• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 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
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• v.17 no.2
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• pp.143-148
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• 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.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.57-63
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• 2014

A number of kidney diseases of childhood may present as isolated proteinuria or/and hematuria, without any overt signs or symptoms. Urinalysis is a simple and inexpensive test used to evaluate various renal disorders. A school urine screening (SUS) program for kidney disease was conducted in Korea in 1998. Several research reports, including case reports and systemic reviews of SUS data, claimed that early detection and confirmatory diagnosis by renal biopsy seems to be helpful for determining the prognosis and intervention of progressive chronic renal disease. However, there is no global consensus as to whether screening for chronic kidney disease (CKD) should be undertaken in children and adolescents. This paper reviews the SUS for CKD in Korea, including the history and structure of the program, its assessment, related research, and associated problems.

• The Journal of the Korea Contents Association
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• v.19 no.6
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• pp.461-470
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• 2019

In diabetes mellitus, renal disease is a common complication, characterized by increased urinary albumin excretion and reduced eGFR. According to KDIGO CKD stage classification, Korean characteristics were analyzed according to urinary albumin and eGFR using the National Health and Nutrition Examination Survey VI raw data. According to KDIGO classification, diabetic patients were classified as Low risk 72.0%, Moderate risk 19.3%, High risk 5.6% and Very high risk 3.0%. Low risk decreased from 74.7% to 52.2%, and moderate to very high risk increased from 25.4% to 47.8% as the duration of diabetes mellitus was prolonged. The risk factors were CKD stage 1 (HR 2.064) to stage 4 (HR 11.049), the highest risk of hypertension. The incidence of renal disease was elevated according to duration of hypertension and HR 0.42 of kidney disease was decreased in the group maintaining proper blood pressure. In the hypertensive patients, the group administered with target blood pressure had a reduction of the kidney disease by 42% than the group with the hypertension. Therefore, controlling and managing hypertension to target blood pressure is important for the prevention of kidney disease.

• Journal of the Korean Society of Radiology
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• v.83 no.2
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• pp.317-330
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• 2022

Purpose To evaluate the various factors that affect renal function following percutaneous radiofrequency ablation (RFA) therapy in patients with renal tumors. Materials and Methods Between 2010 and 2018, 91 patients diagnosed with renal tumors using ultrasonography and CT-guided RFA were enrolled. We retrospectively investigated the serum creatinine (SCr) level and estimated glomerular filtration rates immediately prior to RFA and during post-treatment follow-up. The patients were divided into two groups based on the degree of change in SCr level (0.3 mg/dL). Group comparisons were performed using univariable and multivariable logistic regression analyses to determine the factors impacting renal function. Results Impaired renal function was associated with solitary kidney, chronic kidney disease (CKD) over stage 3, and pyeloureteral injury. Sex, age, other cancers, tumor size, location, growth pattern, and proximity to the collecting system were not significantly associated with impaired renal function. There was a difference in the overall change over time between the association with and without solitary kidney, CKD stage 3, and pyeloureteral injury. Conclusion Among the medical conditions present prior to RFA, solitary kidney and CKD over stage 3 could be considered as risk factors for impaired renal function. Post-procedural pyeloureteral injury can also be considered a risk factor.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.87-94
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• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.217-220
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• 1999

Kimura disease is a chronic benign disorder, primarily seen in asians male during the second and third decades of life, which presents itself as a tumour like lesion with a predilection for the head and neck region. There is high prevalence of associated renal disease. We report two cases of nephrotic syndrome associated with Kimura disease, and this is the first report of Kimura disease with renal involvement in Korean children.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.51-55
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• 2014

Primary renal artery aneurysm has been estimated to account for an incidence of 0.015-1% with associated morbidities including renovascular hypertension and rupture. Renovascular hypertension associated renal artery aneurysms in children is not a common disease. In patients with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be hazardous in these situations. We report a case of a renal artery aneurysm in a 13-year-old Korean child presenting hypertension detected during school health examination. Preoperative workup demonstrated a $2.8{\times}2.1{\times}1.9$ cm saccular aneurysm in the right renal hilum that was not amendable to endovascular repair. A surgical strategy including extracorporeal renal artery reconstruction with autotransplantation was applied in order to restore renal artery anatomy and to treat renovascular hypertension. Immediately he complained of severe right flank pain and postoperative doppler sonography revealed lack of perfusion. On the 5th day after autotransplantation, the patient underwent a transplant nephrectomy. He was well postoperatively and was found to have a normal kidney function and stable blood pressure control without antihypertensive medication. This is the first pediatric case of renal artery aneurysm in Korea who underwent extracorporeal repair followed by autotransplantation failure. More pediatric cases with renal artery aneurysm should be reported to identify therapeutic outcome and long term prognosis.

• Journal of Korean Neurosurgical Society
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• v.30 no.8
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• pp.1019-1022
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• 2001

Although brain metastasis of renal cell carcinoma is a major cause of death in Von Hippel-Lindau disease(VHL), it is not easy to distinguish local recurrence of hemangioblastoma from distant metastasis. In addition, hemangioblastoma has several characteristics suitable for recipient of "tumor-to-tumor metastasis". Authors report a case of Von Hippel-Lindau disease that had metastases of renal cell carcinomas.

• 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.