ABSTRACT
To reduce the mortality and morbidity rates of cystic fibrosis [CF] patients, and to have an effective clinical management, it is important to monitor the progression of the disease. The aim of this study was to evaluate the progression of lung disease in CF patients by means of assessing the correlation of the CT scoring system with clinical status and pulmonary function test at the Pediatric Pulmonary Ward of Masih Daneshvari Hospital in 2008. Pulmonary high resolution computed tomography [HRCT] was performed in 23 CF patients using the Brody's scoring system. Morphologic signs as well as the extent and severity of each sign were scored, and the total sore was calculated. The correlation of HRCT scores[total score as well as the score for each parameter] with Shwachman Kuczycki scoring system and pulmonary function test were examined. The study included 9 female and 14 male patients with an age range of 5-23 years [mean: 13.42 years]. Bronchiectasis [100%] and peribronchial wall thickening [100%] were the most frequent CT abnormalities. Mucus plugging, air trapping and parenchymal involvements were respectively seen in 95.7%, 91.3% and 47.8% of patients. The overall CT score for all patients was 57.6 +/- 24.2 [means +/- SD]. The results of pulmonary function test showed a restrictive pattern; however, in 5.3% of the patients PFT was normal. The overall Shwachman-Kulczycki score was 53.48 +/- 13.8. There was a significantly [P=0.015] negative correlation between the total CT score and Shwachman-Kulczycki score; however, there was no significant correlation between total CT score and the results of PFT [P=0.481]. The Brody's scoring system for high resolution computed tomography seems to be a sensitive and efficient method to evaluate the progression of CF, and can be more reliable when we combine the CT scores with clinical parameters
ABSTRACT
Chronic granulomatous disease [CGD] is a rare disorder of phagocytes, predisposes patients to bacterial and fungal infections. The main purpose of this study was to determine the clinical, radiological, pathologicial features, outcome and response to treatment of children with CGD. Thirteen patients with CGD, who had been referred to National Research Institute of Tuberculosis and Lung Disease [NRITLD], were reviewed during a 6 year period [1999-2005]. There were 10 [76%] male and 3[24%] female cases. The median age of the patients was 9 years [1 month-12 years].Family history of CGD was reported by 7 patients. The median diagnostic age was 8 years, with a diagnostic delay of 4.5 years. The most common manifestations of CGD were pulmonary infections and skin involvement, followed by generalized lymphadenopathy. The most common radiological findings were multiple lymphadenopathy in mediastinal region and fibrotic changes in lung fields. Two patients died of pulmonary infections. Based on the results of this research, immunologic evaluations especially evaluation for CGD is highly recommended in children suffering from recurrent pulmonary infections, cutaneous or hepatic abscesses, or infections caused by uncommon pathogens. Early diagnosis and prophylactic treatment both, prevent further development of the lesions, irreversible complications and decreasing mortality and morbidity rates in children suffering from CGD