• 대한소아치과학회지
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• 제31권3호
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• pp.481-485
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• 2004

Rabson Mendenhall syndrome은 Rabson과 Mendenhall에 의해 1955년 처음 발표된 증후군으로서, 심한 insulin resistance를 보이는 희귀한 상염색체 열성 유전성 질환이다. Insulin receptor의 돌연변이나 insulin이 작용하는 다른 target cell의 결함에 의해 나타나며, 일반적인 증상으로는 흑색가시세포종(acanthosis nigricans), 다모증(hypertrichosis), 손발톱 비대(onychauxis), 성장 지체 (growth retardation), 성 조숙(precocious puberty), 생식기의 비대, 팽만된 복부(protuberant abdomen), 건성 피부(xerotic skin) 등이 나타난다. 악안면 영역에서는 치아의 이형성, 거친 얼굴의 피부, 하악 전돌, 균열 혀 (fissured tongue)와 같은 특징적 증상이 나타난다. 이에 본 증례에서는 Rabson Mendenhall syndrome으로 본원에 내원한 4세 환아에게 관찰된 치과적 특징들에 관하여 보고하는 바이다.

• Journal of Advanced Marine Engineering and Technology
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• 제8권1호
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• pp.49-63
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• 1984

The 5086-H116 Al-Alloy plate specimens having an edge through-thickness notch were investigated to find out the characteristics of the corner crack propagation by the plane bending fatigue. The experiments were also carried out in order to clarify the change of the corner crack propagation behaviour due to the various materials and their thicknesses. In addition, the retardation effect of overload on the corner crack propagation was quantatively studied. Main results obtained are as follows; 1. In the case of estimating the crack propagation rate of the corner crack, it is more reasonable to consider the growth rate of fracture surface area than that of crack length. 2. The shape of the corner crack growing in the plane plate under the bending fatigue can be estimated. 3. The crack propagation rate increases with the increasing of the thickness and the decreasing of the Young's modulus of materials. 4. Regardless of a thickness and kind of materials of specimen, the characteristics of the corner crack propagation can be concluded. 5. The retardation effect of overload is distinct in the corner crack propagation.

• 한국안전학회지
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• 제12권3호
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• pp.38-44
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• 1997

6063-T5 alloys are tested in laboratory air, water and 3% NaCl solution to investigate the effects of corrosive environment on the retardation behavior through single overload fatigue test. Also, the fatigue crack propagation and the crack closure behavior are studied. The results obtained in this experimental study are summarized as follows. 1) Behaviors of fatigue crack growth retardation are observed in water and 3% NaCl solution as they do in air. The number of delay cycles and the size of affected region by single overload decrease greatly in water and 3% NaCl compared with those in air. 2) In fractographic results, the overload marking by single overload appear remarkably in air, but indistinctly in water and 3% NaCl solution. 3) The effect of crack closure on crack propagation is most remarkable in the beginning of crack propagation. With crack propagation, the crack closure level and its effect decrease greatly.

• 대한구순구개열학회지
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• 제3권1호
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• pp.33-36
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• 2000

Cornelia de Lange syndrome is a disorder of unknown biochemical and geneic basis that is recognized on the basis of characteristic facies(low anterior hairline, synophrys, anteverted nares, maxillary prognathism, long philtrum, carp mouth) in association with prenatal and postnatal growth retardation, mental retardation and, in many cases, upper limb anomalies. We treated the patient with incomplete cleft palate related to Cornelia de Lange syndrome.

• Corrosion Science and Technology
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• 제5권6호
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• pp.189-195
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• 2006

Effects of tungsten (W) on the precipitation kinetics of secondary phases and the associated resistance to pitting corrosion of 25%Cr duplex stainless steels were investigated through microstructural and electrochemical noise analyses. With the partial substitution of W for Mo in duplex stainless steel, the potential and current noises of the alloy were significantly decreased in chloride solution due to retardation of the ${\sigma}$ phase precipitation. The preferential precipitation of the $\chi$ phase in the W-containing alloy during the early period of aging contributed to retarding the precipitation of the $\sigma$ phase by depleting W and Mo along grain boundaries. In addition, the retardation of the nucleation and growth of the $\sigma$ phase in the W-containing alloy appears to be attributed to the inherently low diffusivity of W compared with that of Mo.

• Journal of Yeungnam Medical Science
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• 제36권2호
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• pp.152-154
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• 2019

Cornelia de Lange syndrome (CdLS) is a rare multisystemic disorder that is characterized by mental retardation, prenatal and postnatal growth retardation, limb anomalies, and distinctive facial features, which include arched eyebrows that often meet in the middle (synophrys), long eyelashes, low-set ears, small and widely spaced teeth, and a small and upturned nose. Ophthalmic manifestations include long eyelashes, nasolacrimal duct obstruction, myopia, ptosis, and strabismus. There has been no report of surgical treatment for esotropia and unilateral ptosis in patients with CdLS in Korea. I report a patient with CdLS who underwent surgical treatment for esotropia and unilateral ptosis with a good surgical outcome.

• 대한한의학회지
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• 제24권1호
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• pp.1-8
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• 2003

Purpose : Nowadays, there are many studies of growth retardation treatment by oriental medical therapy. This study was performed to evaluate the effect of Hwalhyulsungjang-san (Huoxuechengzhang-san) and KC101 on physical development in growth, Methods : 60 young (3 weeks old) male rats, whose mean weight was $46.8{\pm}0,7g$, were divided into 5 groups : groups A, B, C, and D were fed an experimental diet containing respectively Hwalhyulsungjang-san 2.5%, Hwalhyulsungjang-san 7.5%, KC101 5.0%, and KC101 10.0%, and the other group were fed an AIN-diet containing neither Hwalhyulsungjang-san nor KC101 as a control. Study of each group used 2 rats and was repeated in 6 times. Body weight, daily weight gain, dietary intake, body length, femur length, backbone length and serum concentration of IGF-I and hemoglobin were measured. Result : 1. Body weight and daily weight gain of group D (KC101 10%) were highest; dietary intake of group D was also increased by 13% over the control group. 2. Average body length of group D was increased to 1.69cm, which was 3.2% over the control group. Backbone length of group D was also increased 4% compared to control group. 3. Serum concentration of IGF-I of group D increased 17% compared to control group. 4. Serum concentration of hemoglobin of group B (Hwalhyulsungjang-san 7.5%) increased 8% compared to control group. Conclusion : This study showed that Hwalhyulsungjang-san and KC101 composed of oriental medicinal stuffs have effect on physical development in growing rats. There were not any statistical meaning compared to control group, but this study suggests that intake of these compounds from a young age may lead to increase physical development in growth.

• Clinical and Experimental Pediatrics
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• 제61권7호
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• pp.221-225
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• 2018

Purpose: Congenital hypothyroidism (CH) is the most common endocrine disorder in children. Thyroid hormone deprivation results not only in mental retardation but also growth retardation. This study investigates the final height (FH) in Korean patients with CH detected by newborn screening and examines factors that may affect the FH. Methods: The medical records of Korean CH patients (n=45) were reviewed. The FH was examined and target height (TH) was calculated based on mid-parental height. The FH z score (FHZ) and TH z score (THZ) were computed using the 2007 Korean National Growth Chart. The FHZ and THZ were compared with a Student t test. The impact of the etiology of CH (athyreosis, dyshormonogenesis, ectopic thyoid, hypoplastic thyroid), initial serum thyroid stimulating hormone (TSH) level, initial free thyroxine (T4) level, and time of therapy initiation based on FH was assessed. Results: The mean FHZ was $0.10{\pm}1.01$ for male patients and $-0.11{\pm}1.09$ for female patients. There were no significant differences between FHZ and THZ for both female (P=0.356) and male patients (P=0.237). No significant relationship was found between FH and the etiology of CH, initial TSH level, initial free T4 level, and the time of therapy initiation. Conclusion: Early intervention and satisfactory management do not appear to impede growth in Korean patients with CH. Thus, early detection and proper management of patients with CH detected by newborn screening program are necessary.

• 미생물학회지
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• 제44권2호
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• pp.98-104
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• 2008

mRNA의 핵에서 세포질로의 이동(mRNA export)에 관여하는 것으로 여겨지는 분열효모 Schizosaccharomyces pombe의 rsm1 유전자의 역할을 알아보기 위해 $kan^{r}$ 유전자를 이용하여 결실돌연변이주(deletion mutant)를 제조하였다. rsm1 유전자는 생장에 필수 유전자는 아니지만, rsm1 결실돌연변이주는 야생형에 비해 생장이 조금 늦고 mRNA export도 약간의 결함을 보였다. rsm1 유전자와 mRNA export의 중요 유전자와의 연관관계를 알아보기 위해, 이중돌연변이주(double mutants)를 제작하여 생장결함 정도와 mRNA export 결함 정도를 조사하였다. 조사한 유전자들 중에서 mex67 또는 npp106 돌연변이 유전자는 rsm1 결실돌연변이 유전자와 함께 존재하면 생장과mRNA export가 더욱 악화되었다. 반면, thp1 돌연변이 유전자는 rsm1 결실돌연변이 유전자와 함께 존재하면 오히려 생장과 mRNA export 정도를 야생형과 유사한 정도로 호전시켰다. 이와 같은 결과들은 rsm1 유전자가 mRNA의 핵에서 세포질로의 이동에 중요한 역할을 담당하고 있음을 시사한다.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제14권1호
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• pp.123-127
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• 2003

Cornelia de Lange 증후군은 여러 신체 기관의 선천적 기형과 성장지연, 신경 발달학적 이상을 동반하는 질환이다. 어떠한 생화학적 혹은 염색체 표지자도 아직까지 Cornelia de Lange 증후군에 특이적인 소견은 발견되지 못하였고 진단은 임상적인 관찰에 전적으로 의존하게 되어있다. 따라서 임상가의 경험이 본 질환의 발견에 중요한 만큼 저자들은 정신지체와 자폐장애를 보이는 Cornelia de Lange 증후군 사례를 보고하는 바이다. 본 증례의 환아는 6세 4개월의 여환으로 걷기 등의 운동발달이 늦었다고 하며 현재까지도 한단어도 말하지 못하는 심각한 언어지체를 보였다. 또한 눈 맞춤이 빈약하고 타인과 정서적 교류가 되지 않는 등 사회적 상호작용의 저하와 상동행동을 보였으며 따라서 정신지체와 자폐장애로 진단 할 수 있었다. 환아는 저신장을 보였고 눈썹이 짙고 속눈썹도 길고 짙었으며 치아 간격이 넓고 코 끝이 들려 있었으며 입주위에 수염이 나 있었다. 낮은 헤어라인을 보였고 몸통과 사지에 다모증을 보였으며 손가락은 짧은 편이었다. 방광 요관 역류와 잦은 상기도 감염과 폐렴의 과거력이 있었고 4차례 열성경련이 있었다. 본 증례의 경우 특징적인 얼굴 모습과 성장발달지연, 정신지체, 행동양상, 동반된 내과적 질환의 병력 등으로 Cornelia de Lange 증후군으로 진단할 수 있었다.