• Neonatal Medicine
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• 제18권1호
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• pp.143-147
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• 2011

ASAuria는 요소회로이상증 중의 하나로 드물게 나타나는 상염색체 열성으로 유전되는 대사 질환이다. 체내에 ASA가 축적됨으로서 신생아 시기에 구토, 기면, 수유 곤란, 의식 장애를 보이며 적절한 조치를 하지 않으면 사망에 이르게 되는 치명적인 대사 질환이다. 이 질환은 혈중과 소변에 ASA가 증가하는 것으로 진단할 수 있다. 국내에서는 고전적 형태의 ASAuria가 아직 보고된 사례는 없으며, 이에 본 저자들은 고전적 ASAuria로 진단된 환아에서 유전자 검사를 통해 보인자 부모로부터 출생하였음을 진단한 신생아 환자 1예를 경험하였기에 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권1호
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• pp.61-64
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• 2017

Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.

• Journal of Dairy Science and Biotechnology
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• 제28권1호
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• pp.53-59
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• 2010

The milk product is one of the major foods which provide calcium for nutritional requirement. In addition to calcium, it supplies diverse nutrients including protein, potassium, vitamin A, vitamin $B_1$, and vitamin $B_2$. Adequate calcium intake throughout lifetime ensures optimum bone density and prevents osteoporosis in old age by reducing loss of bone mineral during growth and aging. The nutritional deficiency of calcium intake and spreading of osteoporosis in Korean population accentuated necessity of adequate calcium intake through milk consumption. Obstruction of milk consumption due to lactose intolerance should be overcome by educational programs for adequate milk consumption and low-lactose milk product developments. Consumption of milk products was reported to prevent high blood pressure in clinical studies and epidemiological investigations. As the feeding of milk increased, the body weight and fat of animal decreased in animal studies. The epidemiological investigations revealed that there was a negative correlation between the amount of milk consumption and the body fat. When calcium was provided as dietary supplement in the clinical trials, the body weight and body fat of subjects decreased. When calcium was particularly supplied as a part of milk, the body weight reduction rate was augmented. Calcium, vitamin D, and conjugated linoleic acid have been reported to exert a role in reducing cancer. The paramount importance of milk in nutrition as a source of calcium and other invaluable nutrients emphasizes consistent supply of milk products to adults as well as adolescents by dairy industry.

• 대한한방소아과학회지
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• 제13권2호
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• pp.41-77
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• 1999

The purpose of this research is that infant artificial feeding products is used in clonic with the study on characteristic, ingredients and indication of geneal and special modified milks. The result is as follows. 1. The main ingredients of four company products-Maeil , Namyang, Pasteur, Aebout is similar but the functional is different 2. General infant formula is divided into 100days, 5-6months, 12months, 24months and 36months out of consideration for growth and development of infant. 3. The indication and sorts of the special infant formula used at a hospital is as follows. PKU-1, PKU-2 formula is available for phenylketonuria. MPA formula is available for propionic acidemia and methylmalonic acidomia. UCD is available for urea cycle disorder Leucine-free formula is available for isovaleric acidemia. Maeil LP is available for hypocalcemia. MCT formula is available for indigestion and malabsorption of fat. BCAA-free formula is available for Maple syrup urine disease. Protein-free formula is available for limit of protein uptake or mixture of peculiar amino acid or higher uptake of mineral, vitamin, calory. Methionine-free formula is available for homocystinuria and hypermethioninemia. Premature infant is available for premature and low birth weight. 4. The special infant formula published in nation is as follows. Maeil soy A, Maeil MF1, Namyang hope doctor and Maeil HA is available for diarrhea. Maeil HA, Maeil HA-21 and Namyang hope allergy is available for hypoallergy. Maeil soy A is available for diarrhea of milk allergy. Maeil MF1 or Namyang hope doctor is available for acute bacterial or viral temporal diarrhea. Maeil HA is available for allergic chronic diarrhea. Maeil HA and Namyang hope allergy as eHP-formula is available for chronic diarrhea for lactose intolerance and milk allergy. Maeil-21 as pHP-formula for neonates with allergy family, allergic symptoms such as atopic dermatitis, asthma except digestive system.

• 대한소아치과학회지
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• 제25권1호
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• pp.93-102
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• 1998

Infant formula in nursing bottle, with inappropriate feeding habits, is major factor associated with the development of nursing caries. Although each infant formula has different carbohydrate and protein composition, studies comparing cariogenic potential of many Korean-branded infant formulas are deficient. In addition, it is on the point of being difficult to evaluate the cariogenecity of milk due to development of many infant formulas. In this study, to evaluate the cariogenic potential of many infant formulas, after oral rinse with six Korean-branded infant formulas(three milk based formulas, one soy based formula and two specific formulas for infants with allergy to milk protein and with lactose intolerance) for ten adult volunteers(eight males and two females), plaque pH change was measured with In vivo/In vitro combination technique and results were as follows. 1. All six different kinds of Korean-branded commercial infant formulas dropped the plaque pH significantly(p<0.05) and at an hour after rinse, plaque pH was not recovered in most of subjects. 2. Soy based infant formula and casein-hydrolyzated infant formula containing no casein dropped the plaque pH significantly more than milk based infant formula containing casein (p<0.05). 3. In the milk protein of infant formulas, casein had more effect on buffering the pH change of the infant formula than whey protein and casein-hydrolyzated infant formula had a reduced effect of casein. 4. In infant formulas with similar protein composition, infant formula containing sucrose dropped plaque pH more than infant formula containing lactose, but there was no significant difference (p>0.05).

• Clinical and Experimental Pediatrics
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• 제51권7호
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• pp.704-712
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• 2008

목 적 : 최근 모유수유의 장점이 부각되고 있으나 모유가 미숙아 전용분유에 비해 열량을 포함한 영양소들이 미숙아들의 적절한 성장을 유도하기에는 낮아 이를 극복하기 위한 모유강화제가 개발되었다. 본원에서는 극소저체중출생아들을 대상으로 모유강화제를 첨가한 모유를 수유한 경우와 미숙아 전용분유를 수유한 경우를 비교하여 성장에 어떠한 효과를 미치는지 알아보기로 했다. 방 법 : 2005년 10월부터 2006년 12월까지 동아대학교의료원에서 출생하여 신생아 집중치료실에 입원한 출생체중 1,500 g 이하, 재태주령 33주 미만인 극소저체중출생아들을 대상으로 강화된 모유수유군과 미숙아 전용분유 수유군으로 나누어 전반적인 특징, 영양상태, 성장 추이 및 합병증의 발생 등을 전향적으로 비교 조사하였다. 대상 환자들 중 본원에서 추적관찰이 가능했던 환자들의 경우 외래에서 1, 3, 6개월에 걸쳐 체중, 신장, 두위를 측정하여 비교하였다. 결 과 : 두 군의 평균 재태주령 및 출생체중은 각각 $30^{+1}{\pm}2^{+1}$주과 $29^{+4}{\pm}2^{+1}$주, $1,281.30{\pm}178g$과 $1,234.32{\pm}193g$으로 통계학적으로 차이가 없었으며(P=0.26, P=0.38), 성비, 괴사성 대장염, 패혈증, 신생아호흡곤란증후군, 동맥관개존증, 뇌 내출혈 및 뇌실 내 출혈 등의 합병증의 비율, 평균 재원 기간에서도 유의한 차이는 없었다. 수유 시작 시기는 강화된 모유수유군과 미숙아 전용분유 수유군에서 각각 생후 $8.00{\pm}3.27$일 및 $8.86{\pm}5.37$일(P=0.99), 완전수유 도달 시기는 각각 수유시작 후 $41.78{\pm}20.47$일 및 $36.86{\pm}20.63$일로(P=0.55) 통계적으로 유의한 차이가 없었으며, 총정맥영양 기간 및 수유 곤란증상 횟수에도 유의한 차이는 없었다. 수유시작 시점부터의 체중, 신장, 두위의 성장 속도 및 절대치도 두 군 간에 유의한 차이가 없었으나 각 시점의 평균치를 비교하였을 때 수유 시작 후 7주째 체중은 강화된 모유수유군에서 더 빨리 증가하였다(P=0.03). 환자들의 초기 체중 감소 후 출생체중까지 회복되는데 걸리는 기간도 각각 $22.3{\pm}6.92$일 및 $24.32{\pm}10.68$일로 통계학적 차이는 없었으며(P=0.41), 체중 회복 후부터 퇴원까지의 체중 및 두위, 신장의 증가속도는 두 군에서 유의한 차이는 없었다. 퇴원 후 외래 추적 관찰을 한 각 군의 환자들의 체중, 신장, 두위의 평균치에도 유의한 차이는 없었다. 결 론 : 극소저체중출생아에서 모유강화제를 포함한 모유수유군과 미숙아 전용분유 수유군 간의 전반적인 성장 상태, 생화학적 검사 수치 및 합병증 발생에는 유의한 차이가 없어 모유 강화제는 고열량 수유를 필요로 하는 극소저체중출생아에서 부가적인 선택 사항이라고 하겠다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제14권1호
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• pp.52-58
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• 2011

목 적: 극소저출생체중아 태변 장폐색은 장관 영양을 지연시키고 장폐색을 유발하는 주요 원인 중 하나로 고삼투압 수용성 조영제 관장이 진단과 치료의 유용성에 대한 원칙은 아직 논란 중이다. 본 연구는 태변 장폐색을 보인 극소저출생체중아의 임상 소견을 관찰하고, 고삼투압 수용성 조영 관장 후 장폐색 증상 호전, 장관영양의 진행, 합병증 등을 관찰하고자 하였다. 방 법: 극소저출생체중아 중에 태변 장폐색으로 고삼투압 수용성 조영제 관장을 시행한 14명의 임상 소견과 방사선 소견, 장관 영양의 진행, 고삼투압 수용성 조영제 관장 치료 후 호전 여부, 고삼투압 수용성 조영제 관장과 연관된 합병증 등을 관찰하였다. 이들의 주요임상 소견을 장폐색 증상 없이 수유 진행이 가능하였던 극소저출생체중아 18명과 비교 하였다. 결 과: 1) 태변 장폐색을 보인 극소저출생체중아에서 태변 장폐색이 없었던 극소저출생체중아에 비해 낮은 5분 Apgar 점수를 보였다(p<0.05). 또한, 출생 후 마지막 태변 배출시기(일), 첫 수유 시작 시기(일), 전 장관 영양 도달 시기(일)들도 의미 있게 지연되었다(p<0.05). 2) 고삼투압 수용성 조영제 관장은 14명에서 18 회를 시행하였다. 18회 중 12회에서 조영제가 회장 말단에 도달하였으며 이 중 11회에서 관장 후 다량의 태변 배출과 함께 장폐색 증상의 호전이 있었으나, 1예는 호전이 없어 회장절개술로 태변을 제거하였다. 3) 조영제가 회장 말단에 도달 못한 5명 중 3명에서 증상의 호전이 없어 조영제 관장을 재시행 하여 조영제가 회장말단에 도달하였고 장폐색 증상이 호전되었다. 4) 고삽투압 수용성 조영제 관장과 연관된 특별한 합병증은 없었다. 결 론: 극소저출생체중아 태변 장폐색에서 고삼투압수용성 조영제 관장은 비교적 안전하게 시행할 수 있는 치료 방법이었다.

• Journal of Chest Surgery
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• 제47권5호
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• pp.444-450
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• 2014

Background: We aimed to evaluate the efficacy and safety of early surgical ligation (within 15 days of age) over late surgical ligation (after 15 days of age) by a comparative analysis of very low birth weight (VLBW) infants undergoing surgical correction for symptomatic patent ductus arteriosus (PDA) over the course of 6 years in our hospital. Methods: We retrospectively reviewed all the medical records in the neonatal intensive care unit at Hanyang University Seoul Hospital, from March 2007 to May 2013, to identify VLBW infants (<1,500 g) who underwent surgical PDA ligation. Results: The gestational age (GA) in the late ligation (LL) group was significantly younger than in the early ligation (EL) group (p=0.010). The other baseline characteristics and preoperative conditions did not differ significantly between the two groups. The intubation period before surgery (p<0.001) and the age at surgery (p<0.001) were significantly different. The postoperative clinical outcomes of the study patients, including major morbidity and mortality, are summarized. There were no significant differences in bronchopulmonary dysplasia, sepsis, or mortality between the EL and the LL groups. However, the LL group was significantly associated with an increased risk of necrotizing enterocolitis (p=0.037) and with a prolonged duration of the total parenteral nutrition (p=0.046) after adjusting for GA. Conclusion: Early surgical ligation for the treatment of PDA that failed to close after medical treatment or in cases contraindicated for medical treatment might be desirable to reduce the incidence of necrotizing enterocolitis and to alleviate feeding intolerance in preterm infants.

• 간호행정학회지
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• 제6권1호
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• pp.135-146
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• 2000

The purpose of this study was to develope, based on the Nursing Intervention Classification (NIC) system. a set of standardized nursing interventions which had been validated. and their associated activities. for use with nursing diagnoses related to home health care for women who have had a caesarian delivery and for their newborn babies. This descriptive study for instrument development had three phases: first. selection of nursing diagnoses. second, validation of the preliminary home health care interventions. and third, application of the home care interventions. In the first phases, diagnoses from 30 nursing records of clients of the home health care agency at P. medical center who were seen between April 21 and July 30. 1998. and from 5 textbooks were examined. Ten nursing diagnoses were selected through a comparison with the NANDA (North American Nursing Diagnosis Association) classification In the second phase. using the selected diagnoses. the nursing interventions were defined from the diagnoses-intervention linkage lists along with associated activities for each intervention list in NIC. To develope the preliminary interventions five-rounds of expertise tests were done. During the first four rounds. 5 experts in clinical nursing participated. and for the final content validity test of the preliminary interventions. 13 experts participated using the Fehring's Delphi technique. The expert group evaluated and defined the set of preliminary nursing interventions. In the third phases, clinical tests were held at in a home health care setting with two home health care nurses using the preliminary intervention list as a questionnaire. Thirty clients referred to the home health care agency at P. medical center between October 1998 and March 1999 were the subjects for this phase. Each of the activities were tested using dichotomous question method. The results of the study are as follows: 1. For the ten nursing diagnoses. 63 appropriate interventions were selected from 369 diagnoses interventions links in NlC., and from 1.465 associated nursing activities. From the 63 interventions. the nurses expert group developed 18 interventions and 258 activities as the preliminary intervention list through a five-round validity test 2. For the fifth content validity test using Fehring's model for determining lCV (Intervention Content Validity), a five point Likert scale was used with values converted to weights as follows: 1=0.0. 2=0.25. 3=0.50. 4=0.75. 5=1.0. Activities of less than O.50 were to be deleted. The range of ICV scores for the nursing diagnoses was 0.95-0.66. for the nursing interventions. 0.98-0.77 and for the nursing activities, 0.95-0.85. By Fehring's method. all of these were included in the preliminary intervention list. 3. Using a questionnaire format for the preliminary intervention list. clinical application tests were done. To define nursing diagnoses. home health care nurses applied each nursing diagnoses to every client. and it was found that 13 were most frequently used of 400 times diagnoses were used. Therefore. 13 nursing diagnoses were defined as validated nursing diagnoses. Ten were the same as from the nursing records and textbooks and three were new from the clinical application. The final list included 'Anxiety', 'Aspiration. risk for'. 'Infant behavior, potential for enhanced, organized'. 'Infant feeding pattern. ineffective'. 'Infection'. 'Knowledge deficit'. 'Nutrition, less than body requirements. altered', 'Pain'. 'Parenting'. 'Skin integrity. risk for. impared' and 'Risk for activity intolerance'. 'Self-esteem disturbance', 'Sleep pattern disturbance' 4. In all. there were 19 interventions. 18 preliminary nursing interventions and one more intervention added from the clinical setting. 'Body image enhancement'. For 265 associated nursing activities. clinical application tests were also done. The intervention rate of 19 interventions was from 81.6% to 100%, so all 19 interventions were in c1uded in the validated intervention set. From the 265 nursing activities. 261(98.5%) were accepted and four activities were deleted. those with an implimentation rate of less than 50%. 5. In conclusion. 13 diagnoses. 19 interventions and 261 activities were validated for the final validated nursing intervention set.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권2호
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• pp.133-141
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• 2001

위식도역류는 영아들에 있어서 흔하게 있다고 알려져 있고 특히 미숙아들에게 더 흔히 있다고 알려져 있지만 아직 이에 대한 연구는 부족하다. 목적: 건강하게 자라고 있는 미숙아들에게 위식도역류의 진단에 가장 예민하다고 알려져 있는 24시간 식도 pH 검사를 통하여 그 빈도 및 연관 인자를 찾고자 하였다. 방법: 특이 증상 없이 건강하게 자라고 있는 미숙아 21명(평균 재태기간: $30+{\pm}2+0$주, 출생시의 평균 체중은 $1,468{\pm}329$ g, 검사 당시의 평균 나이는 $29{\pm}8$일, 수태 후 나이(Postconceptional age: 재태기간+출생 후 나이)는 $34+6{\pm}1+4$주, 체중은 $1,750{\pm}329$ g, 남아 : 여아=15:6)을 대상으로 휴대용 레코더가 연결되어 있는 실리콘 재질의 마이크로 소식자를 Stobel의 공식($0.252{\times}$키+5 (cm))에 따라 코로 넣어 그 거리만큼 식도내강에 위치시킨 후 거리를 교정하여 24시간 동안 식도 pH 검사를 시행하였다. 결과: 네 가지 파라미터(number of acid reflux, number of long acid reflux (5 min), longest acid reflux minutes, RI)를 제시하였고 전체 대상환아의 57%에서 유의한 위식도역류가 있음을 보였다. 위식도역류와 number of acid reflux, RI의 두 파라미터가 통계적으로 의미있는 상관관계를 보였으며 이 두 파라미터와 Reflux index of the postprandial 120 min간에도 의미있는 상관관계가 있음을 알 수 있었다. 위식도역류가 있는 환아들을 출생체중, 재태기간, 검사 당시의 나이, 수태 후 나이, 체중, 성별 및 테오필린(theophylline) 투약 여부에 따라 각각 구분하여 그 차이를 살펴보았고 모두 통계학적으로 큰 차이는 없었다. 소식자의 거리는 Stobel의 공식에 의한 거리와 실제길이 사이에 차이가 있어 우리의 미숙아의 경우에는 $0.252{\times}$키+3.7 (cm)로 적용시킬 수 있었다. 결론: 위와 같은 결과로 미숙아들에게 있어서 무증상적, 유의한 위식도역류가 57%의 높은 빈도로 나타났고, 위식도역류증의 진단에 가장 중요한 파라미터는 number of acid reflux, RI와 Reflux index of the postprandial 120 min로 나타났다. 출생체중, 재태기간, 검사 당시의 나이, 수태 후 나이, 체중 및 성별, 테오필린 투약여부 등과 위식도역류와는 통계적으로 유의성이 없었다. 또한 1세 이하의 영아에게 소식자의 위치를 정하는데 사용되는 Strobel의 공식을 본 연구대상 미숙아들에게 적용해본 결과 소식자의 위치가 $0.252{\times}$키+3.7(cm)로 나타났다.