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Biomedical subjects

B Denham

Publications and source records attributed to B Denham.

At least 19 recordsLinked to original sources
PubMed2001

Bronchoscopically administered recombinant human DNase for lobar atelectasis in cystic fibrosis.

Lobar atelectasis is a common complication of cystic fibrosis. The majority of cases respond to intravenous antibiotics and chest physiotherapy. In a subgroup of patients, atelectasis is resistant to medical therapy, and its persistence in the pediatric population is associated with a poor prognosis. Bronchoscopic instillation of human recombinant DNase expanded atelectatic lobes in three children resistant to at least 2 weeks of medical therapy. This method of administration of DNase has been successful in resistant cases of lobar atelectasis in patients with chronic obstructive pulmonary disease, quadriplegia, and status asthmaticus. Purulent cystic fibrosis sputum has a very high DNA content, and DNA has been shown to become more pourable in vitro when treated with rhDNase. Bronchoscopic instillation of rhDNase should be considered in cases of persistent lobar atelectasis unresponsive to medical therapy.

Bronchoscopy↗
PubMed1995

The Irish cystic fibrosis database.

We have found records of 1014 Irish cystic fibrosis patients alive by December 1994, belonging to 883 families. Prevalence in the population is 1/3475 and incidence at birth 1/1461, with a gene frequency of 2.6%. Twenty percent of the patients are aged over 20 years, but at present survival rate falls rapidly after that age. We have identified 85% of the mutations on the CFTR gene in a sample of 29% of the families (506 CF chromosomes). Mutation delta F508 is found in 72% of Irish CF chromosomes, G551D in 6.9%, and R117H in 2%. These are the highest frequencies reported for the latter two mutations world wide. Another seven mutations are found in an additional 4% of CF families. We present new microsatellite haplotype data that could be useful for genetic counselling of CF families bearing some of the 15% of CF mutations still unidentified, and comment on possible uses of our database.

Adolescent↗
PubMed1993

10 year review of cardiac tumours in childhood.

OBJECTIVE: To review the commonest types of cardiac tumours in childhood, their different presentations, and management. DESIGN: A retrospective study of patients with cardiac tumours. SETTING: Cardiac department of a teaching hospital. PATIENTS: Six patients with an age range between one day to three years presented over a period of 10 years. MAIN OUTCOME MEASURES: To determine different presentations, management, and prognosis of cardiac tumours. RESULTS: Three patients presented with an arrhythmia, two with an asymptomatic heart murmur, and the sixth was discovered accidentally. Surgical resection of the tumours was performed in five cases, one patient died during the operation, one developed new tumours after surgery, and the remaining three have had an excellent result. Histology of those operated on showed benign rhabdomyomas in three and fibromas in the other two. The sixth patient was managed conservatively and the multiple tumours he had showed gradual resolution over a period of 10 months follow up. One baby with rhabdomyoma had signs of tuberous sclerosis whereas the others were normal at follow up five months to three years after diagnosis. None of them had a positive family history. CONCLUSION: Cardiac tumours in childhood are extremely rare. The commonest types are rhabdomyomas, then fibromas. Most cases are diagnosed by echocardiography. The prognosis for most patients is excellent.

Child, Preschool↗
PubMed1991

Intussusception in cystic fibrosis.

Two cases of acute intussusception in older children with cystic fibrosis are reported. Both cases presented with symptoms and signs consistent with meconium ileus equivalent, which delayed the final diagnosis. Both cases required abdominal surgery but made full and uneventful recoveries.

Cystic Fibrosis↗
PubMed1990

Cardiac surgery in Down syndrome.

Between January 1976 and December 1987 42 children with Down syndrome and congenital heart disease underwent cardiac surgery. Four children had two operations. Age at the time of surgery ranged from 11 days to 14 years. The commonest operative procedure was repair of a patent ductus arteriosus. Four patients died post-operatively, two following repair of a complete atrio-ventricular canal defect (CAVD), one following correction of tetralogy of Fallot in association with a CAVD, and a fourth following closure of ventricular septal defect and atrial septal defect. The mortality for those who had open heart surgery was 13.3% and for the series as a whole the mortality was 6.6% over a period of follow-up ranging from two months to four years. A relatively conservative approach has been adopted with regard to surgery, based on the shorter natural expectation of life in Down syndrome, the complexity of many of the cardiac lesions involved and the recognition of the frequency of early intellectual deterioration in Down patients.

Adolescent↗
PubMed1989

Cardiovascular manifestations in Kawasaki disease.

Thirteen patients with Kawasaki disease were reviewed. The average age at presentation was 31 months (range: 6 weeks to 8 years). Mean follow up was 36 months, with a range of 5 to 53 months. Cardiovascular manifestations were present in 9 patients. These comprised pericarditis with myocarditis (2), pericardial effusion (4), coronary artery ectasia (3), coronary artery aneurysms (3), peripheral artery aneurysm (1) and peripheral vasculitis (1). Treatment consisted of aspirin alone in 11 and gammaglobulin with aspirin in 2 patients. One patient with coronary artery involvement died following a myocardial infarction. A large coronary artery aneurysm persists, 31 months after initial detection in another patient. Spontaneous resolution of the cardiovascular complications occurred in the remaining seven patients. The importance of clinical awareness and detailed evaluation and follow up of the cardiovascular system in Kawasaki disease is emphasised. There is also a need, however, to exclude other diagnoses which may have a similar clinical presentation.

Cardiovascular Diseases↗
PubMed1989

Bacterial endocarditis and lymphadenopathy mimicking Kawasaki disease.

Two children whose illness initially fulfilled the clinical criteria for the diagnosis of Kawasaki disease were both subsequently found to have acute bacterial endocarditis and one child had a disseminated non-Hodgkins lymphoma. We describe their clinical course and emphasize the importance of the exclusion of other diagnosis as well as strict adherence to the accepted diagnostic criteria of Kawasaki disease.

Child↗
PubMed1988

Regional choroidal atrophy and alopecia. A new syndrome.

Two siblings with total regional choroidal atrophy and other manifestations of ectodermal dysplasia are presented. The mode of inheritance is uncertain, but mild macular disease in the father may represent heterozygote manifestation of a recessive condition.

Adult↗