• 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)

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.109-116
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• 2001

Purpose Long- term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. Patients and Methods : We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was $9.1{\pm}1.4$ years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. Results : On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was $50\%$, which represents 8 children, while remaining 8 children representing $50\%$ of the cases showed no relapse during treatment. During the levamisole therapy period, tile average relapse rate was reduced significantly from $2.18{\pm}0.9/year\;to\;0.77{\pm}0.9/year$(P=0.027). Also the average relapse rate after the therapy was reduced to $1.34{\pm}1.1/year$, which was a significant level compared to the level before treatment(P=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of $12.2{\pm}9.1$ months before the use of levamisole, but it was also $10.1{\pm}6.9$ month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. Conclusion : The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment. (J. Korean Soc Pediatr Nephrol 2001;5 : 109-16)

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.189-196
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• 2009

Purpose : The pathophysiologic mechanism of nephrotic syndrome is not yet known clearly. At least in some cases, certain 'circulating factors' are thought to increase the glomerular protein permeability. Considering the systemic effect of the circulating factor on peritoneal membrane, we evaluated the loss of protein through peritoneal membrane in patients on peritoneal dialysis due to the end stage renal disease (ESRD) caused by steroid resistant nephrotic syndrome (SRNS). Methods : We retrospectively reviewed the medical records of 26 pediatric patients on peritoneal dialysis ensued during the period from 2001 to 2007 at our clinic. Twelve patients had SRNS, while 14 patients had ESRD caused by the congenital anomalies of urinary system. Results : While the other parameters including nPNA indicating the adequacy of protein intake were similar between the two groups, serum albumin was lower in SRNS patients than the non-SRNS patients ($3.7{\pm}0.3$ g/dL vs. $4.0{\pm}0.4$ g/dL, P=0.021). Peritoneal protein loss was higher in SRNS patients than in non-SRNS patients ($3,044.4{\pm}837.6\;mg/m^2$/day vs. $1,791.6{\pm}1,244.0\;mg/m^2$/day, P=0.007). The protein permeability of the peritoneal membrane measured by the ratio of total protein concentration in dialysate to plasma was twice as high in SRNS patients as the non-SRNS ($1.06{\pm}0.46%$ vs. $0.58{\pm}0.43%$, P=0.010). After 1 year, peritoneal protein loss increased in both patient groups, but to a significantly greater degree in non-SRNS patient (P=0.023). Conclusion : The results of our study support the notion that in nephrotic syndrome there are some 'circulating factors' with the systemic effect. Since the greater protein loss through peritoneal membrane in SRNS was confirmed in this study, more meticulous nutritional support and close monitoring on the nutrition are required in these patients.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.505-511
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• 2002

Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may predict the clinical course. Methods : From December 1995 to May 2001, 71 patients with ITP were admitted to Ajou University Hospital. They were treated with IVIg 400 mg/kg for five days and MPV, PDW, platelet count were analysed. Normal control group(n=38) was compared. The study group was divided into acute and chronic forms, and also divided into group A, good early responders whose platelets increased more than $100,000/{\mu}L$ within 5 days and group B who did not. Results : Mean value of MPV at diagnosis in ITP patients was lower than the normal control group(P<0.05). In group A, MPV was abruptly increased on the first day after IVIg and then started to decrease. But in group B, MPV was steadily increased until the fourth day after IVIg. In the normal control group, there were inverse correlations between platelet count and MPV(r=-0.415, P<0.05), but in ITP patients, there were positive relationships between platelet count and MPV(r=0.646, P<0.05) at diagnosis. Conclusions : MPV at diagnosis of ITP was lower than the normal control. MPV and PDW could not predict the course of ITP patients, but MPV could distinguish good early responders. More research is needed to find out the reasons of decreased MPV at diagnosis of ITP.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1253-1259
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• 2003

Backgroud : Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. Methods : A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. Results : The average age at onset of seizures was $6.1{\pm}4.6days$ and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. Conclusion : Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.

• The Journal of the Korean bone and joint tumor society
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• v.16 no.1
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• pp.21-26
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• 2010

Purpose: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). Materials and Methods: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and fi ve were female. The mean age was 34 years. The mean follow-up period was 54 months. Results: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. Conclusion: Comprehensive understanding of clinical, radiological and pathological features of secondary chondro sarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1118-1123
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• 2003

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with previous reports, placing particular emphasis on clinical information. Methods : We collected the clinical data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean or $mean{\pm}SD$ and statistical analysis was performed using Chi-square approximation. P<0.05 was considered as significant. Results : The patient population consisted of 87 boys and 38 girls ranging in age from one to 14 years. HSP occurs throughout the year, but this study shows seasonal skewing, with most patients presenting from fall through spring and a paucity of cases in summer. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities. Purpuric lesions were also scattered on the arms and occaisionally on the face and ears, but the trunk was largely spared. A recurrence of purpura was defined as the reappearance of a rash or other symptoms following resolution of disease for at least two weeks. The mean number of recurrences was 0.51. Eighty eight patients(70.4%), 18 patients(14.4%) and 67 patients(53.6%) complained of abdomianl pain, gastrointestinal bleeding and arthralgia, respectively. Nephritis occurred in 48(38.4%) patients. Fifteen boys (17.2%) developed epididymitis. Neurologic features occurred in 13(10.4%) and two(15%) of these were seizures. Conclusion : HSP all showing purpura as defined is characterized by various clinical features, including abdominal pain, arthralgia, epididymitis and nephritis which could occur before the appearance of purpura. Therefore, we suggest that the possibility of HSP should be considered in children before invasive procedures, even if the above symptoms and signs present without purpura.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.178-187
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• 2002

Purpose : The detection of hydronephrosis(HN) with antenatal ultrasonography was first reported in the 1970s. Prenatal HN is diagnosed with an incidence of 1:100 to 1:500 on antenatal screening. Recently, the purpose of antenatal screening has changed from simple detection to selection for specific diagnosis-based management. this study is to evaluate the usefulness of antenatal sonography for HN and to investigate the differential causes of HN and their clinical outcomes. Patients and methods : 11,783 live neonates with prenatal ultrasonographic examination at Ajou University School of Medicine, from Sep. 1994 to Aug. 2001 were analyzed. Results and conclusion : Hydronephrosis (>10 mm) was detected in 119 (1.0%) cases antenatally and among these, 91 were proved to have HN postnatally Males were three times more affected than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis (47%), followed by multicystic dysplastic kidney, vesicoureteral junction obstruction and vesicoureteral reflux. During 20 months' follow-up(3 to 72 months), 58(48%) renal units showed spontaneous resolution and surgical interventions were necessary in 10 (7.4%) of postnatally confirmed hydronephrotic renal units.

• 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.11 no.1
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• pp.9-15
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• 2007

Purpose : Edema is one of the cardinal features of nephrotic syndrome. Although the pathogenesis of edema is not entirely understood, it is caused by hypovolemia or hypervolemia by different mechanisms. Accordingly it is important to evaluate the volume status of patients in order to treat the edema, but it is difficult to evaluate the patient's volume status only by clinical parameters. The quotient of urine sodium and potassium excretion $U_K/(U_{Na}+U_K)$ is introduced as a more useful way to evaluate volume status. In this study we will propose the usefulness of $U_K/(U_{Na}+U_K)$ in evaluating the volume status of children with nephrotic syndrome. Methods : Primary nephrotic syndrome patients at Yeungnam University Hospital since January 1995 to June 2005, were included in the study. We analyzed clinical parameters such as tachycardia, cardiomegaly, pleural effusion, blood chemistry and urinalysis prospectively. We defined hypovolemia when $U_K/(U_{Na}+U_K)$ exceeded 60%. Intravenous albumin and diuretics were administered to hypovolemic edematous patients. On the other hand, hypervolemic edematous patients were treated only with diuretics. Results : There were 50 cases of primary nephrotic syndrome patients(hypervolemia: 29 vs hypovolemia: 21). There were no significant differences in clinical symptoms and laboratory findings except for FeNa While $F_eNa$ and $U_K/(U_{Na}+U_K)$ had a significant negative correlation, BUN and $U_K/(U_{Na}+U_K)$ had a significant positive correlation. Urine output after edema treatment was effective and there were no treatment-related side effects in both groups. Conclusion : FeNa, BUN and $U_K/(U_{Na}+U_K)$ are a useful parameters for evaluating volume status of edematous nephrotic syndrome patients. We could suggest a therapeutic option for using albumin and/or diuretics according to volemic status by means of measured $U_K/(U_{Na}+UK)$.