Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion.

Background: Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi-organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub-optimal results, such as debilitating long-term neurological complications.

A New Variant Mutation in Gene in Case of Trichohepatoenteric Syndrome.

Trichohepatoenteric syndrome is an autosomal recessive genetic disease with an estimated prevalence of 1:100,000. The mutation of the disease is placed either in or genes. The onset of presentation is variable, but symptoms usually start with intractable diarrhea associated with woolly hair abnormality, immune dysfunction, and sometimes hepatic abnormality.

Effect of prone positioning in patients with acute respiratory distress syndrome: a meta-analysis.

Objective: To review the effectiveness of prone position as compared with supine position, with respect to mortality, improvement in oxygenation, number of days on mechanical ventilation, and ventilator-associated pneumonia.

Data Source: PubMed, EMBASE, Cochrane database, and a manual review of article bibliographies.

Study Selection: Randomized controlled trials comparing > or = 6 hrs of prone position with supine position in adult patients with adult respiratory distress syndrome.

Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh.

Background: Because reports of bronchiolitis obliterans organizing pneumonia (BOOP) are lacking from the Middle East, we conducted a retrospective review of of all histopathologically proven cases of BOOP over a 10-year period at three tertiary care hospitals in Riyadh and describe the clinical features and outcome.

Methods: Charts at the three hospitals were searched using a specific code for BOOP or cryptogenic organizing pneumonia (COP). Lung specimens had to show histological proof of BOOP with a compatible clinical picture.

Bronchiolitis obliterans organizing pneumonia.

Bronchiolitis obliterans with organizing pneumonia (BOOP) is now established as a distinct clinicopathologic entity, yet it may be overlooked by clinicians due to unfamiliarity and its non-specific presentation. It can be either idiopathic or associated with a variety of causes, such as infections, drugs, radiation or connective tissue diseases. A lung biopsy is needed to provide histopathologic confirmation.