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Modaresi, Mohammadreza, Jalalimanesh, Nasrin, Rahmani, Shaghayegh. (1402). Primary Ciliary Dyskinesia Registry. , 12(1), 29-36. doi: 10.22038/psj.2024.75409.1413
Mohammadreza Modaresi; Nasrin Jalalimanesh; Shaghayegh Rahmani. "Primary Ciliary Dyskinesia Registry". , 12, 1, 1402, 29-36. doi: 10.22038/psj.2024.75409.1413
Modaresi, Mohammadreza, Jalalimanesh, Nasrin, Rahmani, Shaghayegh. (1402). 'Primary Ciliary Dyskinesia Registry', , 12(1), pp. 29-36. doi: 10.22038/psj.2024.75409.1413
Modaresi, Mohammadreza, Jalalimanesh, Nasrin, Rahmani, Shaghayegh. Primary Ciliary Dyskinesia Registry. , 1402; 12(1): 29-36. doi: 10.22038/psj.2024.75409.1413

Primary Ciliary Dyskinesia Registry

Journal of Patient Safety & Quality Improvement
مقاله 4، دوره 12، شماره 1، فروردین 2024، صفحه 29-36    اصل مقاله (395.1 K)
نوع مقاله: Original Article
شناسه دیجیتال (DOI): 10.22038/psj.2024.75409.1413
نویسندگان
Mohammadreza Modaresi1؛ Nasrin Jalalimanesh1؛ Shaghayegh Rahmani* 2
1Pediatric Pulmonary Disease and Sleep Medicine Research Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran.
2Clinical Research Development Center, 22 Bahman Hospital, Faculty of Medicine, Mashhad Medical Sciences, Islamic Azad University, Mashhad, Iran.
چکیده
Introduction:
Primary ciliary dyskinesia (PCD) is a rare genetic disease that is estimated to occur in about 1 in 15,000 people. A patient registry is a well-known tool for collecting a sufficient number of patients with a rare disease to evaluate and monitor the patient’s information in a standard and continuous way, as well as to conduct clinical research on the disease, which can be used for early diagnosis. Standard treatment and follow-up of PCD patients will help us. In this study, we developed a PCD registry for the Iranian population.
 Materials and Methods:
In this study, for the first time in Iran, the PCD Registry was presented to record demographic information, clinical diagnostic symptoms, diagnosis method, management, and follow-up of patients called IPOLD (Iranian Pediatric Orphan Lung Disease). This PCD Registry can be used in all provinces of Iran, and a network of PCD treatment centers can be established. Two hundred fifty-six patients diagnosed with PCD, regarding demographic information, diagnostic clinical symptoms, disease diagnosis method, imaging, spirometry, and microbiological findings, were referred to university hospitals and clinics covered by the Tehran University of Medical Sciences from April 1401 to April 1402. We used SPSS to analyze data and performed descriptive tests.
 Results:
In this study, the average age of disease diagnosis was 5.7 years. The most common diagnostic symptom of the patients was chronic cough, with the belief of 94.3%. 56.7% of patients with distress were hospitalized and hospitalized in infancy. Bronchiectasis was seen in CXR or Chest CT in 28 patients (11%), and the severity of bronchiectasis was evaluated using the Bronchiectasis Severity Index (BSI); 13 cases have mild bronchiectasis (46%) and 15 moderate cases (53%). Atelectasis was seen in 47% of patients, with the predominance of RML involvement in 35%. Lung infiltration was reported in 15% of patients. PCD diagnosis method in 46 patients was based on PICADAR clinical diagnosis, 173 patients by nasal nitric oxide test, 31 by genetic test, and six by TEM.
 Conclusion:
The patients’ information was registered in the registration system, IPOLD, Iran, for children's orphan lung disease.
کلیدواژه‌ها
Children؛ Primary ciliary dyskinesia؛ Registry
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