Global Journal of Medical Research, F: Diseases, Volume 22 Issue 6
The strength of this study is that, this is the first study in Bangladesh introducing the SCT by quantitative pilocarpine iontophoresis, the gold standard for the diagnosis of CF, using an indigenously developed and validated low-cost equipment instead of performing sweat chloride test by wrapping the whole body and heating for collection of sweat which is obsolete and too risky for young patients. However, the study has some limitations. Only 38 cases could be diagnosed over 3 years and lack of adequate follow-up services and therapeutic modalities. V. C onclusion Cystic fibrosis does occur in the Bangladeshi population far more than anticipation and in the majority of cases, the diagnosis is delayed and at that time the disease is far advanced. A stronger and more structured system is required for proper diagnosis and effective management of this disease. Creating awareness among the physicians about the disease along with adequate training regarding proper sweat collection and chloride estimation in any suspected CF patient is necessary. Also need to develop a management protocol for CF patients based on locally available recourses. Authors contributions: MSK= Conceptualized the study design, collected data and wrote the initial manuscript. FA- Helped in data collection, Microbiology laboratory tasks and revision of manuscript. JK- Helped in data collection and Biochemistry laboratory work. SI- Analyzed and interpreted the data. Critically analyzed the manuscript. MGDH– Statistical analysis and revision of the manuscript. All authors read and approved the final manuscript. Acknowledgements: The authors wish to thank the patients/parents for their participation in the study. Also wish to thank Ibteshum Khaled and Ishmum Khaled for their kind cooperation in computer typing and designing the manuscript. Availability of data and materials: The datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request. Ethics approval and consent to participate: The study was approved by the IRB (NIDCH/EC/09-2017) and written consent was taken from each patient and/or parent. Funding: The authors received no financial support for this research and/or publication of this article. Conflict of Interests: The authors declared no conflicts of interest with respect to the research, authorship and/or publication of this article. R eferences R éférences R eferencias 1. Zvereff VV, Faruki H, Edwards M, Friedman KJ. Cystic fibrosis carrier screening in a North American population. Genetics in Medicine. 2013; 16 (7): 539 – 546. 2. Karem B, Rommens JN, Bichnan JA. Idetification of cystic fibrosis gene. Genatic Anal sci 1989; 245: 1673 – 1680. 3. Frizzell RA. Functions of cystic fibrosis transmembrane conductance regulator protein. Am J Respir Crit Care Med 1995; 151: S 54 – S 58. 4. Rosenstein BJ, Langbaum TS., Misdiagnosis of cystic fibrosis. Need for continuing follow up and re- evaluation. Clin Pediatr. 1987; 26: 78 – 82. 5. LeGrys VA. Sweat testing for the diagnosis of cystic fibrosis: Practical considerations. J Pediatr. 1996; 129: 892-97. 6. Collie JT, Massie RJ, Jones OH, LeGrys VA, Greaves RF. Sixty-five years since the New York heat wave: Advances in sweat testing for Cystic Fibrosis. Pediatr Pulmonol. 2014; 49: 106 -117. 7. Pamela B. Devis. Cystic Fibrosis since 1938. Am J of Res and Criti Med, 2008; 173: 477-82. 8. Dodge JA, Morison S, Lewis PA, Coles EC, Geddes D, Russel G, et al. Incidence, population and survival of cystic fibrosis in UK 1968-95. UKCF Survey Management Committee. Arch Dis Child 1997; 77: 493-496. 9. Hamosh A, FitzSimmons SC, Macek M, Knowles MR, Rosenstein BJ, Cutting GR. Comparison of the clinical manifestations of cystic fibrosis in black and whites. J Pediatrics 1998; 132: 255 – 259. 10. S. K. Kabra, M. Kabra, R. Lodha, S. Shastri. Cystic Fibrosis in India. Pediatric Pulmonology 2007; 42: 1087 – 1094. 11. Kabra SK, Kabra M, Gera S, Lodha R, Sreedevi KN, Chacko S, et al. An indigenously developed method for sweat collection and estimation of chloride for diagnosis of cystic fibrosis. Indian Pediatr. 2002; 39: 1039-1043. 12. Gibson L, Cooke R. A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. Pediatrics 1959;1: 545 -549. 13. Schales O, Schales SS. A simple and accurate method for determination of chloride in biological fluids. J Biol Chem. 1941; 140: 879-884. 14. Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr, 2017; 181 S: S 4 – S 15 e1. PMID: 28129811 15. Kabir ARML, Roy S, Habib RB, Anwar KS, Mollah MAH, Amin R et al. Cystic fibrosis diagnosed using indigenously wrapped sweating technique: First large-scale study reporting sociodemographic, 25 Year 2022 Global Journal of Medical Research Volume XXII Issue VI Version I ( DD ) F © 2022 Global Journals Cystic Fibrosis– Is it Extremely Rare or Invariably Missed: An Observational Study in Bangladesh Scenario
RkJQdWJsaXNoZXIy NTg4NDg=