Occupation and first episode psychosis in Northern Italy: better outcomes for migrants.

AIMS: Many studies show that migrants have a higher incidence of psychosis compared to natives, but the influence of migration on psychosis outcomes is little investigated. We aimed to evaluate the occupational outcomes of a first episode psychosis (FEP) sample in Bologna (Northern Italy). METHODS: An incidence cohort of FEP patients presenting at the Bologna West Community Mental Health Centers between 2002 and 2009 was assessed at the baseline and at 12th month follow-up. Return to school or work was used as occupational outcome. RESULTS: Most of the patients (82.8%) were still in contact at 12 months. Migrants showed significantly higher rate of return to work compared to natives (adjusted OR 4.45, 95% CI 1.55-12.76). CONCLUSIONS: First generation migrants had better occupational outcomes. Further cross-cultural studies are needed to further explain these findings.

Mild tracheal compression by aberrant innominate artery and chronic dry cough in children.

BACKGROUND: In children with aberrant innominate artery (AIA) one of the most prevalent respiratory symptom is dry cough. How frequently this mediastinal vessels anomaly, that can induce tracheal compression (TC) of different degree, may be detected in children with chronic dry cough is not known. METHODS: In a 3-year retrospective study, the occurrence of mediastinal vessels abnormalities and the presence and degree of TC was evaluated in children with recurrent/chronic dry cough. RESULTS: Vascular anomalies were detected in 68 out of the 209 children evaluated. A significant TC was detected in 54 children with AIA, in eight with right aortic arch, in four with double aortic arch but not in two with aberrant right subclavian artery. In AIA patients, TC evaluated on computed tomography scans, was mild in 47, moderate in six and severe in one. During bronchoscopy TC increased in expiration or during cough, but this finding was more pronounced in children with right aortic arch and double aortic arch in which a concomitant tracheomalacia was more evident. Comorbidities were detected in 21 AIA patients, including atopy, reversible bronchial obstruction and gastroesophageal reflux. Aortopexy was performed in eight AIA patients, while the remaining AIA patients were managed medically and showed progressive improvement with time. CONCLUSION: Mild TC induced by AIA can be detected in a sizeable proportion of children with recurrent/chronic dry cough. The identification of this anomaly, that may at least partially explain the origin of their symptom, may avoid further unnecessary diagnostic examinations and ineffective chronic treatments.

Phenotypic and genetic characterization of a patient with a de novo interstitial 14q24.1q24.3 deletion.

BACKGROUND: Interstitial deletions of chromosome bands 14q24.1q24.3 are very rare with only three reported cases. RESULTS: We describe a 7-year-old boy with a 5.345 Mb de novo interstitial deletion at 14q24.1q24.3 band detected by array-CGH who had a complex phenotype characterized by seizures, congenital heart defects, dysmorphisms, psychomotor delay, and bronchopulmonary, skeletal, and brain anomalies. CONCLUSION: The deleted region contains numerous genes, but we focused our attention on three of them (C14orf169, NUMB, and PSEN1), which could account, at least partially, for the phenotype of the boy. We therefore discuss the involvement of these genes and the observed phenotype compared to that of previously described patients.

Corticosteroids may favor proliferation of thoracic inflammatory myofibroblastic tumors.

Inflammatory myofibroblastic tumor (IMT) was thought to represent a benign post-infectious or post-inflammatory process cured by surgical resection. However, reports of cases with an aggressive clinical course suggest the need for caution about the prognosis. The treatment of choice is a complete surgical resection, while medical treatment options are limited. Corticosteroid therapy has been used with some success in unresectable lesion. However, rapid progression of lung IMT after prednisone treatment has been reported, raising the hypothesis that corticosteroids may favor a tumultuous proliferation of this lesion, possibly through immunosuppression. We here report a similar observation and suggest that other mechanisms may be involved. A 5-year and 6-month-old boy presented with a 72 hr history of breathlessness, initially responsive to albuterol and prednisone. He represented 15 days later with increasing symptoms despite further prednisone treatment. CT chest scan showed a mass lesion in the tracheal lumen, which on biopsy was found to be an IMT. The possibility that prednisone may have an enhancing effect on IMT cell proliferation is demonstrated through IMT cell culture and discussed.

The impact of substance use at psychosis onset on First Episode Psychosis course: results from a 1 year follow-up study in Bologna.

OBJECTIVES: Substance abuse is a well established risk factor for First-Episode Psychosis (FEP), but its influence on FEP course is less clear. Starting from our baseline observation that substance users were younger than non-users at the psychosis onset, we hypothesized that substance use at baseline could be an independent risk factor for a worse clinical course. METHODS: An incidence cohort of patients with FEP collected in an 8year period (2002-2009) at the Bologna West Community Mental Health Centers (CMHCs) was assessed at baseline and at 12month follow-up. Drop-out, hospitalizations and service utilization were used as clinical outcomes. RESULTS: Most of the patients were still in contact with CMHC at 12month follow up. Substance users had a significantly higher rate of hospitalizations during the follow-up after adjusting for age, gender and other potential confounders (OR 5.84, 95% CI 2.44-13.97, p≤0.001). CONCLUSIONS: This study adds to previous evidence showing the independent effect of substance use on FEP course. The identification of a "potentially modifiable" environmental predictor of the course of the illness such as substance use at psychosis onset allows us to envisage the possibility of ameliorating the course of the illness by managing this factor.

Necrotizing sarcoid granulomatosis of the lung in a 12-year-old boy with an atypical clinical course.

Necrotizing sarcoid granulomatosis (NSG) is a disorder of unknown etiology, rarely described in childhood, belonging to the heterogeneous group of the pulmonary angiitis and granulomatosis. One of the characteristics of NSG is to have typically a benign clinical course with minimal treatment with systemic steroids or even with no therapy at all. Here, we report the case of a boy with a lung consolidation, with morphological and histological features consistent with a diagnosis of NSG. Good clinical and roentgenographic response to high dose prednisone treatment was followed three times by relapses, when steroid treatment was tapered. New lesions were detected in different areas of the lung and not in initially affected area, never previously described in NSG and only rarely in other pulmonary angiitides.

Gaslini's tracheal team: preliminary experience after one year of paediatric airway reconstructive surgery.

BACKGROUND: Congenital and acquired airway anomalies represent a relatively common albeit challenging problem in a national tertiary care hospital. In the past, most of these patients were sent to foreign Centres because of the lack of local experience in reconstructive surgery of the paediatric airway. In 2009, a dedicated team was established at our Institute. Gaslini's Tracheal Team includes different professionals, namely anaesthetists, intensive care specialists, neonatologists, pulmonologists, radiologists, and ENT, paediatric, and cardiovascular surgeons. The aim of this project was to provide these multidisciplinary patients, at any time, with intensive care, radiological investigations, diagnostic and operative endoscopy, reconstructive surgery, ECMO or cardiopulmonary bypass. Aim of this study is to present the results of the first year of airway reconstructive surgery activity of the Tracheal Team. METHODS: Between September 2009 and December 2010, 97 patients were evaluated or treated by our Gaslini Tracheal Team. Most of them were evaluated by both rigid and flexible endoscopy. In this study we included 8 patients who underwent reconstructive surgery of the airways. Four of them were referred to our centre or previously treated surgically or endoscopically without success in other Centres. RESULTS: Eight patients required 9 surgical procedures on the airway: 4 cricotracheal resections, 2 laryngotracheoplasties, 1 tracheal resection, 1 repair of laryngeal cleft and 1 foreign body removal with cardiopulmonary bypass through anterior tracheal opening. Moreover, in 1 case secondary aortopexy was performed. All patients achieved finally good results, but two of them required two surgeries and most required endoscopic manoeuvres after surgery. The most complex cases were the ones who had already been previously treated. CONCLUSIONS: The treatment of paediatric airway anomalies requires a dedicated multidisciplinary approach and a single tertiary care Centre providing rapid access to endoscopic and surgical manoeuvres on upper and lower airways and the possibility to start immediately cardiopulmonary bypass or ECMO.The preliminary experience of the Tracheal Team shows that good results can be obtained with this multidisciplinary approach in the treatment of complicated cases. The centralization of all the cases in one or few national Centres should be considered.

Recurrent severe lower respiratory tract infections in a child with abnormal tracheal morphology.

Localized recurrent respiratory infections, leading to severe hypoxia in young children without immunological abnormalities or other risk factors, should raise the suspicion of airway structural abnormalities. In a 24-month-old boy, with recurrent severe post-viral wheezing and a history of RSV-induced bronchiolitis and gastro-esophageal reflux, fiberoptic bronchoscopy demonstrated an abnormal morphology of the distal portion of the trachea, ending in four openings. Computed tomography (CT) scans demonstrated the presence of a right tracheal bronchus and an anomalous upper lobar bronchus, originating at the level of the major carina.

Intermittent gaseous bowel distention: atypical sign of congenital tracheoesophageal fistula.

Three girls, 5-, 9-, and 15-year-old, were evaluated for recurrent airway infections and pneumonia. Chest X-rays, which included the upper portion of the abdomen, showed marked gaseous bowels distention, while computed tomography scans of the chest demonstrated the presence of tracheoesophageal fistula (TEF), confirmed by fiberoptic bronchoscopy. Abdominal gaseous distension, a known possible clinical manifestation of TEF in the neonatal period generated by airflow through the fistula into the oesophagus, has not been reported as a clue to the diagnosis in older children. When detected in patients with recurrent respiratory infection, should raise the suspicion of unrecognized TEF.

HRCT and pulmonary function tests in monitoring of lung involvement in juvenile systemic sclerosis.

OBJECTIVE: To investigate the role of high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) in staging pulmonary involvement in juvenile systemic sclerosis (JSS). METHODS: Clinical charts of 17 JSS patients (pts) were reviewed and high-resolution CT (HRCT) scans and PFTs performed at first and last visit were examined. Forced expiratory volume in 1 sec (FEV(1)), forced vital capacity (FVC) and carbon monoxide diffusing capacity [DL(CO)] were measured (% pred. values) while HRCT changes were graded according to the Schurawitzki method (min-max score: 0-18). RESULTS: At initial assessment, 10 pts (58.8%) had pulmonary involvement (HRCT score >0 and/or PFTs <80% pred. values). PTFs abnormalities were consistent with a restrictive defect, with low FEV(1), FVC and DL(CO) values and normal FEV(1)/FVC ratio. At first visit HRCT scores were inversely correlated with FEV(1) (r = -0.75; P = 0.02), FVC (r = -0.685; P = 0.035), and DL(CO) values (r = -0.71; P = 0.03), but not with the FEV(1)/FVC ratio (r = -0.36; P = 0.31). Stronger inverse correlations were detected at last visit (median time interval: 4.3 years; min-max: 1-10 years) between HRCT and FEV(1) (r = -0.86; P = 0.002), FVC (r = -0.79; P = 0.009) and DL(CO) (r = -0.79; P = 0.009), but not with the FEV(1)/FVC ratio (r = -0.33; P = 0.35). Changes in HRCT scores between first and last visit were correlated with changes in FEV(1) (r = -0.74; P = 0.02), FVC (r = -0.81; P = 0.007), but not in DL(CO) values (r = 0.07;P = 0.84). At the last visit, of the 10 pts with pulmonary involvement at initial assessment 5 showed worsening of lung involvement and 5 either no significant changes in PFTs and HRCT or mild improvement. CONCLUSIONS: PFTs, namely lung volumes, represent a reliable "monitoring" tool in children with JSS to identify pts that need to undergo an HRCT to rule out initial pulmonary involvement and to monitor the course of ILD over time. The weaker clinical value of DL(CO) may relate to poor lung function technique in some younger children.

Correlation between juvenile idiopathic arthritis activity and damage measures in early, advanced, and longstanding disease.

OBJECTIVE: To compare the correlation between juvenile idiopathic arthritis (JIA) measures of disease activity and damage in patients with early and late disease. METHODS: Three cohorts of patients with JIA disease duration < or =1 year (early disease, n = 70), 5-9.9 years (advanced disease, n = 114), and > or =10 years (longstanding disease, n = 39) were studied. Measures included physician's global assessment of overall disease activity (MD global), parent's global assessment of the child's well-being (parent global) and pain (parent pain), joint counts, Childhood Health Assessment Questionnaire (CHAQ), erythrocyte sedimentation rate, C-reactive protein level, and Poznanski score of radiographic damage. RESULTS: In all cohorts, the MD global assessment was generally well correlated with the other variables, except the Poznanski score. The parent global assessment was correlated strongly with the parent pain assessment and moderately with the CHAQ irrespective of disease duration. Correlations between the CHAQ and the joint counts were low in early disease, moderate in advanced disease, and high to moderate in longstanding disease. Correlation between the CHAQ and the Poznanski score was low in early and advanced disease and moderate in longstanding disease. The Poznanski score was highly correlated with the number of joints with restricted motion in longstanding disease. CONCLUSION: We found important differences in the level of correlation between JIA measures of activity and damage in patients with different lengths of disease duration. These findings have important implications for clinical trials because they indicate that the responsiveness of some variables and their correlation with other variables change as disease duration changes.