• Pediatric Infection and Vaccine
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• v.11 no.1
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• pp.126-130
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• 2004

Neisseria meningitidis is a leading cause of meningitis and septicemia in children and young adults. Meningococcemia, the most severe form of infection, represents a part of the various spectrum of the illness, and its clinical manifestations varied from mild fever and occult bacteriemia to fulminant catastrophic events(e.g., sepsis, shock, and death) within a few hours after onset of symptoms. Complement deficiencies, either congenital or acqired, increase the risk for invasive meningococcal disease. Since C3 plays a critical role in the complement cascade, congenital C3 deficiency or conditions that decrease C3(e.g., systemic lupus erythematosus, cirrhosis, nephritis, C3 nephritic factor) increase the risk for invasive disease due to pyogenic bacteria including N. meningitidis. We had experienced a case of meningococcemia and meningococcal meningitis presenting with mild fever, petechiae, and purpura. The patient was a 7-year old male. He had mild fever, vomiting, headache, nuchal rigidity, petechiae and purpura on both lower extremities, which spreaded to the whole body. His blood culture grew Neisseria meningitidis. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics(cefotaxime) for 12 days. However the patient had developed generalized edema, microscopic hematuria & proteinuria on the third day after admission. High ASO titer and low serum C3 level were also noted. The microscopic hematuria returned to normal about 2 months after discharge. The C3 level returned to normal range about 3~4 months after discharge. we report a case of combined acute poststreptococcal glomerulonephritis and acute invasive meningococcal disease with reference review.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.123-129
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• 1999

Purpose: Acute poststreptococcal glomerulonephritis(APSGN) is a renal disease which is characterized by glomerular proliferation and inflammatory changes due to immune reaction. Although the 95% of patients with APSGN seems to recover fully and present as benign course, the remaining patients show poor prognosis. Therefore comparative retrograde study between APSGN with and without nephrotic syndrome was done to find out the any prognostic indicator to predict the outcome in patients with APSGN. Methods: We had retrospectively analyzed seventy-one patients who were diagnosed as APSGN clinically from Mar.1989 to Feb.1999 in Yonsei university medical center. Sixty-four of the patients was APSGN without nephrotic syndrome(Group A) and seven patients were in APSGN with nephrotic syndrome(Group B). Results: Patients who were diagnosed as APSGN with nephrotic syndrome were seven(9.9%) out of seventy-one. In the comparative study, sex ratio was 1:1 in group A and 1.9: 1 in group B, onset mean age was $8.9{\pm}2.6$ in group A and $8.8{\pm}2.6$ in group B. Following clinical profiles were compared but there were no significant difference between these two groups: WBC count($9413{\pm}2964\;vs\;9368{\pm}2650(/mm^3)$), hemoglobin($10.6{\pm}1.2\;vs\;10.0{\pm}0.9(gm/dL)$), ASO($746.1{\pm}640.7\;vs\;614.9{\pm}475.9(IU/ml)$), $C_3(20.1{\pm}17.0\;vs\;16.9{\pm}13.1(mg/dL)$), $C_4(22.8{\pm}9.5\;vs\;22.6{\pm}6.9(mg/dL)$), BUN($25.8{\pm}26.1\;vs\;28.1{\pm}14.5(mg/dL)$), creatinin($0.8{\pm}0.3\;vs\;0.8{\pm}0.3(mg/dL)$), $C_{cr}(80.6{\pm}28.8{\pm}62.4{\pm}31.4(ml/min/1.73\;m^2$)), the duration of edma, gross hematuria, and hypertension. However, we found that there were a significant difference in the duration of proteinuria($1.95{\pm}2.27\;vs\;13.3{\pm}21.1(months)$)(P<0.05), decreased $C_3$ duration($1.9{\pm}2.9\;vs\;7.3{\pm}5.0(weeks)$)(P<0.05) and especially it was proloned according to the amount of early urine protein excretion. Conclusion: Our study showed markedly prolonged duration of proteinuria and decreased $C_3$ duration in patients with APSGN with nephrotic syndrome. We were not able to find the definite prognostic factor that will guide the outcome of patients with APSGN accompaning nephrotic syndrome, but above findings seemed to represent as a relative indication of the outcome of the disease. All patients recovered completely and we did not experience any cases that progressed into the renal failure.

• Clinical and Experimental Pediatrics
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• v.60 no.8
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• pp.266-271
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• 2017

Purpose: The aim of this study was to assess the clinical characteristics of hypertensive encephalopathy according to the underlying etiologies in children. Methods: We retrospectively evaluated 33 pediatric patients who were diagnosed as having hypertensive encephalopathy in Chonbuk National University Children's Hospital. Among the patients, 18 were excluded because of incomplete data or because brain magnetic resonance imaging (MRI) was not performed. Finally, 17 patients were enrolled and divided into a renal-origin hypertension group and a non-renal-origin hypertension group according to the underlying cause. We compared the clinical features and brain MRI findings between the 2 groups. Results: The renal group included renal artery stenosis (4), acute poststreptococcal glomerulonephritis (2), lupus nephritis (2), and acute renal failure (1); the nonrenal group included essential hypertension (4), pheochromocytoma (2), thyrotoxicosis (1), and acute promyelocytic leukemia (1). The mean systolic blood pressure of the renal group ($172.5{\pm}36.9mmHg$) was higher than that of the nonrenal group ($137.1{\pm}11.1mmHg$, P<0.05). Seizure was the most common neurologic symptom, especially in the renal group (P<0.05). Posterior reversible encephalopathy syndrome (PRES), which is the most typical finding of hypertensive encephalopathy, was found predominantly in the renal group as compared with the nonrenal group (66.6% vs. 12.5%, P<0.05). Conclusion: We conclude that the patients with renal-origin hypertension had a more severe clinical course than those with non-renal-origin hypertension. Furthermore, the renal-origin group was highly associated with PRES on brain MRI.

• Clinical and Experimental Pediatrics
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• v.55 no.4
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• pp.136-142
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• 2012

Purpose: Urinary excretion of N acetyl-beta-D glucosaminidase (NAG) and ${\beta}_2$-microglobulin (${\beta}_2$-M) was increased in the presence of proximal tubular damage. Based on these urinary materials, we investigated the ability of expecting renal function in chronic glomerular diseases. In this study, we evaluated the relationship between glomerular filtration rate (GFR) urinary NAG, and urinary ${\beta}_2$-M. Methods: We evaluated 52 children with chronic kidney disease at the Chung-Ang University Hospital between January 2003 and August 2009. We investigated the 24-hour urinalysis and hematologic values in all 52 patients. Serum creatinine, creatinine clearance (Ccr), serum cystatin C, urinary ${\beta}_2$-M and urinary NAG were measured. Results: Out of 52 patients, there were 13 children with minimal change in disease, 3 children with focal segmental glomerulosclerosis, 17 children with immunoglobulin A nephropathy, 15 children with Henoch-Sch$\ddot{o}$nlein purpua nephritis, 3 children with poststreptococcal glomerulonephritis, and 1 child with thin glomerular basement membrane disease. In these patients, there were significant correlation between the Ccr and urinary NAG (r=-0.817; $P$ <0.01), and between the GFR (as determined by Schwartz method) and urinary NAG (r=-0.821; $P$ <0.01). In addition, there was a significant correlation between the GFR (as determined by Bokencamp method) and urinary NAG (r=-0.858; $P$ <0.01). Conclusion: In our study, there was a significant correlation between the GFR and urinary NAG, but there was no correlation between the GFR and urinary ${\beta}_2$-M, suggesting that the GFR can be predicted by urinary NAG in patients with chronic glomerular disease.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.138-145
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• 2009

Purpose : This study was aimed to evaluate the changes of immunologic parameters during hospitalization, and the relationship between IgG and other laboratory or clinical indices in patients with acute poststreptococcal glomerulonephritis (APSGN). Methods : We reviewed the medical charts of 36 children with APSGN who showed ASO titer>250 Todd U/L and C3<70 mg/dL. We evaluated the levels of IgG and other laboratory parameters including C3 and ASO at admission and at discharge (14 cases). Results : The mean age of APSGN patients was $7.5{\pm}2.6$ year of age, and male-to-female ratio was 2.3:1. At presentation, hypertension (systolic blood pressure>125 mmHg), gross hematuria, and weight gain were observed in 27.8% (10/36), 80.1% (29/36), and 80% (24/30) of the patients, respectively. The mean IgG level was $1,432{\pm}322$ mg/dL ($1,025{\pm}234$ mg/dL in control group, P<0.001), and C3 and ASO levels were $26.1{\pm}16.1$ mg/dL and $1,068{\pm}730$ Todd U, respectively. There were no correlation between IgG level and the levels of any of the parameters analyzed (ASO, C3, BUN, creatinine and white blood cell count), and the severity of the disease assessed by the weight-change during admission. The patients aged<6 years of age (10 cases) had less degree of the weight-change, compared to those of the patients aged>8 years of age (15 cases) (-0.6% vs. -5.7%, P=0.01). The IgG and ASO levels did not change, but C3 (P=0.001) and IgM (P=0.02) levels increased during admission. Conclusion : Increased IgG and ASO levels in APSGN did not change, but C3 level increased during admission. IgG level was not correlated with other laboratory parameters (ASO and C3) and the severity of the disease. Younger children seem to have less severe clinical course compare to older children. With our hypothetic pathogenesis of APSGN, further studies are needed to resolve the pathogenesis of the disease including the increase of IgG.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.157-163
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• 2008

Purpose : Several studies have reported the recent increase in the incidence of acute poststreptococcal glomerulonephritis(APSGN). The objective of this study is to see changes of clinical findings/manifwstation in children with APSGN. Methods : Medical records of 63 children who were diagnosed with APSGN in the deparment of Pediatrics, Kyungpook National University Hospital, between January 1992 and December 2006 were reviewed retrospectively. We analyzed various clinical characteristics such as age, sex, degrees of proteinuria, degrees of hematuria, and presence or absence of histories of systemic antibiotic use in children with APSGN, and compared the children with APSGN who were diagnosed between 1992 and 2000 to those who were diagnosed between 2001 and 2006. Results : Age of the patients ranged from 2-14 years(median 7.11 years) at the time of disease onset. Study patients consisted of 41 boys and 22 girls. APSGN followed infection of the throat in 87% of cases. Patient developed an acute nephritic syndrome 12 days after an antecedent streptococcal pharyngitis. Forty patients presented with gross hematuria. Fortyone patients had hypertension at the time of diagnosis. Hypertension disappeared within 7.8$\pm$8.2 days, gross hematuria within 11.3$\pm$17.2 days and microscopic hematuria within 3.5$\pm$3.9 months from the disease onset. Patients in 2001-2006 had significantly higher increase of antistreptolysin O(ASO) titer. However, no significant differences in clinical characteristics were observed. Age, sex, severity of proteinuria, gross or microscopic hematuria, antibiotic therapy did not affect the clinical manifestations of glomerulonephritis. In other words, hypertension, duration of hematuria, recovery of serum C3 level are not different between the two time periods. Conclusion : Our data indicates that patients in 2001-2006 had significantly higher level of ASO titer. However, they did not show significant clinical differences. To evaluate the causes of the resurgence of APSGN, a national epidemic is needed.

• Pediatric Infection and Vaccine
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• v.5 no.2
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• pp.215-220
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• 1998

Purpose : Asymptomatic infections with positive throat culture for group A beta-hemolytic streptococci(GABHS) and high antistreptolysin O(ASO) concentration may lead to sequelae such as rheumatic fever or acute poststreptococcal glomerulonephritis. Children with asymptomatic infections were treated with oral penicillin V to evaluate the effectiveness of penicillin treatment on the asymptomatic infections. Methods : Throat culture and ASO concentration analysis were performed against healthy elementary school children. Thirty-six children with positive throat culture for GABHS and ASO concentrations of 400 IU/mL or more were divided into two groups. Twenty-two children were treated with oral penicillin V for 10 days, and the others were not treated. Eradication rate of GABHS and the change of ASO concentration between the two groups were compared after one month later. Results : Eradication rates of GABHS between treated and untreated children were 91%(20/22) and 50%(7/14) respectively(P<0.05). Children showing elevation of ASO levels more than 100IU/mL were 22%(4/18) in the treated group and 30%(3/10) in the untreated group, while children showing a decrease of more than 200IU/mL in the ASO level were 44%(8/18) and 40%(4/10) respectively. Conclusion : We confirmed the validity of penicillin treatment, because when we treated the asymptomatic children with penicillin V, the GABHS was eradicated effectively. But there was no significant difference of decrease in the ASO levels between the two groups due to long half-life of ASO or poor compliance. Treatment failure was 22% in terms of elevated ASO levels after penicillin treatment.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.26-33
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• 1998

This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.4-12
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• 1997

Purpose: To evaluate the prevalence and clinical manifestations of various glomerulonephritis (GN) in children, a clinicopathological anlysis of 310 biopsied cases were performed. Method: We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 310 cases diagnosed as glomerulonephritis by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results: 1) Male to female ratio was 1.54:1 and the range of age was from 13 months to 15 years 10 months. 2) Among these, 217 (70.0%) patients were belong to primary GN and 93 (30.0%) patients were belong to secondary GN. As a whole, the most common pathologic diagnosis was minimal change lesion (MC, 32.6%), which was followed by IgA nephropathy (IgAN, 15.8%), $Henoch-Sch\"{o}nlein$ purpura nephritis (HSPN, 13.5%), Poststreptococcal glomerulonephritis (PSAGN, 8.1%). 3) Clinical manifestations of patients were asymptomatic urinary abnormality (43.2%), nephrotic syndrome (41.0%), acute glomerulonephritis (14.2%), chronic glomerulonephritis (1.0%), rapidly progressive glomerulonephritis (0.6%). 4) In primary GN, the most common pathologic diagnosis was MC (46.5%), IgAN (22.6%), thin glomerular basement membrane (GBM) disease (7.8%), membranoproliferative glomerulonephritis (MPGN, 5.5%), mesangial proliferative glomerulonephritis (MesPGN,4.6%), focal segmental glomerulosclerosis (FSGS, 4.6%), membranous nephropathy (MN, 0.9%), sclerosing glomerulonephritis (SCGN, 0.9%), crescentic glomerulonephritis (CreGN, 0.5%) and non-specific glomerulonephritis (NonspGN, 6.0%). 5) Major causes of secondary GN were HSPN (45.2%), PSAGN (26.9%), hepatitis B associated glomerulonephritis (HBGN, 17.2%), lupus nephritis (LN, 6.5%), Alport syndrome (2.2%), hemolytic uremic syndrome (1.0%), fibrillary glomerulonephritis (1.0%) in descending order. Conclusions: There are some differences of the results of clinicopathological stuidies of glomerulonephritis in children because of its different indications of renal biopsy, pathologic classification of renal disease and methods of analysis among investigators. In order to establish more reliable data of incidence and classification of childhood glomerulonephritis in Korea, multicenter cooperative study were necessary.