• Pediatric Infection and Vaccine
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• v.3 no.1
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• pp.66-74
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• 1996

There continues to be increasing the number of patients being treated for renal failure day by day due to lot of causes. It is prerequsite for the physician to have a proper understanding of drug use in patients with renal failure since kidney is the major route of elimination for many kinds of drugs and their metabolites. In order to provide practical guidelines for prescribing antibiotics, the literature has been reviewed, and summarized. The tables presented here are made by Dr. William M Bennett et al. and listed the specific pharmacokinetic information such as drug half life, serum levels, and drug removal during dialysis, plasma protein binding, volume of distribution.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.196-199
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• 1998

The classical manifestation of asphyxiating thoracic dystrophy comprise narrow chest, short limbs, and pelvic changes. The degree of respiratory distress varied from negligible to lethal. The development of progressive renal failure may indicate that the condition is asphyxiating thoracic dystrophy. We have experienced a case of asphyxiating thoracic dystrophy who died from chronic renal failure in a 5-month old infant. Abriefreviewofassociatedliteratureisalsopresented.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.145-151
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• 2007

Hypertonicity (hypernatremia) of extracellular fluid causes water movement out of cells, while hypotonicity(hyponatremia) causes water movement into cells, resulting in cellular shrinkage or cellular swelling, respectively. In most part of the body, the osmolality of extracellular fluid is maintained within narrow range($285-295 mOsm/kgH_2O$) and some deviations from this range are not problematic in most tissue of the body except brain. On the other hand, the osmolality in the human renal medulla fluctuates between 50 and $1,200 mOsm/kgH_2O$ in the process of urine dilution and concentration. The adaptation of renal medullary cells to the wide fluctuations in extracellular tonicity is crucial for the cell survival. This review will summarize the mechanisms of urine concentration and the adaptation of renal medullary cells to the hyper tonicity, which is mediated by TonEBP transcription factor and its target gene products(UT-A1 urea transporter etc.).

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.64-66
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• 2018

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

• 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.27 no.2
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• pp.127-132
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• 2023

Cystinuria, a genetically inherited disorder, is a rare cause of kidney stones. It is characterized by impaired transport of cystine and amino acids in the proximal renal tubule and the small intestine. Most patients develop cystine stones throughout their lifetime. Recurrent renal stones need to be extracted by repeated urologic interventions. Treatment options of cystinuria for preventing stone recurrence are limited and poorly tolerated. In this study, we report a pediatric case of cystinuria with a heterozygous SLC3A1 mutation diagnosed by stone analysis, measurement of urine cystine excretion, and genetic analysis. There were recurrent renal stones despite repetitive shock wave lithotripsy and retrograde intrarenal surgery. However, the rate of stone formation seemed to be slower after D-penicillamine was added into adequate hydration and urinary alkalinization.

• Childhood Kidney Diseases
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• v.20 no.2
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• pp.83-87
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• 2016

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is difficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detection of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age. During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hypercellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis suggested a compensatory reaction to the severe nephron loss or glomerular obsolescence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.44-47
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• 2016

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.79-81
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• 1997

Minimal change nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. Children with onset of nephrotic syndrome between the age of 1 and 8 year are likely to have steroid response to minimal chage disease, but we experienced one case of minimal change disease which failed to respond to steroid therapy at beginning and subsequently developed acute renal failure. It was seen in a 5 year-old male child that presented with edema and gross hematuria. Peritoneal dialysis was performed for acute renal failure for 11 days. Patient was completely recorvered from acute renal failure and renal biopsy was done at 27th day after onset of disease which revealed typical picture of minimal change disease complicated by acute tubular necrosis. We beleive this case is very unusual and it may be the first case in the literature in terms of pediatric cases.

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.18-22
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• 2016

Purpose: The previous reports regarding VUR resolution were not precise due to early frequent surgical intervention. We evaluated the spontaneous resolution (SR) rate and the incidence of new renal scars in primary VUR, focusing on severe reflux. Methods: Medical records of 334 patients with primary VUR who were on medical prophylaxis without surgery for 1 to 9 years, were retrospectively reviewed. Medical prophylaxis was initiated with low-dose antibiotic prophylaxis or probiotics. Radioisotope cystourethrography was performed every 1 to 3 years until SR of reflux. New renal scar was evaluated with follow-up $^{99m}Tc$ DMSA renal scan. Results: The SR rates decreased as VUR grades were getting higher (P=0.00). The overall and annual SR were 58.4% and 14.9%/yr in grade IV reflux and 37.5% and 9.3%/yr in grade V reflux. The median times of SR were 38 months in grade IV reflux and 66 months in grade V reflux. The probable SR rates in grade IV and V reflux were 7.8% and 8.9% in the 1st year, 46.0% and 30.8% in the 3rd year and 74.4% and 64.4% in the 5th year. The incidences of new renal scars between low to moderate reflux and severe reflux showed no significant difference (P=0.32). Conclusion: The SR rates of severe primary VUR were higher than previously reported and most new renal scars were focal and mild.