Hyperglycemia is detrimental to postoperative islet cell survival in patients undergoing total pancreatectomy with islet autotransplantation (TPIAT). This makes continuous glucose monitoring (CGM) a useful management tool. We evaluated the accuracy of the Dexcom G6 CGM in pediatric intensive care unit patients following TPIAT. Twenty-five patients who underwent TPIAT had Dexcom G6 glucose values compared to paired serum glucose values. All paired glucose samples were obtained within 5 minutes of each other during the first seven days post TPIAT. Data were evaluated using mean absolute difference (MAD), mean absolute relative difference (MARD), %20/20, %15/15 accuracy, and Clarke Error Grid analysis. Exclusions included analysis during the CGM “warm-up” period and hydroxyurea administration (known drug interference). A total of 183 time-matched samples were reviewed during postoperative days 2–7. MAD was 14.7 mg/dL and MARD was 13.4%, with values of 15.2%, 14.0%, 12.1%, 11.4%, 13.2% and 14.1% at days 2, 3, 4, 5, 6 and 7, respectively. Dexcom G6 had a %20/20 accuracy of 78%, and a %15/15 accuracy of 64%. Clarke Error Grid analysis showed that 77% of time-matched values were clinically accurate, and 100% were clinically acceptable. The Dexcom G6 CGM may be an accurate tool producing clinically acceptable values to make reliable clinical decisions in the immediate post-TPIAT period.

Tyrosine kinase inhibitors that target vascular endothelial growth factor receptor [VEGFR-TKI] are a class of targeted therapies approved for treatment of several malignancies and are increasingly used in the pediatric population. Development of hypothyroidism during VEGFR-TKI therapy is well described in adults; however, there are no available data in children. Importantly, hypothyroidism during childhood can negatively impact growth and neurodevelopment. This retrospective study is the first to document frequency and severity of VEGFR-TKI induced hypothyroidism in pediatric and young adult patients. Patients included were ≤25 years of age and treated with at least one VEGFR-TKI between 2010 and 2018 at Cincinnati Children’s Hospital Medical Center. After review of clinical and demographic data, 69 patients were identified. Of these, 19 (27.5%) developed thyroid dysfunction defined as Thyroid-stimulating hormone≥5 mIU/mL during therapy. Twelve of those patients had overt hypothyroidism with documentation of low free thyroxine and/or levothyroxine initiation. Mean exposure time to VEGFR-TKI before thyroid dysfunction was 2.8 (0.5-10.4) months. These results suggest moderate risk of developing thyroid dysfunction during VEGFR-TKI therapy in pediatric and young adult patients. Baseline thyroid hormone screening should be performed and repeated frequently during the first year of therapy in the pediatric population.

Generalized glucocorticoid (GC) resistance (Chrousos syndrome) results from impaired GC receptor signaling due to variants in the NR3C1 gene. Severe neonatal presentations are extremely rare. We report an 8-week-old male infant born at 31-week gestation, with progressive respiratory failure, refractory hypotension, hypokalemic alkalosis, anasarca, jaundice, and multiorgan dysfunction. Laboratory evaluation demonstrated persistently elevated serum cortisol [>60 µg/dL (SI: >1655 nmol/L) (reference range 2.5-9.1 µg/dL, SI: 69.0-251.0 nmol/L)], adrenocorticotropic hormone [>2400 pg/mL (SI: >528 pmol/L) (reference range 7.2-63.3 pg/mL, SI: 1.6-13.9 pmol/L)], aldosterone [2036.0 ng/dL (SI: 56 nmol/L) (reference range 7.0-99.0 ng/dL, SI: 0.2-2.7 nmol/L)], and androgens despite treatment with escalating doses of dexamethasone (up to 12 mg, > 2000 mg/m2/day of hydrocortisone-equivalency). Whole-genome sequencing identified 2 NR3C1 variants: a maternally inherited variant of uncertain significance (c.2181+5 G>C) and a de novo likely pathogenic ∼7.9 kb deletion encompassing exon 8. In silico analyses suggested both variants could disrupt the GC receptor ligand-binding domain, potentially resulting in complete loss of GC receptor function. Autopsy demonstrated adrenomegaly with marked proliferation of zona fasciculata, cholestasis with bile duct paucity, and chronic neonatal lung disease. This case represents a rare and fatal manifestation of complete generalized GC resistance. It highlights the broad physiological role of GC in neonatal homeostasis.

Aim: Youth‐onset type 2 diabetes and obesity are rising public health burdens. While newer type 2 diabetes medications lower HbA1c, their impact on paediatric weight loss is inconsistent. In contrast, bariatric surgery improves glycemia and weight in youth and adults, though glycemia post‐vertical sleeve gastrectomy (VSG), now the predominant bariatric surgery in youth, remains unstudied in youth‐onset type 2 diabetes. We aimed to assess immediate post‐VSG glycemic patterns using continuous glucose monitoring (CGM) in adolescents with type 2 diabetes. Materials and Methods: In the Surgical or Medical Treatment for Paediatric Type 2 Diabetes (ST ₂ OMP) study, 73 adolescents with type 2 diabetes underwent 14 days of CGM monitoring: 33 participants beginning in the 1–2 days post‐VSG (mean age 17.6 years, 67% female, HbA1c 7.2%, BMI 50.0 kg/m ² ) and 40 participants receiving advanced medical therapy (AMT) (mean age 16.9 years, 53% female, HbA1c 7.8%, BMI 42.2 kg/m ² ). CGM analyses included glucose management indicator (GMI), time in range, coefficient of variability, standard deviation, mean amplitude glycemic excursion, percent > 140, 180, and 200, percent < 70 and 60 mg/dL, 7 a.m., and midnight‐6 a.m. Results: Mean glucose was 84.3 ± 23.4 in the post‐VSG group versus 149.8 ± 78.8 mg/dL in the AMT group ( p < 0.001). Mean CGM GMI was normal (5.3%, 34 mmol/mol) post‐VSG versus in the diabetes range (6.9%, 52 nmol/mol) with AMT ( p < 0.001). VSG participants spent more time < 70 mg/dL, 55 mg/dL, and 45 mg/dL (all p < 0.01). After BMI adjustment, preoperative HbA1c predicted postoperative glucose metrics. Conclusion: Adolescents with type 2 diabetes experience early glycemic normalization post‐VSG, even before major weight loss.