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A Case of Recurred Paraganglioma of the Anterior Mediastinum A Case of Recurred Paraganglioma of the Anterior Mediastinum - A Case Report - (재발된 전종격동 부신경절종 치험 1례)

  • 김주현;김두상;성숙환;김영태
    • Journal of Chest Surgery
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    • v.31 no.2
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    • pp.198-202
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    • 1998
  • The recurrence of an anterior mediastinal mass was discovered incidentally on the chest film for an asymptomatic 72-year-old female. She underwent the mass resection at the same site in 1989 and was diagnosed as a paraganglioma of the anterior mediastinum. She had poorly controlled hypertension which was converted into normal blood pressure after the first operation. During the follow up, the catecholamine levels were within normal limits, and there were no evidence of recurrence of the tumor. However, 6.3 years later, the mass recurred at the same anterior mediastinum. The patient had no hypertension and catecholamine levels were still within normal limits. The recurring mass was successfully removed and had the same histological findings - recurring paraganglioma.

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Radiologic Findings of Local Effect of Right Adrenal Pheochromocytoma on the Adjacent Liver: A Case Report (우측 부신에서 발생한 갈색세포종이 인접 간에 미치는 국소적 영향에 관한 영상의학적 소견 : 증례 보고)

  • Rhim, Jung-Hyo;Cho, Jeong-Yeon;Kim, Seung-Hyup
    • Investigative Magnetic Resonance Imaging
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    • v.16 no.2
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    • pp.173-176
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    • 2012
  • We report the radiological findings of regional enhancement of the liver adjacent to the right adrenal pheochromocytoma. CT and MRI showed focal strong enhancement of adjacent liver tissue in the arterial phase. However during the delayed phase, the lesion showed iso-attenuation with normal hepatic parenchyma and not delineated. The lesion did not show abnormal signal intensity on neither T1 nor T2 weighted images and indistinguishable from normal parenchyma. The enhancing hepatic lesion was spontaneously regressed on postoperative follow up CT which was taken several months after the adrenalectomy.

Fine Needle Aspiration Cytology of Carotid Body Paraganglioma - A Case Report - (부신경절종의 세침 흡인 세포학적 소견 - 1례 보고 -)

  • Kim, Joon-Mee;Chu, Young-Chae
    • The Korean Journal of Cytopathology
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    • v.4 no.1
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    • pp.77-80
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    • 1993
  • Paraganglioma is a benign tumor arising in the paraganglion system scattered throughout the body, but its cytopathologic findings arenot well known. We experienced a case of paraganglioma of carotid body diagnosed by fine needle aspiration. The patient was a 30 year-old female who suffered from the left neck mass for 3 years. The mass was $3\times3cm$ in size without pulsation or bruit. Cytologically, the smear revealed aggregated and singly scattered tumor cells haying abundant pale cytoplasm and indistinct cell borders. Their nuclei were round to oval, but enlarged nuclei were occasionally observed. The nuclear membrane was smooth with fine clumping of chromatin. Differentiation from metastatic follicular carcinoma of the thyroid gland was difficult.

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Influence of Endogenous Catecholamines on Guanabenz- lnduced Inhibition of Micturition Reflex in Rats (Guanabenz 투여에 의한 흰쥐의 배뇨반사억제작용에 미치는 내인성 Catecholamines의 영향)

  • Park, Sang-Yeoul;Sohn, Uy-Dong;Kim, Choong-Young
    • The Korean Journal of Pharmacology
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    • v.25 no.1
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    • pp.67-74
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    • 1989
  • The effect of guanabenz on volume-induced micturition reflex contraction (VIMRC) in urethane-anethetized female rats was examined under adrenalectomy, chemical-sympathectomy, ganglionectomy, alpha-1, or alpha-2 blockade. Intracerbroventricular administration of guanalberz had little effect on VIMRC, but topical application suppressed amplitude and frequency of VIMRC. Guanabenz intravenous injection dose-dependently suppressed amplitude and frequency of VIMRC, with complete inhibition at dose of $100\;{\mu}g/kg$, but phenylephrine had no effect on VIMRC. Intravesicular peak pressure and amplitude of VIMRC were increased by 6-hydroxydopamine (6-OHDA) treatment when compared with control value, but yohimbine-, prazosin-hexamethonium-treatment and adrenalectomy did not show changes in VIMRC. Dose-response curve of guanabenz on amplitude and frequency of VIMRC shifted significantly to the right by treatment of yohimbine and 6-OHDA, and adrenalectomy. Median inhibitory dose $({\mu}g/kg)$ of guanabenz to amplitude of VIMRC showed 27.3 in control group, 381.6 in yohimbine, 294.1 in 6-OHDA and 54.1 in hexamethonium, and 38.8 in prazosin. Those of guanabenz to frequency of VIMRC showed 41.7 in control group, 571.1 in yohimbine, 410.8 in 6-OHDA, 141.4 in adrenalectomy, 59.6 in hexamethoinum and 31.4 in prazosin. These results suggest that guanabenz inhibits VIMRC through alpha-2 receptor stimulation rather than alpha-1 receptor stimulation and that catecholiamines released from sympathetic nerve ending and adrenal gland play a role in the inhibition.

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A Cost-Benefit Analysis of Neonatal Screening Tests for Maple Syrup Urine Disease, Homocystinuria, Galactosemia, and Congenital Adrenal Hyperplasia (한국에서의 단풍당뇨증, 호모시스틴뇨증, 갈락토스혈증, 선천성 부신과형성증에 대한 신생아 선별검사의 경제성 분석)

  • Park, Shin-Young;Kim, Dong-Il;Lee, Dong-Hwan
    • Journal of Genetic Medicine
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    • v.5 no.2
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    • pp.111-118
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    • 2008
  • Purpose: Neonatal screening tests are increasingly being used forearly diagnosis of inborn errors of metabolism (IEM) in the hope of avoiding the severe developmental delay, acute illness, and death that may result from these diseases. In this study, a cost-benefit analysis was performed on the neonatal screening of maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia in Korea. Materials and Methods: This study included 1,259,220 Korean newborns born between January 2005 to December 2007, who were screened for maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia. We calculated and compared the total costs in cases where these four screening tests were implemented, and those where they were not. Results: There were no benefits to screening for maple syrup urine disease or homocystinuria due to their low prevalence for these two tests, the costs exceeded the benefits at benefit:cost ratios of 0.5:1 and 0.6:1, respectively. In contrast, benefits far exceed costs at a ratio of 4.1:1 for galactosemia and 2.9:1 for congenital adrenal hyperplasia. The average benefit:cost ratio for all four tests was 2.0:1. Conclusion: Neonatal screening tests for maple syrup urine disease, homocystinuria, galactosemia, and congenital adrenal hyperplasia are financially viable.

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Influence of Hydrocortisone on Histamine-Evoked Catecholamine Secretion from the Isolated Rat Adrenal Medulla (Hydrocortisone이 적출 흰쥐 부신에서 Histamine의 카테콜아민 분비작용에 미치는 영향)

  • Lim, Dong-Yoon;Choi, Hyeong-Ho;Kim, Jong-In;Kang, Jeong-Won;Choi, Cheol-Hee;Oh, Hyeong-Gueun;You, Ho-Jin
    • The Korean Journal of Pharmacology
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    • v.32 no.1
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    • pp.83-91
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    • 1996
  • The present study was designed to determine the effect of hydrocortisone on CA secretion evoked by histamine from the isolated perfused rat adrenal glands. Histamine (150 ug) given into an adrenal vein produced significantly CA secretion from the rat adrenal medulla. This histamine-evoked CA secretion was enhanced markedly by the pretreatment with the natural glucocorticoid hydrocortisone (30 uM) or the synthetic glucocorticoid dexamethasone 30 (uM) for 20 min, respectively. Hydrocortisone-induced potentiation of CA secretion evoked by histamine was inhibited by preloading with heparin (3.56 U/ml), an $IP_3$ receptor antagonist while more enhanced by forskolin (0.2 uM), a potent stimulator of adenylate cyclase. From the experiment result taken together, it is thought that hydrocortisone (glucocorticoids) can enhance the releasing effect of CA evoked by histamine from the isolated perfused rat adrenal medulla, which seems to be associated to accumulation of inositol phosphate as well as cyclic AMP in the rat adrenomedullary chromaffin cells.

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Studies on Secretion of Catecholamines Evoked By DMPP and McN-A-343 in the Rat Adrenal Gland (흰쥐 적출 부신에서 DMPP 및 McN-A-343의 Catecholamine 분비작용에 관한 연구)

  • Lim, Dong-Yoon;Hwang, Doo-Hwan
    • The Korean Journal of Pharmacology
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    • v.27 no.1
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    • pp.53-67
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    • 1991
  • The characteristics and differences between DMPP and McN-A-343 on the secretory effect of catecholamines(CA) were studied in the isolated perfused rat adrenal glands. DMPP(100 uM) and McN-A-343(100 uM) perfused into an adrenal vein of the gland casued significant increases in CA secretion. On molar basis the secretory effect of McN-A-343 was about one fifth as potent as that of DMPP. Tachyphylaxis to releasing effects of CA evoked by DMPP and McN-A-343 was not observed by repeated perfusion of these agents. The DMPP-evoked CA secretion was significantly inhibited by pretreatment with chlorisondamine, desipramine and profusion of $Ca^{2+}-free$ Krebs solution containing EGTA, while it was not affected by pirenzepine, ouabain and physostigmine. However, pretreatment with atropine rather enhanced CA release by DMPP. The releasing effect of CA induced by McN-A-343 was markedly depressed by pretreatment with atropine, pirenzepine, chlorisondamine, physostigmine, and perfusion of $Ca^{2+}-free$ medium plus EGTA but was not influenced by desipramine, except for the case of ouabain which clearly potentiated CA release by McN-A-343. These experimental results suggest that both DMPP and McN-A-343 cause greatly secretion of CA from the isolated perfused rat adrenal glands by a calcium-dependent exocytotic mechanism. The secretory effect of DMPP is due to the stimulation of cholinergic nicotinic receptors and the secretion by McN-A-343 via activation of selecive $M_{1}-muscarinic$ receptors in the adrenal gland. It is also thought that the DMPP-evoked secretory effect is much greater than McN-A-343-induced effect.

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A Case of Childhood Cerebral Form Adrenoleukodystrophy with Novel Mutation in the ABCD1 Gene (새로운 ABCD1 유전자의 돌연변이를 가지는 소아 대뇌형 부신백질이영양증 1례)

  • Shin, Young-Lim
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.49-53
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    • 2012
  • X-linked adrenoleukodystrophy (ALD) is a rare inherited metabolic disease which results in impaired peroxisomal ${\beta}$-oxidation and the accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex, the myelin of the central nervous system, and the testes. X-linked ALD is caused by mutations in the ABCD1 gene encoding an ATP-binding cassette transporter superfamily located in the peroxisomal membrane. This disease is characterized by a variety of phenotypes. The classic childhood cerebral ALD is a rapidly progressive demyelinating condition affecting the cerebral white matter before the age of 10 years in boys. We report the case of a 8-year-old with childhood cerebral X-linked ALD who developed inattention, hyperactivity, motor incoordination and hemiparesis. We diagnosed ALD with elevated plasma very long chain fatty acid level and diffuse high signal intensity lesions in both parieto-occipital white matter and cerebellar white matter in brain MRI. We identified a novel c.983delT (p.Met329CysfsX7) mutation of the ABCD1 gene. There is no correlation between X-ALD phenotype and mutations in the ABCD1 gene. Further studies for searching additional non-genetic factor which determine the phenotypic variation will be needed.

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Influence of Histaminergic Receptor Activation on Catecholamine Secretion in The Perfused Rat Adrenal Gland (흰쥐 관류부신에서 Histamine 수용체 활성화가 Catecholamine 분비작용에 미치는 영향)

  • Lim, Dong-Yoon;Rho, Sang-Hyun
    • The Korean Journal of Pharmacology
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    • v.29 no.1
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    • pp.43-55
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    • 1993
  • The present study was conducted to examine the characteristics of histamine on catecholamine secretion in the isolated perfused rat adrenal gland and to clarify the mechanism of its secretory action. Histamine (37.5 to 150 ug) injected into an adrenal vein evoked a dose-dependent significant secretory response of catecholamines (CA) from the rat adrenal gland. However, upon the repeated injection of histamine (150 ug) at 120 min intervals, CA secretion was rapidly decreased after third injection of histamine. Tachyphylaxis to releasing effects of CA evoked by histamine was observed by the repeated administration. The histamine-induced CA secretion was markedly inhibited by the pretreatment with chlorisondamine, diphenhydramine, ranitidine, $Ca^{++}-free$ Krebs solution, nicardipine and TMB-8 while was not affected by pirenzepine. Moreover, the CA secretion evoked by ACh was considerably reduced by the prior perfusion of histamine $(6.8{\times}10^{-5} M)$ for 30 min. These experimental data suggest that histamine causes secretion of CA in a calcium dependent manner from the perfused rat adrenal gland and that its secretory effect is mediated through activation of both $H_1-$ and $H_2-histaminergic$ receptors located in adrenal medulla, which may be associated with stimulation of cholinergic nicotinic receptors.

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Effects of Adrenal Function on Reproductive Organ in Immature Rats (미성숙(未成熟)흰쥐의 부현기능(副賢機能)이 생식기관(生植器官)에 미치는 영향(影響))

  • Kwun, Hae Byeng
    • Current Research on Agriculture and Life Sciences
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    • v.3
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    • pp.181-189
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    • 1985
  • This study was conducted to determine the effect of the adrenal function on the reproductive organs in immature rats treated with PMS. Two hundred and ten female rats (Wistar-Imamichi albino rats) of 21 days old (body weight : $58.7{\pm}3.53g$) were disposed in the intact rat group (Int.-) and adrenalectomized rat group (Adx.-) and then each group was devided into 3 subgroups, such as control (-Cont.), PMS treated (-PMS) was administered subcutaneously with 25 IU PMS, and and PMS cortisol treated groups (-PMS+Corti.) with 25 IU PMS and $30.0{\mu}g$ cortisol on 5 th day (aged 26 days old) after adrenalectomy, while the control groups with physiological salt solution by the same way. The reprodutive organs were observed at 48, 54, 60, 66, 72, 78 and 84 hours after hormone treatments. The results obtained were as follows ; 1. The measurments of time average ovary weight in all treated groups were increased with the elapse of time after treatment, and the difference among the treatments was significant (p<0.01) in the all observation time. But the difference of those was not recognized in Int.-Cont. and Adx.-Cont. groups. In the multiple range test. ovary weight of adrenalectomized rat groups (Adx.-PMS and Adx.-PMS+Corti. groups) was significantly (p<0.05) lighter than those of intact rat groups (Int.-PMS and Int.-PMS+Corti. groups), and the effect of cortisol administration was not reconized. 2. The difference of uterus weight was significantly reconized (p<0.01) in all observation time. The weight in Int.-PMS and Int.-PMS+Corti. groups was heavier until 66 hours after treatment, but the values in the adrenalectomized Adx.-PMS and Adx.-PMS+Corti. groups were heavier after 72 hours. The multiple range test showed that the significant difference was not found between Int.-PMS and Int.-PMS+Corti. groups, and Adx.-PMS and Adx.-PMS+Corti. groups. 3. The adrenal weight was not significantly different among the compared groups.

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