• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.23-29
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• 2003

Purpose : This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. Patients and Methods : We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria($<40\;mg/m^2/day$) and had normal renal function and blood pressure at the time of renal biopsy. Based on the clinical and pathological findings at the time of diagnosis, we sought for complications of IgA nephropathy such as heavy proteinuria(${\ge}40\;mg/m^2/day$), hypertension, and chronic renal failure. Results : The mean age at presentation was $9.5{\pm}2.8$ years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26%), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of $7.0{\pm}3.5$ years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events : 4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed Impaired renal function. By univariate analysis using the chisquare test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. Conclusion : We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

• Journal of Nutrition and Health
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• v.46 no.2
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• pp.137-146
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• 2013

The purpose of this study was to evaluate diet-related Quality of Life (QOL) and to analyze the relationship among diet-related QOL, dietary regimen practice, health-related QOL, and gastrointestinal symptoms in hemodialysis patients. Subjects were recruited from an artificial kidney center in Seoul. The self-report questionnaire consisted of socio-demographic characteristics, diet-related QOL, compliance with dietary regimen practice, health-related QOL, and gastrointestinal symptoms was distributed. Diet-related QOL includes a 'Quality of Life and Related to Dietary Change Questionnaire', 'Satisfaction Survey', and 'Dietary Impact Survey'. According to their responses, participants had experienced the greatest difficulty with 'Dietary Impact'. In particular, the mean score for general health was low. Association of dietary regimen practice showed a positive association with 'Cost' and 'Self-care' score. In addition, diet-related QOL showed positive correlation with health-related QOL, particularly in 'taste'. Scores for 'Taste', 'Convenience', and 'Dietary Impact' were lower for subjects with constipation compared to those of subjects without constipation. Scores for 'Taste' and 'Dietary Impact' were lower in subjects with irritable bowel syndrome compared to those of subjects without the syndrome (p < 0.05). Diet-related QOL showed a positive association with health-related QOL (p < 0.05), whereas it showed a negative association with constipation (p < 0.01). In conclusion, hemodialysis patients in Korea suffer from burden of dietary regimen practice and most scores for diet-related QOL and health-related QOL from patients with gastrointestinal symptoms were low. Therefore, appropriate nutrition education considering gastrointestinal symptoms is necessary for improvement of patients' QOL during dietary regimen practice.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.10-21
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• 2010

The overall prognosis of Henoch-Schoenlein purpura (HSP) is favorable, but severe nephritis has a high risk of progression to end stage renal failure. Recent studies emphasize the importance of early treatment in children with severe HSP nephritis, but the treatment of severe HSP nephritis still remains controversial due to the rarity of randomized controlled studies in this field. Nevertheless, several intensive therapies, such as intravenous high-dose methylprednisolone pulse, immunosuppressive/cytotoxic drugs, fibrinolytic therapy, anticoagulants, antiplatelet agent and plasma exchange, have been used in children with severe HSP nephritis. In this review, we focus on the treatment of severe HSP nephritis in children.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.48-56
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• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.164-175
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• 2001

Purpose : Alport syndrome is a hereditary nephrotic disease characterized by progressive nephrotic symptom, sensorineural hearing loss, ophthalmic abnormality, typical microscopic findings, and familial occurrence. In this study, we tried to find the risk factors related with its prognosis by taking a close observation on clinical symptoms of children with Alport syndrome reviewing retrospectively. Materials & methods : We chose children diagnosed as Alport syndrome in renal biopsy during 20 years(from 1980, Jan. until 1999, Dec.) who could receive follow up studies in tile department of pediatrics. They were divided into two groups by comparing renal function at the time of diagnosis and at current status. We compared several clinical aspects in them, and applied nonparametric test for statistical analysis. Results : The sex ratio(male:female) of 24 children was 3:1. The most common clinical symptom presented at their first visit was gross hematuria. Among those 24 children, 11 cases($46\%$) of progressing into chronic renal failure(Group II) were observed. Hypertension, proteinuria and edema were seen much frequently in group II. The level of serum protein, albumin, and creatinine clearance were decreased while BUN, creatinine were relatively increased. All the results were statistically significant. Conclusion Clinically significant risk factors related to prognosis in Alport syndrome were the presence of hypertension, edema, and proteinuria at the time of diagnosis. Also, the level of serum protein, albumin, BUN, creatinine, and glomerular filtration rate were proved to be important factors in predicting prognosis. We believe that studies on these possible risk factors would be of great help in treating and predicting prognosis of children suffering with Alport syndrome. (J Korean Soc Pediatr Nephrol 2001;5 : 164-75)

• Journal of the Korean Society of Radiology
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• v.84 no.5
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• pp.1185-1190
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• 2023

Pseudoaneurysms are among the most serious complications of percutaneous balloon angioplasty. Although pseudoaneurysm rupture rarely happens, when it does, the result can be fatal; thus, early detection and management are crucial. In this report, we disclose the case of a 34-year-old male with end-stage renal disease who presented with a huge symptomatic pseudoaneurysm of the left popliteal artery, following percutaneous balloon angioplasty three months prior. The pseudoaneurysm was successfully excluded using interventional treatment. The patient recovered well, and the follow-up was uneventful, with excellent patency of the covered stent.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.75-83
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• 2009

Purpose : Chronic kidney disease (CKD) and obesity are the worldwide public health problem. Obesity is an already well-established risk factor for CKD. The objective of this study is to evaluate the relationship between high BMI and increased risk for nephropathy by clinical data. Methods : Study group were 26 patients who had $BMI{\geq}25\;kg/m^2$ and control group were 49 patients with BMI<$25\;kg/m^2$. Both groups received renal biopsy in Kyung Hee Medical Center between 2003. Jan.-2007. Dec. BMI was calculated from measured weight and height when they were admitted to the hospital. We collected laboratory data such as CBC and blood chemistry. Results : Our hypothesis was that overweight and obesity are associated with incidence and progression of CKD. From kidney biopsy, we found IgAN 17, MesPGN 5, HSPN 2, Intestitial nephritis 1, IgMN 1 (total 26) in the study group whereas IgAN 22, MesPGN 17, HSPN 3, MGN 3, benign hematuria 2, MPGN 1, Intestitial nephritis 1, (total 49) were found in the control group. There was no significant difference between the two groups. Overweight patients demonstrated significantly higher platelet, TG, ALT, and uric acid level compared to control group. Conclusion : We identified a significant relationship between overweight and development of CKD. These results suggest that overweight children have an increased risk for CKD than those who are not obese. So, we should pay attention to children with overweight who have CKD and earlier weight management is crucial to prevent aggravation of CKD.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.204-210
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• 2003

Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).

• Sleep Medicine and Psychophysiology
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• v.6 no.2
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• pp.126-132
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• 1999

Objectives: The purposes of this study were to investigate 1) the incidence of insomnia, 2) the clinical characteristics of the insomniacs, 3) the correlation of severity of insomnia with somatic complaints and psychological distresses, and 4) the beliefs and attitudes about sleep in patients with chronic renal failure on hemodialysis. Methods: The author evaluated 153 patients, receiving hemodialysis therapy at the four outpatients hemodialysis units in Pusan, Korea. The patients had completed a self-administered questionnaire package, which consisted of basic demographic findings, questions characterizing insomnia, Beck Depression Inventory(BDI), Spielburger's State-Trait Anxiety Inventory(STAI), and visual analogue scales measuring quantitatively the severity of the self-perceived psychological and somatic symptoms. And several laboratory data were collected. Diagnosis of insomnia was made in the base of insomnia criteria of DSM-IV and international classification of sleep disorders. Subjects were dichotomized into those who reported any characteristics of insomnia or those who had no insomnia during the preceding two weeks. Results: Insomnia was found in 100(65.4%) of 153 patients. No statistical differences were found between the patients with and without insomnia in terms of age, gender, education, marital status, mean duration of hemodialysis and all considered laboratory findings except serum albumin. The patients with insomnia had significantly higher BDI score and predialysis systolic blood pressure, and lower serum albumin as compared to non-insomnia group. Significant differences were found between two groups in terms of self-perceived distress such as sadness, anxiety, worry, pruritus, and dysfunction of daily life. The data showed statistically significant correlation between insomnia severity and some variables such as physical dysfunction, pruritus, bone pain, sadness, anxiety, worry, dysfunction of daily life and excessive daytime sleepiness. The patients with insomnia had significantly several dysfunctional beliefs and attitudes about sleep than those without insomnia. Conclusion: These results indicate that insomnia is very common in hemodialysis patients and likely contribute to the impaired quality of life experienced by many these patients. The author suggests that physical and psychological distresses would be reduced and the quality of life could be improved if their sleep disturbances are properly ameliorated in patients on hemodialysis.