Nephrology (Genitourinary)

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Review Article
Nephrology (Genitourinary)
Atypical hemolytic uremic syndrome and eculizumab therapy in children
Seong Heon Kim, Hye Young Kim, Su Young Kim
Clin Exp Pediatr. 2018;61(2):37-42.   Published online February 28, 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is...

Original Article
Nephrology (Genitourinary)
Diagnostic accuracy of urinary biomarkers in infants younger than 3 months with urinary tract infection
Nani Jung, Hye Jin Byun, Jae Hyun Park, Joon Sik Kim, Hae Won Kim, Ji Yong Ha
Clin Exp Pediatr. 2018;61(1):24-29.   Published online January 22, 2018
Purpose

The aim of this study was to evaluate the diagnostic accuracy of urinary biomarkers, such as neutrophil gelatinase-associated lipocalin (uNGAL) and β-2 microglobulin (uB2MG), in early detection of urinary tract infection (UTI) in infants aged <3 months with fever.

Methods

A total of 422 infants aged <3 months (male:female=267:155; mean age, 56.4 days), who were admitted for fever, were retrospectively included in...

Is vaginal reflux associated with urinary tract infection in female children under the age of 36 months?
Yu Bin Kim, Chih Lung Tang, Ja Wook Koo
Clin Exp Pediatr. 2018;61(1):17-23.   Published online January 22, 2018
Purpose

To determine the relationship between vaginal reflux (VR) and urinary tract infection (UTI) in female children aged <36 months.

Methods

A single center retrospective study was performed for 191 girls aged <36 months, with a diagnosis of febrile UTI, who underwent a voiding cystourethrography (VCUG) for assessment of vesicoureteral reflux (VUR) at Sanggye Paik Hospital. Fifty-one girls, who underwent VCUG for assessment...

Review Article
Nephrology (Genitourinary)
Genetics of hereditary nephrotic syndrome: a clinical review
Tae-Sun Ha
Clin Exp Pediatr. 2017;60(3):55-63.   Published online March 27, 2017

Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic...

Case Report
Nephrology (Genitourinary)
A novel mutation of CLCNKB in a Korean patient of mixed phenotype of Bartter-Gitelman syndrome
Hee-Won Cho, Sang Taek Lee, Heeyeon Cho, Hae Il Cheong
Clin Exp Pediatr. 2016;59(Suppl 1):S103-S106.   Published online November 30, 2016

Bartter syndrome (BS) is an inherited renal tubular disorder characterized by low or normal blood pressure, hypokalemic metabolic alkalosis, and hyperreninemic hyperaldosteronism. Type III BS is caused by loss-of-function mutations in CLCNKB encoding basolateral ClC-Kb. The clinical phenotype of patients with CLCNKB mutations has been known to be highly variable, and cases that are difficult to categorize as type III...

Posterior reversible encephalopathy syndrome caused by presumed Takayasu arteritis
Ki Wuk Lee, Sang Taek Lee, Heeyeon Cho
Clin Exp Pediatr. 2016;59(Suppl 1):S145-S148.   Published online November 30, 2016

Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology that affects mainly the aorta, main aortic branches, and pulmonary arteries. Diverse neurological manifestations of TA have rarely been reported in children. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological condition that presents with headache, seizure, visual disturbances, and characteristic lesions on imaging. Inflammatory condition and severe hypertension in...

Eosinophilic gastroenteritis in an 18-year-old male with prolonged nephrotic syndrome
Da Min Choi, Jung Eun Pyun, Hyung Eun Yim, Kee Hwan Yoo, Jung Ok Shim, Eun Jung Lee, Nam Hee Won
Clin Exp Pediatr. 2016;59(Suppl 1):S72-S75.   Published online November 30, 2016

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He...

Successful treatment of tubulointerstitial nephritis and uveitis with steroid and azathioprine in a 12-year-old boy
Ji Eun Kim, Se Jin Park, Ji Young Oh, Hyeon Joo Jeong, Ji Hong Kim, Jae Il Shin
Clin Exp Pediatr. 2016;59(Suppl 1):S99-S102.   Published online November 30, 2016

Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, often underdiagnosed or misdiagnosed in children. We describe the case of a 12-year-old boy who presented to Severance Hospital with a 1-month history of bilateral conjunctival injection. He was first evaluated by an Ophthalmologist in another hospital and diagnosed with panuveitis. Laboratory tests indicated renal failure, and a renal biopsy...

Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis
Kyoung Hee Han, Ji Youn Park, Seung-Kee Min, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Clin Exp Pediatr. 2016;59(5):242-245.   Published online May 31, 2016

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%–5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and...

Review Article
Nephrology (Genitourinary)
Pathogenesis of minimal change nephrotic syndrome: an immunological concept
Seong Heon Kim, Se Jin Park, Kyoung Hee Han, Andreas Kronbichler, Moin A. Saleem, Jun Oh, Beom Jin Lim, Jae Il Shin
Clin Exp Pediatr. 2016;59(5):205-211.   Published online May 31, 2016

Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier...

Case Report
Nephrology (Genitourinary)
Deficiency of antidiuretic hormone: a rare cause of massive polyuria after kidney transplantation
Kyung Mi Jang, Young Soo Sohn, Young Ju Hwang, Bong Seok Choi, Min Hyun Cho
Clin Exp Pediatr. 2016;59(4):202-204.   Published online April 30, 2016

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate...

Original Article
Nephrology (Genitourinary)
Acute tubular necrosis as a part of vancomycin induced drug rash with eosinophilia and systemic symptoms syndrome with coincident postinfectious glomerulonephritis
Kyung Min Kim, Kyoung Sung, Hea Koung Yang, Seong Heon Kim, Hye Young Kim, Gil Ho Ban, Su Eun Park, Hyoung Doo Lee, Su Young Kim
Clin Exp Pediatr. 2016;59(3):145-148.   Published online March 31, 2016

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to...

Usefulness of neutrophil-lymphocyte ratio in young children with febrile urinary tract infection
Song Yi Han, I Re Lee, Se Jin Park, Ji Hong Kim, Jae Il Shin
Clin Exp Pediatr. 2016;59(3):139-144.   Published online March 31, 2016
Purpose

Acute pyelonephritis (APN) is a serious bacterial infection that can cause renal scarring in children. Early identification of APN is critical to improve treatment outcomes. The neutrophil-lymphocyte ratio (NLR) is a prognostic marker of many diseases, but it has not yet been established in urinary tract infection (UTI). The aim of this study was to determine whether NLR is a...

Case Report
Nephrology (Genitourinary)
Recombinant Human Erythropoietin Therapy for a Jehovah's Witness Child With Severe Anemia due to Hemolytic-Uremic Syndrome
Da Eun Woo, Jae Min Lee, Yu Kyung Kim, Yong Hoon Park
Clin Exp Pediatr. 2016;59(2):100-103.   Published online February 29, 2016

Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening...

Original Article
Nephrology (Genitourinary)
Impaired angiogenesis in the enalapril-treated neonatal rat kidney
Hyung Eun Yim, Kee Hwan Yoo, Eun Soo Bae, Young Sook Hong, Joo Won Lee
Clin Exp Pediatr. 2016;59(1):8-15.   Published online January 22, 2016
Purpose

Nephrogenesis is normally accompanied by a tightly regulated and efficient vascularization. We investigated the effect of angiotensin II inhibition on angiogenesis in the developing rat kidney.

Methods

Newborn rat pups were treated with enalapril (30 mg/kg/day) or vehicle (control) for 7 days after birth. Renal histological changes were checked using Hematoxylin & Eosin staining. We also investigated the intrarenal expression of vascular...

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