Renal replacement therapy in neonates with an inborn error of metabolism

Cho, Heeyeon The Korean Pediatric Society 2019 Clinical and Experimental Pediatrics (CEP) Vol.62 No.2

Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates.

Pulmonary thromboembolism: a rare but life-threatening complication of nephrotic syndrome

Cho, Heeyeon The Korean Pediatric Society 2021 Clinical and Experimental Pediatrics (CEP) Vol.64 No.8

Complement regulation: physiology and disease relevance

Cho, Heeyeon The Korean Pediatric Society 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.7

The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation on foreign or modified self surfaces and inhibition on intact host cells. Complement regulators are essential for maintaining this balance and are classified as soluble regulators, such as factor H, and membrane-bound regulators. Defective complement regulators can damage the host cell and result in the accumulation of immunological debris. Moreover, defective regulators are associated with several autoimmune diseases such as atypical hemolytic uremic syndrome, dense deposit disease, age-related macular degeneration, and systemic lupus erythematosus. Therefore, understanding the molecular mechanisms by which the complement system is regulated is important for the development of novel therapies for complement-associated diseases.

Prevention of Pediatric Acute Kidney Injury

Cho, Heeyeon Korean Society of Pediatric Nephrology 2015 Childhood kidney diseases Vol.19 No.2

The incidence of acute kidney injury (AKI) in critically ill pediatric patients has been reported as increasing to 25 %, depending on population characteristics. The etiology of AKI has changed over the last 10-20 years from primary renal disease to the renal conditions associated with systemic illness. The AKI in pediatric population is associated with increased mortality and morbidity, and prevention is needed to reduce the consequence of AKI. It is known that the most important risk factors for AKI in critically ill pediatric patients are clinical conditions to be associated with decreased renal blood flow, direct renal injury, and illness severity. Renal hypoperfusion leads to neurohormonal activation including renin-angiotensin-aldosterone system, sympathetic nervous system, antidiuretic hormone, and prostaglandins. Prolonged renal hypoperfusion can result in acute tubular necrosis. The direct renal injury can be predisposed under the condition of renal hypoperfusion, and appropriate treatment of volume depletion is important to prevent AKI. The preventable causes of AKI include contrast-induced nephropathy, hemodynamic instability, inappropriate mediation use, and multiple nephrotoxic insults. Given the evidence of preventable factors for AKI, several actions such as the use of protocol for prevention of contrast-induced nephropathy, appropriate treatment of volume depletion, vigorous treatment of sepsis, avoidance of combinations of nephrotoxic medications, and monitoring of levels of drugs should be recommended.

Pediatric Hemodialysis

Cho, Heeyeon Korean Society of Pediatric Nephrology 2020 Childhood kidney diseases Vol.24 No.2

Hemodialysis is rarely used in neonates and infants due to the risk of major complications in the very young. Nevertheless, there are clinical situations where hemodialysis is needed and may be helpful in small children. Recently, new developments in specialized hemodialysis equipment and specifically trained personnel have made it possible to implement hemodialysis in neonates and infants. In this review, we will discuss hemodialysis for the treatment of small children with renal replacement therapy-requiring conditions, and consider indications, prescriptions, complications, and ethical issues.

A novel mutation of CLCNKB in a Korean patient of mixed phenotype of Bartter-Gitelman syndrome

Cho, Hee-Won,Lee, Sang Taek,Cho, Heeyeon,Cheong, Hae Il The Korean Pediatric Society 2016 Clinical and Experimental Pediatrics (CEP) Vol.59 No.no.sup1

Bartter syndrome (BS) is an inherited renal tubular disorder characterized by low or normal blood pressure, hypokalemic metabolic alkalosis, and hyperreninemic hyperaldosteronism. Type III BS is caused by loss-of-function mutations in CLCNKB encoding basolateral ClC-Kb. The clinical phenotype of patients with CLCNKB mutations has been known to be highly variable, and cases that are difficult to categorize as type III BS or other hereditary tubulopathies, such as Gitelman syndrome, have been rarely reported. We report a case of a 10-year-old Korean boy with atypical clinical findings caused by a novel CLCNKB mutation. The boy showed intermittent muscle cramps with laboratory findings of hypokalemia, severe hypomagnesemia, and nephrocalcinosis. These findings were not fully compatible with those observed in cases of BS or Gitelman syndrome. The CLCNKB mutation analysis revealed a heterozygous c.139G>A transition in exon 13 [p.Gly(GGG)465Glu(GAG)]. This change is not a known mutation; however, the clinical findings and in silico prediction results indicated that it is the underlying cause of his presentation.

Impact of Depressive Symptoms during the Second Trimester of Pregnancy on Maternal and Fetal Heart Rate Variability

( Innsun Park ),( Heeyeon Kim ),( Kyungun Jhung ),( Dukyong Yoon ),( Jihoon Seo ),( Jin Young Park ),( Hee Young Cho ) 대한산부인과학회 2023 대한산부인과학회 학술대회 Vol.109 No.-

Angiotensin receptor blocker induced fetopathy: two case reports and literature review

Jinwoon Joung,Heeyeon Cho 대한소아신장학회 2023 Childhood kidney diseases Vol.27 No.2

The administration of angiotensin type 2 receptor blockers (ARBs) during pregnancy is known to cause ARB fetopathy, including renal insufficiency. We aimed to analyze the outcomes of two patients who survived ARB fetopathy and perform an accompanying literature review. Case 1 was exposed antenatally from a gestational age of 30 weeks to valsartan because of maternal pregnancy-induced hypertension. The patient presented with oliguria immediately after birth, and renal replacement therapy was administered for 24 days. Seven years after birth, renal function was indicative of stage 2 chronic kidney disease (CKD) with impaired urinary concentration. Case 2 had a maternal history of hypertension and transient ischemic attack and was treated with olmesartan until 30 weeks of pregnancy. Renal replacement therapy was performed for 4 days since birth. After 8 years, the patient is with CKD stage 2, with intact tubular function. Recent reports suggest that ARB fetopathy might manifest as renal tubular dysgenesis and nephrogenic diabetes insipidus, in contrast to mild alterations of glomerular filtration. Tubular dysfunction may induce CKD progression and growth retardation. Patients with ARB fetopathy should be monitored until adulthood. The ARB exposure period might be a critical factor in determining the severity and manifestations of fetopathy.

임신을 원하는 부부의 자연스러운 임신 준비 과정을 위한 IoT 서비스 컨셉 제안

홍은지(Eunji Hong),김희연(Heeyeon Kim),권혜진(Hyejin Kweon),조광수(Kwangsu Cho) 한국HCI학회 2016 한국HCI학회 학술대회 Vol.2016 No.1

현대 사회에서 아이를 갖는 일은 자연스럽고 쉬운 일이 아니다. 특히 맞벌이 부부의 비율이 늘어나 과거집안에만 있던 여성보다 맞벌이 여성의 가정 내 거주시간이 현저하게 줄어들었다. 부부의 바쁜 직장생활과 불임 문제로 인하여 아이 갖는 일이 쉽지만은 않은 현실이다. 문제 없는 부부도 있겠지만, 임신을 위해 큰 노력을 들이는 부부가 늘어나고 있는 현상을 쉽게 찾아 볼 수 있다. 본 IoT 서비스 컨셉은 임신을 계획하고 있는 부부들 중 배란일 계산과 커뮤니케이션 등에 문제를 겪고 있으며 자연 임신을 위해 노력하고 있는 부부를 대상으로 한다. 본고에서는 배란 테스트기를 통해 검출된 데이터를 바탕으로 길일을 계산하고 침실조명색을 제어하는 방식으로 사용자에게 자연스러운 길일 정보를 전달하는 서비스 컨셉을 제안한다. 이를 위해 이론과 사전 인터뷰 조사를 통해 도출된 서비스 컨셉 및 서비스 구성요소 (디바이스 및 데이터 관리)를 소개하고 서비스 이용 시나리오를 본 논문을 통해 제안하고자 한다. Having a child is not a natural or easy chore in the modern society. Particularly the proportion of couples working together has increased, that the working women nowadays stay less at home than the housewives in the past. Busy work life and infertility problems make couples harder to get pregnant. Some couples may not have any problem regarding fertility issue, however it is easy to see currently that many couples put a big effort for pregnancy. This IoT service concept is for the couple who are planning a pregnancy, especially striving for a natural pregnancy, where calculating the ovulation date and communication are huge hurdles. In this paper, we propose a service concept for transmitting love day information in a natural way for the user in a manner of controlling the bedroom lighting color, based on carculating the day using the data detected through the ovulation tester. This research will introduce the service concept and the components of the service (device and data management) that have been derived through the theory and preinterviews, and propose service usage scenarios.

Corrigendum : Effect of Hypertension on Childhood-onset Systemic Lupus Erythematous in a Tertiary Medical Center in Korea

Kim, Jeong Yeon,Cho, Heeyeon Korean Society of Pediatric Nephrology 2021 Childhood kidney diseases Vol.25 No.1