[Occurrence pulmonary tuberculosis in a patient with Bernard soulier syndrome: a case report]

Bernard soulier syndrome [BSS] is a rare congenital bleeding disorder characterized by thrombocytopenia. BSS causes bruising, epistaxis, gingival bleeding, menorrhagia, post partum bleeding, gastrointestinal bleeding and post traumatic hemorrhage, but there is no report of hemoptysis in BSS. A 14 year-old girl was referred to our center due to massive hemoptysis. Her chest x-ray revealed complete collapse of the right lung. Rigid bronchoscopy was preformed and the intrabronchial clots were removed. Smear and culture of direct sputum was positive for Mycobacterium tuberculosis. She received anti-tuberculosis treatment. During treatment, she developed a massive vaginal bleeding, because of drug interaction between rifampin and low dose contraceptive [LD], which she had been taking for control of massive menstrual bleeding. Her vaginal bleeding was controlled by platelet infusion and recombinant factor 7 infusion. After 2 months of anti-TB treatment, sputum smear and culture for BK converted to negative. One year after treatment, the pulmonary tuberculosis was completely cured and no hemoptysis occurred. When hemoptysis occurs in patients with Bernard soulier syndrome, we should consider other differential diagnoses, and further diagnostic evaluation is recommended

correlation of brody high resolution computed tomography scoring system with clinical status and pulmonary function test in patients with cystic fibrosis

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