• Clinical and Experimental Pediatrics
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• 제53권7호
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• pp.735-740
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• 2010

Renal fibrosis, characterized by tubulointerstitial fibrosis and glomerulosclerosis, is the final manifestation of chronic kidney disease. Renal fibrosis is characterized by an excessive accumulation and deposition of extracellular matrix components. This pathologic result usually originates from both underlying complicated cellular activities such as epithelial-to-mesenchymal transition, fibroblast activation, monocyte/macrophage infiltration, and cellular apoptosis and the activation of signaling molecules such as transforming growth factor beta and angiotensin II. However, because the pathogenesis of renal fibrosis is extremely complicated and our knowledge regarding this condition is still limited, further studies are needed.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.18-24
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• 2022

Pediatric rheumatologic diseases are rare systemic diseases that can involve various organs, including the kidneys. Each rheumatologic disease can exhibit characteristic renal involvement, which requires proper treatment and diagnosis. In this review, we discuss renal involvement in classic rheumatologic diseases, including juvenile idiopathic arthritis, Sjogren's syndrome, systemic sclerosis, and juvenile dermatomyositis. Reviews addressing lupus nephritis and antineutrophil cytoplasmic antibody-associated renal disease are complex and tend to cover a wide array of topics, and thus were excluded from this review.

• Journal of Korean Neurosurgical Society
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• 제30권8호
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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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• 제21권2호
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• pp.53-60
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• 2017

Proteinuria is common in pediatric and adolescent patients. Proteinuria is defined as urinary protein excretion at levels higher than $100-150mg/m^2/day$ in children. It can be indicative of normal or benign conditions as well as numerous types of severe underlying renal or systemic disease. The school urine screening program has been conducted in Korea since 1998. Since then, numerous patients with normal or benign proteinuria as well as early stage renal diseases have been referred to the hospital. Benign proteinuria includes orthostatic proteinuria and transient proteinuria. Most causes of proteinuria can be categorized into 3 types: 1) overflow, 2) tubular, and 3) glomerular. Although treatment should be directed at the underlying cause of the proteinuria, prompt evaluation, diagnosis, and long-term monitoring of these pediatric patients can prevent potential progression of the underlying disease process. This article provides an overview of proteinuria: its causes, methods of assessment, and algorithmic suggestions to differentiate benign from pathologic renal disease.

• Journal of Chest Surgery
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• 제48권5호
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• pp.371-374
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• 2015

We present a rare case of a patient with aortoiliac occlusive disease on the background of type A crossed renal ectopia, for whom open surgical intervention was required. Aortic exposure in patients with concomitant crossed renal ectopia can present technical challenges to the vascular surgeon. The knowledge of variations in the ectopic renal blood supply is of paramount importance when performing surgery to treat this condition and affects the choice of surgical exposure. We present and discuss the operative details of our patient and outline an approach to this subset of patients.

• 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.

• 혜화의학회지
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• 제14권2호
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• pp.143-151
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• 2005

Gobjectives and Methods to investigate into the course of incurable and relapse renal stones' differentiation of symptoms and signs and to effectively improve treatment of incurable and relapse renal stones through Oriental Medicine. To search for the papers about treatment of incurable and relapse renal stones from 1994 till 2004, and re-search papers about treatment for indicated differentiation of disease. Result and conclusions : 1. It is the kidney vacuity additionally the deficiency of qi(氣) in middle-burner that the incurable and relapse renal stones' differentiation of disease. 2. The cause is taking an overdose of attacking renal stones to eliminate dampness and heat medicine, aging, chrome disease. a sex act excess, insufficiency of congenital qi(氣), insufficient exercise, wrong treatment and stress. 3. Herb medicines are constituted supplementing kidney drugs for inhibition of renal stone's creation, drugs to diuresis and dissolve calculi, and drugs 10 exclude stasis blood(瘀血) on account of renal stones. 4. Anti-renal stone measures me sufficiently drinking water, not eating greasy food. taking medicine for supplementing kidney, diuresis and dissolve calculi by periods, and drinking substitute water to boll down medical herb as Lysimachiae Herba (金錢草) Polygoni Avicularis Herba(篇蓄).

• Journal of Dental Anesthesia and Pain Medicine
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• 제17권3호
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• pp.215-217
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• 2017

Branchio-oto-renal syndrome (BOR) is a rare autosomal dominant disorder. The features include branchial cysts, hearing loss, ear malformation, preauricular pits, retrognathia, congenital heart disease, and renal abnormalities. However, anesthetic management of these patients has seldom been reported. We report a case in which general anesthesia was performed for dental treatment in a patient with BOR. Airway management, renal function, and hemodynamic changes can be of critical concern during anesthetic management. A 13-year-old girl diagnosed with BOR had severe right hearing loss, right external ear malformation, renal abnormalities, and postoperative patent ductus arteriosus (PDA). Dental extraction under general anesthesia was scheduled for a supernumerary tooth. The procedure was completed with sufficient urine volume, adequate airway management, and stable hemodynamics.

• Childhood Kidney Diseases
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• 제26권2호
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• pp.80-85
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• 2022

Purpose: To determine the prevalence, clinical manifestations, and outcomes of renal involvements in pediatric Alagille syndrome (ALGS). Methods: A total of 21 patients diagnosed with ALGS at age under 18 years who visited Samsung Medical Center from March 1999 to March 2022 were enrolled. ALGS was diagnosed either by clinical manifestations, targeted JAG1 sequencing, and/or liver biopsy. Medical records including sex, age, renal manifestations, urinalysis, serum creatinine, JAG1 sequencing, and ultrasonography were retrospectively reviewed. Results: The male to female ratio was 9:12. The mean age of patients at confirmative diagnosis of ALGS was 18.4 months. Sanger sequencing was performed for 17 patients. Sixteen of 21 patients (76.1%) showed JAG1 mutations. Renal involvement was found in 10 patients (47.6%). The most common type of anomaly was renal dysplasia (40%). One patient having renal dysplasia was pathologically confirmed with glomerular lipid deposition. Two patients (20%) manifested nephrocalcinosis/nephrolithiasis. Among eight renal-involved patients who survived, four (50%) progressed to chronic kidney disease stage 3. Two of these chronic kidney disease patients were diagnosed with hepatorenal syndrome. The other four patients had renal functions preserved, including two without any interventions and two who underwent urological interventions. Conclusions: The current study revealed a high prevalence of renal involvement in Korean pediatric ALGS with diverse phenotypes.

• Journal of Chest Surgery
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• 제23권4호
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• pp.791-798
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• 1990

Takayasu’s disease produces the occlusive and aneurysmal lesions of major branches of the aorta. Angiography is the most important diagnostic procedure in Takayasu’s disease. Surgical treatment is often justified to avoid the possible lethal consequences of hypertension on the heart, kidney, and brain, as well as in the case of aneurysm because of its risk of rupture. We experienced one case of the Takayasu’s disease associated with abdominal coarctation and renovascular hypertension. The patient was 17 years old female and had suffered from hypertension for 14 months. On physical examination, BP was 150/100 mmHg in the right arm and 120/80 mmHg in the left arm. The pulses of the left brachial and femoral arteries were weakly palpable. Aortogram showed the stenosis of the left common and subclavian arteries, coarctation of the abdominal aorta, and stenosis of the right renal artery and complete occlusion of the left renal artery. The stenosis of the right renal artery and the occlusion of the left renal artery produced the renovascular hypertension. She underwent aorta-aortic bypass for the coarctation of the abdominal aorta and aorta-renal bypass for treatment of renovascular hypertension Postoperatively, both femoral pulses were equally palpable. On discharge, antihypertensive drugs were discontinued. She has remained normotensive for last one year.