Serena Panigada

A longitudinal analysis of physical functional disability over the course of juvenile idiopathic arthritis.

Objective: To describe the longitudinal course of physical functioning in children with juvenile idiopathic arthritis (JIA) and identify predictors of long-term functional impairment. Methods: Between January 1987 and December 2002, 227 patients had two or more functional ability questionnaires completed by a parent. The total number of questionnaires was 1356 and the follow-up between first and last questionnaire administration was 949.7 patient years. At each questionnaire administration, patients were assigned to one of three functional disability states (1 = no disability; 2 = mild to moderate disability; 3 = severe disability), based on their functional ability score. Predictor variables included sex, onset age, JIA category, age at visit, disease duration, presence of antinuclear antibodies, joint counts, acute phase reactants and initial disability state. Results: Despite patient variability in the course of physical functioning, the following three longitudinal patterns were observed: (1) a stable state of disability throughout the entire study period, with continued absence of disability in 27.8% of patients and persistently moderate disability in 3.5% of patients; (2) a steady improvement (22.9% of patients) or deterioration (5.7% of patients) in disability over time; (3) a fluctuating course of disability, with deterioration and improvement (40.1% of patients). Younger age at disease onset and a greater restricted joint count were the strongest predictors of long-term functional impairment. Conclusion: A wide within-patient and between-patient variability in the longitudinal course of functional disability was found. Children with early disease onset and a greater number of restricted joints had the highest risk of developing long-term physical disability.

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

To investigate the role of high‐resolution computed tomography (HRCT) and pulmonary function tests (PFTs) in staging pulmonary involvement in juvenile systemic sclerosis (JSS).

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. Pediatr Pulmonol. 2009; 44:192–194.

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. Pediatr Pulmonol. 2012. 47:831–835.

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. Pediatr Pulmonol. 2009; 44:244–248.

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. Pediatr Pulmonol. 2016;51:286–294.

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. Pediatr Pulmonol. 2014; 49:E109–E111.

Bilobar atelectasis as clinical presentation of Mycoplasma pneumoniae infection

A wide variety of radiographic findings have been attributed to Mycoplasma pneumoniae infections. To our knowledge, the occurrence of atelectasis involving one or more lobes as a presenting feature of M. pneumoniae infection and requiring fiberoptic bronchoscopy for resolution has never been reported. A 4-year-old girl with a 3 day history of low-grade fever (<38.0°C), and cough was admitted for progressive dyspnea. Mild respiratory distress (SaO2 94%) and reduced vesicular sounds over the lower right lung were found, with slight elevation of white blood cells (13 480 cells/mL; 74% neutrophils) and of C-reactive protein (1.14 mg/dL; normal range, <0.46 mg/dL). Chest X-ray demonstrated right middle and lower lobe atelectasis (Fig. 1a). To detect respiratory pathogens, real-time polymerase chain reaction (PCR) was performed on oropharyngeal swab, which showed a strong positivity only for the P1 cytoadhesin type 1 and 2 gene of the M. pneumoniae genome. Elevated specific serum IgM titers were detected (59 U/mL; normal range, 217 U/mL). The patient was then treated with nebulized glucocorticoids plus albuterol, chlaritromycin and chest physiotherapy. On hospital day 4, the clinical condition, including the mild respiratory distress, and the physical examination were unchanged. Computed tomography (Fig. 1b,c) confirmed the

Metalloproteolytic balance in asthma: When and how could its regulation?

Matrix metalloproteinases (MMPs) are a family of secreted and cell surface-bound neutral proteinases, active on a large array of extracellular and cell surface proteins under normal and pathological conditions. First described as proteases that can degrade extracellular matrix (ECM) components, MMPs may also act on a variety of extracellular substrates to activate latent forms of mediators, such as antimicrobial peptides, cytokines and growth factors, or alter protein functions, such as shedding of cell-surface molecules. In the lung, as in other organs, MMPs are produced by airway epithelial and mesenchymal cells, but also by a variety of inflammatory cells and their synthesis is tightly regulated, via multiple activators and repressors pathways, by hormones, cytokines, cell adhesion molecules and a variety of environmental factors. Expressed and secreted in inactive form, MMPs undergo multistep activation and inactivation processes in the extracellular milieu, the most important endogenous inhibitors being the ‘‘tissue inhibitors of matrix metalloproteinases’’ (TIMPs). In health and disease, a correct balance between MMPs and TIMPs may prevent an exaggerated ECM deposition or an alteration in its composition, but may also regulate the activation and the cleavage of a variety of molecules involved in wound healing, inflammation and immunity and host defense against pathogens. Imbalance in MMPs/ TIMPs ratio has been detected in the acute and/or the chronic phases of a variety of respiratory disorders, including chronic obstructive pulmonary disease and asthma. In asthma, inappropriate expression and secretion of MMP-2, MMP-3 and MMP-9 and the ADAM family were reported and thought to be part of the physiology of this disorder. Most of the studies performed have been focused on the presence and/or the activity of MMP-9 (also called gelatinase B), a protease found to be highly expressed in the airways of asthmatic patients, compared with normal individuals, and specifically in patients with severe disease or during spontaneous asthma exacerbations. In agreement