• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.48-56
• /
• 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
• /
• v.3 no.1
• /
• pp.72-79
• /
• 1999

Renal scarring associated with vesico-ureteral reflux(VUR) is one of the major causes of end stage renal failure and renal hypertension in children. Urinary ${\beta}_2$-microglobulin(MG) has been suggested as a potential marker for presence of renal tubular damage. This study was designed to evaluate the significance of random urine ${\beta}_2$-MG as a predictor of presence of vesico-ureteral reflux in children with urinary tract infection(UTI). 57 children with urinary tract infection were studied. Patients were devided into two groups; 35($78.9\%$) children have UTI without VUR and 12($21.1\%$) children have UTI and VUR. Beta2-MG and creatinine in random urine sample was measured to decide the excretion ratio(${\beta}_2$-MG/creatinine). Among the 57 children with UTI, 44 children were confirmed by urine culture study and 13 children suspected by compatible clinical feature. Random urine ${\beta}_2$-MG of VUR group ($2.2{\pm}5.91$ mg/L) were significantly higher than that of simple UTI group($0.19{\pm}0.16mg/L$)(P=0.03). The ${\beta}_2$-MG/creatinine ratio of VUR group($32.41{\pm}25.7$) were significantly higher than that of simple UTI group($3.93{\pm}3.44$)(P=0.007). In conclusion, random urine ${\beta}_2$-MG and excretion ratio deserved early predictor of presence of VUR in children with UTI. And this method was more simple and inexpensive than the method of measuring ${\beta}_2$-MG with 24 hour urine collection, so might be a useful screening test for VUR in children with UTI.

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

• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.120-122
• /
• 2002

A 9-year-old boy of B blood group with end-stage renal disease due to IgA nephropathy received group O kidney transplantation from his father On day 9, he developed intravascular hemolysis, and anti-B autoantibody formation was confirmed. We diagnosed as immune hemolytic anemia due to passenger lymphocyte from donor, and cyclosporine withdrawl was done. Anemia resolved spontaneously, but on day 18, graft dysfunction developed, and graft biopsy revealed acute allograft rejection. Although hemolysis due to autoantibody is very rare and often mild, and the role of hemoglobinuria on acute rejection in this case is not certain, we recommend consideration of aggressive management on severe hemolysis after minor mismatched kidney transplantation. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 120-2)

• Tuberculosis and Respiratory Diseases
• /
• v.65 no.3
• /
• pp.225-229
• /
• 2008

Inferior vena cava filters are increasingly being used as an alternative to anticoagulation therapy for the prevention of pulmonary embolism. However, using an Inferior vena cava filter may result in clinically significant complications. Phlegmasia cerulea dolens is a rare disease that presents with acute complete venous occlusion due to extensive thrombosis in the lower extremity. It is characterized by intense pain, edema, decreased pulses and a cyanotic extremity. We report here on a case of phlegmasia cerulea dolens that was accompanied with disseminated intravascular coagulation (DIC) as a complication of the placement of an inferior vena cava filter in a patient who had been previously diagnosed with pulmonary embolism, and the patient had recently developed a cerebral hemorrhage due to a traffic accident.

• Korean Journal of Clinical Pharmacy
• /
• v.15 no.2
• /
• pp.165-172
• /
• 2005

엑세나타이드는 2005년 4월에 미국 FDA로부터 허가된 새로운 계열의 당뇨병치료제로서 적응증은 멧포르민이나 설포닐유레아계열의 당뇨병치료제로서 치료를 받고 있음에도 불구하고 혈당이 목표치로 저하되지 않는 제2형 당뇨병환자에게 기존의 치료법에 부가적으로 사용하는 것으로 제한되어 있다. 엑세나타이드는 39개의 아미노산으로 구성되어 있으며 미국 캘리포니아주에 자생하는 도마뱀의 타액에서 유래된 물질과 조성과 기능이 유사하도록 합성된 펩타이드 약물이다. 이 약물은 혈중포도당의 농도에 의존적으로 인슐린분비를 촉진하며, 비정상적으로 높은 혈중 글루카곤농도를 저하시키며, 음식물의 위통과시간을 연장하며, 식욕을 저하시키는 등의 여러 가지 기전을 통하여 혈당을 조절하는 것으로 알려져 있다. 멧포르민으로 1일 1500 mg을 사용하고 있는데도 불구하고 당화혈색소가 7%를 초과하는 제2형 당뇨병환자 336명을 대상으로 부가적으로 30주간 엑세나타이드 $5{\mu}g$또는 $10{\mu}g$을 1일 2회 피하주사 한 임상시험결과에 의하면, 당화혈색소가 7% 미만인 환자의 비율은 intent-to-treat 로서 각각 27%와 40%로 나타났다. 이는 기존의 치료법과 위약으로 치료받은 군에서의 13%에 비하여 통계적으로 매우 유의성 있는 결과인 것으로 분석되었다(p<0.01). 또 다른 임상시험에서는 상기 임상시험과 유사한 임상시험계획을 바탕으로 하여 설포닐유레아로 치료받고 있었지만 당화혈색소가 7%를 초과하는 제2형 당뇨병환자를 대상으로 임상시험을 실시하였으며, 그 결과에 의하면 엑세나타이드와 설포닐유레아의 병용치료 시 혈당조절에 매우 유리한 것으로 나타났다. 멧포르민과 설포닐유레아의 병용요법으로 치료받고 있던 당뇨병환자를 대상으로 실시한 임상시험에서도 동일한 결과가 나타났다. 이 약의 부작용은 치료개시 후 나타나는 메스꺼움이 문제로 지적되었으며 저 혈당현상은 큰 문제가 되지 않는 것으로 나타났다. 이 약은 인슐린 대용약물이 될 수 없으며 당뇨병성 케토산증의 치료에 사용할 수 없다. 또한 이 약물은 심한 신부전이 있거나 말기신장질환 환자에게 사용해서는 안 된다.

• Childhood Kidney Diseases
• /
• v.12 no.2
• /
• pp.239-244
• /
• 2008

Calcification in patients with endstage renal disease on renal replacement therapy can occur in extraskeletal area such as conjunctiva and cornea. Conjunctival and corneal calcification(CCC) has mostly has been reported in adults with endstage renal disease on hemodialysis. CCC seems to be associated with the duration of renal replacement therapy, and high Ca$\times$P value. We report a 10-year-old girl who was on peritoneal dialysis for 31 months and presented with CCC on both eyes. Her corneal calcification was resolved after the epithelial debridement and ethylenediaminetetraacetic acid(EDTA) soaking therapy.

• Journal of the Korea Convergence Society
• /
• v.11 no.9
• /
• pp.395-404
• /
• 2020

This was a descriptive study to investigate the effect of social support and health literacy on sick role behaviors in hemodialysis patients. A total of 149 outpatients receiving regular hemodialysis at four dialysis centers located in J city were surveyed. Data were analyzed using descriptive statistics, t-test, one-way ANOVA, Pearson correlation coefficient, and multiple linear regression. Factors including social support (β=.37, p<.001), health literacy (β=.16, p=.035), and religion (others) (β=-.25, p=.001) had an effect on sick role behavior. The explanatory power of model in this study was 22.0%. Therefore, the improvement of sick role behavior in patients undergoing hemodialysis requires specific strategies and interventions that are effective in increasing the social support and health literacy.

• Childhood Kidney Diseases
• /
• v.8 no.2
• /
• pp.176-185
• /
• 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
• /
• v.7 no.1
• /
• pp.23-29
• /
• 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.