A5022311304- Renal Diseases and Glomerulopathies
- Parathyroid Disorders and Treatments
- Pediatric Urology and Nephrology Studies
- Hemodynamic Monitoring and Therapy
- Trace Elements in Health
- Lanthanide and Transition Metal Complexes
- Vasculitis and related conditions
- Systemic Lupus Erythematosus Research
- Urinary Tract Infections Management
- Dialysis and Renal Disease Management
- Neuroendocrine Tumor Research Advances
- Complement system in diseases
- Blood groups and transfusion
- Electrolyte and hormonal disorders
- Peptidase Inhibition and Analysis
- Homicide, Infanticide, and Child Abuse
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Cystic Fibrosis Research Advances
- Diphtheria, Corynebacterium, and Tetanus
- Abdominal vascular conditions and treatments
- Neonatal Health and Biochemistry
- Neonatal and Maternal Infections
- Sarcoidosis and Beryllium Toxicity Research
- Magnesium in Health and Disease
- Heart Rate Variability and Autonomic Control
Pusan National University Yangsan Hospital
2023-2024
Pusan National University Hospital
2024
Uijeongbu St. Mary's Hospital
2021-2023
The Korean Society of Nephrology
2022
Seoul National University Children's Hospital
2020-2021
Eulji University
2021
HNF1B mutations, one of the most common causes congenital anomalies kidney and urinary tract, manifest as various renal extrarenal phenotypes. We analyzed genotype-phenotype correlations in 14 pediatric patients with mutations. Genetic studies revealed total gene deletion six (43%). All had bilateral abnormalities, primarily multiple cysts. Twelve exhibited progressive functional deterioration, them progressed to failure. The annual reduction estimated glomerular filtration rate was−2.1...
Purpose: This study evaluated the diagnostic performance of contrast-enhanced voiding urosonography (ce-VUS) using a second-generation ultrasound contrast agent for diagnosis vesicoureteral reflux (VUR) and intrarenal (IRR), compared it with that standard fluoroscopic cystourethrography (VCUG).Methods: Thirty-two consecutive children from April to October 2019 were included in this study. ce-VUS VCUG performed simultaneously by two operators intravesical infusion mixture medium, iodinated...
Abstract The genotype–phenotype correlation of the X-linked Alport syndrome (XLAS) has been well elucidated in males, whereas it remains unclear females. In this multicenter retrospective study, we analyzed 216 Korean patients (male:female = 130:86) with XLAS between 2000 and 2021. were divided into three groups according to their genotypes: non-truncating group, abnormal splicing truncating group. male patients, approximately 60% developed kidney failure at median age 25.0 years, survival...
Background: X-linked hypophosphatemia (XLH) is the most frequent form of hypophosphatemic rickets and caused by mutations in PHEX gene. We analyzed genotype-phenotype correlations XLH patients with proven mutations. Methods: were detected 55 out 81 who clinically presented rickets. The grouped into nontruncating ( n = 9) truncating 46) mutation groups; their initial presentation as well long-term clinical findings evaluated according to these groups. Results: Initial findings, including...
In 2021, a new chapter on the general management of glomerulonephritis (GN) was added to Kidney Disease: Improving Global Outcomes (KDIGO). It emphasizes importance early GN for improving long-term kidney outcomes and prognosis. The introduces glomerular diseases in 18 subchapters. Here, biopsy diagnosis evaluation function complications, such as hypertension, infection, thrombosis, are presented. Moreover, adverse effects glucocorticoids immunosuppressive therapy, which commonly used drugs...
A mercury sphygmomanometer (MS) has been the gold standard for pediatric blood pressure (BP) measurements, and diagnosing hypertension is critical. However, because of environmental issues, other alternatives are needed. Noninvasive BP measurement devices largely divided into auscultatory oscillometric types. The aneroid sphygmomanometer, currently used method, inferior to MS in terms limitations such as validation regular calibration difficult apply infants, whom Korotkoff sounds not...
BACKGROUND Hearing loss (HL) in children may adversely affect their development. HL is more prevalent patients with chronic kidney disease (CKD) than the general population. This study evaluated prevalence of and its underlying diseases childhood-onset CKD. METHODS In this retrospective a tertiary referral center, CKD (stage 2-5, age at onset renal symptom < 18 years) were recruited. We referred to syndromic as cases genetic or diseases, extra-renal anomalies addition RESULTS A total 421...
Purpose This study aimed to assess the feasibility of contrast-enhanced ultrasound (CEUS) for diagnosis acute pyelonephritis (APN) in pediatric patients with febrile urinary tract infection (UTI). Materials and methods Between March 2019 January 2021, participants suspected UTI were assessed APN using ultrasound. Parenchymal echogenicity changes, renal pelvis dilatation, presence a focal lesion conventional grayscale The location decreased perfusion area evaluated color Doppler (CDUS) CEUS....
Abstract Background Ocular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of (APS) that results multiorgan failure and high mortality rate, has rarely been reported. Case presentation A 15-year-old girl presented with sudden vision blurring both eyes. She had marked optic disc swelling macular exudates the right eye intra-arterial white plaques, few retinal blot hemorrhages, ischemic retina left eye. Systemic examination revealed she acute kidney...
Bioimpedance spectroscopy (BIS) is a noninvasive method used to evaluate body fluid volume status in dialysis patients, but reports on its effectiveness pediatrics are scarce. We investigated the correlation between BIS and clinical characteristics identified changes patients whose prescription was modified based BIS. The medical records of children maintenance who had undergone 2017 2019 were reviewed. Of 49 14 overhydrated, >15% proportion overhydration relative extracellular water...
Stimulator of interferon gene (STING)-associated vasculopathy with onset in infancy (SAVI) is an extremely rare autoinflammatory disease. We present the case a female Korean patient early-onset interstitial lung disease who was initially suspected to have systemic lupus erythematosus (SLE) but ultimately diagnosed SAVI. The exhibited signs and cutaneous manifestations before age 1 year continued recurrent fever accompanied by pulmonary infiltrates. Based on positive findings for antibodies...
Abstract Background and Aims Nephrogenic diabetes insipidus (NDI) is a rare inherited disorder, there paucity of studies assessing long-term clinical outcomes in affected individuals. This study aims to evaluate the outcomes, encompassing growth kidney function, pediatric patients with NDI. Method multicenter retrospective included forty-nine children diagnosed The medical records these were retrospectively reviewed, linear mixed model was employed analyze variations function increasing age....
People with group O blood are considered universal organ donors compatible any other group. However, in the case of minor ABO-incompatible transplantation, immune-mediated hemolysis may occur due to concomitant transfer donor B lymphocytes together allograft. These passenger can produce antibodies recipients erythrocytes, causing hemolytic anemia known as lymphocyte syndrome (PLS).A retrospective chart review was performed.A 6-year-old boy (A+) underwent transplantation a kidney from his...
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Here, we present the case of a 2-month-old male infant with hyponatremic hypertensive syndrome resulting from stenosis right proximal and mid-renal arteries. The patient exhibited nephrotic-range proteinuria, low serum albumin, increased creatinine, elevated renin aldosterone levels. Doppler ultrasonography computed tomography angiography revealed decreased vascular flow in small renal artery. Following successful percutaneous balloon angioplasty, experienced decrease blood pressure...
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare and life-threatening disorder. Typical HUS often caused by Shiga toxin-positive Escherichia coli, while aHUS dysregulation of the alternative pathway complement system in association with genetic abnormalities or development autoantibodies. Eculizumab, a humanized anti-complement 5 monoclonal antibody, recommended for treatment aHUS, but its long-term safety efficacy pediatric patients remain under review. In this paper, we...
Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular lymphatic vessels. A 15-year-old boy was initially diagnosed with at the age 8 years based on clinical radiological findings. Following diagnosis, he treated pamidronate, interferon alfa, propranolol, oral corticosteroids, sirolimus. He developed proteinuria 15 progressed into nephrotic range 2 later. renal biopsy revealed focal segmental glomerulosclerosis, not...