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Clinical profile of Saudi children with bronchiectasis
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     Department of Pediatrics, King Faisal Specialist Hospital and Research Centre (KFSH&RC), Riyadh, Saudi Arabia

    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.

    Keywords: Bronchiectasis; Chest infection, Developing countries

    Bronchiectasis was called an orphan disease for the last 2 decades as its incidence decreased markedly and became an uncommon clinical entity among adults and children in developed countries.[1] It is defined as a perminent dilatation of the bronchi,[2] which typically involves the second to 6th order of segmental bronchi.[2] It was first described by Lannec in 1819 based on examination of postmortem specimens.[3] Bierrring (1956) studied 151 patients in Copenhagen following pneumonia and found only one child (0.7 %) to have bronchiectasis.[4] Ruberman and colleagues (1957) evaluated 69 patients with persistent abnormalities on chest radiographs by bronchoscopy.[5] Out of 1711 young adults (18 to 25 years of age) treated for pneumonia at a U.S. army hospital, 29 (1.7 %) found to have bronchiectatic changes.[5] Fileld noted a dramatic decrease in admission rates, for bronchiectasis at 5 British hospitals from an average of 48 per 10,000 in 1952 to 10 per 10,000 total pediatric admissions in 1960.[6] She speculated that improved treatment of lower respiratory tract infections made possible by the increased availability of broad-spectrum antibiotics during that period.[6] Other contributing factors include the prevention of measles and pertussis through immunization and the marked decrease in primary pulmonary tuberculosis in the pediatric population which was brought about by better public health measures and improved treatment regimens for this disease.[3]

    There are very few reports on bronchiectasis in the Middle East.[7] Information on the clinical profile and the underlying diseases have been scarce. In this report, the author presents the experience of a tertiary care center in Saudi Arabia on childhood bronchiectasis.

    Materials and Methods

    A retrospective review of charts for all patients referred to the pulmonary clinic for evaluation of recurrent chest infection during the period Jan. 1993- Aug. 2005 at King Faisal specialist hospital and research center (KFSH&RC)0 in Riyadh region which is considered a tertiary care center for referral of complicated cases in Saudi Arabia. Demographic, radiological patterns, associated diseases, and pulmonary function test data (PFT) were collected.

    Statistical Analysis

    SPSS program for Windows (release 11.0.0) was used for data analysis. Chi-Square (c2) was used to compare categorical variables. Results were presented at a level of significance of p= <0.05

    Definitions

    Progression of Disease a qualitative measurement, defined as a radiological deterioration with more lobes involved in addition to clinical deterioration with increased sputum production, cough and or fever.

    PFT severity: a quantitative measurement of airflow in PFT

    Mild lung changes : defined as forced expiratory volume in one second (FEV1) as 65-75% of predicted values.

    Moderate lung changes: As FEV1 = 55-65% predicted.

    Severe lung changes: As FEV1 < 55% predicted.

    Results

    Of a total of 900 cases were referred with recurrent chest infection to pulmonary clinic during the period Jan 1993- August 2005. 200 patients were diagnosed to have cystic fibrosis (CF). Of the remaining 700, 151 cases were diagnosed as Non-CF bronchiectasis based on chest X-ray in 151(100%) of the patients and/or CT chest on 145(96%) of the patients. Seventy-five (49.7%) were males, 76(50.3%) were females. One hundred and forty-eight (98%) are alive and 3(2%) died. One hundred and forty four (95%) were Saudi and 7(5%) were non-Saudi. One hundred and forty (93%) were full term. Twenty-two (14.6%) from the eastern region, 26(17.2%) from the central region, 39(25.8%) from the western region, 33(21.9%) from the southern region, and 4(2.6%) were from neighboring countries. Ninety-eight (65%) of the families were consanguinous, with 18 patients (12%) having 1-2 siblings with bronchiectasis and 5 patients had 3-4 siblings with similar disease. Age when symptoms started was 2.3 ± 2.2 years. Age at referral to our center was 6.3 ± 4 years. Age of bronchiectasis diagnosis was 7.3 ± 4.1 years. There is a period of (5± 3.2) years between the start of symptoms to the diagnosis of bronchiectasis. Period of follow up 5.5 ± 3.9 years. More than 2/3 of the patients presented with cough, Tachypnea, wheezing, sputum production and failure to thrive. Clubbing was found in 50(33%) of the patients. Cyanosis and oxygen requirement was reported in 35(23%) of the patients. Hemoptysis was only reported in 7(5%) of the cases. Ninety-one (60%) had associated diseases [Table - 1]. Pulmonary diseases were found in 48(32%), immunodefficiency in 27(18%), Central nervous system (CNS) in 18(12%), cardiac in 12(8%), skeletal anomalies in 10(7%) and asthma in 103(68%) of the patients [Table - 1]. Radiological changes that were reported: Consolidation of one or 2 lobes in 137(91%) of the patients, hyperinflation in 103(68%), interstitial pattern in 49(33%), atelectasis in 117(78%), Peribronchial wall thickening in 115(76%) and lymph node enlargement of the Para tracheal region in 33(22%) of the patients. Left lower lobes (LLL) were commonly involved in 114(76%), right middle lobe (RML) in 82(54%), and right lower lobe (RLL) in 76(50%), lingula in 73(48%), right upper lobe (RUL) in 39(26%), and left upper lobe (LUL) in 27(18%) of the patients. Bilateral lobar involvement was noticed in 112(71%) [Table - 2]. A total of 102 patients had sinus X-ray, 18(12%) had CT sinuses. Sixty-eight (67%) of 102 patients "who had sinus radiological investigations" found to have sinusitis. Sixty-nine (46%) of the patients had investigation for gastroesophgeal reflux (GER) by barium swallow and by milk scan in 27 (18%) of the patients. GER was found in a total of 49 (32%) of the patients, 43 from barium swallow group and 13 from milk scan group. Twenty-two of 49 patients with GER (45%) required Nissen fundo- plication. Respiratory cultures were done in 105 (70%) of the patients by sputum cultures if patients were able to do it or by nasopharyngeal aspirates for patients less than 4 years. Hemophilus influenza (H-flue) was cultured in 56(37%), Streptococcus pneumoniae in 25(17%), pseudomonas aeruginosa in 24(16%), Branhamella Cattarrhales in 13(9%), Staphylococcus aureus (Staph.) in 11(7%) and Methicillin resistant staphylococcus aureus (MRSA) in 3 (2%) of the patients. Candida albicans was noticed in 2 (1 %) of the patients. Viral cultures were done in 33 (22%) of the patients: Respiratory Syncytial virus in 3 (9%), and Enterovirus in 1 (3%) of the patients. Seventy-seven (49%) of the patients were able to do pulmonary function test (PFT). Sixty-eight (88%) of them had abnormal PFT changes. Seventeen (22%) had obstructive lung changes, 14(18%) had restrictive lung changes, and 37(48%) had combined obstructive and restrictive lung changes. Sixteen (21%) had mild PFT changes, 30(39%) moderate lung changes and 22(28.5%) had severe lung changes. Disease progression developed in 72 (48%) of the patients and it was related to development of symptoms before 5 years of age, persistent atelectasis of the affected lobes, and involvement of RLL with bronchiectasis [Table - 3] (p <0.05). Unilateral lobectomy was done in 21(14%) of the patients whereas bilateral lobectomies in 3 (2%) of them. Recurrent otitis media was reported in 12(8%) of the patients.

    Discussion

    A recent review of bronchiectasis among Alaska Native children residing in the Yukon-Kuskowkim delta region reported a persistently high incidence of 110/10,000 in 1940s and 140/10.000 persons born in the 1980s.[8] Alaska is considered part of a developed nation, with adequate immunization programs, and Tuberculosis is no longer a major source of pulmonary disease, however, the children reported lived in conditions of relative poverty: small crowded houses heated by wood-burning stoves, with limited access to running water, and 70% of house holds had one or more family members who smoked tobacco which, promotes chronic air way mucous secretion and recurrent respiratory infections.

    Published reports from some developing countries suggest that childhood bronchiectasis may not be disappearing, and that it represents a more common problem than in developed countries. Karakoc from Turkey described 23 children with bronchiectasis and found that factors other than infections have contributed to the development of bronchiectasis, such as immunodefficiency, primary ciliary dyskinesia and asthma.[2] A report by Dawson from United Arab Emirates from Abu Dhabi region described 32 children with bronchiectasis from a population of 300,000.[7] He found that congenital anomalies of the respiratory system, prematurity, immunodefficiency were some of the factors that contributed to the cause of the disease in addition to viral or bacteria infections.[7]

    In the present report, bronchiectasis incidence was found to be 1 in 4 cases that presented with recurrent chest infection in the authors center, which makes it a common problem in this part of the world. Bacterial infection with the common respiratory organisms such as: Staph aureus, H-flue, Pneumococcus, and pseudomonas were found to be common bacteria cultured from 51% of the patients. The Southwestern region accounted for 50% of the reported case. Environmental factors such as humidity and crowd during Pilgrimage time may have contributed to such increase in its incidence. Recurrent aspiration pneumonia due to CNS anomalies or seizure is described for the first time in the literature and might be related to recurrent aspiration of secretions due to swallowing incoordination and or GER. The present report agrees to other reports of early start of symptoms before 5 years of age in 83% of our population,[2] with a delay of diagnosis of bronchiectasis by an average of 5-10 years. Most of the patients had bilateral lobar involvement and severe PFT changes at presentation. Fifty percent of the present study patients had radiological and clinical progression in spite of medical treatment of antibiotic prophylaxis, which may suggest the adoption of surgical intervention in patients with progressive disease as lobectomy has been done in only 16% of the present study population compared to 60-70% in other reports.[2] Asthma was common association in 68% of the patients, which is in accordance with other reports and treatment with inhaled steroid, and B2 agonist may need to be considered in some patients. Immunodefficiency is common in our country due to consanguinity and found to be the 2nd most common disease association after pulmonary disease [Table - 1]. Sinusitis was also a common presentation in the present study patients in 68% and such patients may need to be treated for a longer period of time as suggested by other reports for 4-6 weeks.[3] Persistent atelectasis of the affected lobe has been contributing to the development of bronchiectasis in our population, which may warrant encouragement of chest physiotherapy, and postural drainage in patients with such a problem. Atelectasis is commonly found in many patients with pneumonia, aspiration or asthma and repeat chest X-ray should be done after clinical improvement to ensure the re-expansion of the atelectatic part of the lung. Gastroesophageal reflux and recurrent aspiration is found in 32% of the present study patients and may have contributed to the development of bronchiectasis or complicated its progression. A case control study needs to be done to identify the actual risk factors of developing such disease in our country and efforts should be made to early diagnosis, awareness of contributing factors and early treatment or referral before development of progression.

    References

    1.Callahan CW, Redding G. Bronchiectasis in children: Orphan disease or persistent problem Pediatr Pulmonol 2002; 33: 492-496.

    2.Karakoc GB, Yilmaz M, Altintas DU, Kendiri SG. Bronchiectasis: Still a problem. Pediatr Pulmonol 2001; 32: 175-178.

    3.Brown MA, Leman RJ. Bronchiectasis. In Chernick V, Boat T, eds. Kendig's disorder of the respiratory tract in children. 6th edition, Philadelphia; WB Saunders; 1998. 538-560.

    4.Biering A. Childhood pneumonia, including pertussis, pneumonia and bronchiectasis: a follow-up study of 151 patients. Acta Pediatr 1956; 45 : 348- 351. [PUBMED]

    5.Ruberman W, Shaufer I, and Bioondo T. Bronchiectasis and acute pneumonia. Am Rev Tuber 1957; 76: 761-765.

    6.Field CE. Bronchiectasis: Third report on a follow-up study of medical and surgical cases from childhood. Arch Dis Child 1969; 44: 551-555 [PUBMED]

    7.Dawson K.P., Bakalinova D. Child bronchiectasis in a desert location. Middle East Pediatrics 1996; 1: 6-8

    8.Singleton R, Morris A, Redding G, Poll J, Holck P, Martinez P, Kruse D, Bulkow LR, Peterson KM, Lewis C. Bronchiectasis in Alaska Native children: causes and clinical courses. Pediatr Pulmonol 2000; 29: 182-187.(Banjar Hanaa Hasan)