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1.
Laryngoscope ; 134(5): 2228-2235, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-37933795

RESUMO

BACKGROUND: Head and neck fibromatoses (HNFs) are a rare, diverse group of soft tissue tumors characterized by an abnormal proliferation of fibroblasts. Available literature on these tumors is limited to case reports and small single-institutional studies. OBJECTIVE: We aim to provide demographic, socioeconomic, tumor-related, and treatment characteristics of HNFs. DESIGN: Retrospective cohort analysis using the National Cancer Database (NCDB). METHODS: The NCDB was queried for fibromatosis-related histologic codes located within the head and neck region. Various factors were analyzed. Univariate and multivariate survival analyses were performed. RESULTS: Between 2004 and 2016, 130 patients were included in the analyses. Average age was 57.4 years old with a predominance of White (83.6%) males (61.5%). Non-desmoid HNFs accounted for 60%-70% of the tumors. The salivary gland was the most common location (38.5%) and more than half of the tumors were high grade. The majority were treated surgically (90.8%) and 25% had positive margins. Mean and median overall survival (OS) were 98.9 and 135.4 months, respectively. Surgery is associated with better OS than nonsurgical alternatives. Addition of adjuvant treatments was not associated with differences in survival. CONCLUSION: In the largest study to date, we describe demographic, socioeconomic, tumor-related, and treatment patterns of patients with this rare disease. These tumors are most frequently present in middle-aged males with high-grade histology. Most are treated surgically and positive surgical margins are common. Surgery has better OS than nonsurgical alternatives. While adjuvant radiation has become more common, we found no difference in survival compared to surgery alone. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:2228-2235, 2024.


Assuntos
Fibroma , Neoplasias de Cabeça e Pescoço , Masculino , Pessoa de Meia-Idade , Humanos , Feminino , Estudos Retrospectivos , Análise de Sobrevida , Estudos de Coortes , Cabeça , Neoplasias de Cabeça e Pescoço/terapia
2.
Front Cardiovasc Med ; 10: 1175673, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37396582

RESUMO

Background and aims: Neutrophils drive atheroprogression and directly contribute to plaque instability. We recently identified signal transducer and activator of transcription 4 (STAT4) as a critical component for bacterial host defense in neutrophils. The STAT4-dependent functions of neutrophils in atherogenesis are unknown. Therefore, we investigated a contributory role of STAT4 in neutrophils during advanced atherosclerosis. Methods: We generated myeloid-specific Stat4ΔLysMLdlr-/-, neutrophil-specific Stat4ΔS100A8Ldlr-/-, and control Stat4fl/flLdlr-/- mice. All groups were fed a high-fat/cholesterol diet (HFD-C) for 28 weeks to establish advanced atherosclerosis. Aortic root plaque burden and stability were assessed histologically by Movat pentachrome staining. Nanostring gene expression analysis was performed on isolated blood neutrophils. Flow cytometry was utilized to analyze hematopoiesis and blood neutrophil activation. In vivo homing of neutrophils to atherosclerotic plaques was performed by adoptively transferring prelabeled Stat4ΔLysMLdlr-/- and Stat4fl/flLdlr-/- bone marrow cells into aged atherosclerotic Apoe-/- mice and detected by flow cytometry. Results: STAT4 deficiency in both myeloid-specific and neutrophil-specific mice provided similar reductions in aortic root plaque burden and improvements in plaque stability via reduction in necrotic core size, improved fibrous cap area, and increased vascular smooth muscle cell content within the fibrous cap. Myeloid-specific STAT4 deficiency resulted in decreased circulating neutrophils via reduced production of granulocyte-monocyte progenitors in the bone marrow. Neutrophil activation was dampened in HFD-C fed Stat4ΔLysMLdlr-/- mice via reduced mitochondrial superoxide production, attenuated surface expression of degranulation marker CD63, and reduced frequency of neutrophil-platelet aggregates. Myeloid-specific STAT4 deficiency diminished expression of chemokine receptors CCR1 and CCR2 and impaired in vivo neutrophil trafficking to atherosclerotic aorta. Conclusions: Our work indicates a pro-atherogenic role for STAT4-dependent neutrophil activation and how it contributes to multiple factors of plaque instability during advanced atherosclerosis in mice.

3.
bioRxiv ; 2023 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-36865098

RESUMO

Background and Aims: Neutrophils drive atheroprogression and directly contribute to plaque instability. We recently identified signal transducer and activator of transcription 4 (STAT4) as a critical component for bacterial host defense in neutrophils. The STAT4-dependent functions of neutrophils in atherogenesis are unknown. Therefore, we investigated a contributory role of STAT4 in neutrophils during advanced atherosclerosis. Methods: We generated myeloid-specific Stat4 ΔLysM Ldlr -/- , neutrophil-specific Stat4 ΔS100A8 Ldlr -/- , and control Stat4 fl/fl Ldlr -/- mice. All groups were fed a high-fat/cholesterol diet (HFD-C) for 28 weeks to establish advanced atherosclerosis. Aortic root plaque burden and stability were assessed histologically by Movat Pentachrome staining. Nanostring gene expression analysis was performed on isolated blood neutrophils. Flow cytometry was utilized to analyze hematopoiesis and blood neutrophil activation. In vivo homing of neutrophils to atherosclerotic plaques was performed by adoptively transferring prelabeled Stat4 ΔLysM Ldlr -/- and Stat4 fl/fl Ldlr -/- bone marrow cells into aged atherosclerotic Apoe -/- mice and detected by flow cytometry. Results: STAT4 deficiency in both myeloid-specific and neutrophil-specific mice provided similar reductions in aortic root plaque burden and improvements in plaque stability via reduction in necrotic core size, improved fibrous cap area, and increased vascular smooth muscle cell content within the fibrous cap. Myeloid-specific STAT4 deficiency resulted in decreased circulating neutrophils via reduced production of granulocyte-monocyte progenitors in the bone marrow. Neutrophil activation was dampened in Stat4 ΔLysM Ldlr -/- mice via reduced mitochondrial superoxide production, attenuated surface expression of degranulation marker CD63, and reduced frequency of neutrophil-platelet aggregates. Myeloid-specific STAT4 deficiency diminished expression of chemokine receptors CCR1 and CCR2 and impaired in vivo neutrophil trafficking to atherosclerotic aorta. Conclusions: Our work indicates a pro-atherogenic role for STAT4-dependent neutrophil activation and how it contributes to multiple factors of plaque instability during advanced atherosclerosis in mice.

4.
Circ Genom Precis Med ; 16(2): e003791, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36803080

RESUMO

BACKGROUND: Congenital heart disease (CHD) is the most common major congenital anomaly and causes significant morbidity and mortality. Epidemiologic evidence supports a role of genetics in the development of CHD. Genetic diagnoses can inform prognosis and clinical management. However, genetic testing is not standardized among individuals with CHD. We sought to develop a list of validated CHD genes using established methods and to evaluate the process of returning genetic results to research participants in a large genomic study. METHODS: Two-hundred ninety-five candidate CHD genes were evaluated using a ClinGen framework. Sequence and copy number variants involving genes in the CHD gene list were analyzed in Pediatric Cardiac Genomics Consortium participants. Pathogenic/likely pathogenic results were confirmed on a new sample in a clinical laboratory improvement amendments-certified laboratory and disclosed to eligible participants. Adult probands and parents of probands who received results were asked to complete a post-disclosure survey. RESULTS: A total of 99 genes had a strong or definitive clinical validity classification. Diagnostic yields for copy number variants and exome sequencing were 1.8% and 3.8%, respectively. Thirty-one probands completed clinical laboratory improvement amendments-confirmation and received results. Participants who completed postdisclosure surveys reported high personal utility and no decision regret after receiving genetic results. CONCLUSIONS: The application of ClinGen criteria to CHD candidate genes yielded a list that can be used to interpret clinical genetic testing for CHD. Applying this gene list to one of the largest research cohorts of CHD participants provides a lower bound for the yield of genetic testing in CHD.


Assuntos
Cardiopatias Congênitas , Adulto , Criança , Humanos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/genética , Testes Genéticos , Coração , Genômica , Variações do Número de Cópias de DNA
5.
Comput Biol Med ; 49: 15-8, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24709056

RESUMO

Utilizing electronic data capture (EDC) systems in data collection and management allows automated validation programs to preemptively identify and correct data errors. For our multi-center, prospective study we chose to use TeleForm, a paper-based data capture software that uses recognition technology to create case report forms (CRFs) with similar functionality to EDC, including custom scripts to identify entry errors. We quantified the accuracy of the optimized system through a data audit of CRFs and the study database, examining selected critical variables for all subjects in the study, as well as an audit of all variables for 25 randomly selected subjects. Overall we found 6.7 errors per 10,000 fields, with similar estimates for critical (6.9/10,000) and non-critical (6.5/10,000) variables-values that fall below the acceptable quality threshold of 50 errors per 10,000 established by the Society for Clinical Data Management. However, error rates were found to widely vary by type of data field, with the highest rate observed with open text fields.


Assuntos
Coleta de Dados/métodos , Coleta de Dados/estatística & dados numéricos , Processamento Eletrônico de Dados/estatística & dados numéricos , Software , Humanos , Computação em Informática Médica , Obesidade/epidemiologia , Estudos Prospectivos
6.
Obes Surg ; 23(9): 1404-12, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23475789

RESUMO

BACKGROUND: Past body weight may be a more informative factor than current weight for risk of chronic disease development. Often, investigators must rely on subject recall to gauge past body weights. The Cincinnati Weight History Questionnaire (CWHQ) was developed to aid in the retrospective identification of adults who were obese during adolescence. METHODS: To assess validity, the CWHQ was administered to a subset of National Heart, Lung, and Blood Growth and Health Study (NGHS) participants, a group of young adult females for whom historical measured anthropometrics were available. One hundred ninety-eight NGHS participants were contacted, of whom 191 (97 %) responded (age 26-29). Participants were asked to recall height and weight from ages 13 and 18, which were compared to previously measured values. Multiple indices of validity (Bland-Altman plots, sensitivity, and specificity) were calculated. RESULTS: The CWHQ was moderately sensitive (range, 19-66 %), but highly specific (range, 89-100 %). Recalled height and weight values used to determine body mass index (BMI) underestimated BMI based on recorded height and weight at ages 13 and 18. Differences in calculated BMI based on recalled and measured height and weight were found to increase with BMI calculated using measured values. CONCLUSIONS: The CWHQ proved to be a moderately sensitive, but highly specific instrument for detecting adolescent obesity in a cohort of young adult females. Epidemiologic research seeking to discriminate between adults with adult-onset vs. adolescent-onset obesity may find the CWHQ useful.


Assuntos
Estatura , Peso Corporal , Obesidade Infantil/epidemiologia , Inquéritos e Questionários , Adolescente , Comportamento do Adolescente , Adulto , Fatores Etários , Idade de Início , Índice de Massa Corporal , Feminino , Humanos , Rememoração Mental , Ohio/epidemiologia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Inquéritos e Questionários/normas
7.
Autism ; 15(2): 143-62, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21339248

RESUMO

The aim of this study was to examine the Autism Diagnostic Observation Schedule (ADOS) as it is commonly used in clinical practice. ADOS classifications were compared to final diagnoses given by a multidisciplinary team to 584 children referred for evaluation for possible autism spectrum disorder (ASD) at the Cincinnati Children's Hospital Medical Center. A total of 177 children were evaluated with a Module 1 (87 No Words), 198 with a Module 2 (90 < 5 years) and 209 with a Module 3. Of these, 142 (26%) were diagnosed with autism, 185 (32%) with non-autism ASD, and 257 (44%) with non-spectrum disorders. Sensitivities were moderate to high on both original and revised algorithms, while specificities were substantially lower than those previously reported. This difference is likely attributable to the composition of the sample that included many children with a broad array of developmental and behavioral disorders. The clinical impression of the team member who administered the ADOS was critical to the accuracy of the overall diagnosis. Using numeric scores alone resulted in misclassification from false positive results. The study highlights the importance of the qualitative interactions of the ADOS activities as well as the score in diagnostic decision making.


Assuntos
Transtorno Autístico/diagnóstico , Fatores Etários , Algoritmos , Síndrome de Asperger/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Escalas de Graduação Psiquiátrica , Sensibilidade e Especificidade , Fatores Sexuais
8.
Phys Occup Ther Pediatr ; 29(2): 203-18, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19401932

RESUMO

Speech-sound disorders are common in preschool-age children, and are characterized by difficulty in the planning and production of speech sounds and their combination into words and sentences. The objective of this study was to review and compare the results of the Sensory Profile (Dunn, 1999) in children with a specific type of speech-sound disorder, childhood apraxia of speech (CAS), and to explore the relationship between sensory processing and sound-production deficits. Participants were identified prospectively through an interdisciplinary apraxia clinic at a tertiary care pediatric hospital, and results of the Sensory Profile were compiled and reviewed. Thirty-eight children aged 3 to 10 years with suspected CAS were evaluated from July 2003 to July 2005. The results of the Sensory Profile indicated a difference for these children in several factor clusters when compared to typical peers from the normative population of the Sensory Profile. These findings imply that children with suspected CAS may present with differences in sensory processing in addition to speech impairment. When present, these differences in sensory processing could be addressed with specific therapeutic approaches through occupational therapy or consultation with an occupational therapist.


Assuntos
Apraxias/diagnóstico , Deficiências do Desenvolvimento/diagnóstico , Avaliação da Deficiência , Transtornos de Sensação/diagnóstico , Distúrbios da Fala/diagnóstico , Fonoterapia/métodos , Apraxias/complicações , Apraxias/terapia , Criança , Desenvolvimento Infantil , Pré-Escolar , Deficiências do Desenvolvimento/complicações , Deficiências do Desenvolvimento/terapia , Feminino , Humanos , Masculino , Transtornos de Sensação/complicações , Distúrbios da Fala/complicações , Distúrbios da Fala/terapia
9.
Clin Gastroenterol Hepatol ; 6(6): 621-9, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18434257

RESUMO

BACKGROUND & AIMS: Eosinophilic esophagitis (EE) occurs in families. METHODS: Record review confirmed patient kinship and provided clinical information. Slide review confirmed the diagnosis (threshold peak number > or = 24 eosinophils/high-power field). RESULTS: Fifty-nine members (41 males, 18 females) of 26 families were 3 months to 47 years of age (mean age, 10.3 y) at diagnosis. The only recorded race was Caucasian. In 4 families a parent of an affected male had EE. The most common complaint at diagnosis was dysphagia (68% of patients). Endoscopy showed esophageal mucosal furrows (93% of patients) and exudates (44%). Fifty-one percent had asthma. Skin prick tests to food and aeroallergens were positive in 76% and 71%, respectively. Familial EE characteristics (clinical, endoscopic, pathologic, and global esophageal transcript expression profile analysis) were similar to sporadic EE, except among patients with mucosal furrows: familial patients had lower peak eosinophil counts in the distal esophagus (P = .03) compared with sporadic patients. The basic characteristics of EE (eg, eosinophil levels, rate of atopy) did not vary with patient age. By using genome-wide microarray analysis, no significant differences (P < .05, false-discovery rate) were observed between familial and sporadic EE. Among all patients, chest pain was more common in females (P = .02), and thickened mucosa was more common in males (P = .006). CONCLUSIONS: These data support a familial pattern of inheritance of EE and a pathogenesis shared with sporadic EE. EE should be considered in symptomatic family members of patients who have EE.


Assuntos
Eosinófilos/imunologia , Esofagite/patologia , Esofagite/fisiopatologia , Saúde da Família , Adolescente , Adulto , Asma , Criança , Pré-Escolar , Transtornos de Deglutição/etiologia , Esofagite/genética , Esofagite/imunologia , Esofagoscopia , Feminino , Perfilação da Expressão Gênica , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Mucosa/patologia , Análise de Sequência com Séries de Oligonucleotídeos , População Branca
11.
J Pediatr Gastroenterol Nutr ; 45(1): 22-31, 2007 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-17592361

RESUMO

BACKGROUND: Pediatric eosinophilic esophagitis (EE) is a recently described disorder associated with atopy. Although studies of esophageal tissue suggest that Th2 cytokines and eotaxin-3 may be crucial in disease pathogenesis, little is known about the systemic immunological phenotypes of children with EE. OBJECTIVES: To define the phenotypes of peripheral blood eosinophils and lymphocytes in EE and to examine for correlations between these parameters and tissue eosinophil numbers and disease severity. PATIENTS AND METHODS: Blood was collected from children with EE, atopic control children without EE, and nonatopic control children without EE. Flow cytometry was used to measure eosinophil expression of chemokine receptor 3 (CCR3) and interleukin-5 receptor-alpha (IL-5Ralpha), and intracellular lymphocyte expression of IL-4, IL-5, IL-13, interferon-gamma, and tumor necrosis factor-alpha. Eosinophil numbers and eotaxin-3 mRNA levels were quantitated in esophageal biopsy specimens. RESULTS: Compared with nonatopic control children, EE patients with active disease had increased peripheral blood eosinophil percentages, mean channel of fluorescence (MCF) of CCR3 on eosinophils, and percentage of CD4+ T cells expressing IL-5. Notably, these parameters positively correlated with esophageal eosinophil numbers. Eotaxin-3 tissue expression positively correlated with esophageal eosinophil numbers and peripheral blood eosinophil CCR3 MCF. The percentage of peripheral blood eosinophils, eosinophil CCR3 MCF, and CD4+ T cell expression of IL-5 were lower in EE patients in disease remission than in patients with active disease. CONCLUSIONS: Collectively, these studies demonstrate cooperation between systemic CD4+ Th2-cell-mediated immunity and an enhanced eosinophil-CCR3/eotaxin-3 pathway in EE pathogenesis. Furthermore, the imbalanced Th2 immunity and increased CCR3 expression are reversible with disease remission.


Assuntos
Quimiocinas CC/imunologia , Eosinofilia/imunologia , Eosinófilos/metabolismo , Esofagite/imunologia , Receptores de Quimiocinas/imunologia , Células Th2/imunologia , Antígenos de Superfície , Quimiocina CCL26 , Quimiocinas CC/biossíntese , Criança , Citocinas/biossíntese , Eosinófilos/imunologia , Humanos , Hipersensibilidade Imediata/imunologia , Imunidade Celular , RNA Mensageiro/biossíntese , Receptores CCR3 , Receptores de Quimiocinas/biossíntese
12.
Clin Pediatr (Phila) ; 46(7): 604-11, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17522288

RESUMO

Preschool-aged children with speech-sound disorders may be at risk for associated deficits in fine motor function. The objectives of this study were 2-fold: (1) to determine whether abnormalities in fine motor function could be detected in 2- to 5-year-old children with speech-sound disorders and (2) to determine whether there was a correlation between abnormal oral-motor imitation skills and abnormal fine motor function. Thirty-two children with speech-sound disorders (6 female, 26 male) were prospectively evaluated from July 2003 to July 2005, and the Peabody Developmental Motor Scales and the Kaufman Speech Praxis Test for Children were administered. The presence of abnormal oral-motor imitation skills as measured by the Kaufman Speech Praxis Test was associated with below-average fine motor performance. This finding has important implications for evaluation and treatment of preschool children with severe speech-sound disorders.


Assuntos
Transtornos da Articulação/fisiopatologia , Comportamento Imitativo/fisiologia , Destreza Motora/fisiologia , Fala/fisiologia , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos
13.
Am J Epidemiol ; 166(1): 88-96, 2007 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-17420182

RESUMO

In this 2000-2001 study, the authors compared the effectiveness of the male latex condom and the female polyurethane condom by assessing frequency and types of mechanical failure and by evaluating semen exposure during use. Eligible women from Birmingham, Alabama, were randomly assigned to begin the study with 10 male condoms and then switch to 10 female condoms (n = 55), or vice versa (n = 53), and were trained to use both types. Data collection included questionnaires for each condom use and measurement of prostate-specific antigen in specimens of vaginal fluid taken before and after intercourse. Participants returned 700 male condoms and 678 female condoms, and they reported mechanical problems for 9% and 34%, respectively. Moderate-high postcoital prostate-specific antigen levels (> or = 22 ng/ml) were detected in 3.5% of male condom uses and 4.5% of female condom uses (difference = 1%, 95% confidence interval: -1.6, 3.7). Moderate-high prostate-specific antigen values (> or = 22 ng/ml) were more frequent with mechanical problems (male condom, 9.6%; female condom, 9.4%) but less frequent with other problems (3.0% and 0.9%) or correct use with no problems (2.7% and 2.5%). This study indicates that although mechanical problems are more common with the female condom than with the male condom, these devices may involve a similar risk of semen exposure. Objectively assessed semen exposure is associated with self-reported mechanical problems.


Assuntos
Coito , Preservativos Femininos , Preservativos , Antígeno Prostático Específico/isolamento & purificação , Sêmen , Adulto , Método de Barreira Anticoncepção , Falha de Equipamento , Feminino , Humanos , Látex , Masculino , Pessoa de Meia-Idade , Poliuretanos , Inquéritos e Questionários
14.
J Allergy Clin Immunol ; 119(3): 731-8, 2007 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-17258309

RESUMO

BACKGROUND: Eosinophilic esophagitis (EE) is a gastrointestinal disorder that is increasingly diagnosed in pediatric patients. OBJECTIVE: We aimed to define, in pediatric patients with EE, their demographic and atopic characteristics, the histopathology of all segments of the gastrointestinal tract, and the effect of therapeutic interventions on the natural history. METHODS: We conducted a retrospective analysis of a database of pediatric patients with EE followed over a period of 8 years. RESULTS: In 89 pediatric patients with EE, male sex (78.6%), white race (94.4%), young age at diagnosis, mean +/- SD, 6.2 +/- 4.8 years, and atopy with sensitization to environmental and food allergens in 79% and 75%, respectively, were prevalent. Patients had EE of the proximal and distal esophagus, and 77% had in addition either mucosal eosinophilia or noneosinophilic histopathology in the stomach, duodenum, and colon. EE was chronic, with a duration of mean +/- SD, 0.91 +/- 0.84 years, until first resolution, and was recurrent; of 66% of the patients who had resolution, 79% later relapsed. CONCLUSION: Eosinophilic esophagitis in the pediatric population is a chronic and relapsing condition, associated with atopy and sometimes with subsequent histopathology in segments of the gastrointestinal tract other than the esophagus. CLINICAL IMPLICATIONS: Physicians evaluating pediatric patients with chronic gastrointestinal symptoms should consider the diagnosis of EE, particularly in young white male patients with atopy. Once diagnosed and treated, the physicians should follow the patients over a period of several years because the course of the disease is protracted, other gastrointestinal segments may be affected, and relapses are common.


Assuntos
Eosinofilia/diagnóstico , Esofagite/diagnóstico , Hipersensibilidade Imediata/diagnóstico , Adolescente , Criança , Pré-Escolar , Colo/patologia , Duodeno/patologia , Endoscopia , Esofagite/imunologia , Esofagite/terapia , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Estômago/patologia
15.
Gastroenterology ; 131(5): 1381-91, 2006 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17101314

RESUMO

BACKGROUND & AIMS: Eosinophilic esophagitis is an increasingly recognized disorder with distinctive endoscopic, histologic, and allergic features. Although several therapies are advocated, no placebo-controlled trials have been conducted. We aimed to determine the efficacy of swallowed fluticasone propionate (FP) in the treatment of eosinophilic esophagitis. METHODS: We conducted a randomized, double-blind, placebo-controlled trial of swallowed FP in pediatric patients with active eosinophilic esophagitis. Thirty-six patients were randomly assigned to receive either 880 mug of FP (21 patients) or placebo (15 patients) divided twice daily for 3 months. The primary end point was histologic remission, defined by a peak eosinophil count of

Assuntos
Androstadienos/uso terapêutico , Eosinofilia/tratamento farmacológico , Esofagite/tratamento farmacológico , Adolescente , Fatores Etários , Estatura , Peso Corporal , Linfócitos T CD8-Positivos/imunologia , Criança , Pré-Escolar , Método Duplo-Cego , Eosinofilia/imunologia , Eosinofilia/patologia , Esofagite/imunologia , Esofagite/patologia , Feminino , Fluticasona , Humanos , Hiperplasia , Lactente , Masculino
16.
Arch Otolaryngol Head Neck Surg ; 132(4): 432-6, 2006 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-16618913

RESUMO

OBJECTIVES: To determine the incidence of obstructive sleep apnea syndrome in children aged 2 to 4 years with Down syndrome and to determine parents' ability to predict sleep abnormalities in this patient population. DESIGN: Prospective cohort study. SETTING: Tertiary care pediatric referral center. PATIENTS: Sixty-five children participating in a 5-year longitudinal study in which the otolaryngologic problems seen in Down syndrome were evaluated. Fifty-six completed overnight polysomnography (PSG) between 4 and 63 months of age (mean age, 42 months). INTERVENTIONS: Overnight PSG was performed. Parents also completed a questionnaire regarding their impressions of their child's sleep patterns before PSG. MAIN OUTCOME MEASURES: Polysomnograms were classified as abnormal if the obstructive index was greater than 1, if the carbon dioxide level was greater than 45 mm Hg for more than two thirds of the study or greater than 50 mm Hg for more than 10% of the study, and/or if there was unexpected hypoxemia less than 92% during sleep or repeated intermittent desaturations less than 90%. We also identified a group of children whose PSGs findings were normal except for an arousal index greater than 10 and were associated with increased work of breathing. RESULTS: The PSGs revealed that 57% of the children had abnormal results and evidence of obstructive sleep apnea syndrome. If we also include an elevated arousal index, 80% of the PSGs had abnormal results. Sixty-nine percent of parents reported no sleep problems in their children, but in this group, 54% of PSGs had abnormal results. Of the parents who reported sleep problems in their children, only 36% had abnormal sleep study results. CONCLUSION: Because of the high incidence of obstructive sleep apnea syndrome in young children with Down syndrome, and the poor correlation between parental impressions of sleep problems and PSG results, baseline PSG is recommended in all children with Down syndrome at age 3 to 4 years.


Assuntos
Síndrome de Down/complicações , Programas de Rastreamento , Apneia Obstrutiva do Sono/epidemiologia , Apneia Obstrutiva do Sono/prevenção & controle , Pré-Escolar , Humanos , Incidência , Lactente , Ohio/epidemiologia , Pais , Polissonografia , Estudos Prospectivos
17.
J Clin Invest ; 116(2): 536-47, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16453027

RESUMO

Eosinophilic esophagitis (EE) is an emerging disorder with a poorly understood pathogenesis. In order to define disease mechanisms, we took an empirical approach analyzing esophageal tissue by a genome-wide microarray expression analysis. EE patients had a striking transcript signature involving 1% of the human genome that was remarkably conserved across sex, age, and allergic status and was distinct from that associated with non-EE chronic esophagitis. Notably, the gene encoding the eosinophil-specific chemoattractant eotaxin-3 (also known as CCL26) was the most highly induced gene in EE patients compared with its expression level in healthy individuals. Esophageal eotaxin-3 mRNA and protein levels strongly correlated with tissue eosinophilia and mastocytosis. Furthermore, a single-nucleotide polymorphism in the human eotaxin-3 gene was associated with disease susceptibility. Finally, mice deficient in the eotaxin receptor (also known as CCR3) were protected from experimental EE. These results implicate eotaxin-3 as a critical effector molecule for EE and provide insight into disease pathogenesis.


Assuntos
Quimiocinas CC/metabolismo , Eosinofilia/genética , Esofagite/genética , Perfilação da Expressão Gênica , Animais , Biópsia , Quimiocina CCL26 , Quimiocinas CC/genética , Criança , Eosinofilia/metabolismo , Eosinofilia/patologia , Esofagite/metabolismo , Esofagite/patologia , Feminino , Frequência do Gene , Genótipo , Humanos , Masculino , Mastocitose/genética , Mastocitose/metabolismo , Análise de Sequência com Séries de Oligonucleotídeos , Polimorfismo de Nucleotídeo Único
18.
J Hum Lact ; 21(1): 59-66, 2005 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-15681638

RESUMO

Although there are well-established clinical human milk banks in the United States, there are no milk banks specifically intended to foster research on human milk. The authors' goal was to establish a milk bank with a core data set to support exploratory and hypothesis-driven studies on human milk. Donations to the Cincinnati Children's Research Human Milk Bank are accepted within the context of ongoing, hypothesis-driven research or on an ad hoc basis. Donors must give informed consent, and scientists wishing to use the samples must have Institutional review board approval for their use. Development of more research human milk banks can potentially provide resources for multidisciplinary collaboration and advance the study of human milk and lactation.


Assuntos
Bancos de Leite Humano/estatística & dados numéricos , Leite Humano/química , Leite Humano/fisiologia , Pesquisa , Adulto , Comitês de Ética em Pesquisa , Feminino , Humanos , Lactente , Recém-Nascido , Consentimento Livre e Esclarecido , Estados Unidos
19.
J Pediatr Surg ; 38(6): 916-8, 2003 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-12778393

RESUMO

BACKGROUND: This study was conducted to determine the ability of 2 questionnaires (ie, child and parent versions) to measure physical and psychosocial quality-of-life changes after surgical repair of pectus excavatum. METHODS: The authors administered these questionnaires by telephone interviews with 22 parents and 19 children (ages 8 to 18) before surgery and 6 to 12 months after repair by the Nuss procedure. RESULTS: The instruments had high test-retest reliability (Rho > 0.6 for all retained questions). Children reported significant improvements in exercise intolerance, shortness of breath, and tiredness. Of 9 questions asking the children how they feel or act about their bodies, all but one question showed significant improvement after surgery. Parents also reported significant improvements in their child's exercise tolerance, chest pain, shortness of breath, and tiredness and decreases in the frequency of the child being frustrated, sad, self-conscious, and isolated. CONCLUSIONS: These questionnaires appear to be more than adequate to measure disease-specific quality-of-life changes after surgery. These data confirm for the first time that surgical repair of pectus excavatum has a positive impact on both the physical and psychosocial well-being of the child.


Assuntos
Tórax em Funil/cirurgia , Qualidade de Vida , Adolescente , Adulto , Criança , Humanos , Pais , Satisfação do Paciente , Projetos Piloto , Procedimentos de Cirurgia Plástica/métodos , Inquéritos e Questionários , Procedimentos Cirúrgicos Torácicos/métodos
20.
Pediatrics ; 109(5): 740-4, 2002 May.
Artigo em Inglês | MEDLINE | ID: mdl-11986430

RESUMO

OBJECTIVE: To determine 1) the electronic mail (e-mail) capabilities of families, general pediatricians (GPs), and subspecialty pediatricians (SPs) from an integrated pediatric health care delivery system and 2) the knowledge base and attitudes of these groups regarding the potential issues involved in using e-mail for physician-patient communication. METHODS: Parents were interviewed in the offices of participating practices using a standardized survey tool. Pediatricians and staff were interviewed using a separate instrument. The data were entered into a database for analysis. RESULTS: A total of 325 parents and 37 physicians were interviewed. Fifty-seven percent of the 161 parents who were interviewed at the GP offices and 66% of the 164 families that were interviewed at SP offices had access to e-mail. Parents aged 31 to 40 years were significantly more likely to have access to e-mail than parents of other age groups. Access to e-mail increased with family income and parental education. Most (74%) parents who were interviewed expressed interest in using e-mail to contact their child's physician/physician's office for several purposes, including getting information or test results, scheduling appointments, and/or discussing a particular symptom. Although both groups of parents expressed concerns about confidentiality, parents at the GP offices were significantly more concerned (median(GP) = 95 vs median(SP) = 70). Seventy-four percent of GPs and 100% of SPs had access to e-mail; however, 79% did not want to use e-mail for physician-patient communication, citing concerns about confidentiality and the time demands that patient e-mail might engender. CONCLUSIONS: The majority of parents and pediatricians at both general and subspecialty pediatric offices are capable of communicating electronically. Parents and pediatricians are aware of the issues surrounding e-mail use for patient communication. Most parents express an interest in using e-mail for patient-physician communications, whereas most physicians are opposed to this practice.


Assuntos
Atitude do Pessoal de Saúde , Atitude Frente a Saúde , Redes de Comunicação de Computadores , Pais/psicologia , Pediatria/organização & administração , Relações Médico-Paciente , Prática Profissional/organização & administração , Adolescente , Adulto , Fatores Etários , Comunicação , Confidencialidade , Escolaridade , Feminino , Humanos , Renda , Masculino , Pessoa de Meia-Idade , Relações Profissional-Família
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