• Title/Summary/Keyword: CAH

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Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia

  • Jin, Hye-Young;Choi, Jin-Ho;Kim, Gu-Hwan;Lee, Chung-Sik;Yoo, Han-Wook
    • Clinical and Experimental Pediatrics
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    • v.54 no.3
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    • pp.137-140
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    • 2011
  • Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were $10{\times}6$ cm and $7.5{\times}4.5$ cm, respectively. His height was 155.1 cm (standard deviation score (SDS), -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and $9{\alpha}$-fludrocortisone; thereafter, the reduced testis size has been maintained.

18-year Follow-up of Extended Newborn Screening for Metabolic and Endocrine Disorders (대사 및 내분비 질환에 대한 광범위 신생아 선별 검사의 18년 추적 관찰)

  • Song, Wung Joo;Lee, Sunho;Jeon, Young Mi;Kim, Sook Za;Jang, Mea Young
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.2
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    • pp.35-42
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    • 2018
  • Purpose: To follow up Korean patients with metabolic and endocrine disorders ascertained by Korea Genetics Research Center, and assess the long-term effectiveness of extended newborn screening program in Korea. Methods: From January 2000 to December 2017, tandem mass spectrometry and fluoroimmunoassay were employed in extended newborn screening (NBS). The NBS program obtained dried blood spots from 283,626 babies, 48 hours after birth, and screened for galactosemia, congenital hypothyroidism (CH), congenital adrenal hyperplasia (CAH), and 50 preventable inborn errors of amino acid, fatty acid, and organic acid metabolism. Results: 28 cases of amino acid disorders, 75 cases of organic acid disorders, 27 cases of fatty acid disorders, 51 cases of urea cycle disorders, 127 cases of CH, 14 cases of CAH, and 15 cases of galactosemia were ascertained through NBS and subsequent confirmatory laboratory tests. Patients with amino acid metabolic disorders, galactosemia, CH, or CAH were more likely to have a better long-term outcome if detected early. Early management of MSUD led to much better outcome in over 90%. Despite early intervention, 32% of other organic acidemia cases still resulted in developmental delay and neurological problems. Fatty acid disorders showed varied results; those with EMA and MCAD had a good outcome, but those with VLCAD had serious neurological problems and considerably higher mortality. 75% with UCD experienced serious neurological complications and higher mortality. Conclusion: The nation-wide NBS program must be accompanied by comprehensive long-term management and physician and family education of inborn errors of metabolism for a better outcome.

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Early Hydration Properties of BFS by a Change of pH (pH 변화에 따른 고로수쇄 BFS의 초기 수화 특성)

  • Kang, Hyun Ju;Lee, Woong Geol;Song, Myong Shin;Kang, Seung Min;Kim, Kyeng Nam
    • Journal of the Korean Ceramic Society
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    • v.49 no.5
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    • pp.442-447
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    • 2012
  • This study investigated on the early hydration and physical characteristics of BFS by pH variation. NaOH solution was used as a pH activator. In the range from pH 12 to pH 14, Experiment was compared the hydration propertied of OPC(Ordinary Portland Cement) and BFS(Blast Furnace BFS) and BFS containing 2 wt% of gypsum. It was found that CAH(Calcium Aluminate Hydrates) phases and CSH(Calcium Silicate Hydrates) phases were formed during the early hydration of BFS, and that CAH phases, CSH phases and ettringites were formed during the early hydration of BFS containing 2 wt% of gypsum. Furthermore, early hydration of BFS and BFS containing 2 wt% of gypsum were faster then OPC at pH 14, and the 1 day compressive strength of BFS increased by approximately 30% compared to OPC, and BFS containing 2 wt% of gypsum also increased by approximately 40% compared to OPC.

Purity assignment of 17α-hydroxyprogesterone by mass balance method to establish traceability in measurement

  • Lee, Hwa Shim;Park, Su Jin
    • Analytical Science and Technology
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    • v.32 no.6
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    • pp.225-232
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    • 2019
  • Traceability establishment in chemical measurements is a like a linkage established through an unbroken chain from the measured results to the international system (SI) of units. The primary process for traceability establishment is the purity assignment of a target material to be measured. In this study, we studied the purity assignment of 17α-hydroxyprogesterone (17-OHP). The presence of 17-OHP is indicative of congenital adrenal hyperplasia (CAH) and it builds up due to the deficiency of 21-hydroxylase and 11β-hydroxylase enzyme in the human blood. The purity assignment of 17-OHP was performed by the mass balance method, in which the impurities are categorized into four classes: total related structural impurities, water, residual organic solvents, and nonvolatiles/inorganics. The total related structural impurities were characterized by HPLC-UV; water content was determined by Karl-Fisher coulometer; and the total residual solvents and nonvolatiles/inorganics were determined by TGA. The purity of 17-OHP from a commercial manufacturer was calculated as 993.30 mg/g, and the expanded uncertainty was 0.58 mg/g. The proposed method was validated by uncertainty evaluation and comparing with the actual value of purity.

Evaluation on the Performance of Mortars Made with Calcium Aluminate Cement (칼슘알루미네이트 시멘트 모르타르의 성능 평가)

  • Lee, Seung-Tae
    • Journal of the Korea institute for structural maintenance and inspection
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    • v.19 no.6
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    • pp.80-87
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    • 2015
  • In this study, several properties of mortars made with calcium aluminate cement (CAC) such as hydrated products, strength characteristics, absorption, surface electric resistivity and chloride ions penetration resistance were experimentally investigated. The properties of CAC mortars were compared to those of ordinary portland cement (OPC) mortars. From the test results, it was found that the main hydrated products for CAC mortars were of $C_2AH_8$ and $CAH_{10}$, while CH, ettringite and calcite for OPC mortars. The surface electric resistivity and chloride ions penetration resistance of CAC mortars were significantly beneficial compared to those of OPC mortars. However, it should be noted that the absorption properties of CAC mortars were negatively examined. Thus, it needs to have more study for the improvement of surface absorption of CAC matrices. In addition, the combined mixture of CAC and OPC were ineffective to improve some performances of mortars.

On the Rapid Hardening Cement (II) (초속경시멘트 제조에 관한 연구(제2보 수화반응))

  • 한기성;최상흘;한상목;서일영
    • Journal of the Korean Ceramic Society
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    • v.12 no.4
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    • pp.3-8
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    • 1975
  • Hydration processes of the rapid hardening cement clinkers, which were synthesized from domestic alunite for major alumina source, limestone, kaolin and fluorite, were investigated by means of x-ray diffraction analysis, thermal analysis and microscopic observation etc. The clinkers were composed mainly of alite, calcium fluroaluminate (C11A7.CaF2) minerals. While the hydratio processes of the clinkers are altered by concentration of SO3 in the paste, calcium aluminate hydrates such as C4AH13, CAH10 and calcium monosulfate hydrate (C3A.CaSO4.12H2O) are formed at first and then some of them are transformed into ettringite(C3A.3CaSO4.32H2O) within 30~60 min. when the concentration of SO3 in the paste are enough. However the formed ettringite are changed slowly into calcium monosulfate hydrate as the concentration of SO3 become lowered, and the paste is hardened with these close-packed minerals. When the content of SO3 in clinker is so enough, calcium sulfoaluminate hydrates are found without any addition of anhydrite or hemi-hydrite.

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Diagnostic Evaluation of Enzyme Activity Related to Steroid Metabolism by Mass Spectrometry-Based Steroid Profiling

  • Choi, Man Ho;Chung, Bong Chul
    • Mass Spectrometry Letters
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    • v.5 no.2
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    • pp.35-41
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    • 2014
  • Gas chromatography-mass spectrometry (GC-MS) methods have been used extensively in clinical steroid analyses. Evaluating the metabolic ratios of precursors to products by accurate quantification of individual steroid levels in biological samples can reveal the activities of enzymes associated with steroid metabolism. This review article discusses the impact of GC-MS-based steroid profiling on our understanding of the biochemical role of steroids and their metabolic enzymes in hormone-dependent diseases, such as congenital adrenal hyperplasia (CAH), cortisol-mediated hypertension, apparent mineralocorticoid excess (AME), male-pattern baldness, and breast and thyroid cancers. Steroid profiling is a comprehensive analytical technique that can be applied whenever the highest specificity is required and may be a reasonable initial diagnostic approach.

Surface Modification Method of Stainless Steel using Electrochemical Etching (전기화학적 에칭을 이용한 스테인리스 스틸의 표면 개질)

  • Lee, Chan;Kim, Joonwon
    • Journal of the Korean Society for Precision Engineering
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    • v.31 no.4
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    • pp.353-358
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    • 2014
  • This paper reports a simple, yet effective 1-step surface modification method for stainless steel. Electrochemical etching in dilute Aqua Regia forms hierarchical micro and nanoscale structure on the surface. The surface becomes highly hydrophobic (${\sim}150^{\circ}$) as a result of the etching in terms of static contact angle (CA). However the liquid drops easily pinned on the surface because of high contact angle hysteresis (CAH), which is called a "petal effect": The petal effect occur because of gap between surface microstructures, despite of intrinsic hydrophobicity of the base material. The pore size and period of surface structure can be controlled by applied voltage during the etching. This method can be applied to wide variety of industrial demand for surface modification, while maintaining the advantageous anti-corrosion property of stainless steel.

Synthesis of $CA_2$ -based Clinker by Hydration-Burning Method-II. Hydraulic Properties of the Clinker (수화-소성법에 의한 $CA_2$ 클린커의 합성 -II. 클린커의 수화특성-)

  • 송태웅;한기성
    • Journal of the Korean Ceramic Society
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    • v.27 no.3
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    • pp.383-388
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    • 1990
  • Hydraulic properties of CA2-based clinker synthesized by Hydration-Burning Method was studied by calorimetry, analysis of suspension, thermogravimetry, scanning electron microscopy and X-ray diffractometry. Hydraulic properties of the clinker was so activated that heat of hydration of the clinker evolved faster than that of CA synthesized by conventional method. In suspension of W/C=33, dissolution of the clinker began at nearly same time as that of CA, but precipitation of AH3 and rise of [OH-] occurred fairly faster in the suspension of the clinker than that of CA. From the beginning of hydration, AH3 was produced and became a main phase of the hydrate with minor phase of CAH10 and C2AH8, but C3AH6 was not produced at ambient temperature.

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A Study on the Surface Activation and Quick-setting Characteristics of Blast Furnace Slag (Blast furnace slag의 표면 활성화 특성 및 quick-setting 특성에 관한 연구)

  • Lee, Woong-Geol;Song, Yung-Sin;Kang, Hyun-Ju;Choi, Hun;Song, Yong-Jin
    • Proceedings of the Korea Concrete Institute Conference
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    • 2010.05a
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    • pp.273-274
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    • 2010
  • This study investigated on the early hydration and physical characteristics of blast furnace slag through pH variation. The pH values applied to the experiments were, 12.0 and 13.0 which are the pH values of OPC, and type 3 of pH 14.0 which is a strong alkali condition. A paste and mortar method was used to test blast furnace slag and blast furnace slag containing 2wt% of gypsum. It was found that CAH and CSH phases were formed as key hydrates during the early hydration of blast furnace slag, and ettringites were produced extra during the early hydration of blast furnace slag containing 2wt% of gypsum.

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