• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.22 no.4
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• pp.219-227
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• 2011

Objectives : This study examined maternal object relations, child's and mother's perception on rearing attitude, and children's self-esteem in children with attention-deficit hyperactivity disorder (ADHD) and their mothers. Methods : 64 children with ADHD and their mothers were included in the study group. In the control group, there were 85 children and their mothers. Mothers completed the following tests : Bell object relation inventory (BORI), maternal behavior research instrument (MBRI), Beck Depression Inventory (BDI), and Korean ADHD Rating Scale (K-ARS). Children completed Children's Report of Parental Behavior Inventory (CRPBI) and Rosenberg self-esteem scale. Results : Mothers of ADHD children displayed more rejecting and controlling parenting style than mothers in the control group. ADHD children showed lower self-esteem and perceived their parents as not affectionate, but rejecting and controlling. Mothers with ADHD children who belonged to object relations pathological group showed more rejecting rearing attitude and their children believed that they were more controlling, compared with children and mothers in other conditions. Among factors in mother's object relations, insecure attachment and ego-centricity impacted the rearing attitude. In turn, affective rearing attitude mainly influenced children's self-esteem. Conclusion : This study suggests that the approach focused on mother's object relations may help with the treatment of children with ADHD.

• Clinical and Experimental Pediatrics
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• v.54 no.2
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• pp.90-93
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• 2011

Pseudohypoaldosteronism type 1 (PHA1) is a rare form of mineralocorticoid resistance characterized in newborns by salt wasting with dehydration, hyperkalemia and failure to thrive. This disease is heterogeneous in etiology and includes autosomal dominant PHA1 owing to mutations of the NR3C2 gene encoding the mineralocorticoid receptor, autosomal recessive PHA1 due to mutations of the epithelial sodium channel (ENaC) gene, and secondary PHA1 associated with urinary tract diseases. Amongst these diseases, autosomal dominant PHA1 shows has manifestations restricted to renal tubules including a mild salt loss during infancy and that shows a gradual improvement with advancing age. Here, we report a neonatal case of PHA1 with a NR3C2 gene mutation (a heterozygous c.2146_2147insG in exon 5), in which the patient showed failure to thrive, hyponatremia, hyperkalemia, and elevated plasma renin and aldosterone levels. This is the first case of pseudohypoaldosteronism type 1 confirmed by genetic analysis in Korea.

• Clinical and Experimental Pediatrics
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• v.58 no.3
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• pp.108-111
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• 2015

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.

• Journal of Korean Clinical Nursing Research
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• v.14 no.1
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• pp.173-185
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• 2008

Purpose: The purpose of this study was to identify the effects of multimedia educational program of the prevention of respiratory disorders on knowledge and behavior of preschool children. Method: The research design used a nonequivalent control group pre-posttest. 32 subjects in the control group received no intervention, 33 subjects in experimental group received a multimedia educational program that consists of video, named 'The strong girl and weak girl', 5 different picture boards, method of hand washing, tooth brushing, and reinforcement. The data were analyzed by using $x^2$-test and t-test for determining of homogeneity and testing the hypothesis. Results: There were significant increases in knowledge and behavior in the experimental group compared to the control group. Conclusion: From the results of this study, the multimedia educational program significantly increased knowledge and behaviors of preschool children for preventing respiratory disorders. These findings suggest that a multimedia educational program can be used as an efficient nursing intervention of preschool children for preventing respiratory disorders.

• Archives of Plastic Surgery
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• v.48 no.3
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• pp.338-343
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• 2021

Purpura fulminans is a serious condition that can result in severe morbidity in the pediatric population. Although autologous skin grafts remain the gold standard for the coverage of partial- to full-thickness wounds, they have several limitations in pediatric patients, including the lack of planar donor sites, the risk of hemodynamic instability, and the limited graft thickness. In Singapore, an in-house skin culture laboratory has been available since 2005 for the use of cultured epithelial autografts (CEAs), especially in burn wounds. However, due to the fragility of CEAs, negative-pressure wound therapy (NPWT) dressings have been rarely used with CEAs. With several modifications, we report a successful case of NPWT applied over a CEA in an infant who sustained 30% total body surface area full-thickness wounds over the anterior abdomen, flank, and upper thigh secondary to purpura fulminans. We also describe the advantages of using NPWT dressing over a CEA, particularly in pediatric patients.

• Journal of Yeungnam Medical Science
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• v.40 no.1
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• pp.86-90
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• 2023

Pyocele in infants is rarely described in the literature, but it is an emergent condition that requires rapid recognition and treatment to prevent testicular loss. If peritonitis due to gastrointestinal perforation occurs, abdominal contamination may spread through a patent processus vaginalis in an infant, which may lead to pyocele. We report the cases of three infants with scrotal pyocele due to the spread of infection or inflammatory material from the intraperitoneal cavity through a patent processus vaginalis. Two infants were surgically treated, while the other was treated with percutaneous aspiration and intravenous antibiotic administration. Although rare, pyocele should be considered in the differential diagnosis of acute scrotum in infants, especially in infants who previously had peritonitis due to gastrointestinal perforation.

• Nutrition Research and Practice
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• v.12 no.3
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• pp.215-221
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• 2018

BACKGROUND/OBJECTIVES: The prevalence of malnutrition among hospitalized children ranges between 12% and 24%. Although the consequences of hospital malnutrition are enormous, it is often unrecognized and untreated. The aim of this study was to identify the current status of in-hospital nutrition support for children in South Korea by carrying out a nationwide hospital-based survey. SUBJECTS/METHODS: Out of 345 general and tertiary hospitals in South Korea, a total of 53 institutes with pediatric gastroenterologists and more than 10 pediatric inpatients were selected. A questionnaire was developed by the nutrition committee of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition. The questionnaires were sent to pediatric gastroenterologists in each hospital. Survey was performed by e-mails. RESULTS: Forty hospitals (75.5%) responded to the survey; 23 of them were tertiary hospitals, and 17 of them were general hospitals. Only 21 hospitals (52.5%) had all the required nutritional support personnel (including pediatrician, nutritionist, pharmacist, and nurse) assigned to pediatric patients. Routine nutritional screening was performed in 22 (55.0%) hospitals on admission, which was lower than that in adult patients (65.8%). Nutritional screening tools varied among hospitals; 33 of 40 (82.5%) hospitals used their own screening tools. The most frequently used nutritional assessment parameters were weight, height, hemoglobin, and serum albumin levels. In our nationwide hospital-based survey, the most frequently reported main barriers of nutritional support in hospitals were lack of manpower and excessive workload, followed by insufficient knowledge and experience. CONCLUSIONS: Although this nationwide hospital-based survey targeted general and tertiary hospitals with pediatric gastroenterologists, manpower and medical resources for nutritional support were still insufficient for hospitalized children, and nutritional screening was not routinely performed in many hospitals. More attention to hospital malnutrition and additional national policies for nutritional support in hospitals are required to ensure appropriate nutritional management of hospitalized pediatric patients.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.1
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• pp.1-27
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• 2019

The Committee on Pediatric Obesity of the Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition newly developed the first Korean Guideline on the Diagnosis and Treatment of Obesity in Children and Adolescents to deliver an evidence-based systematic approach to childhood obesity in South Korea. The following areas were systematically reviewed, especially on the basis of all available references published in South Korea and worldwide, and new guidelines were established in each area with the strength of recommendations based on the levels of evidence: 1) definition and diagnosis of overweight and obesity in children and adolescents; 2) principles of treatment of pediatric obesity; 3) behavioral interventions for children and adolescents with obesity, including diet, exercise, lifestyle, and mental health; 4) pharmacotherapy; and 5) bariatric surgery.

• Journal of Genetic Medicine
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• v.10 no.1
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• pp.33-37
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• 2013

Purpose: Myotonic dystrophy 1 (DM1, OMIM 160900) is an autosomal-dominant muscular disorder caused by an expansion of CTG repeats in the 3' UTR of the DMPK gene. Variable expansions of CTG repeats preclude the accurate determination of repeat size. We tried to show the clinical and analytical validity of the application of Southern blotting after long-range PCR was demonstrated in Korean DM1 patients. Materials and Methods: The Southern blotting of long-range PCR was applied to 1,231 cases with clinical suspicion of DM1, between 2000 and 2011. PCR was performed using genomic DNA with forward 5'-CAGTTCACAACCGCTCCGAGC-3' and reverse 5'-CGTGGAGGATGGAACACGGAC-3' primers. Subsequently, the PCR fragments were subjected to gel electrophoresis, capillary transfer to a nylon membrane, hybridization with a labeled (CAG)10 probe. The correlation between clinical manifestations and the CTG repeat expansions were analyzed. Results: Among a total of 1,231 tested cases, 642 individuals were diagnosed with DM1 and the range of the detected expansion was 50 to 2,500 repeats; fourteen cases with mild DM1 ($75{\pm}14$ repeats), 602 cases with classical DM1 ($314{\pm}143$ repeats), and 26 cases with congenital DM1 ($1,219{\pm}402$ repeats). The positive and negative predictive values were 100%. The age at test requested and the CTG repeat numbers were inversely correlated (R=-0.444, P<0.01). Conclusion: This study indicates that Southern blotting after long-range PCR is a reliable diagnostic method DM1.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.57-59
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• 2016

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days -8 to -5), and the target area under the curve was $75,000{\mu}g{\cdot}hr/L$. Fludarabine ($40mg/m^2$) was administered once daily for 6 consecutive days from days -8 to -3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days -4 to -2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.