• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.121-124
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• 2016

Turner syndrome (TS) is a genetic disorder in phenotypic females that has characteristic physical features and presents as partial or complete absence of the second sex chromosome. Growth hormone deficiency (GHD) is a condition caused by insufficient release of growth hormone from the pituitary gland. The concomitant occurrence of TS and GHD is rare and has not yet been reported in Korea. Here we report 2 cases of TS and GHD. In case 1, GHD was initially diagnosed. Karyotyping was performed because of the presence of the typical phenotype and poor response to growth hormone therapy, which revealed 45,X/45,X+mar. The patient showed increased growth velocity after the growth hormone dose was increased. In case 2, a growth hormone provocation test and chromosomal analysis were performed simultaneously because of decreased growth velocity and the typical TS phenotype, which showed GHD and a mosaic karyotype of 45,X/46,XX. The patient showed spontaneous pubertal development. In female patients with short stature, it is important to perform a throughout physical examination and test for hormonal and chromosomal abnormalities because diagnostic accuracy is important for treatment and prognosis.

• Reproductive and Developmental Biology
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• 제35권4호
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• pp.519-525
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• 2011

Growth hormone (GH) is obligatory for growth and development. But, there is controversy on the GH effect about reproductive processes of sexual differentiation, pubertal maturation, gonadal steroidogenesis, gametogenesis and ovulation. This study was conducted to investigate the effect of GH on estrus, ovulation and embryo implantation. The results obtained were as follows. GH stimulated to increase estrus rate (p<0.05), pregnancy rate (p<0.05), and total fetus number in mice treated for superovulation. Also, the correlation between GH and steroids, E2 and P4, at peri-estrus stage/ peri-ovulation stage/ peri-implantation stage of the superovulation-induced mice was examined. Consequently, GH co-injected with PMSG especially increased P4 level (p<0.05) at peri-estrus stage of superovulationinduced mice. In conclusion, GH co-treatment in superovulation system boosted the rate of estrus, pregnancy and total fetus by increasing progesterone level at peri-estrus stage of superovulation-induced mice.

• Journal of Animal Science and Technology
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• 제60권6호
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• pp.11.1-11.7
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• 2018

After pubertal, cohort of small antral follicles enters to gonadotrophin-sensitive development, called recruited follicles. This study was aimed to identify candidate genes in follicular cyclic recruitment via analysis of protein-protein interaction (PPI) network. Differentially expressed genes (DEGs) in ovine granulosa cells of small antral follicles between follicular and luteal phases were accumulated among gene/protein symbols of the Ensembl annotation. Following directed graphs, PTPN6 and FYN have the highest indegree and outdegree, respectively. Since, these hubs being up-regulated in ovine granulosa cells of small antral follicles during the follicular phase, it represents an accumulation of blood immune cells in follicular phase in comparison with luteal phase. By contrast, the up-regulated hubs in the luteal phase including CDK1, INSRR and TOP2A which stimulated DNA replication and proliferation of granulosa cells, they known as candidate genes of the cyclic recruitment.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.286-293
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• 2010

Peak bone mass is established predominately during childhood and adolescence. It is an important determinant of future resistance to osteoporosis and fractures to gain bone mass during growth. The issue of low bone density in children and adolescents has recently attracted much attention and the use of pediatric dual-energy X-ray absorptiometry (DXA) is increasing. The process of interpretation of pediatric DXA results is different from that of adults because normal bone mineral density (BMD) of children varies by age, body size, pubertal stage, skeletal maturation, sex, and ethnicity. Thus, an appropriate normal BMD Z-score reference value with Z-score should be used to detect and manage low BMD. Z-scores below -2.0 are generally considered a low BMD to pediatrician even though diagnoses of osteoporosis in children and adolescents are usually only made in the presence of at least one fragility fracture. This article will review the basic knowledge and practical guidelines on pediatric DXA based on the International Society for Clinical Densitometry (ISCD) Pediatric Official Positions. Also discussed are the characteristics of normal Korean children and adolescents with respect to BMD development. The objective of this review is to help pediatricians to understand when DXA will be useful and how to interpret pediatric DXA reports in the clinical practice for management of children with the potential to develop osteoporosis in adulthood.

• 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.

• Clinical and Experimental Pediatrics
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• 제58권8호
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• pp.294-300
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• 2015

Purpose: It is difficult to differentiate between central precocious puberty (CPP) and premature thelarche (PT) in girls. The aim of this study was to investigate the diagnostic usefulness of pelvic ultrasonography to distinguish between CPP and PT in girls with early breast development. Methods: This study included girls with early breast development who visited the clinic between January 2012 and December 2013. Clinical, laboratory, and pelvic ultrasonographic data were evaluated. CPP and PT were confirmed using the gonadotropin-releasing hormone stimulation test. Results: A total of 248 girls aged 7-8 years were included, among whom 186 (75.0%) had CPP and 62 (25.0%) had PT. The uterine length, transverse diameter, fundus, volume, and cross-sectional area were significantly larger in the CPP group (uterine length, $2.45{\pm}0.50cm$ vs. $2.63{\pm}0.49cm$, P=0.015; uterine volume, $0.95{\pm}0.62cm^3$ vs. $1.35{\pm}0.76cm^3$, P<0.001). However, there were no differences in the fundus/cervix ratio and ovarian measurements. In receiver operating characteristic analysis, a uterine volume of at least $1.07cm^3$ was the most predictive parameter for CPP with an area under the curve of 0.670 (95% confidence interval, 0.593-0.747). Conclusion: Uterine measurements by pelvic ultrasonography in girls with early pubertal development were significantly larger in the CPP group. However, the diagnostic value of ultrasonographic parameters was not high because of a considerable overlap of values between the two groups. Therefore, pelvic ultrasonography in combination with clinical and laboratory tests may be useful to distinguish between CPP and PT in girls.

• Journal of Animal Science and Technology
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• 제56권5호
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• pp.17.1-17.4
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• 2014

Puberty is a stage of sexual development determined by the interaction of many loci and environmental factors. Identification of genes contributing to genetic variation in this character can assist with selection for early pubertal bulls, improving genetic progress in livestock breeding. Thyroid hormones play an important role in sexual development and spermatogenic function. The objective of this study was to evaluate the association between single nucleotide polymorphisms (SNPs) located in thyroglobulin(TG) gene with age of puberty in Angus bulls. Four SNPs were genotyped in 273 animals using SEQUENOM technology and the association between markers and puberty age was analyzed. Results showed a significant association (P < 0.05) between these markers and puberty age estimated at a sperm concentration of 50 million and a progressive motility of 10%. This is the first report of an association of TG polymorphisms with age of puberty in bulls, and results suggest the importance of thyroidal regulation in bovine sexual development and arrival to puberty.

• 대한치과교정학회지
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• 제28권4호
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• pp.581-589
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• 1998

부정교합 환자에서 사춘기 성장 가속화시기의 치료는 악안면 골격 부조화의 교정과 안모의 개선에 상당한 영향을 미치므로 사춘기의 성장을 예측하고 성장 잠재력을 평가하는 것은 매우 중요하다. 따라서 교정학에 있어서 개개인의 성장 가속화시기와 연관해서 신체의 성장 발육상태를 평가하는 것은 매우 중요하며 교정치료시 반드시 고려되어야 한다. 이 에 본 연구에서는 부정교합 분류에 따라 초경시기에 차이가 있는지와 초경시 골성숙도를 조사하기 위해, 1급 부정교합자 64명, II급 부정교합자 51명, III급 부정교합자 38명의 초경 전후 3개월 이내의 수완부골 방사선 사진을 이용하여 골성숙도를 평가한 결과 다음과 같은 결론을 얻었다. 1. 초경시 평균 연령은 $12.50{\pm}1.01$세 였다. 2. 각 군별 초경연령은 I급 부정교합군이 $12.36{\pm}1.04$세, II급 부정교합군이 $12.81{\pm}1.03$세, III급 부정교합군이 $12.32{\pm}0.82$세로서, II급 부정교합군이 I급 부정교합군 과 III급 부정교합군에 비해 늦은 초경연령을 보였다. 3. 초경시 수완부 골성숙도는 부정교합에 따른 차이가 없었다. 4. 초경시 수완부 골성숙도는 SMI 7이 $45.10\%$, SMI 8이 $27.45\%$, SMI 9가 $10.46\%$, SMI 6이 $7.84\%$, SMI 10이 $7.84\%$, SMI 5가 $1.31\%$ 였다. 5. 초경연령과 수완부 골성숙도는 통계적으로 유의한 상관관계를 보였다(p<0.05, r=0.25430).

• Childhood Kidney Diseases
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• 제13권1호
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• pp.1-13
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• 2009

소아청소년기의 당뇨병은 대부분 제1형 당뇨병이나 최근 우리나라를 포함한 서구 사회에서는 제2형 당뇨병의 빈도가 증가하고 있다. 임상소견 상 제1형 당뇨병은 여러 위험인자에 의하여 비교적 전형적인 단계를 거치면서 미세알부민뇨와 당뇨병성 신병증으로 진행하면서 만성 신질환으로 발전하게 되며, 제2형 당뇨병은 비전형적 임상경과를 거치나 신병증 진행율이 높아서, 실제로 당뇨병성 신병증은 전세계 신장대체요법이 필요한 말기 신질환의 가장 많은 원인이며 국내에서도 꾸준히 원인 질환으로서 증가 중이다. 당뇨병이 사춘기 전에 발생하는 경우보다 사춘기나 그 이후에 발생하는 경우에 혈관합병증의 발생이 증가하므로, 사춘기가 위험인자로 작용하며, 이것은 유병기간과 함께 사춘기 전에 소아 당뇨병성 신병증이 발생하는 경우는 매우 드문 이유이다. 제1형과 제2형 당뇨병에서 신병증은 비슷하게 15-25%에서 발병하며, 당뇨병성 신병증과 만성 신질환으로 진행하는 과정 중에 가장 중요한 표식자인 미세알부민뇨는 위험인자이고 병리학적 소견과 관련이 있다.

• Nutrition Research and Practice
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• 제7권5호
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• pp.385-392
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• 2013

Although several studies have assessed the influence of the glycemic index on body weight and blood pressure among adults, limited evidence exists for the pediatric age population. In the current study, we compared the effects of low glycemic index (LGI) diet to the healthy nutritional recommendation (HNR)-based diet on obesity and blood pressure among adolescent girls in pubertal ages. This 10-week parallel randomized clinical trial comprised of 50 overweight or obese and sexually mature girls less than 18 years of age years, who were randomly assigned to LGI or HNR-based diet. Macronutrient distribution was equivalently prescribed in both groups. Blood pressure, weight and waist circumference were measured at baseline and after intervention. Of the 50 participants, 41 subjects (include 82%) completed the study. The GI of the diet in the LGI group was $42.67{\pm}0.067$. A within-group analysis illustrated that in comparison to the baseline values, the body weight and body mass index (not waist circumference and blood pressure) decreased significantly after the intervention in both groups (P = 0.0001). The percent changes of the body weight status, waist circumference and blood pressure were compared between the two groups and the findings did not show any difference between the LGI diet consumers and those in the HNR group. In comparison to the HNR, LGI diet could not change the weight and blood pressure following a 10-week intervention. Further longitudinal studies with a long-term follow up should be conducted in this regard.