• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.13 no.1
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• pp.67-75
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• 2002

Objectives：This study was conducted to investigate the degree of psychopathologies of the family members of children with chronic convulsive disorder and evaluate the structures and dynamics of those families. Methods：The participant patients and family members were recruited from the population attending the outpatient clinic of department of pediatric neurology in Seoul National University Hospital in Korea. All the patients had idiopathic chronic convulsive disorder. Any patient with mental retardation, pervasive developmental disorder and gross brain pathology was excluded. As controls, normal students were chosen and their sex, age, achievement, socioeconomic status were matched to patients. The author interviewed the children and their family members twice and obtained informations about patient-parent relationship, patient-sibling relationship and others. For in-depth evaluation, we used family environment scales(FES), symptom Checklist-90-revised(SCL-90-R), self administered dependency questionnaire for mother(SADQ). Results：After interviewing with the parents of epileptic children, overprotection of parents, hostile feeling of siblings toward index children were higher than controls. The parental conflict was also more expressed than control families. According to results from FES, the scores of the subscales of expression, achievement-orientation, intelligence-orientation and active recreation were significantly lower than control group. The epileptic children showed higher dependency to parents especially in affection, communication and traveling areas of SADQ than control group. Maternal psychopathologies evaluated by SCL-90-R were much higher than the mothers of controls. According to T scores of SCL-90-R, about 40% of mothers with epileptic children had the risk of clinically significant depressive or anxiety disorders. Conclusion：These results suggested that the family members of epileptic children had more relationship problems and psychopathologies than control group and some mothers might have clinically significant depressive or anxiety disorders. so, effective psychiatric family interventions are needed for resolution of conflict and psychopathologies of family members.

• Korean Journal of Family Social Work
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• no.55
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• pp.59-90
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• 2017

The purpose of this study is to identify the overall effect of Family Hope Partner Project conducted by the Korean Association for Children with Leukemia and Cancer (KACLC) for pediatric cancer patients and their families and provide empirical basic data for the support of them. For this purpose, this study applied a qualitative research methodology as follows; 6 parents and 5 children who used the service for more than one year through the Family Hope Partner Project participated in the interviews. The interviews were conducted from June to July 2016, and the collected data were analyzed through the processes of qualitative analysis. The results of this study are as follows. Pediatric cancer patients and their families who were previously confronted with problems such as a 'barrier of severe pain' and 'swirling anxiety and exhaustion' experienced multi-dimensional effects, thanks to the intervention of 'a genuine master serving as a stepping stone toward their recovery and growth', who were linked to the pediatric cancer patients through Family Hope Partner Project. In other words, they experienced positive effects such as 'support for overall rehabilitation of pediatric patients', 'improvement of family relations', 'relief of needs for social relations', and 'liberation from pressure'. The results of this study were meaningful, in that it substantially corroborated the multidimensional effects of the Family Hope Partner Project, and presented the development direction of the Family Hope Partner Project, which was the first of its kind at home and abroad initiated by the Korean Association for Children with Leukemia and Cancer (KACLC) for pediatric cancer patients and their families.

• Journal of Hospice and Palliative Care
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• v.8 no.2
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• pp.216-223
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• 2005

Purpose: The purpose of this study was to compare the need for hospice care between families of children and adults with cancer. Methods: The data was collected from 190 families of children and adult with cancer using self-rating questionnaires from December, 2004 to February, 2005. Data was analyzed using SPSS/Win program by Mean, SD and t-test. Results: The mean score of the need for hospice care in families of children with cancer was greater significantly than in families of adults with cancer (t=-2.126, P=.035). The scores of two factors among the five factors evaluated for the need for hospice care were different significantly. The mean score of control of major terminal physical symptoms' in families of children with cancer was greater significantly than the mean score of adults with cancer (t=-2.165, P=.032). The mean score of 'spiritual care to prepare for death' in families of adults with cancer was greater significantly than the mean score of children with cancer (t=-2.380, P=.018). Conclusion: For improving the quality of life for both patients and families, the hospice service program needs to consider the life cycle of patients.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.36-42
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• 2001

Purpose : Vesicoureteral reflux is the most commonly inherited disease detected in children with urinary tract infection. The incidence of vesicoureteral reflux among siblings of children with known vesicoureteral reflux is 8$\%$ to 45$\%$ according to different authors. Family screening of a patient with vesicoureteral reflux is important in order to prevent reflux nephropathy. The purpose of this study is to determine the incidence of vesicoureteral reflux in asymptomatic family of children with vesicoureteral reflux and the factors which influence the family history. Methods : The study group consisted of 27 families of patients with vesicoureteral reflux. The total number in the group were 79 persons. BUN, Cr, urineanalysis, voidingcystourethrography(VCUG) and 99mTc -dimercaptosuccinic acid(DMSA) renal scan were performed oil tile siblings. As for tile parents the same tests were performed except the VCUG. Results : The abnormality was detected in 7 of 27 families(25.9$\%$). Vesicoureteral reflux was detected in 5 of 20 siblings and renal scar ns detected in 3 of 32 parents. In children with vesicoureteral reflux, renal scar was detected in 24 of 32 children. Between the group with the abnormality in its family(Group A) and the group without the abnormality in its family(Group B), There was no difference of creatinine clearance between two groups. More renal scars were detected in group A according to the DMSA(A:100$\%$, B:75$\%$. t-test P<0.05). There was no difference of grade of VCUG between two groups. There was no difference between one site and both sites in two groups. In tile case of tile siblings with vesicoureteral reflux, there was high incidence of renal scar in a patient with vesicoureteral reflux according to the DMSA. Conclusion : It is important to screen vesicoureteral reflux and renal scar in case of urinary tract infection to prevent reflux nephropathy. This study implies that it is necessary to screen the family of a patient with vesicoureteral reflux especially with renal scar. (J, Korean Soc Pediatr Nephrol 5 : 36- 42, 2001)

• Journal of Hospice and Palliative Care
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• v.7 no.2
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• pp.221-231
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• 2004

Purpose: The purpose of this study was to analyze the need for child hospice care programs in families of children with cancer. Methods: The survey of 104 families who were taking care of children with cancer was conducted. This survey was conducted from February 2004 to July 2004 at two general hospitals in Seoul. The data were collected through a self-reporting questionnaire of 22 items. The items were classified into five areas by factor analysis to identify the construct validity. The reliability of the tool was established by Cronbach's alpha as .94 and the data collected were analyzed by descriptive statistics, t-test and ANOVA. Results: 1) The degree of need for hospice care of the subjects showed a high average of 3.40 (${\pm}3.8$). The need for 'emotional care of children' showed the highest mean (M=3.55), 'management of terminal physical symptoms'(M=3.49), 'control of secondary physical problems' (M=3.41), 'acceptance of the family's difficulty' (M=3.20), 'spiritual care for preparing for death'(M=3.17), respectively. 2) With respect to the demographic characteristics of the subjects, there were statistically significant differences in hospice care needs, according to the child's mother's age (F==4.980, P=.009), whether or not there were cancer patients among their siblings or relatives (t=2.423, P=.017). Conclusion: The family of children with cancer have a heavy burden of ambivalence, especially in relieving the anxiety and fear of their children, communicating about death, and managing physical symptoms. Child hospice care must be provided considering the needs of families of children with cancer. Thus popular needs as well as hospice nurses' higher concern and support for hospice care of children require further education and program development to meet the current demands.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.62-66
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• 2016

Skin and soft tissue infections (SSTIs) caused by community-associated (CA)-methicillin-resistant Staphylococcus aureus (MRSA) have become a worldwide concern. An otherwise healthy 16-month-old Korean girl was admitted because of skin abscess on the left chest wall with a history of recurrent SSTIs since the age of 6 months. Immunologic evaluation including serum immunoglobulin level and nitroblue-tetrazolium (NBT) test were normal. Pus and nasal swab cultures revealed CA-MRSA ST714-SCCmec type IV with the Panton-Valentine leukocidin (PVL) genes, which was initially reported in the Netherlands in 2006 and has not been previously reported in Korea. The skin abscesses were successfully treated by needle aspiration and the use of antibiotics. In addition, nasal mupirocin was applied as a decolonization method. No more episodes of SSTI were observed over a follow-up period of 10 months.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.18 no.10
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• pp.309-322
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• 2017

The purpose of this qualitative study was to describe the experience of mothers who have had children with cancer. Participants were seven mothers who had children diagnosed with cancer between 3 to 36 months. Data were gathered through in-depth interviews and analyzed by Colaizzi's phenomenological methodology. Overall, 216 significant statements, 62 formulated statements, 16 themes and five theme clusters were identified. The five theme clusters were 'heartbreaking sadness', 'arduous journey of battling', 'protect my child', 'feeling varying between gratitude and disappointment', and 'suffering and wishing to be with family'. Mothers who have had children with pediatric cancer receiving chemotherapy have experienced strong emotional fluctuations as well as hope as they cope with their ill child. However, they try to become strong women and embrace their family to cope with their situation. The results of this study will support for health professionals to understand mothers who have had children with cancer and to consider the supportive nursing care in considering mothers' emotional fluctuation.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.13 no.4
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• pp.1706-1713
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• 2012

The purpose of this study was to analyze the need for child hospice care in families of children with cancer for outpatient. The participants were 83 parents of children with cancer. This survey was conducted from January 2011 to March 2011 at four hospitals in Daegu. Data were collected through self-report questionnaires and analyzed by descriptive statistics, t-test and ANOVA using the SPSS/WIN Program. Needs for hospice care for the participants were high. The need for "emotional care of children" showed the highest, "control of secondary physical problems", "acceptance of the family's difficulty", "management for terminal physical symptoms", "spiritual care for preparing for death". With respect on the demographic characteristics of the participants, there were statistically significant differences in hospice care needs, among to the religion, sibling, relatives, whether of the cancer. The above findings indicate that needs for hospice care for the participants were high about emotional care, especially as it is related to children's anxiety. Therefore hospice care, based on emotional part, should be provided systematic hospice care with specialized multidisciplinary child hospice care team, child hospice center.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.302-310
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• 2002

Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients and additional family members in three unrelated XLA families. Mononuclear cells were separated from their blood and the intracellular Btk protein was characterized by a flow cytometry. The mutation analysis was performed using direct sequencing. Results : Cytoplasmic expression of Btk protein in monocytes was not detected in the patients with XLA. We observed a novel deletion and two point mutations within introns(intron 1 and intron 18) resulting in alternative splicings. In XLA family 2, a 980 bp deletion(from intron 9+191 T to intron 10-215 C) including exon 10 was found in patient P2. He was the only sporadic case in this study, because his mother and brother showed a normal Btk expression by flow cytometry. Conclusion : These identified genetic alterations support the molecular heterogeneity of Btk gene in XLA disease. Additionally, by means of flow cytometric analysis, we diagnosed three hypogammaglobulinemia patients as XLA. Advancements in diagnostic methods has facilitated a prompt and definite diagnosis of this disease.

• Neonatal Medicine
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• v.17 no.2
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• pp.250-253
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• 2010

Citrullinemia type I is an urea cycle defect caused by mutations in the argininosuccinate synthetase (ASS1) gene. We report a novel argininosuccinate synthetase gene mutation in a Korean family with type I citrullinemia. Metabolic evaluation revealed significant hyperammonemia. Amino acid/acylcarnitine screening using tandem mass spectrometry showed high level of citrulline. Plasma amino acid analysis showed high level of citrulline and the urine organic acid analysis showed makedly increased level of orotic acid. To confirm diagnosis of citrullinemia we did mutation analysis of the ASS1 gene. The patient was found to have mutations of c.689G>C (p.G230A) and c.892G>A (p.E298K), which were new types of argininosuccinate synthetase gene mutation have never been reported in Korea. We report a novel case of argininosuccinate synthetase 1 gene mutation and suggest that the gene study to the family members is necessary to carry out when a patient is diagnosed as citrullinemia.