Rituximab at a dose of 1 g appears to be beneficial in the treatment of patients with severe myasthenia gravis. Serial monitoring of peripheral blood B lymphocytes appears to be useful in guiding the need for further RTX therapy.
Platypnoea-orthodeoxia is a rare syndrome of postural hypoxaemia accompanied by breathlessness. The predominant symptom, dyspnoea induced by upright posture, can be debilitating and diYcult to discern without thorough evaluation of the patient's pattern of dyspnoea. The precise cause of the syndrome is unclear but patients develop right to left intracardiac shunting in the presence of normal right sided cardiac pressures. Initially, patients should have confirmation of orthostatic desaturation by erect and supine pulse oximetry. However, definitive diagnosis of an orthostatic intracardiac shunt is most readily established by echocardiography. The use of echocontrast with postural manoeuvres may facilitate the diagnosis. The treatment of choice is surgical closure of the intracardiac (usually interatrial) communication, which may result in dramatic symptomatic and haemodynamic improvement. Three cases (a 27 year old man and two women aged 63 and 72 years) are described that exemplify the presentation of this syndrome, and reflect the varied management strategies and outcomes of this condition. (Heart 2000;83:221-223) Keywords: platypnoea-orthodeoxia; echocardiography; intracardiac shunt Platypnoea-orthodeoxia is an uncommon syndrome of dyspnoea induced by upright posture, with associated arterial hypoxaemia, which is subsequently relieved by recumbency. 1The syndrome occurs when there is right to left shunting of blood, usually via an interatrial communication, in the presence of normal pulmonary artery pressure. This is an unusual situation given that most cases of significant right to left intracardiac shunting of blood are associated with increased right sided cardiac pressures.2 The three cases described here exemplify the presentation of this syndrome, and reflect the varied management strategies and outcomes of this condition. Case 1A 27 year old man with a known atrial septal defect (ASD) presented with 24 hours of extreme dyspnoea at rest with a history of four months of worsening exertional dyspnoea and lethargy. The ASD had been diagnosed at 9 years of age but the patient had failed to attend follow up care. He had no other significant medical history but was a smoker with a 12 pack-year history of consumption. He had no symptoms suggestive of parenchymal lung disease, pulmonary infections, obstructive sleep disorders or thromboembolic disease. On physical examination his temperature was 37.5°C, he had a regular pulse at 88 beats/min, respiration rate 24/min, and blood pressure was 110/70 mm Hg. There was central cyanosis and digital clubbing with two subungual haemorrhages. Lung fields were clear to auscultation, and cardiac examination revealed fixed wide splitting of the second heart sound. There were no regurgitant heart murmurs or palpable precordial impulses. With the patient breathing room air, oxygen saturation was 92% supine and 84% in the upright position. Subsequent investigation revealed haemoglobin of 183 g/l, normal pulmonary ventilation perfusion scan, and normal respiratory fu...
Objective To determine (i) the relationship between prescriptions for proton pump inhibitors (PPIs) and upper gastrointestinal conditions, and (ii) compliance with Pharmaceutical Benefits Scheme (PBS) prescribing guidelines for PPIs. Design Drug utilisation evaluation. Setting 800‐bed metropolitan teaching hospital. Participants 253 patients dispensed PPIs from the hospital pharmacy over five consecutive weeks (11 January to 15 February 1999). Main outcome measures Recorded gastrointestinal conditions; previous trial of H2‐ antagonist therapy; compliance with PBS criteria for prescribing PPIs. Results Seventy patients (27.7%) had no appropriate upper gastrointestinal tract investigations, and 62 patients (24%) did not receive an adequate trial of H2‐antagonist therapy before the commencement of a PPI. The major indications for use of PPIs in investigated patients were gastro‐oesophageal reflux in 99 (54%) and peptic ulcer disease in 30 (16.4%). In only 57 patients (22.5%) did PPI prescriptions comply with PBS prescribing guidelines. Clinical indications that failed to meet prescribing criteria included milder forms of gastro‐oesophageal reflux, gastritis/duodenitis, and non‐specific dyspepsia with normal endoscopy results. Conclusion Drug utilisation data indicate widespread use of PPIs outside current prescribing guidelines. Many patients have not had relevant investigations and/or an adequate trial of H2‐antagonist therapy. These findings explain the considerable hospital expenditure on PPIs.
This case series suggests that low-dose courses of rituximab can be used off-label to treat several severe and/or refractory immunological disorders with a reasonable safety profile; however, further trials are required in many off-label indications.
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