Allergic bronchopulmonary aspergillosis, known to be associated with cystic fibrosis in older patients, occurred in 7 young atopic children with cystic fibrosis. The diagnosis was suggested by the onset of, or the increase in, asthmatic symptoms accompanied by major chest x-ray changes ranging from total collapse of a lung or lobe to extensive but changing areas of consolidation. Each of the ch...

BACKGROUND Radiological estimates of hyperinflation are used in several clinical and radiographic scoring systems for cystic fibrosis, but it is not known if these estimates of hyperinflation are related to measured total lung capacity. METHODS Comparison was made of independent clinical estimates of hyperinflation from chest radiographs with objective plethysmographic and radiographic measur...

When a mother has a child with a chronic impairment, the occupational demands of mothering extend to address the specialized needs of that child. This research explores how the type of hidden impairment in a child influences family routines and occupations. This qualitative study consisted of interviews with 22 mothers of children with attention deficit hyperactivity disorder (ADHD), a behavior...

Children with cystic fibrosis frequently have nasal polyps and sinusitis. This study addresses (para-) nasal disease in 39 adult cystic fibrosis patients. Fifteen patients (39%) had recently had serious nasal symptoms and 26% sinusitis. Seventeen (44%) had nasal polyposis. Almost all sinus radiographs taken showed opacification, which was unrelated to symptoms. Polypectomies and antral irrigati...

Cystic fibrosis is one of the commonest genetic diseases among Caucasians and represents an important cause of suffering and death among children and adults. In the past two decades marked prolongation of the life of patients with cystic fibrosis has been achieved as the result of improved case-finding and an extensive regimen of therapies. More recently, a variety of new approaches to therapy ...

Newborn screening for cystic fibrosis promotes early diagnosis and treatment, leading to a reduction in the number of late diagnoses [1]. This is of benefit to patients but contributes to physicians being less aware of the diverse ways in which unscreened patients present. We report a 12-year-old male who presented with pneumomediastinum and hyponatraemic dehydration, in whom subsequent investi...

AIM To examine the gastrointestinal handling and metabolic disposal of emulsified [1-13C]palmitic acid esterified into a triglyceride in nine healthy children and seven patients with cystic fibrosis on enzyme replacement treatment. METHODS After an overnight fast, each child was given 10 mg/kg body weight [1,1,1-13C]tripalmitin with a standardised test meal of low natural 13C abundance. The t...

Aim—To examine the gastrointestinal handling and metabolic disposal of emulsified [1-C]palmitic acid esterified into a triglyceride in nine healthy children and seven patients with cystic fibrosis on enzyme replacement treatment. Methods—After an overnight fast, each child was given 10 mg/kg body weight [1,1,1-C]tripalmitin with a standardised test meal of low natural C abundance. The total enr...

BACKGROUND Mycobacterium chimaera is a recently described species within the Mycobacterium avium complex. Its pathogenicity in respiratory tract infection remains disputed. It has never been isolated during cystic fibrosis respiratory tract infection. CASE PRESENTATION An 11-year-old boy of Asian ethnicity who was born on Réunion Island presented to our hospital with cystic fibrosis after a d...

PURPOSE Classic cystic fibrosis is now known part of cystic fibrosis transmembrane conductance regulator (CFTR)-related disorders. These include a wide spectrum, from multi-system disorders, such as cystic fibrosis, to mono-symptomatic conditions, such as chronic pancreatitis or congenital bilateral absence of the vas deferens. However, respiratory disease is considered typical for the multi sy...