• Title/Summary/Keyword: chronic diseases

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Hemolytic-uremic Syndyome Associated with Bloody Diarrhea (혈변을 동반한 용혈성 요독 증후군 5례)

  • Kim Jung-Sim;Park Eun-Jung;Chung So-Hee;Ko See-Hwan;Uhm Mee-Ryung;Park Moon-Su;Lee Heung-Jea;Jin Dong-Kyu
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.170-175
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    • 1997
  • Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

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Lipoprotein and Lipid Abnormalities in Uremic Children with Maintenance Dialysis (투석 치료중인 만성 신부전 소아에서의 지질 및 지질단백 이상)

  • Kim Jung-Sue;Song Jung-Han;Park Hye-Won;Cheong Hae-Il;Kim Jin-Q;Choi Yong;Ko Kwang-Wook
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.109-116
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    • 1997
  • Purpose : Chronic renal failure is often accompanied by severe dyslipidemia, a known risk factor for cardiovascular disease. Lipoprotein(a) [Lp(a)] has recently been characterized as a risk factor for atherosclerosis and thrombosis. Cardiovascular disease is the leading cause of death in adult patients on dialysis. However, there are only limited data available concerning risk factors for atherosclerosis in uremic children. We have measured serum levels of lipids, lipoproteins, apolipoproteins and Lp(a) in uremic children with maintenance dialysis. Methods : Ten uremic children with hemodialysis (HD) and 14 with peritoneal dialysis (PD) in our dialysis unit were included in this study. The mean age of HD patients was $162{\pm}59$ months and the male to female ratio was 7:3. The mean age and sex ratio of PD patients were $123{\pm}63$ months and 6:8, respectively. The levels of cholesterol, triglyceride, lipoproteins, apolipoproteins and Lp(a) were measured from serum sampled after 14 hours of fasting. The normal control levels were cited from 2 articles presenting the normal blood lipid and lipoprotein levels of primary school and middle school children in Korea. Results : There was no difference in age, sex ratio, body mass index and duration of dialysis between the HD and the PD group. The serum concentration of the cholesterol, triglyceride and apolipoprotein B were significantly elevated in dialysis patients compared with normal subjects. The serum level of Lp(a) was significantly elevated in only PD group. The serum Lp(a) level was below 30 mg/dl in 13 and above 30 mg/dl in 11 patients. The serum albumin level was significantly decreased in high Lp(a) group than in low Lp(a) group. Conclusion : The uremic children receiving dialysis reveal abnormal serum lipid and lipoprotein profiles. These results suggest that they have a higher risk for coronary heart disease, although there has been no clinical evidence of coronary heart disease at present. A long-term follow-up study of these children to clarify the suggestion should be started now.

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Clinical Characteristics and Prognostic Factors of Vesicoureteral Reflux (방광요관역류의 임상적 특징 및 예후인자)

  • Kim, Wun-Kon;Ha, Tae-Sun
    • Childhood Kidney Diseases
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    • v.18 no.1
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    • pp.29-35
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    • 2014
  • Introduction: Persistent vesicoureteral reflux (VUR), a major cause of urinary tract infection (UTI) in children, can result in serious renal complications, such as reflux nephropathy and chronic renal failure. We evaluated the clinical characteristics and prognostic factors of VUR. Methods: From December 1993 to May 2011, we examined 117 children with vesicoureteral reflux who were admitted to the Department of Pediatrics and Urology, Chungbuk National University hospital for a UTI. The patients were managed medically or surgically. Results: Male patients had a slightly higher prevalence of VUR than female patients (55%). The degrees of the 161 refluxing ureters, as classified by the International Reflux Study Committee, were as follows: grade I, 15 ureters; grade II, 32 ureters; grade III, 54 ureters; grade IV, 26 ureters; grade V, 34 ureters. One hundred and sixty-one renal units (115 cases) underwent a 99m TC-DMSA renal scan, and 62% showed abnormal findings. The incidence of renal cortical defects showed a direct correlation with the severity of VUR. Ninety-four refluxing ureters were followed up medically, and 66 ureters (67%) either disappeared or improved. However, 9 refluxing ureters persisted. The spontaneous resolution rate of VUR seemed to be higher in younger patients with lower grades of reflux, and without renal cortical defects. Sixty-seven refluxing ureters (41%) were treated surgically, 62 refluxing ureters (92%) disappeared, and 5 refluxing ureters (8%) persisted. Conclusion: The incidence of renal cortical defects in patients with UTIs was 62% (in a 99m TC-DMSA renal scan), and showed a direct correlation with the severity of VUR. The spontaneous resolution rate seemed to be lower in the patients with higher grades of VUR, older age (over 4 years old) and diffuse renal cortical defects.

Naringin Protects Ovalbumin-induced Asthma through the Down-regulation of MMP-9 Activity and GATA-3 Gene (Naringin에 의한 천식치료 효과연구)

  • Lee, Chang-Min;Chang, Jeong-Hyun;Jung, In-Duk;Jeong, Young-Il;Tae, Noh-Kyung;Park, Hee-Ju;Kim, Jong-Suk;Shin, Yong-Kyoo;Park, Sung-Nam;Park, Yeong-Min
    • Journal of Life Science
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    • v.19 no.6
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    • pp.735-743
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    • 2009
  • The common word flavonoids is often used to classify a family of natural compounds, highly abundant in all higher plants, that have received significant therapeutic interest in recent years. Naringin is associated with a reduced risk of heart disease, neurodegenerative disease, cancer and other chronic diseases; however the molecular basis of this effect remains to be elucidated. Thus we attempted to elucidate the anti-allergic effect of Naringin in ovalbumin (OVA)-induced asthma model mice. The OVA-induced mice showed allergic reactions in the airways. These included an increase in the number of eosinophils in bronchoalveolar lavage (BAL) fluid, an increase in inflammatory cell infiltration into the lung around blood vessels and airways, airway luminal narrowing, and the development of airway hyper-responsiveness (AHR). The administration of Naringin before the last airway OVA challenge resulted in a significant inhibition of all asthmatic reactions. Accordingly, this study may provide evidence that Naringin plays a critical role in the amelioration of the pathogenetic process of asthma in mice. These findings provide new insight into the immunopharmacological role of Naringin in terms of its effects on asthma in mice.

A Clinicopathologipal Study on the Prognosis of IgA Nephropathy in Children (소아 IgA 신병증의 예후와 관련한 임상병리학적 고찰)

  • Kwon Jae-Hun;Choi Eun-Na;Park Jee-Min;Jeung Hyeun-Joo;Lee Jae-Seung
    • Childhood Kidney Diseases
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    • v.7 no.1
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    • pp.23-29
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    • 2003
  • Purpose : This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. Patients and Methods : We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria($<40\;mg/m^2/day$) and had normal renal function and blood pressure at the time of renal biopsy. Based on the clinical and pathological findings at the time of diagnosis, we sought for complications of IgA nephropathy such as heavy proteinuria(${\ge}40\;mg/m^2/day$), hypertension, and chronic renal failure. Results : The mean age at presentation was $9.5{\pm}2.8$ years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26%), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of $7.0{\pm}3.5$ years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events : 4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed Impaired renal function. By univariate analysis using the chisquare test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. Conclusion : We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

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Angiotensinogen M235T Polymorphism in Children with $Henoch-Sch\"{o}nlein$ Purpura Nephritis ($Henoch-Sch\"{o}nlein$ Purpura 신염에서 Angiotensinogen M235T 유전자 다형성)

  • Ha Chang-Woo;Joo Hee-Jung;Park Ji-Kyoung;Chung Woo-Yeong
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.10-17
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    • 2004
  • Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) nephritis has a variable range of prevalence from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the angioten-sinogen(AGT) M235T polymorphism with the clinical manifestations, particularly proteinuria in children with HSP with or without nephritis. Methods : The AGT M235T polymorphism was determined in children with HSP nephritis (n=33) or HSP without nephritis(n=28) who had been diagnosed at Busan Paik hospital from January 1996 to June 2001. The M235T polymorphism of the AGT gene was determined by PCR amplification of the genomic DNA. Results : The M235T polymorphism of AGT gene frequency was MM 75%, MT : 25%, TT : 0% in HSP and MM : 64%, MT : 36%, TT : 0% in HSP nephritis, there was no significant differences in the genotype and allele frequencies between the two groups. No significant differences in clinical manifestations at onset and last follow-up were seen between the two genotypes. When statistical analysis was done according to the presence of the M allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>500 $mg/m^2/day$) at onset and at last follow-up were higher in the MT genotype than in those of in the MM genotype but these difference were not statistically significant. Conclusion : We suggest a lack of association between M235T polymorphism of the AGT gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on sufficient number of patients and long term follow up periods are necessary to confirm the role of M235T polymorphism of AGT gene in children with HSP nephritis.

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Peritoneal Dialysis Associated Peritonitis and Empirical Antibiotics Therapy in Korean Children with Chronic Renal Failure (소아 복막 투석 환자에서 발생한 복막염의 경험적 항생제 치료에 관한 연구)

  • Lee, Sang-Goo;Cho, Joong-Bum;Sohn, Young-Bae;Park, Sung-Won;Kim, Su-Jin;Jin, Dong-Kyu;Paik, Kyung-Hoon
    • Childhood Kidney Diseases
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    • v.12 no.2
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    • pp.213-220
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    • 2008
  • Purpose : This study aims to verify the effectiveness of initial empirical antibiotic choice recommended by the International Society for Peritoneal Dialysis(ISPD) guide among Korean children. Methods : We have collected data on peritonitis from January 2001 to December 2007 in Samsung Medical Center. Results : Of the 42 patients, 48 episodes of peritonitis had occurred in 21 patients. The rate of peritonitis was one episode over 35.3 patient-months. Mean dialysis duration before peritonitis was 18.06$\pm$15.81 months. Gram-positive organisms accounted for 58.3% of all episodes. Of the gram-positive organisms, the most common pathogen was Staphylococcus aureus(29.2%), the next common pathogens were Coagulase negative staphylococcus(14.6%) and Streptococcus species(6.3%). 35.7% of gram-positive pathogens were resistant to 1st cephalosporin. However, in patients younger than 4 years old, 50% of gram-positive pathogens were resistant to 1st generation cephalosporin. 10 episodes of peritonitis were methicillin-resistant and were treated by vancomycin. Of the gram-negative organisms, E. coli was the most common (8.3%). 64.8% of all pathogens were sensitive to cephalothin or ceftazidime. Conclusion : The empirical therapy with 1st generation cephalosporin and ceftazidime can be also effective to peritoneal dialysis associated peritonitis in Korean children. However, in patients younger than 4 years old, glycopeptide should be considered as the first empirical therapy in Korean children.

Clinicopathological Correlation of Intralobar Pulmonary Sequestration (내엽성 폐격리증의 임상적 특성과 조직병리학적 소견의 연관성)

  • Cho, Hyun-Min;Shin, Dong-Hwan;Kim, Kil-Dong;Lee, Sak;Chung, Kyung-Young
    • Journal of Chest Surgery
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    • v.36 no.5
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    • pp.356-362
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    • 2003
  • Background: Intralobar and extralobar pulmonary sequestrations have been considered as congenital lesions that occur at different stages of embryonic life. However, most cases of intralobar pulmonary sequestration (IPS) seem to have an acquired origin, as they are absent in infants and associated anomalies are relatively, uncommon among them. Material and Method: The cases of 25 patients who were diagnosed as IPS after surgical resection from December, 1985 to July, 2002 were included in this study. The medical records wire retrospectively reviewed and the clinical characteristics were age at operation; gender; symptoms at presentation; presence of congenital anomalies; combined diseases; preoperative studies and diagnosis; location of the lesion; method of surgical resection; origin, size and numbers of aberrant artery; histopathological findings; and postoperative complications. Result: There were 17 (68%) female patients and 8 (32%) male patients, their ages ranged from 1 to 57 and mean value was 23 years old. Though 14 patients (56%) complained of respiratory symptoms such as pneumonia and recurrent respiratory infections, a large number of patients (44%) were asymptomatic or had chest pain only when the lesion was discovered. Only 8 patients (32%) were diagnosed as pulmonary sequestration preoperatively and 8 (32%) were suspected as mediastinal or lung tumor, 5 (20%) were congenital or acquired cystic lung disease, and 4 (16%) were lung abscess or bronchiectasis, respectively. The majority of aberrant arteries (86.4%) confirmed during the operation were originated from thoracic aorta and 2 were thoracic and abdominal aorta, 1 was abdominal aorta, respectively. The younger patients (less than 10 years old) had more other congenital anomalies (30% vs 6.7%) but the proportion of congenital IPS was not significantly different (10% vs 6.7%, p>0.05) compared with elder patients. Histopathologically, almost all lesions showed chronic inflammation, cystic changes and similar pleural adhesions regardless of age. Conclusion: The large portion of the patients with IPS (44%) was clinically asymptomatic or presented non-respiratory symptoms at diagnosis and likely to be diagnosed as mediastinal or lung tumor especially in elder patients. Though the younger patients had more other congenital anomalies, most cases of IPS proved to be acquired lesions in terms of the histopathlogical findings and the proportion of congenital evidences.

Influence of Cerebral Protection Methods in Thoracic Aortic Surgery Using Hypothermic Circulatory Arrest (저체온 순환정지를 이용한 흉부 대동맥 수술 시 뇌관류 방법에 따른 수술결과)

  • Kim, Jae-Hyun;Na, Chan-Young;Oh, Sam-Sae
    • Journal of Chest Surgery
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    • v.41 no.2
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    • pp.229-238
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    • 2008
  • Background: Protection of the brain is a major concern during thoracic aortic surgery using hypothermic circulatory arrest (HCA). This study compares the surgical outcomes of two different cerebral protection methods in thoracic aortic surgery using HCA: retrograde cerebral protection (RCP) and antegrade cerebral protection (ACP). Material and Method: We retrospectively reviewed data on 146 patients who underwent thoracic aortic surgery from May 1995 to February 2007 using either RCP (114 patients, Group 1) or ACP (32 patients, Group 2) during HCA. There were 104 dissections (94 acute and 10 chronic) and 42 aneurysms (41 true aneurysms and 1 pseudoaneurysm), and all patients underwent ascending aortic replacement. There were 33 cases of hemiarch replacement, 5 of partial arch replacement, and 21 of total arch replacement. Result: The two groups were similar in preoperative and operative characteristics, but Group 2 had more elderly (over 70 years old) patients (34.4% vs. 10.5%), more coronary artery diseases (18.8% vs. 4.4%), more total arch replacements (46.9% vs. 5.3%) and longer HCA time ($50{\pm}24$ minutes vs. $32{\pm}17$ minutes) than Group 1. The operative mortality was 4.4% (5/114) and 3.1% (1/32), the incidence of permanent neurologic deficits was 5.3% (6/114) and 3.1% (1/32), and the incidence of temporary neurologic deficits was 1.8% (2/114) and 9.4% (3/32) in Groups 1 and 2, respectively. There were no statistical differences between the two groups in operative mortality, postoperative bleeding, or neurologic deficits (permanent and temporary). Conclusion: The early outcomes of aortic surgery using HCA were favorable and showed no statistical difference between RCP and ACP. However, the ACP patients endured longer HCA times and more extended arch surgeries. ACP is the preferred brain protection technique when longer HCA time is expected or extended arch replacement is needed.

Percutaneous Endovascular Stent-graft Treatment for Aortic Disease in High Risk Patients: The Early and Mid-term Results (고위험군의 대동맥류 환자에서 경피적으로 삽입이 가능한 스텐트 그라프트를 이용한 치료: 조기 및 중기성적)

  • Choi, Jin-Ho;Lim, Cheong;Park, Kay-Hyun;Chung, Eui-Suk;Kang, Sung-Gwon;Yoon, Chang-Jin
    • Journal of Chest Surgery
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    • v.41 no.2
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    • pp.239-246
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    • 2008
  • Background: Aortic surgery for high risk patients has high mortality and morbidity rates, and the necessity of performing aortic surgery in cancer patients is questionable because of their short life expectancy. Endovascular repair of aneurysm repair can be considered for high risk patients and cancer patients because it has relatively lower invasiveness and shorter recovery times than aortic surgery does. Especially, percutaneous endovascular stent graft treatment is more useful for high risk patients because it does not require general anesthesia. Material and Method: From July 2003 to September 2007, twelve patients who had inoperable malignancy or who had a high risk of complication because of their combined diseases during aortic surgery underwent endovascular aortic aneurysm repair. he indications for endovascular repair were abdominal aortic aneurysm in 5 patients, descending thoracic aortic aneurysm in 6 patients and acute type B aortic dissection in one patient. The underlying combined disease of these patients were malignancy in 3 patients, respiratory disease in 6 patients, old age with neurologic disease in 6 patients, Behcet's iseae in one patient and chronic renal failure in one patient. Result: Stent grafts were inserted percutaneously in all cases. There were 4 hospital deaths and there were 3 delayed deaths during the follow-up periods. There were no deaths from aortic disease, except one hospital death. There were several complications: a mild cerebrovascular accident occurred in one patient, acute renal failure occurred in 2 patients and ischemic bowel necrosis occurred in one patient. Mild type I endoleak was observed in 2 patients and type II endoleak was observed in a patient after stent graft implantation. Newly developed type I endoleak was observed in a patient during the follow-up period. Conclusion: Percutaneous endovascular stent graft insertion is relatively safe procedure for high risk patients and cancer patients. Yet it seems that its indications and its long term results need to be further researched.