Experience in a PHTS Expertise Centre: Yield of Thyroid Ultrasound Surveillance in Children with PTEN Hamartoma Tumor Syndrome.

Objective: Children with PTEN hamartoma tumor syndrome (PHTS) are at increased risk for developing thyroid abnormalities, including differentiated thyroid carcinoma (DTC). The Dutch PHTS guideline recommends ultrasound surveillance starting from age 18. Since the literature describes PHTS patients who developed DTC before age 18, the Dutch PHTS expertise centre has initiated annual ultrasound surveillance starting from age 12. The purpose of this study was to identify the yield of thyroid ultrasound surveillance in children. Methods: A retrospective single centre cohort study was conducted. Pediatric PHTS patients who received thyroid ultrasound surveillance before age 18 between 2016-2023 were included. Patients' medical records have been reviewed. Primary outcomes included prevalence and time to develop thyroid nodules ≥10mm, nodular growth, goiter, thyroiditis and DTC. Descriptive statistics and Kaplan-Meier analyses were performed. Results: Forty-three patients were included. Two patients (5%) were diagnosed with DTC at ages 12 and 17. Both DTCs were identified as minimally invasive follicular carcinoma at stages pT3NxMx and pT1NxMx respectively. A total of 84% were diagnosed with thyroid abnormalities at a median age of 12 years (range 9-18). Most common findings were benign, including nodular disease (74%), goiter (30%) and autoimmune thyroiditis (12%). Nodular growth was observed in 14 patients (33%) resulting in (hemi)thyroidectomy in 7 patients (16%). Conclusion: Thyroid ultrasound surveillance resulted in the detection of DTC in 2/43 PHTS patients before age 18. These findings support the recommendation to initiate thyroid ultrasound surveillance in children at least from age 12, preferably within an expertise centre.

Variation in the management of adhesive small bowel obstruction in the Netherlands: a prospective cross-sectional study.

BACKGROUND: ASBO is a frequent abdominal surgical emergency and a leading cause of morbidity and mortality in emergency surgery. The aim of this study is to provide insight into the current management of adhesive small bowel obstruction (ASBO) and associated outcomes. METHODS AND MATERIALS: A nationwide prospective cross-sectional cohort study was conducted. All patients with clinical signs of ASBO admitted to participating Dutch hospitals were included during a 6 months inclusion period between April 2019 and December 2020. Ninety-day clinical outcomes were described and compared for nonoperative management (NOM) and laparoscopic and open surgery. RESULTS: In 34 participating hospitals, 510 patients were included, of whom 382 (74.9%) had a definitive diagnosis of ASBO. Initial management consisted of emergency surgery in 71 (18.6%) patients and NOM in 311 (81.4%) patients, 119 (31.1%) of whom required delayed surgery after failure of NOM. Surgical interventions started laparoscopically in 51.1%, of which 36.1% were converted to laparotomy. Intentional laparoscopy resulted in shorter hospital stays compared with open surgery (median 8.0 vs. 11.0 days; P <0.001) and comparable hospital mortality (5.2 vs. 4.3%; P =1.000). Oral water-soluble contrast use was associated with a decreased length of stay ( P =0.0001). Hospital stay for surgical patients was shorter in patients who were operated on within 72 h of admission ( P <0.001). CONCLUSION: This nationwide cross-sectional study demonstrates shorter hospital stay in ASBO patients who received water-soluble contrast, were operated within 72 h of admission or were operated with minimally invasive techniques. Results may support the standardization of ASBO treatment.

COVID-19 vaccination and Atypical hemolytic uremic syndrome.

Introduction: COVID-19 vaccination has been associated with rare but severe complications characterized by thrombosis and thrombocytopenia. Methods and Results: Here we present three patients who developed de novo or relapse atypical hemolytic uremic syndrome (aHUS) in native kidneys, a median of 3 days (range 2-15) after mRNA-based (Pfizer/BioNTech's, BNT162b2) or adenoviral (AstraZeneca, ChAdOx1 nCoV-19) COVID-19 vaccination. All three patients presented with evident hematological signs of TMA and AKI, and other aHUS triggering or explanatory events were absent. After eculizumab treatment, kidney function fully recovered in 2/3 patients. In addition, we describe two patients with dubious aHUS relapse after COVID-19 vaccination. To assess the risks of vaccination, we retrospectively evaluated 29 aHUS patients (n=8 with native kidneys) without complement-inhibitory treatment, who received a total of 73 COVID-19 vaccinations. None developed aHUS relapse after vaccination. Conclusion: In conclusion, aHUS should be included in the differential diagnosis of patients with vaccine-induced thrombocytopenia, especially if co-occuring with mechanical hemolytic anemia (MAHA) and acute kidney injury (AKI). Still, the overall risk is limited and we clearly advise continuation of COVID-19 vaccination in patients with a previous episode of aHUS, yet conditional upon clear patient instruction on how to recognize symptoms of recurrence. At last, we suggest monitoring serum creatinine (sCr), proteinuria, MAHA parameters, and blood pressure days after vaccination.