• Childhood Kidney Diseases
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• 제18권1호
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• pp.7-12
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• 2014

만성 콩팥병의 장기 예후를 결정하는 요인 중 심혈관계 합병증의 중요성은 잘 알려져 있다. 그러나 소아 환자의 경우 전형적인 증상 발현이 적어 그 임상적 중요성이 간과되는 경향이 있다. 현재까지 알려진 심혈관계 합병증의 위험인자로는 고혈압, 당뇨병, 이상지질혈증, 비만과 같은 전통적인 위험 인자와 빈혈, 이차성 부갑상선 기능항진증, 산화 스트레스, 염증과 같은 새로운 위험 인자 등이 알려져 있다. 소아 만성 콩팥병의 경우 주로 좌심실 비대나 경동맥 내중막 두께의 증가 및 관상동맥의 석회화 같이 대부분 겉으로 드러나지 않는 증상이므로 위험 인자에 대한 철저한 관리와 지속적인 추적 관찰이 필수적이다.

• Journal of Veterinary Science
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• 제24권6호
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• pp.75.1-75.8
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• 2023

Background: In veterinary medicine, previous studies regarding the diagnostic performance of shear wave elastography (SWE) in chronic kidney disease (CKD) are not consistent with each other. Moreover, there has been no study evaluating the relationship between symmetric dimethylarginine (SDMA) concentration and renal shear wave velocity (SWV) using two-dimensional SWE (2D SWE) in dogs with CKD. Objectives: This study aimed to evaluate the diagnostic capability of 2D SWE in dogs with CKD and to assess the relationship between renal SWV and SDMA concentration. Methods: Dogs with healthy kidneys and dogs with CKD underwent 2D SWE and SDMA assay. Renal stiffness was estimated as renal SWV in m/s. Results: SDMA concentration had a weak positive correlation with the left (r = 0.338, p = 0.022) and right renal SWV (r = 0.337, p = 0.044). Renal SWV was not significantly different between healthy kidney and CKD groups in the left (p = 0.085) and right (p = 0.171) kidneys. Conclusions: 2D SWE may could not distinguish between dogs with healthy kidney and dogs with early stage of CKD, but it would be useful for assessing the serial change of renal function in dogs.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.40-45
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• 2022

Purpose: Chronic kidney disease (CKD) has various underlying causes in children. Identification of the underlying causes of CKD is important. Genetic causes comprise a significant proportion of pediatric CKD cases. Methods: In this study, we performed whole-exome sequencing (WES) to identify genetic causes of pediatric CKD. From January to June 2021, WES was performed using samples from pediatric patients with CKD of unclear etiology. Results: Genetic causes were investigated using WES in 37 patients (17 males) with pediatric CKD stages 1 (n=5), 2 (n=7), 3 (n=2), 4 (n=2), and 5 (n=21). The underlying diseases were focal segmental glomerulosclerosis (n=9), congenital anomalies of the kidney and urinary tract including reflux nephropathy (n=8), other glomerulopathies (n=7), unknown etiology (n=6), and others (n=7). WES identified genetic causes of CKD in 12 of the 37 patients (32.4%). Genetic defects were discovered in the COL4A4 (n=2), WT1 (n=2), ACTN4, CEP290, COL4A3, CUBN, GATA3, LAMA5, NUP107, and PAX2 genes. WT1 defects were found in patients whose pathologic diagnosis was membranoproliferative glomerulonephritis, and identification of CUBN defects led to discontinuation of immunosuppressive agents. Genetic diagnosis confirmed the clinical diagnosis of hypoparathyroidism, sensorineural deafness, and renal disease; Alport syndrome; and Joubert syndrome in three of the patients with CKD of unknown etiology (COL4A4 [n=2], CUBN [n=1]). Extrarenal symptoms were considered phenotypic presentations of WT1, PAX2, and CEP290 defects. Conclusions: WES provided a genetic diagnosis that confirmed the clinical diagnosis in a significant proportion (32.4%) of patients with pediatric CKD.

• Healthcare Informatics Research
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• 제24권4호
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• pp.292-299
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• 2018

Objectives: The aim of the present study was to assess the frequency of use, and preferences regarding information and communication technologies (ICTs) among Ecuadorian patients with chronic kidney disease (CKD) undergoing hemodialysis. Methods: We conducted an anonymous cross-sectional survey-based study from January 2016 to April 2017, involving 393 patients with end-stage renal disease from 9 hemodialysis centers, in which they rated their use and preferences of various ICTs through a modified version of the Michigan Questionnaire. The questionnaire collected information regarding demographics, patients' interest in obtaining health-related information through ICTs, and interest in using ICTs as a potential way to communicate with their healthcare providers. A chi-square test for association and adjusted regression analyses were performed. Results: Among all patients who participated, 64.3% reported owning a cellphone, with less than a third reporting active Internet connection. The most used ICT for obtaining information about CKD and/or hemodialysis was web-based Internet, followed by YouTube. SMS was rated the highest to receive and seek health-related information, followed by Facebook. Younger age and higher levels of education were associated with a higher overall usage of ICTs. Finally, more than half of the patients reported interest in using WhatsApp for communicating with their healthcare providers. Conclusions: Understanding the preferences of ICTs among patients with CKD undergoing hemodialysis could help to improve their outcomes through the potential uses and benefits of ICTs. Further research is needed to assess their role in improving the care of patients with chronic diseases.

• Journal of Veterinary Science
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• 제21권4호
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• pp.58.1-58.11
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• 2020

Background: Quantitative evaluation of renal cortical echogenicity (RCE) has been tried and developed in human and veterinary medicine. Objectives: The objective of this study was to propose a method for evaluating RCE quantitatively and intuitively, and to determine associations between ultrasonographic renal structural distinction and estimated glomerular filtration rate (eGFR) in canine chronic kidney disease (CKD). Methods: Data were collected on 63 dogs, including 27 with normal kidney function and 36 CKD patients. Symmetric dimethylarginine and creatinine concentrations were measured for calculating eGFR. RCE was evaluated as 3 grades on ultrasonography images according to the distinction between the renal cortex and outer medulla. The RCE grade of each kidney was measured. Results: There was a significant difference in eGFR between the group normal and CKD (p < 0.001). As mean of RCE grades (the mean values of each right and left kidney's RCE grade) increases, the proportion of group CKD among the patients in each grade increases (p < 0.001). Also, severity of RCE (classified as "high" if any right or left kidney evaluated as RCE grade 3, "low" otherwise) and eGFR is good indicator for predicting group CKD (p < 0.001). Conclusions: The degree of distinction between the renal cortex and the outer medulla is closely related to renal function including eGFR and the RCE grade defined in this study can be used as a method of objectively evaluating RCE.

• Childhood Kidney Diseases
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• 제25권2호
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• pp.117-121
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• 2021

Chronic kidney disease (CKD)-mineral and bone disorder (CKD-MBD) is a common complication of CKD, often accompanied by extra-skeletal calcification in adult patients. As increased vascular calcification is predicted to increase cardiovascular mortality and morbidity, the revised Kidney Disease: Improving Global Outcomes guidelines recommend avoiding calcium-containing phosphate chelators. However, extra-skeletal calcification is less commonly noticed in pediatric patients. Here, we report our experience of such a complication in pediatric patients receiving maintenance peritoneal dialysis. Extra-skeletal calcification was noticed at the corneas, pelvic cavity, and soft tissues of the lower leg in 4 out of 32 patients on maintenance peritoneal dialysis. These patients experienced the aggravation of extra-skeletal calcifications during peritoneal dialysis, and 2 of them underwent excisional operations. It is required to monitor extra-skeletal calcifications in children on kidney replacement therapy.

• 대한한방내과학회지
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• 제42권5호
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• pp.727-737
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• 2021

Objectives: This study investigated the effect of Korean medicine on an elderly patient with posterior circulation cerebral infarction, chronic kidney disease (CKD), and early neurological deterioration (END). Methods: The patient, who already had CKD, was treated with Korean medicine, comprising herbal medicine, acupuncture, moxa, and cupping combined with Western medicine (antiplatelet, diabetes) and physical therapy. A manual muscle test (MMT) and a modified Barthel index (MBI) were used to observe the treatment effects, and blood tests were performed to check estimated glomerular filtration rate (eGFR), creatinine and blood urea nitrogen (BUN), which represent renal function. Results: After the treatment, MMT, MBI, and renal function scores had increased. Conclusions: This study suggests that Korean medicine can effectively treat posterior circulation cerebral infarction with END in CKD, but further studies should be conducted.

• Childhood Kidney Diseases
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• 제15권2호
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• pp.116-124
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• 2011

Acute kidney injury (AKI) can result in mortality or progress to chronic kidney disease in hospitalized patients. Although serum creatinine has long been used as the best biomarker for diagnosis of AKI, it has some clinical limitations, especially in children. New biomarkers are needed for early diagnosis, differential diagnosis, and reliable prediction of prognosis in AKI. Up to the present, candidate AKI biomarkers include neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), interleukin-18 (IL-18), livertype fatty acid-binding protein (L-FABP), matrix metalloproteinase-9 (MMP-9), and Nacetyl-$\ss$-D-glucosaminidase (NAG). However, whether these are superior to serum creatinine in the confirmation of diagnosis and prediction of prognosis in AKI is unclear. Further studies are needed for clinical application of these new biomarkers in AKI.

• Childhood Kidney Diseases
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• 제22권2호
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• pp.64-66
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• 2018

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

• 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.