• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권4호
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• pp.217-223
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• 2015

The obesity epidemic is a worldwide problem. Factors predisposing to obesity include genetics, race, socioeconomic conditions, birth by cesarean section, and perinatal antibiotic use. High protein (HP) content in infant formulas has been identified as a potential culprit predisposing to rapid weight gain in the first few months of life and leading to later obesity. In a large multicountry study the effects of lower protein (LP) formula (1.77 and 2.2 g protein/100 kcal, before and after the 5th month, respectively) were compared to those of higher protein (2.9 and 4.4 g protein/100 kcal, respectively). Results indicated that at 24 months, the weight-for-length z score of infants in the LP formula group was 0.20 (0.06, 0.34) lower than that of the HP group and was similar to that of the breastfed reference group. The authors concluded that a HP content of infant formula is associated with higher weight in the first 2 years of life but has no effect on length. LP intake in infancy might diminish the later risk of overweight and obesity. At 6 years of age HP children had a significantly higher body mass index (by 0.51; 95% confidence interval [CI], 0.13-0.90; p=0.009) and a 2.43 (95% CI, 1.12-5.27; p=0.024) fold greater risk of becoming obese than those who received the LP. In conclusion, several factors may influence development of metabolic syndrome and obesity. Breastfeeding should always be encouraged. An overall reduction of protein intake in formula non breastfed infants seems to be an additional way to prevent obesity.

• Archives of Plastic Surgery
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• 제32권1호
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• pp.29-36
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• 2005

Craniosynostosis is the premature fusion of one or more sutures of either cranial vault or base. Fused sutures may impede normal growth of the calvaria, leading to characteristic skull deformities; Morphological craniosynostosis is classified descriptively. Being craniosynostosis uncorrected the deformity progresses continuously and causes an increase of intracranial pressure. The surgical involvement aims at the expansion of intracranial space as well as satisfactory achievement of craniofacial shape. Early surgical correction in infancy prevents the deformity from the further progression and possible associated complication of high intracranial pressure. A long period of follow-up is essential to asses the outcome of an effectiveness of the surgery. measurement of intracranial volume has been concerned in medical personnel and anthropologists for many years. A reliable and accurate measurements of the intracranial volume facilitates to make a diagnosis and treatment of craniosynostosis. Pre-and postoperative change of intracranial volume was evaluated with 3D CT scanning in 12 cases of craniosynostosis who underwent frontal advancement and total cranial vault remodeling. Increased intracranial volume is attributed to surgical release of craniosynostosis and natural growth. We conceive that the intracranial volume is significantly increased after surgical correction of fused cranial sutures and along with natural growing. A procedure of frontal advancement and total cranial vault remodeling is very useful to correct such a deformity as craniosynostosis. And also 2 cases out of five mentally retarded patients improved remarkably and Forehead retrusion or temporal depression followed in another two cases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권1호
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• pp.104-107
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• 2001

저자들은 5개월 된 남아에게 장중첩증 원인으로 흔하지 않은 게실로 판명되었던 1례를 치험하여 문헌 고찰과 함께 보고하는 바이다.

• 대한한방소아과학회지
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• 제19권2호
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• pp.197-213
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• 2005

Objective : The purpose of this study was to investigate chief complaints of pediatric outpatients .Fin the oriental hospital and search for a trend of patients and their symtoms. Method : The study was composed of 2,915 new patients aged between 0 and 20 who had been visited pediatrics in ${\bigcirc}{\bigcirc}$ university oriental hospital from 2001 to 2004. Results : The percentage of new patients decreased every year with 30.7% in 2001, 25.5% in 2002, 24.1% in 2003, 17.8% in 2004. The age distribution showed 9.9% in infancy patients, 62.6% in early childhood, 16.8% in late childhood, 10.6% in adolescence. The percentage of infants decreased every year with 13.3% in 2001, 9.4% in 2002, 8.8% in 2003, 7.1% in 2004. The percentage of adolescents increased every year with 8.0% in 2001, 9.6% in 2002, 12.8% in 2003, 13.5% in 2004. Systemic division of chief complaints was respiratory symtoms(37.6%), general symtoms(26.1%), digestive symtoms(19.2%), skin symtoms(6.8%), nervous and mental symtoms (6.1%), urinary symtoms(2.2%), musculoskeletal symtoms(1.3%) and others(0.7%) in order of frequency. The main chief complaints were nasal mucus/obstruction(17.5%), weakness(16.9%), cough(12.8%), loss of appetite(9.8%), inappropriate growth(4.3%), atopic skin problem(3.4%), repeated common cold(2.4%), abdominal pain(2.4%), vomiting (2.2%), diarrhea(2.0%) and fever (1.9%). Loss of appetite, inappropriate growth and repeated common cold increased every year. Diarrhea and convulsion decreased every year. Conclusions : The two main causes of visit of patients were respiratory problems and improvement of general condition. More data of patients should be accumulated for further study. Not only analysis according to chief complaint, but also analysis according to diagnosis will accomplish more aaccurate information about disease of childrens in the future.

• Child Health Nursing Research
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• 제5권3호
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• pp.241-249
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• 1999

Diaper dermatitis, or commonly called 'diaper rash', is among the most prevalent cutaneous disorders of infancy and early childhood and important issue in nursing. The term itself is not diagnostic since it encompasses a variety of acute inflammatory reactions which are best regarded as a family of disorders arising from a combination of factors specifically attributable to the use of diapers Intensive study of the rash Process has shown that skin wetness and fecal enzyme activity are damaging to skin and lead to the development of diaper rash. This suggests that it is important to keep urine away from babies' skin, so that the skin remains as dry as Possible and maintains its barrier function abilities. Controlling the urine will also minimize the mixing of urine and feces within the diaper. which helps prevent the increased activity of enzymes that attack the skin and cause irritation. Therefore, a diaper that keeps the skin drier and limits the mixing of urine and feces will help Prevent the conditions that lead to diaper rash. Since their introduction about 35 years ago, disposable baby diapers have undergone many design and performance changes. In Particular. the Performance of diapers was advanced by the introduction of absorbent gel materials (AGMs) to Provide advantages in skin care. dryness, and leakage Protection Especially, important was the introduction of AGM which increased the absorbent capacity of the diaper several fold and Yielded marked reductions in the degree of skin hydration occurring under the diaper Studies show not only drier skin but more stable skin pH and less dermatitis with AGM diapers than with home-laundered cloth diapers or single-use diapers without AGM Minimizing diaper area skin wetness is important for managing and Preventing diaper rash. To minimize wetness, parents should use super absorbent diapers.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.262-266
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• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• 대한두개안면성형외과학회지
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• 제12권2호
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• pp.102-106
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• 2011

Purpose: It is accepted universally that correction of the cleft lip nasal deformity requires multiple stages of surgery. Following primary lip repair in infancy or early childhood, secondary surgery to improve the deformity of the lip and nose is frequently necessary. A suitable surgical procedure to correct the accompanying deformity, such as cleft palate and alveolus, must be carried out at an appropriate age. In developing countries, it is common for patients with cleft lip nasal deformity to present severe secondary deformities in adolescence, because of poor follow-up and inappropriate surgery. Methods: The first patient was a 12 year old Mongolian boy. He presented prominent lip scar, short lip, wide columella, asymmetric nostril, palatal fistula, cleft alveolus, and velopharyngeal incompetence. He underwent cheilorhinoplasty, transpositional flap, alveoloplasty by iliac bone graft, and sphincter pharyngoplasty. On follow-up, a bilateral maxillary hypoplasia and a class III malocclusion developed. He underwent LeFort I osteotomy and maxillary advancement at the age of 16 years. The second patient was an 18 year old Eastern Russian girl. She presented with a deviated nose, right alar base depression, short lip, protrusion on vermilion, large palatal fistula, and severe VPI due to short palate. She underwent the combined procedure of cheilorhinoplasty, corrective rhinoplasty, tongue flap for palatal fistula, and superiorly based pharyngeal flap. And the tongue flap was detached at postoperative 3 weeks. Results: The overall results have been extremely pleasing and satisfactory to patients. There were no postoperative complications. Conclusion: We discovered the one stage operation for radical correction was sufficient procedure to provide excellent clinical outcomes in patients with severe cleft lip nose deformity.

• Clinical and Experimental Pediatrics
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• 제61권12호
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• pp.397-402
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• 2018

Purpose: Transcatheter device closure of patent ductus arteriosus (PDA) is challenging in early infancy. We evaluated PDA closure in infants less than 6 months old. Methods: We performed a retrospective review of infants less than 6 months of age who underwent attempted transcatheter device closure in our institution since 2004. To compare clinical outcomes between age groups, infants aged 6-12 months in the same study period were reviewed. Results: A total of 22 patients underwent transcatheter PDA closure during the study period. Patient mean age was $3.3{\pm}1.5months$, and weight was $5.7{\pm}1.3kg$. The duct diameter at the narrowest point was $3.0{\pm}0.8mm$ as measured by angiography. The most common duct type was C in the Krichenko classification. Procedural success was achieved in 19 patients (86.3%). Major complications occurred in 5 patients (22.7%), including device embolization (n=1), acquired aortic coarctation (n=2), access-related vascular injury requiring surgery (n=1), and acute deterioration requiring intubation during the procedure (n=1). Two patients had minor complications (9.1%). Twenty-four infants aged 6-12 months received transcatheter device closure. The procedural success rate was 100%, and there were no major complications. The major complication rate was significantly higher in the group less than 6 months of age (P=0.045). There was a trend toward increased major complication and procedural failure rates in the younger age group (P<0.01). Conclusion: A relatively higher incidence of major complications was observed in infants less than 6 months of age. The decision regarding treatment modality should be individualized.

• Journal of Genetic Medicine
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• 제19권1호
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• pp.22-26
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• 2022

Cerebrotendinous xanthomatosis (CTX) is a rare genetic disease caused by a deficiency of enzymes for the synthesis of bile acid, resulting in the accumulation of cholestanol with reduced chenodeoxycholic acid (CDCA) production and causing various symptoms such as chronic diarrhea in infancy, juvenile cataracts in childhood, tendon xanthomas in adolescence and young adulthood, and progressive neurologic dysfunction in adulthood. Because oral CDCA replacement therapy can effectively prevent disease progression, early diagnosis and treatment are critical in CTX. This study reports the case of CTX in a 10-year-old male who presented with Achilles tendon xanthoma and mild intellectual disability. Biochemical testing showed normal cholesterol and sitosterol levels but elevated cholestanol levels. Genetic testing showed compound heterozygous variants of CYP27A1, c.379C>T (p.Arg127Trp), and c.1214G>A (p.Arg405Gln), which confirmed the diagnosis of CTX. The patient had neither cataracts nor other focal neurologic deficits and showed no abnormalities on brain imaging. The patient received oral CDCA replacement therapy without any adverse effects; thereafter, the cholestanol level decreased and no disease progression was noted. The diagnostic possibility of CTX should be considered in patients with tendon xanthoma and normolipidemic conditions to prevent neurological deterioration.

• Clinical and Experimental Pediatrics
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• 제51권4호
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• pp.372-376
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• 2008

목 적 : 신생아기와 조기 영아기에 발생한 기계환기요법이 필요했던 중증 respiratory syncytial virus (RSV) 감염의 임상적 특성을 고찰하여 그 임상적 특성을 파악함으로써 이 질환의 진료에 도움이 되는 임상 정보를 제공하고자 하였다. 방 법 : 2005년 3월부터 2007년 7월까지 분당서울대학교병원 중환자실에 RSV 감염으로 입원한 6개월 미만의 신생아와 영아들 중 기계환기요법을 필요로 했던 12명의 의무기록을 열람하여 임상적 특성을 분석하였다. RSV 감염은 환아의 비인두흡인물 또는 경기관지흡인액 내에서 RSV 항원이 효소면역법 또는 PCR 방법으로 검출된 경우로 하였다. 결 과 : 입원 시 환아들의 나이는 $35{\pm}15$일(범위, 14-59일)이었고 모두 RSV 유행기인 9월부터 3월 사이에 입원하였다. 환아들의 출생체중은 $2.8{\pm}0.6kg$, 재태연령은 $37{\pm}2$주였다. 12명의 환아들 중 4명은 미숙아였으며 이중 3명은 재태연령 34주에서 36주 사이의 준만삭아였다. 10명의 환아들이 입원 $4{\pm}2$일 전부터 경미한 상기도 감염 증상(비 폐쇄, 기침, 콧물)을 보였고 입원 $0{\pm}1$일 후에는 모든 환아들이 뚜렷한 호흡기 이상 징후(빈호흡, 흉곽함몰, 청색증, 비익확장)를 보였다. 9명의 환아들이 무호흡을 보였고, 이 중에 5명은 무호흡 자체가 기계환기요법의 사유가 되었다. 무호흡이 의료진에 의해 확인된 시점부터 뚜렷한 호흡기 이상 징후 또는 청진 상 나음 또는 천명 소견이 나타난 시점까지 $1{\pm}2$일이 걸렸으며, 이 중에 3명은 무호흡이 의료진에 의해 확인되었을 때 뚜렷한 호흡기 이상 징후 또는 진찰소견을 보이지 않았고 1-3일이 지난 후에야 그러한 징후 또는 진찰소견을 보였다. 환아들의 기계환기요법 기간은 $3{\pm}2$일, 중환자실 체류기간은 $6{\pm}2$일이었고 입원 중 사망한 환아는 없었다. 결 론 : RSV는 미숙아로 태어난 경우가 아니라도 생후 2개월 미만의 신생아 및 조기 영아들에게 중증의 호흡기 감염을 초래할 수 있으며 뚜렷한 호흡기 이상 징후가 나타나기 전에 치명적인 무호흡이 발생할 수 있으므로 이 연령의 영아들이 RSV 유행기에 경미한 상기도 감염 증상을 보이거나 RSV 감염에 노출될 위험이 높은 상황에서는 매우 조심스러운 추적관찰이 필요할 것으로 생각된다.