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Clearance of inhaled technetium-99m diethylenetriaminepentaacetate (99mTc-DTPA) is a potential indicator of disease activity and progression in idiopathic pulmonary fibrosis (IPF). The objective of the present study was to evaluate the prognostic value of 99mTc-DTPA scans in IPF. A total of 22 patients (18 males), aged 33 to 80 years with IPF were followed for six to 20 months (mean 13 months). At diagnosis, high resolution computed tomography (HRCT) scans showed a honeycomb pattern with bibasilar reticular opacities in all cases. At T0 (diagnosis) and T1 (follow-up), each patient had pulmonary function tests (forced vital capacity, diffusing capacity of the lung for carbon monoxide and partial arterial O2 pressure), extension of fibrosis evaluated by HRCT visual score and 99mTc-DTPA lung clearance. Results at T0 and T1 were compared, taking into account the whole population and patients with relatively fast and slow 99mTc-DTPA wash-out. 99mTc-DTPA clearance did not show any significant correlation with functional tests or HRCT score. These findings indicate that clearance of inhaled 99mTc-DTPA is not of value in following the progress of IPF.

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.

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.

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 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.

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 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.

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.

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.

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.

We evaluated the ability of NPV to suppress EMGd and EMGint in seven patients with severe COPD and five normal subjects. Subjects were studied either without (A) or with mouthpiece and nose clip (B). Electromyographic suppression was assessed comparing EMG activity during NPV with the control activity without a mouthpiece and prior to the initiation of the NPV run. In normal subjects, in A, NPV resulted in a partial suppression of EMGd; in B, prior to NPV, EMGd rose compared with A prior to NPV. In patients, in A, NPV resulted in a suppression of both EMGd and EMGint. In B, prior to NPV, both EMGd and EMGint rose compared with A prior to NPV. Thus, it seems that NPV is able to produce a consistent reduction in inspiratory muscle EMG activity. This variable NPV ability would have to be assessed for better selection criteria for patient candidates in a rehabilitation program.

The authors describe the case of a 70 year-old asymptomatic female, who showed nodular pleural growths at an occasional chest roentgenogram. Histological examinations and laboratory findings excluded the possibility of an inflammatory granulomatous process, but they did not allow differentiation between malignant sarcomatoid pleural mesothelioma and malignant fibrous histiocytoma. Only at immunohistochemistry was the differential diagnosis made. In fact, the co-expression of vimentin and cytokeratins by neoplastic cells, along with their negativity for macrophage markers strongly supported the hypothesis that the neoplastic growth originated from subpleural mesenchymal stem cells.

Lymphangioleiomyomatosis (LAM) is an uncommon disease affecting women of reproductive capacity. It is characterized by non-neoplastic proliferation of smooth muscle in the lungs and, occasionally, in lymph nodes and in the thoracic duct. The patients present with dyspnea, chylous pleural effusion, pnx, and hemoptysis. The authors evaluated 4 patients with biopsy-proven LAM, by means of CT. In all cases, CT revealed small well-defined cystic air spaces with smooth and thin walls, scattered in the lungs. CT was very useful because it was more sensitive and specific than conventional radiography. The CT appearance of LAM distinctly differed from that of other diseases than can produce cystic air spaces in the lungs, such as UIP, eosinophilic granuloma, centri-acinar emphysema, and bronchiectasis. Our experience confirms the clinical usefulness of CT in diagnosing LAM.

The role of weight loss in the therapy of obstructive sleep apnea syndrome (OSAS) was investigated in 23 affected patients with various degrees of obesity (body mass index range 26.6-61.0) free of cranio-facial malformations. Weight loss resulted 18.5 +/- 14.7 (s.d.) kg and was significantly correlated with baseline BMI value (r = 0.94; P less than 0.0001). Weight loss significantly reduced the number of apneas + hypopneas per hour of sleep ((A + H)I) from 66.5 +/- 23.0 to 33.0 +/- 26.2 (P less than 0.0001) and improved the mean of oxygen desaturation peaks during apneas (mSaO2) from 81.9 +/- 6.9 to 87.6 +/- 3.9; P less than 0.001). A significant correlation was found between weight loss and changes in the (A + H)I (r = -0.55; P less than 0.01) and the mSaO2 (r = 0.46; P less than 0.05). The (A + H)I significantly improved in both patients who lost more than 10 kg (basal BMI: 42.3 +/- 10.0) and in those who lost less than 10 kg (basal BMI: 30.2 +/- 2.3), whereas the mSaO2 improved only in the former. Obese patients with moderate to heavy ORL pathological findings had worse pretreatment and final OSAS parameters than those with absent or mild ORL lesions. However, both groups showed a significant, although quantitatively different, improvement of the (A + H)I and mSaO2 after weight loss. Compared to those who were cured or improved after the treatment, patients who failed to obtain significant effects on OSAS clinical presentation also had a significantly higher prevalence of ORL pathology. It is concluded that: (1) weight loss improves parameters and clinical presentation of OSAS in the majority of affected obese patients; (2) a relationship exists between the entity of weight loss and that of improvement of the syndrome; (3) weight loss must be encouraged even in patients with mild to moderate overweight; (4) the presence of ORL pathology may represent a confusing factor in the interpretation of the results obtained after weight loss.

Patients with severe COPD may be in a state of ventilatory muscle (VM) fatigue. In these patients, rapid and shallow breathing has been hypothesized to be a compensatory mechanism that prevents more severe fatigue from taking place. To test these hypotheses, we studied the effects of VM resting in a group of patients with severe COPD. Eleven clinically stable patients with COPD and chronic hypercapnia were studied. Six of them (group A) had a seven-day period of negative pressure-assisted ventilation (NPV), and five (group B) with similar functional characteristics served as a control group. Compared with a normal age-matched control group, both A and B groups exhibited significantly lower tidal volume (VT), inspiratory time (TI), total time of the respiratory cycle (Ttot) and Ti/Ttot ratio, decrease in muscle strength, and greater electromyographic activity of diaphragm (EMGd) and parasternal muscles, but similar ventilation and VT/TI. After the study period, group A exhibited significant increase in VT, Ti, and TI/Ttot (p less than 0.05), and decrease in PaCO2 (p less than 0.05), EMGd, and EMGint (p less than 0.05 for both), and a slight but significant increase in maximal inspiratory pressure (MIP) (p less than 0.05). These data suggest that NPV rests VM, increases their strength, and reduces hypercapnia in patients with severe COPD.

A correct staging of sarcoidosis is of the uppermost importance in the diagnosis and treatment of the disease. In the last few years new concepts have been stated: --radiological staging of sarcoidosis is particularly difficult --in no one phase the radiological pattern can be considered typical but sometimes it is highly suggestive for sarcoidosis --anatomical studies recently showed that sarcoid disease seems to begin with a non granulomatous interstitial pneumonia. Following this individual microscopic deposit may conglomerate to form the typical non caseating granulomas. In the lung the disease seems to procede from the interstitium to the lymphnodes. Usually, in the first stage, possible sarcoidosis is diagnosed by the radiologist, but a confident diagnosis needs a pathological confirmation. Transbronchial biopsy guided by fluoroscopy is the first diagnostic choice for obtaining confirmation of the disease and for determining the type and entity of parenchymal lesions.

Hemodynamic effects of orally-administered nifedipine were evaluated in 12 patients with pulmonary hypertension secondary to severe COPD after short-term (30 and 60 minutes) treatment and then again in eight of these 12 patients after long-term (average 55 days) treatment. Pulmonary vascular resistance (PVR) decreased from 426 +/- 52 to 294 +/- 28 dynes.s.cm-5 (p less than 0.001) after therapy with 20 mg sublingual nifedipine (at 60 minutes). Cardiac index (CI) increased from 3.7 +/- 0.2 to 4.6 +/- 0.3 L/min/m2 (p less than 0.001). There was a decrease in mean pulmonary artery pressure (MPAP) only in 4/12 patients after Nifedipine. There was no significant fall in PaO2, while PvO2 and oxygen delivery (CI X CaO2) increased significantly 60 minutes after administration of sublingual nifedipine. PVR decreased from 482 +/- 82 to 374 +/- 44 dynes.s.cm-5 (p less than 0.05) after long-term nifedipine therapy. The changes in PVR and CI 60 minutes after administration of nifedipine in the patients on long-term treatment were similar to those observed with the same doses of nifedipine before initiation of therapy. Despite beneficial hemodynamic effects in two of eight patients, there was progressive clinical worsening. The benefit of long-term administration of nifedipine is difficult to predict on the basis of short-term effects.

A case of Ondine's curse associated with hypothalamic dysfunction in an 8-year-old boy is described. The neuropathological examination revealed a viral encephalitis affecting the hypothalamus and the brainstem. In the medulla the inflammatory process involved the reticular formation and the nuclei considered to control automatic respiration such as the dorsal motor nucleus of the vagus, the nucleus tractus solitarii and the nucleus ambiguous. Although Ondine's curse following viral infection of the central nervous system has been previously reported this represents the first case of viral encephalitis to be pathologically documentated.

In order to detect the dysfunction of the upper airway muscles in awake patients with obstructive sleep apnea syndrome (OSAS), we examined the effect of a chemical stimulus (CO2) on flow-volume loops obtained during tidal breathing. Six flow-volume curves during forced maneuvers and six flow-volume tracings during rebreathing of CO2 were recorded in 20 patients with OSAS. All patients had numerous obstructive apneas (mean apnea index 61 +/- SD 33, percentage of obstructive apneas 76 +/- 29) associated with moderate to severe degrees of arterial oxygen desaturation. Of the 18 patients in whom at least six flow-volume loops could be obtained during CO2 rebreathing, 13 exhibited respiratory flutter (range: grade 1 to 6) during tidal breathing in the flow-volume tracing (RF VtCO2). The greater the degree of RF VtCO2 during wakefulness, the less was the lowest oxygen saturation (LSaO2) during sleep. In fact, all four patients with RF VtCO2 score 6 had LSaO2 below 30%.

A case of pulmonary lymphangioleiomyomatosis is presented, which occurred in a 50-year-old woman suffering from severe dyspnea of 7 year's duration and with a previous history of diseases (polycystic ovaries and endometrial adenocarcinoma) usually related to hyperestrogenism. The patient showed no signs or symptoms of tuberous sclerosis, a condition often associated with pulmonary lymphangioleiomyomatosis. Both the radiological pattern and histological findings had previously been interpreted at other hospitals as consistent with either nodular interstitial fibrosis with emphysematous changes or pulmonary siderosis. Correct diagnosis was suspected on critical review of histological slides and definitely achieved by immunohistochemistry in routine sections, which showed the presence of desmin in the elements constituting the nodular lesions scattered throughout the parenchyma. The criteria for the differential diagnosis are discussed, as well as the role of immunohistochemistry in assessing the muscular nature of proliferating cells.

A comparison between the radiographical findings and the haemodynamic data was made in 53 cases of chronic bronchial obstruction. The results were used in an assessment of the reliability of standard chest X-rays in the diagnosis of pulmonary hypertension. Reference is also made to quantitative indices. Several cases are presented by way of example.

The clinical and radiologic findings of the chronic eosinophilic pneumonia are presented. A personal observation of one case is reported. This should be the first in the Italian roentgenologic literature. The importance of radiology in the diagnosis and in the evolution of the disease is stressed.

The physiopathologic and anatomic findings of bronchial asthma are shortly reviewed. A description follows of the radiologic patterns based on personal experience. Only seldom the radiologic investigations provide the possibility to reach the correct diagnosis of bronchial asthma. But they always complete the clinical evaluation and allow the demonstration of the numerous and frequent complications which influence the evolution and prognosis of the disease.

This report refers to a 51 year old man with the clinical features of central alveolar hypoventilation (CAH). Polysomnographic recordings showed periodic breathing and central apnoeas associated with abnormal oscillations of systemic arterial pressure and heart rate during all sleep stages. Oxygen administration during sleep reduced hypoxia, while the periodic breathing and arterial pressure oscillations persisted. The authors suggest that an impairment of the brain structures that play a role in homeostatic adjustment of autonomic functions in connection with the sleep-wake cycle, is responsible for the unusual sleep-related disturbances shown by this patient.

A new method of studying regional lung functions using 133Xe and the computer-assisted scintillation camera is described. The method and the computer processing give details of the distribution of 133Xe during perfusion and ventilation and also measures of lung function. Some cases are described in detail, and the clinical significance of the method was confirmed in 45 cases.

This study was carried out in order to evaluate changes in pulmonary vascular reactivity and in hemorheology induced by pentoxifylline infusion (100 mg) at rest and during standardized exercise in patients with chronic cor pulmonale secondary to chronic obstructive pulmonary disease. The administration of pentoxifylline at rest was associated with reduction in mean pulmonary artery pressure (p less than 0.01), pulmonary vascular resistance (p less than 0.01) and right ventricular stroke work index (p less than 0.02). Standard exercise performed after pentoxifylline infusion was also associated with significant reduction in mean pulmonary artery pressure and pulmonary vascular resistance. Rheologic tests showed less evident changes. Our data suggest that pentoxifylline significantly improves pulmonary hemodynamics at rest and partly reverses its changes, after exercise, in patients with chronic obstructive pulmonary disease.

Respiratory failure has been described in myotonic dystrophy; it worsens during sleep but its central or peripheral origin has yet to be determined. Moreover, patients may present severely disturbed sleep and daytime somnolence. Eight patients with mild to moderate myotonic dystrophy were studied to assess breathing function while awake and during sleep by means of the pulmonary function tests, nocturnal polysomnographic examination and the multiple sleep latency test (MSLT). Three patients had restrictive respiratory defects; none had signs of airway obstruction. All patients had very disrupted nocturnal sleep. Of six patients who underwent the MSLT only two showed a mild tendency to sleep during the day. Six patients had pathological apnoea plus hypopnoea index [(A+H)I] and there was a prevalence of central apnoeas. The apnoeas occurred while resting but awake and throughout all sleep stages. Only two patients (the ones with the least vital capacity) had episodes of progressive oxygen desaturation during rapid eye movement sleep, similar to those found in other restrictive disorders and in chronic obstructive pulmonary disease. It is concluded that the breathing pattern characteristic of our myotonic dystrophy patients was the occurrence of central apnoeas both at rest while awake and during sleep.

Two groups of patients were studied with serial correlated radiological functional analysis. Each group had a previous history of chronic obstructive pulmonary disease; however, on admission, only one group exhibited the clinical signs of this superimposed disease. The results of this research showed that chronic pulmonary disease is better defined by functional studies while cardiac failure (even in early stages) is always revealed by radiological examination.