Initial steroid regimen in idiopathic nephrotic syndrome can be shortened based on duration to first remission

백희선,박기수,강희경,고철우,조민현 대한소아청소년과학회 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.6

Purpose: The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to remission (early responders [ER] vs. late responders [LR]) for the first idiopathic NS episode in children. Methods: Patients were divided into 4 subgroups (ST+ER, ST+LR, LT+ER, and LT+LR) according to the initial steroid regimen used and rapidity of response; the baseline characteristics, relapse rates, and cumulative percentage of children with sustained remission were then compared among the 4 subgroups. Results: Fifty-four children received ST, and the remaining 45 children received LT. As observed in pre- vious studies, children receiving LT showed significantly lower relapse rates during the first year after the first NS episode than those receiving ST. The ST+ER group showed significantly lower relapse rates during the first one year and two years after the first NS episode than the the ST+LR group, whereas there were no significant differences of the relapse rates and duration to the first relapse between the ST+ER and LT+ER groups. Conclusion: We suggest that the initial steroid regimen in idiopathic NS patients can be shortened according to the duration to remission i.e., LT in patients achieving remission after the first week of steroid therapy, and ST in those achieving remission within the first week of steroid therapy.

위 및 소장에 미치는 Capsaicinoid의 영향

백희선 漢陽大學校 韓國生活科學硏究所 1985 韓國 生活 科學 硏究 Vol.- No.3

Red pepper is one of the world-wide used spices. Especially the Korean (2.5~25 mg/day) of capsaicin which is the pungent essence of red pepper is. In this paper, I investigated the effect of red pepper and capsaicin on rat digestive tract to conform the physiological effect of excess intake of red pepper and capsaicin on a living orgnisms. As a result of sequential experiments, it was found that mucosa of epithelial cells in the small intestine were reduced by excess intake of red pepper powder and digestive movements were inhibited by capsaicin at stomach and small intestine. Finally, these phenomena were indentified by Scanning Electron Microscopic observations.

하악편측치아의 결손에 따른 악관절의 조직반응에 대한 연구

백희선,김영수,Paik, Hyee-Seon,Kim, Yung-Soo 대한치과보철학회 1991 대한치과보철학회지 Vol.29 No.2

The human temporomandibular joint as a ginglymoarthrodial one has much in common with the other synovial joints of the body, but it does possess an unique charachteristic in that it must accomodate the various occlusal relations of dentition during an end point of closure. For that reason, the movement of the condyle in the temporomandibular joint is susceptible to influences from the nature of occlusion. Undue loading to the temporomandibular joint can be applied on the occasion of premature contacts in centric relation, balancing side interferences, change of occlusal surfaces due to excessive attrition, loss of tooth. Such occlusal disharmonies in association with the systemic and emotional factors may give rise to the temporomandibular disorder. On the other hand, the changes of occlusal patterns in the growing body can also have an effect on the growth of the temporomandibular joint through the alterations of functional stresses. The purpose of this study was to observe histopathologic response of the temporomandibular joint in unilateral chewing on one side exclusively for 10 months. Three dogs showing normal masticatory function were chosen. One dog aged about 12 months was for control, two dogs for experimental specimens were about 12 and 18 months old respectively. For chewing on the left side only, unilateral lower right premolar and molar were extracted in two experimental specimens. And then three dogs were sacrificed 10 months later. Frontal histologic sectioning of joints were done for the observation of the effects of one-side chewing. 24 specimens from three dogs were obtained and fixed in 10% formalin and routinly processed with H-E staining for histologic examination. The light microscopic findings were interpretated as follows: 1. Experimental specimen 1 aged about 22 months: In comparison with control and right non-chewing side, the proliferative and hypertrophic zone were increased at the mesial and lateral part of left chewing side condyle. There was no change of the articular tissue of temporal bone. 2. Experimental specimen 2 aged about 28 months: The articular tissues of adult joint were observed. The differences between the chewing and non-chewing side were not seen in the articular tissues of condyle and temporal bone.

Pathogenetic Overlapping of Renovascular Hypertension Developed in a Child with Takayasu Arteritis: ‘One-clip, One and Half-kidney Model’

이수정,백희선,장혜민,김형기,허승,조민현 대한소아신장학회 2016 Childhood kidney diseases Vol.20 No.1

There are two pathogenic models of renovascular hypertension (RVH) originating from renal artery stenosis. We noted a case of a boy who had severe hypertension with atrophic left kidney, hypertrophic right kidney, a segmental stricture of the abdominal aorta, and total occlusion of the right renal artery. Due to the atrophic change of the contralateral, unclipped left kidney, this patient presented with various clinical manifestations related to both models of pathogenesis of RVH oc curring at the same time. We conclude that this patient presented with the middle stage of the two RVH pathogenetic models, so called the ‘one-clip, one and halfkidney model.’

Effect of donor–recipient size mismatch on long-term graft survival in pediatric kidney transplantation: a multicenter cohort study

박민지,백희선,송지연,최나예,안요한,강희경,조민현 대한신장학회 2023 Kidney Research and Clinical Practice Vol.42 No.6

Background: Donor–recipient size mismatching is commonly occurs in pediatric kidney transplantation (KT). However, its effect on graft survival remains unknown. This study aimed to determine the effect of donor–recipient size mismatch on the long-term survival rate of transplant kidneys in pediatric KT. Methods: A total of 241 pediatric patients who received KT were enrolled. The medical records of all patients were retrospectively reviewed, and the correlation between donor–recipient size mismatch and graft function and long-term graft outcome was analyzed according to donor–recipient size mismatch. Results: Recipients and donors’ mean body weight at the time of KT were 34.31 ± 16.85 and 56.53 ± 16.73 kg, respectively. The mean follow-up duration was 96.49 ± 52.98 months. A significant positive correlation was observed between donor–recipient body weight ratio (DRBWR) or donor–recipient body surface area ratio (DRBSR) and graft function until 1 year after KT. However, this correlation could not be confirmed at the last follow-up. The results of long-term survival analysis using Fine and Gray’s subdistribution hazard model showed no significant difference of the survival rate of the transplant kidney according to DRBWR or DRBSR. Conclusion: Donor–recipient size mismatch in pediatric KT is not an important factor in determining the long-term prognosis of transplant kidneys.

Renal involvement in children and adolescents with inflammatory bowel disease

장혜민,백희선,김정은,김주영,이연희,조희연,최연호,Ben Kang,최병호,최봉석,조민현 대한소아청소년과학회 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.10

Purpose: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. Methods: We retrospectively reviewed the medical records of 456 children and adolescents aged <20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. Results: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. Conclusion: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.

Paradigm Shift to Spinal Magnetic Resonance Imaging the Diagnosis of Guillain-Barre Syndrome in Children

김정은,백희선,안지영,황수경,김용선,서혜은,권순학 대한소아신경학회 2015 대한소아신경학회지 Vol.23 No.3

Purpose: This study was aimed to evaluate the clinical value of gadolinium-enhanced spinal magnetic resonance imaging (MRI) in the diagnosis of Guillain-Barre syndrome (GBS) by comparing it with cerebrospinal fluid (CSF) and nerve conduction studies (NCS) in children. Method: A single center, retrospective analysis of clinical investigations undertaken in children with GBS over a 5-year period was performed. The patients’ respective medical records, including spinal MRIs and nerve conduction studies, were reviewed. Results: A total of seventeen children (mean age 5.3±3.6 years; males, 12, females, 5) were enrolled in the study. Twelve out of 17 children (71%) showed gadolinium nerve root enhancement, mostly anterior along with posterior roots (10/12, 83%) at 4.1 days of illness, compared to CSF (2/11, 11%) at 2.5 days and NCS (11/17, 64%) at 7.4 days of illness (P<0.05). In addition, it appeared the more severe symptoms showed more positive findings; however, the difference was statistically insignificant (P>0.05). Conclusion: In conclusion, NCS are a standard diagnostic tool for GBS. This study supports the gadolinium-enhanced spinal MRI as a valuable investigating technique in the early diagnosis of GBS, although it is not necessarily superior. However, further studies are needed to elucidate the mechanisms and to strengthen the results.

Experience with Pediatric Kidney Transplantation, 1985-2016: A Single Regional Center Study

민소윤,조태경,백희선,박숙현,김형기,허승,조민현 대한소아신장학회 2017 Childhood kidney diseases Vol.21 No.2

Purpose: Kidney transplantation (KT) is an ideal treatment for pediatric patients with end-stage renal disease (ESRD). We report the clinical outcomes of pediatric ESRD patients who underwent KT in a single regional center. Methods: We retrospectively investigated the medical records of 60 pediatric patients who were diagnosed with ESRD and underwent KT in our hospital between January 1985 and June 2016. Results: A total of 60 children and adolescents (40 male, 20 female; mean age, 13.86±4.26 years) were included in this study. Six patients (10.0%) underwent KT immediately after receiving the diagnosis of ESRD, while the others underwent KT after dialysis treatment (mean period of dialysis, 368.7±4,41.8 days). The mean donor age (50 living-related [83.3%], 10 deceased [16.7%]) was 40.0±12.85 years and the male:female ratio was 1.07:1. The most common cause of ESRD was chronic glomerulonephritis. The overall survival rates at 1, 3, and 5 years after KT were 98%, 98%, and 96%, respectively, while the graft survival rates at 1, 3, and 5 years were 93%, 86%, and 68%, respectively. Children who underwent KT before 10 years of age had better monthly growth rates than those who underwent KT later than 10 years of age. Conclusions: KT is performed less frequently in children than in adults, but causes of ESRD vary and clinical outcomes after KT greatly affect the growth and development of pediatric patients. Therefore, further analysis and monitoring of clinical progression after KT in pediatric ESRD patients are necessary.

Clinical Characteristics of Febrile UTI First Developed Over 5 Years of Age

노다은,서효림,민소윤,조태경,백희선,조민현 대한소아신장학회 2017 Childhood kidney diseases Vol.21 No.1

Purpose: Febrile urinary tract infection (UTI) is one of the commonest bacterial infections in children. The purpose of this study is to investigate the clinical characteristics of the first episode of febrile UTI occurring in children over 5 years compared to those in infants younger than a year. Methods: We retrospectively reviewed the medical records of 10 patients over 5 years, having febrile UTI, and 25 controls under 1 year. Clinical characteristics including symptoms at admission, the time interval between symptom onset and hospital visit and/or diagnosis, duration of fever, urinalysis, and other laboratory and imaging test results were compared between the two groups. Results: Most patients in the control group showed only high fever at the time of presentation to the hospital. However, 60% of the case group had fever along with gastrointestinal (GI) symptoms such as abdominal and flank pain, vomiting, as well as relatively mild pyuria. The case group showed a longer duration between symptom onset and hospital visit and/or diagnosis. Conclusions: Delay in diagnosis and initiation of treatment of UTI increases the risk of permanent renal scarring and associated complications. Therefore, early diagnosis and treatment of febrile UTI is vital for very young infants, as well as children considering that febrile UTI could be an important cause of febrile illness in children over 5 years.

소아 길랑-바레 증후군 진단에서 척추 MRI로의 패러다임 변화

김정은(Jung Eun, Kim),백희선(Heesun Baek),안지영(Ji Young Ahn),황수경(Su Kyeong Hwang),김용선(Yong Sun Kim),서혜은(Hyeeun Seo),권순학(Soonhak Kwon) 대한소아신경학회 2015 대한소아신경학회지 Vol.23 No.3

목적: 길랑-바레 증후군은 소아의 급성 이완성 마비의 가장 흔한 원인 가운데 하나이다. 길랑-바레 증후군은 신경전도 검사와 뇌척수 액 검사로 진단하는데 다른 원인과의 감별을 위해 조영증강 척추 자기공명영상을 시행하기도 한다. 길랑-바레 증후군에서는 자기공명영 상에서 신경근의 조영증강 소견을 보여 자기공명영상 검사가 진단에 도움을 주기도 한다. 따라서 본 연구는 소아의 길랑-바레 증후군의 진단에 있어 뇌척수액검사와 신경전도검사를 이용한 진단과 비교하여 자기공명영상 검사의 임상적 유용성을 평가하고자 하였다. 방법: 2009년 6월부터 2014년 6월까지 길랑-바레 증후군으로 경북대학교병원에 입원한 17명의 환아를 대상으로 후향적 연구를 시행하였다. 면역글로불린의 치료 전에 뇌척수액검사를 시행하였으며 급성기동안 신경전도검사를 시행하였다. 모든 환아에서 조영증강 척추 자기공명영상 검사를 시행하였다. 결과: 운동증상 발생 후 뇌척수액검사는 증상 발생 후 평균 3일에 시행하였으며 2명(11%)만이 단백세포해리를 보였으며 신경전도검사는 증상 발생 후 평균 7일에 시행하였으며 12명(71%)에서 양성을 나타냈다. 조영증강 척추 자기공명영상 검사는 증상 발생 후 평균 4일에 시행하였으며 12명(71%)의 환아가 신경근의 조영증강을 보였다. 길랑-바레 증후군에서는 전근에서 조영증강이 특이소견인데 이 환아 들은 전근의 조영증강 우세를 동반하여 전근과 후근 모두에서 조영증강 소견을 보였다. 뇌척수액검사와 신경전도검사와 비교할 때 조영증강 자기공명영상 검사는 뇌척수액검사에 비해 더 높은 양성률을 보여 길랑-바레 증후군의 진단에 있어 더 유용한 방법임을 보여주었으며 ( P =0.0013) 증상 정도에 따라 척수 전근에서 조영증강의 차이를 보였 으나 통계학적인 의미는 없었다( P =1.0). 검사의 양성확률 또한 운동 증상의 심한 정도와 관계가 있었으나 통계적으로 유의한 차이는 보이지 않았다( P =0.29). 결론: 본 연구에서 길랑-바레 증후군의 진단시에 뇌척수액검사와 비교하여 조영증강 척추 자기공명영상 검사가 양성 확률이 더 높으며 자기공명영상에서 조영증강을 나타낸 경우 증상의 심한 정도에 따라 조영의 차이를 보였으나 통계학적인 의미는 없었다. 따라서 길랑-바레 증후군의 조기진단에 있어 조영증강 척추 자기공명영상 검사는 유용한 검사방법이 될 수 있을 것이다. Purpose: This study was aimed to evaluate the clinical value of gadolinium-enhanced spinal magnetic resonance imaging (MRI) in the diagnosis of Guillain-Barré syndrome (GBS) by comparing it with cerebrospinal fluid (CSF) and nerve conduction studies (NCS) in children. Method: A single center, retrospective analysis of clinical investigations undertaken in children with GBS over a 5-year period was performed. The patients’ respective medical records, including spinal MRIs and nerve conduction studies, were reviewed. Results: A total of seventeen children (mean age 5.3±3.6 years; males, 12, females, 5) were enrolled in the study. Twelve out of 17 children (71%) showed gadolinium nerve root enhancement, mostly anterior along with posterior roots (10/12, 83%) at 4.1 days of illness, compared to CSF (2/11, 11%) at 2.5 days and NCS (11/17, 64%) at 7.4 days of illness (P0.05). Conclusion: In conclusion, NCS are a standard diagnostic tool for GBS. This study supports the gadolinium-enhanced spinal MRI as a valuable investigating technique in the early diagnosis of GBS, although it is not necessarily superior. However, further studies are needed to elucidate the mechanisms and to strengthen the results.