Sclerosing epithelioid fibrosarcoma (SEF) is a rare and aggressive tumor for which no standardized treatment regimens are available. The occurrence of this tumor in children and adolescents has been rarely reported, especially in the head and neck region. Involvement of the neuraxis is reported only in a few patients. We report a case of a 13-year-old boy with SEF of the skull with intracranial extension. The tumor recurred after initial resection and rapidly spread to the brain parenchyma and the meninges with no response to surgery, chemotherapy and radiation therapy.
Objective
Initiation of continuous positive airway pressure (CPAP) in children with severe obstructive sleep apnea syndrome (OSAS) is challenging and the distinct features of the subset of children requiring CPAP are poorly defined. Asthma often coexists with OSAS in children. The goal of this study was to explore the influence of asthma in the need for CPAP therapy in children with severe OSAS.
Hypothesis
Asthmatic children with severe OSAS have higher probability of needing CPAP than children with severe OSAS without asthma.
Methods
Cross‐sectional study of clinical presentation, individual risk factors, and initial overnight polysomnogram (PSG) parameters in children with severe OSAS. Severe OSAS was defined as an obstructive apnea hypopnea index ≥10/h. The association between asthma and CPAP initiation was studied individually and adjusted by pertinent covariates.
Results
Four hundred eligible children (mean age 7 years, ±SD 5.3) with severe OSAS were enrolled and 133 individuals (33%) were identified to have asthma. The proportion of children needing CPAP was significantly higher in asthmatics with severe OSAS (29%) compared to those with OSAS alone (14%) (P < 0.01). Multivariate analysis demonstrated that the association between asthma and the need of CPAP in pediatric severe OSAS was independent of demographics, OSAS severity, obesity, and history of adenotonsillectomy (P < 0.01).
Conclusion
Asthmatic children with severe OSAS have higher probability of needing CPAP independent of relevant covariables. This study further substantiates the link between OSAS and asthma in children and suggests the diagnosis of asthma may influence the need of CPAP therapy for severe OSAS.
Up to 80% of asthmatic children may experience upper airway symptoms which are often perceived as coming from the lower airways. Currently, there are no validated questionnaires to assess upper airway contribution to pediatric asthma symptoms. The Sino-Nasal 5 (SN-5) questionnaire was previously validated for identifying radiographic confirmed sinus disease in children. In this study, we hypothesize that significant SN-5 scores (≥3.5) are associated with abnormal National Asthma Education and Prevention Program (NAEPP) based asthma impairment and control in asthmatic children. Retrospective data collected on children with asthma referred for pulmonary evaluation included age, gender, ethnicity, NAEPP asthma severity, asthma control (Test for Respiratory and Asthma Control in Kids (TRACK) < 5 years, Asthma Control Test (ACT) 5 years) and pulmonary function testing. Associations between SN-5 scores and asthma impairment and control were identified. Seventy-six children were evaluated; 38% were female with a mean age of 6.9 years. Significant SN-5 scores were associated with decreased control of daytime symptoms (odds ratio (OR): 0.16 (95% confidence interval (CI): 0.06–0.44)), night time awakenings (0.09 (0.03–0.29)), activity interference (0.2 (0.06–0.68)), NAEPP defined asthma control (0.32 (0.12–0.85)) and poor asthma control based on TRACK (p < 0.001) and ACT (p < 0.001). This suggests upper airways may play a larger role in perceived lower airway symptoms, and SN-5 may be beneficial in assessing the contribution of upper airway conditions on asthma control.
Introduction:Hyperthyroidism causing pseudotumor cerebri is very rare in children. case report: We are reporting a case of a 17-year-old girl with uncontrolled Graves' disease presenting with pseudotumor cerebri (Ptc). Patient initially did not respond to common management of Ptc with therapeutic lumbar puncture and acetazolamide, but improved after treating hyperthyroidism by radioablation therapy. conclusion: We emphasize that uncontrolled hyperthyroidism should be suspected as a secondary cause of pseudotumor cerebri in children and treated aggressively to avoid its dreaded complications.
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