Cardiac critical care of the post-operative congenital heart disease patient.

Cardiac critical care has become an increasingly complex subspecialty, involving multiple subspecialists to support patients with congenital heart disease. This requires understanding of their physiology and the impact of medical interventions. The purpose of this article is to provide a concise review of the current strategies utilized by cardiac intensivists to optimize outcomes for this vulnerable patient population, with the goal of broadening the knowledge of other members of the multi-disciplinary team.

Implementation and Maintenance of a Pediatric Severe Burn Guidelines Quality Improvement Project.

Introduction: Critically injured pediatric burn patients require specialized management, yet few verified pediatric burn centers exist in the United States. Many pediatric hospitals have resources to care for severely burned patients but lack standardized care guidelines, which improve outcomes. To improve the morbidity and mortality of severely burned pediatric patients admitted to the pediatric intensive care unit, we created a specialized burn team. We implemented Pediatric Severe Burn Guidelines, focusing on improving fluid resuscitation accuracy and providing timely nutritional support. Methods: This investigation is of a 9-year (2010-2019) retrospective preintervention and postintervention study of the effect of the formation of a multidisciplinary burn leadership committee and development and implementation of Pediatric Severe Burn Guidelines. The primary outcome measures are increasing the accuracy of fluid resuscitation and improving the timely administration of nutritional support. The process measure is the percentage of time the electronic health record power plan was used for burn admissions with burn leadership review of the cases. Balancing measures are pediatric intensive care unit and hospital length of stay. Results: Preprotocol patients received acceptable fluid resuscitation 25% (5/20) of the time compared to 61.5% (8/13) of the time in postprotocol patients (P = 0.04). In postprotocol patients, there is an improvement in the timely placement of postpyloric feeding tube and initiation of feeds 48 hours after admission. Conclusions: Extensive guidelines for standardized care require careful implementation and monitoring of adherence gaps. Creating a specialized burn team and implementing clinical guidelines standardize care leading to improvement in critical patient outcomes.

Adrenal hypoplasia congenita presenting as congenital adrenal hyperplasia.

We report on a patient with genetically confirmed adrenal hypoplasia congenita (AHC) whose presentation and laboratory abnormalities were consistent with the more common condition, congenital adrenal hyperplasia (CAH). The patient presented with failure to thrive and salt wasting. General appearance showed marked hyperpigmentation and normal male genitalia. He displayed mildly elevated 17-hydroxyprogesterone and markedly elevated 11-deoxycortisol levels at baseline and with ACTH stimulation testing. Results were consistent with 11 ß -hydroxylase deficiency. He required glucocorticoids and high doses of mineralocorticoids. The marked elevation in 11-deoxycortisol directed our clinical reasoning away from a hypoplastic condition and towards a hyperplasic adrenal condition. Sequencing of the DAX1 gene (named for dosage-sensitive sex reversal (DSS) locus and the AHC locus on the X chromosome) revealed a missense mutation. A review of the literature revealed that elevated 11-deoxycortisol levels have been noted in kindreds with DAX1 mutations, but only when measured very early in life. A mouse model has recently been described that displays elevated 11-deoxycorticosterone levels and evidence for hyperplasia of the zona glomerulosa of the adrenal gland. We conclude that DAX1 testing may be considered in patients with laboratory evidence of 11 ß -hydroxylase deficiency, especially in those with severe salt wasting.