Association of vitamin D deficiency with clinical outcomes in critically ill Korean children

Won Kyoung Jhang,Da Hyun Kim,Seong Jong Park 한국영양학회 2020 Nutrition Research and Practice Vol.14 No.1

BACKGROUND/OBJECTIVES: Vitamin D is a pleiotropic hormone that affects various body organ systems. We evaluated the prevalence of a vitamin D deficiency (VDD) and its potential role in the clinical condition of critically ill Korean children. SUBJECTS/METHODS: Patients under 18 years old with a 25(OH) vitamin D measurement on the first day of PICU admission were included from among the children admitted to the pediatric intensive care unit (PICU) of our tertiary children’s hospital between October 2017 and January 2019. RESULTS: A total of 172 pediatric patients were enrolled. The mean 25(OH) vitamin D level was 17.5 ± 12.8 ng/mL. There was a 65.1% prevalence of VDD (25(OH) vitamin D level < 20 ng/mL). VDD was associated with age at PICU admission, gastrointestinal/hepatobiliary disorders, International Society of Thrombosis and Hemostasis disseminated intravascular coagulation (ISTH DIC) score, pediatric multiple organ dysfunction syndrome (pMODS) score and with several laboratory test findings including hemoglobin, platelet, C-reactive protein, serum albumin, total bilirubin, prothrombin time, and anti-thrombin III levels. Most of these parameters also showed significant linear correlations with the 25(OH) vitamin D level (P < 0.05). However, no statistically meaningful association was found between VDD and other clinical conditions such as the need for a mechanical ventilator, requirement for vasoactive drugs, duration of the PICU and hospital stays, or PICU mortality. CONCLUSION: There is a high prevalence of VDD in critically ill Korean children. There were significant associations between the 25(OH) vitamin D level and gastrointestinal/hepatobiliary disorders, the pMODS score and with coagulation related factors. Further large-scale studies with more specific subgroup analyses are required to more precisely assess the clinical implications of VDD in critically ill pediatric patients.

Severe hypermagnesemia presenting with abnormal electrocardiographic findings similar to those of hyperkalemia in a child undergoing peritoneal dialysis

Jhang, Won Kyoung,Lee, Yoon Jung,Kim, Young A,Park, Seong Jong,Park, Young Seo The Korean Pediatric Society 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.7

In this report, we present a pediatric case of severe symptomatic hypermagnesemia resulting from the use of magnesium oxide as a laxative in a child undergoing continuous cyclic peritoneal dialysis for end-stage renal disease. The patient showed abnormal electrocardiography (ECG) findings, such as tall T waves, a widened QRS complex, and irregular conduction, which were initially misdiagnosed as hyperkalemia; later, the correct diagnosis of hypermagnesemia was obtained. Emergent hemodialysis successfully returned the serum magnesium concentration to normal without complications. When abnormal ECG changes are detected in patients with renal failure, hypermagnesemia should be considered.

Clinical and molecular characterisation of Holt-Oram syndrome focusing on cardiac manifestations

Jhang, Won Kyoung,Lee, Beom Hee,Kim, Gu-Hwan,Lee, Jin-Ok,Yoo, Han-Wook Cambridge University Press 2015 Cardiology in the young Vol.25 No.6

<B>Abstract</B><P><I>Background:</I> Holt-Oram syndrome is characterised by CHD and limb anomalies. Mutations in <I>TBX5</I> gene, encoding the T-box transcription factor, are responsible for the development of Holt-Oram syndrome, but such mutations are variably detected in 30-75% of patients. <I>Methods:</I> Clinically diagnosed eight Holt-Oram syndrome patients from six families were evaluated the clinical characteristics, focusing on the cardiac manifestations, in particular, and molecular aetiologies. In addition to the investigation of the mutation of <I>TBX5</I>, <I>SALL4, NKX2.5,</I> and <I>GATA4</I> genes, which are known to regulate cardiac development by physically and functionally interacting with <I>TBX5</I>, were also analyzed. Multiple ligation-dependent probe amplification analysis was performed to detect exonic deletion and duplication mutations in these genes. <I>Results:</I> All included patients showed cardiac septal defects and upper-limb anomalies. Of the eight patients, seven underwent cardiac surgery, and four suffered from conduction abnormalities such as severe sinus bradycardia and complete atrioventricular block. Although our patients showed typical clinical findings of Holt-Oram syndrome, only three distinct <I>TBX5</I> mutations were detected in three families: one nonsense, one splicing, and one missense mutation. No new mutations were identified by testing <I>SALL4, NKX2.5,</I> and <I>GATA4</I> genes. <I>Conclusions:</I> All Holt-Oram syndrome patients in this study showed cardiac septal anomalies. Half of them showed <I>TBX5</I> gene mutations. To understand the genetic causes for inherited CHD such as Holt-Oram syndrome is helpful to take care of the patients and their families. Further efforts with large-scale genomic research are required to identify genes responsible for cardiac manifestations or genotype-phenotype relation in Holt-Oram syndrome.</P>

The CCR5 (−2135C/T) Polymorphism may be Associated with the Development of Kawasaki Disease in Korean Children

Jhang, Won Kyoung,Kang, Mi-Jin,Jin, Hyun-Seung,Yu, Jinho,Kim, Byoung-ju,Kim, Bong Seong,Lee, Jong-Keuk,Seo, Eul-Ju,Yoo, Han-Wook,Park, In Sook,Hong, Young Mi,Hong, Soo-Jong Springer-Verlag 2009 Journal of clinical immunology Vol.29 No.1

소아 다발성 경화증 환자들에 대한 임상적 고찰

장원경(Won Kyoung Jhang),유수정(Soo Jung You),김덕수(Deok Soo Kim),고태성(Tae Sung Ko) 대한소아신경학회 2004 대한소아신경학회지 Vol.12 No.2

목적 : 다발성 경화증(multiple sclerosis, MS)은 면역 매개성 탈수초성 질환으로 젊은 성인에서 많이 발병하며 사춘기 이전의 발병은 드문 것으로 알려져 있다. 저자들은 소아 연령에서 발생한 다발성 경화증의 임상양상 및 검사소견, 방사선학적 소견 및 치료 경과 등을 조사하였다. 방법 : 1996년 1월부터 2002년 12월까지 서울 아산병원 소아과에 내원하여 신경학적 증상과 검사소견으로 진단된 다발성 경화증 환아 13명을 대상으로 후향적 의무기록 고찰을 시행하였다. 결과 : Rose 진단 기준에 의거하여 5명의 임상적으로 확실한 다발성 경화증(clinically definite MS)환자와 임상적으로 진단 가능한 다발성 경화증(clinically probable MS)환자 7명, 병리 조직 검사상 진단된 환아 1명이 본 연구에 포함되었다. 이 중 남아가 6명, 여아가 7명이었고 처음 증상 발현 나이는 평균 10.3세였으며 10세 미만인 경우가 4명이었다. 첫 증상 발현으로 시각장애 소견을 나타낸 경우가 8명이었고 보행장애 소견을 나타낸 경우가 3명, 감각 이상을 호소하였던 경우가 3명이었고 발병 당시 2가지 이상의 다발성 증상을 보인 경우는 6명이었다. 뇌척수액 검사상 비정상 단백 소견을 보인 환아가 3명(3/12, 25%)이었으며 oligoclonal band 양성을 보인 환아는 없었다. IgGindex는 4명(4/11, 36%)에서 증가되어 있었다. 뇌자기공명영상에서는 13명 모두 T2 강조 영상에서 음영 증가 소견을 보였고 시각 유발 전위 검사에서 이상 소견을 보인 경우가 7명(7/10, 70%)이었다. 치료로는 모두 정맥용 메탈프레드니솔론에 좋은 반응을 보였고 단기간에 여러 번 재발을 보였던 환아 1명에서 베타 인터베론 사용 후 더 이상의 재발은 없었다. 결론 : 본 연구에 의하여 포함된 환아의 수가 적고 추적 관찰 기간이 짧아 EOMS에 대해 단정적으로 말하기는 어려우나 임상 양상으로는 발병 당시의 증상은 성인 발병에서와 크게 차이는 보이지 않았으며 시각 증상이 가장 많이 나타났고 다발성 증상을 보이는 경우가 많았다. 뇌 자기공명영상 검사는 양성률이 매우 높은 진단검사방법으로 특징적인 병변을 보이며 치료로는 대개는 정백용 메틸프레드니솔론에 좋은 반응을 보였다. 앞으로 성인 환자와의 비교 연구나 소아에서의 치료 약제 개발, 장기간에 걸친 임상 양상의 변화 등 꾸준한 연구 및 추적 관찰이 필요할 것으로 생각된다. Purpose: It is well known that multiple sclerosis(MS) is a demyelinating disease of the central nervous system that mostly starts in the second to third decades. In the present study, we reviewed our own observations of the clinical and paraclinical features of the MS patients whose symptoms started before 16 years of age. Methods: The study concerns the patients admitted to the pediatric department of Asan Medical Center for a period of 5 years(from January 1996 to December 2002). We retrospectively reviewed 13 patients who were diagnosed as early onset multiple sclerosis by Rose criteria on the basis of clinical symptoms and brain MRI findings. Results: Of these 13 patients, 5 patients(38.5%) fulfilled the criteria of clinically definite MS and 7 patients(53.8%) fulfilled the criteria of clinically probable MS. The average age at onset was 10.3 years old. In six patients, the disease onset was polysymptomatic. Diplopia was the most common initial manifestation and occurred in 8 patients (61.5%). Others presented with urinary incontinence, ataxia, seizures and so on. All the patients had typical T2-hyperintensive lesions on brain MRI and brainstem lesions were found in 5 patients(38.5%). All the patients responsed to intravenous methylprednisolone very well. No long-term neurologic sequelae were observed. Conclusions: Although childhood onset MS is rarely seen and may begin with unusual features, it dose not significantly differ from that typically seen in adults in terms of major clinical manifestations and clinical course of the disease. It is also suggested that MRI is a sensitive and effective tool for the diagnosis of MS.

HIV-1 Tat Protein Promotes Amyloid β Generation and Tau Phosphorylation in Rat Hippocampal Slices

Eun Ok Lee,Kyoung A Jhang,Ye Won An,Woong Ju,Young Hae Chong 대한미생물학회 2014 Journal of Bacteriology and Virology Vol.44 No.1

Clinical Characteristics of Patients with Bronchopulmonary Dysplasia Admitted to Pediatric Intensive Care Unit

Yeon Chul Choi,Won Kyoung Jhang,Seong Jong Park 대한신생아학회 2016 Neonatal medicine Vol.23 No.3

Purpose: As the incidence of bronchopulmonary dysplasia (BPD) has increased, it is important to understand the clinical outcomes of BPD patients discharged from neonatal intensive care units (NICU). The purpose of our study was to describe the characteristics of BPD patients who are re-hospitalized in a pediatric intensive care unit (PICU) and to evaluate the prognostic outcome factors. Methods: We retrospectively reviewed the medical records of BPD patients who were admitted to our PICU between May 2006 and November 2014. In total, we identi- fied 101 cases which were divided into two groups, group 1, those who required inten- sive care for an acute illness or disease aggravation (n=62), and group 2, those who were admitted for post-operative care unrelated to having BPD as a control group (n=39). We subsequently compared the characteristics. Results: Most patients in group 1 were aged less than 1 year, with weight below the 3rd percentile for age at the time of their PICU admission. The main cause for their admission was respiratory failure, requiring mechanical ventilation. When compar- ing the two groups, group 1 showed higher gestational age at birth, and a longer duration of mechanical ventilation, oxygen support, and NICU hospitalization than group 2. However, we failed to identify any factor significantly associated with the duration of the PICU stay, hospital stay, and mortality. Further large-scale, long-term follow-up studies will be necessary. Conclusion: As the majority of patients are admitted to PICU because of respiratory symptoms during their infantile period, careful follow-up with supportive care and prevention of respiratory infection are required.

HIV-1 Tat Protein Promotes Amyloid ${\beta}$ Generation and Tau Phosphorylation in Rat Hippocampal Slices

Lee, Eun Ok,Jhang, Kyoung A,An, Ye Won,Ju, Woong,Chong, Young Hae The Korean Society for Microbiology 2014 Journal of Bacteriology and Virology Vol.44 No.1

HIV-1 Tat protein has been implicated as a causative agent in the pathogenesis of HIV-1-associated neurocognitive disorder (HAND) and Alzheimer's disease (AD)-like pathology in HIV-1 infected patients. Here, we provide insights into the potential roles of extracellular HIV-1 Tat protein in amyloid ${\beta}$ ($A{\beta}$) generation and Tau phosphorylation, two major neuropathological features of AD. Exposure of the rat hippocampal slices to the full-length HIV-1 Tat protein (Tat1-86) for 3 days led to the increased levels of $A{\beta}$ precursor protein (APP) accumulation, which accompanied by $A{\beta}$ generation in the hippocampus, the brain region most commonly damaged in HIV-1-associated dementia (HAD). Moreover, extracellular HIV-1 Tat significantly stimulated the level of phosphrylated Tau (pTau) identified using immunoblotting with AT8 antibody, which recognizes abnormally hyperphosphorylated Tau. Collectively, our data suggest that HIV-1 Tat plays important roles in increasing the levels of APP accumulation,$A{\beta}$ generation and Tau phosphorylation in the hippocampus, and thereby might contribute to the development of AD-like pathology in HIV-1-infected patients.

Extracorporeal Membrane Oxygenation Cannula Malposition in the Azygos Vein in a Neonate with Right-Sided Congenital Diaphragmatic Hernia

최승준,Chun Soo Park,Won Kyoung Jhang,Seong-Jong Park 대한중환자의학회 2016 Acute and Critical Care Vol.31 No.2

Malposition of the extracorporeal membrane oxygenation (ECMO) venous cannula in the azygos vein is not frequently reported. We hereby present such a case, which occurred in a neonate with right-sided congenital diaphragmatic hernia. Despite ECMO application, neither adequate flow nor sufficient oxygenation was achieved. On the cross-table lateral chest radiograph, the cannula tip was identified posterior to the heart silhouette, which implied malposition of the cannula in the azygos vein. After repositioning the cannula, the target flow and oxygenation were successfully achieved. When sufficient venous flow is not achieved, as in our case, clinicians should be alerted so they can identify the cannula tip location on lateral chest radiograph and confirm whether malposition in the azygos vein is the cause of the ineffective ECMO.

Pediatric Extracorporeal Membrane Oxygenation in Korea: A Multicenter Retrospective Study on Utilization and Outcomes Spanning Over a Decade

Choi Yu Hyeon,Jhang Won Kyoung,Park Seong Jong,Choi Hee Joung,Oh Min-su,Kwon Jung Eun,Kim Beom Joon,Shin Ju Ae,Lee In Kyung,Park June Dong,Lee Bongjin,Chung Hyun,Na Jae Yoon,Choi Ah Young,Cho Joongbum 대한의학회 2024 Journal of Korean medical science Vol.39 No.3

Background: Over the last decade, extracorporeal membrane oxygenation (ECMO) use in critically ill children has increased and is associated with favorable outcomes. Our study aims to evaluate the current status of pediatric ECMO in Korea, with a specific focus on its volume and changes in survival rates based on diagnostic indications. Methods: This multicenter study retrospectively analyzed the indications and outcomes of pediatric ECMO over 10 years in patients at 14 hospitals in Korea from January 2012 to December 2021. Four diagnostic categories (neonatal respiratory, pediatric respiratory, postcardiotomy, and cardiac-medical) and trends were compared between periods 1 (2012–2016) and 2 (2017–2021). Results: Overall, 1065 ECMO runs were performed on 1032 patients, with the annual number of cases remaining unchanged over the 10 years. ECMO was most frequently used for post-cardiotomy (42.4%), cardiac-medical (31.8%), pediatric respiratory (17.5%), and neonatal respiratory (8.2%) cases. A 3.7% increase and 6.1% decrease in pediatric respiratory and post-cardiotomy cases, respectively, were noted between periods 1 and 2. Among the four groups, the cardiac-medical group had the highest survival rate (51.2%), followed by the pediatric respiratory (46.4%), post-cardiotomy (36.5%), and neonatal respiratory (29.4%) groups. A consistent improvement was noted in patient survival over the 10 years, with a significant increase between the two periods from 38.2% to 47.1% (P = 0.004). Improvement in survival was evident in post-cardiotomy cases (30–45%, P = 0.002). Significant associations with mortality were observed in neonates, patients requiring dialysis, and those treated with extracorporeal cardiopulmonary resuscitation (P < 0.001). In pediatric respiratory ECMO, immunocompromised patients also showed a significant correlation with mortality (P < 0.001). Conclusion: Pediatric ECMO demonstrated a steady increase in overall survival in Korea; however, further efforts are needed since the outcomes remain suboptimal compared with global outcomes.