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Clinical Comparison of $Henoch-Sch\"{o}nlein$ Purpura Nephritis in Children and Adults (소아와 성인 $Henoch-Sch\"{o}nlein$ Purpura(HSP) 신염의 임상적 비교)

  • Kim Ki-Eun;Shin Youn-Ho;Shin Jae-Il;Park Jee-Min;Jeong Hyeon-Joo;Lee Jae-Seung
    • Childhood Kidney Diseases
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    • v.7 no.2
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    • pp.157-165
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    • 2003
  • Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

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A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children (소아에서 일차성 막성 신병증의 임상-병리학적 고찰)

  • Lee Bum-Hee;Cho Hee-Yeon;Kang Ju-Hyung;Kang Hee-Gyung;Ha Il-Soo;Cheong Hae-Il;Lee Hyun-Soon;Choi Yong
    • Childhood Kidney Diseases
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    • v.7 no.2
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    • pp.133-141
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    • 2003
  • Purpose : Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. Methods : Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed aster 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. Results : The median onset age was 13.4 years. Clinical manifestations were nephrotic syn-drome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. Conclusion : With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done.

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Extracorporeal Membrane Oxygenation for Coronavirus Disease 2019: Expert Recommendations from The Korean Society for Thoracic and Cardiovascular Surgery

  • Jeong, In Seok;Kim, Woong-Han;Baek, Jong Hyun;Choi, Chang-Hyu;Choi, Chang Woo;Chung, Euy Suk;Jang, Jae Seok;Jang, Woo Sung;Jung, Hanna;Jung, Jae-Seung;Kang, Pil Je;Kim, Dong Jung;Kim, Do Wan;Kim, Hyoung Soo;Kim, Jae Bum;Kim, Woo-Shik;Kim, Young Sam;Kwak, Jae Gun;Lee, Haeyoung;Lee, Seok In;Lim, Jae Woong;Oh, Se Jin;Oh, Tak-Hyuck;Park, Chun Soo;Ryu, Kyoung Min;Shim, Man-Shik;Son, Joohyung;Son, Kuk Hui;Song, Seunghwan;The Korean Society for Thoracic and Cardiovascular Surgery COVID-19 ECMO Task Force Team
    • Journal of Chest Surgery
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    • v.54 no.1
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    • pp.2-8
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    • 2021
  • Since the first reported case of coronavirus disease 2019 (COVID-19) in December 2019, the numbers of confirmed cases and deaths have continued to increase exponentially despite multi-factorial efforts. Although various attempts have been made to improve the level of evidence for extracorporeal membrane oxygenation (ECMO) treatment over the past 10 years, most experts still hesitate to take an active position on whether to apply ECMO in COVID-19 patients. Several ECMO management guidelines have been published recently, but they reflect some important differences from the Korean medical system and aspects of real-world medical practice in Korea. We aimed to find evidence on the efficacy of ECMO for COVID-19 patients by reviewing the published literature and to propose expert recommendations by analyzing the Korean COVID-19 ECMO registry data.

Discrepancy and Correlation in the renal length between Kidney CT and 99mTc-DMSA Renal scan (신장 CT 검사와 99mTc-DMSA Renal Scan 검사에서 측정한 신장 길이의 차이 및 상관분석)

  • Jung, Woo-Young;Shim, Dong-Oh;Lee, Dong-Hun;Choi, Jae-Min
    • The Korean Journal of Nuclear Medicine Technology
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    • v.25 no.1
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    • pp.15-20
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    • 2021
  • Purpose This article studies the relationship between the length of a kidney measured by two scanning methods: Kidney Computed Tomography (CT) and 99mTc-Dimercaptosuccinic acid (DMSA) renal scan. Kidney CT provides a better anatomic assessment, while 99mTc-DMSA renal scan is superior in the kidney function test. Materials and Methods From January to December of 2019, two hundred patients who had Kidney CT and Tc99m-DMSA renal scan were chosen for this study. SPSS17.0 was selected for statistical analysis. Results Due to the effect of the breathing and resolution of 99mTc-DMSA renal scan, it showed the kidney's relatively longer length than the length of Kidney CT. For the same kidney, the length comparison among different brands' Gamma cameras was negligible. The length difference within the same age group did not show a noticeable discrepancy. However, there was a length difference between the radio technologists. Kidney CT and 99mTc-DMSA renal scan indicated a strong positive correlation between the length of the left and right kidney. Conclusion It is necessary to establish a standardized measurement method for measuring kidney length using 99mTc-DMSA renal scan. The kidney's functional changes and length changes are indications of Kidney diseases. Especially, pediatric patients tend to use 99mTc-DMSA renal scan for assessing the kidney's shape and the function to avoid potential radiation exposure during the Kidney CT. Therefore, it is significant to provide not only the kidney's functional information but also an anatomic analysis, including the kidney's size, length, and location through the 99mTc-DMSA renal scan.

Clinical Analysis of Children with Transitory Minimal Change Nephrotic Syndrome (MCNS) to Focal Segmental Glomerulosclerosis (FSCS) (미세변화형 신증후군(MCNS)으로부터 국소성 분절성 사구체 경화증(FSGS)으로 이행된 환아의 임상양상)

  • Lee Ji Eun;Yook Jinwon;Lee Eui Seong;Kim Ji Hong;Kim Pyung-Kil;Chung Hyun Joo
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.17-24
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    • 2000
  • Purpose: MCNS is found in approximately $85\%$ of the idiopathic nephrotic syndrome in children and shows good prognosis with initial steroid therapy. However in FSGS, there is poor prognosis with initial therapy and shows higher rate of progression to chronic renal failure and relapse after kindney transplantation. We have experienced 8 patients who were diagnosed as MCNS on initial renal biopsy and then progressed to FSGS on follow-up biopsy. So we have investigated their clinical course and risk factors for transition of MCNS to FSGS. Methods: We conducted a retrospective study with a review of histopathologic findings and clinical manifestations of 296 cases of MCNS and FSGS that were diagnosed from January 1988 to May 1999. We classified them into 3 groups according to the histopathologic finding; MCNS, FSGS, MCNS progressed to FSGS in follow-up biopsy. Results: The number of children was 296 cases comprising 241 cases($81.4\%$) showing MCNS, 8 cases($2.7\%$) transition group, 47 cases($15.9\%$) FSGS. The mean onset age was $6.0{\pm}2.6$years in MCNS, transition group $8.3{\pm}2.3$years, FSGS $7.2{\pm4.3$years, and the gender (M:F) ratio was 3.7:1 in MCNS, 3:1 in transition group, 1.8:1 in FSGS. Comparing the presence of initial hematuria, hypertension,24 hour urine protein, serum albumin, serum creatinine, there were significant difference between the transition group and the FSGS group in the following points; 24hour urine protein $684:342mg/m^2/hr$(P<0.05), serum albumin 1.92: 2.47g/dL(P<0.05), serum cholesterol 494:343mg/dL(P<0.05). Refractoriness to steroid therapy was 13.3$\%$ in MCNS. $12.5\%$ in transition group, $29.6\%$ in FSGS; significantly higher in FSGS(P<0.05). Immunosuppressant therapy was performed in $58.5\%$ of MCNS, $100\%$ in transition group, $80.8\%$ in FSGS; transition group showed significantly higher .ate(P<0.05) comparing with MCNS. Mean number of relapse and duration from onset to first relapse showed no significance difference between these groups. Conclusion: 249 patients with MCNS have been followed and $3.2\%$ (8 patients) of them has shown change in pathologic diagnosis from MCNS to FSCS. The risk factor for transition could not be found. Our results point to the need for a follow-up biopsy to certify the possibility of transition to FSCS in some MCNS cases with refractory cases to steroid therepy, frequent relapsing cases, or in case of no remission in spite of vigorous immunosuppressant therapy.

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The Significance of Hyperlipidemia as a Predictive Factor of Relapse in Corticosensitive Nephrotic Syndrome (스테로이드에 반응을 보인 신증후군 환아에서 재발 예측인자로서 고지혈증의 중요성)

  • Jung, Soon-Pil;Hong, Soon-Cheul;Lim, Seong-Joon;Lim, In-Seok;Choi, Eung-Sang
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.136-146
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    • 2001
  • Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)

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Comparison of Acute Clinical Features and Coronary Involvement in Patients with Kawasaki Disease between Those Younger and Older than One Year of Age (1세 미만과 1세 이상의 가와사끼병 환아에서 급성기 임상양상 및 관상동맥 변화에 대한 비교)

  • Kim, So Young;Lim, Seong Joon;Yun, Sin Weon;Lee, Dong Keun;Choi, Eung Sang
    • Clinical and Experimental Pediatrics
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    • v.45 no.6
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    • pp.773-782
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    • 2002
  • Purpose : To identify the necessity of more reasonable diagnostic criteria and the possibility of early prediction of coronary involvement in the higher risk group, we investigated and compared clinical and laboratory findings in the acute phase and coronary involvements in those younger (n=17) and older(n=53) than one year of age in Kawasaki disease(KD). Methods : Retrospective chart reviews were performed on 70 patients with KD who were admitted to the Chung-Ang University Hospital from April 1997 to May 2001. Results : Male were significantly higher in the younger age group(M : F ratio 3.3 : 1 vs. 1.0 : 1, P=0.004). Fever durations before intravenous immunoglobulin(IVIG) and echocardiography were significantly shorter in the younger group($4.6{\pm}1.3$ vs. $6.2{\pm}2.5$, P=0.004 vs. 0.01, respectively). Cases meeting typical diagnostic criteria were significantly less in the younger group(P=0.006). In the laboratory findings, serum albumin, BUN and $K^+$ levels in the acute febrile phase were significantly higher in the younger group(P=0.002, 0.006, <0.001, respectively) and incidences of coronary artery dilatation in the acute phase were significantly higher in the younger group(P=0.01). Conclusion : Although less met the typical diagnostic criteria of KD, infants younger than one year of age are more susceptible to coronary artery change in the acute febrile phase. Therefore, KD should be entertained as a diagnostic possibility in young infants with prolonged fever without distinct fever focus, and echocardiography should be considered as part of the evaluation of these patients, and then early diagnosis and prompt IVIG should be conducted.

Peripheral Neutrophil Count and Respiratory Failure in Preterm Infant (조산아에서 말초혈액 중성구수와 호흡 부전증과의 연관성)

  • Lee, Kum Joo;Yun, Soo Young;Lee, Ran;Hean, Jae Ho;Jung, Ghee Young;Park, Jin Hee;Park, Young Sun
    • Clinical and Experimental Pediatrics
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    • v.45 no.5
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    • pp.596-602
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    • 2002
  • Purpose : The purpose of this study was to analyze the association of peripheral neutrophil count with the development of respiratory failure in preterm infants. Methods : A retrospective study was conducted from January 1993 to December 1999 on 44 preterm infants, who were admitted to the neonatal intensive care unit of St. Francisco hospital. Preterm infants(birth weight 500 to 1,350 gm) who had a complete blood count obtained within 2 hours after delivery. Patients in the lowest of neutrophil count(early neutropenia, < $1.0{\times}10^9/L$) were compared with patients in the remaining group. Results : Low neutrophil count were transient in early neutropenia group. The concentration the circulating neutrophil count rose from $0.85{\pm}0.11{\times}10^9/L$ at average of 2 hours after delivery to $5.3{\pm}2.7{\times}10^9/L$ at 24 hours after delivery in the early neutropenia group and from $3.6{\pm}1.6{\times}10^9/L$ to $5.8{\pm}3.2{\times}10^9/L$ in the non-neutropenia group during the same time period. Compare to the non-neutropenia group, the neutropenia group had a lower birth weight($1,046.50{\pm}180.76gm$ Vs $1,156.70{\pm}124.99gm$), a lower Apgar score(1 min : $3.41{\pm}1.18$ Vs $4.30{\pm}1.46$, 5 min : $5.41{\pm}0.87$ Vs $6.15{\pm}0.95$), and a higher incidence of bronchopulmonary dysplasia(27.27% Vs 7.0%). Patients who had early neutropenia were more likely to require mechanical ventilation, supplemental oxygen and hospital stay. Also, main effect factors for the two groups were birth weight(Odds ratio=5.457, 95% CI=1.551-27.525), initial peripheral blood white cells(odds ratio=8.308, 95% CI=2.054-52.699), and bronchopulmonary dysplasia(odds ratio=0.099, 95% CI=0.017-0.397). Conclusion : A low count of neutrophil in the systemic circulation of premature infants within 2 hours of birth is associated with more severe respiratory distress.

Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨에 관한 고찰)

  • Lee, Jung-Mi;Park, Woo-Saeng;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.25-32
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    • 2000
  • Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

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Clinical Characteristics of Childhood Henoch-Sch$\"{o}$nlein Purpura with Duodenal Involvement by Upper Gastrointestinal Endoscopy (내시경상 십이지장을 침범한 Henoch-Sch$\"{o}$nlein Purpura 환아의 임상적 특징)

  • Park, Sun-Hee;Nam, Yoo-Nee;Park, Sang-Hui;Sim, So-Yeon;Eun, Byung-Wook;Choi, Deok-Young;Sun, Yong-Han;Cho, Kang-Ho;Ryoo, Eell;Son, Dong-Woo;Jeon, In-Sang;Tchah, Hann
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.12 no.2
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    • pp.156-162
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    • 2009
  • Purpose: The aim of this study was to investigate the clinical usefulness of upper gastrointestinal (GI) endoscopy in children with Henoch-Sch$\"{o}$nlein purpura (HSP). Methods: We retrospectively analyzed the clinical, endoscopic, and histopathologic records of children with HSP who had been admitted to the Department of Pediatrics of Gil Hospital and underwent upper GI endoscopy between January 2002 and June 2009. Patients were classified into the following two groups for statistical analysis: duodenal involvement (+) and duodenal involvement (-). Results: Fifty-one children with HSP underwent upper GI endoscopy; the mean age was 7.2${\pm}$2.9 years. The upper GI endoscopy showed abnormalities of the duodenum in 38 cases (74.5%), 22 of which had duodenal ulcers. Among the biopsy specimens obtained from the duodenum of 37 cases, 13 cases (35.1%) had leukocytoclastic vasculitis, neutrophil debri, and/or extravasation of RBCs. Steroid use was more frequent in the duodenal involvement (+) group (86.8%) than the duodenal involvement (-) group (53.8%; p=0.02). The mean length of hospitalization was 13.9${\pm}$8.43 days in the duodenal involvement (+) group and 8.1${\pm}$4.62 days in the duodenal involvement (-) group (p=0.003). The recurrence rate was significantly higher in the duodenal involvement (-) group than the duodenal involvement (+) group (p=0.027), whereas none of the other study parameters, such as the age of onset, renal involvement, and steroid use, led to significantly higher or lower recurrence rates. Conclusion: These results suggest that duodenal involvement can influence the clinical course and prognosis of HSP in children.

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