Growth monitoring to detect children with cystic fibrosis.
نویسندگان
چکیده
BACKGROUND/AIMS Cystic fibrosis (CF) in infancy and childhood is often associated with failure to thrive (FTT). This would suggest that in countries without a newborn screening program for CF, FTT could be used as a clinical screening tool. The aim of this study is to assess the diagnostic performance of FTT for identifying children with CF. METHODS Longitudinal length and weight measurements up to 2.5 years of age were used from CF patients (n = 123) and a reference group (n = 2,151) in The Netherlands. Growth measurements after diagnosis were excluded. We developed five potential screening rules based upon length, weight and body mass index (BMI) standardized by age and gender (SDS). Outcome measures were sensitivity, specificity and positive predictive value (PPV). RESULTS BMI SDS had the highest sensitivity at low false-positive rates. An efficient scenario is a BMI SDS below -2.5 SD in combination with a decrease in BMI SDS of at least 0.5 SD. This scenario had a sensitivity of 32%, a specificity of 98.3% and a PPV of 0.75%. CONCLUSION In the absence of a newborn screening program, young children with FTT for BMI are candidates to consider testing for CF.
منابع مشابه
Prevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran
Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...
متن کاملThe Role of Growth Hormone on Growth of Children with Cystic Fibrosis
Dear Editor,In spite of recent advances in treatment of infection and emphasis on adequate nutritional intake, patients with cystic fibrosis (CF) frequently have less than ideal body weight and adults are often shorter than their target height.1Owing to a state of relative growth hormone (GH) insensitivity, the anabolic and growth promoting effects of recombinant human GH (rhGH) treatment have ...
متن کاملInvestigation of Growth of Microorganisms within the Spacers of Children with Cystic Fibrosis
Background and aim: Cystic fibrosis is a monogenic disorder in several systems. Since spacers used by these patients may be used several times a day, many of them do not know how to wash and use them, so these devices are good places for bacteria to grow. Therefore, this study was performed to identify and determine the relationship between the growth of microorganisms inside spacers and the ef...
متن کاملCardiac Involvement in Mild Cystic Fibrosis Lung Involvement Assessed by Tissue‐Doppler Echocardiography
Background:Cystic Fibrosis (CF) is a systemic disease affecting extra pulmonary dysfunction as a result of CF-related lung disease. Because of lack of enough studies in this field and utilization of TDE in this field, we aimed to evaluate t...
متن کاملThe Effect of Nutritional Consultation on the Level of Malnutrition and Growth Indices in Children with Cystic Fibrosis
Background: Malnutrition is one of the most common disorders among children with Cystic Fibrosis (CF). The present study aimed to investigate the effect of nutritional consultation on the type and severity of malnutrition in children with CF. Materials and Methods: This quasi-experimental stud...
متن کاملOutcome of Cystic Fibrosis in Patients with Bronchiectasis
Introduction: Bronchiectasis is a common problem in children especially under 5 years. Early diagnosis of disease and its causes could be useful in early treatment and preventing probable complications. This study aimed at evaluating the cystic fibrosis (CF) in patients with bronchiectasis. Methods: In a cross-sectional study, 374 children with bronchiectasis were studied. The diagnosis was ma...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Hormone research
دوره 72 4 شماره
صفحات -
تاریخ انتشار 2009