High-Titer Anti-ZSCAN1 Antibodies in a Toddler Clinically Diagnosed with Apparent Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation Syndrome.

Severe obesity in young children prompts for a differential diagnosis that includes syndromic conditions. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a potentially fatal disorder characterized by rapid-onset obesity associated with hypoventilation, neural crest tumors, and endocrine and behavioral abnormalities. The etiology of ROHHAD syndrome remains to be established, but recent research has been focusing on autoimmunity. We report on a 2-year-old girl with rapid-onset obesity during the first year of life who progressed to hypoventilation and encephalitis in less than four months since the start of accelerated weight gain. The patient had a high titer of anti-ZSCAN1 antibodies (348; reference range < 40), and the increased values did not decline after acute phase treatment. Other encephalitis-related antibodies, such as the anti-NDMA antibody, were not detected. The rapid progression from obesity onset to central hypoventilation with encephalitis warns about the severe consequences of early-onset ROHHAD syndrome. These data indicate that serial measurements of anti-ZSCAN1 antibodies might be useful for the diagnosis and estimation of disease severity. Further research is needed to determine whether it can predict the clinical course of ROHHAD syndrome and whether there is any difference in antibody production between patients with and without tumors.

Post-extubation dysphagia in pediatric trauma patients: a single-center case-series study.

We aimed to investigate whether ventilator support time influences the occurrence of dysphagia in pediatric trauma patients. This case-series study was conducted in a single pediatric emergency and critical care center from April 2012 to March 2022. Trauma patients aged < 16 years who underwent tracheal intubation were divided into two groups based on the occurrence of dysphagia within 72 h after extubation, and their data were analyzed. Tracheal intubation was performed in 75 pediatric trauma patients, and 53 of them were included in the analysis. A total of 22 patients had post-extubation dysphagia and head trauma. The dysphagia group tended to have more severe head injuries (Abbreviated Injury Scale (AIS) 4 [4-5] vs. 4 [0-4]; p < 0.05), a longer ventilator support time (7 days [4-11] vs. 1 day [1-2.5]; p < 0.05), and a longer length of hospital stay (27 days [18.0-40.3] vs. 11 days [10.0-21.0]; p < 0.05). Severe head trauma and a long duration of tracheal intubation may be risk factors for dysphagia in pediatric trauma patients. Therefore, early recognition of these risk factors could assist in treatment planning for speech-language pathologist intervention and nutritional routes of administration.