Low glomerular filtration rate (GFR) prior to stem cell transplant (SCT) is associated with increased morbidity and mortality. The implications of abnormally high GFRs, or glomerular hyperfiltration, prior to SCT are unknown. Twenty two of 74 consecutive pediatric SCT patients over 2 years old at a single center were hyperfiltrating prior to SCT, median nuclear medicine GFR 154 mL/min/1.73 m 2 (IQR 146, 170). There was no association between any demographic and hyperfiltration, nor between hyperfiltration and acute kidney injury ( P = 0.8), renal replacement therapy ( P = 0.63), event-free survival ( P = 1), or chronic kidney disease ( P = 0.73).

Myelofibrosis (MF) in the pediatric setting is uncommon and appears to be pathogenically heterogeneous. MF due to intrinsic bone marrow abnormality (IMF) is distinct from adult-type Primary myelofibrosis (PMF) as they can lack the common genetic markers of clonality. To date, all but two reported patients with pediatric MF and mutated MPIG6B have been Arabic, and all reported cases have had a family history of consanguinity. Here we report the first North American patient of European ancestry with pediatric MF in whom novel compound heterozygous mutations of MPIG6B were identified.

Because they can experience neutropenia due to bone marrow failure, patients with Shwachman-Diamond syndrome (SDS) carry increased risk for serious infections compared to the general population; however, it has been unknown whether this predisposes them to COVID-19 infection or more significant complications. We compiled results from a survey distributed to participants in the Shwachman-Diamond Syndrome Registry between May and June 2021. In this report we describe the characteristics and outcomes of patients with SDS who had COVID-19. Patients reported a short clinical course without significant complications or severe cytopenias. Additionally, COVID-19 vaccines were well tolerated with only minor side effects.