• Childhood Kidney Diseases
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• 제24권2호
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• pp.63-68
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• 2020

Despite the many advantages of peritoneal dialysis (PD) in children with end-stage renal disease, there exist redoubtable complications of PD that should be overcome. To prevent and manage these complications, a multidisciplinary team should provide support highly tailored for each child and family, based on the standardized practice guidelines for the management of pediatric PD. In this review, we summarize the clinical manifestations and management of several complications of PD.

• Journal of Chest Surgery
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• 제21권2호
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• pp.347-350
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• 1988

Recent advances in the managements of chronic renal failure have increased the number of the candidates for cardiac operation in patients with chronic renal disease. There have been reports that the operative mortality of the open cardiac surgery in patients with end stage renal diseases was equal to that of the patients with normal renal function. Aortic valve replacement and mitral annuloplasty was successfully performed in a patient with chronic renal failure, and the pre-and postoperative managements are presented.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.189-194
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• 1997

Oligomeganephronia is a rare congenital form of bilateral renal hypoplasia histologically characterized by reduction in number and hypertrophy of nephrons. Clinically, this condition is presented in early infancy with vomiting, polyuria, polydipsia and dehydration. The problems are readily corrected, but slowly progressive renal failure follows accompanied by failure to thrive, short stature, and renal osteodystrophy. We experienced three cases of oligomeganephronia. Case 1. : A 3 2/12 years old female child was incidentally diagnosed as renal failure at age of 2 months when she was hospitalized due to pneumonia. She had open renal biopsy and was diagnosed as bilateral dysplastic kidney. On OPD follow-up, she progressed to end-stage renal failure (BUN/Cr 114/4.6 mg/dl) and had renal transplantation. The specimen was shrunk remarkably and light microscopy showed oligomeganephronia. Case 2. : A 14 8/12 years old female child with proteinuria was detected in an annual urine screening program for school children, she was diagnosed as renal failure (BUN/Cr 33.9/4.1 mg/dl), and had $5{\times}4{\times}3\;cm$ sized mass on abdominal CT scan. She had renal biopsy, and the specimen showed oligomeganephronia. She had hemodialysis for six months, and renal transplantation along with bilateral nephrectomy was performed. Case 3. : A 14 8/12 years old male child was diagnosed having chronic nephritis and chronic renal failure at 3 years old, progressed to end-stage renal failure (BUN/Cr 87/9.6 mg/dl) on OPD follow-up, and had a rephrectomy and renal transplantation. The biopsy specimen showed oligomeganephronic hypoplasia, secondary focal segmental glomerolosclerosis, and chronic interstitial nephritis. We report 3 cases of oligomeganephronia that progressed to end-stage renal failure and had successful renal transplantation with a brief review of related literatures.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.242-247
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• 2009

소아청소년기의 당뇨병은 대부분 제1형 당뇨병으로, 이 때 발생하는 혈관합병증으로서 당뇨병성 신병증은 소아에서 흔하지 않지만 신부전까지 초래할 수 있는 심각한 합병증이다. 혈당조절이 불량하고 사춘기나 그 이후에 당뇨병이 발생하는 경우에 혈관합병증의 발생이 증가하므로 소아청소년기의 당뇨병이 청소년기에 당뇨병성 신병증으로 발현하는 경우는 드물고, 더욱이 말기 신질환으로 진행하는 경우는 매우 드물다. 저자들은 혈당 조절이 불량했던 제 1형 당뇨병 소아 환자에서 혈뇨와 단백뇨가 관찰되어 조직 검사를 통해 사춘기 전에 발생한 당뇨병성 신병증을 확인하고 사춘기에 말기 신질환으로 진행한 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.256-260
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• 2009

Russell-Silver 증후군은 자궁내 성장 지연, 특징적인 얼굴 기형, 저신장을 특징으로 하는 질환이다. Russell-Silver 증후군에서 동반되는 신질환은 말굽 신장, 신세뇨관 산증, 물콩팥증, 요관 깔때기막힘, 방광 요관 역류 등이 있다. 저자들은 Russell-Silver 증후군 환자에서 말기 신부전이 발생한 예를 경험하였으며 문헌 고찰에서 유사한 증례를 찾을 수 없었기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.91-97
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• 2020

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P=0.035). Conclusion: SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

• 한국의료질향상학회지
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• 제7권2호
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• pp.204-216
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• 2000

Background : There has been a concern that the quality of care provided to end-stage renal disease (ESRD) patients in the United States may not be as good as recommended. This paper illustrates a composite measure to assess, the quality of care received by ESRD patients undergoing in-center hemodialysis by incorporating outcomes for 4 major treatment areas. The 4 treatment areas are: dialysis treatments, anemia control, nutritional management, and blood pressure control. Methods : The major data source for the study was the United States Renal Data System (USRDS) Dialysis Morbidity and Mortality Study Wave 1 (DMMS-1) d Sixteen categories of a composite quality indicator were constructed by combining 4 dichotomous variables (16=2*2*2*2). representing the optimal vs. less than optimal level of outcome for each of the 4 treatment outcome measure respectively. Optimal outcome level for each treatment area was defined based on the recommendation from the National Kidney Foundation: (a) delivered dialysis doses (Kt/V) ${\geq}$ 1.2; (b) hematocrit level ${\geq}$ 30%; (c) serum albumin concentration ${\geq}$ 3.8g/dl ; and (d) blood pressure of <140 / <90mmHg. The 16 quality indicator were ranked according to their relative quality weights, which were estimated from its association with the relative risk of survival, adjusting for patient's baseline severity and dialysis facility characteristics. Results : Out of the entire sample of 2,179 patients, only 229 (10%) meet th recommended outcome levels for all 4 treatment areas. Overall, the study patients were distributed evenly over the 16 quality indicators, indicating a great variation in the quality of ESRD care. It appears that the rank of the 16 quality-indicators is driven by serum albumin concentration, suggesting that serum albumin concentration may be the most powerful predictor of ESRD patient survival among the 4 outcome measures. Conclusion : The developed quality indicator has the advantage of describin a range of care for dialysis patients and thus providing a more complete picture of care as compared to previous studies that have focused on only single or few components of the ESRD care.

• 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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• 제27권1호
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• pp.19-25
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• 2023

Proteinuria is an important risk factor for renal and cardiovascular disease. It is associated with a risk for glomerulonephritis, chronic kidney disease, and end-stage renal disease. Therefore, if persistent proteinuria is detected, kidney biopsy is considered to diagnose and treat the underlying disease. Recently, variants in the cubilin (CUBN) gene, which is associated with albuminuria, have been reported. This gene encodes cubilin, a membrane glycoprotein receptor expressed in the renal proximal tubules. Cubilin is a component of the megalin and cubilin-amnionless complex that mediates albumin reabsorption into the proximal tubules through endocytosis. A defect in cubilin leads to a reduction in albumin reuptake, resulting in albumin-dominant proteinuria. Although numerous controversies exist, several reports suggest that cubilin defects lead to proteinuria with a high portion of albuminuria but may not impair renal filtration function. If albuminuria due to reduced cubilin function is confirmed as a benign condition, we can consider using genetic studies to detect CUBN mutations in patients with proteinuria and they may not require any treatment or kidney biopsy. Here, we review recent papers on CUBN mutations and discuss the prognosis and management of individuals with this mutation.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.183-188
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• 1997

Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric\;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.