• 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.

• Journal of Chest Surgery
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• v.33 no.10
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• pp.792-797
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• 2000

Background : Even though there were developments in various treatment techniques for acute arterial occlusion this disease still has high rate of mortalities and limb amputations. We investigated the combined diseases symptoms location of occlusion type of treatment complication and prognosis in our patients. Meterial and methods: This study recruited 48 patients(42 men, 6 women, mean age 57.7 years) who received the operation from January 1995 toDecember 1998. We investigated the post-operation course via medical record review or telephone interview with patients or their family members. Result: The most common combined diseases were atherosclerosis in 30 patients. other diseases were 17 diabetes mellitus 16 hypertension and 12 atrial firillation. Pain and clod sensation were noticed in all patients paresthesia in 5 patients fibrillation. Pain and cold sensation were noticed in all patients paresthesia in 5 patients and lower extremity paralysis in 11 patients. In 29 patients the time interval from the onset of symptom to admission was over 72 hours and 15 patients were admitted within 24 hours. The distribution of arterial occlusion location was at 28 femoral arteries 14 popliteal arteries and 6 iliac arteries. All the patients were received embolectomy and 5 patients were received additional bypass grafting. Postoperative complications were 12 reocclusions. 6 compartment syndromes 6 skin necrosis and 2 acute renal failure. The mortality rate was 16.7%(8/48) and the amputation rate was 25% Conclusion : This study revealed 25% reocclusion 25% limb amputation and 16.7% mortaliyt. To improve the prognosis of acute lower extrements arterial occlusion early diagnosis and understand the underlying diseases prompt treatment and operation additional operation including interventional radiologic examination and thorough postoperative care would be appreciated.

• Microbiology and Biotechnology Letters
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• v.50 no.4
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• pp.441-456
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• 2022

Shiga toxins (Stxs) are major virulence factors from the enterohemorrhagic Escherichia coli (EHEC), a subset of Stx-producing Escherichia coli. Stxs are multi-functional, ribosome-inactivating proteins that underpin the development of hemolytic uremic syndrome (HUS) and central nervous system (CNS) damage. Currently, therapeutic options for the treatment of diseases caused by Stxs are limited and unsatisfactory. Furthermore, the pathophysiological mechanisms underpinning toxin-induced inflammation remain unclear. Numerous works have demonstrated that the various host ribotoxic stress-induced targets including p38 mitogen-activated protein kinase, its downstream substrate Mitogen-activated protein kinase-activated protein kinase 2, and apoptotic signaling via ER-stress sensors are activated in many different susceptible cell types following the regular retrograde transportation of the Stxs, eventually leading to disturbing intercellular communication. Therapeutic options targeting host cellular pathways induced by Stxs may represent a promising strategy for intervention in Stx-mediated acute renal dysfunction, retinal damage, and CNS damage. This review aims at fostering an in-depth understanding of EHEC Stxs-mediated pathogenesis through the toxin-host interactions.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.144-150
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• 1997

Purpose : The kidney is one of the most common sites of cyst formation. Cystic diseases of the kidney are a diverse group of clinicopathologic entities and variable prognosis. They span a wide range of both age of presentation and severity of the renal disease. And many of them are systemic disorders, sharing similar process of cyst formation in other organs. Recently, development of imaging studies has been contributing widely to the diagnosis of the diseases. Treatment, however, is not established satisfactorily. We performed this study to evaluate the occurrence and treatment of cystic diseases of the kidney. Methods : We reviewed retrospectively the medical records of 44 patients with cystic diseases of the kidney in the Department of Pediatrics, during last 11 years. Results : In the 44 patients with cystic diseases of the kidney, 31 patients(71%) had multicystic dysplastic kidney and 11(35%) of them received nephrectomy due to differentiation from neoplasms or severe abdominal distension. Seven patients(16%) had polycystic kidney disease, and all of them were infantile type. Five patients(11%) were diagnosed as having a simple renal cysts. Progression to renal failure was noted in none of the cases. In 14(32%) out of total 44 patients, the diagnosis was made in neonatal or infantile pelted. Conclusion : The incidence of cystic diseases of the kidney appeared very low, but further investigation on their pathogenesis, classification, and indication of treatment is needed.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.125-135
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• 2001

Purpose : Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSCS) have been made but have yielded conflicting results. Reports are rare especially in Pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS Method : Fifty children who diagnosed as biopsy-proven FSGS at department of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. Results : The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was $34\%$ at 5 years, $8\%$ at 10 years Five-year survival rate was $74\%$ in normal renal function group and $27\%$ in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(P<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival( P<0.05). The progression rate to renal failure did not show statistically significant factor. Conclusion : We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors. (J Korean Soc Pediatr Nephrol 2001 ;5 : 125-35)

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.31-37
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• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.

• 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.73-78
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• 2013

Purpose: We aimed to investigate the clinical characteristics and associated diseases in children with a horseshoe kidney and compared these data between children and adults. Method: We performed a retrospective analysis of the medical records and radiological findings of 43 patients diagnosed with a horseshoe kidney in the Busan Paik Hospital. The subjects were divided into the children's group (14 cases, age <18 years) and the adult group (29 cases, age ${\geq}18$ years). Results: The study group consisted of 17 males and 26 females with a median age of 34 years. In the children's group (14 cases), 5 subjects were male and 9 were female, with a mean age of $6.7{\pm}6.2$ years. Most of the subjects were asymptomatic and were incidentally diagnosed with horseshoe kidney during their evaluation for another disease. Among the associated diseases in the children's group, Turner syndrome was the most common (5 cases), whereas ureteropelvic junction (UPJ) stricture was observed in 2 cases (14.2%). None of the children exhibited abnormal renal function during the follow-up period. In the adult group (29 cases), 12 subjects were male and 17 were female, with a mean age of 48 years. Eighteen patients were incidentally diagnosed with horseshoe kidney during their evaluation for another disease, and 11 patients had hematuria or abdominal pain due to renal stones. Among the associated diseases in the adult group, Turner syndrome was the most common (5 cases), and UPJ stricture was observed in 5 cases; the other accompanying diseases included hydronephrosis and overactive bladder. Six patients exhibited decreased renal function (serum creatinine level >1.5) during the follow-up period. Conclusion: Horseshoe kidney is usually diagnosed incidentally in both children and adults. In the present study, we noted that Turner syndrome was the most common associated disease in children. In addition, most children were asymptomatic but had a high risk of urologic complications after the transition to adulthood. Therefore, children with horseshoe kidney require continuous follow-up.

• Childhood Kidney Diseases
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• v.19 no.1
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• pp.23-30
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• 2015

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.1-8
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• 2007

Many children with microscopic hematuria have been found on school screening examinations. There are not, however, nation-wide criteria for us(specifically, pediatric nephrologists) how to take care of them. Recently, quite a few research papers concerning microalbuminuria with microscopic hematuria, which can predict the renal pathological findings, are published. Here I have reviewed articles on microalbuminuria which gives us the information how to manage microscopic hematuria.