First report on the prevalence of bacteria in cystic fibrosis patients (CF) in a tertiary care center in Saudi Arabia.

Introduction: Bacterial infections in CF patients are common and start early in life. The prognosis of the disease is substantially dependent on chronic respiratory infection and inflammation. Pseudomonas aeruginosa (PA) infection or chronic colonization have been established to cause a chronic decline in pulmonary function (PFT), and/or increase CF mortality. Objectives: To obtain the prevalence of all bacterial pathogens in our CF patients and assess their evolution over time. Method: A retrospective review of 327 patients with confirmed CF of all age groups, who had respiratory culture samples at the first visit and on a regular follow-up between January 1, 1990 and December 2018, was conducted. Results: A total of 327 patients had a respiratory culture obtained at presentation. Two hundred and sixteen (66%) of 327 patients are alive, while 111 (34%) have died. Respiratory cultures were taken from nasopharyngeal aspiration (NPA) in 199 patients (61%), tracheal aspirate in 9 (3%), bronchoalveolar lavage (BAL)in one (0.29%), and in 124 patients (38%), sputum was induced. The eastern province contributed to the highest number of patients (122, 37.7%). There is a persistent increase in the prevalence of the common bacteria over the follow-up period of 7 years, namely Hemophilus influenzae (H. influenzae), Staphylococcus aureus (S. aureus), and all Pseudomonas (P. aeruginosa) culture types.Comparing cultures from the first and last follow-up visits, there was an increase in the prevalence of all (P. aeruginosa) cultures from 120 (34%) to 137 (53%), and a decrease in the prevalence of (S. aureus) and (H. influenzae) during the same follow-up period. Conclusion: There is a progressive increase in the number of patients with the most pathogenic types of bacteria because of the advanced age at presentation. As more adult patients are enrolled, there is a need for improved awareness regarding the early eradication of pathogenic bacteria to prevent progressive pulmonary damage.

Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia.

Background: Internationally, Cystic fibrosis-associated liver disease (CFLD) is considered the third leading cause of death, following lung disease and transplantation complications. Aims: To identify the prevalence of CFLD in cystic fibrosis (CF) patients. Methodology: A retrospective chart review for all patients with CF liver disease from a tertiary care center. Result: A total of 341 CF patients were included. The mean age at the diagnosis of liver disease is 13.5 (7.6) years.The first elevated ALT was reported in 190/341 patients (56%), elevated AST in 124 patients (36%), elevated alkaline phosphatase (ALP) in 166 patients (49.1%), elevated GGT in 57 patients (23%), and elevated bilirubin in 24 patients (7%). There was an improvement of the liver enzyme values during the follow-up period, P-value = (<0.05).Ultrasound liver assessments were performed in 258/341 patients (75.7%). One hundred and twelve patients (43%) had abnormal findings. In 14 patients (5.4%), assessment exhibited advanced liver disease (liver cirrhosis and periportal fibrosis). Out of 190 patients, who were given ursodeoxycholic acid for elevated liver enzymes, 180 (94.7%) exhibited improvement. One patient underwent liver transplant at the age of 12. Four patients were submitted for liver biopsy; periportal fibrosis was observed in 4 patients (1.6%), and liver cirrhosis by ultrasound (US) in 10 patients (4%). Conclusion: Patients with CF should be screened early for liver enzymes, and should undergo the US study to detect liver disease at early stages and to prevent its progression.

Correlation of Gut Microbiota, Vitamin D Status, and Pulmonary Function Tests in Children With Cystic Fibrosis.

Background: Children with cystic fibrosis (CF) are expected to have suboptimal serum vitamin D status and altered gut microbiota. The altered gut microbiota is hypothesized to have a pro-inflammatory effect that further complicates the existing respiratory inflammation. Emerging evidence suggests an association between vitamin D and gut microbiota. The aim of this study was to assess the relationships between 25-hydroxyvitamin D [25(OH)D] status, pulmonary function, and fecal bacteria in children with CF. Methods: In this cross-sectional study, a total of 35 children with CF (8.7 ± 2.83 years) and 24 controls without CF (9 ± 2.7 years) were included in this study. Serum 25(OH)D status was measured using the Elecsys vitamin D total II assay. In the CF group, gut microbiota composition was assessed using real-time PCR analysis. Pulmonary function tests (PFTs) were measured using spirometry. Comparisons between the CF and non-CF controls were conducted using the independent sample t-test. In the CF group, one-way analysis of variance (ANOVA) was used to assess differences in PFTs and gut microbiota composition across the three vitamin D subgroups. The correlations between 25(OH)D status and PFTs, or gut microbiota composition, and PFTs with gut microbiota composition were analyzed using the Pearson's correlation coefficient test. Results: Children with CF had significantly lower serum 25(OH)D levels compared with children without CF (44.3 ± 22.4 vs. 59 ± 25.5, respectively, P = 0.026). Children with CF with optimal serum 25(OH)D level had significantly higher levels of Bacteroidetes, Firmicutes, and total bacteria (P = 0.007, P = 0.007, and P = 0.022, respectively). The level of Firmicutes was found to be significantly higher in mild forced expiratory volume in 1 s (FEV1) compared with moderate FEV1 (P = 0.032), whereas the level of the other bacteria species was comparable across FEV1 severity groups. Conclusion: Our findings may encourage studies that target and modify gut microbiota to potentially achieve better outcomes in terms of respiratory function in CF.

The first report on CFTR mutations of meconium ileus in cystic fibrosis population in Saudi Arabia: A single center review.

Introduction: Meconium ileus (MI) is one of the most common causes of intestinal obstruction in newborns. It is the earliest clinical manifestation of cystic fibrosis (CF). MI is suspected if a baby fails to pass meconium shortly after birth and develops symptoms of bowel obstruction, such as distention of the abdomen or vomiting. MI can lead to bowel perforation, a twisting of the bowel, or inflammation and infection of the abdominal cavity. Objectives: To find the incidence and prevalence of meconium ileus in cystic fibrosis patients and to report on the most common gene mutation of MI in CF patients. Methodology: Retrospective review of the medical documentations of all MI patients during the period of 1989-2018. Results: A total of 40 CF confirmed patients were presented with MI. Twenty-nine patients (71%) are alive and 11 patients (29%) died or lost to follow-up. The following CFTR mutations were found: Eight patients (20%) with c.2988+1G>A; Intron 18. Seven patients (17.5%) with c.1418delG; Exon 11. Five patients (12.5%) with c.579+1G>T; Intron 5. Four patients (10%) with c.1911delG; Exon 14. Four patients (10%) with c.1521_1523delCTT; Exon 11. Four patients (10%) with c.416A>T; Exon 13. Three patients (7.5%) with c.2421A>G; Exon 14. Two patients (5%) with c.3908A>C; Exon 21. One patient (2.5%) with c.3889dupT; Exon 24. One patient (2.5%) with c.1657C>T; Exon 12. One patient (2.5%) with c.2547C>A; Exon 14a. Eighteen patients (45%) were presented with vomiting, 38 patients (95%) had postnatal radiological findings, 7 patients (17.5%) had electrolytes imbalance. Five patients (12.5%) had cholestasis and 4 patients (10%) developed chronic liver disease. Thirty-five patients (79.5%) underwent surgical repair and 9 patients (20.5%) were treated medically. Mean age of operation was 2.25 (2) days. Of 9 patients, 6 (66.6%) were treated with gastrograffin enema, 2 patients (22.2%) with oral N-acetylcysteine and 1 patient (11.1%) with saline rectal wash. Thirteen patients (31.5%) required TPN. Five patients had recurrent operation. Conclusion: CF and meconium ileus are commonly present in CF patients in Saudi Arabia. Prognosis is similar to other CFs without MI, if treated early. Thirty percent of our CF/MI patients have intronic mutations.

The first report on the association of celiac disease and cystic fibrosis in a tertiary care center in Saudi Arabia.

Introduction: Celiac disease (CD) has been described before in Saudi Arabia (SA) to be at the range of 1%-2% in the general population, but the association of celiac disease and cystic fibrosis (CF) has never been described before in the Middle East. Objectives: To describe the prevalence of the association of CD and CF in patients with gastrointestinal symptomatology in a tertiary care center. Method: ology: A retrospective charts review of all confirmed CD and CF patients for the years 1989-2018. Results: In a total of 391 confirmed CF patients, 74 of them (19%) had celiac screening due to their symptomatology in the form of (abdominal pain and distension, vomiting, diarrhea despite adequate pancreatic enzyme replacements, and had high antigliadin antibodies and anti-transglutaminase IgA (tTGA). Thirty-five of the 74 patients were male (47.3%, and 39 (52.7%) were female patients. The mean age at diagnosis of CD was 6.1 (3.9), and the mean age at follow up was 7 (5 years). Only 2 of the 74 patients (3%) had bowel biopsies with the typical pathological findings of CD with villous atrophy. Both patients were placed on a gluten-free diet and showed marked improvement in symptomatology and weight gain. Conclusion: CD screening should be considered in all CF patients despite the absence of symptoms. The prevalence of CD in CF patients in SA is similar to or slightly higher than that of the general population. A further study to screen the whole CF population is needed to delineate the actual prevalence, particularly in nonsymptomatic CF.

Geographic distribution of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Saudi Arabia.

INTRODUCTION: Cystic fibrosis (CF) has been reported before in Saudi Arabia and the Gulf area. It has been found that screening for 10 most common cystic fibrosis transmembrane conductance regulator (CFTR) mutations can detect 80% of positive CFTR cases. OBJECTIVES: To determine the geographic distribution of the most common CFTR variants in 5 regions of Saudi Arabia. METHODOLOGY: A retrospective chart review of all CFTR variants conducted from January 1, 1992 to December 1, 2017. RESULTS: The ten most common CFTR mutations in the Saudi population were as follows: p.Gly473GlufsX54 (17%), p.Phe508del (12%), p.Ile1234Val (12%), 3120+1G > A (11%), 711+1G > T (9%), p.His139Leu (6%), p.Gln637Hisfs (5%), p.Ser549Arg (3%), p.N1303K (3%), and delExon19-21 (2%) along with other variants 79 (20%). In terms of the highest frequency, the c.2988+1G > A (3120+1G > A) variant was found in the eastern province (7.3%) of Saudi Arabia, the c.1418delG (p.Gly473GlufsX54) variant in the northern province (6.8%), the c.579+1G > T (711+1G > T) variant in the southern province (4.8%), the c.3700A > G (p.Ile1234Val) variant in the central province (4.8%), and c.1521_1523delCTT (p.Phe508del) variant in the western province (4.3%). CONCLUSION: The eastern and the northern provinces have the highest prevalence of CF, with the c.2988+1G > A (3120+1G > A) and c.1418delG (p.Gly473GlufsX54) variants showing the highest distribution in the Saudi CF population, which may reflect the effect of consanguinity within the same tribe. Proper family screening and counseling should be emphasized.

The first report of Methicillin-resistant Staphylococcus aureus (MRSA) in cystic fibrosis (CF) patients in Saudi Arabia.

INTRODUCTION: Methicillin-resistant Staphylococcus aureus infections have been increasingly reported in patients with cystic fibrosis (CF) who have progressive deterioration in their pulmonary function. OBJECTIVES: To determine the prevalence of MRSA infections in CF in a tertiary care center in Saudi Arabia. METHODOLOGY: This is a retrospective chart review conducted as part of the CF registry data from 1 January 2002 to 1 June 2016. All patients with confirmed CF of all age groups who had a respiratory culture positive for MRSA were included in the study. RESULTS: Among 385 patients with CF who had respiratory samples, 43 (11%) were positive for MRSA at a mean age of 10.4 ±â€¯7.2 years. Twenty-two patients out of the 43 (51%) were treated with different regimens: nasal Bactroban in 13/22 (59%); a combination of nasal Bactroban, oral vancomycin, and rifampicin for 2 weeks in 5 patients (23%); Bactroban and linezolid in one patient (5%); and oral vancomycin and rifampicin in 3 patients (14%). Eight out of the 22 treated patients (36%) achieved MRSA eradication. Six out of the 22 treated (27%) had experienced MRSA recurrence within 3-6 months, and another 5/22 (23%) continued to have MRSA colonization up to 2-4 years of follow-up despite using a proper eradication protocol. Twelve out of the 43 (28%) patients with MRSA infection died. CONCLUSION: MRSA infection in our population with CF is common. Therefore, an eradication protocol should be instituted at an early stage to prevent chronic colonization. Children with persistent MRSA colonization have high morbidity and mortality rate.

The first report on immunoglobulins A, E, G and M levels in cystic fibrosis patients in Saudi Arabia.

BACKGROUND: Previous reports have shown that pulmonary and systemic hypergamma-globulinemia in CF patients is a reflection of chronic pulmonary infection. Infection with Pseudomonas aeruginosa is known to have major prognostic significance in patients CF. This study aims to identify the incidence of immunoglobulins (especially: IgG, and IgE) in a cohort of CF patients. METHODS: A total of 297 patients recruited all over the country's region for this study were a as part of the CF registry data from 1st January 1984 to 1st June 2016. All patients had their immunoglobulin levels measured by enzyme link immunosorbent assay (ELISA) in 3 stages, at presentation and two follow-ups. RESULTS: Of the 297 patients recruited, 139 (46.8%) were males while 158 (53.2%) were females. IgA and IgM levels were found not to have risen above the previously reported levels in healthy individuals in all stages. On the contrary, IgE level increased from 209.51 ± 32.30 KU/L to 303.58 ± 37.11 KU/L from baseline to stage 3 while IgG level rose from 12.26 ± 0.43 mg/mL to 17.17 ± 1.68 mg/mL for baseline and stage 3 respectively all above previously reported levels in healthy individuals. CONCLUSION: This study establishes a potential for the use of IgE and IgG in disease diagnosis as well as the prognostic implications. However, further study is needed to identify the role of infection or medications in relation to the rise of both IgE and IgG with advancement of age and progression of disease severity which may inherently confound the observed results.

The prevalence of viral infections in children with cystic fibrosis in a tertiary care center in Saudi Arabia.

INTRODUCTION: Studies have shown that pulmonary exacerbations in cystic fibrosis (CF) patients are associated with respiratory viruses. The most common agent causing viral infections in patients with CF before the age of 3 years is respiratory syncytial virus. OBJECTIVES: To obtain the prevalence of the different types of viral infection in CF patients and to identify its relation with the type of bacterial infection, (CFTR) mutations and pulmonary function test (PFT). METHODOLOGY: A retrospective charts review of 387 patients with CF of all age groups who were screened for the detection of viruses during respiratory exacerbation from the period of January 1, 1984 to June 1, 2016. RESULTS: A total of 159 CF patients had pulmonary exacerbation and had viral PCR obtained. Fifty-eight patients (36%) had positive viral PCR. Males were more commonly infected in 30/58 patients (52%) compared to females in 28 patients (48%). Forty-five of 58 patients (78%) were alive and 13 patients (22%) died. Rhinovirus was the most frequently reported viral PCR in 33/74 sample (45%). Out of 74 viral PCR, 41 (55.4%) were during the colder seasons (October-February) and 33 (44.5%) during the warmer seasons (March-September). During viral infection and viral recurrence, there was an increase in bacterial colonization specifically of H. influenza and staphylococcus aureus. The most common CFTR mutation for the CF viral infection is: 3120+1G>A in Intron 16 in 11/57 patients (19%). The Eastern Province had the highest viral infection of 24 out of 57 patients (42%). Follow-up PFT post viral infection showed no significant difference in the type and the severity of PFT compared to the initial PFT during the viral illness. CONCLUSION: Viral infections contributed to the increase in morbidity and mortality of CF patients in our population, and rhinovirus was the most common causative agent. Viral infections and viral recurrence increased the prevalence of bacterial infection of specific pathogens such as H. influenza and S. aureus. Physicians should be aware to prevent progressive lung damage in CF patients by treating the concomitant viral and bacterial infections. Viral infection may be associated with some common CFTR mutations.

Genotype patterns for mutations of the cystic fibrosis transmembrane conductance regulator gene: a retrospective descriptive study from Saudi Arabia.

BACKGROUND: Cystic fibrosis (CF) occurs in populations in Saudi Arabia and the Gulf area. Approximately 2000 known variants have been identified for the CF transmembrane conductance regulator (CTFR) gene. Screening for ten of the most common variants can detect 80% of alleles. OBJECTIVE: Determine the pattern of CFTR variants in the CF population of Saudi Arabia. DESIGN: A retrospective, descriptive. SETTING: Tertiary care center. PATIENTS AND METHODS: We examined the medical records of 396 confirmed CF patients of all age groups that were positive for a CFTR variant from the period of 1 January 1998 to 1 December 2017. MAIN OUTCOME MEASURES: Zygosity, morbidity and mortality patterns of different types of CFTR variants. SAMPLE SIZE: 312 families that included 396 patients. RESULTS: Of 48 variants identified, 6 were novel, having not been described in the medical literature. A homozygous state was found in 283 families (90.7%) and compound heterozygosity in 23 (7.4%). Six families were heterozygous (1.9%). Median age (interquartile range) was 10.2 months (4.4 months to 5.7 years) at diagnosis and 9.7 (5.4-16.5) years at follow up. Of 396 patients, 378 patients (95.5%) survived and 18 (4.5%) died. The ten most common variants identified in descending frequency were: p.Gly473GlufsX54 in 98 alleles (16%), p.Ile1234Val in 66 alleles (11%), F508del in 64 alleles (11%), 711+1G>T in 62 alleles (10%), 3120+1G>A in 62 alleles (11%), p.His139Leuin 38 alleles (6.4%), p.Gln637Hisfs in 30 alleles (5.2%), p.Ser549Arg in 27 alleles (4.5%), p.Asn1303Lys in 14 alleles (2.3%), delExon19-21in 10 alleles (1.6%). This analysis identified 79.2% of our CFTR variants. CONCLUSION: CFTR mutational patterns in our CF population are characterized by a high allelic heterogeneity. The high prevalence of homozygous variants reflects the high level of consanguinity between parents. LIMITATIONS: Our CFTR screening reflected only about 80% of CF patients in Saudi Arabia. CONFLICT OF INTEREST: None.

The first case report of double homozygous of 2 different mutations in the CFTR gene in Saudi Arabia.

The first cases of a rare double homozygosity of two different mutations in the cystic fibrosis trans-regulator gene (CFTR) of a cystic fibrosis patient in Saudi Arabia. Details of the family screening and a review of the literature on similar cases are discussed.

Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces.

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

The road for survival improvement of cystic fibrosis patients in Arab countries.

Cystic fibrosis (CF) is a lethal, monogenic disorder that affects multiple organ systems of the body. The incidence has been described before in the Middle East to be 1 in 2000 to 1 in 5800 live births, and the median survival was estimated to be from 10 to 20 years of age. The present article attempts to revisit various facets of this disease and specifically highlights the most important lacunae that exist in treating CF. In addition, it also tries to emphasize the steps in improving the median survival of patients with CF, in these countries.

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates.

The Saudi Association for Pulmonary Hypertension (previously called Saudi Advisory Group for Pulmonary Hypertension) has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008.[1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension (PH). One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH.

Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

Repair of congenital heart disease with associated pulmonaryhypertension in children: what are the minimal investigativeprocedures? Consensus statement from the Congenital HeartDisease and Pediatric Task Forces, Pulmonary VascularResearch Institute (PVRI)

Standardization of the diagnostic routine for children with congenital heart disease associatedwith pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignmentto repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance)may be detrimental and associated with poor outcomes. Thus, members of the Congenital HeartDisease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct asurvey aimed at collecting expert opinion from different institutions in several countries, covering manyaspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnosticprocedures and immediate postoperative support. In privileged communities, the vast majority of childrenwith congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation,and have an uneventful postoperative course, with no residual PAH. However, a small percentageof patients (older at presentation, with extracardiac syndromes or absence of clinical features of increasedpulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk ofcomplications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach,including invasive procedures. The authors emphasize that decision making regarding operability is basednot only on cardiac catheterization data but also on the complete diagnostic picture, which includes theclinical history, physical examination, and all aspects of noninvasive evaluation.

Clinical profile of Saudi children with bronchiectasis.

OBJECTIVE: This study represents the experience of a tertiary care center in Saudi Arabia on non-cystic fibrosis bronchiectasis. METHODS: A retrospective review of all patients with confirmed Non-Cystic Fibrosis (Non-CF) bronchiectasis by chest X-ray and or CT chest in a pulmonary clinic for the period 1993-2005 at a tertiary care center in Riyadh. RESULTS: A0 total of 151 cases were diagnosed as Non-CF bronchiectasis. Siventy-five (49.7%) were males, 76(50.3%) were females. One hundred forty-eight (98%) are alive and 3(2%) died. The southwestern regions constituted 72(50%) of the cases. There is a period (5+/- 3.2) years between the start of symptoms and the diagnosis of bronchiectasis. More than 2/3 of the patients had cough, tachypnea, wheezing, sputum production and failure to thrive. Ninety one (60%) had associated disease: Pulmonary diseases in 48(32%), immunodefficiency in 27(18%), CNS in 18(12%), cardiac in 12(8%) and asthma in 103(68%) of the patients. Left lower lobes were commonly involved in 114(76%) cases. Sixty-eight (67%) were found to have sinusitis. Forty-nine (32%) developed gastroesophgeal reflux (GER). Hemophilus influenza was cultured in 56(37%), Strept pneumoniae in 25(17%) and Pseudomonas aeruginosa in 24(16%) of the patients. 80% of the patients who had pulmonary function test had abnormal changes. Disease progression was related to development of symptoms before 5 years of age, persistent atelectasis and right lower lobe involvement (p<0.05). CONCLUSION: Non-CF bronchiectasis is a common problem in Saudi Arabia. Early recognition and institution of treatment with proper vaccination of available anti-bacterial and anti-viral vaccines are encouraged to prevent progression of the disease.

A review of 151 cases of pediatric noncystic fibrosis bronchiectasis in a tertiary care center.

OBJECTIVE: This study was conducted to review the etiological factors and diseases associated with pediatric noncystic fibrosis bronchiectasis in a tertiary care center in Saudi Arabia. MATERIALS AND METHODS: A retrospective review of all patients with confirmed noncystic fibrosis (Non-CF) bronchiectasis by chest X-ray and/or CT chest in a pulmonary clinic during the period 1993-2005 at a tertiary care center in Riyadh. RESULTS: A total of 151 cases were diagnosed as Non-CF bronchiectasis. Seventy-five (49.7%) were male, 76 (50.3%) were female; 148 (98%) are alive and 3 (2%) died. The southwestern regions constituted 72 (50%) of the cases. There was a period of (5 +/- 3.2) years between the start of symptoms and diagnosis of bronchiectasis. More than two-thirds of the patients had cough, tachypnea, wheezing, sputum production and failure to thrive. Ninety-one (60%) had associated diseases: Pulmonary diseases in 48 (32%), immunodeficiency in 27 (18%), central nervous system anomalies in 10 (7%), cardiac in 10 (7%) and asthma in 103 (68%) of the patients. Left lower lobe was commonly involved in 114 (76%) patients. Sixty-eight (67%) were found to have sinusitis. More than two-thirds of patients had two or more associated diseases. Forty-nine (32%) developed gastroesophageal reflux. Hemophilus influenza was cultured in 56 (37%), strep pneumoniae in 25 (17%) and pseudomonas aeruginosa in 24 (16%) of the patients. Eighty percent of the patients who had pulmonary function test had abnormal changes. Disease progression was related to development of symptoms before 5 years of age, persistent atelectasis and right lower lobe involvement (P< 0.05). CONCLUSION: Non-CF bronchiectasis should be included in the differential diagnosis of recurrent chest infection in Saudi Arabia. Early diagnosis and identification of associated diseases is needed to prevent progression of the disease.

Gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula.

OBJECTIVE: This study represents the experience of a tertiary care center in the Kingdom of Saudi Arabia (KSA) on the long-term effect on the lungs of esophageal atresia (EA) and tracheoesophageal fistula repair (TEF), and to emphasize the magnitude of gastroesophageal reflux (GER) post-EA or post-TEF repair. METHODS: A retrospective review of all patients referred to the pulmonary clinic with EA/TEF or re-operative evaluations from the period 1993-2004 at King Faisal Specialist Hospital and Research Centre, Riyadh, KSA. RESULTS: Forty-one patients with confirmed EA/TEF (26 males and 15 females) were included in the study. Congenital anomalies were associated in 28 (68%). Gastroesophageal reflux developed in 39 (95%) of patients, 24 (59%) needed Nissen fundoplication. Esophageal stricture that required more than 3 dilations developed in 16 (46%) patients, esophageal dysmotility in 37 (90%) and hiatal hernia in 11 (27%). Pulmonary complications developed in >70% of the patients including persistent atelectasis, chronic aspiration pneumonia, asthma and chronic lung disease that required oxygen for more than one month. Tracheomalacia in 12 (29%) and bronchiectasis in 7 (17%). Eighty-eight percent of patients who were able to do pulmonary function test showed abnormal values of moderate obstructive and restrictive lung disease. CONCLUSION: Pulmonary complications cause significant and prolonged morbidities post EA/TEF repair. Gastroesophageal reflux is a common complication after EA/TEF repair and causes significant morbidity that needs a prolonged follow up. Patients with GER may need Nissen fundal plication to improve respiratory problems.