결핵 PCR로 확진된 부신 결핵에 의한 Addison병

문제호 ( Je Ho Mun ),김훈수 ( Hoon Soo Kim ),김수한 ( Su Han Kim ),정도상 ( Do Sang Jung ),고현창 ( Hyun Chang Ko ),김문범 ( Moon Bum Kim ),권경술 ( Kyung Sool Kwon ) 대한피부과학회 2009 대한피부과학회지 Vol.47 No.9

Addison`s disease, or primary adrenal insufficiency, results from progressive destruction of the adrenal glands. The common causes of Addison`s disease are idiopathic autoimmune atrophy and tuberculosis of the adrenals glands. Adrenal insufficiency caused gradual adrenal destruction is characterized by an insidious onset of fatigability, weakness, anorexia, nausea, weight loss, and cutaneous manifestations, such as hyperpigmentation of the skin, especially in sun-exposed areas, palmar creases, frictional areas, recent scars, and oral mucosa, and longitudinal pigmented bands in the nails. After diagnosing Addison`s disease, determining the underlying adrenal abnormality is important for the appropriate treatment of the disease. To date, there have been no reports regarding the cause of Addison`s disease confirmed in the Korean dermatologic literature. We report here a case of Addison`s disease secondary to adrenal tuberculosis confirmed by a tuberculosis-polymerase chain reaction (TB-PCR). (Korean J Dermatol 2009;47(9):1048∼1053)

자가 면역성 갑상선염이 동반된 Addison’s disease 산모에서의 제왕절개술을 위한 마취 관리-증례 보고-

유동근,이상훈,이정현,이미현,정미화,최영룡,최은미 대한마취통증의학회 2013 Anesthesia and pain medicine Vol.8 No.4

Addison's disease is a rare chronic endocrine disorder which results from progressive destruction of the adrenal glands. It is kind of autoimmune polyendocrine syndrome type 2 with autoimmune thyroiditis, and can lead to fatal outcomes when complicated in pregnancy. We report on an anesthetic experience with 38-year-old primigravida with Addison's disease and hypothyroidism who was followed up from the early stage of pregnancy to delivery by a multidisciplinary medical board. Great attention was given to steroid replacement therapy as well as fetal surveillance in order to lower the risk of maternal and fetal mortality. This report describes our experience of the spinal anesthetic management for Cesarean section of a patient with Addison's disease and hypothyroidism.

의식 소실 및 심정지로 발현된 Addison병 1례

엄재호,이동호,조명찬,윤세진,궁성수,김승택,이복희 충북대학교 의과대학 충북대학교 의학연구소 1992 忠北醫大學術誌 Vol.2 No.1

Addison병은 점진적인 부신피질의 파괴에 의하여 나타나는 만성 부신피질 기능저하증으로 주로 자기면역기전에 의할 것으로 추정되는 특발성 부신피질 기능저하증과 결핵에 의해 발병되며, 빈도는 적으나 출혈성 경색, 전이암, 유육종증, 진균증 등에 의해서도 발병될 수 있다. 주요 증상은 전신쇠약, 색소과다침착, 체중감소, 오심, 구토 등이며 혈청 전해질 검사에서 저나트륨 혈증 및 고칼륨혈증이 관찰될 수 있다. 저자들은 심정지로 내원한 32세 남자환자에서 전신쇠약, 색소과다침착, 백반 등의 소견과 일반적인 치료에 반응하지 않는 고칼륨혈증에서 Addison병이 의심되어, 호르몬 검사를 한 결과, 혈장 부신피질 자극 호르몬 179.5 pg/mL, 코티솔 1.48 ㎍/mL로 Addison병을 확진하였으며, hydrocortisone 투여후에 고칼륨혈증과 전신쇠약이 호전된 예를 경험하였기에 보고하는 바이다. Addison's disease is a clinical syndrome which results from chronic, progressive destruction of adrenal cortex. The common causes are idiopathic atrophy and adrenal tuberculosis, and hemorrhagic infarct, metastatic carcinoma, sarcoidosis, chronic granulomatous diseas such as histoplasmosis, coccidioidomycosis and cryptococcosis can also cause primary adrenocortical insufficiency. Addison's disease is characterized by fatigability, weakness, hyperpigmentation, weight loss, nausea, vomiting and electrolyte imbalances including hyponatremia and hyperkalemia. We diagnosed Addison's disease in 32-year old male who showed severe weakness, hyperpigmentation and refractory hyperkalemia. The plasma ACTH and cortisol levels were 179.5 pg/mL and 1.48 ㎍/mL, respectively which showed primary adrenocortical insufficiency. The symptoms of hyperkalemia and weakness were markedly improved by intrevenous administration of hydrocortisone.

Addison씨병 1예 보고 : 부산피질 결핵으로 인한 due to Adrenal Tuberculosis

김종호,김관엽,김홍주,이혁상 인제대학교 1985 仁濟醫學 Vol.6 No.1

Addison's disease is a rare disorder resulting from progressive adrenocortical destruction regardless of the nature of underlying process. We experineced a case of Addison's disease due to adrenal tuberculosis. Patient was a 53-year-old-male, whose main complaints were dark pigmentation of the skin and mucous membrane, general weariness and weight loss. By use of rapid ACTH stimultion test and tissue biopsy, We confirmed the diagnosis of addison's disease. The ACTH stimulation test fails to rise the plasma cortison level and the histopathologic of the adrenal gand shows tuberculous granulomas. This patient recorved with use of cortisone acetate and anti-tuberculous medication.

부신피질 결핵에 의한 Addison씨 병 환자에서 리팜핀 투여 후 혈액투석이 필요하였던 심한 고칼륨혈증

김현경 ( Hyun Gyung Kim ),윤유선 ( Yoo Sun Yoon ),김희언 ( Hee Hun Kim ),김영수 ( Young Soo Kim ),김병수 ( Byung Soo Kim ),윤선애 ( Sun Ae Yoon ),김영옥 ( Young Ok Kim ) 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.1

Rifampicin, a potent inducer of hepatic microsomal enzymes, increases metabolism of steroid hormone. Therefore, concurrent treatment of rifampicin and steroid may lead to decreased bioavailability and increased requirement of steroid hormone. Here we report a case of rifampicin-induced severe hyperkalemia requiring hemodialysis in a patient with Addison`s disease. A 52-year-old woman was admitted due to general weakness. She had been diagnosed with adrenal tuberculosis and consequent Addison`s disease and treated with rifampicin and physiologic dose of steroid. Blood chemistry showed 9.1 mEq/L of potassium and emergent hemodialysis was performed. With increment of dosage of steroid hormone and maintenance of the same dosage of rifampicin, potassium level was normalized.

아디손병 ( Addison ` s Disease )

조양훈 ( Yang Hoon Cho ),이무형 ( Mu Hyoung Lee ),허충림 ( Choong Rim Haw ),유지홍 ( Ji Hong Yoo ) 대한피부과학회 1995 대한피부과학회지 Vol.33 No.6

Addison's disease is a rare disorder resulted from a chronic deficiency of the adreanl cortical hormone. The clinical manifestations are general weakness, weight loss, hyperpigmentation, hypovolemia with hyponatremia and hyperkalemia. We report a case of Addison's disease in a 60-year-old woman who has experienced slowly progressive weakness, weight loss and generalized cutaneous pigmentation, especially sun exposed area, extensor surface and nail bed for the last, 2 years. On a hormonal assay of the adrenal glands, basal plasma cortisol level was decreased and basal plasma ACTH level was markedly elevated. A chest X-ray showed streaky tuberculous infiltration in left, upper lobe field and adrenal CT scan showed calcific densities of both adrenal glands with nodular enlargement of right adrenal gland. There was a clinical improvement with steroid replacement therapy and anti-tuber- culosis chemotherapy. A nearly normal appearance was obtained after 5 months' treatment. (Kor J Dermatol 1995;33(6) : 1148-1153)

결핵에 의한 Addison씨 질환 1예 보고

송영진,최재윤,궁성수,송형근,배윤관,김정욱 충북대학교 의과대학 충북대학교 의학연구소 1994 忠北醫大學術誌 Vol.4 No.1

부신 기능 부전은 가장 심각한 내분비 질환 중의 하나이나, 다행이 그 발생 빈도가 중례 보고할 정도로 희귀하다. 과거에는 가장 흔한 원인이 결핵이었으나 최근 들어 화학요법의 발달로 결핵이 감소됨에 따라 서구에서는 자가면역 질환에 의한 것이 가장 많은 원인으로 보고되고 있다[1]. 그러나 국내의 경우는 아직도 전 인구의 1.8%가 결핵에 감염되어 있으며, 따라서 우리 나라의 경우 아직도 부신피질 기능 저하중의 가장 많은 원인으로 고려되고 있는 것은 결핵이다[2]. 본원에서는 양측 부신 절제로 확인된 결핵성 부신 피질 기능 저하중 1예를 치험하였기에 보고하고자 한다. In 1855 Thomas Addison reported 11 patients with primary adrenal insufficiency, including 5 patients with tuberculous destruction of the adrenal glands. After then, tuberculosis was known as one of the major cause of spontaneous Addison's disease in tuberculosis endemic area. But in western countries, more than 80% of spontaneous causes of adrenal insufficiency are secondary to autoimmune destruction of adrenal glands. In Korea, 70% of spontaneous Addison's disease was suggested due to tuberculosis. But pathologic confirmation was very rare. Most cases of adrenal tuberculosis have extraadrenal tuberculosis and/or adrenal calcification. Authors experienced one case of adrenal tuberculosis without extraadrenal tuberculosis and adrenal calcification confirmed by adrenal pathology.

고칼륨혈증 주기성 마비로 나타난 무통성 갑상선염 동반 애디슨병

고명주 ( Myeong Ju Koh ),서혜미 ( Hye Mi Seo ),김영욱 ( Young Uck Kim ),김성택 ( Seongteag Kim ),이상아 ( Sang A Lee ),유원상 ( Won Sang Yoo ),김소미 ( So Mi Kim ) 대한내과학회 2015 대한내과학회지 Vol.88 No.4

In cases of hyperkalemia with preserved renal function, the differential diagnoses that should be considered are drug-related disorders, primary tubular disease, and hormonal diseases including primary adrenal insufficiency. Addison`s disease represents a rare disorder characterized by primary adrenal failure, general weakness, poor appetite, nausea, dizziness, and hyperpigmentation. It may also cause fatal adrenal crisis, involving hypotension, loss of consciousness, hyperkalemia, or hyperkalemic periodic paralysis under stressful conditions. We describe herein the case of a 54-year-old Korean male who developed Addison`s disease, due to adrenal tuberculosis, in addition to painless thyroiditis, which led to hyperkalemic periodic paralysis.

색소 과다 침착만으로 조기 발견한 소아 부신백질이영양증 1례

박선형,홍용희,Park, Seon-Hyeong,Hong, Yong-Hui 대한유전성대사질환학회 2014 대한유전성대사질환학회지 Vol.14 No.2

X 연관 부신백질이영양증(adrenoleukodystrophy, X-ALD)는 성염색체 Xq28에 위치한 ABCD1 유전자의 돌연변이에 의해 발생하며, 매우 긴 사슬 지방산(saturated very long chain fatty acids, VLCFA) 운반의 장애로 뇌와 척수의 백질과 부신 피질을 포함한 모든 조직에 VLCFA가 쌓이게 되어 중추와 말초 신경 조직 내에 탈수초(demyelination)가 진행되고, 부신 피질의 기능 저하가 나타나게 된다. X-ALD의 임상적 증상은 매우 다양하나, 크게 3가지 표현형으로 나누어 대뇌 부신백질형성 장애(cerebral ALD), 부신 척수 신경병증(adrenomyeloneuropathy), 부신 피질 기능 저하 즉 애디슨병(Addison's disease only ALD)으로 나눌 수 있다2). 부신 피질 기능 저하 증세만을 보이는 Addison's disease only ALD의 경우 주로 2세에서 7.5세에 발생하며, 증세는 구토, 위약, 혼수, ACTH 분비 증가에 따른 색소 과다 침착(hyperpigmentation)으로 나타나고 발생 당시에는 신경학적 증세가 동반되지 않는다. 저자들은 구역, 구토, 탈수, 저혈당 및 저혈압 등의 증상 없이 색소 과다 침착이 부신 피질 기능 저하의 유일한 증세였던 환아를 혈장 VLCFA 검사를 통해 X-ALD로 진단하였고, ABCD1 유전자 분석 검사에서 c.1992-2A>G 변이를 확인하였다. 국내에서 색소 과다 침착만을 보이는 환아가 X-ALD로 진단받은 보고는 찾을 수 없었으며, 국내에서 보고되지 않은 돌연변이로 이를 보고하는 바이다. X-ALD 환아의 장기적인 예후 예측과 자세한 상담을 위해서 유전자형과 표현형의 상관관계에 대한 더 많은 연구가 필요하며, 이를 위해 유전자형에 따른 증상과 예후에 대한 자료가 공유되어야 할 것이다. The X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease by defects of ABCD1 gene on chromosome Xq28 leading to accumulation of saturated very long chain fatty acids (VLCFA), progressive demyelination and adrenal insufficiency. A 4-year-old boy was visited hospital with the chief compliant of hyperpigmentation beginning at 2-years old. Serum adrenocorticotropic hormone (ACTH) and cortisol concentration were compatible with adrenal insufficiency. The elevated plasmatic concentration of VLCFA and genotype analysis with sequencing of ABCD1 gene established the diagnosis of X-ALD. Brain MRI showed no abnormal high signal intensity on the white matter. Steroid replacement was started with good response. He initiated Lorenzo's oil with restriction of VLCFA by reducing the intake of fatty foods. The author highlight the importance of suspecting of X-ALD in the etiology of primary adrenal insufficiency as the first sign of the disease.

애디슨병 환자에게 리팜핀 투여 후 발생한 급성 부신피질기능 저하증 1예

강종식,고광범,이재준,김민수,전성진,최광현,김선목,이우제 영남대학교 의과대학 2012 Yeungnam University Journal of Medicine Vol.29 No.1

Adrenal insufficiency during the treatment of pulmonary tuberculosis is a troublesome condition and can at times be lifethreatening if untreated. Rifampin is one of the most widely prescribed anti-tuberculosis agents. Further- more, rifampin has been known to be capable of affecting the metabolism of various medications, including glucocorticoids. In this paper, a case of recurrent adrenal insufficiency induced by rifampin during the treat- ment of pulmonary tuberculosis is reported. The patient was a 63-year-old man who was diagnosed with Addison’s disease 17 years earlier and had been undergoing glucocorticoid replacement therapy. Five months before, the patient manifested pulmonary tuberculosis and was immediately given anti-tuberculosis medication that included rifampin. After one week of medication, general weakness and hyponatremia occurred. Despite the increased dose of the glucocorticoid medication, the adrenal insufficiency recurred many times. Since the substitution of levofloxacin for rifampin, the episodes of adrenal insufficiency have not recurred so far.