Clinical manifestation, laboratory findings, and outcome of children with cystic fibrosis over a 10-year period in South Iran

Ziyaee, Fateme; Dehghani, Seyed Mohsen; Hosseini, S; Zohalinezhad, Mohammad Ebrahim; Aleyasin, Soheyla; Javaherizadeh, Hazhir

doi:10.1186/s43054-020-00045-9

Cited by 3 publications

(2 citation statements)

References 32 publications

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“…In this study, 62% of the cases were male, which is consistent with the findings of Aziz et al (9), where 58.13% of CF children were male, similar to the current study. In the current study, 40.7% of patients had a sibling with CF; however, in Ziyaee et al's study, only 13.3% of CF patients had a sibling with CF (10).…”

Section: Discussioncontrasting

confidence: 75%

Vitamin D Level, Weight, Height, and BMI Among Children with Cystic Fibrosis: A Retrospective Study

Ahmadi,

Mirkarimi,

Khanifar

et al. 2023

Jundishapur J Chronic Dis Care

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Background: Cystic fibrosis (CF) stands as the most common chronic multisystem and fatal inherited disease. Objectives: The present study aimed to assess the clinical presentation and laboratory findings in children with CF. Methods: This retrospective cross-sectional study reviewed the records of CF patients over a 2-year period beginning in 2018. The diagnosis of CF was confirmed based on clinical manifestations, sweat chloride tests, or genetic studies. Children aged ≥ 2 years were included; nevertheless, patients without sweat chloride tests or genetic studies were excluded. This study recorded demographic features, gastrointestinal manifestations, vitamin D levels, and the number of hospital admissions. A pediatric gastroenterologist conducted fecal elastase and stool fat analyses to evaluate pancreatic insufficiency. Vitamin D levels < 30 ng/mL were considered vitamin D insufficiency; however, levels < 20 ng/mL indicated a vitamin D deficiency. A P-value < 0.05 was considered statistically significant. Results: This study included 59 children (37 male and 22 female) in the study. Low weight, height, and body mass index (BMI) percentiles (< 5) were most frequently observed. Approximately 86.4% of the patients had consanguineous parents, and 40.7% had affected siblings. Failure to thrive (FTT) (49.2%) and recurrent respiratory infections (45.8%) were the most common clinical presentations. A majority (57.7%) of the patients were diagnosed before the age of 1 year. The mean number of admissions in the past 2 years was 3, and 58% of the cases reported gastrointestinal symptoms. A significant correlation was observed between vitamin D levels and the number of hospital admissions (Pearson coefficient = 0.298, P = 0.042). Conclusions: In this study, there was a high prevalence of vitamin D insufficiency and FTT in children with CF. Additionally, there was a significant correlation between vitamin D levels and the number of hospital admissions over a 2-year period.

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Section: Discussioncontrasting

confidence: 75%

Vitamin D Level, Weight, Height, and BMI Among Children with Cystic Fibrosis: A Retrospective Study

Ahmadi,

Mirkarimi,

Khanifar

et al. 2023

Jundishapur J Chronic Dis Care

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“…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

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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.

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Demography of Cystic Fibrosis at a Kashmiri Specialty Hospital

Malik¹,

Wani²,

Farooq³

et al. 2023

ijsr

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INTRODUCTION Cystic brosis (CF) is a recessive genetic disorder caused by a mutation in the epithelial chloride channel—CFTR (CFTR). Our study's primary objective was to examine the demographic prole of cystic brosis at our specialised hospital. MATERIAL AND METHODS The current research was a hospital-based observational study of individuals suspected of having cystic brosis undertaken at the G.B. Pant Children's Hospital in Srinagar between 2019 and 2022. In this research, 200 patients were chosen according to inclusion and exclusion criteria. RESULTS Out of 25, thirteen of our patients were male and twelve were female, for a ratio of 58% males to 42% females. 40% of the patients in our research were between 1 and 24 months of age. 60% were offspring of non-consanguineous marriage. Positive patients who were married consanguineously were third-degree relatives. During the course of our investigation, we discovered that the majority of patients were from Kupwara, totaling 25% of the study group. In our study, 72% of patients presented with recurrent respiratory tract infections. CONCLUSION In our study we concluded that Cystic brosis in Kashmir is more prevalent in men with non-consanguineous marriages and in the Kupwara district.

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Clinical manifestation, laboratory findings, and outcome of children with cystic fibrosis over a 10-year period in South Iran

Cited by 3 publications

References 32 publications

Vitamin D Level, Weight, Height, and BMI Among Children with Cystic Fibrosis: A Retrospective Study

Vitamin D Level, Weight, Height, and BMI Among Children with Cystic Fibrosis: A Retrospective Study

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

Demography of Cystic Fibrosis at a Kashmiri Specialty Hospital

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