• Title/Summary/Keyword: 신 질환

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Congenital Anomaly of Urinary Tract in Children (소아 선천성 요로계 기형에 관한 고찰)

  • Shin Weon Hye;Ko Cheol Woo;Koo Ja Hoon;Chung Sung Kwang
    • Childhood Kidney Diseases
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    • v.3 no.1
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    • pp.88-94
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    • 1999
  • Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

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A study of Diagnostic Significance of Simultaneous Examination of Proteinuria and Hematuria in the Urinary Mass Screening (집단뇨검사(Urinary mass screening) 방법으로 단백뇨와 혈뇨의 동시검사가 가지는 진단적 가치에 대한 연구)

  • Kim, Young-Kyoun;Lee, Chong-Guk
    • Childhood Kidney Diseases
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    • v.3 no.1
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    • pp.57-63
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    • 1999
  • Purpose : To evaluate the diagnostic significance of simultaneous examination of hematuria and proteinuria in the urinary mass screening for early detection ot incipient renal diseases. Method and Object : During the period of 4 months from August to December in 1997, we did urinary mass screening on students of first grade of high school (16 years aged group) nationwide together with Korean Association of Health(KAH). In the first screening test, Comber-10 $N^{(R)}$ M dipsticks were used to detect proteinuria, hematuria, pyuria and nitrite simultaneously. Total 26,508 students (16 years aged group) from 33 high schools in every province in Korea, respectively, complied to the urinary mass screening. After then, one high school in Seoul was selected to reveal the true incidence of incipient renal diseases among students who showed hematuria in the initial screening through intensive examinations. Those who had hematuria and/or proteinuria visited the Paik hospital, and underwent blood tests and ultrasonographic examinations. The results were evaluated. Results 1) The initial screening revealed that the prevalence of proteinuria, hematuria, pyuria and positive nitrite urine, were $0.73\%,\;2.69\%,\;0.23\%\;and\;0.03\%$, respectively. 2) The first urinary screening among 875 students from one high school in Seoul selected fir the second test showed proteinuria, hematuria, pyuria and positive nitrite urine, were $0.91\%,\;4.68\%,\;0.34\%\;and\;0\%$, respectively. a) Total 8 among 875 students showed proteinuria, but one of them had orthostatic proteinuria and those remaining 7 students had transient proteinuria. b) There were 41 students who had hematuria in the initial screening. Among 33 who complied the second test, only one student showed asymptomatic isolated hematuria and those remaining students were normal. Conclusion : 1) Because of high false positive hematuria rate in the urinary mass screening, it dosen't seem to be appropriate that hematuria screening using dipsticks be included in the urinary mass screening. 2) A unified organization is needed from the fret that so various results of urinary mass screening came out. 3) Positive rates of pyuria and nitrite were so low that validity of urinary mass screening for urinary tract infection needs more studies.

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Chronic Dialysis in Infants and Children Under 2 Years of Age (2세 미만 만성 신부전 환아에서의 만성 투석)

  • Sohn, Young-Bae;Nam, Sook-Hyun;Kwak, Min-Jung;Kim, Su-Jin;Jin, Dong-Kyu;Paik, Kyung-Hoon
    • Childhood Kidney Diseases
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    • v.11 no.1
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    • pp.41-50
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    • 2007
  • Purpose : Dialysis in children with chronic renal failure presents with many difficulies. The purpose of this study is to find an improved method in chronic dialysis in infants and children less than 2 years of age by analyzing the experience with 10 cases. Methods : A retrospective review of the medical records of 10 patients(6 boys and 4 girls) was conducted. The patients had chronic renal failure and underwent chronic dialysis at Samsung medical center from March 1999 to February 2007. Results : At Initiation of dialysis, the median age was 3 months old(22 days-20 months), the median body weight was 3.75 kg(2.2-10.3 kg), and the median serum creatinine level was 4.3 mg/dL(2.0-11.4 mg/dL). The median duration of dialysis was 29.5 months(3-62 months). Dysplastic kidney disease was the most common underlying renal disease. Two patients were treated with hemodialysis, 4 patients with peritoneal dialysis, and 4 patients eventually switched dialysis modality. Nine of the 10 patients took erythropoietin and anti-hypertensive drugs. At the end of the follow up period, 1 patient received kidney transplantation, 2 patients died due to sepsis, and 5 patients were treated with peritoneal dialysis. Two patients were lost to follow up. The most common complication of dialysis was infection. Achieving vascular access and maintaining proper catheter function were the most important factors in treating patients with hemodialysis. The growth status of patients was aggravated after 6 month of dialysis but improved after 1 year of dialysis. Patients showed better growth on peritoneal dialysis than hemodialysis. Conclusion : Chronic dialysis can be performed successfully in infants and children under 2 years of age. Vascular access was the main limitation of hemodialysis, and infection was the common problem in both hemodialysis and peritoneal dialysis. To improve the patients survival rate and quality of life, major efforts should be directed toward the prevention of infection and preservation of catheter function. (J Korean Soc Pediatr Nephrol 2007;11:41-50)

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Arthroscopic treatment of septic arthritis of the knee in adults (성인의 화농성 슬관절염의 관절경적 치료)

  • Kyung Hee-Soo;Ihn Joo-Chul;Oh Chang-Wug;Kim Sung-Jung;Kim Joon-Woo
    • Journal of the Korean Arthroscopy Society
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    • v.6 no.1
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    • pp.21-24
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    • 2002
  • Purpose : The purpose was to assess the result of arthroscopic management of the septic arthritis on the knee in compromised patients. Materials and Methods : Fourteen patients with septic knee were analyzed. The mean age was 55 years and the mean follow-up period was 14.6 months. Underlying diseases included 4 cases of diabetes, and history of direct acupuncture in 4 cases. Clinical stage of septic arthritis was judged by $G\ddot{a}chter's$ classification, which was determined by arthroscopic findings. After arthroscopic irrigation and debridement, we observed the results of laboratory data and improvement of clinical findings. Results : Causative organism was identified in 7 cases and no organism was detected in the remaining 7 cases. Stage I was 1, stage II 8, stage III 4, and stage IV 1, respectively. Eleven of 14 cases were improved by one stage operation. Two cases of stage III were recurred and additional arthroscopic management was done. In 1 case of stage IV, symptom was not improved and needed arthrotomy. The result was unsatisfactory in patients with stage III and IV. Serum erythrocyte sedimentation rate and C-reactive protein were normalized after 29.3 and 20.8 days following the operation, respectively. Clinical symptoms disappeared average 2 days following the operation. Conclusion : Arthroscopic management of acute septic arthritis of the knee would be an effective and satisfactory treatment modality in that its postoperative pain and complications are minimal, and it can be done with ease repeatedly.

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A case of Hyper-IgE syndrome with a mutation of the STAT3 gene (STAT3유전자 돌연변이 검사로 확진된 고면역글로불린E 증후군 1례)

  • Kang, Ji-Man;Suh, Jung-Min;Kim, Ji-Hyun;Kim, Hee-Jin;Kim, Yae-Jean;Lee, Hun-Seok;Shin, Young-Kee;Ahn, Kang-Mo;Lee, Sang-Il
    • Clinical and Experimental Pediatrics
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    • v.53 no.4
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    • pp.592-597
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    • 2010
  • Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.

Urinary Tract Infection in Febrile Infants with Pyuria (발열과 농뇨가 있는 영아에서 요로감염에 관한 연구)

  • Lee, Sue Young;Cho, Sung Hee;Kim, Sun Mi;Jeong, Dae Chul;Chung, Seung Yeon;Lee, Kyung Yil;Kang, Jin Han
    • Pediatric Infection and Vaccine
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    • v.11 no.1
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    • pp.90-100
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    • 2004
  • Objective : Urinary tract infection(UTI) is a frequent serious bacterial infection in young infants. The clinical presentation may be non-specific and variable, depends on factors such as the age and the level of infection. Children with renal involvement may be at risk of permanent renal damage. Experimental studies have shown that renal lesions caused by acute febrile UTI may be prevented or diminished by early diagnosis and treatment. Therefore, it is important to find a method that can permit early diagnosis and identification of patients who are at risk for progressive renal damage. We designed this study to identify related factors in culture positive UTI infants, and also to identify related factors in culture negative UTI infants, who are febrile with pyuria, by using renal imaging and functional studies including renal sonography, DMSA scan and VCUG. Methods : Retrospectively analyzed the medical records of 136 febrile infants with pyuria over 2 years(from January 2001 to February 2003). Urine culture was done in all cases, and regardless of urine culture findings, renal imaging study was done if symptomatic UTI suspected. Results : Total 57 organisms were isolated in 53 patients. E. coli was the most common organism(86%), followed by E. faecalis, M. morganii, Proteus species, P. aeruginosa, S. aureus and E. fergusonii. Most of the isolates had high sensitivity on cephalosporins or amikacin and had low sensitivities on aminopenicillins. Abnormal acute phase DMSA scan or VCUG findings were seen in both urine culture positive and negative group without statistical differences(P>0.05). In febrile infants with pyuria, fever over 48 hours, older age and high CRP related to abnormal acute phase DMSA scan findings regardless urine culture results. Conclusion : 1st or 3rd generation cephalosporins with amikacin could be the first choice of treatment for UTI. Febrile infants with positive urine culture dose mean urinary tract infection but not acute pyelonephritis which directly relates to cortical damage which could be confirmed by acute phase DMSA scan. Even cases with negative urine culture findings, acute pyelonephritis should be concerned in febrile infants with pyuria who are older than 3 months of age, has fever over 48 hours or high CRP level. And in such cases, acute phase DMSA scan and VCUG should be evaluated for early treatment and long term prognosis.

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Clinicopathological Analysis of Glomerulonephritis with Asymptomatic Urinary Abnormalities in Children (무증상성 요이상을 동반한 사구체신염 환아의 임상 및 병리학적 분석)

  • Sung Ick-Ho;Yoon Hye-Kyoung;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.136-143
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    • 1997
  • Purpose : To evaluate the prevalence and clinical manifestations of various glomerulonephritis(GN) in children with asymptomatic urinary abnormalities, a clinicopathological analysis of 134 biopsied cases which were subdivided into 3 groups of proteinuria with hematuria, isolated hematuria and isolated proteinuria was done. Methods : We conducted retrospective study with review of histopathologic findings and clinical manifestations of the 134 cases with asymptomatic urinary abnormalities diagnosed by percutaneous renal biopsy which were done between January 1986 and December 1996 at department of pediatrics, Pusan Paik hospital. Results : 1) The proportion of children with asymptomatic urinary abnormalities was 43.2% of all biosied cases. 2) Among these, primary GN were 95 cases and secondary GN were 39 cases, it's ratio was 2.44:1. As a whole, the most common pathologic diagnosis was IgA nephropathy(IgAN, 26.9%), which was followed by $Henoch-Sch\"{o}nlein$ purpura nephritis(HSPN, 17.9%), minimal change lesion(MC, 17.2%), thin GBM disease(12.7%), Hepatitis B associated glomerulonephritis(HBGN, 6.0%), poststreptococcal glomerulonephritis(PSAGN, 3.0%), mesangial proliferative glomerulonephritis(MesPGN, 2.2%), membranoproliferative glomerulonephritis (MPGN, 2.2%), Alport syndrome (1.5%) and Fibrillary nephritis(0.7%). 3) In proteinuria with hematuria, the most common pathologic diagnosis was IgAN(34.6%), which was followed by HSPN(19%), MC(17.7%), thin GBM disease(8.9%), HBGN(6.3%), PSAGN(3.6%), MesPGN(1.2%), MPGN(1.2%) and Alport syndrome(1.2%). 4) Major causes of isolated hematuria were thin GBM disease(19.6%), IgAN(17.6%), HSPN(17.6%), MC(11.8%). 5) Isolated proteinuria was due to of 3 cases of MC and 1 case of HBGN. Conclusion : The prevalence of glomerulonephritis with asymptomatic urinary abnormalities in children were 43.2% of all biopsed cases. When these children were subdivided into 3 groups, proteinuria with hematuria was accounted 58.9%(79 cases) and then isolated hematuria was 38.1%(51 cases), isolated proteinuria was only 3%(4 cases) respectively. The most common pathologic diagnosis was IgA nephropathy in patient with proteinuria and hematuria, and thin GBM disease in patient with isolated hematuria.

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Predicting the Progression of Chronic Renal Failure using Serum Creatinine factored for Height (소아 만성신부전의 진행 예측에 관한 연구)

  • Kim, Kyo-Sun;We, Harmon
    • Childhood Kidney Diseases
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    • v.4 no.2
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    • pp.144-153
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    • 2000
  • Purpose : Effects to predict tile progression of chronic renal failure (CRF) in children, using mathematical models based on transformations of serum creatinine (Scr) concentration, have failed. Error may be introduced by age-related variations in creatinine production rate. Height (Ht) is a reliable reference for creatinine production in children. Thus, Scr, factored for Ht, could provide a more accurate predictive model. We examined this hypothesis. Methods : The progression of of was detected in 63 children who proceeded to end-stage renal disease. Derivatives of Scr, including 1/Scr, log Scr & Ht/Scr, were defined fir the period Scr was between 2 and 5 mg/dl. Regression equation were used to predict the time, in months, to Scr > 10 mg/dl. The prediction error (PE) was defined as the predicted time minus actual time for each Scr transformation. Result : The PE for Ht/Scr was lower than the PE for either 1/Scr or log Scr (median: -0.01, -2.0 & +10.6 mos respectively; P<0.0001). For children with congenital renal diseases, the PE for Ht/Scr was also lower than for the other two transformations (median: -1.2, -3.2 & +8.2 mos respectively; P<0.0001). However, the PEs for children with glomerular diseases was not as clearly different (median: +0.9, +0.5 & +9.9 respectively). In children < 13 yrs, PE for Ht/Scr was tile lowest, while in older children, 1/Scr provided the lowest PE but not significantly different from that for Ht/Scr. The logarithmic transformation tended to predict a slower progression of CRF than actually occurred. Conclusion : Scr, floored for Ht, appears to be a useful model to predict the rate of progression of CRF, particularly in the prepubertal child with congenital renal disease.

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Validation of Onen's Alternative Grading System for Congenital Hydronephrosis (선천성 수신증을 위한 Onen 등급 분류법의 임상적인 타당성)

  • Woo, Da Eun;Lim, Myung Hee;Kim, Myung Uk;Kim, Sae Yoon;Park, Yong Hoon
    • Childhood Kidney Diseases
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    • v.18 no.2
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    • pp.77-84
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    • 2014
  • Purpose: The purpose of this study was to compare the validity of Onen's alternative grading system (AGS) with that of the APDRP and SFU grading systems in patients with isolated and complicated congenital hydronephrosis. Methods: We retrospectively reviewed the medical records of 153 patients (204 renal units) diagnosed with congenital hydronephrosis between January 2002 and December 2011. We classified patients into 2 groups; isolated or complicated hydronephrosis. All renal units were graded according to anterior-posterior diameter of renal pelvis (APDRP), Society for Fetus Urology (SFU) and Onen's grading systems. We analyzed the prognosis of hydronephrosis, according to each grading system, at 2 years of age. Results: There were 152 renal units with isolated hydronephrosis and 52 renal units with complicated hydronephrosis. The isolated hydronephrosis group had a lower grade according to Onen's AGS, and showed more frequent spontaneous remission by 2 years of age. There was more frequent obstruction (P =0.000) and surgical treatment (P =0.000) of units with high-grade hydronephrosis according to Onen's AGS. In the complicated hydronephrosis group the frequencies of spontaneous remission (P =0.015) and renal dysfunction (P =0.013) were significantly higher than those in high-grade hydronephrosis, as indicated by Onen's AGS. There were no significant differences in clinical outcomes among the highest grade groups, according to the 3 systems, in either isolated or complicated hydronephrosis. Conclusion: Onen's AGS reflects the prognosis of hydronephrosis as well as other grading systems in those with isolated hydronephrosis. It was better predictor of renal dysfunction in those with complicated hydronephrosis. However, Onen's AGS was not superior to the other grading systems in terms of predicting prognosis, especially in high-grade hydronephrosis.

Clinical Report of 46 Intracranial Tumors with LINAC Based Stereotactic Radiosurgery (선형가속기를 이용한 뇌종양 46예의 뇌정위다방향방사선치료 성적)

  • Yoon Sei C;Suh Tge S;Kim Sung W;Kang Ki M;Kim Yun S;Choi Byung O;Jang Hong S;Choi Kyo H;Kim Moon C;Shinn Kyung S
    • Radiation Oncology Journal
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    • v.11 no.2
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    • pp.241-247
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    • 1993
  • Between July 1988 and December 1992, we treated 45 patients who had deep seated inoperable or residual and/or recurrent intracranial tumors using LINAC based stereotactic radiosurgery at the Department of Therapeutic Radiology, Kangnam St. Mary's Hospital, Catholic University Medical College. Treated intracranial tumors included pituitary tumors (n=15), acoustic neurinomas (n=8), meningiomas (n=7), gliomas (n=6), craniopharyngiomas (n=4), pinealomas (n=3), hemangioblastomas (n=2), and solitary metastatic tumor from lung cancer (n=1). The dimension of treatment field varied from 0.23 to 42.88 $cm^3\;(mean;\;7.26\;cm^3)$. The maximum tumor doses ranging from 5 to 35.5 Gy (mean; 29.9 Gy) were given, and depended on patients' age, target volume, location of lesion and previous history of irradiation. There were 22 male and 23 female patients. The age was varied from 5 to 74 years of age (a median age; 43 years). The mean duration of follow-up was 35 months (2~55 months). To date, 18 $(39.1\%)$ of 46 intracranial tumors treated with SRS showed absent or decrease of the tumor by serial follow-up CT and/or MRI and 16 $(34.8\%)$ were stationary, e.g. growth arrest. From the view point of the clinical aspects, 34 $(73.9\%)$ of 46 tumors were considered improved status, that is, alive with no evidence of active tumor and 8 $(17.4\%)$ of them were stable, alive with disease but no deterioration as compared with before SRS. Although there showed slight increase of the tumor in size according to follow-up imagings of 4 cases (pituitary tumor 1, acoustic neurinomas 2, pinealoma 1), they still represented clinically stable status. Clinically, two $(4.4\%)$ Patients who were anaplastic astrocytoma (n=1) and metastatic brain tumor (n=1) were worsened following SRS treatment. So far, no serious complications were found after treatment. The minor degree headache which could be relieved by steroid or analgesics and transient focal hair loss were observed in a few cases. There should be meticulous long term follow-up inall cases.

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