• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.4
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• pp.356-365
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• 2020

Purpose: There have been many efforts to develop generalizable severity markers in children with acute pancreatitis (AP). Expert opinion panels have developed consensus guidelines on management but it is unclear if these are sufficient or valid. Our study aims to assess the effect of clinical and laboratory variables, in addition to treatment modality on hospital length of stay (LOS) as a proxy variable for severity in pediatric patients admitted with AP. Methods: We conducted a retrospective chart review of patients between ages of 0-18 years, who were admitted with AP at 2 institutions between 2013-2018, John R. Oishei Children's Hospital (Buffalo, NY, USA) and Medical University of South Carolina Children's Hospital (Charleston, SC, USA). We constructed three linear regression models to analyze the effect of clinical signs of organ dysfunction, laboratory markers and fluid intake on hospital LOS. Results: Ninety-two patients were included in the study. The mean age was 12 years (range, 7.6-17.4 years), 55% were females, and median LOS was 3 days. The most frequent cause of AP was idiopathic. Our study showed that elevated blood urea nitrogen (BUN) on admission (p<0.005), tachycardia that lasted for ≥48 hours (p<0.001) and need for fluid resuscitation were associated with increase LOS. Total daily fluid intake above maintenance did not have a significant effect on the primary outcome (p=0.49). Conclusion: Elevated serum BUN on admission, persistent tachycardia and need for fluid resuscitation were associated with increase LOS in pediatric AP. Daily total fluid intake above recommended maintenance did not reduce LOS.

• Clinical and Experimental Pediatrics
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• v.62 no.7
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• pp.274-280
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• 2019

Purpose: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. Methods: Twenty-three patients with prepubertal NS treated at Pusan National University Children's Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were positive for mutations of the PTPN11, RAF1, and SOS1 genes. The five genes undetected (FGU) group was negative for PTPN11, RAF1, SOS1, KRAS, and BRAF gene mutations. The influence of genotype was retrospectively analyzed by comparing the growth parameters after GH therapy. Results: The mean chronological age at the start of GH treatment was $5.85{\pm}2.67years$. At the beginning of the GH treatment, the height standard deviation score (SDS), growth velocity (GV), and lower levels of insulin-like growth factor-1 (IGF)-1 levels were not statistically different among the groups. All the 23 NS patients had significantly increased height SDS and serum IGF-1 level during the 3 years of treatment. GV was highest during the first year of treatment. During the 3 years of GH therapy, the PTPN11, RAF1, and SOS1 groups showed less improvement in height SDS, IGF-1 SDS, and GV, and less increase in bone age-to-chronological age ratio than the FGU group. Conclusion: The 3-year GH therapy in the 23 prepubertal patients with NS was effective in improving height SDS, GV, and serum IGF-1 levels. The FGU group showed a better response to recombinant human GH therapy than the PTPN11, RAF1, and SOS1 groups.

• Korean Journal of Cleft Lip And Palate
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• v.11 no.2
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• pp.59-63
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• 2008

Craniofacial Centre at Children's Hospital Boston is a worldwide leader in the care of children and adolescents with craniofacial anomalies especially with cleft lip and/or cleft palate, which provides a team approach to the evaluation, diagnosis and treatment of children and adults with congenital (present at birth) or acquired facial deformities. This is staffed by an experienced team of clinicians, such as in oral and maxillofacial surgery, plastic surgery, neurosurgery, dentistry, audiology, speech and language pathology, genetics, psychiatry, otolaryngology, and social work, all with specialized training in the care of children with craniofacial anomalies. Here, there is a short introduction of history, attending surgeons, works, and sequential treatment for cleft lip/palate patients about this institution.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.84-87
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• 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

• Childhood Kidney Diseases
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• v.25 no.2
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• pp.117-121
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• 2021

Chronic kidney disease (CKD)-mineral and bone disorder (CKD-MBD) is a common complication of CKD, often accompanied by extra-skeletal calcification in adult patients. As increased vascular calcification is predicted to increase cardiovascular mortality and morbidity, the revised Kidney Disease: Improving Global Outcomes guidelines recommend avoiding calcium-containing phosphate chelators. However, extra-skeletal calcification is less commonly noticed in pediatric patients. Here, we report our experience of such a complication in pediatric patients receiving maintenance peritoneal dialysis. Extra-skeletal calcification was noticed at the corneas, pelvic cavity, and soft tissues of the lower leg in 4 out of 32 patients on maintenance peritoneal dialysis. These patients experienced the aggravation of extra-skeletal calcifications during peritoneal dialysis, and 2 of them underwent excisional operations. It is required to monitor extra-skeletal calcifications in children on kidney replacement therapy.

• The Journal of Pediatrics of Korean Medicine
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• v.20 no.1
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• pp.195-206
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• 2006

Objective : The purpose of this study is to investigate the current trends of children patients with convulsion. Children convulsion patients who visited the Pediatric Department of the Oriental Medicine hospital were investigated and analyzed. Method : 90 children complaining convulsion who visited the Department of Pediatrics of Kyung Hee University Oriental Medicine Hospital, between March, 2005 and October 2005 were statistically analyzed. Results : The percentage of boys outnumbered the girls. The percentage of children under the age of 5 18 66.7%. The percentage of children with family history is 43%. The majority of children had visited the hospital twice (n=21). As for intercurrent diseases, there were URI, C.P., ADHD, CHD, sequela of encephalitis and etc. As for the result of EEG examinations, 25 children were normal and 18 were abnormal. As for the appearance of seizures, Tonic-Clonic seizure was the most common seizure among the children (n=13). As for the type of convulsion, simple febrile convulsion was the most common (n=35). As for the curative effect. the percentage of children who showed improvement to treatment was 41.2%. Jangdambosintang, sunbangpaedoktang, gamichodeungeunm, gamichungeumchowitang and etc were the most frequently prescribed medicines.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.3
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• pp.187-192
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• 2015

Purpose: Measurement of serum ceruloplasmin level is the first step in screening for Wilson's disease (WD). Despite the rarity of WD in the general population, ceruloplasmin levels are routinely measured through hepatitis screening in both adults and children. Herein, we evaluated the diagnostic value of ceruloplasmin for the diagnosis of WD among children with hepatitis. Methods: We retrospectively reviewed data on serum ceruloplasmin levels measured as a serologic marker for patients with hepatitis at Asan Medical Center (Seoul, Korea) between from January 2004 to November 2013. The diagnosis of WD was confirmed by the identification of pathogenic variants in the ATP7B gene. To determine the diagnostic accuracy of ceruloplasmin, receiver operation characteristic (ROC) curves were constructed and the area under curve (AUC) were calculated. Results: Measurements of serum ceruloplasmin were performed in 2,834 children who had hepatitis. Among these, 181 (6.4%) children were diagnosed with WD. The sensitivity, specificity, and accuracy of a ceruloplasmin level of <20 mg/dL in the discrimination of WD were 93.4%, 84.2%, and 84.8%, respectively. In this study, 418 (14.7%) false-positive cases and 12 (0.4%) false-negative cases were noted. Using a ROC curve, a ceruloplasmin level of ${\leq}16.6mg/dL$ showed the highest AUC value (0.956) with a sensitivity of 91.2%, a specificity of 94.9%, and an accuracy of 94.7%. Conclusion: The measurement of serum ceruloplasmin was frequently used for the screening of WD in children, despite a low positive rate. The diagnostic value of ceruloplasmin may be strengthened by adopting a new lower cut-off level.

• Clinical and Experimental Pediatrics
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• v.55 no.11
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• pp.408-413
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• 2012

Moyamoya disease (MMD) is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

• The Journal of Pediatrics of Korean Medicine
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• v.20 no.3
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• pp.11-22
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• 2006

Objectives : Molluscum Contagiosum is a contagious disease most often seen in children. It is caused by molluscum contagiosum virus(MCV-1,2). The purpose of this study is to report three cases of Molluscum Contagiosum children treated by oriental medicine. Methods : The subjects are in regard to children diagnosed as Molluscum Contagiosum. We treated three children with herbal medicine(Gwakhyangjeonggisangamibang). Results : After treatment, the symptoms(papule on the whole body and pruritus) of Molluscum Contagiosum were improved. Two children of them were cured completely and the one child still appealed a little itching. But, the one child had atopic dermatitis, and suffered from itching primarily, so after treatment the itching sign decreased rather than before an attack of Molluscum Contagiosum. Conclusions : This study shows that oriental medicine can be an effective treatment for Molluscum Contagiosum. And the further study is needed with more cases and longer duration.

• Korean Institute of Interior Design Journal
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• v.25 no.4
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• pp.105-112
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• 2016

Nurses in a children's hospital have to meet a special condition with their younger patients who need continuous supervision and cares. The planning of the ward where the nurse as well as the patient and his/her caregivers stay all day long should cover all the users need. This study focused on the nurse's need for the ward in children's hospital. The nurse stay longer than any users in hospital and their treatment have to be based on deep understanding of their patients. The survey research followed the literature review on the children's hospital and the nurses' task and behavior. 119 nurses answered the structural questionnaire and their answers were analyzed using the statistical process such as basic descriptive statistics, ANOVA, and actor analysis. Results and conclusions are as follows. (1) The subjects least satisfied with the accessibility for the children and the nature-and child-friendly design features among physical environment design factors of the hospital. (2) The Subject regarded the patients' room to a private place of the patients and their caregivers not to the work places. (3) The design factors of the nursing station were classified into four: the functionality-, the privacy-, the supervision-and the restfulness-factor. The functionality and supervision factor were highly required as a workplace, the privacy factor between the patients, their caregivers and subject were also represented high score, but the restfulness factor were least required.