• Annals of Pediatric Endocrinology and Metabolism
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• 제23권4호
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• pp.176-181
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• 2018

Noonan syndrome (NS) is an autosomal dominant disorder that involves multiple organ systems, with short stature as the most common presentation (>70%). Possible mechanisms of short stature in NS include growth hormone (GH) deficiency, neurosecretory dysfunction, and GH resistance. Accordingly, GH therapy has been carried out for NS patients over the last three decades, and multiple studies have reported acceleration of growth velocity (GV) and increase of height standard deviation score (SDS) in both prepubertal and pubertal NS patients upon GH therapy. One year of GH therapy resulted in almost doubling of GV compared with baseline; afterwards, the increase in GV gradually decreased in the following years, showing that the effect of GH therapy wanes over time. After four years of GH therapy, ~70% of NS patients reached normal height considering their age and sex. Early initiation, long duration of GH therapy, and higher height SDS at the onset of puberty were associated with improved final height, whereas gender, dosage of GH, and the clinical severity did not show significant association with final height. Studies have reported no significant adverse events of GH therapy regarding progression of hypertrophic cardiomyopathy, alteration of metabolism, and tumor development. Therefore, GH therapy is effective for improving height and GV of NS patients; nevertheless, concerns on possible malignancy remains, which necessitates continuous monitoring of NS patients receiving GH therapy.

• Journal of Yeungnam Medical Science
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• 제38권1호
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• pp.47-52
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• 2021

Background: Short stature is defined as a height below the 3rd percentile or more than two standard deviations below the mean for a given age, sex, and population. There have been inconsistent results regarding craniofacial morphology in short-statured children. This study aimed to analyze the differences between short-statured children with growth hormone deficiency, idiopathic short-statured children, and normal children. Methods: Thirty-one short-statured children with growth hormone deficiency, 32 idiopathic short-statured children, and 32 healthy children were enrolled in this study. The measurements of their craniofacial structures from lateral cephalograms were evaluated. Results: There were statistically significant differences among the three groups seven variables (anterior cranial base length, posterior cranial base length, total cranial base length, upper posterior facial height, posterior total facial height, mandibular ramus length, and overall mandibular length) in the linear measurement and five variables (saddle angle, gonial angle, mandibular plane angle, position of mandible, and maxilla versus mandible) in the angular measurement. Conclusion: Compared to the control group, many linear and angular measurements of the craniofacial structures were significantly different in the two short-statured groups (p <0.05). Treatment plans by orthodontists should include these craniofacial structure characteristics.

• 한방비만학회지
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• 제5권1호
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• pp.97-107
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• 2005

Objectives The purpose of this study was to investigate correlation between short stature and obese degree. And we also wanted to know the characteristics of patients visited clinic in the name of 'short stature'. Methods Height, body weight, BMI, fat mass, lean body mass, percent body fat were taken intended for 236 patients in the name of short stature. To all the patient questionnaire for growth clinic was drawn up, and ultrasound scan was taken through calcaneous of the right foot. The patients were classified to three groups - short, average, tall group - according to relative position of mid-parental height. It was analysed that the differences between groups in obese degree. Also was investigated correlation between position of short stature and obese degree, and between obese degree and bony maturity. Results & Conclusion 1. The average ages of patients in the name of 'short stature' were $12.69{\pm}3.93$ years old in boys, $10.66{\pm}3.67$ years old in girls. And it seemed to be just before second rapid maturing period. 2. The average BMI were $20.58{\pm}4.07kg/m^2$ in boys, $18.65{\pm}2.85kg/m^2$ in girls, and average percent body fat were $21.99{\pm}7.35%$ in boys, $26.01{\pm}6.35%$ in girls. 3. The numbers of obese children were 34(31.2%) in boys, 19(14.9%) in girls on the basis of BMI. And the numbers were 39(35.8%) in boys, 53(41.7%) in girls on the basis of percent body fat. There was a big difference in case of girls. 4. The numbers of AG(average group) were 48(44.0%) in boys, 60(47.2%) in girls, the numbers of SG(short group) were 35(32.1%) in boys, 31(24.4%) in girls, and numbers of TG(tall group) were 26(23.9%) in boys, 35(27.6%) in girls. 5. There were no significant differences among the groups in BMI, lean body mass, fat mass, percent body fat. Only significant difference in DI(disease index). 6. There were no significant correlation between PH(percent height) and BMI, leanbody mass, fat mass, percent body fat. Only significant correlation in DI(disease index). 7. There were no significant differences between DA(difference between bone age and chronorogical age) and BMI, leanbody mass, percent body fat. Only significant correlation in fat mass.

• 대한소아치과학회지
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• 제38권2호
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• pp.161-169
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• 2011

상악 치조골은 상악골의 일부로 치근을 감싸고 지자하는 역할을 하며 치아의 결손 시 지지구조물로 치료에 중요한 역할을 하게 된다. 지금까지 치조골의 성장에 관한 연구는 교정적 치료의 관점에서 이루어져 영구치 결손을 위한 치료법을 위한 자료로 사용하기에는 한계가 있다. 따라서 본 연구에서는 성장기에 있는 한국인 여학생들의 측모두부규격방사선사진들을 연도 별로 수집하여 상악 치조골의 성장 양상을 분석하였다. 한국인 여학생들의 만 8세에서 만 14세까지 2년 간격으로 측모두부규격방사선 사진, 키, 역연령을 수집하였다. 여섯 항목을 계측하여 각 항목의 평균 최소값 및 최대값을 구하고 각 예측 항목과 역연령, 키 사이의 상관관계 및 동일 항목에서 나이 군사이의 상관관계를 분석하였으며 성장량 예측을 위한 선형회귀분석을 시행하였다. 상악 치조골은 14세까지 계속 성장하였으나 성장속도는 점차 감소하여 키의 성장 양상과 차이를 보였다. 역연령 및 키와 치조골 높이 사이의 상관 계수는 차이를 보이지 않았으며, 역연령 군 사이의 상관계수는 매우 높게 나타났다. 따라서 역연령을 기초로 하는 선형회귀분석을 시행하여 14세 때의 치조골 높이 예측 공식을 작성하였다. 이러한 결과는 소아 상악 치조골의 성장 양상 이해와 성장량을 예측을 가능하게 하여 임플란트 시술과 같은 적극적 치료에 도움을 줄 수 있을 것으로 기대된다.

• 산업공학
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• 제13권2호
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• pp.147-156
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• 2000

It is known that the human stature and its related dimensions generally increase until the late teens or early twenties, remain relatively constant throughout early adulthood, and decline from early-to-middle adulthood into old age. However, there is exception that the length of the human ears, which continue to grow throughout life. In this study, 600 Korean male and female subjects aging from 17 to 89 were selected. Four different points of external ears including Pinna length, ear connection point length, Concha length, and Lobule thickness were measured along with other relative subjects' demographic data(e.g., age, stature, weight) to determine the relationship among these obtained data. The results showed that the age, sex, and different ethnic populations were functions of ear dimensions similar to the human stature. For example, the Pinna length increases as the age increases(r= 0.689, p<0.00l). The primary objective of this study was thus to provide anthropometric dimensions of Korean ear for the product designers and recommend the appropriate product design solutions in relation to human ear. Based on the results of this study, it is suggested that the continuing growth of the human body parts should be considered in the application of anthropometric data to the design of the things especially for human use.

• 사회복지연구
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• 제45권2호
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• pp.173-202
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• 2014

본 연구는 저신장장애 자녀를 둔 부모의 양육경험을 탐색적으로 고찰하기 위해 질적연구를 수행하였다. 내부자 관점을 반영할 수 있는 심층면담을 수행하여 저신장장애 자녀를 양육하면서 겪게 되는 양육자의 고유한 현실과 그 본질적 의미를 도출하였다. 저신장장애 자녀를 키우는 부모의 고유한 양육경험을 관통하는 대주제는 '자라날 수 없는 아이'에서 '자라날 수 있는 아이'로 키우기"로 확인되었다. 참여자들은 자녀의 '자라날 수 없음'에 대한 큰 충격과 실망을 경험하지만 자녀를 '자라날 수 있는 아이'로 만들기 위해 혼신을 다한다. 그러나 자녀의 '키 키우기'의 한계와 자녀가 겪는 사회적 낙인과 어려움을 함께 감내하면서 쉽지 않지만 결국 자녀의 장애를 인정하게 되고 자녀의 자라날 수 없는 '키' 가 아닌 자녀가 '자라날 수 있는' 심리적 사회적 성장에 집중할 수 있는 대안적 관점을 갖게 된다. 이러한 결과를 근거로 본 연구는 저신장장애인 당사자와 부모들을 지원할 수 있는 사회복지적 방안을 제안하였다.

• Clinical and Experimental Pediatrics
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• 제52권2호
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• pp.142-151
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• 2009

The incidence of small for gestational age (SGA) births is frequent, accounting for 2.3% to 8% of all live births. Several childhood and adult diseases are related to early postnatal growth and birth size, and 10% of children born SGA may have a short stature throughout postnatal life. Additionally, they may have abnormal growth hormone (GH)-insulin like growth factor axis, HPA axis, and gonadal function. Permanent changes are detrimental in an environment of nutritional abundance, and predispose SGA children to an array of diseases in adolescence and adulthood. Such changes may also cause premature pubarche, adrenarche, and precocious puberty. The varying results from clinical studies necessitate more prospective case control studies. Reproductive tract abnormalities and reproductive dysfunction are related to SGA births. GH treatment is required for SGA infants who do not experience catch-up growth.

• Clinical and Experimental Pediatrics
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• 제49권7호
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• pp.703-709
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• 2006

Since the advent of growth hormone(GH), children with a wide variety of growth disorders have received GH treatment. In GH deficiency(GHD), Turner syndrome, chronic renal failure, children born small for gestational age, Prader-Willi syndrome, and idiopathic short stature, the therapeutic effects and safety profile of GH are reviewed. GH therapy has been clearly shown to improve height velocity and final adult height in a variety of pediatric conditions in which growth is compromised irrespective of GHD. Early initiation and individualization of GH treatment has the potential to normalize childhood growth. The supra-physiological doses of GH have been shown to increase height velocity during childhood and final height in non-GHD conditions. Adverse events during GH therapy are uncommon and often not drug related. However continued surveillance into adult life is crucial, especially in children receiving supra-physiological doses or whose underlying condition increases their risk of adverse effects.

• 구강회복응용과학지
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• 제30권2호
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• pp.170-175
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• 2014

성장장애는 두개안면부의 골격성장과 영구치의 조기상실에 영향을 미친다. 본 증례는 성장장애 환자에서 치아를 조기 상실한 부위에 무피판 술식으로 임플란트를 식립 시 컴퓨터 단층촬영(CT)을 통한 인접치아의 확인과 임플란트 가이드의 필요성을 살펴보았다. 특발성 저신장과 3급 정신지체의 20세의 여환에서 구치의 회복을 위해 임플란트 고정성 보철을 계획하였다. CT 영상에서 인접치가 심하게 협측으로 위치되어 있었고, 이를 반영하여 가이드를 제작후 임플란트를 식립하였다. 이상 위치된 인접치를 기준으로 임플란트를 식립할 경우 협측 골의 천공이 발생할 수 있다. 그러므로 성장장애 환자에서 치아 조기 상실부에 임플란트를 식립 시 CT를 통한 치아의 위치 확인과 가이드의 제작이 요구된다.

• 대한소아치과학회지
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• 제29권1호
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• pp.51-56
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• 2002

러셀-실버 증후군(Russell-Silver syndrome)은 출생시 저신장, 편측성 비대칭과 성기관 발육의 다양성 및 그 외 cafe-aulait 반점, 만지증 등의 특징과 태아기부터 발현되는 성장지연을 보이는 질환이다. 이 신드롬과 관련된 안면 특징은 작고 삼각형의 얼굴과 짧은 안면고경, 구각부가 아래로 쳐진 입모양(shark's mouth) 작은 하악골과 흔히 좌우 비대칭이 있는 것이다. 현재까지 보고되고 있는 러셀-실버 증후군의 주요한 구강내 소견은 높은 구개궁(high-arched palate), 맹출 지연, 왜소치와 총생이다. 현재까지 세계적으로 약 150 증례가 보고되고 있으나 치의학적으로는 극히 드물다. 본 증례는 출생전 성장지연, 저신장, 저체중 등 임상소견을 통해 러셀-실버 증후군으로 진단받았고 성장호르몬 치료를 받았고, 현재 치료 중이다. 이 두 증례를 통해 러셀-실버 증후군의 구강내 특징을 보고하고, 관련 문헌을 고찰해 보고자 한다.