• Childhood Kidney Diseases
• /
• 제26권1호
• /
• pp.31-39
• /
• 2022

Alport syndrome (AS) is a progressive hereditary nephritis that is often accompanied by sensorineural hearing loss and ocular abnormalities. It is inherited in three modes of X-linked AS (XLAS), autosomal recessive AS (ARAS), and autosomal dominant AS (ADAS). XLAS is caused by pathogenic variants in COL4A5, while ARAS and ADAS are caused by those in COL4A3 or COL4A4. There is currently no curative treatment for AS; however, angiotensin-converting enzyme inhibitors (ACEi) can improve the outcome of AS. In the past decade, multiple studies have shown that early intervention with ACEi upon isolated microscopic hematuria or microalbuminuria could delay disease progression, and early diagnosis is crucial for early treatment. Therefore, a new classification of AS based on molecular diagnoses has been proposed, including the paradigm shift of re-classifying female "carriers" to "patients" and "thin basement membrane nephropathy" to "ADAS." In addition, with the detection of COL4A mutations in some patients with biopsy-confirmed IgA nephropathy, focal segmental glomerulosclerosis, and chronic kidney disease of unknown origin, it is suggested that the phenotype of AS should be expanded. In this review, we highlight the landmark studies and guidelines published over the past decade and introduce strategies for early diagnosis and treatment to improve the outcomes of AS.

• Asian Pacific Journal of Cancer Prevention
• /
• 제16권17호
• /
• pp.7603-7606
• /
• 2015

Background: The chemotherapeutic agent oxaliplatin can cause acute and chronic forms of peripheral neuropathy. The aim of this study was to evaluate the incidence of chronic neuropathy and its risk factors in colorectal cancer (CRC) patients treated with FOLFOX or XELOX regimens in the Oncology Ward of Hazrate-Rasoul Hospital in Tehran. Materials and Methods: A total of 130 patients with CRC were entered into our study, aged over 18 years, without history of receiving other neurotoxic agents or other predisposing factors such as diabetes or neurologic diseases and kidney and liver dysfunction. For the FOLFOX regimen, patients received oxaliplatin, 85mg/m2, every 2 weeks for 12 courses and with the XELOX regimen, oxaliplatin was $130mg/m^2$, every 3 weeks for 8 courses. Based on Common Toxicity Criteria (CTC or NCI-CTC v.3), the patients were divided into 5 groups (grades) based on the severity of their symptoms. Results: Fifty-seven patients (43.8%) were male and 73(56.2%) female. Some 19 patients (14.7%) had BMI<20, 97(74.6%) were between 20-25 and 14 (10.8%) ${\geq}25$. In 105 patients (80.7%) neuropathy was found. There was significant correlation between BMI, hypomagnesaemia and especially, severity of anemia in patients with neuropathy compared to those without. Conclusions: Oxaliplatin regimens can induce chronic neuropathy in CRC patients, with anemia, high BMI and hypomagnesaemia as risk factors that can predispose to this kind of neurotoxicity.

• Clinical and Experimental Pediatrics
• /
• 제57권3호
• /
• pp.135-139
• /
• 2014

Purpose: Adult Korean patients on chronic dialysis have a 9-year survival rate of 50%, with cardiovascular problems being the most significant cause of death. The 2011 annual report of the North American Pediatric Renal Trials and Collaborative Studies group reported 3-year survival rates of 93.4% and relatively poorer survival in younger patients. Methods: In this study, we have reviewed data from Korean Pediatric Chronic Kidney Disease Registry from 2002 to 2010 to assess survival rates and causes of death in Korean children on chronic dialysis. Results: The overall estimated patient survival rates were 98.4%, 94.4%, and 92.1% at 1, 3, and 5 years, respectively. No significant difference was observed in survival rates between patients on peritoneal dialysis and those on hemodialysis. Patients for whom dialysis was initiated before 2 years of age (n=40) had significantly lower survival rates than those for whom dialysis was initiated at 6-11 years of age (n=140). In all, 26 patients had died; the mortality rate was 19.9 per 1,000 patient years. The most common causes of death were infections and comorbidities such as malignancy and central nervous system (CNS) or liver diseases. Conclusion: The outcomes observed in this study were better than those observed in adults and comparable to those observed in pediatric studies in other countries. To improve the outcomes of children on chronic dialysis, it is necessary to prevent dialysis-related complications such as infection, congestive heart failure, or CNS hemorrhage and best control treatable comorbidities.

• Journal of Chest Surgery
• /
• 제56권5호
• /
• pp.304-310
• /
• 2023

Background: The late progression of tricuspid regurgitation (TR) after mitral valve surgery is well known. However, few reports have described the progression of TR after aortic valve surgery. We investigated the incidence of and risk factors for the development of significant TR after isolated aortic valve replacement (AVR). Methods: This study analyzed patients with less than moderate TR who underwent isolated AVR at Seoul National University Hospital from January 1990 to December 2018. Significant TR was defined as moderate or higher. Echocardiographic follow-up was performed in all patients. The Fine-Gray model was used to identify clinical risk factors for the development of significant TR. Results: In total, 583 patients (61.7±14.2 years old) were included. Operative mortality occurred in 9 patients (1.5%), and the overall survival rates at 10, 20, and 25 years were 91.1%, 83.2%, and 78.9%, respectively. Sixteen patients (2.7%) developed significant TR during the follow-up period (13 moderate; 3 severe). The cumulative incidence of significant TR at 10, 20, and 25 years was 0.77%, 3.83%, and 6.42%, respectively. No patients underwent reoperation or reintervention of the tricuspid valve. Hemodialysis or peritoneal dialysis for chronic kidney disease (hazard ratio [HR], 5.188; 95% confidence interval [CI], 1.154-23.322) and preoperative mild TR (HR, 5.919; 95% CI, 2.059-17.017) were associated with the development of significant TR in the multivariable analysis. Conclusion: TR progression after isolated AVR in patients with less than moderate TR is rare. Preoperative mild TR and hemodialysis or peritoneal dialysis for chronic kidney disease were significant risk factors for the development of TR.

• Childhood Kidney Diseases
• /
• 제27권1호
• /
• pp.34-39
• /
• 2023

Purpose: This article was to collect data on the safety of coronavirus disease 2019 (COVID-19) vaccines in children with underlying medical conditions. Methods: We constructed a prospective cohort of children and adolescents aged 5 to 19 years who had received at least one dose of COVID-19 vaccine. Patients diagnosed with and treated for chronic kidney disease, autoimmune disease, or other chronic conditions at the Seoul National University Children's Hospital were recruited from June to December 2022. A mobile survey questionnaire was sent to their guardians. The presence of adverse events on the day (day 0), 3 weeks (day 21), and 6 months (day 180) after the 1st dose of COVID-19 vaccine was recorded by the guardians. Results: A total of 73 children participated. The median age was 14 years, and 64.4% of the patients were male. On the day of immunization, 65.8% of the patients reported at least one adverse event. Pain at the injection site, fatigue, headache, arthralgia, and myalgia were the most common symptoms. The prevalence of adverse events decreased over time (65.8% on day 0, 27.4% between days 0 and 21, and 24.6% between days 21 and 180). Severe acute respiratory syndrome coronavirus 2 infection after the 1st dose occurred in 17 patients (23.3%) and one of the patients (5.88%) was hospitalized due to infection. Conclusions: Adverse events after COVID-19 vaccination were generally mild in children and adolescents with underlying medical conditions. Our findings provide evidence for the safety of COVID-19 vaccination in the vulnerable pediatric population.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제16권4호
• /
• pp.207-218
• /
• 2013

Long term outcomes after liver transplantation are major determinants of quality of life and of the value of this heroic treatment. As short term outcomes are excellent, our community is turning to take a harder look at long term outcomes. The purpose of this paper is to review these outcomes, and highlight proposed treatments, as well as pressing topics needing to be studied. A systemic review of the English literature was carried in PubMed, covering all papers addressing long term outcomes in pediatric liver transplant from 2000-2013. Late outcomes after pediatric liver transplant affect the liver graft in the form of chronic liver dysfunction. The causes include rejection particularly humoral rejection, but also de novo autoimmune hepatitis, and recurrent disease. The metabolic syndrome is a major factor in long term cardiovascular complication risk. Secondary infections, kidney dysfunction and malignancy remain a reality of those patients. There is growing evidence of late cognitive and executive function delays affecting daily life productivity as well as likely adherence. Finally, despite a good health status, quality of life measures are comparable to those of children with chronic diseases. Long term outcomes are the new frontier in pediatric liver transplantation. Much is needed to improve graft survival, but also to avoid systemic morbidities from long term immunosuppression. Quality of life is a new inclusive measure that will require interventions and innovative approaches respectful not only on the patients but also of their social circle.

• Journal of Yeungnam Medical Science
• /
• 제37권4호
• /
• pp.269-276
• /
• 2020

Fibrosis is characterized by excessive accumulation of extracellular matrix components. The fibrotic process ultimately leads to organ dysfunction and failure in chronic inflammatory and metabolic diseases such as pulmonary fibrosis, advanced kidney disease, and liver cirrhosis. Idiopathic pulmonary fibrosis (IPF) is a common form of progressive and chronic interstitial lung disease of unknown etiology. Pathophysiologically, the parenchyma of the lung alveoli, interstitium, and capillary endothelium becomes scarred and stiff, which makes breathing difficult because the lungs have to work harder to transfer oxygen and carbon dioxide between the alveolar space and bloodstream. The transforming growth factor beta (TGF-β) signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis and scarring of the lung tissue. Recent clinical trials focused on the development of pharmacological agents that either directly or indirectly target kinases for the treatment of IPF. Therefore, to develop therapeutic targets for pulmonary fibrosis, it is essential to understand the key factors involved in the pathogenesis of pulmonary fibrosis and the underlying signaling pathway. The objective of this review is to discuss the role of kinase signaling cascades in the regulation of either TGF-β-dependent or other signaling pathways, including Rho-associated coiled-coil kinase, c-jun N-terminal kinase, extracellular signal-regulated kinase 5, and p90 ribosomal S6 kinase pathways, and potential therapeutic targets in IPF.

• 동아시아식생활학회지
• /
• 제11권1호
• /
• pp.1-10
• /
• 2001

Since the Industrial Revolution, people have enjoyed an abundant life, owing to the technological innovations of science. However, because of changes in the environment or polution to the environment, it has brought on many chronic diseases. Especially, even though it may be a small amount, if cadmium gets into a human body, because its biological half life is long, it fatally causes a kidney disease and damage to human organisms. It was reported that Metallothioneins(MT), a substance that is closely related with a free radical that comes from environmental pollution and the course of which cadmium, which causes chronic addition in the body, is formed, along with harmful metals, make the toxicity reduce. MT compound led into the body by cadmium indirectly functions as an antioxidation, supplied adequate amount of vitamin E, and suppresses the accumulation of cadmium in heart, liver and blood. Cadmium content found in Korean soil according to the test conducted from 1992 to 1996 was 0.02~0.03mg/kg in cereals, pulses, and potatoes. Free radical scavenger, finding solution for metal substance that comes from environmental pollution from food and natural substances implicates a better future for the study of food science.

• Childhood Kidney Diseases
• /
• 제5권2호
• /
• pp.164-175
• /
• 2001

목 적 ; Alport 증후군은 1927년 Alport에 의해 처음 보고된 유전성 신질환으로 전신적으로 기저막에 영향을 미쳐 계속적으로 진행하는 신질환과 감각신경성 난청, 눈의 이상, 전자현미경상 특징적 소견, 그리고 대부분의 경우 가족력 동반을 특징으로 한다. 저자들은 Alport 증후군 환아의 임상적 특징을 중심으로 관찰하여 이 질환의 예후에 관련된 위험요인을 알아보고자 후향적 조사를 시행하였다. 대상 및 방법 : 1980년 1월부터 1999년 12월까지 20년동안 소아과에서 시행한 신장조직검사에서 Alport 증후군으로 진단 후 추적관찰이 가능하였던 환아 24명을 대상으로 하였다. 처음 내원 당시와 현재의 신장기능을 비교하여 현재까지 정상의 신장기능을 유지하고 있는 군(Group I)과 만성신부전으로 진행한 군(Group II)으로 나누어 Group I과 Group II간의 여러 가지 임상양상을 비교하였으며 통계학적 방법으로는 비모수검정법을 이용하였다. 결 과 : 24명의 환아 중 남녀의 비는 3:1이였고 신증상 발현시의 연령은 평균 $5.2{\pm}3.6세$였으며 내원 당시 나이는 평균 $7.8{\pm}4.4세$였다. 처음 내원 당시의 주증상은 육안적 혈뇨가 15예($62\%$)로 가장 많았고, 진단 당시의 환아들의 임상양상으로는 가족력이 있는 경우가 17예($70\%$), 육안적 혈뇨는 18예($75\%$), 단백뇨가 있었던 경우가 14예($58\%$), 부종은 14예($58\%$), 고혈압은 6예($25\%$)에서 관찰되었다. 전체 24명의 환아 중 만성신부전으로 진행된 경우는 11예($46\%$)였고, 이 중 말기신부전으로 진행되어 신장이식 후 관찰 중인 경우가 7예($29\%$) 있었다. 청각이상소견을 보인 경우는 12예($50\%$)였으며, 안과적 정밀검사상 이상소견을 보인 경우는 5예($21\%$)였다. 신증상 발현이후 계속해서 신장기능이 유지되고 있는 집단을 Group I(n=13), 만성신부전으로 진행한 집단을 Group II(n=11)라고 할 때, 이 두 집단 사이의 진단당시의 임상양상의 차이를 보면 고혈압, 단백뇨 및 부종의 빈도가 Group I에서 통계적으로 유의하게 증가되어 있으며, 임상병리검사 소견에서는 혈중 총단백량, 알부민, creatinine 청소율이 Group I에서 통계적으로 유의하게 감소되어 있고, BUN, creatinine은 통계적으로 유의하게 증가되었다. 결 론 : Alport 증후군 환아에서 임상적으로 유의미한 예후와 관련된 위험요인으로는 진단시의 고혈압, 부종, 단백뇨 유무 여부와 혈중 총단백량, 알부민, BUN, creatinine, 사구체 여과율이 중요하며, 이런 가능한 위험요인의 분석과 관찰이 Alport 증후군 환아의 치료와 예후 인지에 많은 도움이 되리라 사료된다.

• 적정기술학회지
• /
• 제6권2호
• /
• pp.163-173
• /
• 2020

ODA 사업을 성공적으로 수행하기 위해서는 수요 국가에서 취약하고 필요한 분야가 무엇인지 정확히 아는 것이 중요하다. 보건분야는 대부분의 개발 도상국에서 우선순위의 첫 번째를 차지하고 있다. 이 연구는 ODA 프로젝트 계획에 도움이 되고자 피지에서의 비전염병 질환(NCD) 상황을 소개하기 위해 수행되었다. 2016년 피지의 주요 사망 원인은 당뇨병, 허혈성심장병, 뇌혈관질환, 만성신장질환, 하기도감염, 천식 등이다. 같은 해 한국의 주요 사망 원인은 암, 허혈성심장병, 뇌혈관질환, 폐렴, 자살, 당뇨병 순이다. 비전염성 질환으로서의 만성 질환은 생활 습관 및 소비 패턴의 변화와 인구 고령화로 인해 지속적으로 증가하고 있다. 이러한 세계적인 추세는 피지와 한국에서도 명백하며 NCD의 치료 및 관리를위한 사망률 및 개인 비용 증가에 반영된다. 많은 연구에서 개별 환자에 맞춘 지속적이고 포괄적인 치료의 필요성이 제안되었으며, 이러한 치료를 제공하기 위해 NCD 환자를 관리하는 체계적인 프로그램 개발이 권장되었다. 피지 정부는 2015-2019 년 NCD 전략계획을 개발했으며 요인별로 NCD의 유병률을 낮추려고 노력해왔다. 국가 차원의 NCD 정책을 안내하는 WHO 글로벌 행동 계획은 지역 사회 수준의 NCD 예방 및 관리 모델을 필요로 한다. 이런 측면에서 ODA도 직접적인 지원만이 아니라 시스템관리 지원도 고려할 만하다.