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M X FitzGerald

Publications and source records attributed to M X FitzGerald.

At least 55 records · Page 3Linked to original sources
PubMed1991

Comparison of oxygen desaturation during sleep and exercise in patients with cystic fibrosis.

Patients with cystic fibrosis (CF) desaturate during sleep and during exercise but by different mechanisms. To determine the need for supplemental oxygen, many centers measure resting and exercise arterial oxygen saturation (SaO2). We examined the associations among resting, sleep, and exercise SaO2 to ascertain the validity of this approach. We studied 21 adult and adolescent CF patients, eight of whom were hypoxemic (SaO2 less than 95 percent; group A) and 13 of whom were nonhypoxemic (SaO2 greater than or equal to 95 percent; group B) by overnight oximetry and treadmill exercise testing. The whole group desaturated more during sleep than during exercise, the change in SaO2 being 10.59 +/- 8.35 vs 6.25 +/- 4.44 (p less than 0.002). Group B desaturated significantly more during sleep than during exercise, with a reduction in SaO2 of 7.9 +/- 3.3 vs 3.3 +/- 1.49 (p less than 0.05). Group A desaturated more during exercise than group B, with a reduction of 11 +/- 3.2 vs 3.3 +/- 1.5 (p less than 0.001). Despite a strong correlation between awake SaO2 and mean sleep SaO2 (r = 0.68; p less than 0.001), minimum sleep SaO2 (r = 0.55; p less than 0.01), and minimum exercise SaO2 (r = 0.92; p less than 0.001), there was no correlation between awake SaO2 and sleep-related desaturation or between exercise- and sleep-related desaturation. In conclusion, clinically significant oxygen desaturation during sleep may be missed unless specifically checked in CF patients, and awake and exercise SaO2 may not give an indication of the degree of sleep-related desaturation.

Adolescent↗
PubMed1991

Analysis of patients lost to follow up at a sarcoid clinic.

An analysis was made of an outpatient population attending a specialized sarcoidosis clinic in order to identify and quantify the number of patients who had become lost to follow up. In addition we wished to elucidate the reasons why they had not returned and to determine the consequences, if any, of not having continuing medical surveillance. Ninety-four patients were identified as being lost to follow up from our specialized sarcoidosis clinic and were invited to return for review. Forty-six patients (49%) responded and were evaluated. The main reason for clinic non-attendance given by 91% (42/46) of the clinic reattenders was that they were asymptomatic. Comparing this group with a cohort of 50 patients who are regular clinic attenders, we found that the lost to follow up patients had a strikingly more benign disease profile. In spite of this, analysis of this respondent group revealed some evidence of continuing disease activity or progression, with 37% (17/46) of the group fulfilling at least one of our defined criteria for disease progression. While none of these patients warranted the immediate addition of corticosteroid medication at their first recall visit, their continuing disease activity warrants close supervision at a specialized sarcoidosis clinic. We conclude that educational strategies require to be developed in order to minimize clinic non-attendance by patients with sarcoid. Furthermore more positive action needs to be taken by clinicians in attracting those patients, who become lost to follow up, to return for clinic review.

Adult↗
PubMed1990

Frequency of deletion 508 among Irish cystic fibrosis patients.

We estimate the incidence of cystic fibrosis in Ireland to be at least 1 case per 1838 live births. We have analysed DNA from 44 Irish CF patients for the presence of deletion 508, using the polymerase chain reaction. The deletion was found in 76% of their chromosomes, and approximately 58% of the patients are homozygous for this deletion. Our results are not significantly different from those found in Canadian or UK patient populations, in which frequencies are higher than those found in Southern European countries.

Chromosome Deletion↗
PubMed1990

Pseudomonas colonization in cystic fibrosis: lack of correlation with secretion of ABO blood group antigens.

In cystic fibrosis, a majority of patients develop persistent lifelong respiratory tract colonisation with pseudomonas aeruginosa while a minority do not appear to become colonised. There is some evidence that non-secretion of ABO Blood Group antigens into body secretions is a marker for susceptibility to certain gram negative infections. Therefore, we studied 47 adult patients with cystic fibrosis to identify an association, if any, between secretor status and the presence or absence of pseudomonas aeruginosa in their sputum. Overall, we found no difference between the prevalence of non-secretors in our patient group and in the normal population. Furthermore, there was no association between secretor status and presence or absence of pseudomonas aeruginosa in sputum. We conclude that secretor status is unlikely to play a major role in susceptibility to pseudomonas aeruginosa infection in patients with cystic fibrosis.

ABO Blood-Group System↗
PubMed1990

Talc lung in a drug abuser.

We report a case of intravenous talcosis in a 36 year old woman. Although she initially denied drug abuse clinical suspicion was aroused by the finding of obliterated peripheral veins, a pleural rub and a peripheral nodular lesion on chest x-ray. Diagnosis of intravenous talcosis was confirmed by finding birefringent particles on transbronchial biopsy. Confronted with this evidence the patient admitted long-standing drug abuse, including intravenous injection of crushed methadone tablets which contain talc filler.

Adult↗
PubMed1990

Skin reactivity to atypical mycobacteria in cystic fibrosis.

Atypical mycobacterial disease has been described in a small number of patients with cystic fibrosis. Apart from one uncontrolled study, there is little information regarding atypical mycobacterial skin reactivity in this group of patients. We evaluated delayed cutaneous hypersensitivity to purified extracts of Mycobacterium avium, Mycobacterium intracellular, Mycobacterium kansasii and Mycobacterium bovis in 23 healthy controls and 43 adult and adolescent patients with cystic fibrosis. Fifteen of the cystic fibrosis group were receiving regular corticosteroids. Additionally, direct smear examination and Lowenstein Jensen culture were performed on sputum from the cystic fibrosis group. The prevalence of positive skin reactions was similar in the group with cystic fibrosis (30%) and in the control group (57%). Subgroup analysis showed that those cystic fibrosis patients receiving corticosteroids had a markedly lower prevalence of positive reactions (7%) compared to controls (P less than 0.01). When this subgroup was excluded from analysis, the prevalence of positive skin reactions among patients with cystic fibrosis was 43%. In the prospective sputum bacteriology study, one of the 43 cases grew Mycobacterium avium-intracellulare and had clinical and radiological evidence of this disease. Of note, this patient showed positive skin tests to all four mycobacterial species tested. Our data show no difference in the prevalence rate of positive skin reactions to atypical mycobacterial antigens between a control population and an adult cystic fibrosis population. In addition, the predictive value of skin testing is low in cystic fibrosis due to the high prevalence of cross-reactivity between different mycobacterial species and the high prevalence of anergy among those patients with advanced disease receiving treatment with corticosteroids.

Adolescent↗
PubMed1990

Bronchoalveolar lavage in patients with mild and severe rheumatoid lung disease.

The reported prevalence of interstitial lung disease in patients with rheumatoid arthritis has varied from 10% to 50%, yet less than 5% of patients with arthritis develop severe fibrosing interstitial lung disease. This suggests that subclinical disease may not always presage progressive disease. Bronchoalveolar lavage fluid from patients with rheumatoid arthritis and either clinically evident interstitial lung disease or subclinical disease was examined for the presence of factors with a putative role in the development of interstitial fibrosis. Patients with subclinical disease were identified by prospective radiographic and lung function screening of 93 patients with rheumatoid arthritis. Fourteen patients were identified in this manner and an association between subclinical disease and smoking history was noted. Eleven patients with established interstitial lung disease had increased neutrophils (p less than 0.05), collagenase, and type III procollagen N terminal peptide levels (p less than 0.01) in the bronchoalveolar lavage fluid. Preliminary characterisation of the bronchoalveolar lavage collagenase suggested that it originated from neutrophils. Ten patients with subclinical interstitial lung disease underwent bronchoalveolar lavage. Of these, one had increased neutrophils and two had increased collagenase concentrations--abnormalities associated with advanced interstitial lung disease and a poor prognosis. These results suggest that in arthritis patients with evidence of subclinical pulmonary interstitial disease bronchoalveolar lavage might be useful in identifying those who may require careful monitoring in the hope that early treatment will prevent severe fibrosis.

Adult↗
PubMed1989

Pulmonary alveolar proteinosis: primary and secondary, a report of three cases.

Three cases of the rare disorder pulmonary alveolar proteinosis (PAP) presented to our unit over the past thirteen years. Two of the patients conformed to the classical description of idiopathic or "primary" PAP. One patient appeared to have co-existing extrinsic allergic alveolitis with "secondary" PAP, an association not previously described. This patient has required continued steroid therapy, a mode of treatment usually contraindicated in PAP.

Adult↗
PubMed1989

Type 3 procollagen peptide in bronchoalveolar lavage fluid. Poor indicator of course and prognosis in sarcoidosis.

To investigate the role of bronchoalveolar lavage type 3 procollagen peptide as a prognostic indicator in sarcoidosis, we measured type 3 procollagen N-terminal peptide levels in lavage fluids from 84 sarcoidosis patients and monitored disease progress in these patients for a period of 12 months. Lavage procollagen peptide levels were significantly elevated in sarcoidosis patients compared to control subjects (p less than 0.001). No association was observed between lavage type 3 procollagen peptide and disease severity, as assessed by lung function tests. Follow-up monitoring of patients failed to demonstrate any relationship between subsequent functional deterioration and initial lavage type 3 procollagen peptide. These results suggest that elevated lavage type 3 procollagen peptide concentrations in sarcoidosis may reflect increased type 3 collagen synthesis associated with the inflammatory process rather than signal an early event in the development of chronic disease.

Adult↗
PubMed1988

Tuberculosis.

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BCG Vaccine↗
PubMed1988

Investigation of aminoglycoside nephrotoxicity in cystic fibrosis patients.

Cystic fibrosis patients are a special risk group for nephrotoxic effects of amino-glycosides (AG). The traditional methods of assessing renal damage are very insensitive, and toxicity is not detected until serious functional damage is evident. The aims of the present study were to monitor early markers of nephrotoxicity in cystic fibrosis patients treated with gentamicin. Urinary excretion of beta-N-acetylglucosaminase (beta-NAG, a lysosomal enzyme) and low molecular weight proteins as identified on sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE) were prospectively studied in cystic fibrosis outpatients and inpatients. Urinary excretions of beta-NAG and low molecular weight proteins were normal in cystic fibrosis patients not being treated with gentamicin. All patients being treated with intravenous gentamicin (average dose, 2.5 g over 9 days) showed markedly elevated urinary excretion of beta-NAG. SDS-PAGE of serial urine samples from CF patients during intravenous AG therapy showed increasing excretion of low molecular weight proteins. Protein excretion profiles from control subjects showed no change over equivalent time periods. CF patients receiving nebulized AG did not show altered protein excretion. beta-NAG excretion patterns were similar to changes detected in the SDS-PAGE system. No gross nephrotoxicity developed in these patients. Blood urea nitrogen, plasma creatinine, and plasma magnesium concentrations remained within normal limits. The pattern of protein excretion on SDS-PAGE was consistent with renal tubular rather than glomerular damage. The SDS-PAGE system provides an additional means of monitoring early subclinical nephrotoxicity of AG in CF patients.

Acetylglucosaminidase↗
PubMed1988

Acute hypomagnesaemia complicating the treatment of meconium ileus equivalent in cystic fibrosis.

Meconium ileus equivalent (MIE) is a common and often recurrent complication in adolescent and adult patients with cystic fibrosis (CF). MIE is characterized by partial or complete bowel obstruction, resulting from abnormally viscid mucofaeculant material in the terminal ileum and right colon. Patients present with recurrent abdominal pain, intestinal obstruction, and/or a palpable faecal mass. Conventional treatment consists of the oral and rectal administration of the mucolytic agent N-acetylcysteine, and hypertonic solutions of sodium diatrizoate. We describe the occurrence of acute decreases in plasma magnesium in all of seven patients treated with this regimen with marked hypomagnesaemia (less than 0.70 mmol/l) in four of the seven patients. No changes in plasma sodium, potassium, or calcium were observed.

Acetylcysteine↗
PubMed1987

Popliteal cyst rupture and the pseudothrombophlebitis syndrome.

Fifteen patients presented during a four-year period with a pseudothrombophlebitis syndrome, subsequently confirmed arthrographically as being due to popliteal cyst rupture. Initially the majority of patients (73%) were erroneously diagnosed as having calf vein thrombosis, and were anticoagulated for periods of three to ten days. Clinical differentiation from venous thrombosis was usually impossible. Contrast arthrography was the definitive diagnostic investigation, revealing popliteal cysts in all patients, and an active synovial leak into the calf in 13 cases (87%). The mean delay in performing arthrography was 5.3 days, the procedure usually being performed after normal contrast venography. Doppler and isotope venography yielded misleading or equivocal results (42%) delaying diagnosis, and prolonging periods of potentially dangerous anticoagulation. We suggest that the marked overlap in the emergency presentation of popliteal cyst rupture and calf vein thrombosis mandates the aggressive use of arthrography combined with venography in all patients presenting with a painful swollen leg.

Adolescent↗