. Clinical outcome according to initial clinical presentation showed no relationship to the normalization of urinalysis at follow-up periods. However, 15-20% of children with initial heavy proteinuria showed persistent heavy proteinuria ( 3 out of 20 cases at 1-2 years, and 2 out of 10 case at 3-4 years of follow-up periods). CONCLUSION: The majority of children with HS nephritis (histopathologic grade I, II, III) improved within 3-4 years, and persistent heavy proteinuria was seen only in a few of children with initial clinical presentation of heavy proteinuria.