1.A rare case of childhood-onset systemic lupus erythematosus associated end-stage renal disease with cerebral abscess and hemorrhage
Jee Hyun KIM ; Jae Il SHIN ; Ji Hong KIM ; Keum Hwa LEE
Childhood Kidney Diseases 2024;28(1):44-50
Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, a cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.
2.Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children presenting orthostatic proteinuria
Eun Jae HWANG ; Ji Hong KIM ; Mi-Jung LEE ; Haesung YOON ; Jae Il SHIN ; Keum Hwa LEE
Childhood Kidney Diseases 2024;28(2):74-79
Purpose:
To compare the Doppler sonographic findings of the left renal vein (LRV) of children diagnosed with nutcracker syndrome with and without orthostatic proteinuria.
Methods:
Fifty and 53 consecutive children with and without orthostatic proteinuria, respectively, underwent renal Doppler ultrasonography examinations. The peak velocity (PV) was measured at the hilar portion of the LRV and between the aorta and superior mesenteric artery. Renal Doppler ultrasonography findings and clinical data including urine protein-to-creatinine ratio (UPCR) were compared according to the presence or absence of orthostatic proteinuria.
Results:
Between the two groups, no significant differences were observed in terms of age or sex. The PV ratio between the aortomesenteric and hilar portions was 7.79±2.65 and 6.32±3.01 in children with and without orthostatic proteinuria, respectively (P=0.009). No significant differences were observed between the two groups in terms of the UPCR in the first morning urine sample. However, the UPCR in the afternoon urine sample was significantly higher in children with orthostatic proteinuria than in those without orthostatic proteinuria (0.49±0.46 vs. 0.11±0.04 mg/mg, P<0.001). Furthermore, the PV ratio between the aortomesenteric and hilar portions revealed a positive correlation with the ratio of UPCR of the afternoon and that of first morning urine samples (R=0.21, P=0.034).
Conclusions
This study suggests that there can be a significant correlation of the PV ratio between the aortomesenteric and hilar portion of the LRV with orthostatic proteinuria in pediatric patients with nutcracker syndrome.
3.C3 glomerulonephritis with genetically confirmed C3 deficiency in a pediatric patient: a case report
Hae Min KIM ; Jae Il SHIN ; Ji Hong KIM ; Jiyoung OH ; Ji-Man KANG ; Hee Gyung KANG ; Seong Heon KIM ; Byoung Soo CHO ; Keum Hwa LEE
Childhood Kidney Diseases 2024;28(3):124-130
Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.
4.Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD
Na Ri KANG ; Yo Han AHN ; Eujin PARK ; Keum Hwa LEE ; Hee Sun BAEK ; Seong Heon KIM ; Heeyeon CHO ; Min Hyun CHO ; Jae Il SHIN ; Joo Hoon LEE ; Hae Il CHEONG ; Hee Gyung KANG ; Young Seo PARK ; Il-Soo HA ; Duk-Soo MOON ; Kyoung Hee HAN
Journal of Korean Medical Science 2021;36(20):e138-
Background:
Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD.
Methods:
Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years).
Results:
The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs.
Conclusion
On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.
5.Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD
Na Ri KANG ; Yo Han AHN ; Eujin PARK ; Keum Hwa LEE ; Hee Sun BAEK ; Seong Heon KIM ; Heeyeon CHO ; Min Hyun CHO ; Jae Il SHIN ; Joo Hoon LEE ; Hae Il CHEONG ; Hee Gyung KANG ; Young Seo PARK ; Il-Soo HA ; Duk-Soo MOON ; Kyoung Hee HAN
Journal of Korean Medical Science 2021;36(20):e138-
Background:
Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD.
Methods:
Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years).
Results:
The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs.
Conclusion
On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.
6.A Comprehensive Review of Coronavirus Disease 2019: Epidemiology, Transmission, Risk Factors, and International Responses
Han LI ; Seung Won BURM ; Sung Hwi HONG ; Ramy Abou GHAYDA ; Andreas KRONBICHLER ; Lee SMITH ; Ai KOYANAGI ; Louis JACOB ; Keum Hwa LEE ; Jae Il SHIN
Yonsei Medical Journal 2021;62(1):1-11
Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has caused a worldwide pandemic. The first reports of patients with COVID-19 were provided to World Health Organization on December 21, 2019 and were presumably associated with seafood markets in Wuhan, China. As of October 25, 2020, more than 42 million cases have been confirmed worldwide, with more than 1.1 million deaths. Asymptomatic transmission contributes significantly to transmission, and clinical features are non-specific to the disease. Thus, the diagnosis of COVID-19 requires specific viral RNA testing. The disease demonstrates extensive human-to-human transmissibility and has infected healthcare workers at high rates. Clinical awareness of the epidemiology and the risk factors for nosocomial transmission of COVID-19 is essential to preventing infection. Moreover, effective control measures should be further identified by comprehensive evaluation of hospital and community responses. In this review, we provide a comprehensive update on the epidemiology, presentation, transmission, risk factors, and public health measures associated with COVID-19. We also review past insights from previous coronavirus epidemics [i.e., severe acute respiratory syndrome (SARS) and Middle East respiratory syndrome (MERS)] to suggest measures to reduce transmission.
7.The Development of Evaluation Methods for Outcomes in Medical Humanities Curriculum of a Medical School
Hye-Jin PARK ; Sun-Young KWON ; Dong-Yoon KEUM ; Dae-Hyun KIM ; Dong-Eun KIM ; Jae-Bum KIM ; Jin-Hee KIM ; Won-Ki BAEK ; Jung-Sook HA ; Il-Seon HWANG ; Jung-Jeung LEE ; Ae-Hwa LEE ; Seon-Kyoung KIM ; Ha-Young JUNG ; Won-Kyun PARK
Keimyung Medical Journal 2021;40(2):77-97
This study was performed to select the proper assessing methods for learning outcomes in undergraduate education of medical humanities (MH), and to evaluate whether student assessments in MH curricula are related to the graduate outcomes (GO)and/or periodic phase outcomes (PO). We searched the reasonable assessing methods for GO and PO of MH curricula of Keimyung University School of Medicine (KUSM). The outcomes are composed of six competencies including patient care, communication, patient support, professionalism, problem solving and research, and self-development. Then, we analyzed whether student assessments carried out during formal MH curricula properly achieved their PO, furthermore their GO. Four competencies including communication, patient support, professionalism, self-development were lightened to be closely related to outcomes for MH. Only the component of problem solving was settled to be related to MH in the competency of problem solving and research. The competency of patient care was excluded from the relationship with MH. The assessing methods for the GO and three PO recommended from educational experts, and there were various available assessing methods based on medical situations and clinical contexts including direct observation of clinical skills, 360 degree feedback, peer review, self-assessment, project-based assessment, portfolio-based assessment, discussion & presentation-based assessment, log-based assessment. For the outcome-achieving from formal MH curricula, the MH programs of phase-1 (1st and 2nd grades) almost accomplished the PO of communication, patient supporting and professionalism, and considerably accomplished the PO of problem solving and self-development. The MH programs of phase-2 (3rd and 4th grades) accomplished considerably their PO as the competencies of professionalism and problem solving, and partially as communication, patient supporting and self-development. However, as only one program, public health law, was provided for MH program in phase-3 (5th and 6th grades), the extra methods to evaluate their MH outcomes are needed. Many assessing methods can be available for the most MH competencies consisting of the GO of KUSM, and the proper assessing methods for each MH competency should be selected based on programs and learning contexts in MH education. While formal MH curricula of the school variously accomplished the MH competencies of GO according to periodic phases of curricula, it is recommended to enhance the feasibility and effectiveness of evaluation for GO in MH curricula of the school.
8.Characteristics of pediatric rhabdomyolysis and the associated risk factors for acute kidney injury: a retrospective multicenter study in Korea
Sukdong YOO ; Min Hyun CHO ; Hee Sun BAEK ; Ji Yeon SONG ; Hye Sun LEE ; Eun Mi YANG ; Kee Hwan YOO ; Su Jin KIM ; Jae Il SHIN ; Keum Hwa LEE ; Tae-Sun HA ; Kyung Mi JANG ; Jung Won LEE ; Kee Hyuck KIM ; Heeyeon CHO ; Mee Jeong LEE ; Jin-Soon SUH ; Kyoung Hee HAN ; Hye Sun HYUN ; Il-Soo HA ; Hae Il CHEONG ; Hee Gyung KANG ; Mee Kyung NAMGOONG ; Hye-Kyung CHO ; Jae-Hyuk OH ; Sang Taek LEE ; Kyo Sun KIM ; Joo Hoon LEE ; Young Seo PARK ; Seong Heon KIM
Kidney Research and Clinical Practice 2021;40(4):673-686
Background:
The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases.
Methods:
This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016.
Results:
Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels.
Conclusions
Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.
9.A Review of Pre-Exposure Prophylaxis Adherenceamong Female Sex Workers
Ramy Abou GHAYDA ; Sung Hwi HONG ; Jae Won YANG ; Gwang Hun JEONG ; Keum Hwa LEE ; Andreas KRONBICHLER ; Marco SOLMI ; Brendon STUBBS ; Ai KOYANAGI ; Louis JACOB ; Hans OH ; Jong Yeob KIM ; Jae Il SHIN ; Lee SMITH
Yonsei Medical Journal 2020;61(5):349-358
Globally and in Africa specifically, female sex workers (FSWs) are at an extraordinarily high risk of contracting human immunodeficiencyvirus (HIV). Pre-exposure prophylaxis (PrEP) has emerged as an effective and ethical method with which to preventHIV infection among FSWs. PrEP efficacy is, however, closely linked to adherence, and adherence to PrEP among FSWs is a complexand interrelated process that has been shown to be of importance to public health policies and HIV control and interventionprograms. This comprehensive review categorizes barriers to and facilitators of adherence to HIV PrEP for FSWs, and describes fivestrategies for promoting PrEP adherence among FSWs. These strategies encompass 1) a long-term educational effort to decreasethe stigma associated with sex work and PrEP use, 2) education on how PrEP works, 3) lifestyle modification, 4) research on nextgenerationPrEP products to address the inconvenience of taking daily pills, and 5) integration of PrEP into existing services, suchas social services and routine primary care visits, to reduce the economic burden of seeking the medication. Our review is expectedto be useful for the design of future PrEP intervention programs. Multidisciplinary intervention should be considered to promotePrEP adherence among FSWs in order to help control the HIV epidemic.
10.Expression of Phospholipase A2 Receptor in PediatricHepatitis B Virus-Related Membranous Nephropathy
Sung-Eun CHOI ; Yoon Sung BAE ; Keum Hwa LEE ; Jae Il SHIN ; Hyeon Joo JEONG ; Beom Jin LIM
Childhood Kidney Diseases 2020;24(1):36-41
Purpose:
Hepatitis B virus (HBV) infection is among etiologies of secondarymembranousnephropathy (MN) in pediatric patients. We evaluated expressionof phospholipase A2 receptor (PLA2R), a specific target antigen of primary MN, inpediatric HBV-related MN.
Methods:
We retrospectively reviewed patients with biopsy-proven HBV-relatedMN from the renal biopsy registry and electronic medical records of SeveranceHospital, Seoul, Korea, from 1993 to 2004. Paraffin-embedded human kidneytissues were retrieved and immunohistochemically stained for PLA2R.
Results:
Ten pediatric patients with 13 biopsied specimens were reviewed. Thepredominant pathological stage was stage II–III, and second was stage II. Theintensity of staining for IgG was greatest, with less intense staining for IgM, IgA,C3, C4, and C1q. All the patients had angiotensin-converting enzyme inhibitorcombined with glucocorticoid, and four patients converted to cyclosporine treatmentfrom glucocorticoid monotherapy. Urinalysis of all the patients normalizedafter variable period. PLA2R staining was demonstrated in the outer glomerulus in3 out of 13 biopsies, 2 of which were obtained from the same patient over a 5-yearinterval.
Conclusions
PLA2R was expressed in a small number of cases diagnosed aspediatricHBV-related MN, indicating that some HBV-related MN cases may beprimary MN concurrent with HBV infection.

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