Idiopathic pulmonary haemosiderosis in a child with Down's syndrome: case report and review of the literature.

We report the case of a female child with Down's syndrome affected by idiopathic pulmonary haemosiderosis (IPH), who was successfully treated with hydroxychloroquine. First-line conventional treatment of IPH is traditionally based on systemic corticosteroids; however, many steroid-sparing agents are being increasingly used as adjuncts to corticosteroids in children with recurrent or refractory bleeding. The use of these drugs is particularly promising for maintenance treatment, because it tends to avoid the adverse effects of long-term corticosteroids.

High frequency dynamics in liquid nickel: An inelastic x-ray scattering study.

Owing to their large relatively thermal conductivity, peculiar, nonhydrodynamic features are expected to characterize the acousticlike excitations observed in liquid metals. We report here an experimental study of collective modes in molten nickel, a case of exceptional geophysical interest for its relevance in earth interior science. Our result shed light on previously reported contrasting evidences: In the explored energy-momentum region, no deviation from the generalized hydrodynamic picture describing nonconductive fluids is observed. Implications for high frequency transport properties in metallic fluids are discussed.

Bronchiolitis obliterans-organizing pneumonia: an Italian experience.

The purpose of this study was to describe the clinical features at onset and outcome and the diagnostic approach in subjects with bronchiolitis obliterans-organizing pneumonia (BOOP). Over a 7-year period we observed 78 cases of biopsy-proven bronchiolitis obliterans-organizing pneumonia, in which well documented clinical and radiographic data were available. The final diagnosis of BOOP was validated when patients presented: (i) negative microbiological analysis on BAL fluid; (ii) a well documented improvement either spontaneous, or after steroid treatment or (iii) cases with progressive respiratory failure and increasing radiographic shadows, an open lung biopsy or autopsy that excluded other entities. There were 42 males and 36 females; the mean age was 61+/-12 years (range 12-85 years). Forty-two (54%) patients were current smokers, 25 (32%) had never smoked and 11 (14%) were ex-smokers. The clinical pattern at presentation of BOOP was more frequently similar to classical acute or sub-acute infectious pneumonia. Fever (63%), dyspnoea (58%) and dry cough (53%) were the typical symptoms on admission. A flu-like syndrome preceeding BOOP was observed in 21 cases (27%). Inspiratory crackles (78%) were the most typical finding at physical examination. However, 13% of the patients were asymptomatic and an abnormal chest X-ray film was the reason for seeking medical attention. Radiographically the most frequent pattern of BOOP was a unilateral consolidation (44%) with lower field predominance. A migratory behaviour was present in 22% of the cases. High-resolution computed tomographic (HR-CT) scan when performed, was more sensitive in detecting ground glass infiltrates, sub-pleural or peri-bronchovascular distribution or the presence of nodules or cavitation. Most patients (68%) were classified as having idiopathic BOOP. However, the same clinical-roentgenological pattern was observed in patients after radiotherapy for ductal breast carcinoma (6%), in collagen-vascular diseases (6%), related to drugs (9%), to infections serologically documented (4%), and to graft vs. host disease (4%). Four patients (all of whom had idiopathic BOOP) presented a rapid progressive respiratory failure needing mechanical ventilation. In another two cases respiratory failure appeared after a long period during which patients experienced exertional dyspnoea and low grade fever. BAL profile was characterized by lymphocytosis with a reduction of the CD4/CD8 ratio, associated with a slight increase of neutrophils and eosinophils and scattered mast cells. However in two cases we had an increased CD4/CD8 ratio and in one case the presence of a significant 12% of polyclonal B cells. In a few cases atypical (cytokeratin-positive cells) epithelial cells were detected: these cells were constantly present in the BAL fluid of patients with rapidly progressive respiratory failure. From the diagnostic point of view this series documents that transbronchial lung biopsy (coupled with BAL) can be the first diagnostic step. However, therapy can be started on the basis of BAL data (when a characteristic morphological and phenotypical profile is evident) in cases in which the clinical presentation is suggestive and a biopsy cannot be made. Most patients showed a rapid and good response to steroid therapy. However, three patients died (4%) in spite of steroid therapy (two cases) and steroid and cyclophosphamide therapy (one case). In conclusion, although clinical findings, chest X-ray film and CT Scan findings usually suggest the diagnosis a definite confirmation requires transbronchial lung biopsy and BAL and, less frequently, open lung biopsy.

Bronchoalveolar lavage and transbronchial lung biopsy in alveolar and/or ground-glass opacification.

In order to assess the diagnostic yield of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) in pulmonary diseases with a ground-glass and/or alveolar pattern on high-resolution computed tomography (HRCT) scan, a prospective study was undertaken. Thirty-six patients (17 males, 19 females), mean age 53 yrs, selected on the basis of the presence of an alveolar and/or ground-glass pattern on chest HRCT scan, were submitted to fibreoptic bronchoscopy. All patients underwent BAL. TBBs were performed in 33 cases. A specific diagnosis was achieved, taking into account data obtained by means of serology, microbiology, cytology and histopathology in appropriate clinical settings. Twelve (33%) patients only had the appearance of a ground-glass opacity, whereas 24 (67%) had associated areas of airspace consolidation. BAL was performed in all cases and gave a definitive diagnosis in 21 (58%). The diagnostic yield of BAL in patients with only ground-glass opacities was no different from that in those patients also showing areas of alveolar consolidation (58 versus 58%). In eight patients (six with ground-glass opacity and two with alveolar consolidation), BAL provided useful but not definitive information. In these patients, a definitive diagnosis was achieved by means of TBB in seven cases and by open lung biopsy in one case. TBB was performed in 33 out of 36 patients and gave positive results in 25 (76%). The diagnostic yield of TBB in patients showing areas of alveolar consolidation was significantly higher than in those with pure ground-glass opacity, i.e. 95% (21 of 22) and 36% (4 of 11) respectively (p < 0.001). BAL and TBB were performed during the same bronchoscopy in 33 patients, and an accurate diagnosis was achieved in 30 (91%). Overall, the diagnostic yield of TBB (76%) and BAL (56%) did not differ significantly in the whole patient group (p = 0.12), or in patients with a ground-glass opacification (58 versus 36%, p = 0.3). However, in patients with areas of alveolar consolidation, the diagnostic sensitivity of TBB (95%) was significantly greater than the diagnostic sensitivity of BAL (54%) (p = 0.03). In conclusion this study shows that high-resolution computed tomography can be helpful in predicting the diagnostic accuracy of bronchological procedures, in particular of bronchoalveolar lavage and transbronchial biopsy, and that alveolar and/or ground-glass are favourable patterns for these diagnostic tools.

A rare association of inlet patch with laryngospasm: a report of two children and literature review.

The inlet patch (IP) is an area of heterotopic gastric mucosa in the cervical esophagus commonly seen during upper endoscopy. Although generally asymptomatic IP has been associated with esophageal and supraesophageal symptoms and, though rare, in adult with malignant transformation. We describe two cases of recurrent episodes of laryngospasm associated with IP. In both cases there was a good response to prolonged acid suppression therapy.

Swyer-James syndrome: bronchoalveolar lavage findings in two patients.

Swyer-James syndrome (SJS) is a rare constrictive bronchiolitis that appears to be the result of acute bronchiolitis in infancy or early childhood. In the present study the cytological and immunophenotypical profile of bronchoalveolar lavage (BAL) was studied in two patients with SJS who showed a different spectrum of clinical outcome. The total BAL yield was markedly increased in the patient with chronic cough and acute episodes of dyspnoea but not in the patient with decreased exercise tolerance and longer duration of disease. In the two patients, the differential cell counts in percentage were characterized by a significant increase of neutrophils and a slight increase of lymphocytes. The analysis of lymphocyte subsets showed a significant increase of CD8+ cells (T-suppressor-cytotoxic) in both cases, resulting in a decreased CD4/CD8 ratio. In addition, an increase of cells bearing a B-phenotype (CD19+ cells) was noted in the fluid recovered from the patient with chronic cough and acute episodes of dyspnoea. In conclusion, our data suggest that SJS is an active process with inflammatory characteristics. Further studies are needed to explain the mechanism leading to the expansion in situ of immunocompetent cells.

The diagnostic value of bronchoalveolar lavage and transbronchial lung biopsy in cryptogenic organizing pneumonia.

In order to determine the diagnostic value of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) in cryptogenic organizing pneumonia (COP) a prospective study was carried out. Thirty seven consecutive patients (20 males and 17 females) with clinicoradiological features of COP were enrolled in the study. The statistical analyses were completed in 35 cases. Twenty eight patients were diagnosed to have COP, all of them with a confirmatory biopsy. In seven cases, a different diagnosis was made. BAL cytological and phenotypical criteria considered for the diagnosis of COP were: a lymphocytosis of more than 25% (with a CD4/CD8 ratio less than 0.9); combined with at least two of the following data (foamy macrophages of > 20%, and/or neutrophils of > 5%, and/or eosinophils of > 2% and < 25%). TBLB specimens were classified as positive for COP if they showed: buds of granulation tissue within the centrilobular air spaces; infiltration of alveolar walls with chronic inflammatory cells; and preservation of alveolar architecture. BAL was performed in 34 patients; 17 cases were consistent with the final diagnosis of COP (sensitivity 63%), and four cases were correctly classified as negative (specificity 57%). BAL had a positive predictive value (PPV) of 85% and a negative predictive value (NPV) of 29%. TBLB was performed in 32 patients; it correctly identified COP in 16 cases (sensitivity 64%), and six cases were correctly classified as negative (specificity 86%). TBLB had a PPV of 94% and a NPV of 40%. The accuracy of the examinations, that is the probability of correctly diagnosing both diseased and nondiseased patients by BAL or TBLB, was 62 and 69%, respectively. Our findings suggest that the combination of cytological bronchoalveolar lavage and histological transbronchial lung biopsy data obtained during a fibreoptic procedure appears to be an effective method for the initial investigation in cryptogenic organizing patients pneumonia presenting with patchy radiographic shadows.

Pulmonary function abnormalities in children with Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is a widespread necrotizing vasculitis affecting small vessels characterized by nonthrombocytopenic purpura. Pulmonary involvement is a rare fatal complication with diffuse alveolar haemorrhage. The objective of this study was to evaluate possible early lung function abnormalities and to establish any relationship with the clinical activity of the disease. Fifteen children with HSP and without clinical or radiological evidence of lung involvement underwent pulmonary function study at the onset of the disease. A sample of 28 subjects matched by age, height, and weight was chosen as a control group. After a mean of 21 months (range 12-43) lung function tests were repeated in 10 of the previously studied children. During the acute phase of the disease the transfer factor for carbon monoxide, measured by steady-state (TL,COss) and single-breath (TL,COsb) methods, was found to be significantly lower in children with HSP than control subjects. There was no significant relationship between pulmonary function tests with symptoms and signs at onset, nor was there any correlation between variables and serum immunoglobulin A (IgA) concentration. In all but two patients, clinical recovery was observed within 6 weeks from the onset of the disease. In one case relapses of purpuric skin lesions were observed during the first 3 months of follow-up. The second case had relapses of purpuric skin lesions and microscopical haematuria during the 12 months following the onset of the disease with characteristic IgA mesangial deposition on renal biopsy. Although the overall mean value of TL,COsb improved from baseline to the second investigation, in both patients the recurrences of clinical signs were associated with a slight impairment of TL,COsb at the second evaluation. These data suggest an early subclinical lung impairment in children with Henoch-Schönlein purpura during the active phase of the disease. The presence of isolated pulmonary function abnormalities was not associated with the subsequent development of lung disease.