Enhancing Impact: The Use of VA-ECMO in a Critically Ill Pediatric Patient with Sickle Cell Disease, Acute Chest Syndrome, and Streptococcal Toxic Shock Syndrome

Age-dependent pathophysiology of acute chest syndrome in children with sickle cell disease.

1. Department of Haematology, Guy’s and St Thomas’ NHS Foundation Trust 2. Lane Fox Respiratory Unit, St Thomas’ Hospital, Guy’s and St Thomas’ NHS Foundation Trust 3. Department of Haematology, North Middlesex Hospital 4. Department of Paediatric Haematology, Bristol Royal Hospital for Children 5. Department of Haematological Medicine, King’s College Hospital 6. Department of Haematology, Whittington Hospital

Maximizing Survival: VA-ECMO As a Lifeline in a Critically Ill Pediatric Patient with Sickle Cell Disease, Acute Chest Syndrome, and Streptococcal Toxic Shock Syndrome

Blood exchange transfusion with dexamethasone and Tocilizumab for management of hospitalized patients with sickle cell disease and severe COVID-19: Preliminary evaluation of a novel algorithm.

Sputum IL-6 level as a potential predictor of acute chest syndrome during vaso-occlusive crisis in children with sickle cell disease: Exploratory prospective prognostic accuracy study.

Prognosis of patients with sickle cell disease and COVID-19: a French experience

Exosomes from Patients with Sickle Cell Disease and History of Acute Chest Syndrome Alter Endothelial Integrity In Vitro

Venous Thromboembolism In Pregnant Women With Sickle Cell Disease

28-Year-Old Man With Joint Pain

Acute Chest Syndrome at the Emory University Georgia Comprehensive Sickle Cell Center at Grady Health System

Secretory Phospholipase A2(sPLA2) in Patients with Sickle Cell Disease (SCD) Hospitalized for Vaso-Occlusive Pain Episodes (VOE)

1. Kathryn Westphal, MD* 2. Respicius Bakalemwa, MD† 3. Elizabeth Groothuis, MD, MPH* 1. *Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL 2. †Department of Pediatrics, Bugando Medical Centre, Mwanza, Tanzania A 12-year-old boy with sickle cell disease (SCD) is admitted to a hospital in Mwanza, Tanzania, with acute onset of periorbital and scalp swelling. Associated symptoms include fever, headache, difficulty breathing, and back pain. Because resources in sub-Saharan Africa are limited, access to comprehensive care for patients with SCD is variable. The general principles of management focus on early diagnosis, parental education, prevention of infection with vaccinations and prophylactic medications, prompt treatment of infection and pain, the use of hydroxyurea for stroke prevention, and blood transfusions for life-threatening anemia. This patient has a known diagnosis of SCD but has infrequent pain episodes and no previous transfusions. He is not receiving hydroxyurea or prophylaxis against bacterial infections or malaria. He has no history of stroke and no known head trauma. On presentation he is afebrile and his heart rate is 120 beats/min, respiratory rate is 40 breaths/min, and blood pressure is 105/75 mm Hg. His oxygen saturation is 92% in room air. Physical examination is significant for pallor, periorbital edema, scalp edema, dyspnea without adventitious lung sounds, and splenomegaly with diffuse abdominal tenderness. His scalp edema is fluctuant and extends posteriorly from the periorbital region, encompassing the entire scalp. Swelling is most prominent in the frontal and parietal areas. The overlying skin is nonerythematous and nontender but is warm to the touch. His neurologic examination has no focal findings, although generally he appears tired. Initial laboratory results demonstrate a white blood cell count of 84,000/μL (84×109/L) with a lymphocytic predominance (57%), a hemoglobin level of …

HIV and sickle cell disease (SCD) are significant causes of morbidity and mortality in sub-Saharan Africa. Given their separate roles in immune dysregulation, our objective was to characterise the impact that SCD has on the presentation and progression of paediatric HIV. The study was a retrospective cohort study (study period 2004-2018). Cases of HIV+and SCD-afflicted patients (HIV+/SCD+) were obtained via electronic chart review from a paediatric HIV clinic in Kampala, Uganda and matched 1:3 with HIV+controls without SCD (HIV+/SCD-). Thirty-five HIV+/SCD+subjects and 95 HIV+/SCD- controls were analysed (39% female (51/130), age 3.6years (SD3.9)). At baseline, WHO clinical stage (64% total cohort Stage III/IV) and nutritional status (9.4% severe acute malnutrition) were similar for both groups, whereas HIV+/SCD+had higher though non-significant baseline CD4 count (1036 (SD713) vs 849 (SD638) cells/microlitre, P=0.20, two-tailed t-test). There were 19 deaths, 6 (17%) HIV+/SCD+and 13 (14%) HIV+/SCD-, with unadjusted/adjusted models showing no significant difference. Nutritional progression and clinical stage progression showed no significant differences between groups. Kaplan-Meier analysis showed a slower rate of treatment failures in the HIV+/SCD+cohort (P=0.11, log-rank survival test). Trajectory analysis showed that in the time period analysed, the HIV+/SCD+cohort showed a more rapid rise and higher total CD4 count (P=0.012, regression analysis). The study suggests that SCD does not adversely affect the progression of HIV in patients on ART. Further, HIV+/SCD+achieved higher CD4 counts and fewer HIV treatment failures, suggesting physiological effects due to SCD might mitigate HIV progression.

Complement Drives Acute Chest Syndrome in a Model of Sickle Cell Disease

On March 12th 2021 the Italian Government decided to implement a national lockdown in almost all the regions of the country. It was the second most severe measure taken after the March 2020 national lockdown, due to the rising of coronavirus disease 2019 (COVID-19) cases and the overcrowding of the hospitals. The first peak of 29 000 hospitalised individuals, including children, except those in intensive care units (ICUs) was recorded in April 2020. Most hospitals had to rapidly implement strategies to ensure care for non-COVID-19 patients. The majority of children with sickle cell disease (SCD) live in the Northern regions of Italy. Therefore, the dramatic scenario represented an opportunity to explore the challenges presented for children with SCD who lived in Italy during the first outbreak, so that the lessons learned could be used to guide clinical management in the upcoming months.