• Childhood Kidney Diseases
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• v.6 no.1
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• pp.48-55
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• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.61-67
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• 2002

Purpose: The urinary mass screening program in school aged population has been performed since 1981, but the consensus on the follow-up schedule and the management of isolated proteinuria has not been reached yet. The aim of this study was to investigate the cause of isolated proteinuria and to propose a guideline for the treatment and follow-up afterwards Methods: The medical records of 114 cases of isolated proteinuria detected through the analysis of urinary mass screening and evaluated at the pediatric outpatient clinic of Asan Medical Center from January 1990 to July 2001 have been reviewed. Results: The classification of isolated proteinuria was as follows. Transient proteinuria $32\%$, orthostatic proteinuria $65\%$, persistent proteinuria $3\%$, In orthostatic proteinuria group, daytime and nighttime proteinuria were $319.2{\pm}189.1\;mg/dL$ and $56.5{\pm}56.1\;mg/dL$. In persistent proteinuria group, daytime and nighttime proteinuria were $1140{\pm}540.5\;mg/dL$ and $289{\pm}58\;mg/dL$. After 30 month follow-up, 2 cases of persistent proteinuria were needed renal biopsy and 1 case revealed focal segmental glomerular sclerosis. In all cases, serum creatinine, albumin and complements levels were normal. In the orthostatic proteinuria group, no significant renal diseases were detected. Conclusion: Since most of the isolated proteinuria detected through the school urinary mass screening were orthostatic proteinuria or transient proteinuria, initially aggressive diagnostic method such as renal biopsy is not needed and regular follow- up with quantitation of proteinuria is warranted.(J Korean Soc Pediatr Nephrol 2002 ; 6 61-7)

• 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.2 no.2
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• pp.118-124
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• 1998

Purpose : There is no specific treatment guidelines for Henoch-$Sch{\ddot{o}}nlein$(HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine Methods : Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenolisly 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following 1 month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. Results : Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean $7.4{\pm}7.4$ weeks. Clinical remission were seen in 4 cases out of 5 ($80\%$). Mean time period with disappearance of proteinuria and microscopic hematuria were $5{\pm}2.4$ month and $13.3{\pm}2.9$ month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up tidneybiopsiesafterlyearstreatment. Conclusion : As there is no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.117-122
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• 1997

Purpose : To clarify the clinical findings, laboratory findings and disease course of EP complicated with CAPD and to find out possible predisposing factors to EP. Methods : The medical records of 34 children who underwent CAPD at our hospital between Jan. '94 and Dec. '96 were retrospectively reviewed. The clinical features and laboratory findings of EP were analyzed, and several parameters were evaluated as predisposing factors of EP. Results : EP developed in 7(21%) out of 34 patients. The major symptom of EP was turbid peritoneal fluid without fever, abdominal pain or disturbance of drainage in all cases. The microbiologic culture studies of the peritoneal fluid resulted negative in all cases. Patients with peripheral blood eosinophilia before insertion of CAPD catheter had higher risk of EP than those without eosinophilia (P=0.002). And peripheral blood eosinophilia, noted after insertion of hemodialysis catheter in cases with previous hemodialysis before CAPD, showed significant correlation with the occurrence of EP (P=0.016), too. However, there was no significant correlation between peripheral blood eosinophilia noted after insertion of CAPD cathter and the occurrence of EP. Identification of eosinophils in peritoneal fluids was more accurate with cytospin analysis. Conclusions : An early and accurate diagnosis of EP in patients with CAPD can prevent unnecessary treatment of antibiotics. Peripheral blood eosinophilia before insertion of CAPD catheter is one of the predisposing factors of EP. And, cytospin analysis of peritoneal fluid is an accurate method for diagnosis of EP.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.60-66
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• 2003

Purpose : Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. Materials and Methods : Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and $Tc^{99m}-mercaptoacetyl$ triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. Results : Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. Conclusion : We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.49-56
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• 2013

Purpose: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. Methods: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, ${\bigcirc\bigcirc}$ University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. Results: The mean age of the SUS and Sx groups was $10.8{\pm}2.7$ and $9.5{\pm}3.4$ years (P >0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P <0.001) and edema (P =0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P =0.007) and lower hemoglobin (P =0.007) and albumin (P =0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. Conclusion: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.

• Radiation Oncology Journal
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• v.19 no.2
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• pp.142-145
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• 2001

Purpose : Acute leukemia with hyperleukocytosis (more than $10^5/mm^3$) is at high risk of early sudden death, usually from intracerebral hemorrhage. Emergency cranial irradiation is a relatively simple approach to solve this the problem. We summarized our experience of cranial irradiation in 24 leukemic children who presented with hyperleukocytosis. Methods and Materials : Between 1990 and 1998, 40 children with acute leukemia presenting with hyperleukocytosis were referred for emergency cranial irradiation. Among these patients, 24 children were evaluable. There were 16 boys and eight girls, their ages ranged from 2 to 13 years (median 9.5 years). The initial leukocyte counts ranged $109,910/mm^3\;to\;501,000/mm^3$. Peripheral blood smear was peformed in all patients and noted the morphology of the blast. Introduction of emergency cranial irradiation was determined by the leukocyte counts (more than 100,000/mm) and the existence of the blast in peripheral blood smear. All patients were treated with intravenous hydration with alkaline fluid and oral allopurinol. Cranial irradiation started on the day of diagnosis. With 2 Gy in one fraction in 4 patients, 4 Gy in two fractions in 20 patients. Results : The WBC count had fallen in 19 patients (83%) and no intracerebral hemorrhage occurred after irradiation. There were five cases of early deaths. Four patients died of metabolic complications, and one patient with intracerebral hemorrhage. He died 5 hours after cranial irradiation. No patient had any immediate side effect from cranial irradiation. Conclusion : Our data suggest, that emergency cranial irradiation can be safely chosen and effective in childhood leukemic patients presenting with high leukocyte counts.

• Sleep Medicine and Psychophysiology
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• v.15 no.2
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• pp.94-99
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• 2008

The most common cause of obstructive sleep apnea syndrome (OSAS) in childhood is adenotonsillar hypertrophy. Adenotonsillectomy improves the symptoms quite well in most cases. However, some patients could experience the OSAS again after adenotonsillectomy, who might have several risk factors such as incomplete operation, misdiagnosis, combined anatomical malformation, sinusitis or chronic allergic rhinitis, obesity, initial severe OSAS, and early onset OSAS. We report a case of 11-year-old obese boy who presented with snoring for several years. He was obese with body mass index (BMI) of $26.3kg/m^2$ and also found to have fatty liver by ultrasonogram. Initial polysomnography (PSG) showed that he met the criteria of severe OSAS with the apnea-hypopnea index (AHI) of 70.5. He underwent adenotonsillectomy and symptoms improved immediately. Four months later symptoms were relieved with AHI of 0, but 1 year after the adenotonsillectomy he started to complain snoring again and the subsequent PSG results showed that OSAS has relapsed with AHI of 43. Paranasal sinus X-ray and physical examination showed sinusitis and re-growth of adenoid. Obesity was proved not to be a contributing factor because his BMI decreased to normal range ($23.1kg/m^2$) after diet control and regular exercise. Also, liver transaminase was normalized and fatty liver was disappeared on follow-up abdominal ultrasonogram. After treatment of sinusitis, symptoms were relieved with decreased AHI (8.5). This case suggests that simple adenotonsillectomy might not be the end of OSAS treatment in childhood. Patients who had adenotonsillectomy should be followed by subsequent PSG if symptoms recur. It is also important to be aware of risk factors in the recurrent OSAS for the proper intervention according to the cause.