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Biomedical subjects

J A Kuzemko

Publications and source records attributed to J A Kuzemko.

At least 19 recordsLinked to original sources
PubMed1994

Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period.

All newborn infants in East Anglia are screened for cystic fibrosis by blood immunoreactive trypsin assay at 7 days. Thirty eight infants with cystic fibrosis were randomised to treatment with either continuous oral flucloxacillin 250 mg/day (group P, n = 18) or with episodic antimicrobials as clinically indicated (group E, n = 20). Their progress was monitored from diagnosis to 24 months by a nurse coordinator who visited all infants regularly, at home and in hospital, to collect anthropometric, dietary, clinical, and microbiological data. Mean (range) age of confirmation of diagnosis was 5.7 weeks (1-14 weeks). There was no significant difference in birth weight, genotype, immunoreactive trypsin concentration, neonatal history, symptoms at diagnosis, pancreatic enzyme supplementation, or parental smoking history between the groups. Infants in group E had more frequent cough and a greater number of Staphylococcus aureus isolates than infants in group P. More infants of group E were admitted to hospital, had higher admission rates during the second year (19 v 5), for longer periods (6.4 v 2.2 days), despite receiving more than double the number of courses of antibiotics than group P infants (in addition to flucloxacillin). Continuous prophylactic flucloxacillin from early diagnosis of cystic fibrosis is associated with improved clinical progress during the first two years of life.

Bacterial Infections↗
PubMed1993

Cystic fibrosis identified by neonatal screening: incidence, genotype, and early natural history.

The incidence of cystic fibrosis over the last 10 years in East Anglia (a region of the United Kingdom with a population of 2.1 million) has halved. This has happened during the establishment of a neonatal screening programme, which has enabled early diagnosis, genetic counselling, and lately the option of prenatal diagnosis in subsequent pregnancies. One hundred and seven children were born with cystic fibrosis between 1981 and 1990, eight of whom were siblings. The Guthrie blood spots of 82 infants detected by neonatal immunoreactive trypsin screening between 1981 and 1990 were examined for the presence of the most common cystic fibrosis gene mutation (delta F508). It was present in 135 (82%) of the 164 cystic fibrosis genes analysed with 54 (66%) cases being homozygous and 27 (33%) heterozygous. Sixty nine per cent of infants were symptomatic at the time of diagnosis regardless of genotype. No association was found between the early clinical or biochemical features of the disease and homozygosity or heterozygosity for this mutation. Screening for cystic fibrosis using the blood immunoreactive trypsin assay alone remains an effective method of identifying infants with the disease soon after birth, thereby allowing early therapeutic intervention. Genetic counselling and prenatal diagnosis have contributed to a reduction in the number of children born with cystic fibrosis, but may not entirely explain the decreasing incidence of the disease.

Cystic Fibrosis↗
PubMed1989

Twenty years of sodium cromoglycate treatment: a short review.

Sodium cromoglycate (Intal) was first synthesized from khellin, a naturally occurring plant chromone, by Roger Altounyan and his colleagues in 1965. It was introduced as a therapeutic agent in 1968 and marked a new era in the management of asthma. Numerous studies on the use of sodium cromoglycate in the treatment of asthma have since been published. Both short-term and long-term controlled studies indicate unequivocally that sodium cromoglycate is of significant clinical benefit in 60-70% of asthmatic children and adults. Clinical experience during the last 20 years has indicated that it should be the drug of first choice in the short-term treatment of asthma provoked by irritants, allergens and exercise, as well as in chronic asthma. Side effects are usually minor and sodium cromoglycate is the safest therapeutic agent in current use for the treatment of asthma.

Asthma↗
PubMed1988

Does monotherapy of pulmonary infections in cystic fibrosis lead to early development of resistant strains of Pseudomonas aeruginosa?

We report the development of ceftazidime-resistant strains of Pseudomonas aeruginosa in a small population of cystic fibrosis patients who had ceftazidime monotherapy over a 5-year period as clinically indicated. The background rate of less than 30% of patients with a ceftazidime-resistant strain of P. aeruginosa in their sputum each month is similar to the resistance rate to other anti-pseudomonas antibiotics that have seldom been used here. In practice this resistance has meant that for about 10% of patients each month consideration has to be given to the use of an agent other than ceftazidime.

Ceftazidime↗
PubMed1988

Home treatment of pulmonary infection in cystic fibrosis.

The concept of home care therapy for acute respiratory exacerbations in cystic fibrosis (CF) has been developed in a number of treatment centers during the last few years. Objective and prospective clinical observations have demonstrated that home care therapy is as effective as structured hospital treatments in improving pulmonary function and body weight in patients with CF. Moreover, the medical, social, and financial benefits of home care suggest that such an approach to the management should be developed further in order to improve the quality of life of patients with CF.

Acute Disease↗
PubMed1984

Improved sweat test method for the diagnosis of cystic fibrosis.

We describe a new technique of collecting sweat for measurement of osmolality and sodium concentrations. Eighty two subjects were studied--39 controls and 43 patients with cystic fibrosis. Adequate amounts of sweat were obtained in 81 subjects and sweat was analysed for both osmolality and sodium concentrations in 73 subjects. The 34 controls gave sweat osmolality and sodium values ranging from 62 to 196 mmol/kg and 9 to 72 mmol/l respectively. The 39 cystic fibrosis patients gave osmolality values ranging from 220 to 416 mmol/kg and sodium concentrations ranging from 60 to 150 mmol/l. Sweat osmolality alone was determined in eight infants under 50 days of age--four later developed the clinical features of cystic fibrosis and four, in whom cystic fibrosis was suspected, were later excluded. Sweat osmolality values in these two groups ranged from 255 to 345 mmol/kg and 87 to 123 mmol/kg respectively. The simplicity of collecting sweat and the measurement of osmolality offer distinct advantages over techniques previously described.

Adolescent↗
PubMed1982

Screening for cystic fibrosis by died blood spot trypsin assay.

Immunoreactive trypsin was measured in dried blood specimens from 14,000 infants. A second test was performed in 0 . 2% of the population in whom blood trypsin levels were greater than 80 ng/ml. Five infants with cystic fibrosis were then detected, with only one case of persistent hypertrypsinaemia in whom this diagnosis could not be established. No false-negative test results have yet been identified. Seventeen infants with cystic fibrosis were tested inthe first 2 weeks of life, only one of whom had a blood trypsin concentration less than 80 ng/ml.

Cystic Fibrosis↗
PubMed1979

Sensitive trypsin assay for dried-blood specimens as a screening procedure for early detection of cystic fibrosis.

An immunoreactive-trypsin assay uses small dried-blood spots (diameter 1.25 mm) and is therefore suitable for incorporation in established neonatal screening schemes. Blood specimens from neonates with cystic fibrosis had trypsin levels greater than those in control subjects, thus confirming earlier findings. Trypsin levels were below normal in several older patients with cystic fibrosis.

Adolescent↗