Cystic Fibrosis - An Update

Ahsan, Mohammed Rizwanul; Alam, Hossain Shahid Kamrul; Hasan, Ahmed Rashedul; Islam, Mirza Ziaul; Hossain, A

doi:10.3329/bjch.v40i3.33060

Cited by 2 publications

(4 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…While Kabra et al conducted an 8-years long (1995-2002) study in India 11 nothing much was done in Bangladesh, except 3 short drives. While the 1st one remains a simple literature review (2005), 17 the 2nd one was a report on 2 CF-cases only (2016). 24 However, Probir et al gave a 3rd drive in the form of a leading article 25 yet a review.…”

Section: Principal Driven Force For Conducting the Studymentioning

confidence: 99%

“…Unfortunately, to our knowledge there is no organized data-base system for CF in developing countries including Bangladesh until now, mainly due to non-existent of broad-based research-which Ahasan et al pointed out rightly 17 and because of limited knowledge and skill on CF (among physicians). Thus, it is perceived that a lot of children with CF remain undiagnosed and do not receive structured management of CF and succumb to death.…”

Section: Introductionmentioning

confidence: 99%

“…11,12 So, it is logically presumed that CF cases are either missed by the physicians due to knowledge gap, as our recent observation of a KAP-study 13 or often delayed by parental lack of awareness in seeking doctor's advice in time-that Kabra et al assumes due to lower suspicion index. 4,5,[14][15][16] Unfortunately, to our knowledge there is no organized data-base system for CF in developing countries including Bangladesh until now, mainly due to nonexistent of broad-based research-which Ahasan et al pointed out rightly 17 and because of limited knowledge and skill on CF (among physicians). Thus, it is perceived that a lot of children with CF remain undiagnosed and do not receive structured management of CF and succumb to death.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country

Kabir

Roy

Habib

et al. 2020

Global Pediatric Health

View full text Add to dashboard Cite

Due to lack of robust data on childhood cystic fibrosis (CF) in Bangladesh we sought to evaluate their clinico-epidemiology. A cross-sectional observation was conducted adopting CF-foundation consensus-panel-diagnostic criteria in 3 tertiary-care-hospitals in Bangladesh from 2000 to 2017. Clinically suspected 95 CF-cases were subjected to sweat-chloride testing using locally-developed a fast, cheap and effective indigenously body-wrapped sweating technique measured by US-Easy Lyte-automated microprocessor-controlled analyzer marking ≥60 mmol/L as positive. Mean-age of CF-cases at disease-onset was 16.9 ± 26.6 months that significantly differed with age-at-diagnosis ( P < .02). Pulmonary syndromes included chronic wet cough in 100%, respiratory distress in 90.5%, digital-clubbing in 78%, mucopurulent-sputum in 74%-cases, and crepitation in 82%. Radio-imaging revealed bronchiectasis in 60%, hyperinflation/peribronchial-thickening in 22% and, pan-sinusitis in 89%-cases. While 37% had history-of malabsorption, high-fecal-fat revealed in 53%-cases. Malnutrition prevailed as severe-underweight in 87%-cases and all CF-cases (100%) had high sweat-chloride (mean = 118 ± 53.34 mmol/L). Thus, children with pulmonary features coupled with severe malnutrition and associated radio-imaging bronchiectasis should be screened for CF with a fast, cheap and effective sweat test in resource poor settings.

show abstract

Section: Principal Driven Force For Conducting the Studymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country

Kabir

Roy

Habib

et al. 2020

Global Pediatric Health

View full text Add to dashboard Cite

show abstract

“…Cough occurs mainly due to repeated infection of the airway. Hence, recurrent respiratory infections and failure to thrive are the most commonly reported symptoms in studies involving CF [15,16,[19][20][21]. Children with CF frequently pass bulky offensive stools due to fat malabsorption from the gut, and malnutrition occurs due to inadequate absorption of proteins, fats, and fat-soluble vitamins because of pancreatic insu ciency [22].…”

Section: Resultsmentioning

confidence: 99%

Clinico-Epidemiological Characteristics of Children with Cystic Fibrosis: A Tertiary Care Experience

Sarkar

Akand

Tahura

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Cystic fibrosis (CF) is considered to be rare among individuals from Bangladesh. The objective of the study was to delineate the clinico-epidemiological characteristics of pediatric cystic fibrosis cases.Method: This observational study included pediatric patients (up to 14 years of age) with a clinical diagnosis of CF. Data were collected within the period from February 2021 and October 2021. Written informed consent was obtained from the accompanying parent. Clinical and epidemiological characteristics were analyzed on the basis of demographic data, medical history, laboratory tests, and outcome information. Data analysis was done with SPSS 26.Result: A total of 50 patients (66% male) with a mean age of 39.7 ±30.75 (SD) months were included. Twenty-eight patients (57.14%) had siblings with CF, and 41.67% of parents had a history of consanguineous marriage. The majority of them were stunted (86%) and underweight (86%), and half of them had wasting (54%). Median disease duration was 12 months (range: 2 – 72). Cough (100%) and purulent sputum (100%) were the predominant respiratory symptoms, while failure to thrive (98%) and bulky offensive stools (86%) were prime gastrointestinal symptoms. Among the signs, malnutrition (94%), short stature (72%), digital clubbing (64%), and bronchiectasis (40%) were most frequent. Pulmonary hypertension (48%, n=24) was the most common comorbidity identified in the study participants. In hospital, mortality was 16% (n=8). Digital clubbing, bronchiectasis, pancreatic insufficiency, and abnormal liver function tests were significantly higher in the patient who died.Conclusion: Children with cystic fibrosis most commonly present with undernutrition and respiratory symptoms. Failure to thrive was almost a global phenomenon. Pulmonary hypertension was the most common complication found in echocardiography.

show abstract

Cystic Fibrosis - An Update

Abstract: Cystic fibrosis (CF) is said to be the most common lethal inherited

Cited by 2 publications

References 18 publications

Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country

Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country

Clinico-Epidemiological Characteristics of Children with Cystic Fibrosis: A Tertiary Care Experience

Contact Info

Product

Resources

About