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1.
Proc Natl Acad Sci U S A ; 117(25): 14405-14411, 2020 06 23.
Article in English | MEDLINE | ID: mdl-32518111

ABSTRACT

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. We queried two European-American cohorts and one Turkish cohort (total n = 231) of individuals with PFAPA for common variants previously associated with two other oropharyngeal ulcerative disorders, Behçet's disease and recurrent aphthous stomatitis. In a metaanalysis, we found that a variant upstream of IL12A (rs17753641) is strongly associated with PFAPA (OR 2.13, P = 6 × 10-9). We demonstrated that monocytes from individuals who are heterozygous or homozygous for this risk allele produce significantly higher levels of IL-12p70 upon IFN-γ and LPS stimulation than those from individuals without the risk allele. We also found that variants near STAT4, IL10, and CCR1-CCR3 were significant susceptibility loci for PFAPA, suggesting that the pathogenesis of PFAPA involves abnormal antigen-presenting cell function and T cell activity and polarization, thereby implicating both innate and adaptive immune responses at the oropharyngeal mucosa. Our results illustrate genetic similarities among recurrent aphthous stomatitis, PFAPA, and Behçet's disease, placing these disorders on a common spectrum, with recurrent aphthous stomatitis on the mild end, Behçet's disease on the severe end, and PFAPA intermediate. We propose naming these disorders Behçet's spectrum disorders to highlight their relationship. HLA alleles may be factors that influence phenotypes along this spectrum as we found new class I and II HLA associations for PFAPA distinct from Behçet's disease and recurrent aphthous stomatitis.


Subject(s)
Behcet Syndrome/genetics , Fever/genetics , Genetic Predisposition to Disease , Lymphadenitis/genetics , Pharyngitis/genetics , Stomatitis, Aphthous/genetics , Alleles , Behcet Syndrome/immunology , Child , Cohort Studies , Fever/immunology , Genes, MHC Class I/genetics , Genes, MHC Class I/immunology , Genes, MHC Class II/genetics , Genes, MHC Class II/immunology , Genetic Loci/immunology , Humans , Lymphadenitis/immunology , Pharyngitis/immunology , Polymorphism, Single Nucleotide , Risk Factors , Stomatitis, Aphthous/immunology , Syndrome
2.
Pediatr Radiol ; 52(9): 1619-1626, 2022 08.
Article in English | MEDLINE | ID: mdl-34841448

ABSTRACT

Vocal cord paralysis is a common cause of respiratory and feeding problems in the pediatric population. While the causes of vocal cord paralysis are multiple, iatrogenic injury of the recurrent laryngeal nerve after cardiovascular surgery is the most frequent cause. Vocal cord paralysis increases the risk of swallowing dysfunction, tracheal aspiration and pneumonia. It also increases the need for nasoenteric feeds and gastrostomy tube placement. Flexible nasopharyngolaryngoscopy is considered the gold standard for diagnosing vocal cord paralysis, but it has significant drawbacks: it is uncomfortable, it can trigger a cardiovascular event in children with unstable cardiovascular status, it can be challenging to perform, and it can be difficult to interpret. Laryngeal US has become a popular imaging modality to evaluate the function of the vocal cords. Laryngeal US is well-tolerated, easy to perform, simple to interpret and has a lower physiological impact compared to flexible nasopharyngolaryngoscopy. Laryngeal US is an accurate and low-cost diagnostic test for vocal cord paralysis. In this review, we describe the anatomy of the larynx and recurrent laryngeal nerve; the causes, symptoms and pathophysiology of vocal cord paralysis; laryngeal US technique; diagnostic criteria for vocal cord paralysis; and a reporting system.


Subject(s)
Larynx , Vocal Cord Paralysis , Child , Humans , Infant , Laryngoscopy/adverse effects , Larynx/diagnostic imaging , Recurrent Laryngeal Nerve , Vocal Cord Paralysis/diagnostic imaging , Vocal Cord Paralysis/epidemiology , Vocal Cords/diagnostic imaging
3.
J Craniofac Surg ; 26(8): e788-91, 2015 Nov.
Article in English | MEDLINE | ID: mdl-26595008

ABSTRACT

BACKGROUND: Long-segment congenital tracheal stenosis (CTS) is characterized by segmental tracheal stenosis, complete tracheal rings, and absent posterior pars membranosa for >50% of tracheal length. Slide tracheoplasty on cardiopulmonary bypass (CPB) has traditionally been the procedure of choice for airway reconstruction. Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. The authors and others, have demonstrated the efficacy of mandibular distraction osteogenesis (MDO) to avoid tracheostomy in severe cases of PRS. METHODS: The authors present a unique case of the multidisciplinary management of long-segment CTS and concomitant PRS via total airway reconstruction off CPB, involving our otolaryngology, cardiothoracic, and plastic surgery teams. RESULTS: This 36-week baby girl, prenatally diagnosed with PRS and polyhydramnios concerning for airway obstruction, was delivered via planned ex utero intrapartum treatment (EXIT). Tracheostomy was aborted because of long-segment CTS. A 2.5-French endotracheal tube (ETT) was temporarily sutured in before transfer to our facility for definitive airway management.Bilateral MDO was performed without complication at 2 weeks old (distraction to 20mm by postoperative day 25). At 6 weeks old, delayed slide tracheoplasty avoiding cardiopulmonary bypass was followed by an uneventful recovery. Most recent follow-up demonstrates airway patency without signs of obstruction. CONCLUSIONS: This patient's case is the first reporting combined MDO and slide tracheoplasty to relieve multilevel neonatal airway obstruction. Unique and challenging, it demonstrates the importance of multidisciplinary management of complex neonatal airway obstruction.


Subject(s)
Airway Obstruction/surgery , Constriction, Pathologic/surgery , Mandible/surgery , Osteogenesis, Distraction/methods , Pierre Robin Syndrome/surgery , Plastic Surgery Procedures/methods , Trachea/abnormalities , Airway Management/methods , Anastomosis, Surgical/methods , Bronchoscopy/methods , Female , Follow-Up Studies , Humans , Infant, Newborn , Intubation, Intratracheal/methods , Patient Care Team , Piezosurgery/methods , Trachea/surgery , Tracheostomy/methods
4.
Pediatr Rev ; 41(Suppl 1): S50-S53, 2020 Oct.
Article in English | MEDLINE | ID: mdl-33004582

Subject(s)
Hypertrophy , Adolescent , Humans
5.
Otolaryngol Head Neck Surg ; 170(4): 1167-1172, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38193359

ABSTRACT

OBJECTIVE: Paradoxical vocal fold motion (PVFM) is characterized by inappropriate adduction of vocal folds during inspiration causing dyspnea. While anxiety is suspected to be a predisposing factor, incidence has been understudied. STUDY DESIGNS: Retrospective review. SETTING: Multidisciplinary PVFM hospital clinic. METHODS: We used patient-reported outcome measures to examine anxiety and depression in consecutive patients aged 10 to 17 years using Pediatric SFv1.1 Anxiety 8b and Level 2-Depression inventories (parents completed proxy forms). T-scores were classified as normal (none to slight <55) or elevated (mild 55-59.9, moderate 60-69.9, severe >70). RESULTS: Twenty-three pediatric patients and 20 parents completed surveys. Mean age was 13.74 years. For anxiety, 69.6% of patients and 40% of parents identified elevated levels. For depression, 30.4% of patients and 15% of parents identified elevated levels. Therapy need for the sample was 65.2% (34.8% active in services and 30.4% referred). Child anxiety scores were significantly higher in the therapy need group, U = 17, P = .004. CONCLUSION: This study of adolescents with PVFM confirmed elevated anxiety and depression scores in 2/3 of the participants. Anxiety likely precedes diagnosis and is a predisposing factor. Referral for individualized intervention targeting anxiety and depression is indicated.


Subject(s)
Mental Health , Vocal Cord Dysfunction , Adolescent , Humans , Child , Vocal Cords , Dyspnea , Patient Reported Outcome Measures
6.
Ann Allergy Asthma Immunol ; 111(5): 337-41, 2013 Nov.
Article in English | MEDLINE | ID: mdl-24125137

ABSTRACT

BACKGROUND: Patients with chronic rhinosinusitis (CRS) and nasal polyps (NPs) may be subdivided into aspirin-sensitive (AS) and aspirin-tolerant (AT) populations. These cohorts are not well characterized. OBJECTIVE: To examine phenotypic characteristics and determine the extent of medical/surgical interventions in patients with CRS+NP and to compare the AS with the AT subset in the CRS+NP sample. METHODS: Retrospective chart review was performed at a tertiary academic respiratory hospital. Data included patient demographics, asthma severity, peripheral eosinophilia, Lund-Mackay computed tomographic score, symptomatic dysosmia, and therapeutic interventions. RESULTS: Of the 182 patients included, 81 had aspirin sensitivity (45%) and 101 had aspirin tolerance (55%). Asthma was present in 94% of patients with CRS+NP (100% in AS subgroup vs 89% in AT subgroup, P = .001). Eighty-eight percent of the CRS+NP sample had moderate to severe persistent asthma. In the AS and AT subgroups, asthma severity was similar (P > .6). The CRS+NP sample showed a mean computed tomographic score of 14.0 (44% with eosinophilia and 46% with dysosmia). More severe sinus disease was noted in the AS group (Lund-Mackay computed tomographic scores, P = .002; olfactory symptoms, P = .001). Serum eosinophil levels were not statistically different between groups (51% in AS group, 39% in AT group, P > .1). CONCLUSION: This study is one of the broadest reviews of patients with CRS+NP, with unique findings in the high prevalence of asthma in AS and AT patients, greater olfactory dysfunction in AS patients, and a minority of patients with CRS+NP and circulating eosinophils. Most AS patients do not have increased circulating eosinophils, as is often believed. These results shed further light on the association between asthma and upper respiratory tract disease in those with nasal polyposis.


Subject(s)
Aspirin/therapeutic use , Asthma/drug therapy , Nasal Polyps/drug therapy , Sinusitis/drug therapy , Adolescent , Adult , Aged , Aged, 80 and over , Asthma/diagnosis , Asthma/surgery , Child , Drug Resistance , Eosinophils/immunology , Female , Humans , Male , Middle Aged , Nasal Polyps/diagnosis , Nasal Polyps/surgery , Olfaction Disorders , Paranasal Sinuses/pathology , Retrospective Studies , Severity of Illness Index , Sinusitis/diagnosis , Young Adult
7.
Laryngoscope ; 133(4): 970-976, 2023 04.
Article in English | MEDLINE | ID: mdl-35730686

ABSTRACT

OBJECTIVE: To explore patient-reported outcome measures of pediatric paradoxical vocal fold motion through a multi-institutional study of geographically diverse United States medical facilities to assess long-term management and outcomes. METHODS: Eligible participants >8 years of age diagnosed with PVFM over a 10-year period from 7 tertiary pediatric hospitals were invited to complete a survey addressing study objectives. RESULTS: 65 participants completed the survey, of whom 80% were female, 75% reported a 3.5 grade point average or better, and 75% identified as competitive athletes or extremely athletic individuals. Participants rated their perceived efficacy of 13 specific treatments. Only five treatments were considered effective by a majority of the participants who tried them. The treatments that participants tried most often were breathing exercises (89.2%), bronchodilator treatments (45%), and allergy medications (35.4%). 78.8% of participants reported receiving more than one treatment and 25% reported receiving a combination of bronchodilators, anticholinergics, and steroids. At the time of PVFM diagnosis, 38% of participants had no idea when their symptoms would completely resolve. 23.3% of participants did not experience symptom resolution until greater than 1 year after diagnosis. CONCLUSIONS: Traditional management tools such as breathing exercises and biofeedback treatments may not provide the long-term benefit that providers anticipate. In addition to these commonly used management strategies, highly efficacious techniques such as counseling and lifestyle management should be incorporated into the long-term management of patients whose symptoms are refractory to traditional care. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:970-976, 2023.


Subject(s)
Laryngoscopes , Vocal Cord Dysfunction , Humans , Female , Child , Male , Vocal Cord Dysfunction/diagnosis , Vocal Cord Dysfunction/therapy , Biofeedback, Psychology , Breathing Exercises , Patient Reported Outcome Measures , Vocal Cords
8.
Clin Neuropathol ; 31(1): 24-30, 2012.
Article in English | MEDLINE | ID: mdl-22192701

ABSTRACT

Ectopic pituitary adenomas usually occur within sphenoid sinus or nasopharynx, and seldom within the clivus. There is only a single reported example of ectopic adenoma with clinical apoplexy, albeit not from clivus. We report a 78-year-old male with known prostate carcinoma admitted with acute onset of blurred vision, suggestive of apoplexy. Work-up revealed unilateral cranial nerve VI palsy and neuroimaging showed a mass confined to the clivus; sellar region was normal. Preoperative considerations included chordoma, chondrosarcoma, or metastatic prostate carcinoma to bone. Resection was via endoscopic transsphenoidal approach to the clivus. An ectopic null cell pituitary adenoma with bland infarction was identified as the cause of the patient's clinical apoplexy. No antecedent precipitating factors for apoplexy were present; specifically the patient had not received leuprolide preoperatively, a known precipitant of pituitary apoplexy in prostate cancer patients who receive drug. We review the literature on ectopic clival pituitary adenomas, apoplexy in ectopic adenomas, and the link between apoplexy and leuprolide usage.


Subject(s)
Adenoma/pathology , Neoplasms, Second Primary/pathology , Pituitary Gland/pathology , Skull Neoplasms/pathology , Stroke/etiology , Adenoma/complications , Adenoma/surgery , Aged , Cranial Fossa, Posterior/pathology , Humans , Magnetic Resonance Imaging , Male , Pituitary Gland/surgery , Prostatic Neoplasms/pathology , Skull Neoplasms/complications , Skull Neoplasms/surgery , Stroke/pathology , Tomography, X-Ray Computed
9.
Ann Allergy Asthma Immunol ; 107(4): 353-9, 2011 Oct.
Article in English | MEDLINE | ID: mdl-21962096

ABSTRACT

BACKGROUND: Matrix metalloproteinases (MMPs) are key enzymes responsible for extracellular matrix degradation contributing to the progressive histological changes seen in lower airway disease, including asthma. MMP-9 and TIMP-1 have also shown some role in the pathogenesis of chronic rhinosinusitis (CRS) and nasal polyposis (NP). OBJECTIVE: We aim to determine variability in expression of MMP-9 and its inhibitor, tissue inhibitor of metalloproteinase-1 (TIMP-1), in sinus tissue from distinct patient populations presenting with nasal polyposis. METHODS: The expression of MMP-9 and TIMP-1 was investigated in nasal polyp tissue from 6 aspirin-sensitive (AS) and 6 aspirin-tolerant (AT) patients undergoing endoscopic sinus surgery for chronic rhinosinusitis with nasal polyposis (CRSwNP). Sinus mucosa from 6 patients with chronic rhinosinusitis without nasal polyposis (CRSsNP) was used as control. The MMP-9 and TIMP-1 expression was measured using immunofluorescence technique and graded using manual and computerized methods. RESULTS: Expression of TIMP-1 was significantly reduced in the AS group when compared with both the AT and CRSsNP (control) groups (P < .001). The MMP-9/TIMP-1 ratio was significantly increased in the AS group when compared with other patient groups (P < .001). The MMP- 9 expression was similar between study and control groups. CONCLUSION: These results support the importance of MMP-9 and TIMP-1 expression in nasal polyp formation. The decreased expression of TIMP-1 in AS patients may promote the effects of MMP-9 expression and thus contribute to tissue remodeling and inflammatory changes. This finding may lead to further understanding of disease severity and resistance to treatment in this group of patients, as well as the pathogenesis of nasal polyps.


Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Aspirin/administration & dosage , Matrix Metalloproteinase 9/biosynthesis , Nasal Polyps/metabolism , Tissue Inhibitor of Metalloproteinase-1/biosynthesis , Adult , Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Aspirin/adverse effects , Biopsy , Female , Histocytochemistry , Humans , Male , Microscopy, Fluorescence , Middle Aged , Nasal Mucosa/drug effects , Nasal Mucosa/enzymology , Nasal Mucosa/surgery , Nasal Polyps/enzymology , Nasal Polyps/surgery , Retrospective Studies , Young Adult
10.
Ann Otol Rhinol Laryngol ; 129(12): 1195-1209, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32527140

ABSTRACT

OBJECTIVES: To explore long-term patient reported outcome (PRO) measures of pediatric paradoxical vocal cord motion (PVCM) including ease of diagnosis, management, symptom duration and effect on quality of life. METHODS: All children >8 years of age diagnosed with PVCM at a tertiary pediatric hospital between 2006 and 2017 were invited to complete a survey addressing study objectives. RESULTS: 21/47 eligible participants could be contacted and 18/21 (86%) participated. 78% were female with a mean age at diagnosis of 11.6 and 15.0 years at survey completion. Common PVCM symptoms reported were dyspnea (89%), globus sensation (56%), and stridor (50%). The median time to diagnosis was 3 months (IQR 2-5 months). Nearly all reported being misdiagnosed with another condition, usually asthma, until being correctly diagnosed usually by an otolaryngologist. Participants reported undergoing 3.7 diagnostic studies (range 0-8); pulmonary function testing was most common. Of numerous treatments acknowledged, breathing exercises were common (89%) but only reported helpful by 56%. Use of biofeedback was recalled in 1/3 of subjects but reported helpful in only 14% of them. Anti-reflux, allergy, anticholinergics, inhalers and steroids were each used in >50%, but rarely reported effective. PVCM was reportedly a significant stressor when initially diagnosed but despite 2/3 of participants still reporting ongoing PVCM symptoms, the perceived stress significantly decreased over time (Z = 3.26, P = 0.001). CONCLUSIONS: This first PVCM PRO study endorses that diagnosis is often delayed and prescribed treatments often viewed as ineffective. While biofeedback and breathing exercises may be critical for short-term control of PVCM episodes, lifestyle changes and stress reduction are likely necessary for long-term management. Increased awareness and improvements in management are needed for this condition.


Subject(s)
Biofeedback, Psychology , Breathing Exercises , Dyspnea/physiopathology , Globus Sensation/physiopathology , Respiratory Sounds/physiopathology , Stress, Psychological/psychology , Vocal Cord Dysfunction/therapy , Adolescent , Asthma/diagnosis , Child , Diagnostic Errors , Female , Gastroesophageal Reflux/diagnosis , Humans , Male , Patient Reported Outcome Measures , Relaxation Therapy , Respiratory Hypersensitivity/diagnosis , Vocal Cord Dysfunction/diagnosis , Vocal Cord Dysfunction/physiopathology , Vocal Cord Dysfunction/psychology
11.
BMJ Case Rep ; 12(6)2019 Jun 22.
Article in English | MEDLINE | ID: mdl-31229983

ABSTRACT

We report a significant complication after tracheocutaneous fistula (TCF) excision with closure by secondary intention in a 4-year-old boy who had been tracheostomy dependent since infancy. He had a persistent 3 mm TCF one year after decannulation. On postoperative day 2 the patient developed profound subcutaneous emphysema and pneumomediastinum. He was extubated after 2 days and discharged from the hospital on postoperative day 7. At follow up he had complete resolution of subcutaneous emphysema and complete closure of the TCF. The main methods of TCF closure and management of subcutaneous emphysema are discussed along with the lessons learned from this case.


Subject(s)
Cutaneous Fistula/complications , Subcutaneous Emphysema/etiology , Trachea/surgery , Child, Preschool , Cutaneous Fistula/etiology , Cutaneous Fistula/surgery , Device Removal/adverse effects , Humans , Intubation, Intratracheal/methods , Male , Mediastinal Emphysema/etiology , Postoperative Complications , Subcutaneous Emphysema/diagnostic imaging , Subcutaneous Emphysema/therapy , Tracheal Diseases/complications , Treatment Outcome
12.
Ann Otol Rhinol Laryngol ; 128(5): 401-405, 2019 May.
Article in English | MEDLINE | ID: mdl-30739476

ABSTRACT

OBJECTIVE: Multidisciplinary vascular anomaly clinics (VACs) offer important value to pediatric patients with complex vascular anomalies whose care overlaps specialties. These clinics are labor intensive and costly to operate since providers see fewer patients compared to their individual specialty clinic. Our North American tertiary care institution's VAC specialists include a pediatric otolaryngologist, pediatric surgeon, pediatric plastic surgeon, pediatric dermatologist, and interventional radiologist. To assess financial feasibility, we conducted a cost analysis of our VACs comprised of 2 half-day multidisciplinary physician attended clinics (5 specialists at our main campus and 2 specialists at a satellite clinic) and a half-day nurse practitioner clinic. METHOD: Assessment of net revenue based on net collections for clinic, professional, operative, hospital setting, and facility charges generated during 12 consecutive monthly VACs beginning July 1, 2015. Expense calculations included provider and staff salaries, benefits, supply costs, and clinic leasing costs. RESULTS: There were 469 clinic visits, of which 202 were new patient evaluations. Sixty-eight patients underwent 93 procedures under general anesthesia, including procedures performed by our interventional radiologist, most commonly sclerotherapy or embolization (n = 37), surgical interventions including endoscopy (n = 36), or laser procedures (n = 20). Three patients were admitted. Fifty-seven patients received a new diagnosis different from that for which they were referred. Gross revenue was $1 810 525, and net revenue was 42.5%, or $783 152. Expenses totaled $453 415 for a net positive revenue of $329 737. CONCLUSION: When including direct downstream revenue, particularly from operative procedures, our VAC program operates on a net positive margin, making the program financially feasible.


Subject(s)
Ambulatory Care Facilities/economics , Vascular Malformations/therapy , Anesthesia, General/statistics & numerical data , Costs and Cost Analysis , Embolization, Therapeutic/economics , Embolization, Therapeutic/statistics & numerical data , Endoscopy/economics , Endoscopy/statistics & numerical data , Feasibility Studies , Health Personnel/economics , Humans , Laser Therapy/economics , Laser Therapy/statistics & numerical data , North America , Patient Admission/economics , Patient Admission/statistics & numerical data , Pediatrics , Reimbursement Mechanisms , Retrospective Studies , Salaries and Fringe Benefits/economics , Sclerotherapy/economics , Sclerotherapy/statistics & numerical data
13.
JAMA Otolaryngol Head Neck Surg ; 149(4): 368-369, 2023 04 01.
Article in English | MEDLINE | ID: mdl-36729472

ABSTRACT

A 12-year-old girl presented to the clinic with a 3-month history of intermittent stridor; her symptoms were initially most prominent while playing sports and were suspected to be due to asthma or seasonal allergies. What is your diagnosis?


Subject(s)
Trachea , Child , Humans , Trachea/pathology
14.
Ann Otol Rhinol Laryngol ; 126(5): 401-406, 2017 May.
Article in English | MEDLINE | ID: mdl-28397559

ABSTRACT

OBJECTIVE: Multidisciplinary clinics offer important value to pediatric patients with complex conditions that overlap specialties; however, such labor-intensive clinics are difficult to facilitate. We performed a cost analysis of our pediatric multidisciplinary aerodigestive clinic (MADC) to assess its financial feasibility at our tertiary care institution. METHOD: Revenue was based on net collections for clinic, professional, and hospital setting charges generated during 12 consecutive monthly MADCs beginning August 2013. Clinic charges included facility and speech pathologist fees. Professional charges included clinic and operative fees generated by providers and anesthesiologist. Hospital setting fees included facility and material charges for technical procedures. Direct expense calculations included all providers and staff salaries, benefits, and supply costs. RESULTS: Charge capture for 54 consecutive patients seen during the study time included new visits 99203-99205 (n = 63), consults 99243-99245 (n = 60), and follow-up visits 99212-99215 (n = 196). Sixty percent of patients underwent a clinic nasopharyngeal or laryngeal endoscopy (92511 or 31575), and 60% underwent subsequent intraoperative procedures with 1 (n = 8) or 2 to 3 services (n = 24). Program net revenue totaled $828 136 and direct costs $518 867, accounting for a net positive margin of $309 269. CONCLUSIONS: When including direct downstream revenue, our MADC operates on a net positive margin, making it financially feasible.


Subject(s)
Digestive System Diseases , Pediatrics , Respiratory Tract Diseases , Tertiary Care Centers , Child , Cost-Benefit Analysis , Digestive System Diseases/diagnosis , Digestive System Diseases/therapy , Feasibility Studies , Humans , Patient Care Team/economics , Patient Care Team/organization & administration , Pediatrics/economics , Pediatrics/methods , Respiratory Tract Diseases/diagnosis , Respiratory Tract Diseases/therapy , Retrospective Studies , Tertiary Care Centers/economics , Tertiary Care Centers/organization & administration , Tertiary Care Centers/statistics & numerical data , United States
15.
JAMA Otolaryngol Head Neck Surg ; 143(7): 712-717, 2017 07 01.
Article in English | MEDLINE | ID: mdl-28472239

ABSTRACT

Importance: Ibuprofen used in postoperative management of pain after tonsillectomy has not been shown to increase the overall risk for posttonsillectomy hemorrhage (PTH). The severity of bleeding is difficult to quantify but may be a more important outcome to measure. Objective: To evaluate the association between ibuprofen use and severity of PTH using transfusion events as a marker of severity. Design, Setting, and Participants: This retrospective cohort study identified 8868 patients who underwent tonsillectomy from January 20, 2011, through June 30, 2014, at the tertiary academic Children's Hospital of Philadelphia. Of these patients, 6710 met the inclusion criteria. Data were collected using electronic database acquisition and query. Main Outcomes and Measures: Multivariate analysis was performed to identify independent prognostic factors for PTH and receipt of transfusion. Results: Of the 6710 patients who met criteria for analysis (3454 male [51.5%] and 3256 female [48.5%]; median age, 5.4 years [interquartile range, 3.7-8.2 years]), 222 (3.3%) presented with PTH that required surgical control (sPTH). A total of 15 of the 8868 patients required transfusion for an overall risk for transfusion after tonsillectomy of 0.2%. Fifteen of 222 patients undergoing sPTH (6.8%) received transfusions. No significant independent increased risk for sPTH was associated with use of ibuprofen (adjusted odds ratio [OR], 0.90; 95% CI, 0.68-1.19). A significant independent association was found in the risk for sPTH in patients 12 years or older (adjusted OR, 2.74; 95% CI, 1.99-3.76) and in patients with a history of recurrent tonsillitis (adjusted OR, 1.52; 95% CI, 1.12-2.06). When using transfusion rates as a surrogate for severity of sPTH, transfusion increased by more than 3-fold among ibuprofen users compared with nonusers (adjusted OR, 3.16; 95% CI, 1.01-9.91), and the upper limit of the 95% CI suggests the difference could be nearly 10 times greater. Conclusions and Relevance: The risk for sPTH is not increased with use of postoperative ibuprofen but is increased in patients 12 years or older and patients undergoing tonsillectomy with a history of recurrent tonsillitis. Hemorrhage severity is significantly increased with ibuprofen use when using transfusion rate as a surrogate marker for severity.


Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Ibuprofen/adverse effects , Pain, Postoperative/prevention & control , Postoperative Hemorrhage/chemically induced , Postoperative Hemorrhage/surgery , Tonsillectomy , Blood Transfusion , Child , Child, Preschool , Female , Humans , Male , Retrospective Studies , Risk Factors , Severity of Illness Index
16.
Int J Pediatr Otorhinolaryngol ; 78(8): 1360-4, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24958162

ABSTRACT

UNLABELLED: Otolaryngologic problems are common in the 22q11.2 deletion syndrome (DS) population. Structural anomalies and retrognathia may predispose these patients to obstructive sleep apnea (OSA). The current association of OSA in this population is not defined. OBJECTIVE: (1) Define the frequency of OSA in 22q11.2 DS patients referred for polysomnography (PSG). (2) Determine if OSA is present before and/or after surgery to correct velopharyngeal insufficiency (VPI). (3) Determine effect of prior adenotonsillectomy on OSA following VPI surgery. METHODS: Retrospective review of children treated from 2006 to 2013 in a tertiary care setting identified by ICD-9 758.32 (velocardiofacial syndrome) and 279.11 (DiGeorge syndrome). Surgical history and PSG data were abstracted from the identified records. RESULTS: We identified 323 patients with 22q11.2 DS; 57 (18%) were screened at any point in care using PSG and 15 patients had PSG at multiple time points in care. In most cases, indication for PSG was sleep disordered breathing or pre-operative planning. Overall, 33 patients met criteria for OSA on PSG, accounting for 10.2% of our study population; however, the percentage of patients with OSA was significantly higher within the group of 57 patients (58%) who were screened with PSG. Twenty-one of the screened patients (54%) had PSG prior to any pharyngeal surgery and had mild to severe OSA (obstructive apnea/hypopnea index (AHI): median 5.1/h, range 1.9-25.6). Eighteen patients had PSG after adenotonsillectomy; 8 of these patients (44%) had mild to moderate OSA (median AHI 2.95/h, range 1.9-5.4). Seventeen patients had PSG after VPI surgery (palatopharyngeal flap (PPF) n=16, sphincteroplasty n=1). Nine of these patients (53%) had mild to severe OSA (median AHI 3/h, range 1.9-15). Patients who underwent adenotonsillectomy prior to VPI surgery had similar prevalence of OSA (50%, n=12) than those who did not (OSA: 60%, n=5, p=0.70). Most children had mild OSA. CONCLUSION: Prevalence of OSA in this population of 22q11.2 DS patients is higher than expected in the general population. OSA risk is highest after VPI surgery, and may be decreased by adenotonsillectomy. Providers should have awareness of increased prevalence of OSA in patients with 22q11.2 DS. Close monitoring for OSA is warranted given the likelihood of subsequent surgical intervention that can worsen OSA.


Subject(s)
DiGeorge Syndrome/complications , Sleep Apnea, Obstructive/diagnosis , Adenoidectomy , Adolescent , Child , Child, Preschool , DiGeorge Syndrome/surgery , Female , Humans , Infant , Male , Polysomnography , Retrospective Studies , Severity of Illness Index , Tonsillectomy , Velopharyngeal Insufficiency/surgery
18.
Otolaryngol Head Neck Surg ; 146(3): 467-72, 2012 Mar.
Article in English | MEDLINE | ID: mdl-22140204

ABSTRACT

OBJECTIVE: To determine benefits of early intervention in neonates with symptomatic micrognathia who underwent bilateral mandibular distraction osteogenesis within the first 90 days of life as relates to growth, need for supportive care, and further invasive procedures. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care, academic children's hospital. SUBJECTS AND METHODS: Review of neonates with symptomatic micrognathia who underwent bilateral mandibular distraction osteogenesis in the past 5 years. Inclusion criteria included mandibular distraction osteogenesis performed within the first 90 days of life. Outcome measures included hospital course, growth curves, supportive home care needs, and airway at cleft repair. RESULTS: Twenty-four patients met inclusion criteria. The mean age at distraction was 30 days, and the average discharge was postoperative day 14. One patient required home oxygen, 50% were able to feed exclusively by oral diet, and no patients required tracheotomy. In addition, airway results were substantial, with 90% of patients showing objective improvement in airway grade from time of mandibular distraction to time of cleft repair. CONCLUSION: We present our initial outcomes on mandibular distraction osteogenesis in neonates with symptomatic micrognathia. Early intervention allows discharge to home with minimal supportive care needs by avoiding tracheostomy and facilitating transition to oral feeds. The airway improvement is significant and is sustained and allows for easier intubation at time of cleft repair.


Subject(s)
Airway Obstruction/surgery , Mandible/surgery , Micrognathism/surgery , Osteogenesis, Distraction/methods , Airway Obstruction/etiology , Cohort Studies , Early Medical Intervention/methods , Female , Follow-Up Studies , Hospitals, Pediatric , Humans , Infant , Infant, Newborn , Male , Mandible/abnormalities , Micrognathism/complications , Osteogenesis, Distraction/adverse effects , Postoperative Care/methods , Postoperative Complications/diagnosis , Postoperative Complications/epidemiology , Retrospective Studies , Risk Assessment , Severity of Illness Index , Time Factors , Treatment Outcome
19.
Int Forum Allergy Rhinol ; 1(3): 157-60, 2011.
Article in English | MEDLINE | ID: mdl-22287365

ABSTRACT

BACKGROUND: Nasal polyps in patients with cystic fibrosis (CF) are believed to be phenotypically different than polyps affecting non-CF patients. The objective of this study was to characterize the expression of inflammatory cytokines within nasal polyps from CF and non-CF, aspirin-tolerant patients. "Regulated on activation, normal T-cell expressed and secreted" (RANTES) is a chemotactic cytokine involved in the recruitment and activation of eosinophils. Multiple molecular studies of non-CF polyps have established that RANTES may play an important role in nasal polyposis. Our study suggests RANTES may be upregulated in CF polyps relative to non-CF polyps. METHODS: Nasal polyps were prospectively obtained from CF and non-CF, aspirin-tolerant patients. The Quantibody™ Human Cytokine Array I from RayBiotech, Inc., was used to identify differences in cytokine expression between protein extracts of two polyp groups. Four CF polyp extracts and 4 non-CF, aspirin-tolerant polyp extracts were each incubated on identical antibody subarrays, each containing 20 human cytokines in quadruplicate. Western blot analysis confirmed expression of RANTES. RESULTS: The protein microarray suggests a greater than 4-fold upregulation of RANTES in CF polyps relative to non-CF polyps. Western blot analysis confirmed expression of RANTES in CF polyps. CONCLUSION: Chemokines such as RANTES are responsible for the activation of inflammatory cells within the lamina propria of nasal polyps. We have demonstrated that RANTES may be an important cytokine associated with CF polyps.


Subject(s)
Chemokine CCL5/metabolism , Cystic Fibrosis/metabolism , Nasal Polyps/metabolism , Blotting, Western , Enzyme-Linked Immunosorbent Assay , Humans , Prospective Studies , Protein Array Analysis , Up-Regulation
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