Severe labile hypertension in a patient with catecholamine-secreting neuroblastoma: a case report.

Neuroblastoma is a common pediatric tumor arising from the post-ganglionic sympathetic nervous system and is associated with hypertension in 25% of cases. We describe an unusual case of labile, multi-drug resistant hypertension associated with chemotherapy administration for neuroblastoma and provide potential management strategies in this scenario. We report the case of a 4-year-old female with a history of headaches who presented with hypertensive emergency and evidence of end-organ damage, including posterior reversible encephalopathy syndrome, acute cerebral infarct, concentric left ventricular hypertrophy, and growth failure secondary to a large, abdominal catecholamine-secreting neuroblastoma, which compressed the kidney vasculature and inferior vena cava. She was classified as intermediate risk according to Children's Oncology Group criteria and underwent chemotherapy, complicated by labile hypertension, followed by surgical resection. Vigilance in monitoring and treatment of hypertension is recommended during chemotherapy for neuroblastoma due to the potential catecholamine release in the setting of tumor lysis.

Recent Advances in Sickle-Cell Disease Therapies: A Review of Voxelotor, Crizanlizumab, and L-glutamine.

Sickle-cell disease (SCD) is an inherited hemoglobinopathy, causing lifelong complications such as painful vaso-occlusive episodes, acute chest syndrome, stroke, chronic anemia, and end-organ damage, with negative effects on quality of life and life expectancy. Within the last five years, three new treatments have been approved: L-glutamine in 2017 and crizanlizumab and voxelotor in 2019. We conducted a literature search of these three medications, and of the 31 articles meeting inclusion criteria, 6 studied L-glutamine, 9 crizanlizumab, and 16 voxelotor. Treatment with L-glutamine was associated with decrease in pain crises, hospitalizations, and time to first and second crises, with a decrease in RBC transfusion rate. Barriers to filling and taking L-glutamine included insurance denial, high deductible, and intolerability, especially abdominal pain. Crizanlizumab was associated with a reduction in pain crises and time to first crisis, with reduction in need for opioid use. Adverse effects of crizanlizumab include headache, nausea, insurance difficulty, and infusion reactions. Voxelotor was associated with increased hemoglobin and decreased markers of hemolysis. Barriers for voxelotor use included insurance denial and side effects such as headache, rash, and diarrhea. These three medications represent exciting new therapies and are generally well-tolerated though price and insurance approval remain potential barriers to access. Other studies are ongoing, particularly in the pediatric population, and more real-world studies are needed. The objective of this article is to evaluate post-approval studies of crizanlizumab, voxelotor, and L-glutamine in SCD, with a focus on real-world efficacy, side effects, and prescribing data.

Accuracy of Parent Perception of Comprehension of Discharge Instructions: Role of Plan Complexity and Health Literacy.

OBJECTIVE: Inpatient discharge education is often suboptimal. Measures of parents' perceived comprehension of discharge instructions are included in national metrics given linkage to morbidity; few studies compare parents' perceived and actual comprehension. We 1) compared parent perceived and actual comprehension of discharge instructions and 2) assessed associations between plan complexity and parent health literacy with overestimation of comprehension (perceive comprehension but lack actual comprehension). METHODS: Prospective cohort study of English/Spanish-speaking parents (n = 192) of inpatients ≤12 years old and discharged on ≥1 daily medication from an urban public hospital. We used McNemar's tests to compare parent perceived (agree/strongly agree on 5-point Likert scale) and actual comprehension (concordance of parent report with medical record) of instructions (domains: medications, appointments, return precautions, and restrictions). Generalized estimating equations were performed to assess associations between low parent health literacy (Newest Vital Sign score ≤3) and plan complexity with overestimation of comprehension. RESULTS: Medication side effects were the domain with lowest perceived comprehension (80%), while >95% of parents perceived comprehension for other domains. Actual comprehension varied by domain (41%-87%) and was lower than perceived comprehension. Most (84%) parents overestimated comprehension in ≥1 domain. Plan complexity (adjusted odds ratio 3.6; 95% confidence interval 2.9-4.7) and low health literacy (adjusted odds ratio 1.9; 1.3-2.6) were associated with overestimation of comprehension. CONCLUSIONS: Parental perceived comprehension of discharge instructions overestimated actual comprehension in most domains. Plan complexity and low health literacy were associated with overestimation of comprehension. Future interventions should incorporate assessment of actual comprehension and standardization of discharge instructions.