Serial lung function tests in primary immune deficiency.

Pulmonary complications are common in patients with primary immune deficiency (PID). The aim of this study was to assess the usefulness of lung function tests (LFTs) in the management of these patients, and in particular to see if carbon monoxide transfer factor (TLCO) is needed in addition to spirometry. We studied 20 patients (11 female) with PID in a tertiary referral clinic, with a mean age of 47.6 years. Serial LFTs, spanning a mean of 101 months, were correlated with immunoglobulin levels and antibiotic usage. Seven patients showed a decline in forced expiratory volume in 1 second over the period of the study. An additional five patients showed a decline in TLCO. Of these 12 patients, two had no radiographic evidence of lung disease. Higher levels of immunoglobulin were associated with slower decline in LFTs (P < 0.05). The analysis of antibiotic usage and LFTs failed to show a statistically significant effect, although there was a trend towards a slower rate of decline with greater use of antibiotics. LFTs decline slowly in patients with PID. Annual testing (both spirometry and transfer factor) is useful in the assessment of these patients, and should not be confined to those with radiological evidence of lung disease.

Respiratory involvement in inherited primary muscle conditions.

Patients with inherited muscle disorders can develop respiratory muscle weakness leading to ventilatory failure. Predicting the extent of respiratory involvement in the different types of inherited muscle disorders is important, as it allows clinicians to impart prognostic information and offers an opportunity for early interventional management strategies. The approach to respiratory assessment in patients with muscle disorders, the current knowledge of respiratory impairment in different muscle disorders and advice on the management of respiratory complications are summarised.

Trends in survival from muscular dystrophy in England and Wales and impact on respiratory services.

Respiratory failure is an important terminal event in muscular dystrophy, but increasingly is effectively treated by non-invasive ventilation. This study was designed to assess mortality statistics in this patient group in order to get an indication of future demand. Mortality data for all deaths from muscular dystrophy registered by death certification in England and Wales between 1993 and 1999 were analysed. In total, 817 deaths from muscular dystrophy were registered between 1993 and 1999. Annual number of deaths was unchanged over this period. Median age at death (interquartile range) for all cause muscular dystrophy increased from 20 (17-42.5) years in 1993, to 26 (17.5-63) years in 1999. Respiratory failure was the primary or contributory cause of death in 82% of cases. Two thirds of these deaths were during acute infection. We can expect 100 patients with muscular dystrophy to develop respiratory failure in England and Wales each year, so non-invasive ventilation services probably need to be able to provide for 0.2 new patients per 100,000 population annually. Respiratory services also need to provide adequate monitoring and early treatment of infection in these patients.

The effect of a mechanical glottis on peak expiratory flow rate and time to peak flow during a peak expiratory flow manoeuvre: a study in normal subjects and patients with motor neurone disease.

We have evaluated a mechanical glottis in healthy volunteers and in patients with bulbar motor neurone disease. In healthy volunteers, the mechanical glottis increased peak flow rate and decreased the time to peak flow during forced expiration, but cough produced even higher flow rates and shorter times to peak flow. In patients, the mechanical glottis increased peak flow rate and decreased the time to peak flow. The mechanical glottis also produced higher peak flow rates when compared to the cough manoeuvres, and the time to peak flow was also significantly shorter with the mechanical glottis. We have shown that the use of a mechanical glottis tends to convert the airflow profile of a peak expiratory flow manoeuvre into that of a cough in both healthy volunteers and patients with motor neurone disease. Its potential role as an aid to clearance of airway secretions in patients with impaired laryngeal function remains to be seen.

Patterns of mortality in patients with motor neurone disease.

OBJECTIVE: Motor neurone disease (MND) is a rapidly fatal condition with survival of less than 4 years. Patients can deteriorate quickly in the preterminal stages resulting in inappropriate resuscitation or admission to intensive care units (ICU) or accident and emergency (A & E). MATERIAL AND METHODS: We looked at patterns of mortality with emphasis on the place of death. A retrospective study was performed of all patients attending an MND clinic, who had died within a 10-year period. RESULTS: Of 179 patients (63 female), 81 patients (45%) died at home, in a hospice or in a nursing home. Sixty-five patients (36%) died in hospital (11 in ICU or A & E). Nine of the latter were previously known to have MND and six admissions were probably avoidable. Most ward patients died of respiratory causes and were treated conservatively. CONCLUSION: The proportion of patients dying in A & E or ICU was small but could have been reduced further. A number of those who died on the wards could probably have been managed conservatively at home. Older patients and those with bulbar disease had a poorer prognosis.