• Childhood Kidney Diseases
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• 제22권2호
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Journal of Chest Surgery
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• 제23권3호
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• pp.482-487
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• 1990

The leading cause of death in patients with chronic renal failure is cardiovascular diseases. The problems relevant to cardiac surgery in these patients are occurring more frequently with a growing number of patients at risk. Among these, important risk factors related to uremic patients undergone open heart surgery are fluid and electrolytes imbalance, coagulopathy, increased susceptibility to infection. Since 1968 when Lansing and colleagues reported the first successful aortic valve replacement in patients with chronic renal failure and infective endocarditis, there have been increasing reports of the cardiopulmonary bypass surgery in chronic renal failure patients with acceptable perioperative morbidity and mortality From Jan. 1988 to Nov. 1989 we have experienced four uremic patients necessitating open heart surgery ; one needing a coronary artery bypass graft and the other 3 needed cardiac valve replacement. Based on our observations we would like to suggest followings 1]Intraoperative ultrahemofiltration during C-P bypass thought to be an excellent means for the control of hyperkalemia and fluid balance. 2] The immediate postoperative application of peritoneal dialysis instead of hemodialysis is beneficial in controlling fluid and electrolyte imbalance. 3]The cause of one early postoperative death was not associated to renal failure, rather it was the result of an accidental rupture in the right ventricular wall.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.115-119
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• 2020

Distal renal tubular acidosis (dRTA) is a rare renal tubular disorder characterized by normal anion gap metabolic acidosis, hypokalemia, and high urine pH. It can be inherited or acquired. In untreated pediatric patients with dRTA, rickets and growth retardation are common. We report the case of a 12-year-old Lao girl who presented with typical clinical features of dRTA with severe bone deformities that developed after a bed-ridden state due to a bicycle accident at the age of 8 years. Initial laboratory tests revealed metabolic acidosis with a normal anion gap, hypokalemia, and alkali urine. Renal ultrasonography revealed bilateral medullary nephrocalcinosis. Whole exome sequencing revealed no pathogenic mutations. After treatment with oral alkali, potassium, and vitamin D, she could walk and run. Later, she underwent corrective orthopedic surgeries for bony deformities. Thus, in pediatric dRTA patients, despite severe symptoms remaining untreated, accurate diagnosis and proper management can improve quality of life.

• Childhood Kidney Diseases
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• 제23권1호
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• pp.48-52
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• 2019

The ketogenic diet (KD) has been used as an effective antiepileptic therapy for intractable childhood epilepsy. However, various adverse effects have been reported with use of the KD. We report a case of a child who developed acute tubular necrosis subsequent to therapy with KD. A 5-year-old girl had myoclonic epilepsy with developmental delay. She was under the treatment with antiepileptic drugs since the age of 3 months and on the KD during the past 18 months. Proteinuria persisted intermittently with the initiation of the KD and subsequently increased in the past 2 months. She was admitted with intermittent mild fever, vomiting, and lethargy for the past 3-4 weeks. At the time of admission, she presented with hypertriglyceridemia, heavy proteinuria, renal Fanconi syndrome, and acute kidney injury. Renal sonography showed a marked increase in the size and parenchymal echogenicity of both kidneys. A renal biopsy revealed acute tubular necrosis accompanied by early interstitial fibrosis. After the withdrawal of the KD and supportive therapy, without changing other anticonvulsants and their dosages, improvement of renal function was observed. Proteinuria had disappeared after 1 month and kidney size returned to normal after 8 months. It is hypothesized that the KD can induce and/or aggravate the renal tubulointerstitial injury in some patients who are under the treatment with anticonvulsants.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.30-37
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• 2008

목적 : 소아 연령에서 입원 당시 혹은 다른 질환으로 입원하여 치료를 받던 중에 발생한 신성 급성 신부전의 임상적 특징과 경과 그리고 예후에 대해 분석하였다. 방법 : 2000년 1월부터 2006년 6월까지 부산백병원 소아청소년과에 입원한 환자 중 신성 급성 신부전으로 진단된 59명을 대상으로 하였다. 진단 기준은 혈청 크레아티닌치가 1.2 mg/dL 이상이거나 기저치에 비해 2배 이상 증가한 경우로 하였다. 핍뇨는 소변량이 0.5 mL/kg/hr 이하이며 무뇨는 <50 mL/day로 정의하였다. 결과 : 전체 환자의 성별 비는 2.2:1로 남아에게 많았으며 연령별 분포는 신생아 7명, 2개월-2세 10명, 3-6세 12명, 7-12세 21명, 13-16세 9명이었다. 신부전의 진단까지 평균 기간은 3.1${\pm}$2.8일이었다. 소변량에 따른 분류는 핍뇨군 21명, 비핍뇨군 36명 및 무뇨군 2명이었다. 원인 질환별 분류에서는 원발성 신질환군 30명, 감염군 14명, 종양군 9명 및 기타군 6명이였다. 연령별 분류에서는 신생아는 감염군, 2개월-2세는 신질환과 감염군과 3세 이상에서는 신질환군이 많았다. 계절별 발생 빈도는 차이가 없었으나 여름철에 용혈성 요독증후군의 발생이 많았다. 투석치료는 4명에서 복막투석을 시행하였다. 호전까지의 평균기간은 10.0${\pm}$6.7일이었다. 경과 중 18명이 사망하였는데 비핍뇨군의 사망률이 낮았다. 연령별로는 신생아가 원인별로는 종양군의 사망률이 각각 높았다. 결론 : 소아에서의 신성 급성 신부전은 조기진단과 기저질환의 적절한 관리로 대부분의 환자에서 정상적인 신기능의 회복을 기대할 수 있으며 성인에 비해 양호한 임상적 경과를 보였다.

• Childhood Kidney Diseases
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• 제19권2호
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• pp.131-135
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• 2015

Purpose: The aim of this study was to establish a simple formula to predict renal length in children using a Technesium-99m dimercaptosuccinic acid (DMSA) scan data, and to compare it with the formula derived from ultrasonography, which is widely accepted. Methods: Children who underwent a DMSA scan and ultrasonography were reviewed retrospectively, and those who had anatomical urinary tract abnormalities or urinary tract infections were excluded. Results: A total of 230 children (84 males and 146 females; age, 1 month to 16 years; mean age, $16.8{\pm}27.4$ months). Mean renal length measured by DMSA scan was longer than that by ultrasonography ($6.38{\pm}1.16$ vs. $6.02{\pm}1.14cm$; P < 0.001). Renal length was correlated with age, weight, height, and body surface area on the DMSA scan and ultrasonography, and showed the strongest positive correlation with height. The following formulae were established to predict renal length: mean renal length (cm) = 5.433 ${\times}$ height (m) + 2.330 (R2, 0.833) using the DMSA scan data, and mean renal length (cm) = 5.367 ${\times}$ height (m) + 2.027 (R2, 0.853) using ultrasonography data. Conclusion: We propose a simple height-based formula to predict renal length in children using a DMSA scan data, and validate it by comparing with ultrasonography formula.

• Childhood Kidney Diseases
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• 제12권1호
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• pp.105-110
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• 2008

신성 고혈압은 소아기 이차성 고혈압의 흔한 원인이며, 그 중 신동맥 협착에 의한 신혈관성 고혈압은 치료 가능한 고혈압의 가장 흔한 원인이다. 신혈관성 고혈압 치료로 최근 경피적 신동맥 혈관 성형술은 비침습적인 방법으로 각광 받고 있다. 저자들은 작은 직경을 가진 신동맥 분지 혈관 협착에 의한 신혈관성 고혈압 소아에서 관상동맥용으로 쓰이는 풍선 카테터를 이용하여 경피적 신동맥혈관 성형술을 성공적으로 시행한 경험을 하였으므로 보고하는 바이다.

• Journal of Chest Surgery
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• 제32권9호
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• pp.835-839
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• 1999

1974년에 말기 신부전증 환자에서 심폐바이패스를 이용한 관상동맥우회술이 처음 보고된 이래 고위 穩\ulcorner인 만성 신부전증 환자들에서의 관상동맥우회술에 대한 많은 연구 논문들이 발표되어 왔다. 만성 신부전증 환자들은 고혈압, 당뇨 등을 동반하는 경우가 종종 있으며, 이 질환들의 합병증 혹은 만성 신부전증 자체가 관상동맥우회술의 단기및 장기 생존율에 영향을 미칠수 있다. 개심술을 받은 말기 신부전증 환자들에서는 감염과 패혈증 등의 합병증의 발생률이 높으며, 수술전후의 수액량과 전해질 장애 등으로 수술 위험도가 증 가하는 것으로 알려져 왔다. 저자들은 3예의 만성 신부전증 환자들에서 심폐바이패스를 사용하지 않고 관상 동맥우회술을 시행하여 그 결과를 발표하고자 한다.

• Childhood Kidney Diseases
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• 제17권1호
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• pp.6-12
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• 2013

사구체에 면역글로불린은 음성이면서 C3 만 침착되는 경우, 다른 원인이 없는 경우 C3GN을 의심하여야 한다. 광학현미경으로는 세포의 증식이 있는 경우와 없는 경우가 있지만, 전자현미경으로는 EDD가 내피세포 밑으로 혹은 메산지움 등에 침착함을 확인하여야 한다. 대체보체경로의 조절이상이 원인임으로 혈청 C3 치를 비롯하여 CFH, CFI, CFB 등을 측정하여야 하며, C3 nephritic factor, antifactor H 자가항체 등을 조사하는 것이 필요하다. 보체 조절이상을 일으키는 유전자 돌연변이를 조사해 보아야 한다. 예후는 안정적이지만, 치료는 대증적이다. 이론적으로 보체의 활성화를 저지하는 약으로 가능할 것으로 생각되며, C5 항체인 eculizumab으로 치유된 보고도 있다.

• 대한임상검사과학회지
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• 제38권2호
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• pp.111-116
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• 2006

One of the cardinal findings of the renal diseases is proteinuria, which appears in the early phase of kidney diseases and is very important in diagnosis, prognosis and decision making in the treatment process and results of the treatment. The study subjects were 126 patients who visited the nephrology department of Kangbuk Samsung Hospital. Serum was requested for urine protein electrophoresis. Total protein was measured with Bayer Advia 1650 (Biuret). Quantitation of each fraction was done by multiplying the percentage of each fraction by the total protein. Serum creatinine and BUN were also measured with Bayer Advia 1650 (Jaffe and Urease). Serum protein EP was done with REP(rapid electrophoresis) using Helena Kit reagents (REP Ultra SPE Kit, Ponceau S stain, Acetic acid, Methanol, EP Control). Concentrated urine was used for urine protein EP. The SPSS package was used for statistics analysis. Percentage and quantitation of the level of albumin in renal diseases were significantly lower than those in healthy controls. Total protein was correlated with albumin. In terms of proportion, ${\alpha}1$-globulin, ${\alpha}2$-globulin, ${\beta}$-globulin, and ${\gamma}$-globulin fractions were increased in the disease group. But, in the quantified level, ${\alpha}2$-globulin was increased and ${\beta}$-globulin and ${\gamma}$-globulin were decreased. ESRD patients showed an increased secretion of high molecular proteins in urine protein EP. A decreased level in serum total protein correlated with the decreased level of serum albumin and the total amount of urine total protein. This study revealed the variety in the level of serum and urine proteins and their subgroups by EP.