1.Familial Aortic Dissection without Marfan Syndrome: A Report of Four Cases in a Family
Kenji Iino ; Masahiro Seki ; Kengo Kawakami ; Naoki Sakakibara
Japanese Journal of Cardiovascular Surgery 2004;33(6):399-402
Aortic dissection with multiple familial members is rare. It is commonly associated with Marfan syndrome. Several authors have reported familial aortic dissection without Marfan syndrome. We encountered 4 cases of aortic dissection in a family. The aortic dissection occurred in the mother and all of her children. No case had clinical manifestations of Marfan syndrome or other connective tissue disease. Histopathological examination of the aorta did not show cystic medial necrosis in 2 operated cases. Many members in the family had systemic arterial hypertension. The presence of multiple incidence of aortic dissection in one family suggests underlying connective tissue disease, irrespective of the absence of typical features of Marfan syndrome. Therefore we propose that other close relatives as well as the members with aortic dissection should be followed-up in the same way used for families with typical connective tissue disease like Marfan syndrome.
2.A Case of Progressive Respiratory Failure Resulting from Chronic Bird Fancier's Disease after Postoperative Chemotherapy
Yoko SHINOHARA ; Yusuke KIYOKI ; Keita ANDO ; Tyuta OKAWA ; Takashi YAMANA ; Naoki NISHIYAMA ; Naoki KAWAKAMI ; Yoko WAKAI ; Takaaki YAMASHITA ; Kazuhito SAITO ; Takuya ONUKI ; Masaharu INAGAKI
Journal of the Japanese Association of Rural Medicine 2016;65(1):62-69
75-year-old man had the right lower lobe resected because of pulmonary adenocarcinoma (stage IIB) and received 4 courses of postoperative chemotherapy 4 years earlier. Thereafter, he continued to complain of cough, sputum, and progressive exertional breathlessness. The preoperative chest CT showed ground glass opacity (GGO) at the bottom of both lung fields, and over time the GGO changed to honeycombing with traction bronchiectasis. He was administered prednisolone, clarithromycin, and pirfenidone but with little improvement. He exhibited hypoxemia (PaO2 56 mmHg) and was admitted. An interview revealed that he had worked in the poultry farming business for 45 years having had contact with and breeding 3,000 game fowl at the time of hospitalization. We suspected bird-related hypersensitivity pneumonitis. Results of the reaction to pigeon dropping extracts (PDE) were high, with PDE IgG 0.697 and PDE IgA 0.445. He was diagnosed with chronic bird-related hypersensitivity pneumonitis. Although the chest CT appearance was difficult to distinguish from that of idiopathic interstitial pneumonia, the test for PDE and the interview were useful for reaching a diagnosis.
3.Abdominal Aortic Aneurysm Repair in Patients with Ischemic Heart Disease.
Hiroshi Urayama ; Kenji Kawakami ; Fuminori Kasashima ; Yuhshi Kawase ; Takeshi Harada ; Yasushi Matsumoto ; Hirofumi Takemura ; Naoki Sakakibara ; Michio Kawasuji ; Yoh Watanabe
Japanese Journal of Cardiovascular Surgery 1995;24(1):31-35
Ischemic heart disease (IHD) poses a major complicating factor for abdominal aortic aneurysm (AAA) repair. To identify patients with IHD, we evaluated patients scheduled to undergo AAA repair with dipyridamole-thallium scintigraphy (DTS) and coronary angiography (CAG). If indicated, coronary revascularization was performed. Finally, an assessment of the effectiveness of these preventive measures was made. One hundred and ten patients scheduled to undergo AAA repair were identified and treated accordingly over a 20-year period. As the pre-operative evaluation and prophylactic surgical revascularization strategies were instituted in 1983, the patients were divided into 2 groups: 25 patients between 1973-1982 (group A) and 85 patients between 1983-1992 (group B). The mean age of patients in group A was 65.3 years. The male/female ratio within this group was 21:4. One patient in the group had a history of IHD and 9 had hypertention. The mean age of patients in group B was 67.7 years. The male/female ratio within this group was 77:8. Fourteen patients in this group had a history of IHD and 27 had hypertension. Screening and treatment of IHD in group B was as follows. All patients with a history of IHD underwent CAG. Of the 32 patients with cardiac risk factors, including hypertension and hyperlipidemia, or ECG abnormalities who underwent DTS, 8 were referred for CAG. Thirty-nine patients with no risk factors and a normal ECG proceeded to AAA repair without further workup. Perioperative myocardial infarction occurred in 2 patients in grouzp A, leading to death in 1 patient. Coronary revascularization was performed in 5 patients in group B. No perioperative myocardial infarction occurred in this group. Pre-operative identification of high-risk cases with DTS, CAG, and coronary revascularization in patients with IHD may prevent cardiovascular complications in patients undergoing AAA repair.
4.A Case of Cryptococcosis with a Large Lung Mass
Tomohiro MORIYA ; Takuya SHINMURA ; Naoki KAWAKAMI ; Yoko WAKAI ; Kazuhito SAITO
Journal of the Japanese Association of Rural Medicine 2019;67(5):591-
In many patients with pulmonary cryptococcosis, chest radiographs show solitary or multiple nodular shadows, but pulmonary cryptococcosis with a large mass is rare. A previously healthy 45-year-old man presented to the hospital complaining of fever and cough. Contrast-enhanced computed tomography showed a tumor shadow with a long diameter of 64 mm in the lower lobe of the right lung. We considered the possibility of a lung abscess, but because it was refractory to antibiotics, we suspected the possibility of a malignant tumor. Transbronchial biopsy was performed, and Cryptococcus neoformans was detected. There were no complications of meningitis or immune deficiency, including acquired immune deficiency syndrome. He was administered antifungal drugs, and shrinkage of the shadow was achieved. Pulmonary cryptococcosis should also be considered when a large lung mass is observed.