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1.
J Pediatr ; 256: 5-10.e2, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36403673

RESUMO

OBJECTIVE: To validate a novel biomarker, airway impedance for extraesophageal disease. STUDY DESIGN: We prospectively recruited patients with respiratory symptoms undergoing combined endoscopy and direct laryngoscopy for the evaluation of symptoms. The direct laryngoscopy was performed and videotaped for blinded scoring by 3 otolaryngologists and an impedance catheter was placed onto the posterior larynx to obtain measurements. Following this, an endoscopy was performed and impedance measurements and biopsies were taken at 3 esophageal heights. Impedance values were compared within and between patients. RESULTS: Eighty-eight patients were recruited, of which 73 had complete airway and endoscopic exams. There was no significant correlation between airway impedance values and mean reflux finding scores (r2 = 0.45, P = .07). There was no significant positive correlation between airway impedance and esophageal impedance values (r2 = 0.097-0.138, P > .2). Patients taking proton pump inhibitors had significantly lower mean airway impedance values (706 ± 450 Ω) than patients not taking them (1069 ± 809 Ω, P = .06). Patients who had evidence of aspiration on video fluoroscopic swallow studies had lower airway impedance (871 ± 615 Ω) than patients without aspiration (1247 ± 360 Ω, P = .008). Inhaled steroids did not impact airway impedance levels (P = .7). CONCLUSIONS: Airway impedance may be an important diagnostic tool to diagnose gastroesophageal reflux or aspiration, eliminating the subjectivity of airway appearance alone.


Assuntos
Refluxo Gastroesofágico , Humanos , Impedância Elétrica , Refluxo Gastroesofágico/diagnóstico , Laringoscopia , Inflamação , Inibidores da Bomba de Prótons , Endoscopia Gastrointestinal , Monitoramento do pH Esofágico
2.
J Pediatr Gastroenterol Nutr ; 77(1): 86-92, 2023 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-37084338

RESUMO

OBJECTIVES: The objective of this study is to determine demographic and clinical characteristics of infants and toddlers <2 years with eosinophilic esophagitis (EoE) and to assess treatment response in this rarely studied pediatric age group. METHODS: Retrospective study of children <2 years diagnosed with EoE at a single center from 2016 to 2018. EoE was defined by ≥15 eosinophils per high power field (eos/hpf) on at least 1 esophageal biopsy. Demographics, symptoms, and endoscopic findings were collected via chart review. EoE treatment [proton pump inhibitor (PPI), swallowed steroids, dietary restriction, or a combination] and treatment responses on all follow-up endoscopies were reviewed, with remission defined as <15 eos/hpf. RESULTS: Forty-two children ages 1.3 ± 0.4 years underwent 3.8 ± 2.3 endoscopies over 3.6 ± 1.7 years of follow-up. Thirty-six children (86%) were male, and comorbidities included atopy (86%), reflux (74%), and a history of cow's milk protein allergy (40%). Common symptoms were feeding difficulties in 67% of patients (with gagging or coughing with feeding in 60% and difficulty with progression to pureed or solid foods in 43%), vomiting (57%), and coughing/wheezing (52%). Of the 37 patients with follow-up endoscopies, 25 (68%) had histologic remission. There was an effect of therapy type on histologic response ( P = 0.004) with the best responses seen on combinations of diet/steroids or diet/PPI and the worst response seen on PPIs alone. All patients showed improvement in ≥1 symptom at the time of first follow-up endoscopy. CONCLUSIONS: EoE should be considered in young children with feeding difficulties, vomiting, or respiratory symptoms. All patients improved clinically with standard medical or dietary interventions, however there is dissociation between clinical and histologic response with only 2 of 3 patients achieving histologic remission.


Assuntos
Esofagite Eosinofílica , Feminino , Animais , Bovinos , Masculino , Esofagite Eosinofílica/diagnóstico , Esofagite Eosinofílica/epidemiologia , Esofagite Eosinofílica/terapia , Estudos Retrospectivos , Inibidores da Bomba de Prótons/uso terapêutico , Vômito/etiologia , Vômito/tratamento farmacológico
3.
Crit Care Med ; 49(3): e291-e303, 2021 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33405412

RESUMO

OBJECTIVES: Hemoptysis is uncommon in children, even among the critically ill, with a paucity of epidemiological data to inform clinical decision-making. We describe hemoptysis-associated ICU admissions, including those who were critically ill at hemoptysis onset or who became critically ill as a result of hemoptysis, and identify predictors of mortality. DESIGN: Retrospective cohort study. Demographics, hemoptysis location, and management were collected. Pediatric Logistic Organ Dysfunction-2 score within 24 hours of hemoptysis described illness severity. Primary outcome was inhospital mortality. SETTING: Quaternary pediatric referral center between July 1, 2010, and June 30, 2017. PATIENTS: Medical/surgical (PICU), cardiac ICU, and term neonatal ICU admissions with hemoptysis during or within 24 hours of ICU admission. INTERVENTIONS: No intervention. MEASUREMENTS AND MAIN RESULTS: There were 326 hemoptysis-associated ICU admissions in 300 patients. Most common diagnoses were cardiac (46%), infection (15%), bronchiectasis (10%), and neoplasm (7%). Demographics, interventions, and outcomes differed by diagnostic category. Overall, 79 patients (26%) died inhospital and 109 (36%) had died during follow-up (survivor mean 2.8 ± 1.9 yr). Neoplasm, bronchiectasis, renal dysfunction, inhospital hemoptysis onset, and higher Pediatric Logistic Organ Dysfunction-2 score were independent risk factors for inhospital mortality (p < 0.02). Pharmacotherapy (32%), blood products (29%), computerized tomography angiography (26%), bronchoscopy (44%), and cardiac catheterization (36%) were common. Targeted surgical interventions were rare. Of survivors, 15% were discharged with new respiratory support. Of the deaths, 93 (85%) occurred within 12 months of admission. For patients surviving 12 months, 5-year survival was 87% (95% CI, 78-92) and mortality risk remained only for those with neoplasm (log-rank p = 0.001). CONCLUSIONS: We observed high inhospital mortality from hemoptysis-associated ICU admissions. Mortality was independently associated with hemoptysis onset location, underlying diagnosis, and severity of critical illness at event. Additional mortality was observed in the 12-month posthospital discharge. Future directions include further characterization of this vulnerable population and management recommendations for life-threatening pediatric hemoptysis incorporating underlying disease pathophysiology.


Assuntos
Estado Terminal/mortalidade , Hemoptise/mortalidade , Índice de Gravidade de Doença , Adolescente , Criança , Pré-Escolar , Feminino , Hemoptise/terapia , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Pediátrica , Masculino , Alta do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Taxa de Sobrevida
4.
J Pediatr ; 238: 42-49.e2, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34186104

RESUMO

OBJECTIVE: To determine whether the use of acid suppression and thickened feeds impact laryngomalacia outcomes in infants, including supraglottoplasty risk, time to supraglottoplasty, and hospitalization risk. STUDY DESIGN: We performed a retrospective cohort study to compare risk and time with supraglottoplasty and frequency and duration of hospitalizations for infants diagnosed with laryngomalacia at Boston Children's Hospital between January 1 and December 31, 2017. The primary outcomes were supraglottoplasty requirement, time to supraglottoplasty, and hospitalization risk. Multivariate analyses were performed to determine predictors of supraglottoplasty and hospitalization risk after adjusting for laryngomalacia severity and comorbidities in addition to propensity score adjustment. Kaplan-Meier curves were created to determine the impact of acid suppression use on time to supraglottoplasty. RESULTS: In total, 236 subjects with mean age 62.6 ± 4 days were included in the analysis; 55% were treated with acid suppression. Subjects treated with acid suppression had a greater risk of supraglottoplasty (hazard ratio 3.36, 95% CI 1.36-8.29, P = .009), shorter time to supraglottoplasty (5.64 ± 0.92 vs 7.98 ± 1.92 months, P = .006), and increased respiratory hospitalization risk (relative risk 1.97, 95% CI 1.01-3.85, 0.047), even after adjustment for covariates. Subjects receiving thickening had fewer respiratory hospitalization nights and longer time to supraglottoplasty (9.3 ± 1.7 vs 4.56 ± 0.73 months, P = .004), even after adjustment. CONCLUSIONS: Acid suppression use does not reduce the frequency of supraglottoplasty and related hospitalizations compared with untreated subjects. However, patients treated with thickening have decreased hospitalization and longer time to supraglottoplasty, suggesting that thickening of feeds may be a preferred intervention over acid suppression.


Assuntos
Antiulcerosos/administração & dosagem , Transtornos de Deglutição/terapia , Refluxo Gastroesofágico/prevenção & controle , Laringomalácia/complicações , Antiulcerosos/efeitos adversos , Transtornos de Deglutição/etiologia , Feminino , Refluxo Gastroesofágico/etiologia , Glote/cirurgia , Hospitalização , Humanos , Lactente , Laringomalácia/cirurgia , Laringomalácia/terapia , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento
5.
J Pediatr Gastroenterol Nutr ; 68(2): 218-224, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30320668

RESUMO

OBJECTIVES: To determine if children with laryngeal penetration on videofluoroscopic swallow study (VFSS) who received feeding interventions (thickened liquids, change in liquid flow rate, and/or method of liquid delivery) had improved symptoms and decreased hospitalizations compared with those without intervention. METHODS: We performed a retrospective cohort study of children under 2 years with laryngeal penetration on VFSS at our institution in 2015 to determine initial and follow-up VFSS findings, symptom improvement at follow-up, and hospitalization risk before and after VFSS. Proportions were compared with Fisher exact test and hospitalizations with paired t tests. RESULTS: We evaluated 137 subjects with age 8.93 ±â€Š0.59 months who had laryngeal penetration without aspiration on VFSS. Fifty-five percent had change in management, with 40% receiving thickening and 15% a change in flow rate. There was significant improvement in symptoms for children that had feeding intervention and this improvement was the greatest with thickening (OR 41.8, 95% CI 12.34-141.69, P < 0.001). On repeat VFSS, 26% had evidence of aspiration that was not captured on initial VFSS. Subjects had decreased total and pulmonary hospitalizations with feeding intervention and decreased pulmonary nights with thickening (P < 0.05). CONCLUSIONS: Laryngeal penetration appears to be clinically significant in children with oropharyngeal dysphagia and interventions to decrease its occurrence are associated with improved outcomes including decreased symptoms of concern and hospitalization nights. Thickening or other feeding intervention should be considered for all symptomatic children with laryngeal penetration on swallow study.


Assuntos
Transtornos de Deglutição/terapia , Doenças da Laringe/terapia , Apoio Nutricional/métodos , Deglutição , Transtornos de Deglutição/diagnóstico , Transtornos de Deglutição/fisiopatologia , Feminino , Fluoroscopia , Seguimentos , Humanos , Lactente , Doenças da Laringe/diagnóstico , Doenças da Laringe/fisiopatologia , Masculino , Estudos Retrospectivos , Resultado do Tratamento
6.
Anesth Analg ; 129(4): 1053-1060, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-30300182

RESUMO

BACKGROUND: The Pediatric Perioperative Surgical Home (PPSH) model is an integrative care model designed to provide better patient care and value by shifting focus from the patient encounter level to the overarching surgical episode of care. So far, no PPSH model has targeted a complex airway disorder. It was hypothesized that the development of a PPSH for laryngeal cleft repair would reduce the high rates of postoperative resource utilization observed in this population. METHODS: Institutional review board approval was obtained for the purpose of data collection and analysis. A multidisciplinary team of anesthesiologists, surgeons, nursing staff, information technology specialists, and finance administrators was gathered during the PPSH development phase. Standardized perioperative (preoperative, intraoperative, and postoperative) protocols were developed, with a focus on preoperative risk stratification. Patients presenting before surgery with ≥1 predefined medical comorbidity were triaged to the intensive care unit (ICU) postoperatively, while patients without severe systemic disease were triaged to a lower-acuity floor for overnight observation. The success of the PPSH protocol was defined by quality outcome and value measurements. RESULTS: The PPSH initiative included 120 patients, and the pre-PPSH period included 115 patients who underwent laryngeal cleft repair before implementation of the new process. Patients in the pre-PPSH period were reviewed and classified as ICU candidates or lower acuity floor candidates had they presented in the post-PPSH period. Among the 79 patients in the pre-PPSH period who were identified as candidates for the lower-acuity floor transfer, 70 patients (89%) were transferred to the ICU (P < .001). Retrospective analysis concluded that 143 ICU bedded days could have been avoided in the pre-PPSH group by using PPSH risk stratification. Surgery duration (P = .034) and hospital length of stay (P = .015) were found to be slightly longer in the group of pre-PPSH observation unit candidates. Rates of 30-day unplanned readmissions to the hospital were not associated with the new PPSH initiative (P = .093). No patients in either group experienced emergent postoperative intubation or other expected complications. Total hospital costs were not lower for PPSH observation unit patients as compared to pre-PPSH observation unit candidates (difference = 8%; 95% confidence interval, -7% to 23%). CONCLUSIONS: A well-defined preoperative screening protocol for patients undergoing laryngeal cleft repair can reduce postoperative ICU utilization without affecting patient safety. Further research is needed to see if these findings are applicable to other complex airway surgeries.


Assuntos
Anormalidades Congênitas/cirurgia , Cuidados Críticos/organização & administração , Procedimentos Clínicos/organização & administração , Prestação Integrada de Cuidados de Saúde/organização & administração , Laringoscopia , Laringe/anormalidades , Assistência Centrada no Paciente/organização & administração , Boston , Criança , Pré-Escolar , Anormalidades Congênitas/diagnóstico , Técnicas de Apoio para a Decisão , Hospitais Pediátricos , Humanos , Lactente , Laringoscopia/efeitos adversos , Laringe/cirurgia , Tempo de Internação , Período Perioperatório , Complicações Pós-Operatórias/terapia , Valor Preditivo dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
7.
Gut ; 67(1): 36-42, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-27742763

RESUMO

OBJECTIVE: The clinical presentation and course of Crohn's disease (CD) is highly variable. We sought to better understand the cellular and molecular mechanisms that guide this heterogeneity, and characterise the cellular processes associated with disease phenotypes. DESIGN: We examined both gene expression and gene regulation (chromatin accessibility) in non-inflamed colon tissue from a cohort of adult patients with CD and control patients. To support the generality of our findings, we analysed previously published expression data from a large cohort of treatment-naïve paediatric CD and control ileum. RESULTS: We found that adult patients with CD clearly segregated into two classes based on colon tissue gene expression-one that largely resembled the normal colon and one where certain genes showed expression patterns normally specific to the ileum. These classes were supported by changes in gene regulatory profiles observed at the level of chromatin accessibility, reflective of a fundamental shift in underlying molecular phenotypes. Furthermore, gene expression from the ilea of a treatment-naïve cohort of paediatric patients with CD could be similarly subdivided into colon-like and ileum-like classes. Finally, expression patterns within these CD subclasses highlight large-scale differences in the immune response and aspects of cellular metabolism, and were associated with multiple clinical phenotypes describing disease behaviour, including rectal disease and need for colectomy. CONCLUSIONS: Our results strongly suggest that these molecular signatures define two clinically relevant forms of CD irrespective of tissue sampling location, patient age or treatment status.


Assuntos
Doença de Crohn/genética , Adulto , Fatores Etários , Estudos de Casos e Controles , Criança , Colo/metabolismo , Doença de Crohn/classificação , Doença de Crohn/metabolismo , Doença de Crohn/terapia , Feminino , Perfilação da Expressão Gênica/métodos , Regulação da Expressão Gênica , Estudo de Associação Genômica Ampla , Humanos , Íleo/metabolismo , Masculino , Fenótipo , Análise de Componente Principal , Prognóstico
8.
Eur J Immunol ; 46(8): 1912-25, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27159132

RESUMO

Intestinal macrophages (IMs) are uniquely programmed to tolerate exposure to bacteria without mounting potent inflammatory responses. The cytokine IL-10 maintains the macrophage anti-inflammatory response such that loss of IL-10 results in chronic intestinal inflammation. To investigate how IL-10-deficiency alters IM programming and bacterial tolerance, we studied changes in chromatin accessibility in response to bacteria in macrophages from two distinct niches, the intestine and bone-marrow, from both wild-type and IL-10-deficient (Il10(-/-) ) mice. We identified chromatin accessibility changes associated with bacterial exposure and IL-10 deficiency in both bone marrow derived macrophages and IMs. Surprisingly, Il10(-/-) IMs adopted chromatin and gene expression patterns characteristic of an inflammatory response, even in the absence of bacteria. Further, when recombinant IL-10 was added to Il10(-/-) cells, it could not revert the chromatin landscape to a normal state. Our results demonstrate that IL-10 deficiency results in stable chromatin alterations in macrophages, even in the absence of bacteria. This supports a model in which IL-10-deficiency leads to chromatin alterations that contribute to a loss of IM tolerance to bacteria, which is a primary initiating event in chronic intestinal inflammation.


Assuntos
Cromatina/metabolismo , Inflamação/imunologia , Interleucina-10/genética , Intestinos/fisiopatologia , Macrófagos/metabolismo , Animais , Citocinas/metabolismo , Ensaio de Desvio de Mobilidade Eletroforética , Expressão Gênica , Humanos , Tolerância Imunológica , Intestinos/imunologia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout
9.
J Pediatr ; 183: 127-131, 2017 04.
Artigo em Inglês | MEDLINE | ID: mdl-27979581

RESUMO

OBJECTIVE: To determine if the reflux finding score (RFS), a validated score for airway inflammation, correlates with gastroesophageal reflux measured by multichannel intraluminal impedance (MII) testing, endoscopy, and quality of life scores. STUDY DESIGN: We performed a prospective, cross-sectional cohort study of 77 children with chronic cough undergoing direct laryngoscopy and bronchoscopy, esophagogastroduodenoscopy, and MII testing with pH (pH-MII) between 2006 and 2011. Airway examinations were videotaped and reviewed by 3 blinded otolaryngologists each of whom assigned RFS to the airways. RFS were compared with the results of reflux testing (endoscopy, MII, symptom scores). An intraclass correlation coefficient was calculated for the degree of agreement between otolaryngologists' RFS. Receiver operating characteristic curves were created to determine the sensitivity of the RFS. Spearman correlation was calculated between the RFS and reflux measurements by pH-MII. RESULTS: The mean ± SD RFS was 12 ± 4. There was no correlation between pH-MII variables and mean RFS (|r| < 0.15). The concordance correlation coefficient for RFS between otolaryngologists was low (intraclass correlation coefficient = 0.32). Using pH-metry as a gold standard, the positive predictive value for the RFS was 29%. Using MII as the gold standard, the positive predictive value for the RFS was 40%. There was no difference in the mean RFS in patients with (12 ± 4) and without (12 ± 3) esophagitis (P = .9). There was no correlation between RFS and quality of life scores (|r| < 0.15, P > .3). CONCLUSIONS: The RFS cannot predict pathologic gastroesophageal reflux and an airway examination should not be used as a basis for prescribing gastroesophageal reflux therapies.


Assuntos
Tosse/diagnóstico , Refluxo Gastroesofágico/diagnóstico , Edema Laríngeo/diagnóstico , Adolescente , Criança , Pré-Escolar , Doença Crônica , Estudos de Coortes , Estudos Transversais , Diagnóstico Diferencial , Impedância Elétrica , Monitoramento do pH Esofágico , Esofagoscopia/métodos , Feminino , Humanos , Concentração de Íons de Hidrogênio , Laringoscopia/métodos , Masculino , Variações Dependentes do Observador , Otolaringologia/métodos , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença
10.
J Pediatr ; 166(4): 1048-54.e1-5, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25681199

RESUMO

OBJECTIVES: To test the hypothesis that somatic phosphatidylinositol-4,5-bisphospate 3-kinase, catalytic subunit alpha (PIK3CA) mutations would be found in patients with more common disorders including isolated lymphatic malformation (LM) and Klippel-Trenaunay syndrome (KTS). STUDY DESIGN: We used next generation sequencing, droplet digital polymerase chain reaction, and single molecule molecular inversion probes to search for somatic PIK3CA mutations in affected tissue from patients seen at Boston Children's Hospital who had an isolated LM (n = 17), KTS (n = 21), fibro-adipose vascular anomaly (n = 8), or congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (n = 33), the disorder for which we first identified somatic PIK3CA mutations. We also screened 5 of the more common PIK3CA mutations in a second cohort of patients with LM (n = 31) from Seattle Children's Hospital. RESULTS: Most individuals from Boston Children's Hospital who had isolated LM (16/17) or LM as part of a syndrome, such as KTS (19/21), fibro-adipose vascular anomaly (5/8), and congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (31/33) were somatic mosaic for PIK3CA mutations, with 5 specific PIK3CA mutations accounting for ∼ 80% of cases. Seventy-four percent of patients with LM from Seattle Children's Hospital also were somatic mosaic for 1 of 5 specific PIK3CA mutations. Many affected tissue specimens from both cohorts contained fewer than 10% mutant cells. CONCLUSIONS: Somatic PIK3CA mutations are the most common cause of isolated LMs and disorders in which LM is a component feature. Five PIK3CA mutations account for most cases. The search for causal mutations requires sampling of affected tissues and techniques that are capable of detecting low-level somatic mosaicism because the abundance of mutant cells in a malformed tissue can be low.


Assuntos
Anormalidades Múltiplas , DNA/genética , Síndrome de Klippel-Trenaunay-Weber/genética , Anormalidades Linfáticas/genética , Mutação , Fosfatidilinositol 3-Quinases/genética , Malformações Vasculares/genética , Criança , Pré-Escolar , Classe I de Fosfatidilinositol 3-Quinases , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Síndrome de Klippel-Trenaunay-Weber/diagnóstico , Síndrome de Klippel-Trenaunay-Weber/metabolismo , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/metabolismo , Masculino , Fosfatidilinositol 3-Quinases/metabolismo , Reação em Cadeia da Polimerase , Malformações Vasculares/diagnóstico , Malformações Vasculares/metabolismo
11.
Clin Transl Gastroenterol ; 15(2): e00670, 2024 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-38146854

RESUMO

INTRODUCTION: Our understanding of the epidemiology of inflammatory conditions of the pouch and effectiveness of treatment is largely based on selected populations. We created a state-level registry to evaluate the incidence of pouchitis and the effectiveness of treatments used in an initial episode of pouchitis. METHODS: In a state-level retrospective cohort of all patients undergoing proctocolectomy with ileal pouch-anal anastomosis (IPAA) for ulcerative colitis between January 1, 2018, and December 31, 2020, we evaluated the incidence of pouchitis and compared the proportion of patients developing recurrent pouchitis and chronic antibiotic-dependent pouchitis according to initial antibiotic therapy. RESULTS: A total of 177 patients underwent surgery with 49 (28%) developing pouchitis within the 12 months after the final stage of IPAA. Patients with extraintestinal manifestations of inflammatory bowel disease (IBD) were significantly more likely to develop pouchitis within the first 12 months after IPAA (adjusted odds ratio 2.45, 95% confidence interval 1.03-5.81) after adjusting for family history of IBD (adjusted odds ratio 3.50, 95% 1.50-8.18). When comparing the proportion of patients who developed recurrent pouchitis or chronic antibiotic-dependent pouchitis with those who experienced an isolated episode of pouchitis, there were no significant differences among the initial antibiotic regimens used. DISCUSSION: In a state-level examination of outcomes after IPAA for ulcerative colitis, patients with extraintestinal manifestations of IBD were more likely to develop pouchitis; however, the initial antibiotic regimen chosen did not seem to affect long-term outcomes.


Assuntos
Colite Ulcerativa , Doenças Inflamatórias Intestinais , Pouchite , Humanos , Pouchite/epidemiologia , Pouchite/etiologia , Colite Ulcerativa/epidemiologia , Colite Ulcerativa/cirurgia , Colite Ulcerativa/complicações , Estudos Retrospectivos , Fatores de Risco , Complicações Pós-Operatórias/epidemiologia , Doenças Inflamatórias Intestinais/epidemiologia , Doenças Inflamatórias Intestinais/cirurgia , Doenças Inflamatórias Intestinais/complicações , Antibacterianos/uso terapêutico
12.
Int J Pediatr Otorhinolaryngol ; 176: 111810, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38147730

RESUMO

OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.


Assuntos
Otolaringologia , Lactente , Criança , Humanos , Consenso , Inquéritos e Questionários , Técnica Delphi
13.
Paediatr Anaesth ; 23(4): 334-41, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23368883

RESUMO

INTRODUCTION: Laryngeal cleft is a rare congenital malformation that is being reported with increasing frequency. Diagnosis requires suspension microlaryngoscopy under general anesthesia during spontaneous respiration. Repair may be attempted by a minimally invasive endoscopic approach or open surgical repair. The authors report on their experience with total intravenous anesthesia (TIVA) and spontaneous ventilation without an endotracheal tube during suspension laryngoscopy and CO2 laser application for this specific surgical procedure. Of particular interest were the rate at which this technique failed and rescue techniques were employed and the ability to predict patients in whom this might occur. METHODS: Between July 2004 and September 2012, 110 endoscopic laryngeal cleft repairs were completed under TIVA with spontaneous ventilation without an endotracheal tube. Anesthetic induction was achieved by inhalation of sevoflurane and oxygen by mask or infusion of propofol at 300 mcg kg(-1) min(-1) and remifentanil at 0.05-1.0 mcg kg(-1)  min. The vocal cords and surgical site were sprayed with up to 2 mg kg(-1) of 4% lidocaine. If the oxygen saturation decreased during the procedure or the patient became apneic, a rescue process utilizing jet ventilation or intermittent intubation was instituted. RESULTS: Ten (9.1%) of the 110 cases required rescue (95% confidence interval [CI]: 5.0-15.8%). The most prevalent comorbidities included reactive airway disease, chronic lung disease, failure to thrive, developmental delay, and an unrelated syndrome. Thirty-nine patients (36%) had reactive airway disease and twelve (11%) had chronic lung disease. Intraoperative complications included six cases requiring a brief, temporary period of intubation (5.5%) and four cases requiring a brief period of jet ventilation (3.6%). CONCLUSION: The technique of TIVA with spontaneous respirations without an endotracheal tube is a safe and effective technique for laryngeal cleft repair. Although the potential for intraoperative adverse events may be high, the actual rate was very low. The need to convert to other techniques is not significant although the children who did require brief periods of jet ventilation or intubation tended to have reactive airway disease or chronic lung disease.


Assuntos
Anestesia por Inalação , Anestesia Intravenosa , Anormalidades Congênitas/cirurgia , Laringe/anormalidades , Procedimentos Cirúrgicos Otorrinolaringológicos/métodos , Apneia/epidemiologia , Apneia/terapia , Pré-Escolar , Estudos de Coortes , Endoscopia , Feminino , Ventilação em Jatos de Alta Frequência , Humanos , Complicações Intraoperatórias/epidemiologia , Laringoscopia , Laringe/cirurgia , Terapia a Laser , Pneumopatias/complicações , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Oxigênio/sangue
14.
Int J Pediatr Otorhinolaryngol ; 164: 111398, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36455431

RESUMO

BACKGROUND: Interviews for Pediatric Otolaryngology fellowship rapidly transitioned to virtual interviews mid-cycle in March 2020 due to the COVID-19 pandemic. OBJECTIVE: This study aims to describe perspectives on virtual versus in-person interviews for both applicants and program directors. METHODS: Cross-sectional study. Surveys were conducted of all Pediatric Otolaryngology fellowship applicants participating in the San Francisco Match and program directors in 2020 and 2021. RESULTS: Out of 32 U.S. trained fellowship applicants, 24 completed the survey in 2020 and 18 in 2021. While 70% of applicants felt they did not get the same experience with virtual interviews, 75% did not feel it changed how they ranked programs. Applicant perception of virtual interviews improved in 2021, with the majority (56%) preferring virtual interviews if provided an option. Twenty out of 36 fellowship directors completed the survey in 2020, and eighteen in 2021. While fellowship directors continued to prefer in-person in 2021, an increased number (10% in 2020, 30% in 2021) felt continuing with virtual interviews may increase the number of applicants in the future. CONCLUSION: Based on the survey, both applicants and fellowship directors had a less favorable perception of virtual interviews compared to in-person interviews initially; however, applicant perception favored virtual interviews in 2021, while Program Directors continued to prefer in-person.


Assuntos
COVID-19 , Internato e Residência , Otolaringologia , Criança , Humanos , Estudos Transversais , Bolsas de Estudo , Pandemias , COVID-19/epidemiologia , Atitude , Inquéritos e Questionários
15.
Int J Pediatr Otorhinolaryngol ; 168: 111547, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37079945

RESUMO

OBJECTIVE: Mandibular tumors in the pediatric population are rare. These malignancies are variable in their histology, and combined with their rarity, has made it difficult to describe their clinical course, and treatment guidelines. The aim of this paper is to describe the experience of Boston Children's Hospital, a pediatric tertiary referral center, with treating malignant mandibular malignancies, as well as provide multi-disciplinary team approach in managing this clinical entity. METHODS: A retrospective search was performed for mandibular malignancies in pediatric patients between 1995 and 2020 via the pathological database at Boston Children's Hospital. Only patients with malignant solid mandibular neoplasms were included, leaving 15 patients for final analysis. RESULTS: The median age at presentation was 10.1 ± 10.3 years. Nine of 15 patients (60%) presented with jaw mass which was the most common clinical presentation. The most commonly identified histological diagnosis was rhabdomayosarcoma and osteosarcoma (n = 4, 26% each). A mandibulectomy was performed in 12 (80%) cases. Reconstruction of the mandible was performed using a fibular free flap in 6 (40%) cases, and a plate in 3 (20%) cases. Mean follow-up was 4.6 ± 4.9 years. CONCLUSION: Malignant tumors most commonly present with a jaw mass, however asymptomatic and incidental presentations follow closely and pathologies can vary greatly. Surgical resection and reconstruction is often indicated, multidisciplinary tumor board review is required to determine when children are best treated with neo-/adjuvant treatment with chemo- and radiotherapy.


Assuntos
Retalhos de Tecido Biológico , Neoplasias Mandibulares , Procedimentos de Cirurgia Plástica , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Neoplasias Mandibulares/diagnóstico , Neoplasias Mandibulares/cirurgia , Estudos Retrospectivos , Mandíbula/patologia , Transplante Ósseo
16.
Inflamm Bowel Dis ; 2023 Aug 22.
Artigo em Inglês | MEDLINE | ID: mdl-37607334

RESUMO

BACKGROUND: Our understanding of outcomes after proctocolectomy with ileal pouch-anal anastomosis (IPAA) for ulcerative colitis (UC) is largely based on analyses of selected populations. We created a state-level registry to evaluate the epidemiology of IPAA surgery and pouch-related outcomes across the major healthcare systems performing these surgeries in our state. METHODS: We created a retrospective cohort of all patients undergoing restorative proctocolectomy with IPAA for UC at 1 of 4 centers between January 1, 2018, and December 31, 2020. The primary outcomes of this study were the rate of complications and all-cause readmissions within the first 30 days of the final stage of IPAA surgery. RESULTS: During the study period, 177 patients underwent IPAA surgery with 66 (37%) experiencing a complication within 30 days. After adjusting for the number of stages in IPAA surgery, patients with extensive UC (odds ratio, 3.61; 95% confidence interval, 1.39-9.33) and current or former smokers (odds ratio, 2.98; 95% confidence interval, 1.38-6.45) were more likely to experience a complication. Among all patients, 57 (32%) required readmission within 30 days. The most common reasons for readmission were ileus/small bowel obstruction (22%), peripouch abscess (19%), and dehydration (16%). CONCLUSION: In this first state-level examination of the epidemiology of IPAA for UC, we demonstrated that the complication rate after IPAA for UC was 37%, with one-third of patients being readmitted within 30 days. Extensive disease at the time of colectomy appears to be an indicator of more severe disease and may portend a worse prognosis after IPAA.

17.
Int J Pediatr Otorhinolaryngol ; 166: 111469, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36764081

RESUMO

INTRODUCTION: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. OBJECTIVES: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. METHODS: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. CONCLUSION: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation.


Assuntos
Linfadenite , Infecções por Mycobacterium não Tuberculosas , Otolaringologia , Criança , Humanos , Micobactérias não Tuberculosas , Linfadenite/microbiologia , Antibacterianos/uso terapêutico , Excisão de Linfonodo , Infecções por Mycobacterium não Tuberculosas/diagnóstico
18.
Otolaryngol Clin North Am ; 55(6): 1321-1335, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36224059

RESUMO

While the majority of the initial attention to symptomatic COVID-19 focused on adult patients as well as adult critical care and first responders, the pandemic drastically altered care throughout the entire health care industry. COVID-19 has had a profound effect on the treatment and care of pediatric patients within pediatric otolaryngology. The objective of this article is to highlight the unique ramifications of COVID-19 in general and its effect within pediatric otolaryngology, with a focus on the immediate and potential long-term shifts in practice. This article addresses several aspects of care within pediatric otolaryngology including safety, diagnosis, and treatment of COVID-19 detailing the unique effects of the pandemic on the pediatric otolaryngology specialty and opportunities.


Assuntos
COVID-19 , Otolaringologia , Adulto , Humanos , Criança , SARS-CoV-2 , Pandemias
19.
Int J Pediatr Otorhinolaryngol ; 161: 111251, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35988373

RESUMO

OBJECTIVES: To outline an expert-based consensus of recommendations for the diagnosis and management of pediatric patients with congenital tracheal stenosis. METHODS: Expert opinions were sought from members of the International Pediatric Otolaryngology Group (IPOG) via completion of an 18-item survey utilizing an iterative Delphi method and review of the literature. RESULTS: Forty-three members completed the survey providing recommendations regarding the initial history, clinical evaluation, diagnostic evaluation, temporizing measures, definitive repair, and post-repair care of children with congenital tracheal stenosis. CONCLUSION: These recommendations are intended to be used to support clinical decision-making regarding the evaluation and management of children with congenital tracheal stenosis. Responses highlight the diverse management strategies and the importance of a multidisciplinary approach to care of these patients.


Assuntos
Otolaringologia , Procedimentos de Cirurgia Plástica , Criança , Consenso , Constrição Patológica , Humanos , Lactente , Procedimentos de Cirurgia Plástica/métodos , Traqueia/anormalidades , Traqueia/cirurgia , Estenose Traqueal/congênito , Resultado do Tratamento
20.
Curr Opin Otolaryngol Head Neck Surg ; 29(6): 487-491, 2021 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-34710067

RESUMO

PURPOSE OF REVIEW: The aim of this study was to present the evaluation and current management of congenital paediatric nasal dermoid. RECENT FINDINGS: There has been a trend towards less invasive surgical excision techniques, including purely endoscopic excision, endoscopic-assisted approaches and midline excision with nasal bone osteotomies and bone flap. These approaches allow adequate access for both total resection and nasal contour and skull base reconstruction. Following resection, if nasal bone osteotomies are insufficient for restoring nasal appearance, free temporoparietal fascial graft and/or conchal cartilage can be considered. For nasal tip deformities, interdomal sutures and free fat grafting are a suitable option. SUMMARY: Complete surgical excision remains the treatment of choice for nasal dermoid lesions. The surgical approach taken and reconstruction depends on the type of lesion (cyst versus sinus or fistula), location (intranasal versus extranasal), whether or not there is intracranial extension, and experience of the surgical team.


Assuntos
Cisto Dermoide , Neoplasias Nasais , Criança , Cisto Dermoide/diagnóstico , Cisto Dermoide/cirurgia , Endoscopia , Humanos , Nariz , Neoplasias Nasais/diagnóstico , Neoplasias Nasais/cirurgia , Base do Crânio
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