BV equivalence with boundary.

An extension of the notion of classical equivalence of equivalence in the Batalin-Vilkovisky (BV) and Batalin-Fradkin-Vilkovisky (BFV) frameworks for local Lagrangian field theory on manifolds possibly with boundary is discussed. Equivalence is phrased in both a strict and a lax sense, distinguished by the compatibility between the BV data for a field theory and its boundary BFV data, necessary for quantisation. In this context, the first- and second-order formulations of nonabelian Yang-Mills and of classical mechanics on curved backgrounds, all of which admit a strict BV-BFV description, are shown to be pairwise equivalent as strict BV-BFV theories. This in particular implies that their BV complexes are quasi-isomorphic. Furthermore, Jacobi theory and one-dimensional gravity coupled with scalar matter are compared as classically equivalent reparametrisation-invariant versions of classical mechanics, but such that only the latter admits a strict BV-BFV formulation. They are shown to be equivalent as lax BV-BFV theories and to have isomorphic BV cohomologies. This shows that strict BV-BFV equivalence is a strictly finer notion of equivalence of theories.

General Relativity and the AKSZ Construction.

In this note the AKSZ construction is applied to the BFV description of the reduced phase space of the Einstein-Hilbert and of the Palatini-Cartan theories in every space-time dimension greater than two. In the former case one obtains a BV theory for the first-order formulation of Einstein-Hilbert theory, in the latter a BV theory for Palatini-Cartan theory with a partial implementation of the torsion-free condition already on the space of fields. All theories described here are BV versions of the same classical system on cylinders. The AKSZ implementations we present have the advantage of yielding a compatible BV-BFV description, which is the required starting point for a quantization in presence of a boundary.

Matching oceanography and genetics at the basin scale. Seascape connectivity of the Mediterranean shore crab in the Adriatic Sea.

Investigating the interactions between the physical environment and early life history is crucial to understand the mechanisms that shape the genetic structure of marine populations. Here, we assessed the genetic differentiation in a species with larval dispersal, the Mediterranean shore crab (Carcinus aestuarii) in the Adriatic Sea (central Mediterranean), and we investigated the role of oceanic circulation in shaping population structure. To this end, we screened 11 polymorphic microsatellite loci from 431 individuals collected at eight different sites. We found a weak, yet significant, genetic structure into three major clusters: a northern Adriatic group, a central Adriatic group and one group including samples from southern Adriatic and Ionian seas. Genetic analyses were compared, under a seascape genetics approach, with estimates of potential larval connectivity obtained with a coupled physical-biological model that integrates a water circulation model and a description of biological traits affecting dispersal. The cross-validation of the results of the two approaches supported the view that genetic differentiation reflects an oceanographic subdivision of the Adriatic Sea into three subbasins, with circulation patterns allowing the exchange of larvae through permanent connections linking north Adriatic sites and ephemeral connections like those linking the central Adriatic with northern and southern locations.

Rare diffuse diseases of the lung. Pulmonary alveolar proteinosis, lymphangioleiomyomatosis, amyloidosis.

Pulmonary alveolar proteinosis (PAP), lymphangioleyomiomatosis (LAM) and amyloidosis are three unrelated diseases of rare occurrence, with characteristic histopathological features. A pattern of alveolar filling with granular pink material accumulation is characteristic of PAP. This material can be recognized in lung biopsies, but also in bronchial lavage fluid. PAP is clinically related to the abnormal clearance of alveolar surfactant, most commonly due to the disruption of the granulocyte macrophage-colony stimulating factor signalling pathway. Whole lung lavage is the treatment of choice. LAM is characterized by cystic lung degeneration and interstitial proliferation of LAM cells, which express both melanocyte and smooth muscle cell markers, has a typical cystic pattern on CT scan, can be associated clinically with abdominal angiomyolipomas and limphangioleiomyomas, and occurs in female patients, either in isolation or as a manifestation of tuberous sclerosis. Sex hormone manipulation is the therapy of choice in this otherwise progressive disease. Diffuse interstitial or perivascular amyloid deposits in the lung can form in the context of systemic amyloidosis, usually associated with myeloma or monoclonal gammopathy, and less often with chronic inflammatory diseases. Nodular amyloid deposits, in contrast, are not associated with systemic lung disease, and present instrumentally as a coin lesion or lung mass. Isolated tracheobronchial amyloidosis is another rare form that is not related to systemic disease. In all conditions, amyloid has a typical waxy, amorphous, slightly eosinophilic stain, stains red with Congo red and presents a characteristic apple-green birefringence under polarized light, which is essential for diagnosis.

Clear-cell proliferation of the lung with lymphangioleiomyomatosis-like change.

AIMS: To describe two cases of a peculiar pulmonary lesion, which expand both the morphological and the immunophenotypic spectrum of perivascular epithelioid cell (PEC)-related disorders. METHODS AND RESULTS: One man and one female, with and without the tuberous sclerosis complex (TSC), respectively, showed pulmonary cysts and small nodules on computed tomography scan. In the former, lymphangioleiomyomatosis (LAM) was suspected. In both cases, an open lung biopsy was performed, whose cut surface displayed numerous cysts lined by thin/thick septa. Microscopically, the septa were associated with micronodular or interstitial proliferation of medium/large-sized elements with abundant clear (periodic acid-Schiff-positive/diastase-sensitive) cytoplasm and distinct cell borders, embedded in fibrous tissue. The elements were CD34+, vimentin-positive and, to a lesser extent, HMB-45+ and MART-1+. The stains for specific muscle actin, desmin, S100 protein, CD31, FVIIIRAg, cytokeratins, CD45, CD68, oestrogen and progesterone receptors were all negative. Ki67 labelling was <1%. Electron microscopy displayed cytoplasmic vacuoles containing glycogen particles. The TSC1 and TSC2 gene status could not be assessed because of poor DNA preservation. In the man with TSC, a focus of micronodular pneumocyte hyperplasia was also found. CONCLUSIONS: Because of the coexpression of CD34 and melanoma-associated antigens and the occurrence of TSC in one patient, the cases described here add a new piece to the puzzle of PEC lesions and contribute to the open discussion on the origin of LAM and LAM-like proliferations.

EDU-CARE, a randomised, multicentre, parallel group study on education and quality of life in COPD.

Chronic obstructive pulmonary disease (COPD) represents one of the main causes of morbidity and mortality in the western world. Unfortunately, its therapy is largely palliative, the key aims of treatment being to reduce exacerbations, minimise symptoms, and improve patients' ability to perform their usual daily activities. In the absence of true disease-modifying treatments, the concept of rehabilitation has become important. In addition, it has been shown that educational and self-management programmes may play a role in the general treatment of COPD patients. This study was promoted by the Italian Association of Hospital Pulmonologists (AIPO) with the aim to verify changes and improvements induced by an educational programme validated by AIPO in patients with COPD. Edu-Care is a 6-month, multicentre, randomised, controlled, parallel-group study. In addition to treatment within the usual therapeutic schemes for COPD, patients were randomised to either the 'Educational' group, i.e. to receive a formal and structured educational programme, or the 'Normal General Advice' group, i.e. to receive the usual general advice given by general practitioners on life-style and on the disease's risk factors and treatment. A number of evaluations were performed: pulmonary function test, walking distance, quality of life, locus of control, register of number of exacerbations and hospital admissions. To date, of the 1,230 patients enrolled interim data are available from 1,003 patients. Males represent 85% of the study population. Smoking habit is quite a common status (21%). In the year prior to enrolment 34% of patients had one exacerbation, 49% 2-3 exacerbations, and 17% more than 3 exacerbations. Seventy-two percent of patients were not hospitalised over the year prior to enroLlment, while 22% were hospitalised once and 6% had more than 2 hospitalisations. Edu-Care is the first large study aimed to evaluate the efficacy of an educational programme for patients with COPD. AIPO wishes to make a contribution to this important field. This is the reason why Edu-Care includes a very large number of patients in numerous Italian centres throughout northern and southern Italy.

First report of the Italian register for diffuse infiltrative lung disorders (RIPID).

RIPID was established in 1998 as a joint project of the major Italian scientific societies for Respiratory Medicine, with the aim to create an Italian Register on diffuse infiltrative lung disorders that can provide the basis for epidemiological and clinical studies of adequate sample size. In the period from May 1998 to December 2000, 1,382 cases were submitted from 54 Centers in 15 regions of Italy, 54.2% males (mean age +/- SD 50.5 +/- 16.8 years) and 45.8% females (50.2 +/- 15.3 years). A current smoking habit emerges in 18% of subjects; former smokers and never-smokers represent 26% and 56% of the total case series, respectively. The most frequent disease registered is idiopathic pulmonary fibrosis (37.6%), followed in decreasing order by sarcoidosis (29.2%), and Langherans' cell hystiocytosis (6.6%). High resolution computed tomography (HRCT) was considered as the most important tool for final diagnosis in the majority of cases (74.4%); 39.4% of patients underwent transbronchial biopsies, 39.2% bronchoalveolar lavage (BAL). A surgical biopsy was performed in 20.5% of patients. A web site has been activated from December 2000 (www.pneumonet.it/ripid), allowing prompt access to all information and scientific material concerning the project and to an electronic form for data collection that can be completed on-line.

Altered thymic endocrine activity along with impairments of peripheral zinc metabolism and T-lymphocyte populations are associated with myasthenia gravis: a follow-up study.

Thymic endocrine activity was assessed by a bioassay to determine the basal activity of thymulin (TH), a zinc dependent hormone, and its in vitro reactivation in two different age groups of patients with myasthenia gravis (MG). Before thymectomy, basal TH plasma levels were increased in patients over the age of 50 years. Plasma zinc levels were increased in all patients, this increment being very high in old patients. One year after thymectomy both TH and zinc plasma levels decreased. While zinc plasma levels were within the normal ranges for their respective ages, TH levels were lower in young and higher in old patients than in age comparable controls. Young patients with MG showed increased CD3,DR positive peripheral T-cells as well as lymphocytes with the CD16,CD56 phenotype. An increment of CD3 positive cells along with CD4 and CD16,CD56 positive cells were found in older patients. Thymectomy partially affected blood lymphocyte representation only in young patients, since CD3,DR T-cells decreased one year after surgery. No significant variations in T-cell representation were found in old patients after thymectomy. Immunosuppression in thymectomized patients did not significantly affected TH and zinc plasma levels. Very high levels of TH and the presence of additional alterations in T-lymphocyte subsets in old patients suggested that differential age related pathogenetic immunological mechanisms might be associated with the disease.

Central alveolar hypoventilation (Ondine's curse) treated with negative pressure ventilation.

A 22 year old woman with central alveolar hypoventilation (CAH) underwent repeated nocturnal polysomnographic recordings to assess the efficacy of negative pressure ventilation (NPV) administered by pneumowrap. The NPV successfully prevented hypoxia during most of the recording time; however, a sudden severe hypoxic episode not related to an apnoea occurred during a recording night with NPV. This made home use advisable together with an ear oximeter fitted with an alarm.

External negative pressure ventilation techniques.

External ventilation was introduced at the beginning of the century to treat acute and chronic respiratory failure. Long-term negative pressure ventilation (NPV) has proved useful in patients with respiratory failure, secondary to a restrictive impairment of neuromusculoskeletal origin. Although NPV may be successfully used in acute-on-chronic respiratory failure in patients with chronic airflow obstruction (CAO), its use in the long-term management of this type of patient seems much less promising. The various types of ventilators, iron lung, pneumo-wrap and cuirass are discussed. All these prostheses, except for the pneumo-wrap, are connected to a pump able to generate negative and positive pressure. NPV must be a controlled ventilation, because an effective trigger is not yet available. The most important feature of the ventilator must be the ability to vary the ventilatory pattern to adapt the machine drive to the patient drive. The goal of NPV is not only to normalize gas exchange, but also to restore an effective spontaneous ventilation. This kind of ventilation has no negative effects on haemodynamics, because it does not vary physiological gradients of transthoracic pressure. However, due to lack of coordination between the respirator and the pharyngeal muscles of the patient, obstructive apnoeas may occur during NPV in sleeping patients.

Lymphangioleiomyomatosis: clinical course.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of smooth muscle proliferation that generally leads to death, due to respiratory failure, within 10 yrs. The disease almost exclusively affects women of child-bearing age and rarely in postmenopausal years. High resolution computed tomographic (CT) patterns are characteristic and almost pathognomonic for LAM. Attempts to treat this disease with hormonal therapy have shown that a number of hormonal manipulations may not be helpful, but that surgical or radiant oophorectomy alone is the most effective treatment, even if the disease is so rare as to preclude randomized trials. Castration, as a means of early therapy, has been associated with a stable respiratory picture in our patients.

[Relapsing polychondritis]. / La policondrite recidivante.

Relapsing polychondritis is a rare disease of unknown origin consisting in recurrent inflammatory episodes of cartilaginous structures as well as other organs such as the eye, the inner ear and the kidney. Symptoms of autoimmune or rheumatic disorders frequently precede those typical of relapsing polychondritis and thus the contemporaneous presence of these diseases is a common observation. Consequently some diagnostic concerns connected to the great variability of the early symptoms and also to the lack of a semiotic test do exist. It is widely recognized that a serious involvement of the tracheo-bronchial cartilage the lumen diameter with a consequent heavy dyspnoea which reduces occurs in relapsing polychondritis. In these cases the study of the thorax area by means of CT was of fundamental importance. Furthermore, histological analysis of bioptic specimens from tissues only apparently not affected, such as the pinna of the ears, better defines the nature of the disease. The Authors present a case report, characterized by the presence of a saddle-nose deformity as well as a dyspnoea caused by a lumen stenosis related to tracheo-bronchial cartilage chondritis.

Breathing disorders during sleep in chest wall diseases.

Chest wall deformity due to kyphoscoliosis may present with two altered breathing patterns during sleep; 1) progressive O2 desaturation particularly during REM sleep and 2) central apnoea also during REM sleep but rarely accompanied by O2 desaturation. No typical breathing pattern abnormality is seen in the rigid spine syndrome.

[Myasthenia and muscle relaxants]. / Miastenia e miorilassanti.

Eighteen myasthenic patients have been operated on under general anaesthesia; 14 subjected to thymectomy and 2 to emergency procedures (caesarean section and laparotomy because of intestinal obstruction). Atracurium (0.3 mg/kg) and vecuronium (0.06 mg/kg) exhibited a long duration of action only in the two cases affected by the more severe signs and symptoms of the disease.

Intermittent negative pressure ventilation in patients with restrictive respiratory failure.

Thirty one patients in stable respiratory failure (arterial oxygen tension (PaO2) 67 +/- 20 mmHg (8.9 +/- 2.7 kPa) and arterial carbon dioxide tension (PaCO2) 59 +/- 10 mmHg 7.9 +/- 1.3 kPa)), secondary to non-obstructive ventilatory impairment, were treated by intermittent negative pressure ventilation (INPV), using a pneumowrap or poncho during the night. Daytime arterial blood gas measurements, taken before and after the ventilation, revealed a substantial improvement in PaO2, PaCO2 and maximal inspiratory pressure (Pimax). This improvement persisted during the six month follow-up of home nocturnal ventilation. The polysomnographic, recording during mechanical ventilation by poncho, in five patients, showed a general improvement in the quality and structure of sleep. All patients returned to normal arterial oxygen saturation (SaO2) values (> 90%), except for one. In three patients, the appearance of obstructive events was noted but the desaturation that they caused was, remarkably, lower than that caused by central events in baseline recording. We conclude that non-invasive treatment by INPV, in patients with neuromuscular and chest wall disease, is the method of choice as an alternative to intermittent positive pressure ventilation and to tracheostomy.