• Advances in pediatric surgery
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• v.17 no.1
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• pp.72-80
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• 2011

Although laparoscopic surgery for hepatobiliary disease in children is possible, it is technically challenging. In an attempt to overcome these difficulties, the da Vinci Robotic Surgical System$^{(R)}$ was used to facilitate the minimally invasive treatment of choledochal Cyst in six children. In early consecutive three cases, we experienced three complications; a case of laparotomy conversion, a case of late stenosis of the hepaticojejunostomy, and a case of leakage from a hepaticojejunostomy. However, in the last three cases the complete resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed using the robotic surgical system without complication. We think robot-assisted choledochal cyst resection in children appears safe and feasible, and may increase the variety of complex procedures in pediatric surgical fields.

• Journal of Genetic Medicine
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• v.15 no.1
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• pp.13-16
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• 2018

X-linked juvenile retinoschisis (XLRS) is characterized by the progressive loss of visual acuity and vitreous hemorrhage. XLRS is caused by a mutation of retinoschisin 1 (RS1) gene at Xp22.13. In the current report, a 2-year-old Korean patient with XLRS was described. The germline deletion of exon 1 was identified in the RS1 gene. Considering X-linked inheritance pattern, validation of a carrier state of a patient's mother is important for the genetic counseling of other family members and for the future reproductive plan. To confirm the carrier state of his mother, the multiplex ligation-dependent probe amplification analysis was done using peripheral leukocytes and found the heterozygous deletion of exon 1 in his mother.

• 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 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.

• Journal of Chest Surgery
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• v.47 no.3
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• pp.275-279
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• 2014

A 13-year-old girl, who had undergone the total correction of partial atrioventricular septal defect at the age of 4 years, was admitted with severe tricuspid regurgitation in echocardiography. She had received one-and-a-half ventricle repair during follow-up. Her right ventricle showed global akinesia, and the ejection fraction of the left ventricle was 25% with paradoxical interventricular septal motion. We performed right ventricular exclusion adjunct to the Fontan procedure. She is doing well two years after the operation without complications.

• Journal of Korean Neurosurgical Society
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• v.59 no.3
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• pp.227-232
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• 2016

Various operative techniques are available for the treatment of craniosynostosis. The patient's age at presentation is one of the most important factors in the determination of the surgical modality. Minimally invasive suturectomy and postoperative helmet therapy may be performed for relatively young infants, whose age is younger than 6 months. It relies upon the potential for rapid brain growth in this age group. Its minimal invasiveness is also advantageous. In this article, we review the advantages and limitations of minimally invasive suturectomy followed by helmet therapy for the treatment of craniosynostosis.

• Journal of Trauma and Injury
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• v.36 no.3
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• pp.295-297
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• 2023

Penetrating gallbladder injuries are uncommon in the pediatric population. The treatment varies according to the severity of the injury and the patient's hemodynamics. We present the case of an 11-year-old male with an accidental pellet gunshot wound to the right upper abdomen that resulted in a grade III liver laceration and damage to the anterior gallbladder wall. The patient underwent laparoscopic cholecystectomy with drain placement. Postoperative radiography of the surgical specimen confirmed the presence of the pellet in the gallbladder. The patient recovered uneventfully and was discharged home on postoperative day 3. Laparoscopic cholecystectomy is a feasible treatment option for penetrating gallbladder injuries in hemodynamically stable patients.

• Child Health Nursing Research
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• v.11 no.3
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• pp.273-281
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• 2005

Purposes: The purposes of this study were to evaluate parent satisfaction with inpatient hospital services in children's hospitals and to identify variables related to parent satisfaction. Method: A descriptive correlation study was conducted. Parents of 165 children who were inpatients in two children's hospitals participated in the study. Data were collected using structured questionnaire at the time of discharge. For statistical analysis, t-test, ANOVA, and Pearson correlation analysis were used. Results: The highest parent satisfaction domain was nursing service, and the lowest parent satisfaction domain was hospital service and accommodations. Parents were less likely to be satisfied with hospital facilities, equipment, noise and cleaning and less likely to be satisfied with the lack of information they received and with the lack of communication with health care professionals. Parents with longer length of stay and with older children reported higher satisfaction than their counterparts. Moreover, parent satisfaction was related to their intention to revisit and related to intention to recommend this surveyed hospital over others. Conclusions: Efforts to improve parent satisfaction in children's hospital service and accommodation are needed to improve the quality of health care. Communication by health care professionals with parents and a partnership between parents and health care professionals are necessary to improve quality of care.

• Journal of Genetic Medicine
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• v.16 no.2
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• pp.49-54
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• 2019

Purpose: Potocki-Lupski syndrome (PTLS), is a recently identified, rare genomic disorder. The patients are affected by infantile hypotonia, poor growth and developmental delay. Facial dysmorphism may not be obvious in some patients. PTLS is associated with microduplication at chromosome 17p11.2. In the current study, three Korean patients are reported with their clinical and genetic features. Materials and Methods: The clinical findings of each patient were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were done for genetic diagnoses. Results: All the patients did not have the characteristic dysmorphic features, such as broad forehead, triangular face, asymmetric smile and palpebral fissures. On the other hand, all three patients were affected by variable degree of developmental delay, poor oral intake, failure to thrive, and language development disorders. Chromosome 17p11.2 duplication was identified by conventional karyotyping analysis only in one patient, whereas the other confirmed by MLPA analyses. Conclusion: Delayed development was mostly commonly observed in our patients without distinct dysmorphic facial features. In this respect, genomic screening in patients with developmental delay would identify more cases with PTLS to understand their long-term clinical courses with the development of adequate psychological and rehabilitation education program.