Objectives To investigate the outcomes of pediatric tracheostomy as influenced by demographics and comorbidities. Study Design Retrospective national database review. Setting Fifty-two children’s hospitals across the United States. Subjects and Methods Hospitalization records from Pediatric Health Information System database dated 2010 to 2018 with patients younger than 18 years and procedure codes for tracheostomy were extracted. The primary outcome was total length of stay. The secondary outcomes were 30-day readmission, mortality, and posttracheostomy length of stay. Results A total of 14,155 children were included in the analysis. The median total length of stay was 77 days and increased from 59 to 103 days between 2010 and 2018 ( P < .001). The median posttracheostomy length of stay was 34 days and also increased from 27 to 49 days ( P < .001). On multivariate regression analyses, the total and posttracheostomy lengths of stay were significantly increased in children younger than 1 year, patients of black race, hospitals in the non-West regions, those discharged to home, and those with comorbidities. Socioeconomic indicators such as insurance type and estimated household income were associated with no difference or small effect sizes. Regions and comorbidities were associated with differences in 30-day readmission (overall 26%), while in-hospital mortality was primarily associated with age and comorbidities (overall 8.6%). Conclusion Pediatric tracheostomy requires substantial health care resources with length of stay escalating over recent years. Age, race, region, discharge destination, and comorbidities were associated with differences in length of stay.
Given that the SNOT-22 is easy to administer and inexpensive, this sinus disease-specific questionnaire seems to be an appropriate tool for routine use by respirologists when assessing patients with CF to help predict subclinical nasal polyps.
BackgroundHelios (encoded byIKZF2), a member of the Ikaros family of transcription factors, is a zinc finger protein involved in embryogenesis and immune function. Although predominantly recognised for its role in the development and function of T lymphocytes, particularly the CD4+regulatory T cells (Tregs), the expression and function of Helios extends beyond the immune system. During embryogenesis, Helios is expressed in a wide range of tissues, making genetic variants that disrupt the function of Helios strong candidates for causing widespread immune-related and developmental abnormalities in humans.MethodsWe performed detailed phenotypic, genomic and functional investigations on two unrelated individuals with a phenotype of immune dysregulation combined with syndromic features including craniofacial differences, sensorineural hearing loss and congenital abnormalities.ResultsGenome sequencing revealedde novoheterozygous variants that alter the critical DNA-binding zinc fingers (ZFs) of Helios. Proband 1 had a tandem duplication of ZFs 2 and 3 in the DNA-binding domain of Helios (p.Gly136_Ser191dup) and Proband 2 had a missense variant impacting one of the key residues for specific base recognition and DNA interaction in ZF2 of Helios (p.Gly153Arg). Functional studies confirmed that both these variant proteins are expressed and that they interfere with the ability of the wild-type Helios protein to perform its canonical function—repressingIL2transcription activity—in a dominant negative manner.ConclusionThis study is the first to describe dominant negativeIKZF2variants. These variants cause a novel genetic syndrome characterised by immunodysregulation, craniofacial anomalies, hearing impairment, athelia and developmental delay.
Calcific tendonitis of the longus colli (CTLC) muscle is an underrecognized cause of spontaneous acute or subacute neck pain, dysphagia, or odynophagia. Imaging may reveal a retropharyngeal fluid collection leading to the presumed diagnosis of retropharyngeal abscess. Recognition of this uncommon presentation is important to prevent unnecessary surgical incision and drainage. A 44-year-old otherwise healthy male presented with a 2-week history of progressive neck pain, stiffness, and odynophagia. A noncontrast CT scan of the cervical spine revealed a retropharyngeal fluid collection with a small area of calcification anterior to C2. There was a presumed diagnosis of retropharyngeal abscess. The patient was afebrile with normal vital signs. Flexible nasolaryngoscopy was unremarkable. C-reactive protein was elevated but all other bloodwork was normal with no evidence of an infective process. A CT scan was repeated with IV contrast showing no enhancement around the fluid collection. A diagnosis of CTLC was made. The patient was successfully managed with a short course of intravenous steroids and oral NSAIDs with complete resolution of symptoms. Clinically CTLC can mimic more serious disease processes. Identifying pathognomonic imaging findings often confirms the diagnosis. Awareness of this condition by the otolaryngologist will ensure proper patient management and avoidance of unnecessary procedures.
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