• Childhood Kidney Diseases
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• v.12 no.2
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• pp.250-255
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• 2008

Diabetes mellitus(DM) is a metabolic syndrome caused by deficiency of insulin secretion and a consequence of insulin resistance. Poor glycemic control is a common finding in children with Type 1 DM(T1DM). Approximately 60% of the young patients with T1DM develop abnormalities in the eyes and 15-20% in the kidney. Diabetic nephropathy (DN) is a serious metabolic complication of T1DM that leads to renal failure. Some clinical studies report that the duration of prepubertal diabetes may contribute less to the development of microvascular complications than pubertal and postpubertal duration. There have been few cases of DN in prepubertal patients with T1DM in Korea. Thus we report a case of a 12-year-old female with T1DM who had poor glycemic control and was diagnosed as DN in a prepubertal period. It was proven by renal biopsy after microscopic hematuria and proteinuria were detected through the mass school urinary screening program.

• 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.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.40-47
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• 2000

Purpose: Renal involvement is the most important prognostic factor of HSP. Therefore, the pathogenesis and prognostic factors in renal involvement have been studied by many researchers. The aim of this study was to evaluate the clinical risk factors and the role of TNF-$\alpha$ in renal involvement of HS purpura. Methods: The subjects of this study were 12 patients of HS purpura, 7 patients of HS nephritis, and 5 age-matched controls. We have analysed the rist factors for renal involvement in clinical symptoms and collected the sera and urines of all subjects in acute and convalescent stage. The concentration of TNF-$\alpha$ in the collected sera and urines were measured by sandwich ELISA and compared with that of age-matched controls. Results: Statistical analysis showed that persistent purpura increased the risk of developing renal involvement (P=0.0018). and serum TNF-$\alpha$ levels in the acute stage of patients with renal involvement($11.45{\pm}7.01$ pg/ml) were significantly higher than those of without renal involvement($6.32{\pm}1.31$pg/m1) and of age-matched controls($5.99{\pm}1.34$pg/m1)(P=0.012, 0.027, respectively). However, urine TNF-$\alpha$ levels have no correlation with renal involvement. On investigation of serum TNF-$\alpha$ levels in acute stage of HS purpura, persistent purpura had a significantly higher increase(P=0.038). Conclusion: Serum concentration of TNF-$\alpha$ is a risk factor and has a predictable value along with clinical risk factors, such as, persistent purpura for renal involvement in HS purpura. Also, the effectiveness of the specific treatment fur antagonizing TNF-$\alpha$ in HS nephritis may need further study.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.194-201
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• 2003

Malperfusion of major organs which frequently accompanies acute aortic dissection is one of the major causes of death. Malperfusion does not only develop before surgery, but also during or after surgery in various manifestations according to the aortic branches involved. Expeditious diagnostic and therapeutic measures based on high degree of clinical suspicion are mandatory for successful treatment. The authors report four cases of acute aortic dissection accompanied by malperfusion of various organs that were successfully treated.

• Journal of Chest Surgery
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• v.37 no.7
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• pp.613-616
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• 2004

Systemic lupus erythemotosus (SLE) is an autoimmune disorder with dermal, renal, and cardiac manifestations. It frequently has cardiovascular complications such as pericarditis, myocarditis, and valvular heart diseases. Valvular heart diseases in SLE comes mainly in the form of mitral or aortic insufficiencies. Report of aortic stenosis is extremely rare. Surgical treatments of valvular heart disease in SLE are not done frequently because of complications in other organs. Aortic stenosis developed in a 59 year-old woman with SLE, and aortic valve replacement was done successfully.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.256-261
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• 2008

Rhabdomyolysis is a syndrome involving the breakdown of skeletal muscle causing myoglobin and other intracellular proteins and electrolytes to leak into the circulation. There are various causes of acute rhabdomyolysis in childhood, such as direct trauma to muscle, muscle necrosis from ischemia, inflammation in muscle, or exposure to drugs and toxins. The most-important complication of this disorder is acute renal failure (ARF). However, the contributing factors to the development of ARF in children with rhabdomyolysis remain obscure. We report two cases of rhabdomyolysis after excessive exercise.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.178-181
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• 2007

Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

• Tuberculosis and Respiratory Diseases
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• v.65 no.2
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• pp.116-120
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• 2008

Rifampin is one of the first line drugs for treating tuberculosis, but it might be associated with serious adverse effects, including renal failure. We report here on a case of a 57-year-old patient who developed Henoch-$Sh{\ddot{o}}nlein$ purpura during antituberculosis therapy that included rifampin. The patient converted to negative on the AFB smear for tuberculosis two weeks after the initial administration of antituberculosis medication. After treatment for 60 days, this patient was diagnosed with Henoch-$Sh{\ddot{o}}nlein$ purpura by the purpura lesion on the lower legs, the leukocytoclastic vasculitis, the renal impairment and the pathological examination. After stopping rifampin, the skin lesions disappeared in about 10 days and his renal function gradually improved. This case study showed that Henoch-$Sh{\ddot{o}}nlein$ purpura can be caused by rifampin during antituberculosis therapy and we recommend that the use of rifampin should be restrained when clinical symptoms of Henoch-$Sh{\ddot{o}}nlein$ purpura are observed.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.408-413
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• 2010

Purpose : We aimed to prove the relative limitation of $^{99m}Tc-DMSA$ scintigraphy (DMSA) compared to computed tomography (CT) in diagnosing acute pyelonephritis (APN) in children. Methods : Since September 2006, after a 64-channel CT was imported, 10 DMSA false-negative patients have been identified: these patients underwent a CT scan for acute abdomen or acute febrile symptoms and were diagnosed as having APN; however, their DMSA scans were clear. We focused on these 10 DMSA false-negative patients and analyzed their clinical findings and CT results. We used Philips Brilliance Power 64-channel CT scanner for the CT scan and Siemens Orbitor Nuclear Camera 60 Hz for the DMSA scan. Results : The 10 DMSA false-negative patients were mostly males (80%) and infants (80%). They had fever for a mean of 1.1-day duration before admission and showed increase in acute reactants: leukocyte, erythrocyte sedimentation rate, and C-reactive protein. The CT findings of renal lesions were focal in 6 (60%) cases and diffuse in 4 (40%) cases, and most of the lesions were unilateral in 80% of patients. CT proved that 22 renal lesions were neglected by DMSA. Differential renal function test by DMSA was also of no use in the evaluation of renal lesions. Conclusion : In this study, DMSA scan showed limitation in finding renal cortical lesions of CT-proven APN patients. DMSA false-negative results seem to occur at early-phase disease of infantile age, but more prospective studies are needed to determine the reasons and their prevalence.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.184-190
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• 2011

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Sch$\ddot{o}$nlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.