Systematic Literature Review of the Presentation and Management of Pediatric Relapsing Polychondritis.

INTRODUCTION: Pediatric Relapsing Polychondritis (RP) is a rare autoimmune disorder that causes inflammation and damage to cartilage in children. Common symptoms include pain, swelling and deformities in the ears, nose, trachea, joints, and eyes. The lack of research on the pediatric population necessitates further evaluation of the literature on pediatric RP to summarize existing patterns in presentation, management, and treatment. METHODS: A systematic review was conducted on PubMed and Embase from 1947 to April 2023 on RP in patients under 21 years old abiding by the 2020 PRISMA checklist. Only patient presentations meeting McAdam criteria for RP and including information on management were included. RESULTS: From the 304 initial studies, 54 studies were included for final analysis with a total of 68 patients, who were predominantly female (65%). With a median diagnostic delay of 1 year, the mean age of onset was 12 years old. The most common symptoms on presentation included bilateral auricular chondritis (69%), nasal cartilage inflammation (62%), and respiratory tract chondritis (63%). The most commonly reported information in the literature for the initial workup usually included CT/MRI (72%), bronchoscopy (57%), biopsy (51%), and labs (88%), which most commonly displayed elevated ESR (59%). The most common medications were corticosteroids (91%) and methotrexate (35%) and the most common procedural treatment was tracheostomy (38%). The most efficacious treatment options were monoclonal antibodies (87%, n = 15) and corticosteroids (66%, n = 62) used in 22% and 91% of patients, respectively. The most commonly used monoclonal antibody therapy was infliximab (13%, n = 9). CONCLUSION: The most common presentation for pediatric RP includes chondritis of the ear, nose, and respiratory tract. The most effective treatment options include corticosteroids and monoclonal antibody therapy, such as infliximab. Our findings highlight increasing remission achieved with anti-rheumatic drugs and monoclonal antibody treatment, especially alongside corticosteroids.

Management of Vertebral Defects, Anal Atresia, Cardiac Defects, Tracheoesophageal Fistula or Atresia, Renal Anomalies, and Limb Abnormalities (VACTERL) in a Child With Complex Medical Needs in the Foster Care System: A Pediatric Case Study.

Vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula or atresia, renal anomalies, and limb abnormalities (VACTERL) association is a complex congenital condition characterized by the presence of malformations that affect various organ systems. Most children born with VACTERL association require surgery shortly after birth, often undergoing multiple procedures during infancy, which can lead to a wide range of physical challenges. The unique combination of malformations in these children in addition to having complex care needs that need to be met can result in physical and social difficulties in their daily lives, affecting both their own and their caregivers' quality of life. In some cases, children with complex medical needs are placed in foster care. When children with complex health needs enter the foster care system, there is a risk of overwhelming the caretaker, leading to their needs continuing to be unmet. Pediatricians have a role not only in helping support families but also in knowing what resources are available to meet these needs, which can be dependent on what their communities offer. Pediatricians require current training to navigate their state's foster care system. This training allows pediatricians to effectively collaborate with foster families while also assisting and coordinating complex care to support these families. We present a case of a child with complex health needs placed in the foster care system, facing multiple healthcare challenges, with care delayed due to difficulty attending appointments. Highlighted is the importance of delivering supportive, personalized, and multidisciplinary care to families with children who have complex health needs, including when caretakers are within the foster care system.

Higher area deprivation index is associated with poorer local control and overall survival in non-metastatic nasopharyngeal carcinoma.

BACKGROUND: Neighborhood socioeconomic deprivation impacts outcomes in various cancers. We examined this association in nasopharyngeal carcinoma (NPC) patients using the area deprivation index (ADI). METHODS: We conducted a single-institution retrospective cohort study on NPC patients treated with definitive radiotherapy from 1980 to 2023. ADI was used as the primary exposure measure. Higher ADI indicates higher levels of socioeconomic deprivation. RESULTS: Of 561 patients, those with higher ADI (6-10 vs. 1-5) presented more commonly with AJCC stage III/IV compared to I/II (87% vs. 76%, p = 0.03). Increasing ADI decile score correlated with poorer overall survival (HR 1.14, 95% CI 1.01-1.28, p = 0.04). Local control was worse in patients from the most deprived quartile in the cohort ADI 5-10 (HR 2.11, 95% CI 1.01-4.41, p = 0.05). CONCLUSIONS: NPC patients from more disadvantaged neighborhoods undergoing radiotherapy had worse local control and survival outcomes. Interventions to address structural determinants of health and neighborhood disparities may improve these outcomes.

Auditory discomfort in visually sensitive individuals.

Introduction: Sensory discomfort occurs in clinical and non-clinical populations. While some of the parameters that evoke visual discomfort have been identified, the parameters of sounds that evoke auditory discomfort are largely unknown. Methods: We presented various sounds and asked participants to rate the discomfort they experienced. In Experiments 1 and 2 tones were presented at frequencies between 0.25-8 kHz and modulated sinusoidally in amplitude at frequencies between 0-32 Hz. In Experiment 3 tones were swept in frequency from 500 Hz-2 kHz at sweep rates of 5-50 per second. In Experiment 4, sweeps varied in frequency range and central frequency. Results: Discomfort increased with frequency. The effects of the amplitude modulation and sweep rate on discomfort were relatively small and were experienced mainly at low modulation frequencies and high sweep rates. Individuals who experienced visuo-perceptual distortions in the Pattern Glare (PG) Test reported greater auditory discomfort. Discussion: This suggests that sensory sensitivity in one modality may occur in another.