• Childhood Kidney Diseases
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• 제14권2호
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• pp.223-229
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• 2010

신성 고혈압은 신동맥의 협착 혹은 폐색으로 인해 유발되는 전신성 고혈압이다. 최근에는 신동맥의 재개통을 위해 PTRA가 선호되고 있지만, 복잡한 경우에 있어서는 신 자가이식이 대안적인 치료방법으로서 시행되어 왔다. 저자들은 심각한 고혈압 증상을 보인 14세 남자 환아를 PTRA 및 신 자가이식을 시행하여 성공적으로 치료하였기에 보고하는 바이다. 도플러 초음파 검사와 CT 혈관조영술상 우측 신동맥 기시부에서 약간의 좁아짐과 다발적인 측부순환을 동반한 좌측 신동맥 기시부의 완전폐색이 확인되었다. 우측 신동맥 협착의 치료를 위해 PTRA와 스텐트 삽입술을 시행하였으나 좌측 신동맥은 완전폐색으로 인해 시술이 불가능하여 좌측 신장을 절제한 후 동측 장골와에 자가이식하였다. 수술 후 시행한 도플러 초음파 검사와 MAG-3 신주사 검사상 신혈류와 신기능은 정상이었다. 혈압은 정상화되었으며 항고혈압제 투여도 점차 중단하였다. 임상적인 특성을 고려할 때 신성 고혈압의 원인은 섬유근성 형성이상으로 생각되었다. 결론적으로 신 자가이식은 혈관내 중재적 시술이 실패하였거나 불가능한 경우에 선택할 수 있는 치료방법이다.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.41-46
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• 2017

Purpose: Thin glomerular basement membrane nephropathy (TBMN) is, along with the IgA nephropathy, the most common cause of asymptomatic hematuria in Korean children. TBMN is usually a benign renal disease not requiring treatment and is associated with a good prognosis, but some cases hematuria is indicative of a state of progressive renal insufficiency. We aimed to retrospectively evaluate clinical manifestations and renal prognosis of patients with TBMN. Methods: Among the 428 renal biopsies performed on children at Yeungnam University Hospital between January 2000 and February 2017, 167 patients were diagnosed as having TBMN. We retrospectively investigated 167 pediatric patients and identified 59 children with follow-up duration >3 years. Results: Among 59 patients, there were 33 boys and 26 girls. Mean age of onset of hematuria was $7.18{\pm}2.64$ years, and mean time from onset of disease until a renal biopsy was performed was $2.48{\pm}2.10$ years. There were no clinical features or laboratory findings among studied children to indicate decreased renal function during follow-up; however, one case progressed to chronic kidney disease (CKD) due to an unknown cause. There were seven patients among these related a positive family history of hematuria or renal insufficiency. Concluson: Although almost all patients had normal renal functions during follow-up, there were one patient who progressed to CKD and seven patients with family history of hematuria or renal insufficiency. Moreover, four among the 428 patients over 17 years underwent repeat renal biopsies, which showed results different from their earlier biopsies.Thus, large-scales studies may be required to determine long-term prognosis of TBMN in children, and further evaluation for Alport syndrome in TBMN cases is essential.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.47-52
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• 2017

Purpose: Renal ultrasonography has been widely used in children with renal disease. However, the relationship of renal echogenicity with renal pathology and function in children is not well known. Method: Ultrasound examination was performed in 75 patients undergoing renal biopsy for suspected renal disease in Konkuk University Medical Center from August 2005 to November 2015. We compared renal echogenicity to pathologic findings and renal function. Renal echogenicity was scored as 0 to 2 by comparing adjacent liver echogenicity. Three histologic characteristics were evaluated: glomerular changes, interstitial infiltration or fibrosis, and tubular atrophy. These were graded as 0 to 3, according to increasing severity. Laboratory results included urine albumin excretion and estimated glomerular filtration rate (eGFR). Results: Among pathologic findings, renal echogenicity revealed a positive correlation with interstitial infiltration or fibrosis (r=0.259, P=0.025), and with tubular atrophy (r=0.268, P=0.02). Renal echogenicity and glomerular changes were not correlated. Renal echogenicity showed a positive correlation with microalbuminuria (r=0.283, P=0.014), but a negative correlation with eGFR (r=-0.352, P=0.002). Conclusion: Increased renal echogenicity suggested severe interstitial infiltration or fibrosis and tubular atrophy among the pathologic findings. Moreover, increased echogenicity is correlated with increased urine albumin excretion and decreased eGFR. Echogenicity on ultrasonography is useful for determining the status of renal pathology and function.

• Journal of Chest Surgery
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• 제20권1호
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• pp.171-176
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• 1987

Takayasu`s arteritis is one of chronic inflammatory disease characteristically involving the aorta and its major branches. Symptoms and signs of the disease are various depending on the involved area. We experienced a surgical case of Takayasu`s arteritis mainly involving both renal arteries with renovascular hypertension in a 13 year old girl. Hypertension was not controlled by medical treatment including diuretics and captopril [160/140 mmHg]. Aortogram showed severely stenosed right renal artery, nearly obstructed left renal artery and not visulalized superior mesenteric artery. Angioplasty was performed for the right renal artery but aorta-renal bypass graft with greater saphenous vein was inevitable for the left renal artery. Blood pressure was controlled sufficiently with some adjunct of captopril postoperatively [130/90 mmHg]. While the patient was discharged with much improvement, she was lost follow up and died of not identified definitive cause 3 months later.

• 한국동물위생학회지
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• 제38권4호
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• pp.249-252
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• 2015

Primary renal tumors are uncommon in dogs with prevalence rate of approximately 1%. Renal carcinoma originating from epithelium of proximal convoluted tubules are more likely to be affected to Middle-aged dogs (average age, 8y), males about twice as often as bitches. A 10-year-old, female, German Shepherd dog with history of anorexia, vomitting and hematuria was referred to the Animal Disease Diagnostic Division in Animal and Plant Quarantine Agency. The dog was necropsied and several organs were collected, fixed in 10% phosphate-buffered formalin, embedded in paraffin wax and sectioned for histopathology. Grossly, the kidneys were bilaterally enlarged ($18{\times}12{\times}8cm$; left, $18{\times}10{\times}8cm$; right). The numerous cysts varying sizes from 3 to 6 cm in diameter were protruding from the surface of both kidney. A large nodule ($10{\times}6{\times}6cm$) was discovered between cardiac and diaphragmatic lobe in the right lung. Immunohistochemical examination revealed strong positive reaction to cytokeratin and ki-67 in the nuclei of the epithelial tumor cells. But showed negative reactions to vimentin and CD10. Based on the pathological and immunohistochemical examination, we diagnosed as the bilateral renal cystadenocarcinoma in German shepherd dog.

• Clinical and Experimental Pediatrics
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• 제52권10호
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• pp.1075-1081
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• 2009

Renal transplantation is the treatment of choice for children with end-stage renal disease. The outcome of pediatric kidney transplantation has improved dramatically in recent years, with lower acute rejection rates, superior graft survival, and low mortality. These improvements have allowed increased attention to other aspects of care for long-term survivors. Taking this into consideration, this review article will focus on the key issues related to pediatric kidney transplantation such as growth, neurocognitive function, nonadherence, and posttransplantation infectious complications, including lymphoproliferative disease, to broaden the understanding of pediatricians who provide pre-and postoperative care to children with end-stage renal disease.

• Childhood Kidney Diseases
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• 제11권2호
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• pp.294-298
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• 2007

모야모야병은 주로 뇌혈관 협착을 유발하여 측부순환을 생성하는 질환으로 최근 뇌혈관 이외의 혈관에도 침범할 수 있음이 보고되고 있다. 지금까지 모야모야병에서 폐동맥, 관상동맥, 말초동맥 및 신동맥의 협착을 동반한 증례들이 발표되고 있다. 이 중 신동맥 협착을 동반한 경우 이로 인한 신성고혈압을 보이는 증례들이 보고되고 있으며 이는 모야모야병에서 지속적인 고혈압을 보여 2차적인 뇌경색이나 뇌출혈 등의 합병증을 유발할 수 있으므로 모야모야 환자의 치료에 있어서 항상 면밀하게 평가되어야 할 부분이다. 저자들은 모야모야병으로 양측 뇌경질막동맥간접문합술을 시행 받고 경과관찰 하던 환아에서 지속되는 고혈압으로 인해 발견된 신동맥 협착 및 이로 인한 신성 고혈압을 경험하였기에 이를 보고하는 바이다.

• Childhood Kidney Diseases
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• 제21권2호
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• pp.75-80
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• 2017

Purpose: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. Methods: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. Results: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the $mean{\pm}standard$ deviation (SD) age at infection was $13.3{\pm}3.9$ years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was $13.1{\pm}3.9$ years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. Conclusions: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

• 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.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.86-91
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• 2013

목적: 본 연구는 가와사키 질환의 신장 침범에 대하여 비교적 덜 침습적이며, 특이도와 민감도가 높은 검사인 Technetium-99m dimercaptosuccinic acid scintigraphy (DMSA renal SPECT)을 통하여 밝혀보고자 한다. 방법: 2011년 3월부터 10월까지 국민건강보험 일산병원에서 가와사키로 진단된 15명의 환아들을 대상으로 진행하였다. 15명의 환아 모두 가와사키의 급성기에 DMSA renal SPECT를 시행하였다. 또한 신장 세뇨관 손상 지표인 요중 ${\beta}2$-microglobulin (${\beta}2$-MG)을 측정하여 이를 통한 가와사키 환아에서 신장 손상의 조기 진단 가능 여부를 연구해보았다. 결과: 환아 15명 모두의 신기능 검사는 정상이었다. 소변 검사상 현미경적 혈뇨와 농뇨가 각각 13%, 33%에서 관찰되었다. 요중 ${\beta}2$-MG는 46%에서 증가된 소견을 보였다. 또한 환아들을 요중 ${\beta}2$MG를 기준으로 증가되어 있는 군과 증가되지 않은 군으로 나누어 비교, 분석해 보았으며, 두군간에 임상 증상, 임상 검사, 초음파 검사 및 심초음파 검사에서 유의한 차이를 보이지 않았다. 모든 환아에서 DMSA renal SPECT는 정상 소견을 보였다. 결론: 본 연구에서 가와사키 질환의 신장 침범은 경한 소변 검사 이상 및 일부 환아에서의 요중 ${\beta}2$-MG의 상승 소견을 보였으며, DMSA renal SPECT에서 관찰될 정도의 신장 침범은 보이지 않았다.