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OBJECTIVE: The aim of the study is to investigate factors affecting total sleep time (TST) during infant polysomnography (PSG) and assess if <4 hours of TST is sufficient for accurate interpretation. STUDY DESIGN: Overall, 242 PSGs performed in 194 infants <6 months of chronological age between March 2013 and December 2015 were reviewed to identify factors that affect TST, including age of infant, location and timing of study, presence of medical complexity, and presence of nasal tubes. A continuum of apnea-hypopnea index (AHI) in relation to TST was reviewed. Data were examined in infants who had TST <4 hours and low AHI. RESULTS: Greater TST (p < 0.001) was noted among infants during nocturnal PSGs, at older chronological and post-menstrual ages, and without medical complexity. The presence of nasogastric/impedance probes reduced TST (p = 0.002). Elevated AHIs were identified even in PSGs with TST <4 hours. Short TST may have affected interpretation and delayed initial management in one infant without any inadvertent complications. CONCLUSION: Clinical factors such as PMA and medical complexity, and potentially modifiable factors such as time of day and location of study appeared to affect TST during infant PSGs. TST < 4 hours can be sufficient to identify high AHI allowing physician interpretation. KEY POINTS: · Less than 4 hours of TST is enough for interpretation of infant polysomnography.. · Shorter TST appears related to infant age, medical complexity, and higher apnea-hypopnea index.. · Modifiable factors seen with higher TST were time of day, environment, and presence of nasal tubes..
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Apneia , Sono , Humanos , Lactente , PolissonografiaAssuntos
Regulador de Condutância Transmembrana em Fibrose Cística/efeitos dos fármacos , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/tratamento farmacológico , Combinação de Medicamentos , Regulação da Expressão Gênica/efeitos dos fármacos , Mutação/efeitos dos fármacos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aminofenóis/uso terapêutico , Benzodioxóis/uso terapêutico , Criança , Feminino , Humanos , Indóis/uso terapêutico , Masculino , Pessoa de Meia-Idade , Pirazóis/uso terapêutico , Piridinas/uso terapêutico , Pirrolidinas/uso terapêutico , Quinolonas/uso terapêutico , Adulto JovemRESUMO
Background Pre-lung transplant (LTx) panel reactive antibody (PRA) levels are associated with adverse outcomes in adult LTx recipients, but their impact in pediatric LTx recipients is unknown. Methods The United Network for Organ Sharing registry was queried from 2004 to 2013 to compare survival between pediatric LTx recipients with PRA class I and II levels = 0 versus > 0. Results Overall, 333 pediatric LTx recipients had data on class I or II PRA and were included in the analysis. Univariate analysis demonstrated that PRA > 0 was not associated with survival benefit for class I (hazard ratio [HR] = 0.985; 95% confidence interval [CI]: 0.623, 1.555; p = 0.947) or class II (HR = 1.080; 95% CI: 0.657, 1.774; p = 0.762) PRA. Multivariate Cox models confirmed no significant association with mortality hazard for both class I (HR = 1.230; 95% CI: 0.641, 2.363; p = 0.533) and class II (HR = 0.847; 95% CI: 0.359, 1.997; p = 0.704) PRA. Multivariate logistic regression models identified no association between class I or class II and acute rejection within 3 years of LTx. Conclusions Pretransplant class I and II PRA levels > 0 were not associated with mortality or acute rejection in pediatric LTx recipients.
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Teste de Histocompatibilidade , Histocompatibilidade , Isoanticorpos/sangue , Transplante de Pulmão , Doença Aguda , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto/imunologia , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Transplante de Pulmão/efeitos adversos , Transplante de Pulmão/mortalidade , Masculino , Análise Multivariada , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Obtenção de Tecidos e Órgãos , Resultado do Tratamento , Estados UnidosRESUMO
OBJECTIVE: To assess whether geographic location influences hospitalizations for pulmonary exacerbations for patients with cystic fibrosis (CF) in the US, as there is no existing literature regarding this subject. STUDY DESIGN: The CF Foundation Patient Registry was analyzed during the years 2007-2012 via geographic grouping of states. The impact of geographic region on recovery from hospitalization, hospitalization length, and time to next hospitalization were analyzed using multivariate models. RESULTS: Posthospitalization lung function and nutritional measures were similar among regions for 1 year following hospitalization. The West region was associated with risk of longer hospital stays (OR 1.60, CI 1.45-1.77), however, dornase alfa use (OR 3.85, CI 1.15-12.92) was the only specific factor. History of allergic bronchopulmonary aspergillosis (OR 1.58, CI 1.11-2.25) and adult age (OR 2.48, CI 1.17-5.25) in the Northeast, chronic macrolide use in the South (OR 1.36, CI 1.03-1.79), and infection with Candida albicans (OR 1.47, CI 1.18-1.82) and Pseudomonas aeruginosa (OR 1.44, CI 1.02-2.04) in the Midwest were associated with increased hospitalization length. There was a significantly decreased risk for subsequent hospitalizations in the Northeast compared with other regions (P = .038). Sociodemographic analysis identified Caucasians in the South having a significantly lower risk of future hospitalization compared with African Americans (hazard ratio 0.79, CI 0.69-0.91, P = .0009). CONCLUSIONS: There is significant regional variability in hospitalization length and risks for subsequent hospitalizations for patients with CF in the US. Regional variation should be subject to further study to determine if benchmarking standards can be achieved nationally.
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Fibrose Cística/terapia , Progressão da Doença , Hospitalização/estatística & dados numéricos , Adolescente , Adulto , Criança , Pré-Escolar , Fibrose Cística/fisiopatologia , Bases de Dados Factuais , Feminino , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Análise Multivariada , Modelos de Riscos Proporcionais , Estados Unidos , Adulto JovemRESUMO
Background: Emerging data reveal higher-than-expected prevalence of cystic fibrosis (CF) among non-European populations worldwide including in the Indian subcontinent. Systematic analyses of the CFTR mutation profile, and genotype-phenotype correlations among people with CF from south, east, or northeast India have not been reported before. We wanted to identify CFTR mutations in people with CF, and highlight novel variants, selective phenotypic correlations, and regional variances within India. Methods: A retrospective study was conducted at Christian Medical College, Vellore, India (single tertiary referral hospital) from September 2010 to August 2022, involving 120 people with CF from (i) four south Indian states (Tamil Nadu, Andhra Pradesh, Kerala, Karnataka), (ii) in and nearby regions of West Bengal, India and (iii) Bangladesh. Comprehensive CFTR mutation analyses were done by Next-Generation Sequencing, and variants were categorized per American College of Medical Genetics guidelines and compared with validated Locus-specific databases. Demographic characteristics, mutation profile, novel mutations, selective phenotype correlations, and regional variances were assessed. Findings: In 120 people with CF, 55 CFTR variants were identified, including six novel variants. F508del was the predominant mutation, yet with a lower allele frequency than reported among European populations (27% versus 70%). Phenotypic correlations suggested high mutational pathogenicity causing severe multi-organ morbidity, and death in 27%. Milder variants associated with pancreatic sufficiency were also evident in 23% of people with CF. Statistically significant regional variances were noted in genotype frequency, and clinical phenotype among people with CF from the two regions. Hotspot exons and introns that could potentially help create targeted mutation panels were identified. Interpretation: The identification of 55 different CFTR variants among 120 people with CF describes the diversity of mutations noted in India, while also revealing the challenges that providers may encounter in timely diagnosis and treatment of CF. However, these single-centre data have specific limitations and cannot be generalised to all people with CF from India or to those of non-European origin. Our data on regional CFTR mutations contribute to the emerging national registry on CF epidemiology in India, help formulate diagnostic and newborn screening algorithms, help optimise clinical care, and highlight urgency to improve access to life-changing modulator therapy. Funding: Cystic Fibrosis Foundation, USA (towards the CF-India Demonstration Project) and Christian Medical College, Vellore, India.
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Multiple strains of Mycobacteria cause tuberculosis (TB), a chronic, specific infectious granulomatous disease. It mainly occurs with pulmonary involvement when compared to extrapulmonary involvement. Primary oral occurrence is uncommon and oral lesions are usually secondary to pulmonary involvement. When there are no active pulmonary clinical manifestations of TB, the diagnosis of the very rare entity of primary gingival TB poses a great challenge to clinicians. In this case report, we discuss a case of primary gingival TB in a 24-year-old lactating mother. This article briefs the onset and course of the lesion during pregnancy and postpartum, elaborates the pathway to diagnosis, various investigations performed and the regimen of antitubercular therapy for 6 months, followed by complete resolution of the lesion without recurrence. This report also describes the significance of considering TB as a differential diagnosis in oral lesions and the various diagnostic methods available. It also emphasizes the sole importance of histopathology in the early detection of the lesion and its management.
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5q spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease caused by absence of the SMN1 gene with three FDA approved genetic therapies which significantly improve outcomes. The AAV9 mediated gene replacement therapy, onasemnogene abeparvovec, has the greatest potential for side effects. Here we report the safety and outcomes from 46 children treated with onasemnogene abeparvovec in the state of Ohio between December 2018 and January 2023. In our cohort, onasemnogene abeparvovec treatment remained safe and no child experienced any significant adverse events, including thrombotic microangiopathy, liver failure or death. All children experienced benefit, although the benefit in those with 2 copies of SMN2 was variable. 79 % of the children treated when symptomatic had a SMN2 modifying therapy added on. With careful screening and post treatment monitoring, onasemnogene abeparvovec is safe and effective for children with SMA in the state of Ohio, but more work needs to be done to ensure optimal outcomes for all children with 2 copies of SMN2.
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Produtos Biológicos , Atrofia Muscular Espinal , Doenças Neurodegenerativas , Proteínas Recombinantes de Fusão , Atrofias Musculares Espinais da Infância , Criança , Humanos , Ohio , Terapia GenéticaRESUMO
Vitamin D deficiency and asthma are common conditions that share risk factors such as African American ethnicity, inner-city residence, and obesity. This review provides a critical examination of current experimental and epidemiologic evidence of a causal association between vitamin D status and asthma or asthma morbidity, including potential protective mechanisms such as antiviral effects and enhanced steroid responsiveness. Because most published epidemiologic studies of vitamin D and asthma or asthma morbidity are observational, a recommendation for or against vitamin D supplementation as preventive or secondary treatment for asthma is not advisable and must await results of ongoing clinical trials. Should these trials confirm a beneficial effect of vitamin D, others will be needed to assess the role of vitamin D supplementation to prevent or treat asthma in different groups such as infants, children of school age, and ethnic minorities.
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Asma/epidemiologia , Deficiência de Vitamina D/epidemiologia , Asma/tratamento farmacológico , Asma/etiologia , Conservadores da Densidade Óssea/uso terapêutico , Medicina Baseada em Evidências , Humanos , Fatores de Risco , Resultado do Tratamento , Estados Unidos/epidemiologia , Vitamina D/uso terapêutico , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/tratamento farmacológicoRESUMO
BACKGROUND: Cystic Fibrosis (CF) and autism spectrum disorder (ASD) are life-long conditions with intense treatment burdens for patients and families. Patients with a concurrent diagnosis (CF-ASD) experience unique obstacles to CF care. This study describes the experiences of our multidisciplinary CF team in caring for patients with CF-ASD and provides insight into provider and parental perspectives on clinical management. METHODS: This is a three-part qualitative study involving (1) retrospective chart review of patients with CF-ASD, (2) surveys with multidisciplinary care team members, and (3) semistructured interviews with caregivers of patients with CF-ASD. Challenges in clinical management of this specific cohort were compiled using data from chart review and care team surveys. Strategies to address these concerns were identified and rated by individual families based on relevance and practicality. RESULTS: Within our CF center, 12 patients have an official diagnosis of ASD. Median age of patients with CF-ASD was 8.5 years (range 3-20 years), 67% were male, and 83% were on highly effective modulator therapy. Clinical barriers included sensory processing issues, environmental overstimulation, intolerance to procedures and to disrupted routines. Potentially impactful strategies include patient-specific coping plans, guided behavioral interventions, parental advocacy, and improved communication between the family and multidisciplinary team. CONCLUSION: Children with CF-ASD face extraordinary challenges beyond the experience of neurotypical children with CF. Increased awareness of this complex dual diagnosis will help providers be sensitive to the unique needs of these patients, help build consistent and trustworthy relationships with their families and deliver effective clinical care despite limitations.
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Transtorno do Espectro Autista , Fibrose Cística , Humanos , Criança , Masculino , Pré-Escolar , Adolescente , Adulto Jovem , Adulto , Feminino , Transtorno do Espectro Autista/terapia , Fibrose Cística/complicações , Fibrose Cística/terapia , Estudos Retrospectivos , Adaptação Psicológica , PaisRESUMO
Importance: Cystic fibrosis (CF) is a multiorgan genetic disease with progressive upper and lower airway involvement. The effects of CF transmembrane conductance regulator (CFTR) modifier therapies on CF-related upper airway disease, specifically chronic rhinosinusitis (CRS), are not characterized. Objective: To determine the outcome of elexacaftor-tezacaftor-ivacaftor (ETI) on CRS as measured by changes in sinus computed tomography (CT) metrics and on clinical parameters in individuals with CF. Design, Setting, and Participants: This prospective longitudinal cohort study was conducted at the CF center of a tertiary care hospital between October 1, 2019, and July 31, 2021. A total of 64 participants with CF were included in the analysis. Intervention: Sinus CT was obtained within 1 month of initiation of ETI therapy (baseline), and within 1 month of 1 year of ETI therapy. Images were independently analyzed by pulmonology, radiology, and otolaryngology physicians, using the Lund-Mackay and Sheikh-Lind scoring systems. Percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and microbiologic data collected at initiation of ETI therapy and 3-month intervals for 1 year were also measured. Main Outcomes and Measures: The study hypothesis was that ETI therapy will improve CRS as measured by changes in sinus CT at initiation and 1 year after ETI therapy and clinical parameters in individuals with CF. Results: Among the 64 participants (39 [60.9%] female; median age, 18.5 [IQR, 16.0-28.5] years; 64 [100%] White), improvement in CRS was noted by improvements in sinus CT scans using both sinus CT scoring systems after 1 year of ETI therapy. The reduction in the median total score using the Lund-Mackay sinus CT scoring system (from 5.8 [IQR, 5.0-7.0] to 3.3 [IQR, 2.6-4.2]) and the Sheikh-Lind scoring system (from 3.8 [IQR, 3.0-5.0] to 2.2 [IQR, 2.0-2.5]) was noted. Increases in ppFEV1 and BMI were also observed by 3 months of ETI therapy with persistent improvement through 1 year of treatment. Similarly, after 1 year of ETI therapy, participants with CF had reductions in positivity for Pseudomonas aeruginosa and Staphylococcus aureus in oropharyngeal cultures. Conclusion and Relevance: This cohort study found that use of ETI therapy was associated with improved CRS outcomes in participants with CF as quantified by improved sinus CT scans measured by 2 radiographic scoring systems and was also associated with improved clinical outcomes. Despite improvement in CT scan scores, most people with CF continue to have scores that indicate severe sinus disease.
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Fibrose Cística , Feminino , Humanos , Adolescente , Masculino , Fibrose Cística/tratamento farmacológico , Estudos de Coortes , Estudos Longitudinais , Estudos Prospectivos , Regulador de Condutância Transmembrana em Fibrose Cística/genética , MutaçãoRESUMO
Technology advancement, industrialisation, and globalisation have been significant reasons for air pollution outdoors and indoors. It may surprise us that we spend about 80% of our time indoors breathing toxic, stale, polluted air, making us sluggish and fretful. In contrast to outdoor air, indoor air does not recycle consistently. It traps and builds pollutants from wood and coal stoves, furniture and building materials, paints and solvents, cigarette smoke, and cleaning supplies. The prolonged exposure to these hidden pollutants can prompt respiratory disorders such as lung disease, pneumonitis, asthma, pulmonary hypertension, and chronic obstructive pulmonary disease (COPD). It is enduring and is not curable, which has been a threat to humanity for ages. COPD's major cause is airborne particulate matter and other toxic compounds emitted from indoor and outdoor sources. Outdoor air pollution can be controlled only by acquiring changes in the vast population, wherein for indoor, every individual may create a major impact on improving air purity, thereby promoting health. The proposed design model for monitoring indoor air quality was tested in a normal and stimulating environment where we live. Parameters tested included temperature, humidity, amount of PM2.5, and the concentration of CO, CO2, and NH3. These parameters were monitored for five to 6 h per day for 8 days. Results indicate that the total air quality lies in the moderate range. Further study will be helpful to utilise this module as an effective Indoor air quality (IAQ) monitoring system. HIGHLIGHTS: ⢠A simple, effective, inexpensive integrated gas sensor module (IGSM) has been proposed in this study to monitor the indoor air quality index (IAQI). ⢠Indoor air quality was tested in a normal and stimulating environment for 8 days. ⢠The integrated gas sensor module (IGSM) was composed of sensors in series, and the outputs for the respective parameter were measured easily. ⢠Among the parameters tested, CO and PM2.5 lie in the moderate range, while other pollutants within the normal range reveal that the tested air quality is moderate.
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Poluentes Atmosféricos , Poluição do Ar em Ambientes Fechados , Doença Pulmonar Obstrutiva Crônica , Humanos , Poluição do Ar em Ambientes Fechados/análise , Poluentes Atmosféricos/análise , Monitoramento Ambiental/métodos , Material Particulado/análise , GasesRESUMO
PURPOSE: Little progress has been made in understanding the effect of Trypanosoma brucei brucei infection that was allowed to run its course without treatment on human and animal carbohydrate metabolism even though most of the symptoms associated with the disease can be clearly linked with interference with host energy generation. The present study therefore assessed the course of untreated Trypanosoma brucei brucei infection on hepatic glycogen, hepatic hexokinase and glucokinase activities. METHODS: Mice were grouped into two: control and infected group. Trypanosomiasis was induced by intraperitoneal inoculation of 1 × 104 parasites/mice in 0.3 ml of phosphate saline glucose. The infection was allowed to run its course until the first mortality was recorded with all the mice showing chronic symptoms of the second stage of the disease before the research was terminated. Blood and liver samples were collected from the mice in each group for the assessment of hepatic glycogen and total protein, hepatic hexokinase and glucokinase activities, liver biomarkers, blood glucose and protein with packed cell volume. RESULTS: The infection resulted in decrease in blood glucose, hepatic glycogen, liver protein, PCV, hepatic hexokinase and glucokinase activities, but increase in serum total protein and liver biomarkers. CONCLUSION: Trypanosomiasis negatively affects hepatic integrity, resulting in the depletion of hepatic glycogen content and suppression of both hepatic hexokinase and glucokinase activities. The suppression of hepatic hexokinase and glucokinase activities suggested that trypanosomiasis affected the oxidation of glucose and host energy generation via glycolysis. This probably denied the host of the needed energy which is likely the reason for early death in untreated African trypanosomiasis.
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Hipoglicemia , Tripanossomíase , Animais , Glicemia/metabolismo , Metabolismo dos Carboidratos , Glucoquinase/metabolismo , Glucose/metabolismo , Hexoquinase/metabolismo , Hipoglicemia/induzido quimicamente , Hipoglicemia/metabolismo , Fígado/enzimologia , Fígado/metabolismo , Glicogênio Hepático/metabolismo , Camundongos , Trypanosoma brucei brucei , Tripanossomíase/metabolismoRESUMO
BACKGROUND: Reactive oxygen metabolites (ROMs) produced in periodontitis could contribute to excessive tissue damage. Thus, treatment of chronic periodontitis may decrease the ROM levels. The aim of this study is to evaluate the ROM levels in plasma, saliva, and gingival crevicular fluid (GCF) in generalized chronic periodontitis (GCP) patients before and after nonsurgical periodontal treatment. MATERIALS AND METHODS: Two groups were included in this study. Group I consisted of 30 healthy controls (C) and Group II consisted of 30 subjects with GCP. Plaque index (PI), papillary bleeding index, Probing Depth (PD), and clinical attachment level were recorded. GCF, saliva, and plasma samples were collected from both groups. ROM levels were assessed. A baseline comparison was made between the two groups. Nonsurgical periodontal treatment was carried out for Group II subjects. Two months posttreatment, the clinical parameters and ROM levels in GCF, saliva, and plasma were reassessed in Group II, and the data were compared with their baseline values. Statistical analysis was done using SPSS 20 software and results were derived. RESULTS: Two months posttreatment, Group II exhibited significant reduction in ROM levels in plasma, saliva, and GCF with significant decrease in PI, bleeding on probing, probing depth, and attachment loss. CONCLUSION: Thus, significant oxidative stress may occur in chronic periodontitis and nonsurgical periodontal therapy may be regarded as an effective treatment modality to treat the diseased periodontium, thereby preventing possible systemic diseases in future.
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BACKGROUND: Early diagnosis via newborn screening is crucial to improve clinical outcomes in patients with cystic fibrosis (CF). In resource-limited areas where newborn screening is unavailable and CF-related morbidity is high, clinical tools such as palmar aquagenic wrinkling (AW) have been considered. We report the utility of AW for possible early identification of CF in children <2 years old. METHODS: This pilot case-control study included 55 total children, 20 with confirmed CF, 10 CF carriers, and 25 healthy controls. The time to wrinkling (TTW) after hand immersion in water was recorded, and relationships between TTW, demographic and clinical variables, and validated diagnostic tests were analyzed. RESULTS: Wrinkling was observed in children <2 years of age, and median TTW was significantly lower among those with CF (3 min) compared to carriers or healthy controls (12 and 14 min, respectively). Higher immunoreactive trypsinogen and sweat chloride levels were associated with lower TTW (p < 0.001). In this predominantly Caucasian cohort, children with F508del had the lowest TTW. Six minutes of hand immersion offered a sensitivity of 85% and a specificity of 91%, suggesting a practical and effective test duration for this age. There was no evidence that nutritional status affected TTW. CONCLUSION: Our data confirm the role of AW in CF, validate test utility among young children, and analyze relationships between TTW, immunoreactive trypsinogen, sweat chloride levels, and CF-causing mutations. Despite test limitations, in children with suspected CF from non-screened populations, utility of AW in enabling early referral and diagnosis needs further exploration.
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Fibrose Cística/diagnóstico , Dermatoses da Mão/etiologia , Programas de Rastreamento/métodos , Água , Feminino , Humanos , Imersão , Lactente , Recém-Nascido , Masculino , Encaminhamento e Consulta , Suor/química , Fatores de TempoRESUMO
The phenotype of spinal muscular atrophy (SMA) has been changing with the recent availability of three FDA-approved treatments: intrathecal nusinersen, intravenous onasemnogene abeparvovec-xioi, and enteral risdiplam. The degree of improvement in muscle strength and respiratory health varies with SMA genotype, severity of baseline neuromuscular and pulmonary impairment, medication used, and timing of the first dose. A spectrum of pulmonary outcomes has been reported with these novel medications when used early and in conjunction with proactive multidisciplinary management of comorbidities. In this review, we summarize the reported impact of these novel therapies on pulmonary well-being and the improving trajectory of pulmonary morbidity, compared to the natural history of SMA. The importance of ongoing clinical monitoring albeit the improved phenotype is reiterated. We also discuss the limitations of the current SMA-therapy trials and offer suggestions for future clinical-outcome studies and long-term monitoring.
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Atrofia Muscular Espinal , Produtos Biológicos , Terapia Genética , Genótipo , Humanos , Atrofia Muscular Espinal/tratamento farmacológico , Fenótipo , Proteínas Recombinantes de Fusão , Atrofias Musculares Espinais da Infância/terapiaRESUMO
BACKGROUND: Asthma control in African Americans (AA) is considered more difficult to achieve than in Caucasian Americans (CA). The aim of this study was to compare asthma control over time among AA and CA children whose asthma is managed per NAEPP (EPR-3) guidelines. METHODS: This was a one-year prospective study of children referred by their primary care physicians for better asthma care in a specialty asthma clinic. All children received asthma care per NAEPP guidelines. Results were compared between CA and AA children at baseline and then at three-month intervals for one year. RESULTS: Of the 345 children, ages 2-17 years (mean = 6.2 ± 4), 220 (63.8%) were CA and 125 (36.2%) were AA. There were no significant differences in demographics other than greater pet ownership in CA families. At baseline, AA children had significantly more visits to the Emergency Department for acute asthma symptoms (mean = 2.3 [Formula: see text] compared to CA (1.4 ± 2.3, P = 0.003). There were no other significant differences in acute care utilization, asthma symptoms (mean days/month), or mean asthma control test (ACT) scores at baseline. Within 3-6 months, in both groups, mean ACT scores, asthma symptoms and acute care utilization significantly improved (P < 0.05 for all) and change over time in both groups was comparable except for a significantly greater decrease in ED visits in AA children compared to CA children (P = 002). CONCLUSION: Overall, improvement in asthma control during longitudinal assessment was similar between AA and CA children because of consistent use of NAEPP asthma care guidelines.
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Asma , Fidelidade a Diretrizes , Adolescente , Negro ou Afro-Americano , Asma/diagnóstico , Asma/prevenção & controle , Criança , Pré-Escolar , Serviço Hospitalar de Emergência , Humanos , Estudos ProspectivosRESUMO
CASE PRESENTATION: A 17-year-old male patient who was diagnosed with Becker muscular dystrophy (nonsense mutation [c.3822C>A] within exon 28 of the DMD gene) at 6 years of age was evaluated in the multidisciplinary neuromuscular clinic for loss of ambulation for 1 year. From a pulmonary perspective, there were no acute or chronic respiratory symptoms, and no history of pneumonia or aspiration. Clinical examination revealed a nonambulant teenager, with normal oxygen saturation and end-tidal CO2 when awake, no respiratory distress, and symmetrically diminished aeration due to obesity (BMI 40 kg/m2). Results of pulmonary function testing revealed FVC of 83% predicted with actual volume of 3.5 L and peak cough flow of 445 L/min (all within normal limits).
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Distrofia Muscular de Duchenne/complicações , Apneia do Sono Tipo Central/diagnóstico , Apneia do Sono Tipo Central/etiologia , Adolescente , Humanos , Masculino , PolissonografiaRESUMO
BACKGROUND: Mycobacterium porcinum is a non-tuberculous mycobacterium (NTM) identified in potable water. The identification and clinical impact of M. porcinum in patients with cystic fibrosis (CF) has not been described. In our institution, M. porcinum was isolated exclusively during hospitalization in a cluster of patients with CF. METHODS: Patients with CF who were hospitalized between September 2016 and September 2018 and could expectorate sputum were included, and samples were processed per institutional guidelines. Post-hospitalization and one-year clinical outcomes on those who isolated M. porcinum in respiratory cultures were reviewed. Whole genome sequencing was performed on M. porcinum isolates obtained from patients and environmental sources to identify source of acquisition. RESULTS: Review of 14 CF patients with 16 M. porcinum isolates revealed rapid time to culture positivity within 0.8 (0.04-8.0) days after admission. M. porcinum was isolated in teenagers and adults irrespective of baseline pulmonary function, body mass index, or CF genotype. Whole genome sequencing suggested all isolates belong to the same M. porcinum strain and confirmed the source of acquisition to the ice machine. Review of patients' clinical course, including three patients who underwent lung transplantation, suggested a pseudo-outbreak with minimal clinical impact. CONCLUSIONS: NTM, including M. porcinum, are ubiquitous in potable water and institutional water reservoirs. Our findings suggest M. porcinum is a transient colonizer rather than a pathogen. Challenges exist in discerning the role of NTM as a contributor of pulmonary morbidity in patients with CF, and adherence to established guidelines regarding NTM related pulmonary disease remains important.
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Fibrose Cística , Contaminação de Equipamentos/prevenção & controle , Equipamentos e Provisões Hospitalares/microbiologia , Mycobacteriaceae , Infecções por Mycobacterium não Tuberculosas , Adolescente , Adulto , Fibrose Cística/epidemiologia , Fibrose Cística/genética , Fibrose Cística/microbiologia , Fibrose Cística/fisiopatologia , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Mycobacteriaceae/genética , Mycobacteriaceae/isolamento & purificação , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Infecções por Mycobacterium não Tuberculosas/microbiologia , Infecções por Mycobacterium não Tuberculosas/prevenção & controle , Testes de Função Respiratória/métodos , Escarro/microbiologia , Estados Unidos/epidemiologia , Sequenciamento Completo do Genoma/métodosRESUMO
BACKGROUND: Glucose intolerance is observed more in patients with HCV infection compared with control subjects with liver disease, Initial studies suggested that Hepatitis C virus infection may be an additional risk factor for the development of diabetes mellitus. This study was therefore carried out to determine the correlation of HCV infection and diabetes. METHODS: Three hundred (300) confirmed type 2 diabetic patients were screened for hepatitis C virus antibodies at the Plateau state specialist hospital, Jos, using Grand diagnostic test strip. Questionnaire comprising of age, sex, family history on diabetes, duration of disease and marital status were issued to subjects. RESULTS: Overall result showed that the prevalence rate of HCV infection was 33(11%). In response to diabetic status, females subjects had a higher prevalence of 178(59.3%) compared to males 122(40.7%). Those aged 47-57 recorded the highest seroprevalence 10(30.3%) to the Hepatitis C Virus, while Patients without family history of diabetes showed a higher seroprevalence of 13(39.4%). Subjects who never had any blood transfusion recorded a prevalence rate of 6(18.2%). Marital status showed no significant difference [(P = 0.275; P.0.05)]. Considering duration of developing diabetes, patients within the range of 1-10 years diabetic status recorded the highest prevalence rate 25(75.8%) compared to other ranges considered. CONCLUSION: This study hence, suggests a relatively strong association between HCV infection and diabetes, this therefore call for an urgent approach strategy in the control and management of this disease of the endocrine system.