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Background: The rare monogenic syndrome Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) leads to multisystemic autoimmunity with possible lung involvement. Autoimmune pneumonitis is a rare manifestation, with bronchiectasis being the most frequent radiologic pattern, and may lead to fatal outcome. The Sardinian population in Italy has a high incidence of APECED, although no case of lung manifestation has been reported yet in this cohort. This is the case of a Sardinian APECED patient referred to a bronchiectasis clinic. Our aim is to raise awareness and screen these patients earlier for pulmonary involvement and to initiate multidisciplinary treatment for better outcome.
Case Presentation: A 49-year-old female native of Sardinia from consanguineous parents was diagnosed with APECED in childhood and was referred to our bronchiectasis clinic in March 2023. In addition to typical APECED features, she reported recurrent respiratory infections since childhood, chronic purulent sputum and a hospitalization for pneumonia. She came to our attention with a recent isolation of P. aeruginosa on sputum culture and diffuse cylindrical and varicoid bronchiectasis on her first CT scan. She underwent aetiologic screening for bronchiectasis with no evidence of another cause of disease. Lung treatment was optimized according to bronchiectasis guidelines, and during follow-up the patient developed methicillin-resistant Staphylococcus aureus (MRSA) infection and M. intracellulare pulmonary disease. The patient was offered P. aeruginosa eradication treatment with intravenous antibiotics and initiation of antimycobacterial therapy.
Conclusion: This is the first documented lung involvement case of APECED in a Sardinian patient, and the first patient reported to enter a bronchiectasis program. The patient was prescribed lung imaging late in time when bronchiectasis complications were already present. Our case report highlights the need for early pulmonary screening and multidisciplinary management in patients with APECED.
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http://dx.doi.org/10.1186/s12890-024-03149-9 | DOI Listing |
Expert Rev Respir Med
December 2024
Division of Thoracic Surgery, Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Introduction: Situs inversus is a rare congenital condition where the organs in the chest and abdomen are reversed, thus complicating surgeries such as lung transplantation. Kartagener syndrome (KS), associated with situs inversus, includes chronic sinusitis and bronchiectasis, which can progress to end-stage lung disease requiring transplantation. This review discusses the unique surgical considerations, technical challenges, and outcomes of lung transplantation in patients with situs inversus, particularly KS.
View Article and Find Full Text PDFCureus
November 2024
Pediatric Surgery, Mohammed VI University Hospital, Oujda, MAR.
Primary pulmonary abscess is a rare but serious localized bacterial infection of the lung parenchyma, occurring without prior lung conditions like bronchiectasis or necrotizing pneumonia. We report the case of an 11-month-old child with a 22-day history of productive cough and fever, unresponsive to initial antibiotics. Clinical examination showed a stable, eupneic child with mild fever and reduced oxygen saturation.
View Article and Find Full Text PDFRespir Med
December 2024
New York City Health & Hospitals, Woodhull; NYU Grossman School of Medicine, Division of Pulmonary Medicine, New York City Health and Hospitals, Woodhull, 760 Broadway, 8(th) Floor, Brooklyn, NY 11206, USA. Electronic address:
Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly is an uncommon disease of the central airways. It is characterized by pathological dilatation of the trachea and main bronchi and inevitably leads to recurrent respiratory infections, bronchiectasis, hospitalizations, and results in considerable morbidity and mortality. Despite numerous case reports, there is a shortage of evidence on clinical outcomes and limited data on interventions, thus presenting a significant gap in the literature.
View Article and Find Full Text PDFNon-tuberculous Mycobacterial Pulmonary Disease (NTM-PD) is a chronic disease characterised by progressive inflammatory lung damage due to infection by non-tuberculous mycobacteria (NTM). Global prevalence of NTM-PD is generally low but is rising, likely due to a combination of increased surveillance, increasing multimorbidity and improved diagnostic techniques. Most disease is caused by Mycobacterium avium complex species.
View Article and Find Full Text PDFRespir Investig
December 2024
Department of Pulmonology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, Kanagawa, 236-0004, Japan.
Background: No previous studies have compared respiratory clinics and respiratory specialized facilities regarding causative diseases for bloody sputum and hemoptysis in Japan.
Methods: We retrospectively compared causative diseases for bloody sputum and hemoptysis between 3 respiratory clinics (clinic group) and 7 departments of respiratory medicine at hospitals (hospital group) in Japan.
Results: We collected data from 231 patients (median age, 51 years; age range, 24-96 years; 109 men (47.
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