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A rare cause of respiratory failure
  1. S P L Meghjee,
  2. S E Enright,
  3. H O'Beirne,
  4. S Williams
  1. Pinderfields Hospital, Wakefield, West Yorkshire, UK
  1. Dr Salim P L Meghjee, c/o Dr Williams' secretary, Department of Cardiorespiratory Medicine, Pinderfields Hospital, Aberford Road, Wakefield, West Yorkshire WF1 4AN, UK

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Answers on p 211.

A 68 year old man with a 60 year pack history (20 cigarettes per day for 60 years) of smoking was admitted via casualty with acute confusion, lethargy, and dyspnoea over three days. He denied any cough or haemoptysis, but was unable to give a clear history. Information from his wife indicated increasing exertional dyspnoea over two years with recent swelling of the ankles. She described increased spinal curvature over the past year with weakness of his arms and legs. On clinical examination he had marked kyphosis and was apyrexial. Pulse was 100 beats/min with a blood pressure of 140/70 mm Hg. Heart sounds were normal. His respiratory rate was 14 beats/min with poor chest expansion and decreased breath sounds bilaterally. He was confused and slightly drowsy with a Glasgow coma scale of 14/15. Cranial nerves were intact. Upper limb examination was normal with no evidence of muscle wasting or fasciculation. Lower limb examination showed right foot drop. Full blood count and electrolytes were normal. Arterial blood gases on admission on 24% oxygen showed pH 7.40, arterial carbon dioxide tension 9.8 kPa, arterial oxygen tension 8.1 kPa, bicarbonate 44 mmol/l, and a base excess of +15. The chest radiograph is shown in fig 1.

Six weeks before this admission he had been seen in a respiratory clinic for investigation of his dyspnoea but no mention of muscular weakness was made. His past medical history included a neck injury after a road traffic accident 14 years previously with no associated neurological deficit. Chest examination at that time revealed kyphosis with markedly decreased chest expansion with reduced air entry.

Cardiovascular examination was normal. Detailed neurological examination was not recorded. Full lung function tests showed peak flow 320 l/m (predicted 505 l/m). Forced expiratory volume in one second was 2.2 l/sec (predicted 2.65 l/sec). Forced vital capacity was 2.4 litres (predicted 3.65 litres), and transfer factor of carbon monoxide per unit of volume was 19.4 ml/min/mm Hg (predicted 17.6). Cervical radiography is shown in fig 2. Arrangements were made to review him after further investigation.

Figure 2

Cervical radiograph.


How would you interpret the arterial blood gases and the lung function tests?
What is the differential diagnosis?
What do the chest and cervical radiographs show?
What further investigations might be helpful?
What would be your initial management of this patient?
What is the long term respiratory management of this condition?