Tuberous sclerosis with pulmonary involvement.

Pulmonary tuberous sclerosis produced interstitial disease in a woman with normal-sized lungs; numerous hemosiderin-laden macrophages were found in the fluid obtained through bronchoalveolar lavage. The pathological changes seen in the lungs were identical to those of pulmonary lymphangiomyomatosis, in which the constellation of clinical signs usually found in tuberous sclerosis is absent. The two conditions are sufficiently similar in clinical presentation, pathological changes and prognosis to be considered variants of the same disease. The recent findings of progestin receptors in lung tissue from patients with pulmonary lymphangiomyomatosis will likely direct future management towards hormonal manipulation.     

Mycosis fungoides with pulmonary involvement. Cytopathologic findings

In order to define the cytologic features of pulmonary involvement by mycosis fungoides, 15 respiratory cytology specimens from four patients with biopsy-proven pulmonary mycosis fungoides were reviewed. The presence in sputum smears of occasional small or large cerebriform mononucleated cells against a background of numerous atypical lymphocytic cells permitted an antemortem cytologic diagnosis of probable or definite dissemination of mycosis fungoides with pulmonary involvement. Similar cells were seen in aspiration smears. The lymphocytic infiltrates were similar to those in corresponding skin biopsies in each case. The distinctive cytologic findings in these cases may therefore help to determine the underlying etiology of pulmonary lesions and may contribute to the antemortem diagnosis of visceral dissemination of mycosis fungoides.     

Cardiac involvement in Caucasian patients with pulmonary sarcoidosis

Background

Cardiac sarcoidosis (CS) is a potentially life-threatening condition. At present, there is no consensus with regard to the optimal non-invasive clinical evaluation and diagnostic procedures of cardiac involvement in patients with sarcoidosis. The aim of this study in a large homogenous Scandinavian sarcoidosis cohort was therefore to identify risk factors of cardiac involvement in patients with sarcoidosis, and the value of initial routine investigation with ECG and cardiac related symptoms in screening for CS.

Methods

In this retrospective study a cohort of 1017 Caucasian patients with sarcoidosis were included. They were all screened with ECG at disease onset and investigated for CS according to clinical routine.

Results

An abnormal ECG was recorded in 166 (16.3%) of the 1017 patients and CS was later diagnosed in 22 (13.2%) of them, compared to in one (0.1%) of the 851 sarcoidosis patients with a normal ECG (p < 0.0001). The risk for CS was higher in patients with a pathologic ECG combined with cardiac related symptoms (11/40) (27.5%) compared to those with pathologic ECG changes without symptoms (11/126) (8.7%) (p < 0.01). Furthermore, patients with Löfgren’s syndrome had a reduced risk for CS compared to those without (p < 0.05) the syndrome.

Conclusions

This study on an unusually large and homogenous sarcoidosis population demonstrate the importance of an abnormal ECG and cardiac related symptoms at disease onset as powerful predictors of a later diagnosis of cardiac sarcoidosis. In contrast, CS is very rare in subjects without symptoms and with a normal ECG. This knowledge is of importance, and may be used in a clinical algorithm, in identifying patients that should be followed and investigated extensively for the presence of CS.     

Paxillin-associated focal adhesion involvement in perinatal pulmonary arterial remodelling.

Birth is followed by remodelling of the actin cytoskeleton of pulmonary arterial smooth muscle cells, then by extracellular matrix deposition. Hypothesising that the cell/matrix adhesions would also be remodelled, we investigated the expression, localisation and biochemical characteristics of the focal adhesion protein paxillin in vivo, in vessels from normal and pulmonary hypertensive neonatal piglets. Initially we showed that in intact porcine pulmonary arteries exposed to cytochalasin D there was a reduction filamentous actin accompanied by a reduction in paxillin-associated focal adhesions, similar to that seen in cultured pulmonary arterial smooth muscle cells. Vessels from normal and hypoxic animals were found to have two isoforms of paxillin, of 60 and 66 kDa with pI values of 6.7-4.2. Transient changes occurred during the first 14 days of life. Between birth and 6 days there was a reduction in the amount of both paxillin isoforms, a shift to more acidic pI values and an increase in paxillin phosphorylation. Simultaneously, immunostaining showed a transient reduction in paxillin expression, a change temporally and spatially associated with a previously demonstrated reduction in actin. Findings are consistent with an immediate postnatal spatial reorganisation of paxillin-associated focal adhesions. Paxillin content and remodelling was abnormal in pulmonary hypertensive arteries, the response varying according to postnatal age.     

Thromboxane-induced pulmonary vasoconstriction: involvement of calcium

Infusion of tert-butyl hydroperoxide (t-bu-OOH) or arachidonic acid into rabbit pulmonary arteries stimulated thromboxane B2 (TxB2) production and caused pulmonary vasoconstriction. Both phenomena were blocked by cyclooxygenase inhibitors or a thromboxane synthase inhibitor. The increase in pulmonary arterial pressure caused by either t-bu-OOH or arachidonic acid infusion correlated with the concentration of TxB2 in the effluent perfusate. The concentration of TxB2 in the effluent perfusate, however, was always 10-fold greater after arachidonic acid infusion. In the rabbit pulmonary vascular bed lipoxygenase products did not appear involved in the vasoactive response to t-bu-OOH or exogenous arachidonic acid infusion. Calcium entry blockers or a calcium-free perfusate prevented the thromboxane-induced pulmonary vasoconstriction. Calmodulin inhibitors also blocked the pulmonary vasoconstriction induced by t-bu-OOH without affecting the production of TxB2 or prostacyclin. These results suggest that thromboxane causes pulmonary vasoconstriction by increasing cytosol calcium concentration.     

慢性阻塞性肺疾病患者继发真菌性医院肺炎的病原菌分布及耐药性分析

目的探讨慢性阻塞性肺疾病（Chronic obstructive pulmonary diseases,COPD）患者继发真菌性医院肺炎的病原菌分布及耐药情况。方法回顾性分析2011年1月至2012年12月间31例COPD患者继发肺部真菌感染的情况。结果 31例COPD继发肺部真菌感染的患者送检标本中共检到34株真菌,以曲霉菌属居首位,占29.41%,其次为白色假丝酵母菌,占26.41%。7种常用抗真菌药物对真菌显示有不同的抗菌活性,尤以伊曲康唑、氟康唑、5-氟胞嘧啶为佳。结论 COPD患者继发肺部真菌感染多为曲霉菌属和白色假丝酵母菌。临床疑为COPD患者继发肺部真菌感染病例须及时采集标本培养,以早期发现病原性真菌和选择有效药物治疗。     

Mycosis fungoides. The importance of pulmonary cytology in the diagnosis of a case with systemic involvement

A patient with cutaneous mycosis fungoides developed pulmonary lesions while under radiation therapy. Bronchial cytologic specimens demonstrated malignant lymphocytes, which open lung biopsy confirmed to be mycosis cells. We believe this is the first report to document pulmonary involvement of mycosis fungoides by the use of bronchial cytology.