Food insecurity and quality of life in patients with sickle cell disease.

Little is known about the relationship between quality of life (QOL) and food insecurity (FI) among patients with sickle cell disease (SCD). We hypothesized FI is associated with lower QOL in children and young adults with SCD. Overall (N = 99), 22% screened positive for FI. Supplemental Nutrition Assistance Program (SNAP) enrollment was 50 and 71% among people from food secure and FI households, respectively. A higher FI score was correlated with lower overall QOL (r = -0.22, p = .03), specifically lower QOL in worry and communication domains. Interventions for FI beyond SNAP may be important for QOL among people living with SCD.

Sickle cell trait and multisystem trauma: an unaddressed urgent knowledge gap.

Sickle cell trait (SCT) has historically been considered a benign condition, but SCT-positive patients have increased baseline risk of venous thromboembolism and chronic kidney disease, as well as increased risk of sickled erythrocytes in settings of hypoxia, acidosis, and hypovolemia. Multisystem traumatic injuries are a common clinical scenario, in which hypoxia, acidosis, and hypovolemia occur; however, little is known about how SCT-positive status impacts outcomes in multisystem trauma. We conducted a scoping literature review to investigate what was known about SCT in the setting of multisystem trauma. In the 110+ years that sickle cell hemoglobinopathies have been known, only three studies have ever examined the relationship between SCT and multisystem traumas. All three articles were case reports. None of the articles intentionally measured the association between SCT and multisystem trauma outcomes; they only incidentally captured information on SCT. Our article then examines historical reasons why so little research has studied the pathophysiology of the multisystem trauma in patients with SCT. Among the reasons is that historical and logistical factors have long prevented patients from knowing their SCT-status: historical discriminations against SCT-positive patients in the 1960s and 1970s delayed federal mandating of SCT newborn screening until 2006, whereas difficulties communicating known SCT-status to afflicted children also contributed to lack of patient knowledge. In light of our findings, we offer specific calls to action for the trauma surgery research community: (1) consider testing for SCT in trauma patients that have unexpected complications, particularly venous thromboembolism, rhabdomyolysis, or renal failure and (2) support research to understand how SCT impacts multisystem trauma outcomes. We also offer specific guidelines about how to 'proceed with caution' in implementation of these goals in light of the troubled history of SCT testing and policy in the USA.

A neonate with a vesiculopustular rash.

Transient myeloproliferative disorder (TMD) is a unique form of acute megakaryocytic leukemia (AMKL) that spontaneously regresses and is found in 3% to 10% of neonates with Down syndrome (DS). We report the case of a neonate with DS who presented with a widespread vesiculopustular eruption as an initial sign of TMD. Complete blood count was normal but peripheral smear revealed circulating megakaryoblasts. The severity of skin lesions correlated with the blast count. By age 2 months the TMD resolved and the patient remains disease-free after 18 months of follow-up. Several important features of TMD are highlighted: skin findings may provide an important clinical clue to TMD diagnosis; manual review of the peripheral smear is necessary when TMD is suspected; and patients with a history of TMD have a very high (∼30%) risk of recurrence with a persistent AMKL within the first 3 years of life.