Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A

문지미,서영진,김주형,박주리,이윤정,김희영,김신곤,최동섭,김윤정,최혜윤 대한내분비학회 2009 Endocrinology and metabolism Vol.24 No.4

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause. We describe here a case of ectopic ACTH syndrome with bilateral pheochromocytoma in a 29-year-old man with MEN 2A presenting with medullary thyroid carcinoma and hyperparathyroidism. MEN 2A was confirmed by the detection of the RET proto-oncogene mutation. The laboratory results were compatible with ectopic ACTH syndrome. Immunohistochemical studies of the pheochromocytoma tissue confirmed the etiology of the ACTH secretion. This is the first confirmed case of the ectopic ACTH syndrome with bilateral pheochromocytoma in a Korean patient with MEN 2A. Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause. We describe here a case of ectopic ACTH syndrome with bilateral pheochromocytoma in a 29-year-old man with MEN 2A presenting with medullary thyroid carcinoma and hyperparathyroidism. MEN 2A was confirmed by the detection of the RET proto-oncogene mutation. The laboratory results were compatible with ectopic ACTH syndrome. Immunohistochemical studies of the pheochromocytoma tissue confirmed the etiology of the ACTH secretion. This is the first confirmed case of the ectopic ACTH syndrome with bilateral pheochromocytoma in a Korean patient with MEN 2A.

자가면역 췌장염 (PPT)

문지미,김재선 대한췌담도학회 2010 대한췌담도학회지 Vol.15 No.3

노인 관상동맥질환자의 질병 관련 지식, 우울, 가족지지가 건강행위에 미치는 영향

문지미,신소영 한국노인간호학회 2019 노인간호학회지 Vol.21 No.3

Purpose: The purpose of this study was to identify the degrees and relationships of disease-related knowledge, depression, family support and health behaviors of older patients with coronary artery disease, and the factors influencing their health behaviors. Methods: The subjects included 139 older patients with coronary artery disease who visited the outpatient clinic at one general hospital located in metropolitan city B. Data collection was performed from December, 2018 to February, 2019, using a structured self-reported questionnaire. Descriptive statistics, t-test, one-way ANOVA, Pearson correlation coefficients, and multiple linear regression were used to analyze the data. Results: Disease-related knowledge (r=-.17, p=.050) and depression (r=-.32, p<.001) had significant negative correlations with health behaviors. While, a significant positive correlation was noted between family support and health behaviors (r=.67, p<.001). In the final multiple regression analysis, medication intake status (β=-.17, p=.009), depression (β=-.15, p=.017) and family support (β=.61, p<.001) significantly influenced health behaviors of the subjects. The explanatory power of the subjects’ medication intake status, disease-related knowledge, depression and family support on health behaviors was 49.0% (F=33.97, p<.001). Conclusion: Developing an effective program to improving medication status, depression, and family support is recommended for the enhancement of their health behaviors and ultimately for their recovery and well-being.

조기 하인두암의 치료로 내시경 점막하 박리술을 시행한

김승한,최원재,최정완,박자인,오주연,문지미,최세호,김기현,김우현,김태형,이범재,김재선,박영태,박종재 대한내과학회 2010 대한내과학회 추계학술대회 Vol.2010 No.-

신장 조직 검사에서 사구체 면역침착을 보인 베게너 육아종증 1예

서영진 ( Young Jin Seo ),김윤정 ( Yoon Jung Kim ),문지미 ( Ji Mi Moon ),최혜윤 ( Hae Yoon Choi ),우진현 ( Jin Hyun Woo ),최성재 ( Seong Jae Choi ),이영호 ( Young Ho Lee ),지종대 ( Jong Dae Ji ),정운용 ( Woon Yong Jung ),송관규 ( G 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.6

Rapidly progressive glomerulonephritis (RPGN) in Wegener`s granulomatosis patients typically has been characterized by pauci-immune glomerulonephritis (PIGN). In some patients, however, significant amount of glomerular immune deposits was detected and reported that they may have poor prognosis. A 30 year-old-female visited due to the skin rash of both lower extremities, arthralgia and nasal stiffness. She had sinusitis, lung opacity, and proteinuria. Serologic PR-3 ANCA was positive and histologic findings of nasal cavity and lung also showed necrotizing vasculitis and granuloma. Thus we could diagnose Wegener`s granulomatosis. However, gross hematuria developed and renal function worsened in spite of treatment with high dose prednisolone and oral cyclophosphamide. Therefore we performed a kidney biopsy. The kidney biopsy showed crescentic glomerulonephritis with Ig A deposition in the mesangium. We experienced a case of Wegener`s granulomatosis patient with significant IgA deposition in glomeruli. We report this case with brief review of the literature.

A Novel Technique for Retrieval of a Drug-Eluting Stent After Catheter Break and Stent Loss

Sunil P. Wani,나승운,박지영,Kanhaiya L Poddar,Lin Wang,Sureshkumar Ramasamy,문지미,류상렬,신승용,최운정,박창규,서홍석,오동주,최철웅,임홍의 대한심장학회 2010 Korean Circulation Journal Vol.40 No.8

Break of a stent delivery catheter and subsequent stent loss (SL) has been a rare event in the drug-eluting stent (DES) era. We here report a case of successful retrieval of a stent after a break if the delivery catheter and SL from a balloon catheter at a culprit lesion. We finally resolved this situation using a simple balloon technique for both the broken stent catheter inside of the guide catheter and the unexpanded stent in the culprit lesion. Thus balloons are an important weapon in our armamentarium in the cardiac catheterization laboratory for urgent retrieval of a lost stent. Their apt use definitely allowed our patient to avoid undergoing emergency cardiovascular thoracic surgery.

부갑상선 선종 제거술 22년 후에 재발한 이소성 부갑상선 선종 1예

김윤정,서지아,서영진,김혜숙,김신곤,최경묵,백세현,최동섭,김난희,문지미,최혜윤 대한내분비학회 2009 Endocrinology and metabolism Vol.24 No.4

Persistent or recurrent primary hyperparathyroidism after initial parathyroid surgery occurs at rates of 1.5~10%. A single missed parathyroid adenoma accounts for the majority of persistent hyperparathyroidism, whereas metachronous parathyroid adenoma is a rare cause of recurrent hypercalcemia. We report a case of a 39-year-old female who presented with recurrent pancreatitis. She had symptoms of hyperparathyroidism such as hypercalcemia, hypophosphatemia, hypercalciuria, nephrocalcinosis, and osteoporosis. She had a 2-cm firm neck mass under the right submandibular area. She was diagnosed with primary hyperparathyroidism 22 years ago. At that time, the right upper and lower parathyroid glands were removed after exploration of all parathyroid glands, and a right upper parathyroid adenoma was diagnosed. Now, she had a second surgery to remove the right submandibular mass with intraoperative PTH monitoring, which was diagnosed as a parathyroid adenoma in an ectopic supernumerary parathyroid gland. Because of hungry bone syndrome, she received calcium carbonate replacement therapy and has no evidence of recurrence. Here, we report a recurrent parathyroid adenoma in the undescended, supernumerary parathyroid gland after a long interval from the initial surgery. Persistent or recurrent primary hyperparathyroidism after initial parathyroid surgery occurs at rates of 1.5~10%. A single missed parathyroid adenoma accounts for the majority of persistent hyperparathyroidism, whereas metachronous parathyroid adenoma is a rare cause of recurrent hypercalcemia. We report a case of a 39-year-old female who presented with recurrent pancreatitis. She had symptoms of hyperparathyroidism such as hypercalcemia, hypophosphatemia, hypercalciuria, nephrocalcinosis, and osteoporosis. She had a 2-cm firm neck mass under the right submandibular area. She was diagnosed with primary hyperparathyroidism 22 years ago. At that time, the right upper and lower parathyroid glands were removed after exploration of all parathyroid glands, and a right upper parathyroid adenoma was diagnosed. Now, she had a second surgery to remove the right submandibular mass with intraoperative PTH monitoring, which was diagnosed as a parathyroid adenoma in an ectopic supernumerary parathyroid gland. Because of hungry bone syndrome, she received calcium carbonate replacement therapy and has no evidence of recurrence. Here, we report a recurrent parathyroid adenoma in the undescended, supernumerary parathyroid gland after a long interval from the initial surgery.