Global Journal of Medical Research, F: Diseases, Volume 22 Issue 6

and also brought to light the phenotypic spectrum of CF in this country. In the present study, sweat chloride test was conducted in 400 patients with high clinical suspicion and diagnosis of CF was based on the CF Foundation guidelines in consensus report in 2008 for diagnosis of CF i.e., presence of characteristic clinical features of CF or history CF in a sibling or a positive newborn screening test result plus a positive sweat chloride test or presence of two CF causing mutations or abnormal nasal potential differences. 17 Among 400 patients included in the study, the sweat chloride test was positive in 38 patients (9.5%). In India, Kabra et al. 18 conducted a study in the All-India Institute of Medical Sciences (AIIMS) pediatric chest clinic and found sweat test was positive in 3.5% of patients which is lower than our study. Another study conducted in India by Manzoor A. Raina et al. 19 found sweat chloride test positive in 22.5% of patients which is much higher than the present study. These differences might be due to differences in the number of study population, age at presentation of disease symptoms and variation in geographical and ethnic populations. No such study has been conducted previously in this country and further studies are needed to get the actual scenario of CF in Bangladesh. The mean age at diagnosis of CF in our study was 8 . 92 ± 6.72 yrs with a range of 2 – 32 yrs which is close to the studies reported by Homash et.al 9 and Kawoosa et al. 20 where the age at diagnosis was 9.6 yrs and 10.5 yrs respectively. The age at diagnosis of CF is much higher in the Indian sub-continent 21 in contrast to the patients of USA where 71% of CF cases are diagnosed by 1 st year of life. 22 Reality might be due to low index of suspicion among the treating physicians and lack of proper diagnostic facilities. Regarding gender discrimination, there was a male preponderance of the disease in SCT positive patients in our study (63 % male vs 37% female) which is statistically significant (p < .05). This could be related to greater attention received by the male child and greater provision of medical care to them. Consanguinity was present in 18.42% of SCT positive patients in present study, not significant statistically (p >0.05) but this finding is supported by a study in India 18 where 19.2% of CF patients were presented with consanguinity. However, a higher rate of consanguinity was reported in CF patients in studies repo rted from Middle East countries. 23,24 Statistically significant number of CF patients (18, 47.4%) in present study were from rural area and 29 % and 18 % patients from urban and sub urban areas respectively (p < .05). This might reflect the aforementioned thoughts about CF being a rare disease and also financial constraints and long travels to get sweat test done. The most frequent mode of clinical presentation among the study participants was repeated respiratory tract infection. FTT, digital clubbing, recurrent pneumonia, nasal polyps and rectal prolapse were the significant clinical manifestations among SCT positive patients (p <.05). On the other hand, persistent pneumonia and hemoptysis were present significantly in borderline SCT group. These clinical manifestations are almost similar to the studies reported by Raina et al. from India 19 , El Falaki et al. 25 from Egypt and Farahmand et al. 26 from Iran with few differences in percentages in some points of clinical involvement which might be due to differences at the age of CF diagnosis and also big differences in study samples. Failure to thrive (FTT) was present in 84.21 % of the SCT positive patients in present study, highly significant statistically (p<.001). Shaha et al. 27 reported FTT in 83.9 % and Kabra et al. 18 reported in 90 % of CF patients in their studies, which supports the present study. Clinical presentations of pancreatic insufficiency such as ch. Diarrhea, steatorrhea was present in 28.94 % and 18.42 % of patients respectively in SCT positive group, not significant statistically in present study. Raina et al. 19 from India reported diarrhea in 31.7 % and steatorrhea in 85.3 % of patients in their study. El – Falaki et al. 25 from Egypt reported steatorrhea in 66.7 % of CF patients which is much higher than the present study. Pancreatic insufficiency might be less in Bangladeshi population than others due to different genetic variants could be a speculation for this reason and could be a matter of thinking for future researchers in their next studies. Regarding radiological profile, bronchiectasis and consolidation were the significant radiological findings in both X -ray and HRCT of the chest in SCT positive patients (p < .05). Almost similar radiological findings were shown by Aziz DA et al. from Pakistan and Kawoosa et al. from India. 28,20 The presence of bronchiectasis, an end-stage pulmonary disease in the majority of CF patients at the time of diagnosis indicates the delay in diagnosis and advancement of the disease deterioration. The microbiological profile obtained from sputum and throat swab culture revealed the preponderance of P.aeruginosa (57.89 %) and Klebsiella (44.73 %) in SCT positive patients and highly significant statistically ( p = .001). This finding was supported by Indika et al. from Srilanka (60 %) and Shah et al. from Pakistan (87 %). 29,27 Bowler et al. in their study found the growth of this pathogen at a significantly earlier age in Asian patients and may adversely affect the outcome. 30 It was not possible for us to sub type the P. aeruginosa into mucoid or non-mucoid strains. A more accurate pattern of lung infection would emerge from bronchoalveolar lavage (BAL) fluid study after bronchoscopy. Cystic Fibrosis– Is it Extremely Rare or Invariably Missed: An Observational Study in Bangladesh Scenario 24 Year 2022 Global Journal of Medical Research Volume XXII Issue VI Version I ( D ) F © 2022 Global Journals