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.

Haemodynamic effects of intravenous acetaminophen in critically ill paediatric patients: a retrospective chart review.

OBJECTIVES: Haemodynamic changes following intravenous acetaminophen are well studied in adults. Limited data are published in critically ill paediatric patients, especially from the Middle East. We aim to investigate haemodynamic effects and incidence of hypotension with intravenous acetaminophen in critically ill children, with a focus on understanding factors influencing these effects. METHODS: We retrospectively reviewed patients who received intravenous acetaminophen between July and December 2022. A haemodynamic event was defined as drop of >15% in systolic blood pressure (SBP) or mean arterial blood pressure (MAP) within 120 min after drug administration. Hypotension was defined as either drop in SBP below the 5th percentile for age, or a haemodynamic event associated with tachycardia, increased lactate or treatment with fluid/vasopressors. Logistic regression was performed to quantify relationships between patients' characteristics and the occurrence of haemodynamic event and hypotension. RESULTS: A haemodynamic event was observed in 50/156 patients (32%) post-acetaminophen. Mean MAP (SD) before and after acetaminophen was 69.6 mm Hg (14.8) and 67.4 mm Hg (13.9), respectively (p=0.001). Mean SBP (SD) before and after acetaminophen was 95.4 mm Hg (18.2) and 92.8 mm Hg (19.2), respectively (p=0.006). Baseline MAP, median (interquartile range (IQR)) was 76.0 (64.0-85.3) and 66.0 (57.0-74.5) in patients with and without haemodynamic events, respectively (p=0.004). Only 38/156 patients (24%) met the definition for hypotension. Baseline MAP, median (IQR) was 62.0 (51.8-79.0) in patients with, and 68.5 (62.0, 79.3) in patients without hypotension (p=0.036). Baseline shock, vasoactives, mechanical ventilation and paediatric sequential organ failure assessment were not significantly associated with hypotension. Only MAP was found to be associated with both haemodynamic event (adjusted odds ratio (AOR) 1.05, 95% CI 1.02-1.10) and hypotension (AOR 1.06, 95% CI 1.02-1.10) even after controlling for other confounders. CONCLUSIONS: Administration of intravenous acetaminophen in critically ill children can lead to haemodynamic changes, including clinically significant hypotensive events.

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.

Clinical course of COVID-19 among immunocompromised children: a clinical case series.

Infection with SARS-CoV-2 represents a great source of concern and a new threat for immunocompromised patients. Limited studies are available on COVID-19 in immunocompromised children. This case series aimed to evaluate the clinical and laboratory characteristics, management and outcomes of COVID-19 in five children immunocompromised due to different underlying conditions. All had mild symptoms or were asymptomatic at presentation. All had a benign course of illness. No changes or delays in their treatment regimens occurred, and none experienced a relapse of the original disease, developed severe COVID-19 or died. However, these cases showed a prolonged duration of virus shedding. This report suggests that immunocompromised paediatric patients may not be at a higher risk of developing severe COVID-19. However, further studies are required to elaborate on the pathogenesis of COVID-19 in this vulnerable group.

Diagnosing inborn error of immunity following the presentation of a complicated acquired infection after MMRV vaccine administration.

Live vaccine-acquired infection should attest for the occurrence of inborn errors of immunity. Autosomal recessive immunodeficiency 31B, a result of a signal transducer and activator of transcription 1 genetic mutation, results in defected interferon pathways: interferon alpha/beta and interferon gamma. These interferons are crucial for the defence against viral and mycobacterial infections. Recognition is important for preventive and therapeutic approaches. Herein, we report the presentation of a newly diagnosed 13-month-old child with immunodeficiency 31B after presenting with disseminated measles and varicella infection after Measles, Mumps, Rubella and Varicella vaccination.

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.

Adenovirus as a rare cause of acute necrotising encephalitis.

Adenovirus is a common cause of upper and lower respiratory tract infections. Rarely, neurological manifestations may occur, ranging from mild aseptic meningitis to potentially fatal acute necrotising encephalopathy (ANE). Very little is known in regards to the exact pathogenesis of ANE in association with adenovirus. This report describes the presentation of a previously healthy 14-month-old girl diagnosed with adenovirus-induced ANE. Herein, we highlight the clinicoradiological manifestation of this uncommon association with adenovirus in order to maintain a high index of suspicion for early diagnosis and a better outcome.

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.

A Retrospective Evaluation of Echocardiograms to Establish Normative Inferior Vena Cava and Aortic Measurements for Children Younger Than 6 Years.

OBJECTIVES: The ability to plot the inferior vena cava (IVC) size on a normal curve for pediatric patients may prove beneficial. First, in patients with normal cardiac anatomy who present in shock, assessing IVC size may be valuable for evaluating the degree of dehydration. Second, in children with heart disease, understanding how a child's IVC size compares to normal could be particularly beneficial for patients with right heart disease. We sought to create normal curves for the IVC and aorta in children younger than 6 years. METHODS: Data were gathered from 347 echocardiograms of healthy children younger than 6 years in a retrospective study at a quaternary care children's hospital. From the subcostal long- and short-axis images, maximum diameters in the transverse and longitudinal views were obtained for both the IVC and the aorta. RESULTS: Both IVC and aortic dimensions increased in a linear fashion and had excellent correlations with the body surface area, body mass, and height (IVC, r = 0.78-0.81; P < .0001; aorta, r = 0.82-0.86; P < .0001). CONCLUSIONS: In children younger than 6 years, the IVC and aorta increase linearly as the children grow. Such normal curves will be beneficial for assessing a pediatric patient's hydration status or right heart function in patients with congenital heart disease.

Inhaled ß2-Agonist Therapy Increases Functional Residual Capacity in Mechanically Ventilated Children With Respiratory Failure.

OBJECTIVES: To test the hypothesis that in mechanically ventilated children with respiratory failure, aerosolized albuterol modifies functional residual capacity, lung mechanics, oxygen consumption, and hemodynamics. DESIGN: Prospective, self-control clinical trial. SETTING: A 24-bed PICU in a quaternary care, academic children's hospital. PATIENTS: 25 children (age range, 1-18 yr) undergoing mechanical ventilation to treat respiratory failure. Entry criteria included previously prescribed inhaled ß2 agonists. Physiologic measurements were performed prior to and 20 minutes after administration of aerosolized albuterol solution. Functional residual capacity was determined via nitrogen washout. INTERVENTIONS: Functional residual capacity, oxygen consumption, respiratory mechanics, and vital signs were measured were measured prior to and 20 minutes after administration of aerosolized albuterol solution. Functional residual capacity was determined via nitrogen washout. MEASUREMENT AND MAIN RESULTS: At baseline, functional residual capacity is only 53% of predicted. After aerosolized albuterol, functional residual capacity increased by 18.3% (p = 0.008). Overall, aerosolized albuterol had no effect on airway resistance. However, in patients with an endotracheal tube size of more than or equal to 4.0 mm, resistance decreased from 33 ± 3 to 25 ± 3 (p < 0.02). Inhaled albuterol administration had no effect on oxygen consumption despite an increase in heart rate from 116 ± 2 to 128 ± 2 beats/min (p < 0.0001). CONCLUSIONS: In pediatric patients with respiratory failure, aerosolized albuterol increases functional residual capacity without a decrease in resistance. In infants and children, aerosolized albuterol might favorably enhance pulmonary mechanics and thereby represent a novel strategy for lung recruitment in children with respiratory failure.

Haemophagocytic lymphohistiocytosis associated with coccidiomycosis.

Haemophagocytic lymphohistiocytosis (HLH) is a rapidly fatal disease caused by dysregulated histiocytes leading to an excessive inflammatory reaction. While genetic forms of HLH exist, the most common form is acquired, frequently associated with infection. Here we report the first case of HLH associated with a coccidiomycosis infection. This patient is a 13-year-old previously healthy boy who presented with a flu-like illness, which rapidly progressed to refractory shock, severe ARDS, multiorgan failure and death despite maximal medical therapy, including broad-spectrum antibiotics to treat well-established causes of acquired HLH. Autopsy findings revealed the diagnosis of HLH in the setting of pulmonary coccidiomycosis. Antifungal therapy should be considered in cases of acquired HLH when the underlying aetiology is not clear.

Severe lactic acidosis and multiorgan failure due to thiamine deficiency during total parenteral nutrition.

A 16-year-old perioperative paediatric patient presented with refractory lactic acidosis and multiorgan failure due to thiamine-deficient total parenteral nutrition during a recent national multivitamin shortage. Urgent empiric administration of intravenous thiamine resulted in prompt recovery from this life-threatening condition. Despite readily available treatment, a high index of suspicion is required to prevent cardiovascular collapse and mortality.