Adult sickle cell disease a five-year experience of intensive care management in a university hospital in Oman

Sickle cell disease [SCD] is an inherited disease caused by an abnormal type of haemoglobin. It is one of the most common genetic blood disorders in the Gulf area, including Oman. It may be associated with complications requiring intensive care unit [ICU] admission. This study investigated the causes of ICU admission for SCD patients. This was a retrospective analysis of all adult patients >/= 12 years old with SCD admitted to Sultan Qaboos University Hospital [SQUH] ICU between 1st January 2005 and 31st December 2009. A total number of 49 sickle cell patients were admitted 56 times to ICU. The reasons for admission were acute chest syndrome [69.6%], painful crises [16.1%], multi-organ failure [7.1%] and others [7.2%]. The mortality for SCD patients in our ICU was 16.1%. The haemoglobin [Hb] and Hb S levels at time of ICU admission were studied as predictors of mortality and neither showed statistical significance by Student's t-test. The odds ratio, with 95% confidence intervals, was used to study other six organ supportive measures as predictors of mortality. The need for inotropic support and mechanical ventilation was a good predictor of mortality. While the need for non-invasive ventilation, haemofiltration, blood transfusions and exchange transfusions were not significant predictors of mortality. Acute chest syndrome is the main cause of ICU admission in SCD patient. Unlike other supportive measures, the use of inotropic support and/or mechanical ventilation is an indicator of high mortality rate SCD patient

Scapular bronchogenic cyst: a case report and literature review

Bronchogenic cysts originate from abnormal budding of the tracheal diverticula during the embryological period. Inaccuracy in the process of growing of the ventral foregut will give rise to bronchogenic cyst. Scapular bronchogenic cyst is an extremely rare form of this anomaly. A three years old boy suffered for 2 years with left sided suprascapular cystic lesion which was gradually increasing in size. The swelling was 4x3 cm in size and non tender. The cyst was evaluated by CT scan that showed complex cystic lesion over the left scapular spine. Total excision of the cyst was done and histopathology showed cutaneous bronchogenic cyst. The proposed mechanism for such cutaneous lesion is that the accessory buds from the tracheobronchial tree/primitive foregut migrated from the thorax in an aberrant manner to lie in periscapular positions. The definitive treatment of scapular lesions is total surgical excision. The final diagnosis is based on the histopathological findings in the majority of cases

Interstitial cells of Cajal. Pathology, injury and repair

Interstitial cells of Cajal [ICC] are specialised cells located within the musculature of the gastrointestinal tract [GIT]. Although they form only 5% of the cells in the musculature of the GIT, they play a critical role in regulating smooth muscle function and GIT motility in coordination with the enteric nervous system. C-kit is a transmembrane glycoprotein that plays a critical role in ICC development and maturation. Physiological conditions such as ageing, as well as pathological conditions that have different disease processes, negatively affect ICC networks and function. Absent or disordered ICC networks can be associated with disorders in GIT motility. This review highlights the mechanism of ICC recovery from various types of injury which entails understanding the development of ICC and the factors affecting it. ICC transformation into malignant tumours [gastrointestinal stromal tumours] and their potential as contributors to therapeutic resistance is also discussed