Recurrent haemorrhagic venous infarct in a patient with sickle cell disease.

Cerebrovascular accidents are uncommon but devastating complications of sickle cell disease (SCD). Notably, cerebral sinovenous thrombosis is rarely reported in SCD and poses a therapeutic dilemma regarding anticoagulation. Herein, we describe a challenging case of a patient with sickle thalassaemia admitted to the hospital with recurrent haemorrhagic infarct secondary to cerebral sinus thrombosis. The patient was successfully treated with anticoagulation without neurological deficit. No embolic or other thrombotic aetiology was found, and the stroke was presumed due to sickle cell disease, leading to a hypercoagulable state. Our case report highlights the value of early recognition of this rare but potentially life-threatening condition in SCD, considers other options of anticoagulation therapy and emphasises the importance of close multidisciplinary follow-up particularly post hospital discharge.

Efficacy of Prostacyclin Anticoagulation in Critically Ill Patients Requiring Extracorporeal Support: A Systematic Review and Meta-Analysis.

Extracorporeal support modalities are highly prothrombotic. Anticoagulation is frequently used for patients receiving Continuous Renal Replacement Therapy (CRRT), Molecular Adsorbent Recirculating System (MARS), and Extracorporeal Membrane Oxygenation (ECMO). The objective of this systematic review and meta-analysis is to determine if prostacyclin-based anticoagulation strategies are effective compared to other anticoagulation strategies, in critically ill children and adults who needs extracorporeal support, such as continuous renal replacement therapy. We conducted a systematic review and meta-analysis using multiple electronic databases and included studies from inception to June 1, 2022. Circuit lifespan, proportion of bleeding, thrombotic, and hypotensive events, and mortality were evaluated. Out of 2,078 studies that were screened, 17 studies (1,333 patients) were included. The mean circuit lifespan was 29.7 hours in the patients in the prostacyclin-based anticoagulation series and 27.3 hours in the patients in the heparin- or citrate-based anticoagulation series, with a mean difference of 2.5 hours (95%CI -12.0;16.9, p=0.74, I2=0.99, n=4,003 circuits). Bleeding occurred in 9.5% of the patients in the prostacyclin-based anticoagulation series and in 17.1% of the patients in the control series, which was a statistically significant decrease (LogOR -1.14 (95%CI -1.91;-0.37), p<0.001, I2=0.19, n=470). Thrombotic events occurred in 3.6% of the patients in the prostacyclin-based anticoagulation series and in 2.2% of the patients in the control series, which was not statistically different (LogOR 0.97 (95%CI -1.09;3.04), p=0.35, I2=0.0, n=115). Hypotensive events occurred in 13.4% of the patients in the prostacyclin-based anticoagulation series and in 11.0% of the patients in the control series, which was not statistically different (LogOR -0.56 (95%CI -1.87;0.74), p=0.40, I2=0.35, n=299). The mortality rate was 26.3% in the prostacyclin-based anticoagulation series, and 32.7% in the control series, which was not statistically different (LogOR -0.40 (95%CI -0.87;0.08), p=0.10, I2=0.00, n=390). The overall risk of bias was low to moderate. In this systematic review and meta-analysis of 17 studies, prostacyclin-based anticoagulation was associated with fewer bleeding events, but with similar circuit lifespans, thrombotic events, hypotensive events, and mortality rates. The potential benefits of prostacyclin-based anticoagulation should be explored in large randomized controlled trials.

Abdominal compartment syndrome secondary to chronic constipation in MECP2 duplication syndrome.

Abdominal compartment syndrome (ACS) is an infrequently encountered life-threatening disorder characterised by elevated abdominal pressure with evidence of new organ dysfunction. It is rarely reported in paediatrics. We describe an extremely unusual presentation of a 13-year-old boy with long-standing constipation who developed ACS complicated by refractory septic shock and multiorgan failure. He was treated with emergent decompressive laparotomy and supportive critical care. This case highlights the need for early diagnosis and timely management of ACS to improve its outcome.

United Arab Emirates' Future Perspective: Converting Potential Organ Donors Into Actual Organ Donors in an Academic Setting.

OBJECTIVES: The wide gap between organ demand and supply has been exponentially increasing worldwide, resulting in longer wait lists for patients. In response, a few countries have started deceased-donor programs. Thus, establishing national self-sufficiency and optimizing the deceased donation process are crucial steps. Recently, a deceased-donor program was initiated in the United Arab Emirates, and a process is being implemented to enhance the donation rate and to decrease the number of missed organ donors. This study aimed to estimate the number of potential brain-dead donors and eligible donors who are medically suitable and have been declared dead based on neurologic criteria. Additionally, the study aimed to highlight latent obstacles that could add valuable strategic input for establishing a successful organ donation process for new programs. MATERIALS AND METHODS: For this retrospective study, we reviewed all patients who died at various intensive care units at Shaikh Khalifa Medical City (Abu Dhabi, UAE) from January 2018 to December 2018. Patients were classified as potential donors when meeting the neurologic criteria of brain dead and as eligible donors if no medical contraindication was shown. Barriers to donation were analyzed systematically. RESULTS: In 2018, 411 inpatient deaths were recorded. Of the 39 patients identified as potentially brain dead, 25 were confirmed as brain dead, with 20 of these confirmed as eligible donors. However, none were converted to actual donors. The lack of proper identification and referral of potential organ donors constituted significant obstacles to the organ donation process. CONCLUSIONS: This study described the potential organ donation program of our hospital and suggested a systemic approachthattargets the system,organ/donor factors, and consent process to improve the donor conversion rate. New programs might benefit highly from in-house organ donation coordinators, the education and proactive collaboration of hospital staff, and organizational support and management.

Thrombocytopenia-associated multiple-organ failure (TAMOF): recognition and management.

Thrombocytopenia-associated multiple-organ failure (TAMOF) is an increasingly fatal phenomenon that may be associated with sepsis. TAMOF results from immune dysregulation and impaired activity of A Disintegrin And Metalloproteinase with ThromboSpondin type 1 motif, member 13. Early recognition of this premorbid condition and specific management results in a significantly improved outcome. Herein, we report the presentation and management of a 2-year-old child with TAMOF who was successfully treated with plasma exchange and recovered without long-term sequelae.

Rare Bacteria Infecting the Heart and Affecting the Kidney of a Young Child.

Rare bacteria can lead to infective endocarditis, which may lead to renal involvement as severe glomerulonephritis. We report our experience of a 12-year-old child who presented with infective endocarditis and blood culture-grown Gemella morbillorum - a rarely reported bacteria. The clinical picture was further complicated with severe glomerulonephritis. Renal biopsy was suggestive of crescentic glomerulonephritis. The child was managed with antibiotics, steroids, and plasmapheresis and responded well to the treatment. To our knowledge, this is the first report of G. morbillorum endocarditis with immune complex deposition and necrotizing glomerulonephritis in a child.