• Childhood Kidney Diseases
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• v.24 no.2
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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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• v.21 no.2
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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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• v.1 no.2
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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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• v.13 no.2
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• pp.242-247
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• 2009

Type 1 diabetes mellitus (T1DM) commonly occurs in childhood and adolescence and diabetic nephropathy is a serious metabolic complication of T1DM that leads to serious morbidity. With poor glycemic control prepubertal diabetes duration contributes to the risk of long-term microvascular complications, however, the younger age at onset or longer prepubertal diabetes duration seems to prolong the time to development of microalbuminuria or later end-stage renal disease (ESRD). Therefore, there have been a few cases of diabetic nephropathy in prepubertal patients and therefore the ESRD cases developed during adolescence in T1DM children were very rare. Here we report an adolescent with T1DM who had poor glycemic control and was diagnosed as diabetic nephropathy in a prepubertal period and leading to end-stage renal disease during adolescence.

• 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.

• Childhood Kidney Diseases
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• v.24 no.2
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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.

• Quality Improvement in Health Care
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• v.7 no.2
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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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• v.32 no.9
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• pp.835-839
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• 1999

Since the first report of coronary artery bypass grafting (CABG) in patients with end-stage renal disease in 1974, numerous reports have documented the feasibility of CABG in patients with chronic renal diseases. Patients with chronic renal failure often have comorbid disorders such as hypertension, and diabetes mellitus, each with their own complications and associated impact on both short and long-term survivals. In addition, infection and sepsis have been identified as significant causes of morbidity and mortality in most series of patients with end-stage renal disease undergoing cardiac surgical procedure. As a result of these and other factors such as perioperative volume and electrolyte disturbances, patients with chronic renal failures are at an increased risk of complication and mortality after CABG. We report 3 cases of "Off-pump" CABG in the chronic renal failure patients. patients.

• Childhood Kidney Diseases
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• v.27 no.1
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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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• v.1 no.2
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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.