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Abdomen
Published in Bobby Krishnachetty, Abdul Syed, Harriet Scott, Applied Anatomy for the FRCA, 2020
Bobby Krishnachetty, Abdul Syed, Harriet Scott
Functional asplenia occurs when splenic tissue is present but does not function properly and is characterised by the loss of phagocytosis. It occurs in autoimmune diseases, inflammatory bowel disease, sickle cell disease, beta thalassemia, chronic graft-versus-host disease and can be caused by splenic tissue infiltration by tumour cells, sickled erythrocytes, etc.
Overwhelming Post-Splenectomy Infections in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
In order to focus the treating clinician appropriately, the patient without a spleen or with an underlying condition that may produce a functional hyposplenism should have a medical alert necklace or bracelet proclaiming this fact. Additionally, since truth can have a short half-life, the patient and his or her relatives should be aware of relating this fact to the healthcare deliverer at every visit involving an acute febrile illness. Since the splenectomy may be remote in time to the illness, this is even more important. As stated, rarely congenital asplenia may present with severe infections in adulthood, so evidence to support the diagnosis of the absent spleen should be looked for in every presentation of significant febrile illness [14].
Hemoglobin CC and SC Red Cells
Published in Ronald L. Nagel, Genetically Abnormal Red Cells, 2019
Mary E. Fabry, Ronald L. Nagel
Both splenomegaly and asplenia occur in SC patients. Asplenia is less common than in SS patients. The relative risk of bacteremia is smaller for SC patients than for SS patients when correction is made for the unhospitalized population at risk,58 but is much larger than that for the normal population; however, the type of systemic infection most commonly found in SC patients (Gram negative bacteria) was less life-threatening than those most commonly found in SS patients (pyogenic bacteremia).
Streptococcal pyomyositis in asplenia and underlying connective tissue disease
Published in Baylor University Medical Center Proceedings, 2023
John Nguyen, Pardeep Singh, Tapas Gajjar
This patient had increased risk of S. pneumoniae infection given his immunocompromised state from asplenia. He also had a confirmed diagnosis of Stickler syndrome with retinal detachment and genetic testing in childhood. The extent to which Stickler syndrome contributed to his pyomyositis is unclear; however, the compromised connective tissue in his joints possibly increased the risk of bacterial seeding. The significance of his elevated anti-CCP levels is also unclear since he did not have synovitis on exam to suggest rheumatoid arthritis. We suspect this patient had subclinical S. pneumoniae pneumonia with bacterial seeding in his left supraspinatus tear, right knee following minor trauma, and further seeding into his thoracic spine and lower extremity muscles. His prolonged pain and weakness in the knees, hips, and shoulders were also possibly related to his Stickler syndrome. In hospitalized patients with multiple underlying conditions, it is important to consider how their chronic medical conditions contribute to their acute illness.
Strategies to increase access to basic sickle cell disease care in low- and middle-income countries
Published in Expert Review of Hematology, 2022
Meghna Dua, Halima Bello-Manga, Yvonne M. Carroll, Aisha Amal Galadanci, Umma Abdulsalam Ibrahim, Allison A. King, Ayobami Olanrewaju, Jeremie H. Estepp
SCD is a monogenic red blood cell (RBC) disorder, where normal hemoglobin (HbA) is replaced by sickle hemoglobin (HbS). It is inherited as an autosomal codominant trait [1], and common types of SCD include homozygous hemoglobin SS disease, hemoglobin SC disease, and sickle beta-thalassemia[1]. It is a chronic and debilitating condition characterized by hemolytic anemia and endothelial dysfunction, with findings of vaso-occlusive crises, acute chest syndrome, increased risk of stroke, and cumulative multiorgan damage. Children with SCD are also at heightened risk of morbidity and mortality from specific infections due to functional asplenia. There are approximately 300,000–400,000 babies born with SCD globally, with over 75% of them born in Africa [1]. Nigeria, India, and the Democratic Republic of the Congo alone account for over 50% of patients with SCD [2]. This geographic distribution is attributed to the ‘malaria hypothesis’ that the HbS carrier state is protective against malaria infection, which was substantiated by the coexistence of high HbS carrier rates and malaria infections in Africa [3].
Bringing Sickle Cell Disease Care Closer to Home: Feasibility and Efficacy of a Quality Improvement Initiative at a Community Hospital
Published in Hemoglobin, 2022
Sickle cell disease is the most common clinically significant monogenic disorder with over 300,000 children born worldwide, annually [1]. Sickle cell disease is characterized by severe pain, stroke, avascular necrosis, retinopathy and increased susceptibility to infections [2]. This results in a natural history of early death with childhood mortality of 50.0–90.0% in low resource settings [3]. Access to comprehensive care centers for sickle cell disease in developed nations has dramatically improved childhood mortality rates to less than 7.0% [4–6]. This is in part attributable to a significant reduction in pain, hospitalizations and mortality with hydroxyurea (HU), the only widely available treatment for sickle cell disease [7,8]. Other important features of comprehensive care for this condition include access to regular clinic visits for patient education, screening for complications, vaccinations for functional asplenia and penicillin prophylaxis. These have also been shown to improve morbidity and mortality in this population [9]. However, frequent pain in childhood and early adult mortality remain challenges in treating this population [5]. Contributing to this is a lack of education in patients and health care providers regarding sickle cell disease care [10,11]. Transition age patients with sickle cell disease have been demonstrated to be at particularly high risk of morbidity and mortality due to factors related to transfer of care from pediatric to adult care and psychosocial factors related to young adulthood [6,12].