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Biomedical subjects

D R McCluskey

Publications and source records attributed to D R McCluskey.

10 recordsLinked to original sources
PubMed1992

Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor.

A boy whose chronic granulomatous disease (CGD) manifested in infancy, and whose elder brother had died at 7 years of age, had phagocytes with complete lack of functional cytochrome B-245 and which could not be induced by interferon gamma to achieve adequate staphylococcal killing. He underwent an elective displacement bone marrow transplant from a volunteer unrelated donor at the age of 8 months. This has achieved 100% replacement of the CGD granulocytes by those of the normal volunteer and the boy has since had a normal childhood for 3 years. Six previous transplants for CGD are briefly reviewed and illustrate that the host abnormal marrow must be completely displaced using an adequate dose of busulphan to ensure 100% stable engraftment of the donor's marrow and that this is best done under elective conditions before septic foci and irreversible organ damage have occurred. Criteria need to be developed to identify early those patients likely to have severe morbidity.

Bone Marrow Transplantation↗
PubMed1992

CD4 lymphocyte subset abnormalities associated with impaired delayed cutaneous hypersensitivity reactions in patients with X-linked agammaglobulinaemia.

Circulating CD4 lymphocyte subset (CD45RA; CD45RO; CD29; Leu8) levels were determined in nine patients with X-linked agammaglobulinaemia (XLA), nine patients with common variable immunodeficiency (CVI) and in 18 age- and sex-matched controls. CD4CD45RO and CD4CD29 cells were significantly lower (P less than 0.01) in the XLA patient group (CD45RO, 15.7 +/- 10.2%; CD4CD29, 32.1 +/- 14.6%) compared with CVI patients (61.8 +/- 25.4%; 60.1 +/- 11.2%) and normal controls (43.7 +/- 22.3%, 54.5 +/- 22.0%). The levels of CD4CD45RA and CD4Leu8 cells were not abnormal in the XLA patient group. No selective reduction in CD4 subsets was observed in the CVI patient group. Delayed cutaneous hypersensitivity testing of five XLA and five CVI patients revealed a significantly reduced response to recall antigens in patients with XLA. This may relate to the deficiency of circulating memory T cells observed in these patients.

Adolescent↗
PubMed1992

Chronic fatigue syndrome.

Chronic Fatigue Syndrome appears to represent a spectrum of disorders in which a variety of pathophysiological mechanisms may operate. While the initiating event in the majority of patients is a pyrexial illness, possibly due to enterovirus infection, evidence of persisting infection or inflammatory changes in muscle and/or brain remain unconvincing. CFS patients display a definite reduced aerobic work capacity compared to normal control subjects, but this may reflect a state of deconditioning resulting from prolonged physical inactivity. They also have an altered perception of their level of exertion and premorbid fitness. The characteristic fluctuation in symptoms, with periods of relapses and partial remissions, may indicate that some central disorder of sensory perception is operational. It may be that a primary sleep disorder results in a reduced sensory threshold for afferent stimuli from muscle. This could well account for many of the subjective symptoms which patients experience. Much more research is clearly necessary if we are to achieve a better understanding of this distressing and at present enigmatic disorder.

Adult↗
PubMed1991

Selective C7 complement deficiency causing recurrent meningococcal infection.

We report on two sisters both with complete absence of the 7th component of complement. This congenital immunodeficiency disorder is associated with recurrent bacterial infection, especially that due to Neisseria species. These cases illustrate many of the well-recognised features of this disorder, but in one patient the illness was complicated by infective endocarditis due to N. meningitidis, a feature not previously reported.

Adolescent↗
PubMed1991

Treatment of chronic fatigue syndrome.

Chronic Fatigue Syndrome is a disorder which is characterised by profound fatigue together with a variety of other subjective clinical features which persist over a prolonged period of time. The aetiology remains at present uncertain and therefore rational therapeutic strategies are difficult to plan. This paper reviews currently used forms of treatment aimed at correcting the possible pathophysiological mechanisms and discusses the problems associated with the management of this condition.

Diagnosis, Differential↗
PubMed1990

Aerobic work capacity in patients with chronic fatigue syndrome.

OBJECTIVE: To determine the aerobic work capacity of patients with the chronic fatigue syndrome and compare it with that of two control groups, and to assess the patients' perception of their level of activity before and during illness. DESIGN: A symptom limited exercise treadmill test with on line gas analysis and blood sampling was used. Subjects were assessed by one investigator, who was blind to the group which they were in. SETTING: Department of medicine, Royal Victoria Hospital, Belfast. SUBJECTS: 13 Patients (10 women, three men) who fulfilled the diagnostic criteria for chronic fatigue syndrome. Two control groups of similar age, sex, and body weight: 13 normal subjects (10 women, three men) and seven patients (five women, two men) with the irritable bowel syndrome. MAIN OUTCOME MEASURES: Aerobic work capacity as assessed by several variables such as length of time on treadmill, heart rate, and biochemical measurements; Borg score; and visual analogue scores of perceived level of physical activity. RESULTS: The patients with the chronic fatigue syndrome had a reduced exercise capacity compared with that of the other subjects, spending a significantly shorter time on the treadmill. They had a significantly higher heart rate at submaximal levels of exertion and at stage III exertion had significantly higher blood lactate concentrations. Using a Borg score, they showed a significantly altered perception of their degree of physical exertion with a mean score of 8.2 compared with 6.6 and 5.3 for the normal subjects and patients with the irritable bowel syndrome respectively. Using a visual analogue scale they indicated that they had a greater capacity for activity before illness than had the patients with the irritable bowel syndrome, but the scores were not significantly different between the two groups. Both groups of patients indicated reduced activity at the time of testing. Normal controls and patients with the irritable bowel syndrome aspired to a greater level of activity than their current level, but the patients with the chronic fatigue syndrome aspired to a level similar to that which they had had before their illness. CONCLUSIONS: Patients with the chronic fatigue syndrome have reduced aerobic work capacity compared with normal subjects and patients with the irritable bowel syndrome. They also have an altered perception of their degree of exertion and their premorbid level of physical activity.

Adult↗
PubMed1988

Wegener's granulomatosis and autoantibodies to neutrophil antigens.

We report five cases of Wegener's granulomatosis all of whom had clinical and histological evidence of disease activity at presentation and in whom autoantibodies to neutrophil antigens were detected. This test may prove useful for the diagnosis of this serious condition and help to monitor disease activity during treatment.

Adult↗
PubMed1986

Assessment of cell-mediated immunity in a British population using multiple skin test antigens.

An in vivo method of assessing the competence of the cell-mediated immune system (Multitest CMI) was used in 200 healthy volunteers (age range 17-88 years). The profile of reactivity to seven individual antigens was determined. A positive reaction was obtained in 96.5% of the subjects who reacted positively to at least one antigen with 78% reacting to two or more antigens. The number of positive responses and the degree of reactivity was significantly reduced in elderly subjects and in females aged 17-65 years. The Multitest CMI system provides a rapid and convenient method of assessing cell-mediated immunity (CMI) in vivo and could have a wide range of applications in the investigation of immunological, infective and neoplastic conditions.

Adolescent↗