• Journal of Veterinary Clinics
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• v.31 no.5
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• pp.389-393
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• 2014

Medical records of 48 pet ferrets that underwent adrenalectomy were retrospectively reviewed to investigate the frequency and clinical outcomes of adrenal diseases in pet ferrets. These diseases were most commonly diagnosed in ferrets aged 3 to 5 years and in neutered females (58.3%). Adrenal disease occurred most frequently in the left adrenal gland (72.9%), followed by involvement of both adrenal glands (16.7%) and the right adrenal gland (10.4%). The mean sizes (length * thickness) of the adrenal glands as determined by ultrasonography were 8.96 * 5.08 mm and 12.91 * 8.26 mm for the left and right adrenal glands, respectively. In the ferrets with adrenal disease, alopecia (82.2%) was the main presenting clinical signs in both sexes, and vulvar swelling was seen in 32.1% of the females with adrenal disease. The common incidental findings included renal cysts (29.2%) and splenomegaly (25.0%). Histological findings showed pheochromocytoma, adenoma, and hyperplasia in 44.7%, 14.9%, and 12.8% of cases, respectively. The survival rates at 1- and 2- years after surgery were 87.5% and 74.0%, respectively. Alopecia and vulvar swelling improved within an average of 3.4 months and 12 days after surgery.

• The Korean Journal of Nuclear Medicine
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• v.31 no.1
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• pp.108-115
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• 1997

$^{131}I-6{\beta}$-iodomethyl-19-norcholesterol(NP-59) has an advantage to assess adrenal dysfunction caused by adrenal cortical disorders. The aim of this study is to evaluate the clinical usefulness of NP-59 scintigraphy in each adrenal disease. Ten patients who did eleven NP-59 adrenal scintigraphies at Dong-A University Hospital from March 1990 to December 1996 were selected as the subject. Among the subject there were 5 cases of Cushing's syndrome, 2 cases of incidentaloma, 1 case of metastatic adrenal tumor, liver cirrhosis with hirsutism and hypertension respectively. Among 5 cases of Cushing's syndrome, there were 2 cases of Cushing's disease, 2 cases of adrenal adenoma and 1 case of adrenal carcinoma. There are no disagreement between clinical diagnosis and scan finding in Cushing's syndrome. In 2 incidentaloma cases, even though one is interpretated as a functioning tumor, both of 2 cases could avoid unnecessary biopsy according to scintigraphy result. One case of hirsutism, clinically adrenal originated, revealed the normal scintigraphic finding after dexamethasone suppression scan. It could suggest that the etiology of hirsutism was extra-adrenal origin. One case of hypertension took the study to exclude the possibility of primary aldosteronism. Normal suppression scan finding revealed that primary aldosteronism did not exist in this case. In conclusion, NP-59 scintigraphy was very useful in diagnosis and differential diagnosis of Cushing's syndrome and it could avoid unnecessary biopsy in the incidental adrenal tumor.

• The Korean Journal of Medicine
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• v.93 no.6
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• pp.532-537
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• 2018

• Tuberculosis and Respiratory Diseases
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• v.67 no.1
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• pp.47-51
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• 2009

Loss of appetite is an important factor in the quality of life for advanced cancer patients. Megestrol acetate is used to stimulate appetite, but it can cause suppression of the pituitary adrenal axis due to the affinity of the glucocorticoid receptor. Adrenal insufficiency is a life threatening disorder if left, untreated, but the initial clinical symptoms of the patients are vague. Awareness of the glucocorticoid-like activity of megestrol acetate and its side effects are important for the diagnosis of adrenal insufficiency. We present a case of secondary adrenal insufficiency associated with megestrol acetate in a patient with lung cancer.

• 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.

• Journal of the Korean Society of Radiology
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• v.81 no.2
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• pp.459-464
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• 2020

Leiomyosarcoma is a malignant tumor that typically originates from either the uterus or the retroperitoneum. Furthermore, primary adrenal leiomyosarcoma is an extremely rare condition. Owing to its radiological non-specificity, differentiating leiomyosarcoma from other tumor types in the adrenal gland is difficult. We report the imaging findings of a primary adrenal leiomyosarcoma in a patient who presented with left upper quadrant abdominal pain, which increased by more than 1 cm in diameter in two years. Primary adrenal leiomyosarcoma was diagnosed considering the subsequent surgical and histopathologic findings.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.332-336
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• 2006

Toxic epidermal necrolysis (TEN) is a rare, acute and life-threatening cutaneous drug reaction. TEN is characterized by the sudden onset of extensive necrosis in the epidermis and frequent mucous membrane involvement. The pathogenesis has not yet been elucidated. In addition, no particular treatment for TEN has been established. We report a case of TEN in a 14-year-old-boy, which might have been caused by steroids with enalapril treatment for membranous nephropathy. He recovered after intravenous immunoglobulin therapy.

• Journal of Oral Medicine and Pain
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• v.25 no.3
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• pp.279-289
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• 2000

현대에는 날로 복잡해지는 생활양식의 변화에 따라 다양한 스트레스에 노출되고 그로 인한 면역계, 신경계 또는 각종 장기의 기능이상이 점차로 증가되고 있다. 최근 치과에 내원하는 환자들 중 구강건조증을 호소하는 환자들의 다수는 스트레스에 노출되어 있고, 임상적으로도 방사선 타액선 기능 검사에서 타액선, 특히 악하선의 기능이 현저하게 저하되어 있는 소견을 관찰할 수 있 다. 더욱이 이는 타액선 조영술에서 이미 보고된 바 있는 면역성 질환인 $Sj\ddot{o}gren$ 증후군과는 다른 양상으로 관찰되고 있어, 이에 본인은 백서에 스트레스와 관련이 깊은 내분비적 환경 변화를 유도함으로써 이와같은 변화가 악하선 조직의 병리적 변화와 어떠한 연관성을 갖는 지를 관찰하고자 본 실험을 시행하였다. 생후 7주된 Sprague-Dawley계 웅성 백서(165-209g/bw) 40마리를 2 개의 실험군(부신 제거군 : ADX 군, 부신 제거 후 dexamethasone 투여군 : DEX 군)으로 나누어 실험하였다. ADX 군은 외과적으로 부신을 제거한 후 다른 조건을 부여하지 않았고, DEX 군은 외과적으로 부신을 제거한 후 매일 dexamethasone($1.5*10^{-46}mg/g$ I.V./day)을 투여하였으며, 이들을 각각 실험 후 즉시, 1일, 3일, 5일, 7일에 희생시켰다. 그 후에는 즉시 악하선을 적출하여 면역조직화학법으로 Clusterin의 발현 정도 및 부위를 관찰하였다. 그 결과는 다음과 같았다. 1. 광학현미경 하에서, 양 군 모두 유의할만한 조직학적 변화는 관찰되지 않았다. 2. ADX 군에서는 실험기간 전반에 걸쳐 도관세포와 선포세포에서 clusterin이 발현되었다. 3. DEX 군에서는 소수의 선세포에서 국소적으로 clusterin이 관찰되었으나, 전반적으로 도관세포, 선포세포에서 공히 clusterin이 관찰되지 않았다. 부신을 제거한 군에서는 실험기간 전반에 걸쳐 clusterin이 발현되었는데, 이는 clusterin이 스트레스의 생리적 반응의 결과로서 세포보호작용을 한다는 사실에 기초하여 볼 때, 부신을 제거하였을 때도 스트레스를 받았을 때와 같은 영향이 백서의 악하선에 가해졌을 것으로 생각된다. 반면, 부신제거후 glucocorticoid agonist인 dexamethasone을 투여하였을 때, clusterin이 감소 내지 관찰되지 않았던 것은 부신제거에 의해 악하선에 가해졌던 영향을 dexamethasone이 길항하여 나타난 결과로 볼 수 있어, 스트레스에 의해 부신으로부터 분비되는 glucocorticoid가 타액선을 보호하는데 중요한 작용을 하는 것을 시사한다.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.54-59
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• 2014

Adrenoleukodystrophy is an X-linked genetic disease resulting from mutations in the ABCD1 gene. Cerebral adrenoleukodystrophy is one of the phenotypes of adrenoleukodystrophy and shows progressive demyelination of brain white matter and adrenal insufficiency. We report a nine year old male who presented with rapidly progressive cognitive and neurologic deterioration. He had abnormal findings in brain imaging and elevated very long chain fatty acid level in serum. Mutation analysis of ABCD1 revealed a c. 1252C>T (p.Arg418Trp) mutation which was previously known but not reported in Korea.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.33-43
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• 2005

Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.