• Childhood Kidney Diseases
• /
• v.10 no.2
• /
• pp.125-131
• /
• 2006

Purpose : High interleukin-1 beta(IL-$1{\beta}$) expression in the skin biopsy specimens of patients with Henoch-$Sch\ddot{o}nlein$ Purpura(HSP) has been observed. We examined IL-$1{\beta}$ gene polymorphism in patients with HSP. The purpose of this study is to examine the relationship between IL-$1{\beta}$ gene polymorphism and renal involvement in HSP. Methods : Patients from mideast Korea with HSP were studied. All patients had at least 6 months of follow up. Patients and ethnically matched controls were genotyped for IL-$1{\beta}$ gene polymorphism by polymerase chain reaction-restriction fragment length polymorphism(PCR-RFLP). Results : Thirty-four patients(all younger than 15 years old) who had been diagnosed with HSP and 27 controls were examined. No allele or genotype differences between the HSP and control groups were observed. No significant association between the carriage of IL-$1{\beta}$(-511) T allele and renal involvement(P=0.525, OR:1.417, CI:0.545-3.686) was found. Conclusion : In unselected patients with HSP, carriage of IL-$1{\beta}$(-511) T allele does not appear to influence renal involvement.

• Childhood Kidney Diseases
• /
• v.21 no.2
• /
• pp.114-120
• /
• 2017

Purpose: The aim of this study was to determine the clinical characteristics, frequency of renal abnormalities and benefits of a top-down approach in children with their first febrile urinary tract infection (UTI). Methods: We reviewed 308 patients retrospectively who were admitted to Yeungnam University Hospital and were treated for their first febrile UTI from February 2006 to December 2013. We performed a comparative analysis of laboratory findings and results of imaging techniques including a Tc-99m dimercaptosuccinic acid (DMSA) renal scan. Results: Among the patients, 69% (213/308) were males, and 90% (277/308) had their first UTI episode during infancy. A DMSA renal scan was performed on all patients, and showed positive findings in 60% (184/308) of cases. Laboratory indices of inflammation were significantly higher in the DMSA-positive group (P< 0.05). There was a statistically significant difference in the age distribution between the two groups. In the DMSA-positive group, 165 patients underwent voiding cystourethrography (VCUG), and 58 (35%) cases demonstrated vesicoureteral reflux. In total, 110 patients in the DMSA-positive group, underwent repeat scanning at 6 months; 33 children (30%) demonstrated static scarring, but 77 (70%) had improved completely. The concordance of the ultrasonography (US) and VCUG was low. Older patients had more renal scarring. Conclusion: DMSA is a sensitive method for assessing the severity of inflammation and kidney injury. However, the ability of US to predict renal parenchymal damage was limited. A top-down approach in children with their first febrile UTI showed significant value.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.26 no.6
• /
• pp.652-657
• /
• 2000

The kidneys play a vital role in the maintenance of normal body fluid volumes and in the composition of the extracellular fluid compartments. There are normally more than 2 million functioning glomeruli that regulate total body water and solute concentrations. As renal failure progresses, there is a decrease in the number of functioning nephrons. Chronic renal failure(CRF) is the consequence of a multitude of diseases that cause permanent destruction of the nephron. Azotemia is an elevation in blood urea nitrogen(BUN) and serum creatinine levels subsequent to a decreased glomerular filtration rate(GFR), which results in uremia. This loss of renal function can cause functional and metabolic abnomalities of body. For this problem, oral & maxillofacial surgeons have demanded to routinely treat patients with CRF. However, there has not been a reported case of orthognathic surgery by bilateral sagittal split ramus osteotomy(BSSRO) in patients with CRF, which can cause multiple complications in healthy patients. We report developmental mechanism of complication associated with CRF and preop. and postop. care of orthognathic surgery by BSSRO in Cl III patient with severe chronic renal failure.

• Childhood Kidney Diseases
• /
• v.15 no.2
• /
• pp.107-115
• /
• 2011

Acute kidney injury (AKI) is associated with mortality and may lead to increased medical expense. A modified criteria (pediatric RIFLE [pRIFLE]: Risk, Injury, Failure, Loss, and End-stage renal disease) has been proposed to standardize the definition of AKI. The common causes of AKI are renal ischemia, nephrotoxic medications, and sepsis. A majority of critically ill children develop AKI by the pRIFLE criteria and need to receive intensive care early in the course of AKI. Factors influencing patient survival (pediatric intensive care unit discharge) are known to be low blood pressure at the onset of renal replacement therapy (RRT), the use of vasoactive pressors during RRT, and the degrees of fluid overload at the initiation of RRT. Early intervention of continuous RRT (CRRT) has been introduced to reduce mortality and fluid overload that affects poor prognosis in patients with AKI. Here, we briefly review the practical prescription of pediatric CRRT and literatures on the outcomes of patients with AKI receiving CRRT and associations among AKI, fluid overload, and CRRT. In conclusion, we suggest that an increased emphasis should be placed on the early initiation of CRRT and fluid overload in the management of pediatric AKI.

• Childhood Kidney Diseases
• /
• v.11 no.2
• /
• pp.299-305
• /
• 2007

Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.

• Childhood Kidney Diseases
• /
• v.11 no.2
• /
• pp.152-160
• /
• 2007

Twenty-six renal allograft biopsies which showed acute rejection and had renal capsule and medulla in the same specimen were selected in order to compare the severity of acute rejection between superficial cortex, deep cortex and medulla. Disregarding the mid cortical region, the superficial cortex was considered as being one-third of the distance from the renal capsule to the medulla and the deep cortex as being that one-third of the cortex which was adjacent to the medulla. Using semiquantitative histologic analysis the following parameters were compared in superficial cortex, deep cortex, and medulla: interstitial inflammation, edema, tubulitis, and acute tubulointerstitial rejection grade. Also, the presence of lymphocyte activation and polymorphonuclear leukocytes was evaluated. Significantly greater histologic changes of acute rejection were found in the deep cortex vs. supeficial cortex for the following parameters: interstitial inflammation(P=0.013), edema (P=0.023) and tubulointerstitial rejection grade(P=0.016). These findings support the view that biopsies in which deep cortex is not included may result in underestimation of the severity of renal allograft rejection.

• Childhood Kidney Diseases
• /
• v.18 no.1
• /
• pp.42-46
• /
• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Childhood Kidney Diseases
• /
• v.12 no.1
• /
• pp.111-115
• /
• 2008

Vitamin D-dependent rickets(VDDR) is a rare autosomal disorder, characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type I VDDR is due to congenital defects of renal 1${\alpha}$-hydroxylase, the enzyme responsible for the conversion of 25-(OH)D3 to 1,25-$(OH)_2D3$. Type II VDDR arise from target organ resistance to 1,25-$(OH)_2D3$. Unilateral renal aplasia is generally thought to result from a lack of induction of the metanephric blastema from the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. The incidence of unilateral renal aplasia is approximately 1/500-3,200. Type 1 VDDR associated with unilateral renal aplasia has not been reported yet. Thus we report a case of a 3 month old female infant diagnosed as type 1 VDDR with unilateral aplasia of kidney.

• Childhood Kidney Diseases
• /
• v.12 no.2
• /
• pp.239-244
• /
• 2008

Calcification in patients with endstage renal disease on renal replacement therapy can occur in extraskeletal area such as conjunctiva and cornea. Conjunctival and corneal calcification(CCC) has mostly has been reported in adults with endstage renal disease on hemodialysis. CCC seems to be associated with the duration of renal replacement therapy, and high Ca$\times$P value. We report a 10-year-old girl who was on peritoneal dialysis for 31 months and presented with CCC on both eyes. Her corneal calcification was resolved after the epithelial debridement and ethylenediaminetetraacetic acid(EDTA) soaking therapy.

• Childhood Kidney Diseases
• /
• v.17 no.2
• /
• pp.143-148
• /
• 2013

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.