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Biomedical subjects

M Schiavina

Publications and source records attributed to M Schiavina.

At least 19 recordsLinked to original sources

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.

Adolescent

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.

Adult

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.

Humans

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.

Adult

[Myasthenia and muscle relaxants].

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.

Adolescent

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.

Adult

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.

Adult

Suppression of ventilatory muscle activity in healthy subjects and COPD patients with negative pressure ventilation.

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.

Adult

Bronchopulmonary involvement in ulcerative colitis.

We report two cases of pulmonary involvement in ulcerative colitis. The first patient, a 37-year-old woman, had bilateral basal bronchiectasis full of mucopurulent secretion, with a marked improvement of pulmonary function and roentgenographic appearance after a conservative approach. The second patient had severe pulmonary fibrosis of autoimmune nature and died owing to a pulmonary infection. In the second patient, a sulfasalazine reaction as an etiologic factor was excluded, while in the first this possibility seemed unlikely. Therefore, we take these two cases as examples of the extraintestinal manifestations of ulcerative colitis.

Adolescent

Changes in ventilatory muscle function with negative pressure ventilation in patients with severe COPD.

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.

Aged

[Pulmonary lymphangioleiomyomatosis. Radiologic aspects and the diagnostic role of computed tomography].

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.

Adult

Treatment of obese patients with obstructive sleep apnea syndrome (OSAS): effect of weight loss and interference of otorhinolaryngoiatric pathology.

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.

Adult

Sleep-related breathing impairment in myotonic dystrophy.

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.

Adult