• Childhood Kidney Diseases
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• 제20권1호
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• pp.33-36
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• 2016

There are two pathogenic models of renovascular hypertension (RVH) originating from renal artery stenosis. We noted a case of a boy who had severe hypertension with atrophic left kidney, hypertrophic right kidney, a segmental stricture of the abdominal aorta, and total occlusion of the right renal artery. Due to the atrophic change of the contralateral, unclipped left kidney, this patient presented with various clinical manifestations related to both models of pathogenesis of RVH occurring at the same time. We conclude that this patient presented with the middle stage of the two RVH pathogenetic models, so called the 'one-clip, one and half-kidney model.'

• Childhood Kidney Diseases
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• 제14권1호
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• pp.89-93
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• 2010

조영제 사용으로 유발된 중증 저나트륨혈증이 일어난 경우는 매우 드물다. 저자들은 조영제를 사용한 복부전산화단층촬영을 시행했던 환아에서 중증 저나트륨혈증과 경련이 발생하였으나, 신경학적 검사에서 특별한 이상이 발견되지 않았고, 조영제 이외의 저나트륨혈증의 다른 원인을 발견할 수 없었던 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제17권2호
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• pp.35-41
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• 2013

Routine urinalysis is a simple, economical, and useful test that facilitates the detection of urinary system diseases and monitoring of renal disease progression. It consists of 4 parts of specimen evaluation, gross examination, a dipstick urinalysis, and a sediment microscopic urinalysis. Urine specimens should first be evaluated in terms of acceptability, and thereafter, the gross appearance is examined for color, turbidity, and odor. In particular, a dipstick urinalysis is an easy and rapid test that provides information on the multiple physicochemical properties of the urine sample. Moreover, although a sediment microscopic urinalysis is time-consuming, it provides information on the cells, microorganisms, casts, and crystals. In the present report, the clinical significance of the routine urinalysis and the problems concerning interpretation are summarized.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.138-142
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• 2020

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare and life-threatening disorder. Typical HUS is often caused by Shiga toxin-positive Escherichia coli, while aHUS is caused by dysregulation of the alternative pathway of the complement system in association with genetic abnormalities or development of autoantibodies. Eculizumab, a humanized anti-complement 5 monoclonal antibody, is recommended for the treatment of aHUS, but its long-term safety and efficacy in pediatric patients remain under review. In this paper, we report a pediatric case of aHUS with anti-complement factor H autoantibodies, who was treated successfully with eculizumab.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.126-130
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• 2020

Nephrogenic syndrome of inappropriate antidiuresis (NSIAD) is a rare X-linked genetic condition caused by a gain-of-function mutation of arginine vasopressin receptor 2 gene, AVPR2. We report the case of a male neonate diagnosed with NSIAD based on his DNA sequence of the AVPR2 gene and the clinical course. He demonstrated a complete correction of hyponatremia using oral urea. We suggest that (1) sequencing analysis of the AVPR2 gene ought to be done in newborns with prolonged euvolemic hyponatremia, hypo-osmolality, high urinary sodium and normal/low or undetectable AVP levels, and that (2) oral urea is a safe and effective treatment option in infants diagnosed with NSIAD until the patients are grown-up.

• Kidney Research and Clinical Practice
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• 제37권4호
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• pp.315-322
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• 2018

The high mortality rates associated with acute kidney injury are mainly due to extra-renal complications that occur following distant-organ involvement. Damage to these organs, which is commonly referred to as multiple organ dysfunction syndrome, has more severe and persistent effects. The brain and its sub-structures, such as the hippocampus, are vulnerable organs that can be adversely affected. Acute kidney injury may be associated with numerous brain and hippocampal complications, as it may alter the permeability of the blood-brain barrier. Although the pathogenesis of acute uremic encephalopathy is poorly understood, some of the underlying mechanisms that may contribute to hippocampal involvement include the release of multiple inflammatory mediators that coincide with hippocampus inflammation and cytotoxicity, neurotransmitter derangement, transcriptional dysregulation, and changes in the expression of apoptotic genes. Impairment of brain function, especially of a structure that has vital activity in learning and memory and is very sensitive to renal ischemic injury, can ultimately lead to cognitive and functional complications in patients with acute kidney injury. The objective of this review was to assess these complications in the brain following acute kidney injury, with a focus on the hippocampus as a critical region for learning and memory.

• Kidney Research and Clinical Practice
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• 제34권1호
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• pp.53-56
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• 2015

MYH9-related disorder is an autosomal dominant disease caused by a mutation in the MYH9 gene, which encodes nonmuscle myosin heavy chain IIA (NMMHC-IIA). This disease is characterized by giant platelets, thrombocytopenia, granulocyte inclusion bodies, proteinuria, and high-pitch sensorineural deafness. Nephropathy has been observed in 30% of patients with MYH9-related disorder. The characteristic features are early onset proteinuria and rapidly progressing renal disorder. However, the prognosis of MYH9 nephropathy remains unclear. Herein, we describe a 36-year-old woman who presented with proteinuria and was diagnosed with MYH9 nephropathy via renal biopsy and gene analysis. Her proteinuria improved after administration of an angiotensin II receptor blocker, but was aggravated after changing to a calcium channel blocker.

• Childhood Kidney Diseases
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• 제23권2호
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• pp.121-123
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• 2019

Urinary tract infection is common in the pediatric population. The most common causative agents are bacteria, among which Escherichia coli is the most frequent uropathogen. Although fungal urinary tract infection is rare in the healthy pediatric population, it is relatively common among hospitalized patients. Fungus may be isolated from the urine of immunocompromised patients or that of patients with indwelling catheters. The most common cause of funguria is Candida albicans. Although more than 50% of Candida isolates belong to non-albicans Candida, the prevalence of non-albicans candiduria is increasing. Herein, we report a case of community-acquired candiduria in a 4-month-old immunocompetent male infant who had bilateral vesicoureteral reflux and was administered antibiotic prophylaxis. He was diagnosed with urinary tract infection caused by Candida lusitaniae and was managed with fluconazole.

• Childhood Kidney Diseases
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• 제23권1호
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• pp.1-6
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• 2019

Nephrotic syndrome (NS) is the most common glomerular disorder in childhood, and a vast majority of cases are idiopathic. The precise cause of this common childhood disease is not fully elucidated despite significant advancements in our understanding of podocyte biology. Idiopathic NS has been considered "a disorder of T-cell function" mediated by a circulating factor that alters podocyte function resulting in massive proteinuria since the last four decades. Several circulatory factors released from T-cells are considered to be involved in pathophysiology of NS; however, a single presumptive factor has not been defined yet. Extended evidence obtained by advances in the pathobiology of podocytes has implicated podocytes as critical regulator of glomerular protein filtration and podocytopathy. The candidate molecules as pathological mediators of steroid-dependent NS are CD80 (also known as B7-1), hemopexin, and angiopoietin-like 4. The "two-hit" hypothesis proposes that the expression of CD80 on podocytes and ineffective inhibition of podocyte CD80 due to regulatory T-cell dysfunction or impaired autoregulation by podocytes results in NS. Recent studies suggest that not only T cells but also other immune cells and podocytes are involved in the pathogenesis of MCNS.

• Childhood Kidney Diseases
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• 제23권1호
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• pp.7-21
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• 2019

Nephrotic syndrome (NS) is a common chronic glomerular disease in children characterized by significant proteinuria with resulting hypoalbuminemia, edema, and hyperlipidemia. Renal biopsy findings of diffuse foot processes effacement on electron microscopy and minimal change disease, focal segmental glomerulosclerosis (FSGS), or diffuse mesangial proliferation on light microscopy. It has been speculated that circulating permeability factors would be implicated in the pathogenesis of NS because they have been reportedly detected in the sera of patients and in experimental models of induced proteinuria. Moreover, a substantial portion of the patients with primary FSGS recurrence shortly after transplantation. This report reviews the current knowledge regarding the role of circulating permeability factors in the pathogenesis of proteinuria in NS and suggests future targeted therapeutic approaches for NS.