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C M Mellis

Publications and source records attributed to C M Mellis.

At least 73 records · Page 4Linked to original sources

Diagnostic delay in cystic fibrosis: lessons from newborn screening.

Newborn screening for cystic fibrosis (CF) by dried blood spot immunoreactive trypsin (IRT) assay is now feasible, but the benefits are disputed. We have studied the symptoms and signs at diagnosis in 48 babies detected during a newborn screening programme, and also the delay between presentation with symptoms and diagnosis in all 33 babies diagnosed at our CF clinic in the two years before screening began. Eleven of the 48 screened babies had meconium ileus, 16 had gastrointestinal symptoms only, and 14 had both respiratory and gastrointestinal symptoms at the time of diagnosis. Five of the remaining 7 babies developed clear cut symptoms or signs soon after diagnosis. Thus, 96% (46 of 48) of the babies had symptoms by 3 months of age. Of the 33 infants diagnosed clinically in the two years immediately before screening, 13 (39%) were over 12 months of age at diagnosis. Moreover, the mean delay between presentation with symptoms and diagnosis of CF in these infants was 2.6 years. Our data show that the delay between onset of symptoms and diagnosis is far greater than previously supposed and that most babies detected by our screening programme already had symptoms that warranted treatment at the time of their diagnosis.

Age Factors↗

Pseudomembranous croup.

During a 2-year period, 7 children were seen with a severe form of laryngotracheobronchitis associated with sloughing of the respiratory epithelium and profuse mucopurulent secretions. We have called this condition pseudomembranous croup. The children had severe upper airways obstruction, appeared toxic with high fever, and were older than the typical age group for viral laryngotracheobronchitis. Lateral x-ray films of the airways showed subglottic narrowing and often these suggested the presence of radio-opaque foreign material in the tracheal lumen. At endoscopy, in addition to pseudomembrane in the subglottic region and trachea, there was thick mucopus and debris, and in some cases these changes extended into the bronchi. An artificial airway was required in all except one, and even after intubation it proved difficult to maintain the airway. Staphylococcus aureus was the most common pathogen isolated from tracheal cultures but other organisms were grown.

Airway Obstruction↗

Immediate food hypersensitivity reactions on the first known exposure to the food.

We report 8 infants with immediate hypersensitivity reactions to foods (milk, egg, or peanut), occurring at the first-known exposure. Each developed symptoms within the first hour, but these generally settled within 2 hours. Sensitisation to the food concerned was demonstrated by positive immediate allergen skin prick tests in every case. Symptoms experienced included irritability, erythematous rash, urticaria, angio-oedema, vomiting, rhinorrhoea, and cough. Five infants were being followed prospectively and 4 were clinically tolerant of the food by age 16 months. The most likely route of sensitisation was via breast milk. None of the infants experienced similar reactions while being breast fed, suggesting that the reaction was dose dependent. As 5 out of a group of 80 infants being followed prospectively developed an immediate reaction at their first known exposure to a food, this appeared to be a not uncommon presentation of food hypersensitivity in infancy.

Allergens↗

The significance of nasal eosinophils and mast cells in children with nasal symptoms.

Nasal smears from children with symptoms of nasal obstruction and/or discharge were examined for the presence of eosinophils, mucus-containing cells and mast cells. The presence of more than one eosinophil or any mast cells was significantly associated with atopy as determined by immediate hypersensitivity on skin prick testing. Twelve children with markedly increased numbers of nasal smear mast cells are described. In these children symptoms frequently commenced in the first 6 months of life. Nasal eosinophilia was not noted in any of the cases. Nasal smear mastocytosis was associated with significant perennial symptoms and would be missed if nasal smears are examined only for eosinophilia.

Child↗

Comparison of peak expiratory flow rate and forced expiratory volume in one second in histamine challenge studies in children.

The difficulty in performing FEV1 in young children prompted us to assess whether PEFR could be used to measure the response to inhaled histamine. In 123 children with recurrent respiratory symptoms we found a highly significant correlation (r = 0.83, P less than 0.001) between the percentage fall from base-line of FEV1 and PEFR. We also performed histamine challenge tests using PEFR alone on 56 children too young to manage spirometry and found our procedure reliable. PEFR is an adequate measurement in histamine provocation studies and can be used interchangeably with FEV1. The criterion for a positive test is a 20% fall in either PEFR or FEV1.

Adolescent↗

Antimicrobial therapy in whooping cough.

We compared the ability of erythromycin and co-trimoxazole in clearing Bordetella pertussis from the nasopharynx of 22 children admitted to hospital with whooping cough. Both agents appeared effective. Nevertheless, 10 of the 22 patients gave positive cultures four or more days after antimicrobial administration was begun.

Bordetella pertussis↗

Value of combining B2 sympathomimetic metered aerosol and oral theophylline in children with asthma.

We report the results of a double blind controlled trial on 12 children with clinical asthma. Each child received three different treatment regimens; terbutaline sulphate 0.5 mg (Bricanyl) via a metered aerosol; orally administered theophylline 5 mg/kg (Somophyllin); or a combination of both. As expected, the metered aerosol therapy produced significantly greater early bronchodilatation than therapy with theophylline alone. In addition, the combined therapy produced a significantly greater bronchodilatation after three hours than the metered aerosol alone (P less than 0.05). This trend was also observed after one and two hours, but did not reach significance (0.1 greater than P greater than 0.05). We conclude that there is value in combining a B2 sympathomimetic metered aerosol and oral theophylline in children with asthma.

Administration, Oral↗

Bronchial reactivity in cystic fibrosis with normal pulmonary function.

We studied 20 children with cystic fibrosis who had relatively normal pulmonary function (forced expiratory volume greater than or equal to 70% of predicted) to determine whether demonstration of heightened bronchial reactivity in this group reflects coexistent asthma. Using a standardized histamine provocation, we found that eight (40%) had a positive response. These responders were significantly younger than the nonresponders, had a greater incidence of clinical asthma, and all were atopic on skin testing. However, they had a significantly higher ratio of residual volume to total lung capacity than the nonresponders. Although as a group they were less sensitive to histamine than children with asthma, four of the responders with cystic fibrosis had histamine sensitivity similar to a group of asthmatic patients. We concluded that, in the presence of mild lung disease, the demonstration of heightened bronchial reactivity in children with cystic fibrosis may provide confirmatory evidence of coexistent asthma, particularly if the children are highly sensitive to histamine.

Adolescent↗

Allergen skin-prick testing in asthmatic children.

Allergen skin-prick tests to the common airborne allergens were performed on 534 children with asthma. A positive reaction to one or more allergens was observed in 81%, and the prevalence increased with age. The specific allergen reactions were characteristic with a high prevalence of reactions to house-dust mite (Dermatophagoides pteronyssinus) (88%), house dust (79%, and cat fur (62%), and a low prevalence of grass-pollen reactions (12% to 25%). We found that the diagnosis of atopy could be made in virtually all (96%) of these children by using only three allergens: D. pteronyssinus, cat fur, and rye grass.

Adolescent↗