Microbiological data of cystic fibrosis patients in a tertiary care center in Saudi Arabia

To determine the microbiological patterns of cystic fibrosis patients in a tertiary care center in Saudi Arabia and their relation to mortality. Material and Aretrospective review of records of all confirmed Cystic Fibrosis [CF] patients followed up during the period November 1992 - November 1998. Of 96 CF patients, 81 [84%] patients are alive, whereas 15 [16%] have died. Fifty patients [52%] were male and 46 [48%], female. The most common bacteria that were grown from the first culture samples were: Pseudomonas aeruginosa in 42 [44%] patients, Hemophilus influenzae in 16 [17%], Staphylococcus aureus in 15 [15%], Streptococcus pneumoniae in 6 [6%], methicillin resistant Staphylococcus aureus [MRSA] in 4 [4%], and Branhamella catarrhalis in 6 [6%]. Follow up culture after 1-6 months of treatment showed an increase of Branhamella catarrhalis culture to 13 [13%] [p=0.03], and MRSA to 6 [6%] [p = 0.02]. Patients in whom Pseudomonas aeruginosa developed resistance to gentamicin, had the shortest lifespan [2 +/- 3 years] after initial diagnosis. The most important factor that was significantly related to early mortality in CF patients was the early development of antibiotic resistance [p value < 0.01]. Early development of Pseudomonasaeruginosa colonization and of other multi-resistant organisms, contributes significantly to early mortality. Cohort isolation should be encouraged in CF centers. Early treatment of chronic Pseudomonas aeruginosa colonization should be adopted to improve survival

Sickle cell disease and cystic fibrosis

Simultaneous occurrence of both cystic fibrosis and sickle cell disease in the same patient is a rare phenomenon and has occurred only in 3 reports in the world literature. This phenomenon has never been described before in Arab population. In this report, we describe the first reported case of both diseases in one patient in the Arab population, and discuss its complications and review the literature in this aspect

Pediatric scoliosis and the lung

To determine the pattern of scoliosis in the pediatric age group and to determine the course of the disease and the result of surgical intervention. A retrospective review of all charts of patients referred to the Pulmonary Clinic of the King Faisal Specialist Hospital, Riyadh, Kingdom of Saudi Arabia [KSA], for scoliosis and preoperative evaluation from the period November 1992 to October 2001. Demographic and clinical data, preoperative and postoperative pulmonary function test [PFT] and effect of surgery on the lung were presented. A total of 66 patients with scoliosis were followed. The age at diagnosis was 8.5 +/- 4.4 years. Thirty-four [52%] males and 32 [48%] females. Fifty-seven [86%] were alive and 9 [14%] died. A period of follow up was 5.3 +/- 3.7 years. Idiopathic scoliosis was found in 20 patients [30%], congenital anomalies associated with scoliosis constituted 35 [53%] of the total scoliosis referral and secondary type due to trauma or malignancy formed 11 [17%]. Mean Cobb s angle of 70 +/- 30 degrees. The most common curvatures were: thoracic 31 [47%], lumbar 12 [18%] and thoracolumbar 30 [45%]. Thirteen patients [20%] had 2 or 3 types of curvatures simultaneously. Baseline PFT showed moderate restrictive lung disease in approximately two thirds of the patient population. Scoliosis surgery was carried out in 34 [52%] of the patients. Posterior approach was more commonly used in 30 [88%] of the patients. Pneumonia and atelectasis were the most common complication encountered in 15 [44%] of the patients. Recurrence of scoliosis after operation recurred in 12 [35%] of the population. Comparisons between both groups have shown that patients with congenital anomalies and scoliosis were diagnosed earlier, had significant postoperative complications and higher mortality compared to those with idiopathic scoliosis [p<0.05]. Changes in PFT for patients who underwent surgery for scoliosis with Cobb s angle >40 degrees showed less deterioration compared to those with angle <40 degrees [p=0.004], due to progressive deterioration without surgery. Scoliosis is a common problem in KSA. Patients need to be referred early for proper intervention before it becomes a severe magnitude and difficult to be fixed and to prevent permanent complications. Patients with congenital anomalies and scoliosis had poor prognosis compared to those with idiopathic scoliosis

Diabetes mellitus and cystic fibrosis in 2 Saudi siblings

Diabetes mellitus and cystic fibrosis [CF] have been reported before in the literature, but they have never been reported in the same patient in the Middle East. We present the first reported case of insulin dependent diabetes mellitus [IDDM] and CF in 2 siblings of the same family. Both siblings were diagnosed early in life with IDDM, and their diabetes was well controlled on insulin. Cystic fibrosis was diagnosed in the first case one year after IDDM was diagnosed due to history of chronic cough and in the 2nd case by family screening. Both had severe failure to thrive, recurrent chest infections and gastro-esophageal reflux. With treatment both showed clinical improvement, but continued to have moderate lung disease radiologically and by pulmonary function test

Spontaneous regression of pulmonary Lymphangion-Hemangiomatosis / Lymphangio-Haemangiomatosis

Pulmonary capillary haemangiomatosis and pulmonary lymphangiomatosis are both rare and are usually fatal disorders. The combined disorder has not been described before. In this report, we describe a case of combined haemangiomatosis of the chest wall and pulmonary lymphangiomatosis with involvement of the left clavicle, humerus and scapula and spontaneous resolution of lung changes