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Biomedical subjects

M X Fitzgerald

Publications and source records attributed to M X Fitzgerald.

At least 55 records · Page 3Linked to original sources

Semi-quantitative gallium-67 lung scanning as a measure of the intensity of alveolitis in pulmonary sarcoidosis.

Gallium-67 (67Ga) lung scanning has been applied for some years in pulmonary sarcoidosis to assess the activity of the alveolitis. Interpreting the scans, however, is difficult due to the low uptake of 67Ga in the disease process relative to background activity. In this study we have measured the mean parenchymal lung activity of 67Ga and have compared the lung activity to that at three remote sites, the liver, the abdomen and the thigh. The results obtained were compared with the percentage of lymphocytes in broncho-alveolar lavage fluid. There was a very good correlation with the lung-to-thigh uptake measurements and a much poorer correlation with the lung-to-liver and the lung-to-abdomen measurements. It was observed that steroid therapy reduced dramatically the correlation between the broncho-alveolar lavage findings and the 67Ga scan measurements. The results suggest that in patients not on steroid therapy, the 67Ga lung-to-thigh measurements may be used, similarly to the broncho-alveolar lavage lymphocyte counts, to identify those with high-intensity alveolitis from those with low-intensity alveolitis.

Adult↗

Ventilation and gas exchange during sleep in patients with interstitial lung disease.

Ventilation and gas exchange during overnight sleep was studied in a group of seven patients with severe interstitial lung disease (mean vital capacity 50%, mean diffusing capacity 46% predicted), to see whether clinically significant oxygen desaturation occurred. Patients with a history of loud snoring or clinically significant airflow obstruction were excluded. Sleep was fragmented in these patients, but all achieved rapid eye movement (REM) sleep. All patients showed episodes of oxygen desaturation during sleep--mean (SEM) awake arterial oxygen saturation (SaO2) was 92.9% (0.3%) compared with a mean minimum SaO2 during sleep of 83.2% (2.1%) (p less than 0.01). These episodes were, however, transient, and mean SaO2 showed only a slight fall between wakefulness and sleep (non-REM 91.5%, REM 90.4%; NS). Furthermore, SaO2 during non-REM sleep correlated well (p less than 0.001) with SaO2 during wakefulness. Respiratory frequency showed a significant fall between wakefulness and sleep--21.1 (1.8) versus 17.3 (1.5) breaths per minute (p less than 0.02). Our data suggest that nocturnal oxygen treatment need not be considered in patients with interstitial lung disease unless the level of oxygenation while they are awake indicates the need for such treatment.

Humans↗

High prevalence of familial sarcoidosis in an Irish population.

Previous studies in the Republic of Ireland have demonstrated a high national prevalence of sarcoidosis. Observations in our sarcoid clinic suggested a high prevalence of the disease among siblings and prompted a survey to quantify this phenomenon. The study group comprised 114 index patients with biopsy proved sarcoidosis and a total sibling pool of 534 individuals. Eleven of the index patients (9.6%) were found to have at least one sibling with sarcoidosis. Of the 13 siblings thus identified, eight had biopsy proof of the disease, while the remaining five showed highly suggestive clinical and radiological evidence of sarcoidosis. There was no significant difference in the mode of presentation between the sibling pairs or between familial and non-familial cases and there was an equal distribution of like sex and unlike sex pairs. In only two instances was the temporal profile of onset of the disease suggestive of intrafamilial spread of a transmissible agent. The high prevalence of sarcoidosis among siblings reported here (2.4%) suggests that genetic factors significantly predispose to the development of sarcoidosis and that family members of affected patients should be screened for this disease.

Adolescent↗

Atopy, immunological changes, and respiratory function in bronchiectasis.

Cystic fibrosis has been reported to be associated with an increased prevalence of atopy and reversible airways obstruction. To determine whether such features can also result from other chronic suppurative lung infections, we studied 23 patients with proved bronchiectasis, and 23 age and sex matched normal controls. A personal or family history of atopy was reported with equal frequency in the two groups. Although the groups displayed a similar prevalence of positive immediate hypersensitivity skinprick test responses, the positive patients reacted to more antigens (p less than 0.05) and had larger weal diameters (p less than 0.01) than the positive controls. Other indices, such as blood eosinophil counts and serum IgE, did not differ significantly. Serum concentrations of immunoglobulins G, A, and M and of the four IgG subclasses tended to be higher in patients than controls, but only in the case of IgA (p less than 0.01) was this difference significant. No case of IgG subclass deficiency was noted. The patients displayed significant airflow obstruction, the mean basal one second forced expiratory volume (FEV1), forced vital capacity (FVC), and peak expiratory flow rate (PEFR) being 67%, 77%, and 67% of their predicted values. There was evidence of a significant reversible obstructive component in that FEV1 or PEFR or both increased by 15% or more in nine of the 23 patients after inhalation of fenoterol, the mean increases in FEV1, FVC, and PEFR for the whole group being 9.5%, 11%, and 16.9%. These results indicate that while bronchiectasis provokes a hyperimmune response it differs from cystic fibrosis in that there is no significant increase in the prevalence of atopy. The finding of reversible airways obstruction, however, suggests that bronchodilators may be useful adjuncts to treatment.

Adolescent↗

Meconium ileus equivalent in older patients with cystic fibrosis.

Meconium ileus equivalent is one of the lesser known manifestations of cystic fibrosis, and occurs most often in older patients. With the improved overall survival of patients with cystic fibrosis, one would expect to see this condition more often in the future. Of 53 patients attending our cystic fibrosis clinic for adolescents and adults, eight had experienced a total of 25 episodes of meconium ileus equivalent. Recurrent attacks occurred in seven patients, of whom four had at least four separate, well documented episodes: these episodes were associated with obvious recognised precipitating factors in only three patients. All patients responded to appropriate medical treatment with acetylcysteine or sodium diatrizoate, including three who had previously undergone surgery for meconium ileus equivalent elsewhere. Controversies remain, however, concerning the role of prophylactic medical management with acetylcysteine, sodium diatrizoate, and pancreatic supplements.

Acetylcysteine↗

Agnogenic myeloid metaplasia preceded by repeated leukemoid reactions and persistent acquired Pelger-Huët anomaly of granulocytes: case report with review of acquired Pelger-Huët anomaly.

A case report of agnogenic myeloid metaplasia (AMM) is presented in which a patient had a preliminary 18-month period characterized by leukemoid reactions to repeated infections alternating with normal health, throughout which an acquired Pelger-Huët anomaly persisted. This is the only reported case of the anomaly as a premonitory hematologic marker of AMM. The literature concerning the acquired Pelger-Hut anomaly is reviewed.

Bone Marrow Examination↗

Does pseudomonas cross-infection occur between cystic-fibrosis patients.

Over a 12-months period respiratory Pseudomonas aeruginosa isolated from CF patients were typed by serology and pyocin production to determine whether cross-infection was occurring. Results of typing were interpreted in relation to the degree of contact patients had with each other. One strain appeared in 4 unrelated patients. However, since none of these patients had been in contact with each other the strains considered to have been acquired from the environment. Each of six pairs of siblings shared the same strain, but the pairs of strains were distinct from each other. These results suggest that the environment is the most important source of Pseudomonas strains for CF patients and that for cross-infection to occur prolonged intimate contact is required.

Adolescent↗

Atopy and cystic fibrosis. A study of CF sibling pairs and their families.

We have investigated the prevalence of atopy within families of cystic fibrosis (CF) patients and compared its frequence in CF patients, their parents and their non-CF siblings. By studying families with two CF patients it was also possible to evaluate the factors influencing the development of atopy in one CF patient relative to the other patient within the same family. A significant correlation with age (P less than 0.001) for skin test positivity to common allergens within CF sibling pairs was observed. In 14/18 families studied, only the older CF patient was atopic. The presence of atopy in CF patients was independent of the atopic status of their parents.

Adolescent↗

Atopy and bronchial reactivity in older patients with cystic fibrosis.

We studied 25 adolescent and adult patients with cystic fibrosis (CF) and 25 control subjects to determine if the prevalence of atopy and bronchial hyperreactivity was increased in this disease. Results showed that atopic symptoms, as defined by history, were more frequently present in the CF patients. Prick testing of the skin produced positive reactions in 88% of the CF group and 36% of the control subjects (p less than 0.001), and the mean number of reactions per subject was significantly higher in the former group (p less than 0.001); reactions to fungal antigens were strikingly positive in the CF group. The CF patients had a significantly higher mean serum IgG4 (p less than 0.001), IgE (p less than 0.01), and higher mean eosinophil count (p less than 0.05). Clear-cut bronchial hyperreactivity was demonstrated in the CF group compared with control subjects. Bronchial provocation with 400 micrograms of histamine led to a greater than 15% fall in the preinhalation FEV1 in 35% of the CF subjects compared with 4% of the control group, with a mean percentage fall of 15% and 3% respectively (p less than 0.001). In the CF group a greater than 15% rise in PEFR occurred in 32% after inhalation of the parasympatholytic, ipratropium bromide (54 micrograms), and in 27% after inhalation of the sympathomimetic, fenoterol (400 micrograms). No correlation was found between bronchial reactivity and atopic status, HLA phenotype pattern, or disease severity. The cause of the increased prevalence of atopy and bronchial reactivity in CF patients remains unknown. However, it is clear that a trial of bronchodilator therapy is warranted in adolescents and young adults with CF.

Adolescent↗