Case report: Early molecular confirmation and sodium polystyrene sulfonate management of systemic pseudohypoaldosteronism type I.

Introduction: Type 1 pseudohypoaldosteronism (PHA) consists of resistance to aldosterone. Neonatal presentation is characterized by salt wasting, hyperkalemia, and metabolic acidosis with high risk of mortality. Type 1 PHA can be autosomal dominant (renal type 1) or autosomal recessive (systemic type 1). Renal PHA type 1 can be feasibly managed with salt supplementation; however, systemic PHA type 1 tends to have more severe electrolyte imbalance and can be more refractory to treatment. Case Presentation: We present a case of a 3-year-old girl with systemic PHA type 1, diagnosed and confirmed molecularly in infancy, who has been successfully managed with sodium polystyrene sulfonate decanted into feeds along with sodium supplementation. On day 5 of life, a full-term female infant presented to the ED for 2 days of non-bloody, non-bilious emesis, along with hypothermia to 94°F. Laboratory results were notable for hyponatremia (Na) of 127, hyperkalemia (K) of 7.9, and acidosis with bicarbonate level of 11.2. Genetic testing ordered within a week of life confirmed PHA type 1 with a homozygous pathogenic frameshift variant in SCNN1A c.575delA (p.Arg192GlyfsX57). Sodium polystyrene sulfonate and feeds were decanted until the age of 16 months, and she was also continued on NaCl supplementation. She was gradually transitioned to directly administered sodium polystyrene sulfonate without any electrolyte issues. She has overall done well after gastrostomy-tube (G-tube) placement without severe hyperkalemia even with several hospitalizations for gastrointestinal or respiratory illnesses. Discussion/Conclusion: A treatment approach to systemic PHA and sodium polystyrene sulfonate administration in neonates and infants is described.

When Duty Calls, Resident Physicians Answer: Learning to Take Transfer Calls.

BACKGROUND AND OBJECTIVE: Transitions of care are error-prone. Standardized handoffs at transitions improve safety. There are limited published curricula teaching residents to perform interfacility transfer calls or providing a framework for performance evaluation. The objective of this study was to measure the impact of a workshop utilizing a standardized handoff tool on resident-reported confidence in taking transfer calls and observed behavioral change in a simulated environment. METHODS: A pre- and posteducational intervention trial was performed at a large children's hospital in March 2021. A 1-hour session highlighting the importance of phone communication, outlining an evidence-based handoff tool, and reviewing cases was delivered to 44 of 75 residents who attended scheduled didactics. The workshop's effectiveness was measured by rating behavioral change in a simulated environment. Calls were scored by using a 0 to 24 summative score checklist created from the handoff tool. A paired t test was used to analyze the differences in pre- and postintervention scores. Resident confidence, knowledge of the call process, and perceived importance of skill were measured with an internally developed retrospective pre- and postsurvey. The survey results were analyzed with a Wilcoxon rank test and Kruskal-Wallis test. RESULTS: Behaviors in a simulated environment, measured by an average score on the grading checklist, had a mean score increase of 6.52 points (P <.0001). Of the participants, 95% completed the survey, which revealed that reported confidence, knowledge of the transfer call process, and importance of transfer call skills increased significantly (P <.0001). CONCLUSIONS: This workshop improved resident behaviors in a simulated environment, confidence, and knowledge of the transfer call process, demonstrating the utility of providing a standardized tool and education to improve transitions of care.