• Childhood Kidney Diseases
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• v.2 no.1
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• pp.73-76
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• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Pediatric Infection and Vaccine
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• v.8 no.1
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• pp.114-117
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• 2001

We experienced a case of streptococcal toxic shock-like sundrome in a 12 year old boy. Symptoms such as fever, sore throat, diffuse erythematous rashes on whole body developed 3 days before admmision. His symptoms rapidly aggravated to develop hypotension, hepatic and renal dysfunction and thrombocytopenia. After admission, intravenous fluid and antibiotic therapy were done and he was succesfully treated. Attention should be paid to recognize and diagnose this fatal disese. We report this case with review of related literatures.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.159-166
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• 2005

Purpose : Recently the merits of 6 weeks of initial prednisolone treatment for pediatric primary nephrotic syndrome have been reported, and the use of the 6 week regimen is increasing. We compared our experiences with the 6 week treatment versus the 4 week treatment for Korean patients. Methods : We conducted a retrospective analysis of 69 children who had primary nephrotic syndrome and who were followed up for at least 12 months in the 4 major medical centers in Daegu. The remission rate, the relapse rate, the frequency of relapse and complication of steroid treatment were compared between the 4 weeks and 6 weeks treatment group. Results : Of the 69 children, 42 were in the 4 week treatment group and 27 were in the 6 week group. The median age, blood pressure, serum total protein, serum albumin, cholesterol, creatinine, estimated creatinine clearance, 24 hour urine protein and 12 month cumulative dose did not differ between the two groups. Among the children who relapsed after steroid treatment, the relapse time was significantly later for the 6 week treatment group. The relapse rate after 1 year of treatment was 62$\%$ in the 4 week treatment group and 52$\%$ in the 6 week treatment group; however, there was no statistically significant difference between the two groups. The frequency of relapse at 12 months was $1.5{\pm}1.2$ times in the 4 week treatment group and $1.1{\pm}1.2$ times in the 6 week treatment group, and there was not different between the two groups. The most common side effects of steroid treatment were an increase of appetite and a cushingoid appearance, and there was no statistical difference between the two groups. Among the 27 children who had kidney biopsies performed, 21 suffered from minimal change nephrotic syndrome. Conclusion : The first relapse time after steroid treatment was significantly later in the 6 week steroid treatment group. The frequency of relapse and the 12 month cumulative dose of steroid were lower in the 6 week treatment group, but there was no statistical significance between the two groups. The side effects of steroid treatment did not differ between the two groups. We need to study the long term side effects and the advanced regimens of steroid treatment in the future.(J Korea Soc Pediatr Nephrol 2005;9:159-166)

• 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.2
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• pp.240-246
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• 2002

Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha($TNF-{\alpha}$) is related to intrarenal or serum concentration of $TNF-{\alpha}$. So, we studied the relationship between the concentration of $TNF-{\alpha}$ and aggravation of glomerular damage in the Henoch-$Sch{\ddot{o}}nlein$ nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of 21 patients with Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) and 22 patients with INS visited Chungbuk National University hospital from March 1998 to March 2001. The concentration of $TNF-{\alpha}$ in the sera and urines were measured by sandwich ELISA. Results : Serum $TNF-{\alpha}$ levels in the HSP patients with renal involvement were significantly higher than those without renal involvement(P=0.009). But urine $TNF-{\alpha}$ levels have no correlation with renal involvement(P=0.088). In the HSN patients, proteinuria have a significant correlation with serum $TNF-{\alpha}$ levels(P=0.004) but less correlation with urine $TNF-{\alpha}$ levels(P=0.053). Otherwise, proteinuria have no correlation with serum $TNF-{\alpha}$ levels(P=0.763) but have a significant correlation with urine $TNF-{\alpha}$ levels(P=0.007) in INS. Conclusion : These result suggest that the serum concentration of $TNF-{\alpha}$ would be important to glomerular involvement in HSP. And, it is interesting that proteinuria shows a significant relation with serum $TNF-{\alpha}$ levels in the HSN, but with urine $TNF-{\alpha}$ levels in the INS. This means the major production of $TNF-{\alpha}$ may be originated by extrarenal inflammation in the HSN and by intrarenal tubulo-interstitial damage due to proteinuria in the INS.

• Journal of the Korean Arthroscopy Society
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• v.6 no.2
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• pp.195-199
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• 2002

This article is to report a new technique for reconstruction of the anteromedial and posterolateral bundles of anterior cruciate ligament by separate tensioning and fixation of the each bundle. Method : Tibial and femoral tunnels were made with conventional technique of anterior cruciate ligament reconstruction. Tibial tunnel was enlarged $5\~7$ mm in anterior-posterior direction to make oval it in cross section. When preparing the Achilles tendon allograft, bone plug portion was trimmed as the conventional technique. The tendinous portion was trimmed as two separate bundles by dividing the tendinous portion longitudinally, so the graft is shaped like 'Y'. The bone plug portion of allograft was inserted into the femoral tunnel and fixed with absorbable cross pins. Two ligamentous portionss of the distal part of the grafts were tensioned separately at the external orifice. Anteromedial bundle was fastened under maximum tension with the knee flexed 90 degrees by post-tie method. The posterolateral bundle was fixed by the same technique with the knee in full extension. Then, an absorbable interference screw was inserted between the two bundles upto the upper end of the tibial tunnel, to get more initial rigidity of the reconstructed graft as well as to locate the two bundles in more anatomic position.

• 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)

• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1067-1072
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• 2006

Purpose : The purpose of this study was to examine the clinical courses and long-term outcomes of children with Allagille syndrome in Korea, and to evaluate the prognostic potentials of identified variables. Methods : We reviewed the clinical manifestations and outcomes of 30 children with Alagille syndrome, investigated from 1984 to 2006 until the end of this study (defined as death or last visit; mean follow-up : 5 years). Results : Cholestasis occurred in 100 percent, cardiovascular abnormalities in 83.3 percent, butterfly vertebrae in 30.0 percent, posterior embryotoxon in 43.3 percent, and a characteristic facial appearance in 100 percent. At study conclusion, of these 30 patients, eight had died (26.7 percent); six related to Alagille syndrome. Five patients died of a liver disease complication. Liver transplantation was carried out in five of the 30 patients (16.7 percent) and one of these died due to hyperacute rejection. At age two, cholestasis improved in 17 of the 30 patients. Those who had severe cholestasis at 2 years of age tended to have a complication, such as liver cirrhosis or liver transplantation, or to have died. Conclusion : Hepatic complications account for the most mortalities in patients with Alagille syndrome. Careful and complete assessments should be made in children who have cholestasis at 2 years of age. Further investigations of more cases are required.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.266-271
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• 2002

There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome Is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.57-62
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• 2004

Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.