Patients with familial amyloid polyneuropathy show a high incidence of myocardial adrenergic denervation with viable myocardium that can be identified very early in cardiac amyloidosis, before the development of clinically apparent heart disease, ventricular wall thickening, significant LV systolic and diastolic dysfunction and positive findings on Tc-99m PYP myocardial scanning.

E En nd do ot th he el li in n--1 1 a an nd d i in nt te er rl le eu uk ki in n--8 8 i in n h hi ig gh h a al lt ti it tu ud deABSTRACT: We present a case of high altitude pulmonary oedema (HAPE) with pulmonary hypertension and polymorphoneuclear leucocyte (PMN) accumulation in bronchoalveolar lavage fluid (BALF), which occurred in a 21 year old man. Plasma endothelin-1 (ET-1) and interleukin-8 (IL-8) concentration in BALF were elevated on admission, and returned to normal level at recovery, when the pulmonary artery pressure and the PMN counts in BALF were normal. In addition, E-selectin and intercellular adhesion molecule-1 (ICAM-1) in BALF were also slightly increased on admission.These findings suggest that endothelin-1 is a vasoconstrictor which contributes to the pulmonary hypertension in high altitude pulmonary oedema, and that some of the inflammatory mediators play an important role in chemotaxis and accumulation of polymorphonuclear leucocytes in the development of high altitude pulmonary oedema. Eur Respir J., 1996Respir J., , 9, 1947Respir J., -1949. High altitude pulmonary oedema (HAPE) is a severe type of acute mountain sickness. It is a serious, sometimes fatal illness related to rapid exposure to altitude. Although the pathophysiological mechanism of HAPE remains unclear, it has long been suggested that pulmonary vasoconstriction and increased pulmonary vascular permeability are important contributory factors [1,2]. Recent studies have suggested that endothelin is related to pulmonary vasoconstriction [3][4][5], and that the increased pulmonary vascular permeability is probably caused by inflammatory mediators [6][7][8][9][10]. However, no studies have been conducted on the role of endothelin and cytokines in patients with HAPE. We report a case of HAPE, documenting the possible role of endothelin-1 (ET-1), and polymorphoneuclear leucocyte (PMN) chemotactic mediators, such as interleukin-8 (IL-8). Case reportA Japanese male university student, aged 21 yrs, who was healthy before climbing, arrived at the foot of the Japan Alps at 1,000 m above sea level. The next morning, he started climbing to a snowy valley about 2,000 m above sea level and still felt well. On the third day, whilst climbing to Mt Yari (3,180 m), he developed general fatigue, followed by headache, cough, sputum, poor appetite and fever (38˚C). After staying all day to rest at 3,000 m, his condition progressively deteriorated. On the fifth day, he was lethargic, comatose, cyanotic, and had a cough with foamy sputum. He was rescued, transported by helicopter, and admitted to our Shinshu University Hospital (660 m).On admission, he had cyanosis and was unconscious. His temperature was 38˚C, pulse rate 140 beats·min -1 , respiration rate 26 breaths·min -1 , and his blood pressure 140/90 mmHg. Coarse crackles were audible over both sides of the lung. He showed decerebrate rigidity. An examination of the ocular fundi showed papilloedema and multiple flame-shaped and blot haemorrhages in both fundi. A chest roentgenogram revealed patc...

The role of systemic chemotherapy and optimal regimen in thymic carcinoma remains uncertain. We evaluated the clinical responsiveness of ADOC (cisplatin, doxorubicin, vincristine, and cyclophosphamide) chemotherapy for advanced thymic carcinoma that have distant metastatic or unresectable lesions. From 1996 to 2000, we treated eight cases of thymic carcinoma. According to the classification by Masaoka et al., the clinical stage in one case was IVa, whereas the others were IVb. Histologic subtypes were as follows: four cases were squamous cell carcinoma, two cases were undifferentiated, and two were small-cell carcinoma. All patients received 50 mg/m2 of cisplatin and 40 mg/m2 of doxorubicin intravenously on day 1, 0.6 mg/m2 of vincristine intravenously on day 3, and 700 mg/m2 of cyclophosphamide intravenously on day 4, ADOC regimen, respectively, at 3- to 4-week intervals. Six patients obtained a partial response after ADOC chemotherapy and the overall clinical response rate was 75%. There were no life-threatening side effects noted. Cisplatin plus VP-16 chemotherapy (PVP) was performed in three cases before the ADOC regimen, but PVP chemotherapy did not show beneficial effects in two patients. Median survival time was 19 months. ADOC chemotherapy appears to have significant activity against thymic carcinoma.

A 67-year-old female patient with biopsy proven AL systemic amyloidosis developed rapidly progressive dyspnea. Chest roentgenogram and CT scan revealed a large right pleural effusion in addition to nodular lesions with bilateral hilar lymphadenopathy. The patient's serum showed IgG lambda type monoclonal gammopathy and she also had Bence Jones proteinuria. The pleural effusion was an exudate that contained many mononuclear cells and a high concentration of protein. Cardiac function was not seriously disturbed. Except for amyloidosis, no other causes for the severe pleural effusion were found. This patient was treated with chemical pleurodesis using Picibanil and a low dose of prednisolone. Eighteen months after this treatment, her right pleural effusion did not recur. Bronchopulmonary tissues are known to be frequently involved by AL systemic amyloidosis, but a nodular pattern of pulmonary amyloid deposition and a unilateral large pleural effusion are rare clinical manifestations in this disease.