• Title/Summary/Keyword: 신증후군

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A Case of Gitelman Syndrome Presented with Epileptic Seizure (간질 발작으로 내원하여 진단된 Gitelman 증후군 1례)

  • Park Jee-Min;Kim Jeong-Tae;Shin Jae-Il;Kim Heung-Dong;Kim Tae-Young;Cheong Hae-Il;Lee Jae-Seung
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.68-73
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    • 2004
  • Both Gitelman syndrome and Bartter syndrome are autosomal recessively inherited renal tubular disorders characterized by hypokalemic metabolic alkalosis, salt wasting and normal to low blood pressure. Gitelman syndrome is caused by mutations in the thiazide-sensitive Na-Cl cotransporter (NCCT) and distinguished from Bartter syndrome, which is associated with mutations of several genes, by the presence of hypomagnesemia and hypocalciuria. In most of the patients with Gitelman syndrome, the disease manifests with transient episodes of muscular weakness and tetany in the adult period, but, often, is asymptomatic. We report here an 11 years-old female with Gitelman syndrome who presented with aggravation of epileptic seizure. The diagnostic work-up showed typical clinical features of metabolic alkalosis, hypokalemia, hypomagnesemia and hypocalciuria. We also identified a heterozygote mutation($^{642}$CGC(Arg)>TGC(Cys)) and an abnormal splicing in the SLC12A3 gene encoding NCCT.

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Efficacy of 23-valent Pneumococcal Polysaccharide Vaccine in Steroid Responsive Nephrotic Syndrome (스테로이드 반응성 신증후군 환아에서 23-valent pneumococcal polysaccharide vaccine의 예방효과)

  • Hahn, Hye-Won;Ha, Il-Soo;Cheong, Hae-Il;Lee, Hoan-Jong;Choi, Yong
    • Childhood Kidney Diseases
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    • v.6 no.1
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    • pp.56-60
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    • 2002
  • Purpose Streptococcus pneumoniae is a major pathogen in both adults and children, causing significant morbidity and mortality In patients with nephrotic syndrome, Streptococcus pneumoniae is a major cause of spontaneous peritonitis, and the increasing incidence of penicillin-resistance strain facilitates the development of effective vaccine. The limitation of current pneumococcal polysaccharide vaccine prompted development of polysaccharide- protein conjugate vaccine. Methods: We reviewed the medical record of total 225 steroid responsive nephrotic patients to ascertain the effectiveness of 23- valent pneumococcal polysaccharide vaccine. Results. Twenty- eight patients have developed peritonitis during the courses, and 7 of those have recurrent peritonitis. Fifty- five patients were vaccinated and followed- up for 1- 108 months (mean 38.5 months), and during the follow- up period, pneumococcus related peritonitis was not detected. Vaccine- related relapse of nephrotic syndrome w as absent. Conclusion: In spite of the non- consensus about the efficacy of PPV23, clinically it benefits, and until the clinical trial of PCV7 is completed, PPV23 will be recommended. (J Korean Soc Pediatr Nephrol 2002;6: 56-60)

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A case of restless legs syndrome in a child presenting with growing pains (성장통으로 발견된 하지 불안 증후군 1예)

  • Kim, Dong Soon;Shin, Hong Beom;Ahn, Young Min
    • Clinical and Experimental Pediatrics
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    • v.51 no.11
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    • pp.1222-1227
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    • 2008
  • Restless legs syndrome (RLS) is a common neurological sleep disorder in adults characterized by the following diagnostic criteria: an urge to move that is usually associated with unpleasant sensations and symptoms that are worse at rest, relieved by movement, and most severe at night. The definite diagnosis of RLS in children is stricter and consists of self-description of leg discomfort or the presence of 2 of 3 supportive criteria combined with 4 essential criteria for diagnosis in adults. RLS in childhood has often been misdiagnosed as growing pains or a part of normal development. As a result, physicians have often missed the chance for proper management. We diagnosed a case of RLS in a 5-year-old boy presenting with growing pains, whose mother was found to have had RLS since childhood. We confirmed RLS by using a polysomnograph, in which the indices of periodic limb movement syndrome (PLMS) and periodic limb movement during wakefulness (PLMW) were recorded to be compatible with RLS criteria. The patient's ferritin level was low normal, and his symptoms improved after taking iron supplements.

Pulmonary Embolism In Childhood Minimal Change Nephrotic Syndrome (소아 미세변화 신증후군에서 폐색전증에 대한 연구)

  • Sung, Seung-Joon;Hong, Ki-Woong;Kim, Eun-Ryoung;Kim, Il-Soo;Cho, Byung-Soo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.100-108
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    • 2001
  • Purpose : We investigated the incidence and predisposing factors of pulmonary embolism in minimal change nephrotic syndrome(MCNS). Methods : Lung perfusion scan using 99mTC-MAA were done on 14 patients who were diagnosed to minimal change nephrotic syndrome. Group h: Five patients who had perfusion defects on scan, Group B; Nine patients who had no perfusion defect on scan. Between the two groups, the differences of platelet number, hematocrits, albumin, cholesterol, triglyceride, proteinuria were evaluated. Results : Five patients were found to have perfusion defect consistent with pulmonary embolism($35.7\%$). However, there were minimal or no respiratory symptoms and signs. In our laboratory studies, the mean proteinuria on admissions was $676{\pm}31\;mg/m2/hr$ in the group with pulmonary embolism, and $313{\pm}28\;mg/m2/hr$ in the group without pulmonary embolism. There were more severe proteinuria in group with pulmonary embolism(P<0.05). The mean platelet count at early stage of remission after steroid treatment was $746,600{\pm}280,000/mm3$ in the group with pulmonary embolism, $511,890{\pm}90,000/mm3$ in the group without pulmonary embolism. There were significant difference of platelet count between the two groups(P<0.01). In patients with pulmonary embolism, there were more higher and sustained increasement of platelet count. All cases of pulmonary embolism were treated with dipyridamole(5 mg/kg). In 4 cases the perfusion defects were improved in two weeks, however, one case showed persistent perfusion defect after 1 month. Conclusion : Our study suggested that pulmonry embolism might be one of tile major complications in childhood MCNS The occurrence rate was correlated with severity of proteinuria before treatment and sustained increasement of platelet counts in early remission state after steroid treatment. Therefore, the scintigraphic pulmonary perfusion study is mandatory in childhood MCNS, especially in the high risk patients, such as the patients with severe proteinuria and sustained increasement of platelet count. (J Korean Soc Pediatr Nephrol 2001;5 : 100-8)

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Evaluation of Blood Volume State Using the Quotient of Urine Sodium and Potassium Excretion in Primary Nephrotic Syndrome in Children (일차성 신증후군 환아에서 소변 나트륨과 칼륨 농도를 이용한 저혈량증 평가)

  • Choi, Jung-Youn;Park, Yong-Hoon
    • Childhood Kidney Diseases
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    • v.11 no.1
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    • pp.9-15
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    • 2007
  • Purpose : Edema is one of the cardinal features of nephrotic syndrome. Although the pathogenesis of edema is not entirely understood, it is caused by hypovolemia or hypervolemia by different mechanisms. Accordingly it is important to evaluate the volume status of patients in order to treat the edema, but it is difficult to evaluate the patient's volume status only by clinical parameters. The quotient of urine sodium and potassium excretion $U_K/(U_{Na}+U_K)$ is introduced as a more useful way to evaluate volume status. In this study we will propose the usefulness of $U_K/(U_{Na}+U_K)$ in evaluating the volume status of children with nephrotic syndrome. Methods : Primary nephrotic syndrome patients at Yeungnam University Hospital since January 1995 to June 2005, were included in the study. We analyzed clinical parameters such as tachycardia, cardiomegaly, pleural effusion, blood chemistry and urinalysis prospectively. We defined hypovolemia when $U_K/(U_{Na}+U_K)$ exceeded 60%. Intravenous albumin and diuretics were administered to hypovolemic edematous patients. On the other hand, hypervolemic edematous patients were treated only with diuretics. Results : There were 50 cases of primary nephrotic syndrome patients(hypervolemia: 29 vs hypovolemia: 21). There were no significant differences in clinical symptoms and laboratory findings except for FeNa While $F_eNa$ and $U_K/(U_{Na}+U_K)$ had a significant negative correlation, BUN and $U_K/(U_{Na}+U_K)$ had a significant positive correlation. Urine output after edema treatment was effective and there were no treatment-related side effects in both groups. Conclusion : FeNa, BUN and $U_K/(U_{Na}+U_K)$ are a useful parameters for evaluating volume status of edematous nephrotic syndrome patients. We could suggest a therapeutic option for using albumin and/or diuretics according to volemic status by means of measured $U_K/(U_{Na}+UK)$.

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A Case of Infantile Nephrotic Syndrome (부신 석회화가 동반된 영아형 신증후군)

  • Lee, Kyung-A;Shin, Son-Mun;Park, Yong-Hoon
    • Journal of Yeungnam Medical Science
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    • v.9 no.2
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    • pp.427-435
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    • 1992
  • We have experienced a case of infantile nephrotic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed growth and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.

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No Effect of IV cyclophosphamide in Children with Steroid Resistant Nephrotic Syndrome (스테로이드 저항성 신증후군 소아 3례에서 Cyclophosphamide 정맥투여요법의 치료실패)

  • Min, Su-Jeen;Lee, Chang-Yeun
    • Childhood Kidney Diseases
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    • v.2 no.2
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    • pp.183-186
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    • 1998
  • Steroid Resistant Nephrotic Syndrome(SRNS) in children has poor prognosis and no effective therapy. In 1994, Ravi Elhence have reported that IV cyclophosphamide therapy was effective against SRNS of children. So, we evaluated the efficacy of IV cyclophosphamide in 3 children with biopsy proven steroid-resistant MCNS. And the result was the rapeutic failure. In conclusion, IV cyclophosphamide therapy wass not effective against SRNS of children.

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Chronic Renal Failure in Russell-Silver Syndrome (Russell-Silver 증후군에서의 만성 신부전)

  • Ahn, Yo-Han;Lee, Se-Eun;Kang, Hee-Gyung;Ha, Il-Soo;Cheong, Hae-Il;Choi, Yong
    • Childhood Kidney Diseases
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    • v.13 no.2
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    • pp.256-260
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    • 2009
  • The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.

A Study on Hydraulic Experiment for Improvement of Dredging Efficiency (준설 효율 향상을 위한 수리실험 연구)

  • Chae, Dong-Seok;Park, Jae-Hyeon;Kim, Young-Do;Lee, Man-Soo
    • Proceedings of the Korea Water Resources Association Conference
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    • 2007.05a
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    • pp.1373-1377
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    • 2007
  • 준설은 수중굴착, 운반(배송), 매립 기술로 구분되는데, 국내에서는 아직까지 각 기술을 통합한 체계적인 연구가 수행되지 못하고 있다. 준설분야는 토질, 수리 등의 토목 분야 기술뿐만 아니라 기계, 제어, 환경 등 여러 분야의 기술들이 매우 복잡하게 연계되어 있으며, 제한된 소수의 기술자들과 특수 대형 장비-준설선에 의해 수행되기 때문이다. 최근에는 장비의 대형화, 첨단화가 진행되면서 선진국과의 기술격차로 인해 국제 경쟁력이 약화되어 심각한 위기에 처해있는 현장기술 분야이다. 유럽의 델프트 공대, IHC, 준설협회(CEDA)는 세계 기술 개발을 주도하고 있으며, 미국에서는 육군공병단의 수로국에서 준설을 담당하고 있으며 DOER(Dredging Operation & Environmental Research) 기술 개발을 주도하는 등 선진국에서는 이미 여러 연구가 진행되었으며 준설기술이 정착되어있다. 본 연구에서는 현재 현대건설에서 보유중인 아산3호를 모델로 축소모형을 제작하여 실험하였다. 본 연구에서는 선행실험으로 성층형성 및 석션펌프 작동시 물입자의 운동방향을 PIV로 촬영한 결과에 커터헤드를 추가 장착 시행하여 이를 바탕으로 수치해석시 발생할 수 있는 오류를 수정 보완 할 것이다. 차후 실험에서는 준설토의 조건과 기계적 조건 및 환경적인 영향력을 고려한 여러 가지 방법으로 진행할 계획이며, 실험 조건에서는 각 매개변수를 달리하여 이를 토대로 데이터를 구성하고, 측정을 통해서 얻어지는 결과 값을 기초로 하여 준설시에 환경과 매개변수의 조건에 따른 준설량의 특성을 파악하고 효율을 향상시킬 수 있는 방향을 제시할 것이며, 이를 통해서 기본적인 메뉴얼을 구성할 계획이다. 수리실험의 결과값을 바탕으로 이를 현실무 작업에 적용할 경우 효율적인 준설작업을 수행할 수 있을 것으로 판단된다. HDL-콜레스테롤 함량은 유의차가 없었다. 간조직 중 총지질, 총콜레스테롤 및 중성 지방 함량은 HC군이 NC군에 비하여 유의하게 증가되었다. HC-LREL 군과 HC-LREH군은 HC군에 비하여 간조직 중 총지질 함량만이 유의하게 저하되었다. 이상의 결과 연근 에탄올 추출물은 in vitro에서 HMG-CoA reductase 활성을 농도 의존적으로 저하시켰으며, in vivo 에서는 고콜레스테롤 식이급여로 증가되어진 LDL-콜레스테롤 및 중성지질 함량을 감소시키고, 감소되어진 인지질 함량을 증가시킴으로써 고콜레스테롤 혈증 및 지방간의 예방과 치료에 효과가 있을 것으로 사료되나 연근의 어떠한 성분의 작용 기전에 의한 것인지에 대해서는 더욱 체계적인 연구가 요구된다.}nlein$ nephritis가 3례로 가장 많았다. 미세변화 신증후군 71명 중 비재발군이 16명으로 22.5%, 비빈발 재발군 49.3%, 빈발 재발군 18.3%, 스테로이드 의존군 9.9%를 보였다. 결론 : 대전시에서의 신증후군 환아의 발생빈도는 15세이하 소아 10만명당 약 5명으로 추정되었으며 10여 년전과 비교하여 큰 변화를 보이지는 않았다. 또한 저자들의 임상병리학적 연구결과가 다른 문헌에서 보고된 소아 신증후군의 연구결과와 큰 차이를 보이지 않음을 알 수 있었다. 자극에 차이가 있지 않나 추측되며 이에 관한 추후 연구가 요망된다. 총대장통과시간의 단축은 결장 분절 모두에서 줄어들어 나타났으나 좌측결장 통과시간의 감소 및 이로 인한 이 부위의 통과시간 비율의 저하가 가장 주요하였다. 이러한 결과는 차가운 생수 섭취가 주로 결장 근위부를 자극하는 효과를 발휘하는 것이 아닌가 해석된다. 이와 같은 연구결과를 통해 생다시마를 주원료로 개발된 생다시마차와 생다시마 음료가 만성 기능성 변비 증세를 개선하는 효

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Influence of Premenstrual Syndrome and Stress Response on Learning Flow in Nursing Students (간호대학생의 월경전증후군과 스트레스반응이 학습몰입도에 미치는 영향)

  • Shim, Chung-sin
    • The Journal of the Convergence on Culture Technology
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    • v.5 no.2
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    • pp.83-91
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    • 2019
  • The purpose of this study was to investigate the relationship among the variables and the influence of premenstrual syndrome and stress response on the learning flow of nursing students. Method : The participants were 162 nursing students in K city, who were surveyed between June 1 and June 10, 2018, using self-report questionnaire. Result: There were positive correlation between premenstrual syndrome of participants and stress response(r=.389, p<.001). There were negative correlation between premenstrual syndrome and learning flow(r=-1.93, p<.014), between stress response and learning flow(r=-.216 p<.001). The variables for learning flow where stress response(${\beta}=-.314$, p<.001) and Psychological change of menstruation(${\beta}=-.358$, p<.001), with a coefficient of determination($R^2$) of 30.3%. The result of this study will be useful in preparing basic date for improvement of the learning flow of the nursing students and it will be necessary to develop educational programs and strategies related with the premenstrual syndrome and stress response disposition.