• 조명전기설비학회논문지
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• 제26권11호
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• pp.1-8
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• 2012

The road lightings have made our living environment safe and active, since it was first installed in 1900 in Korea. As the industry developed, various kind of road lightings have been installed in an outdoor area. But the spilt light from the road lightings have caused health problem like the sleeplessness, the depression, the failure to thrive and the nearsightedness to children. The light trespass caused by road lightings vary with the height of road lightings, the distance from road lightings to the window and the BUG rating of road lightings. In this paper, we analyze the effect on the light trespass by the spatial installation position of road lightings using by RELUX program. And we derive the characteristics of the light trespass according to the spatial installation position of the road lightings in residential area near road.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권1호
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• pp.55-59
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• 2015

Gastroesophageal reflux disorder (GERD) is the most common esophageal disorder in children. Achalasia occurs less commonly but has similar symptoms to GERD. A nine-year old boy presented with vomiting, heartburn, and nocturnal cough. The esophageal impedance-pH monitor revealed nonacidic GERD (all-refluxate clearance percent time of 20.9%). His symptoms persisted despite medical treatment for GERD, and he was lost to follow up. Four years later, he presented with heartburn, solid-food dysphagia, daily post-prandial vomiting, and failure to thrive. Endoscopy showed a severely dilated esophagus with candidiasis. High-resolution manometry was performed, and he was diagnosed with classic achalasia (also known as type I). His symptoms resolved after two pneumatic dilatation procedures, and his weight and height began to catch up to his peers. Clinicians might consider using high-resolution manometry in children with atypical GERD even after evaluation with an impedance-pH monitor.

• Clinical and Experimental Pediatrics
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• 제45권11호
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• pp.1430-1434
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• 2002

저자들은 성장 장애, 만성적인 구토, 저칼륨혈증, 저염소성 대사성 알카리혈증, 고레닌혈증, 고알도스테론혈증, 정상 혈압과 부종결여 등의 Bartter 증후군의 임상형태를 보였으나 요중 클로라이드 농도가 감소되어 있고 초음파 검사상 비후성 유문 협착증으로 진단수술 후 칼륨과 프로스타글란딘 억제제 등의 약물치료 없이도 회복된 가성 Bartter 증후군을 1례 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of mucopolysaccharidosis and rare diseases
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• 제3권2호
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• pp.41-43
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• 2017

Prader-Willi syndrome (PWS) is a genetic disorder that is considered, especially on child, to cause poor feeding, hypotonia, failure to thrive, developmental delay and hypogonadism which is known to affect between 1 in 10,000 and 30,000 people. The children with PWS are viewed as affected by growth hormone (GH) insufficiency, although the exact mechanisms of GH deficiency are not fully understood. However, the benefits of GH treatment in children with PWS are well established. Myers, et al. (2006), Grugni, et al. (2016) indicated its positive effects on linear growth, body composition, motor function, respiratory function and psychomotor development. Despite of its effectiveness and advantages had been well known and proven in many other studies, there is only one recombinant GH product that is approved for PWS in Korea, $Genotropin^{(R)}$, till now. A phase III clinical study of GH treatment with $Eutropin^{TM}$, in 34 Korean PWS children is in progress, which is expected to have comparable effects and safety profile with the active control by assessing auxological changes such as height standard deviation score, body composition changes such as lean body mass and percent body fat, motor and cognitive development using Bayley scale, and safety profiles.

• Journal of Genetic Medicine
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• 제9권2호
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• pp.89-92
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• 2012

Campomelic dysplasia (CMD) is a rare, often lethal, genetic disorder characterized by multiple congenital anomalies and abnormal development of the reproductive organs in males. Mutations in the SOX9 gene are known to cause CMD. We present a Korean CMD girl with a normal 46,XX karyotype and a female reproductive organ phenotype. She was born at 2.35 kg at 38 weeks of gestation and showed characteristic phenotypes, including cleft palate, micrognathia, hypertelorism, flat nasal bridge, congenital bowing of limbs, hypoplastic scapulae, deformed pelvis, and 11 pairs of ribs. She also had an atrioseptal defect of the heart and marked laryngotracheomalacia requiring tracheostomy and tracheopexy. SOX9 mutation analysis revealed the presence of a novel nonsense mutation, $p.Gln369^*$, and the patient was genetically confirmed to have CMD. Although she showed marked failure to thrive and neurodevelopmental delay, she is now 40 months of age and is the only surviving patient with CMD in Korea.

• Neonatal Medicine
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• 제17권1호
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• pp.127-131
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• 2010

저자들은 저긴장증과 다발성 선천성 기형을 보인 신생아에서 염색체 검사 및 FISH로 확인된, 부계의 균형적 전좌에 의한 Emanuel 증후군 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.240-244
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• 1999

저자들은 만성설사와 범발성 혈관내 응고 증후군을 동반한 2개월된 남아에서 장병성 선단 피부염을 의심하여 아연을 경구 투여한 후, 증상호전을 보인 장병성 선단 피부염 환아 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.115-119
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• 2020

Distal renal tubular acidosis (dRTA) is a rare renal tubular disorder characterized by normal anion gap metabolic acidosis, hypokalemia, and high urine pH. It can be inherited or acquired. In untreated pediatric patients with dRTA, rickets and growth retardation are common. We report the case of a 12-year-old Lao girl who presented with typical clinical features of dRTA with severe bone deformities that developed after a bed-ridden state due to a bicycle accident at the age of 8 years. Initial laboratory tests revealed metabolic acidosis with a normal anion gap, hypokalemia, and alkali urine. Renal ultrasonography revealed bilateral medullary nephrocalcinosis. Whole exome sequencing revealed no pathogenic mutations. After treatment with oral alkali, potassium, and vitamin D, she could walk and run. Later, she underwent corrective orthopedic surgeries for bony deformities. Thus, in pediatric dRTA patients, despite severe symptoms remaining untreated, accurate diagnosis and proper management can improve quality of life.

• Clinical and Experimental Pediatrics
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• 제53권10호
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• pp.863-871
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• 2010

The prevalence of pediatric obstructive sleep apnea syndrome (OSAS) is approximately 3% in children. Adenotonsillar hypertrophy is the most common cause of OSAS in children, and obesity, hypotonic neuromuscular diseases, and craniofacial anomalies are other major risk factors. Snoring is the most common presenting complaint in children with OSAS, but the clinical presentation varies according to age. Agitated sleep with frequent postural changes, excessive sweating, or abnormal sleep positions such as hyperextension of neck or abnormal prone position may suggest a sleep-disordered breathing. Night terror, sleepwalking, and enuresis are frequently associated, during slow-wave sleep, with sleep-disordered breathing. Excessive daytime sleepiness becomes apparent in older children, whereas hyperactivity or inattention is usually predominant in younger children. Morning headache and poor appetite may also be present. As the cortical arousal threshold is higher in children, arousals are not easily developed and their sleep architectures are usually more conserved than those of adults. Untreated OSAS in children may result in various problems such as cognitive deficits, attention deficit/hyperactivity disorder, poor academic achievement, and emotional instability. Mild pulmonary hypertension is not uncommon. Rarely, cardiovascular complications such as cor pulmonale, heart failure, and systemic hypertension may develop in untreated cases. Failure to thrive and delayed development are serious problems in younger children with OSAS. Diagnosis of pediatric OSAS should be based on snoring, relevant history of sleep disruption, findings of any narrow or collapsible portions of upper airway, and confirmed by polysomnography. Early diagnosis of pediatric OSAS is critical to prevent complications with appropriate interventions.

• Journal of Chest Surgery
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• 제14권4호
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• pp.381-387
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.