• Advances in pediatric surgery
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• 제3권1호
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• pp.54-60
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• 1997

Eight pediatric patients with Peutz-Jeghers syndrome were treated from 1984 to 1994 at the Department of Surgery, Seoul National University Children's Hospital. We reviewed the clinical features of our own 8 cases as well as 56 cases reported in Korean literatures. The results were compared to those of western reports. Gastrointestinal complications in our series were more frequent than in the Korean series, but the distribution of polyps was similar. The Korean series showed the following characteristics compared to the western reports; demographic backgrounds were similar; gastrointestinal symptoms were more common; location of the polyp was more frequent in colon (2 times); and the most prevalent site of malignant change was the colon in Korean cases.

• Journal of Genetic Medicine
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• 제15권1호
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• pp.38-42
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• 2018

WAGR (Wilms tumor, aniridia, genitourinary anomalies, and mental retardation) syndrome is a rare contiguous gene deletion syndrome caused by deleting genes including WT1 and PAX6 genes in 11p13 region, which is characterized by Wilms tumor, aniridia, genitourinary abnormalities, and intellectual disability. We report the clinical and cytogenetic characteristics of one Korean patient with WAGR syndrome. The patient shows bilateral sporadic aniridia and genital anomalies at 2 months of age. A heterozygous 14.5 Mb interstitial deletion of 11p14.3p12 region was detected by array comparative genomic hybridization. At 2 years and 10 months of age, Wilms tumor is found through regularly abdominal ultrasonography and treated by chemotherapy, radiation therapy and surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.479-486
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• 2019

Progressive familial intrahepatic cholestasis (PFIC) is a group of severe genetic disorders, inherited in an autosomal recessive manner, causing cholestasis of hepatocellular origin, later progressing to biliary cirrhosis and liver failure. This is the first report of PFIC type 1 with novel compound heterozygous mutations in Korea. The patient was presented with intrahepatic cholestasis, a normal level of serum ${\gamma}-glutamyl$ transferase, steatorrhea, and growth failure. Genetic testing of this patient revealed novel compound heterozygous mutations (p.Glu585Ter and p.Leu749Pro) in the ATP8B1 gene. After a liver transplantation at age 19 months, the patient developed severe post-transplant steatohepatitis.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.1-10
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• 2022

Congenital isolated hydronephrosis encompasses a spectrum of physiologic states that spontaneously resolve and pathologic obstruction that necessitates surgical intervention. Distinguishing patients whose condition will resolve, those who will require stringent follow-up, and those who will eventually need surgical intervention present a challenge to clinicians, particularly because no unified guidelines for assessment and follow-up have been established. The recognition of the natural course and prognosis of hydronephrosis and a comprehensive understanding of the currently proposed consensus guidelines may aid in multidisciplinary treatment and in providing proper counseling to caregivers. In this review, we aimed to summarize the literature on the grading systems and management strategies for congenital isolated hydronephrosis.

• 대한병원협회지
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• 제22권7.8호통권206호
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• pp.24-30
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• 1993

• 의료ㆍ복지 건축 : 한국의료복지건축학회 논문집
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• 제3권5호
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• pp.97-105
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• 1997

According as the medical demands have been high, ward dept, in general Hospital become more specifying. One thing of its sample is Children ward in general Hospitals and Children's medical demand become increasing. Therefore, this study aims to represent the standard for the architectural planning of the children ward in general Hospital. For it, the present conditions and space programs of 4 general Hospitals were investigated, analyzed. And to present children ward's functional programs, plans of the 2 domestic children hospitals and foreign children hospitals were analyzed. Then one children ward in general hospital was selected, architectural remodeing process was performed by the whole those sources.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제21권3호
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• pp.168-173
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• 2010

Objectives: This study was conducted to compare emotional problems in children with neurosurgical diseases prior to surgery as well as levels of parenting stress experienced by mothers of children treated with surgery and those without surgery. The goals was to provide a basis on which comprehensive treatment interventions could be established. Methods: Subjects included 78 elementary school children who visited the Department of Pediatric Neurosurgery as well as their mothers. The Children's Depression Inventory (CDI) and the Revised Children Manifest Anxiety Scale (RCMAS) were administered to children. The Parenting Stress Indexs (PSI) and Beck Depression Inventory (BDI) were administered to their mothers. Results: The children with neurosurgical diseases showed higher levels of depression prior to surgery. In their mothers, parenting stress was relatively higher and parenting competence was lower. In particular, for those children who underwent surgery, depression was significantly higher. Specifically, they exhibited more negative expectations, and negative self-image. In mothers of children who underwent surgery, depression was significantly higher. Conclusion: Our results suggest higher levels of emotional distress and parental stress in children who undergo neurosurgical operations and their mothers, respectively. Based on this, the necessity for surgical and comprehensive psychological intervention is suggested.

• 대한임상검사과학회지
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• 제38권2호
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• pp.125-134
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• 2006

This study was carried out to confirm the arguments that the role of the parents in sampling blood specimens from their children should be increased to reduce the anxiety and fear of children. The support and confidence of the parents, the reaction of the children and the subsequent action by phlebotomists should help to make an efficient sampling process for children. We randomly selected 50 children and their parents and had them fill out questionnaires before and after phlebotomy. The results were: 1. Detailed explanation by parents made children more active in sampling; 2. Higher confidence levels made parents more active in supporting children and relieving them from pain; 3. Confidence levels were higher in male children than in female children; 4. The confidence levels of parents were higher in parents of children with experience of multiple samplings and the highest level of confidence was observed in parents of children aged 61 to 120 months; 5. Only children showed the highest reaction level and very negative reactions; 6. The reaction of the phlebotomist was positive with children in good moods and negative with ones in bad moods. These results could serve as a basic tool in coping with children in phlebotomy.

• Advances in pediatric surgery
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• 제17권1호
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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 Chest Surgery
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• 제51권1호
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• pp.81-84
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• 2018