1.To treat or not to treat: CUBN-associated persistent proteinuria
Yun Young CHOI ; Yo Han AHN ; Eujin PARK ; Ji Hyun KIM ; Hee Gyung KANG ; Hyun Kyung LEE
Kidney Research and Clinical Practice 2024;43(5):663-670
Persistent proteinuria is an important indicator of kidney damage and requires active evaluation and intervention. However, tubular proteinuria of genetic origin typically does not improve with immunosuppression or antiproteinuric treatment. Recently, defects in CUBN were found to cause isolated proteinuria (mainly albuminuria) due to defective tubular albumin reuptake. Unlike most other genetically caused persistent albuminuria, CUBN C-terminal variants have a benign course without progression to chronic kidney disease according to the literature. Here, we present Korean cases with persistent proteinuria associated with C-terminal variants of CUBN. Methods: We identified Korean patients with CUBN variants among those with an identified genetic cause of proteinuria and evaluated their clinical features and clinical course. We also reviewed the literature on CUBN-associated isolated proteinuria published to date and compared it with Korean patients. Results: All patients presented with incidentally found, asymptomatic isolated proteinuria at a median age of 5 years. The proteinuria was in the subnephrotic range and did not significantly change over time, regardless of renin- angiotensin system inhibition. Initial physical examination, laboratory findings, and kidney biopsy results, when available, were unremarkable other than significant proteinuria. All patients maintained kidney function throughout the follow-up duration. All patients had at least one splicing mutation, and most of the variants were located C-terminal side of the gene. Conclusion: We report Korean experience of CUBN-related benign proteinuria, that aligns with previous reports, indicating that this condition should be considered in cases with incidentally found asymptomatic isolated proteinuria, especially in young children.
2.Asymptomatic hematuria in children: Korean Society of Pediatric Nephrology recommendations for diagnosis and management
Eujin PARK ; Sang Woon KIM ; Su Jin KIM ; Minki BAEK ; Yo Han AHN ; Eun Mi YANG ; Myung Hyun CHO ; Hyun Kyung LEE ; Kyoung Hee HAN ; Yae Lim KIM ; Miyoung CHOI ; Hee Gyung KANG ; Jin-Soon SUH ;
Kidney Research and Clinical Practice 2024;43(5):565-574
Hematuria is a relatively common condition among school-aged children. Because international guidelines for asymptomatic hematuria in children are unavailable, developing practical guidelines for the diagnosis and management of asymptomatic hematuria based on scientific evidence while considering real-world practice settings, values, and patient and physician preferences is essential. The Korean Society of Pediatric Nephrology developed clinical guidelines to address key questions regarding the diagnosis and management of asymptomatic hematuria in children.
3.To treat or not to treat: CUBN-associated persistent proteinuria
Yun Young CHOI ; Yo Han AHN ; Eujin PARK ; Ji Hyun KIM ; Hee Gyung KANG ; Hyun Kyung LEE
Kidney Research and Clinical Practice 2024;43(5):663-670
Persistent proteinuria is an important indicator of kidney damage and requires active evaluation and intervention. However, tubular proteinuria of genetic origin typically does not improve with immunosuppression or antiproteinuric treatment. Recently, defects in CUBN were found to cause isolated proteinuria (mainly albuminuria) due to defective tubular albumin reuptake. Unlike most other genetically caused persistent albuminuria, CUBN C-terminal variants have a benign course without progression to chronic kidney disease according to the literature. Here, we present Korean cases with persistent proteinuria associated with C-terminal variants of CUBN. Methods: We identified Korean patients with CUBN variants among those with an identified genetic cause of proteinuria and evaluated their clinical features and clinical course. We also reviewed the literature on CUBN-associated isolated proteinuria published to date and compared it with Korean patients. Results: All patients presented with incidentally found, asymptomatic isolated proteinuria at a median age of 5 years. The proteinuria was in the subnephrotic range and did not significantly change over time, regardless of renin- angiotensin system inhibition. Initial physical examination, laboratory findings, and kidney biopsy results, when available, were unremarkable other than significant proteinuria. All patients maintained kidney function throughout the follow-up duration. All patients had at least one splicing mutation, and most of the variants were located C-terminal side of the gene. Conclusion: We report Korean experience of CUBN-related benign proteinuria, that aligns with previous reports, indicating that this condition should be considered in cases with incidentally found asymptomatic isolated proteinuria, especially in young children.
4.Asymptomatic hematuria in children: Korean Society of Pediatric Nephrology recommendations for diagnosis and management
Eujin PARK ; Sang Woon KIM ; Su Jin KIM ; Minki BAEK ; Yo Han AHN ; Eun Mi YANG ; Myung Hyun CHO ; Hyun Kyung LEE ; Kyoung Hee HAN ; Yae Lim KIM ; Miyoung CHOI ; Hee Gyung KANG ; Jin-Soon SUH ;
Kidney Research and Clinical Practice 2024;43(5):565-574
Hematuria is a relatively common condition among school-aged children. Because international guidelines for asymptomatic hematuria in children are unavailable, developing practical guidelines for the diagnosis and management of asymptomatic hematuria based on scientific evidence while considering real-world practice settings, values, and patient and physician preferences is essential. The Korean Society of Pediatric Nephrology developed clinical guidelines to address key questions regarding the diagnosis and management of asymptomatic hematuria in children.
5.Dental Age Estimation in Children Using Convolution Neural Network Algorithm: A Pilot Study
Byung-Yoon ROH ; Hyun-Jeong PARK ; Kyung-Ryoul KIM ; In-Soo SEO ; Yeon-Ho OH ; Ju-Heon LEE ; Chang-Un CHOI ; Yo-Seob SEO ; Ji-Won RYU ; Jong-Mo AHN
Journal of Oral Medicine and Pain 2024;49(4):118-123
Purpose:
Recently, deep learning techniques have been introduced for age estimation, with automated methods based on radiographic analysis demonstrating high accuracy. In this study, we applied convolutional neural network (CNN) techniques to the lower dentition area on orthopantomograms (OPGs) of children to develop an automated age estimation model and evaluate its accuracy for use in forensic dentistry.
Methods:
In this study, OPGs of 2,856 subjects aged 3-14 years were analyzed. The You Only Look Once (YOLO) V8 object detection technique was applied to extract the mandibular dentition area on OPGs, designating it as the region of interest (ROI). First, 200 radiographs were randomly selected, and were used to train a model for extracting the ROI. The trained model was then applied to the entire dataset. For the CNN image classification task, 80% of OPGs were allocated to the training set, while the remaining 20% were used as the test set. A transfer learning approach was employed using the ResNet50 and VGG19 backbone models, with an ensemble technique combining these models to improve performance. The mean absolute error (MAE) on the test set was used as the validation metric, and the model with the lowest MAE was selected.
Results:
In this study, the age estimation model developed using mandibular dentition region from OPGs achieved MAE and root mean squared error (RMSE) values of 0.501 and 0.742, respectively, on the test set, and MAE and RMSE values of 0.273 and 0.354, respectively, on the training set.
Conclusions
The automated age estimation model developed in this study demonstrated accuracy comparable to that of previous research and shows potential for applications in forensic investigations. Increasing the sample size and incorporating diverse deep learning techniques are expected to further enhance the accuracy of future age estimation models.
6.To treat or not to treat: CUBN-associated persistent proteinuria
Yun Young CHOI ; Yo Han AHN ; Eujin PARK ; Ji Hyun KIM ; Hee Gyung KANG ; Hyun Kyung LEE
Kidney Research and Clinical Practice 2024;43(5):663-670
Persistent proteinuria is an important indicator of kidney damage and requires active evaluation and intervention. However, tubular proteinuria of genetic origin typically does not improve with immunosuppression or antiproteinuric treatment. Recently, defects in CUBN were found to cause isolated proteinuria (mainly albuminuria) due to defective tubular albumin reuptake. Unlike most other genetically caused persistent albuminuria, CUBN C-terminal variants have a benign course without progression to chronic kidney disease according to the literature. Here, we present Korean cases with persistent proteinuria associated with C-terminal variants of CUBN. Methods: We identified Korean patients with CUBN variants among those with an identified genetic cause of proteinuria and evaluated their clinical features and clinical course. We also reviewed the literature on CUBN-associated isolated proteinuria published to date and compared it with Korean patients. Results: All patients presented with incidentally found, asymptomatic isolated proteinuria at a median age of 5 years. The proteinuria was in the subnephrotic range and did not significantly change over time, regardless of renin- angiotensin system inhibition. Initial physical examination, laboratory findings, and kidney biopsy results, when available, were unremarkable other than significant proteinuria. All patients maintained kidney function throughout the follow-up duration. All patients had at least one splicing mutation, and most of the variants were located C-terminal side of the gene. Conclusion: We report Korean experience of CUBN-related benign proteinuria, that aligns with previous reports, indicating that this condition should be considered in cases with incidentally found asymptomatic isolated proteinuria, especially in young children.
7.Asymptomatic hematuria in children: Korean Society of Pediatric Nephrology recommendations for diagnosis and management
Eujin PARK ; Sang Woon KIM ; Su Jin KIM ; Minki BAEK ; Yo Han AHN ; Eun Mi YANG ; Myung Hyun CHO ; Hyun Kyung LEE ; Kyoung Hee HAN ; Yae Lim KIM ; Miyoung CHOI ; Hee Gyung KANG ; Jin-Soon SUH ;
Kidney Research and Clinical Practice 2024;43(5):565-574
Hematuria is a relatively common condition among school-aged children. Because international guidelines for asymptomatic hematuria in children are unavailable, developing practical guidelines for the diagnosis and management of asymptomatic hematuria based on scientific evidence while considering real-world practice settings, values, and patient and physician preferences is essential. The Korean Society of Pediatric Nephrology developed clinical guidelines to address key questions regarding the diagnosis and management of asymptomatic hematuria in children.
8.To treat or not to treat: CUBN-associated persistent proteinuria
Yun Young CHOI ; Yo Han AHN ; Eujin PARK ; Ji Hyun KIM ; Hee Gyung KANG ; Hyun Kyung LEE
Kidney Research and Clinical Practice 2024;43(5):663-670
Persistent proteinuria is an important indicator of kidney damage and requires active evaluation and intervention. However, tubular proteinuria of genetic origin typically does not improve with immunosuppression or antiproteinuric treatment. Recently, defects in CUBN were found to cause isolated proteinuria (mainly albuminuria) due to defective tubular albumin reuptake. Unlike most other genetically caused persistent albuminuria, CUBN C-terminal variants have a benign course without progression to chronic kidney disease according to the literature. Here, we present Korean cases with persistent proteinuria associated with C-terminal variants of CUBN. Methods: We identified Korean patients with CUBN variants among those with an identified genetic cause of proteinuria and evaluated their clinical features and clinical course. We also reviewed the literature on CUBN-associated isolated proteinuria published to date and compared it with Korean patients. Results: All patients presented with incidentally found, asymptomatic isolated proteinuria at a median age of 5 years. The proteinuria was in the subnephrotic range and did not significantly change over time, regardless of renin- angiotensin system inhibition. Initial physical examination, laboratory findings, and kidney biopsy results, when available, were unremarkable other than significant proteinuria. All patients maintained kidney function throughout the follow-up duration. All patients had at least one splicing mutation, and most of the variants were located C-terminal side of the gene. Conclusion: We report Korean experience of CUBN-related benign proteinuria, that aligns with previous reports, indicating that this condition should be considered in cases with incidentally found asymptomatic isolated proteinuria, especially in young children.
9.Asymptomatic hematuria in children: Korean Society of Pediatric Nephrology recommendations for diagnosis and management
Eujin PARK ; Sang Woon KIM ; Su Jin KIM ; Minki BAEK ; Yo Han AHN ; Eun Mi YANG ; Myung Hyun CHO ; Hyun Kyung LEE ; Kyoung Hee HAN ; Yae Lim KIM ; Miyoung CHOI ; Hee Gyung KANG ; Jin-Soon SUH ;
Kidney Research and Clinical Practice 2024;43(5):565-574
Hematuria is a relatively common condition among school-aged children. Because international guidelines for asymptomatic hematuria in children are unavailable, developing practical guidelines for the diagnosis and management of asymptomatic hematuria based on scientific evidence while considering real-world practice settings, values, and patient and physician preferences is essential. The Korean Society of Pediatric Nephrology developed clinical guidelines to address key questions regarding the diagnosis and management of asymptomatic hematuria in children.
10.Hematuria in children: causes and evaluation
Eujin PARK ; Sang Woon KIM ; Su Jin KIM ; Minki BAEK ; Yo Han AHN ; Myung Hyun CHO ; Hyun Kyung LEE ; Kyoung Hee HAN ; Yae Lim KIM ; Miyoung CHOI ; Hee Gyung KANG ; Jin-Soon SUH ; Eun Mi YANG ;
Childhood Kidney Diseases 2024;28(2):66-73
Hematuria is the presence of blood in the urine and is classified as either gross hematuria or microscopic hematuria. There are many causes of hematuria, and the differential diagnosis depends on the presence or absence of comorbidities and whether it is glomerular or non-glomerular. When hematuria in children is symptomatic or persistent, an evaluation of the cause is essential. The causes of hematuria and basic approaches to its diagnosis are discussed in this review.

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