• Childhood Kidney Diseases
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• 제19권2호
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• pp.184-189
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• 2015

Poststreptococcal glomerulonephritis (PSGN) is one of the most well-known and important infectious renal diseases resulting from a prior infection with group A ${\beta}$-hemolytic streptococcus. The typical clinical characteristics of the disease reflect acute onset with gross hematuria, edema, hypertension and moderate proteinuria after the antecedent streptococcal infection. In children, usually PSGN is healed spontaneously but if it combines with fast progressing glomerulonephritis, it would be developed to chronic renal failure. Therefore, it is important to make a fast diagnosis and treatment by simple tools to predict the course and the prognosis of disease. Sonography is a simple tool for diagnosis but there is no typical renal sonographic finding in PSGN, so it is difficult to predict the course and the prognosis of disease by sonographic findings. In comparison between two cases of renal sonographic findings in PSGN, a patient who showed more increased echogenicity in more extended area of renal sonography had the severe results of renal pathology, prolonged treatment period and low serum C3 level. Here, we report the different findings of renal sonography and pathology depending on the degree of severity between two patients. Thus, it is necessary to gather more information from further studies to make a consensus about the relationship between the renal sonography and the prognosis of disease in PSGN.

• Childhood Kidney Diseases
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• 제24권1호
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• pp.42-46
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• 2020

Disturbances in water and salt balances are relatively common in children after brain tumor surgery. However, the coexistence of different diseases of water and sodium homeostasis is challenging to diagnose and treat. The coexistence of combined central diabetes insipidus (CDI) and cerebral salt wasting syndrome (CSWS) is rare and may impede accurate diagnosis. Herein, we report the case of an 18-year-old girl who underwent surgery for a germinoma and who presented prolonged coexistence of CDI and CSWS. The patient was diagnosed with panhypopituitarism with CDI at presentation and was treated with hydrocortisone, levothyroxine, and desmopressin. Postoperatively, she developed polyuria of more than 3L/day, with a maximum daily urine output of 7.2 L/day. Her serum sodium level decreased from 148 to 131 mEq/L. Polyuria was treated with desmopressin at incremental doses, and hyponatremia was managed with fluid replacement. At 2 months after surgery, she presented with hyponatremia-induced seizure. Polyuria and hyponatremia combined with natriuresis indicated CSWS. Treatment with fludrocortisone were initiated; then, her electrolyte level gradually normalized. CSWS is self-limiting and generally resolves within 2 weeks. However, the patient in this study still required treatment with vasopressin and fludrocortisone at 16-months after surgery. Hyponatremia in a patient with CDI may be erroneously interpreted as inadequate CDI control or syndrome of inappropriate antidiuretic hormone secretion, leading to inappropriate treatment. The identification of the potential combination of CDI and CSWS is important for early diagnosis and treatment.

• Childhood Kidney Diseases
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• 제22권2호
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• pp.91-96
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• 2018

Nephrotic syndrome in the first year of life, characterized by renal dysfunction and proteinuria, is associated with a heterogeneous group of disorders. These disorders are often related to genetic mutations, but the syndrome can also be caused by a variety of other diseases. We report an infant with nephrotic syndrome associated with a neuroblastoma. A 6-month-old girl was admitted with a 10% weight loss over 10 days and nephrotic-range proteinuria. She was ill-looking, and her blood pressure was higher than normal for her age. Her cystatin-C glomerular filtration rate was decreased, and levels of plasma renin, aldosterone, and catecholamines were elevated. Renal ultrasonography and abdominal computed tomography showed a retroperitoneal prevertebral mass encasing both renal arteries and the left renal vein. The mass was partially resected laparoscopically, and the pathologic diagnosis was neuroblastoma. Findings on a simultaneous renal biopsy were unremarkable. The patient was treated with chemotherapy and several anti-hypertensive drugs, including an alpha blocker. Two months later, the mass had decreased in size and the proteinuria and hypertension were gradually improving. In an infant with abnormal renin-angiotensin system activation, severe hypertension, and nephrotic-range proteinuria, neuroblastoma can be considered in the differential diagnosis.

• Tuberculosis and Respiratory Diseases
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• 제50권2호
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• pp.245-251
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• 2001

저자들은 신장의 혈관근지방종을 동반한 폐임파관평활근종증 1예를 경험하였기에 폐임파관평활근종증에 대한 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제21권1호
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• pp.15-20
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• 2017

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.

• Childhood Kidney Diseases
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• 제21권1호
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• pp.8-14
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• 2017

Purpose: To classify the results of renal biopsy in pediatric patients and to compare pathological findings with clinical features. Methods: This study included data of 318 children who underwent renal biopsy at our hospital between December 1987 and November 2014. Biopsy specimens were examined histopathologically using light, immunofluorescence, and electron microscopy. Results: Asymptomatic urinary abnormalities was the most common clinical diagnosis (35.9%), followed by nephrotic syndrome (29.3%), and acute glomerulonephritis (18.0%). Glomerular disease was identified in 98.1% of the renal biopsy specimens. The most common primary cause of glomerulonephritis was IgA nephropathy, with gross hematuria in 61.9% of the patients, hypertension in 14.2%, proteinuria >1.0 gm/24-hr in 33.3%, and impaired renal function in 3.6% patients. Conclusion: The most common clinical diagnosis was asymptomatic urinary abnormalities, with primary glomerular disease being the most common renal biopsy finding, and IgA nephropathy the most common histopathological lesion. This study provides a 27-year overview of pediatric renal disease at our center and underlines the importance of renal biopsy for accurate diagnosis and proper management.

• 한국의사학회지
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• 제30권2호
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• pp.83-100
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• 2017

Research purpose : Review of the systematized medical theory of Huangdi within the first series of conversations including questions and answers between Leigong and Huangdi. Conclusions : 1. "Huang-di-Nei-Jing" records the 5 theories of Huangdi's system of thinking (黃帝學派). Among these, the contents of the questions and answers with Leigong led to the establishment of the first of Huangdi's treatises. 2. In the questions and answers exchanged between Leigong and Huangdi, the Suwen (素問) Chapter deals with the areas of Yinyang, Zangfu, Cause and Mechanism of diseases, as well as medical ethics. Moreover, the "Lingshu (靈樞)" Chapter discusses distribution of meridian system, routes of circulation of energies, symptoms, acupuncture therapy and diagnosis, etc. 3. Characteristics of the medicine of Leigong and Huangdi can be summarized as follows: Firstly, 12 meridian systems of the "Jingmai (經脈)" section describes the meridian system that ceaselessly circulates in the shape of loops with the inner and outer aspects linked with each other. Secondly, regarding the methods of diagnosis, the "Yinyangleilun (陰陽類論)" section describes the method of diagnosis through palpation of pulse. In addition, the "Wuse (五色)" section describes visual diagnosis that uses five colors. The Wuhang combination of spleen, liver, kidney of "Shicongronglun (示從容論)" section also includes the Wuhang theory of "Jinwenshangshu (今文尙書)". The "Wuse" section discusses areas of the face and five colors, while the "Yinyangleilun" discusses the 'Yinyancixiongmaifa (陰陽雌雄脈法)', the "Jingmai" discusses the 'Yinyancunkoumaifa (人迎寸口脈法)', and the "Zhengsishilun" and "Fangchengshuailun" discuss 'chifuzhenfa (尺膚診法)'. Thirdly, Huangdi's school of thinking did not neglect bencao (本草) and tangye (湯液), which are discussed in the "Jiejingweilun" and "Shicongronglun" sections.

• Childhood Kidney Diseases
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• 제20권1호
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• pp.29-32
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• 2016

Purpose: Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common anomalies identified in newborns. This study aims to demonstrate the prevalence of CAKUT including hydronephrosis diagnosed by antenatal and postnatal ultrasound over a five-year period. Methods: The records of births between May $1^{st}$, 2009 and April $30^{th}$, 2014 at our hospital were collected. The number of infants who underwent renal ultrasound after birth for the detection of CAKUT was counted. The incidence of each type of CAKUT such as hydronephrosis, size abnormality, horseshoe kidney, and Multicystic dysplastic kidney (MCDK) was retrospectively evaluated for antenatal screening and postnatal follow-up examination. Results: During the study period, 33,276 infants were born and 521 neonates underwent postnatal renal ultrasound. 183 cases of CAKUT were detected prenatally and 140 postnatally using ultrasonographic examinations at the following time: (i) 3-7 days postnatally in 123 newborns (87.9%), (ii) during 1-3 months in 11 newborns (7.9%), and (iii) later than 3 months in 6 newborns (4.3%). Among diagnosed CAKUT, hydronephrosis was the most common anomaly with 113 newborns diagnosed prenatally and 46 postnatally. Duplex kidney was the second most common anomaly followed by horseshoe kidney, simple cysts in the kidney and so on. Conclusion: The detection of CAKUT is an important part of the prenatal ultrasound. This study analyzed the prevalence of CAKUT detected on prenatal screening and compared the results to those detected postnatally. Prenatal ultrasound screening fulfills the needs of postnatal examinations and therefore, both antenatal and postnatal sonographic investigations are of vital importance for diagnosis of renal and urinary tract anomalies.

• Childhood Kidney Diseases
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• 제1권1호
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• pp.53-59
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• 1997

목적 : 한쪽 신장이 완전히 기능이 없는 무형성신이나 거의 기능이 없는 다낭성이형성신 환아에서 하나 남은 신장마저 방광요관역류가 동반되어 손상되는 사례가 최근 많이 보고되고 있어 일측성 무형성신, 저형성신, 다낭성이형성신을 일측성 신발생 이상이라는 하나의 군으로 묶어 일측성 신발생 이상 반대측으로 방광요관역류가 잘 동반되는지를 조사하고자 하였다. 방법 : 1987년 1월 1일부터 1996년 12월 31일까지 세브란스 병원, 영동 세브란스 병원에서, 또 1994년 1월1일부터 1996년 12월 31일까지 아주대학 병원에서 일측성 무형성신, 저형성신, 다낭성이형성신으로 진단받은 96명의 소아를 대상으로 후향적 연구를 시행하였다. 진단은 방사선 소견에 근거하였으며, 96명의 대상 환아 중 48명의 환아가 배뇨성 방광요도조영술을 시행하였다. 결과 : 1. 일측성 신발생 이상을 가진 96명 환아군의 성별 분포는 남아가 58명으로 60%였고, 여아가 38명으로 40%였다. 2. 일측성 신발생 이상의 분포는 좌측이 45례로 47%였고, 우측이 51례로 53%였다. 3. 일측성 신발생 이상을 진단받게 된 동기를 보면 총 96례 중 산전 진단에 의한 경우가 41례로 가장 많았으며, 요로 감염의 검사 도중이나 다른 동반 기형의 평가 도중 발견된 경우가 각각 18례, 16례로 많았다. 질환별로 볼 때 일측성 무형성신이나 저형성신은 요로 감염의 검사 도중이나 다른 동반 기형의 평가 도중 발견된 경우가 많았으나 다낭성이형성신은 산전 진단으로 발견된 경우가 대부분이었다. 4. 배뇨성 방광요도조영술을 시행한 환아들의 경우 일측성 신발생 이상의 진단 연령은 $1.8{\pm}3.3$세였고, 배뇨성방광요도 조영술의 시행 연령은 $2.5{\pm}3.8$세로 통계적으로 유의한 격차가 있었다(P<0.01). 5. 일측성 무형성신으로 진단받은 18명의 환아 중 9명, 일측성 저형성신으로 진단받은 11명의 환아 중 5명, 일측성 다낭성이형성신으로 진단받은 19명의 환아 중 3명에서 반대측 방광요관역류가 있었다. 6. 방광요관역류가 있었던 환아들은 역류의 정도가 평균 3등급 이상으로 중등도 이상이었다. 반대측 방광요관역류가 있었던 17명의 환아 중 3명은 만성 신부전 상태에 있었고, 6명은 방광요관문합술을 시행받았다. 결론 :한쪽 신장의 기능이 없거나 거의 없다고 여겨지는 일측성 신발생 이상 환아에서 반대측 방광요관역류를 조기에 발견하기위해 선별적 검사로 배뇨성 방광요도조영술을 반드시 시행해야 한다고 결론지을 수 있었다.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.157-165
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• 2003

목적 : $Henoch-Sch\"{o}nlein$ purpura(HSP)는 여러 장기를 침범하는 전신성 혈관염으로 신장손상이 예후를 좌우하는 중요한 원인이 된다. 성인 HSP 신염은 빈도가 낮고 임상 경과에 대한 연구가 부족한 편이다. 소아와 성인 HSP 신염의 임상 및 경과를 전반적으로 비교함으로서 각각의 예후에 영향을 주는 인자를 알아보고자 하였다. 방법 : 1986년 1월부터 2003년 5월까지 17년 4개월간 연세대학교 세브란스병원에 내원한 15세 미만의 소아 81명과 15세 이상의 성인 25례를 대상으로 후향적으로 고찰하였다. 결과 : 남녀비는 소아 1.5:1, 성인 1.3:1이었고, 발병 계절은 두 군 모두 가을과 겨울에 발병 빈도가 증가하였다. 감염이 선행한 경우가 소아 39례(48.1%), 성인 16례(64.0%)였고, 약물을 복용한 경우는 소아 8례(9.9%), 성인 4례(16.0%)였다. 모든 환자에서 현미경적 혈뇨가 있었고 신증 범위의 단백뇨를 보인 예는 소아 13례(16.0%), 성인 7례(28.0%)있었다(Table 3). 추적 관찰에서 요검사가 정상화된 경우는 소아 34례(42.0%), 성인 4례(16.0%)였고, 무증상 요검사 이상의 예는 소아 41례(50.6%), 성인 18례(72.0%)이었다. 신증후군 또는 고혈압의 합병증이 초래된 경우는 소아 3례(3.7%), 성인 2례(8.0%)이었고, 투석 또는 이식을 받은 경우는 소아 3례(3.7%), 성인 1례(4.0%)였다(Table 4). 소아 21례에서 추적 신생검을 시행하였고 이 중 10례는 조직학적 등급의 변화를 보이지 않았고, 낮은 등급으로 호전된 경우가 9례, 높은 등급으로 악화된 경우가 2례있었다. 진단 당시 신증 범위의 단백뇨가 있었던 경우와 조직학적 등급이 높았던 경우에 나쁜 예후를 보였고(P<0.05) 이는 두 연령군에서 통계학적 차이가 없었다(Table 5). 결론 : 본 연구에서는 소아와 성인에서 임상 양상 및 경과가 두 군에서 다르지 않음을 확인하였고, 소아와 성인 두 군 모두 진단 당시 신증 범위의 단백뇨가 있거나 조직학적 등급이 높을수록 나쁜 예후를 보이므로 그에 따른 장기적인 계획과 치료가 필요할 것으로 생각된다.