Adrenal insufficiency in pediatric kidney transplantation recipients.

BACKGROUND: Immunosuppression of pediatric kidney transplant (PKT) recipients often includes corticosteroids. Prolonged corticosteroid exposure has been associated with secondary adrenal insufficiency (AI); however, little is known about its impact on PKT recipients. METHODS: This was a retrospective cohort review of PKT recipients to evaluate AI prevalence, risk factors, and adverse effects. AI risk was assessed using morning cortisol (MC) and diagnosis confirmed by an ACTH stimulation test. Potential risk factors and adverse effects were tested for associations with MC levels and AI diagnosis. RESULTS: Fifty-one patients (60.8% male, age 7.4 (IQR 3.8, 13.1) years; 1 patient counted twice for repeat transplant) were included. Patients at risk for AI (MC < 240 nmol/L) underwent definitive ACTH stimulation testing, confirming AI in 13/51 (25.5%) patients. Identified risk factors for AI included current prednisone dosage (p = .001), 6-month prednisone exposure (p = .02), daily prednisone administration (p = .002), and rejection episodes since transplant (p = .001). MC level (2.5 years (IQR 1.1, 5.1) post-transplant) was associated with current prednisone dosage (p < .001), 6-month prednisone exposure (p = .001), daily prednisone administration (p = .006), rejection episodes since transplant (p = .003), greater number of medications (ß = -16.3, p < .001), 6-month hospitalization days (ß = -3.3, p = .013), creatinine variability (ß = -2.4, p = .025), and occurrence of acute kidney injury (ß = -70.6, p = .01). CONCLUSION: Greater corticosteroid exposure was associated with a lower MC level and confirmatory diagnosis of AI noted with an ACTH stimulation test. Adverse clinical findings with AI included greater medical complexity and kidney function lability. These data support systematic clinical surveillance for AI in PKT recipients treated with corticosteroids.

Designing a Collaborative Patient-Centered Digital Health Platform for Pediatric Diabetes Care in British Columbia: Formative Needs Assessment by Caregivers of Children and Youths Living With Type 1 Diabetes and Health Care Providers.

BACKGROUND: Digital health apps are becoming increasingly available for people living with diabetes, yet data silos continue to exist. This requires health care providers (HCPs) and patients to use multiple digital platforms to access health data. OBJECTIVE: In this study, we gathered the perspectives of caregivers of children and youths living with type 1 diabetes (T1D) and pediatric diabetes HCPs in the user-centered design of TrustSphere, a secure, single-point-of-access, integrative digital health platform. METHODS: We distributed web-based surveys to caregivers of children and youths living with T1D and pediatric diabetes HCPs in British Columbia, Canada. Surveys were designed using ordinal scales and had free-text questions. Survey items assessed key challenges, perceptions about digital trust and security, and potential desirable features for a digital diabetes platform. RESULTS: Similar challenges were identified between caregivers of children and youths living with T1D (n=99) and HCPs (n=49), including access to mental health support, integration of diabetes technology and device data, and the ability to collaborate on care plans with their diabetes team. Caregivers and HCPs identified potential features that directly addressed their challenges, such as more accessible diabetes data and diabetes care plans. Caregivers had more trust in sharing their child's data digitally than HCPs. Most caregivers and HCPs stated that an integrative platform for T1D would support collaborative patient care. CONCLUSIONS: Caregiver and HCP perspectives gathered in this study will inform the early prototype of an integrative digital health platform. This prototype will be presented and iterated upon through a series of usability testing sessions with caregivers and HCPs to ensure the platform meets end users' needs.

The use of CGM to identify hypoglycemia and glycemic patterns in congenital hyperinsulinism.

OBJECTIVES: Unrecognized hypoglycemia, especially in the neonatal population, is a significant cause of morbidity and poor neurologic outcomes. Children with congenital hyperinsulinism (HI) are at risk of hypoglycemia and point of care testing (POCT) is the standard of care. Studies have shown that continuous glucose monitoring (CGM) improves glycemic control and reduces the frequency of hypoglycemia among children with type 1 diabetes. There is limited experience with the use of CGM in children with HI. To assess the glycemic pattern of children with HI on stable therapy and evaluate the frequency of undetected hypoglycemia using Dexcom G6® CGM. METHODS: A cross-sectional, observational pilot study was done in 10 children, ages 3 months to 17 years. Each child had a clinical or genetic diagnosis of HI on stable medical therapy. Participants were asked to continue their usual POCT blood glucose monitoring, as well as wear a blinded Dexcom G6® CGM during a 20-day study period with the potential of unblinding if there was severe hypoglycemia detected during the study trial. RESULTS: During the study period, 26 hypoglycemic events were noted by CGM in 60 % of the participants with 45 % occurring between 0600 and 0800. CONCLUSIONS: CGM can help detect hypoglycemia and blood glucose trends during a time when there is usually no POCT, which can guide medical management. 30 % of our population had a dose adjustment in their medications. This study was limited by population size.

A Pediatric Case of Reninoma Presenting with Paraneoplastic SIADH.

INTRODUCTION: A reninoma (juxtaglomerular cell tumour) is a rare cause of secondary hypertension that can present with headaches alongside the triad of severe hypertension, hypokalemia, and metabolic alkalosis. CASE PRESENTATION: We describe a case of a 15-year-old previously healthy girl who presented with headaches and hypertensive urgency who had severe hypokalemia, moderate hyponatremia and elevated aldosterone and renin levels. Abdominal ultrasound and MRI with contrast revealed a unilateral mass localized to the right kidney. Despite treatment of her hypertension, she had persistent hyponatremia with clinical euvolemia which was consistent with the paraneoplastic syndrome of inappropriate antidiuretic hormone secretion (SIADH). She underwent radical nephrectomy which normalized her blood pressure and aldosterone and renin values. The pathology findings were consistent with a reninoma with a mitotic rate of 1-2 mitoses per 10 high power fields. DISCUSSION/CONCLUSION: Hypertension in the pediatric age group requires work-up to rule out secondary causes. The classic triad of hypertension, hypokalemia, and metabolic alkalosis warrants assessment for aldosterone-mediated hypertension which can be a result of a renin-producing tumour. Curative approach requires surgical resection of the tumour. Reninomas may rarely manifest with a paraneoplastic phenomenon including SIADH, as seen in our case. Although reninomas are benign tumours, there are also a few reports of malignant transformation and metastases. Features uncommon in reninomas such as mitotic activity warrant long-term surveillance.

Continuous Glucose Monitoring to Diagnose Hypoglycemia Due to Late Dumping Syndrome in Children After Gastric Surgeries.

Gastrostomy tubes (G-tubes) and Nissen fundoplication are common surgical interventions for feeding difficulties and gastroesophageal reflux disease in children. A potential yet often missed, complication of these procedures is dumping syndrome. We present 3 pediatric patients with postprandial hypoglycemia due to late dumping syndrome after gastric surgeries. All patients received gastrostomy tubes for feeding intolerance: 2 had Nissen fundoplication for gastroesophageal reflux disease, and 1 had tracheoesophageal repair. All patients underwent multiple imaging studies in an to attempt to diagnose dumping syndrome. Continuous glucose monitoring (CGM) was essential for detecting asymptomatic hypoglycemia and glycemic excursions occurring with feeds that would have gone undetected with point-of-care (POC) blood glucose checks. CGM was also used to monitor the effectiveness of treatment strategies and drive treatment plans. These cases highlight the utility of CGM in diagnosing postprandial hypoglycemia due to late dumping syndrome, which is infrequently diagnosed by imaging studies and intermittent POC blood glucose measurements.

Youth with Type 1 Diabetes Had Improvement in Continuous Glucose Monitoring Metrics During the COVID-19 Pandemic.

Background: The impact of the coronavirus disease-2019 (COVID-19) pandemic on glycemic metrics in children is uncertain. This study evaluates the effect of the shelter-in-place (SIP) mandate on glycemic metrics in youth with type 1 diabetes (T1D) using continuous glucose monitoring (CGM) in Northern California, United States. Methods: CGM and insulin pump metrics in youth 3-21 years old with T1D at an academic pediatric diabetes center were analyzed retrospectively. Data 2-4 months before (distant pre-SIP), 1 month before (immediate pre-SIP), 1 month after (immediate post-SIP), and 2-4 months after (distant post-SIP) the SIP mandate were compared using paired t-tests, linear regression, and longitudinal analysis using a mixed effects model. Results: Participants (n = 85) had reduced mean glucose (-10.3 ± 4.4 mg/dL, P = 0.009), standard deviation (SD) (-5.0 ± 1.3 mg/dL, P = 0.003), glucose management indicator (-0.2% ± 0.03%, P = 0.004), time above range (TAR) >250 mg/dL (-3.5% ± 1.7%, P = 0.01), and increased time in range (TIR) (+4.7% ± 1.7%, P = 0.0025) between the distant pre-SIP and distant post-SIP periods. Relationships were maintained using a mixed effects model, when controlling for other demographic variables. There was improvement in SD, TAR 180-250 mg/dL, and TIR for participants with private insurance, but changes in the opposite direction for participants with public insurance. Conclusions: Improvement in CGM metrics in youth with T1D during the COVID-19 pandemic suggests that diabetes management can be maintained in the face of sudden changes to daily living. Youth with public insurance deserve more attention in research and clinical practice.