Search Results
National Center for Children’s Health, Beijing, China
Search for other papers by Yi Wang in
Google Scholar
PubMed
Search for other papers by Yingying Xu in
Google Scholar
PubMed
Search for other papers by Rongrong Xie in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Bingyan Cao in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Yuan Ding in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Jiayun Guo in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Xiaoqiao Li in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Xiaolin Ni in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Zheng Yuan in
Google Scholar
PubMed
Search for other papers by Linqi Chen in
Google Scholar
PubMed
Search for other papers by Liyang Liang in
Google Scholar
PubMed
National Center for Children’s Health, Beijing, China
Search for other papers by Chunxiu Gong in
Google Scholar
PubMed
Objective
Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is rare, and manifestations of autonomic dysregulation are diverse and may be overlooked. We aimed to evaluate the incidence of these manifestations.
Methods
Patients with ROHHAD syndrome reported before and after 2019 were divided into groups 1 and 2. Patients who were diagnosed at three regional hospitals in China were included in group 3. We collected the age of each specific term of the ROHHAD (neurogenic tumor, NET) acronym and the detailed manifestations of each term, and compared them among the three groups.
Results
A total of 16 patients were diagnosed within the 2-year period. Two had neurogenic tumors and cognitive and behavioral abnormalities before developing rapid obesity. At least 93.8% of the patients had ≥ 4 symptoms of autonomic dysregulation. When comparing autonomic dysregulation among groups 1–3, the rates of cardiovascular manifestations were NA vs 12.8% vs 81.2%; gastrointestinal disturbances were 11.4% vs 8.5% vs 62.5%; strabismus was 25.7% vs 12.8% vs 62.5%; sleep disturbance was NA vs 6.4% vs 50.0%; and abnormal pain threshold was NA vs 10.6% vs 25.0% (all P < 0.05). The rates of cognitive and behavioral abnormalities were NA vs 29.8% and 87.5% (P < 0.01).
Conclusion
Rapid-onset obesity is not always the first sign of ROHHAD syndrome. Higher rates of autonomic dysregulation and cognitive and behavioral abnormalities with multiple manifestations of autonomic dysregulation coexisted in our cohort, indicating that evaluations of autonomic function and the limbic system should be strengthened when assessing this condition.
Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
Search for other papers by Jesper Krogh in
Google Scholar
PubMed
Department of Clinical Biochemistry, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark
Search for other papers by Peter Plomgaard in
Google Scholar
PubMed
Department of Clinical Biochemistry, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark
Search for other papers by Ruth Frikke-Schmidt in
Google Scholar
PubMed
Search for other papers by Sten Velschow in
Google Scholar
PubMed
Department of Pediatrics, Copenhagen University Hospital - Herlev & Gentofte, Copenhagen, Denmark
Search for other papers by Jesper Johannesen in
Google Scholar
PubMed
Search for other papers by Linda Maria Hilsted in
Google Scholar
PubMed
Search for other papers by Malene Schrøder in
Google Scholar
PubMed
Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
Search for other papers by Ulla Feldt-Rasmussen in
Google Scholar
PubMed
Repeated blood sampling is required in certain clinical and research settings, which is currently performed by drawing blood from venous catheters requiring manual handling of each sample at the time of collection. A novel body-worn device for repeated serial samples, Fluispotter®, with automated extraction, collection, and storage of up to 20 venous dried blood spot samples over the course of 20 h may overcome problems with current methods for serial sampling. The purpose of this study was to assess the performance and safety of Fluispotter for the first time in healthy subjects. Fluispotter consists of a cartridge with tubing, a reservoir for flushing solution, pumps and filterpaper, and a multi-lumen catheter placed in the brachial vein. We recruited healthy subjects for testing in an in-hospital setting. Fluispotter was attached by an anesthesiologist to 22 healthy subjects of which 9/22 (40.9%) participants had all 20 samples taken, which was lower than the goal of complete sampling in 80% of the subjects (P = 0.02). The main reason for sample failure was clogging of blood flow which was observed in 11/22 (50%) of the participants. No serious adverse events occurred, and the participants rated the pain from the insertion and the removal of catheter as very low. A cortisol profile showed nadir values at midnight and highest values at 05:00 h. Although full sampling was not successful in all participants, the Fluispotter technology proved safe and highly acceptable to the participants producing the expected cortisol profile without the requirement of staff during sample collection.
Department of Medicine, Division of Endocrinology and Centre for Endocrine Tumors, Leiden University Medical Centre, Leiden, The Netherlands
Department of Neurosurgery, University Neurosurgical Centre Holland (UNCH), Leiden University Medical Centre, Haaglanden Medical Centre and Haga Teaching Hospitals, Leiden and The Hague, The Netherlands
Search for other papers by Amir H Zamanipoor Najafabadi in
Google Scholar
PubMed
Search for other papers by Merel van der Meulen in
Google Scholar
PubMed
Search for other papers by Ana Luisa Priego Zurita in
Google Scholar
PubMed
Search for other papers by S Faisal Ahmed in
Google Scholar
PubMed
Search for other papers by Wouter R van Furth in
Google Scholar
PubMed
Search for other papers by Evangelia Charmandari in
Google Scholar
PubMed
Search for other papers by Olaf Hiort in
Google Scholar
PubMed
Search for other papers by Alberto M Pereira in
Google Scholar
PubMed
Search for other papers by Mehul Dattani in
Google Scholar
PubMed
Search for other papers by Diana Vitali in
Google Scholar
PubMed
Search for other papers by Johan P de Graaf in
Google Scholar
PubMed
Search for other papers by Nienke R Biermasz in
Google Scholar
PubMed
Objective
The European Reference Network on Rare Endocrine Conditions (Endo-ERN) aims to organize high-quality healthcare throughout Europe, including care for pituitary adenoma patients. As surgery is the mainstay of treatment, we aimed to describe the current surgical practice and published surgical outcomes of pituitary adenoma within Endo-ERN.
Design and Methods
Systematic review and meta-analysis of studies reporting surgical outcomes of pituitary adenoma patients within Endo-ERN MTG6 pituitary reference centers between 2010 and 2019. A survey was completed by reference centers on their current surgical practice.
Results
A total of 18 out of 43 (42%) reference centers located in 7 of the 20 (35%) MTG6-represented countries published 48 articles. Remission rates were 50% (95% CI: 42–59) for patients with acromegaly, 68% (95% CI: 60–75) for Cushing’s disease, and 53% (95% CI: 39–66%) for prolactinoma. Gross total resection was achieved in 49% (95% CI: 37–61%) of patients and visual improvement in 78% (95% CI: 68–87). Mortality, hemorrhage, and carotid injury occurred in less than 1% of patients. New-onset hypopituitarism occurred in 16% (95% CI: 11–23), transient diabetes insipidus in 12% (95% CI: 6–21), permanent diabetes insipidus in 4% (95% CI: 3–6), syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in 9% (95% CI: 5–14), severe epistaxis in 2% (95% CI: 0–4), and cerebrospinal fluid leak in 4% (95% CI: 2–6). Thirty-five (81%) centers completed the survey: 54% were operated endoscopically and 57% were together with an ENT surgeon.
Conclusion
The results of this study could be used as a first benchmark for the outcomes of pituitary adenoma surgery within Endo-ERN. However, the heterogeneity between studies in the reporting of outcomes hampers comparability and warrants outcome collection through registries.