Background
Delta granule storage pool deficiency (δ‐SPD) is a rare platelet disorder in which a deficiency of platelet granules leads to poor aggregation, resulting in varying clinical bleeding phenotypes. Children with δ‐SPD have variable laboratory results, making the proper diagnosis and evaluation controversial.
Objectives
To describe the demographic and laboratory trends of this population and to assess the value of electron microscopy in diagnostic evaluation and its correlation to bleeding symptoms.
Methods
We performed a retrospective review of 109 pediatric patients diagnosed with δ‐SPD. We collected demographic information and bleeding scores using a validated bleeding assessment tool. A descriptive and exploratory analysis was performed.
Results
The majority of patients were female, with an average age at diagnosis of 11.61 years. Females were diagnosed at a significantly older age presenting most often with menorrhagia, while males presented most commonly with epistaxis. The majority showed normal lumiaggregometry, the mean platelet electron microscopy (PEM) value was 2.37, and the mean bleeding score was 6. Bleeding assessment tool and PEM had a significantly weak correlation.
Conclusions
Patients with more dense granules per platelet had higher bleeding scores than those with fewer dense granules per platelet. The current body of evidence does not favor the use of PEM in routine clinical practice, and results are difficult to interpret. In patients with severe mucocutaneous bleeding symptoms and normal platelet aggregation studies, consideration should be given to an alternative diagnosis and further evaluation is warranted.
We present a case series of three febrile episodes in neutropenic pediatric cancer patients who wore a Food and Drug Administration approved high-frequency temperature monitoring (HFTM) wearable device (WD) at home. The WD detected fever events when temperature monitoring by thermometer did not detect fever or was not feasible to perform. Two of the episodes were associated with bloodstream infections and the WD detected fevers 5 and 12 h prior to fevers detected by thermometer, triggering earlier medical evaluation and more prompt administration of antibiotics. These observations provide a basis for future investigation of home-based HFTM to improve infection-related outcomes in pediatric oncology.
A 5-month-old previously healthy female presented with a one-week history of fever and increased fussiness. Her presentation revealed an ill-appearing infant with an exam and cerebrospinal fluid (CSF) studies concerning bacterial meningitis; CSF cultures grew Pasteurella multocida. Additionally, brain magnetic resonance imaging (MRI) demonstrated cervical osteomyelitis. Despite multiple days of antibiotic therapy, she remained febrile with continued pain; MRI showed oligoarticular effusions, and aspiration of these joints yielded bloody aspirates. Evaluations for coagulopathy and immune complex-mediated arthropathy were negative. The patient improved following appropriate antibiotic therapy and spontaneous resolution of hemarthroses, and was discharged to a short-term rehabilitation hospital. P. multocida is a small, encapsulated coccobacillus that is part of the commensal oral flora of animals. It most commonly causes skin infections in humans, yet is a rare cause of meningitis in the pediatric population, especially in children <1 year of age. Transmission due to P. multocida is most commonly due to direct contact with animals. To our knowledge, this is the first case of oligoarticular hemarthroses and cervical osteomyelitis complicating P multocida meningitis. This case highlights the physician’s potential for cognitive bias and premature anchoring, and the resulting implications in delivering excellent patient care.
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