1 Childrens Pl Ste C
Saint Louis MO 63110
Medical School: Other - Unknown
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License #: 2004014829
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An Official American Thoracic Society Clinical Practice Guideline: Pediatric Chronic Home Invasive Ventilation. - American journal of respiratory and critical care medicine
Children with chronic invasive ventilator dependence living at home are a diverse group of children with special health care needs. Medical oversight, equipment management, and community resources vary widely. There are no clinical practice guidelines available to health care professionals for the safe hospital discharge and home management of these complex children.To develop evidence-based clinical practice guidelines for the hospital discharge and home/community management of children requiring chronic invasive ventilation.The Pediatric Assembly of the American Thoracic Society assembled an interdisciplinary workgroup with expertise in the care of children requiring chronic invasive ventilation. The experts developed four questions of clinical importance and used an evidence-based strategy to identify relevant medical evidence. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to formulate and grade recommendations.Clinical practice recommendations for the management of children with chronic ventilator dependence at home are provided, and the evidence supporting each recommendation is discussed.Collaborative generalist and subspecialist comanagement is the Medical Home model most likely to be successful for the care of children requiring chronic invasive ventilation. Standardized hospital discharge criteria are suggested. An awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child's care. Standardized equipment for monitoring, emergency preparedness, and airway clearance are outlined. The recommendations presented are based on the current evidence and expert opinion and will require an update as new evidence and/or technologies become available.
Shared clinician-patient decision-making about treatment of pediatric asthma: what do we know and how can we use it? - Current opinion in allergy and clinical immunology
Shared decision-making (SDM) is an emerging field that promises to improve healthcare. We aim to explore the concept of SDM, how it has been studied or applied in the treatment of asthma, and how it might be implemented to improve adherence and outcomes in pediatric asthma.Healthcare providers often fail to involve their patients in clinical decision-making by not presenting all available options, associated risks and benefits, in light of the patient's values, preferences, concerns, lifestyle, and perceived barriers to following various treatment regimens. It has been argued that SDM is preferable to a clinician-controlled approach and may improve patient outcomes (increase satisfaction with care, reduce decisional conflict and decisional regret, improve health-related quality of life, and increase decision-specific knowledge). This may be especially important in managing chronic conditions in which adherence to treatment regimen may increase if the patient was actively involved in the decision-making. In pediatrics, the decision process is further complicated by the clinician-parent(s)-child interaction. We found no studies on how to effectively involve and communicate with children at different developmental levels, or how to coalesce the parent and child's perspective to work as a unit.SDM has the promise to improve satisfaction with disease management, treatment adherence and patient-centered outcomes in pediatric asthma, but further research is needed to determine its effectiveness and to establish guidelines on how to implement SDM in the clinical setting and incorporate the input and preferences of all stakeholders' perspectives.
Do oral corticosteroids reduce the severity of acute lower respiratory tract illnesses in preschool children with recurrent wheezing? - The Journal of allergy and clinical immunology
Oral corticosteroids (OCSs) are recommended for severe wheezing episodes in children. However, limited evidence supports this intervention in preschool children with outpatient wheezing illnesses.We sought to investigate whether OCSs reduce symptom scores during acute lower respiratory tract illnesses (LRTIs) in preschool children with recurrent wheeze.We performed post hoc and replication analyses in 2 outpatient cohorts of children aged 1 to 5 years with episodic wheezing participating in clinical trials. We compared symptom scores during LRTIs that were or were not treated with OCSs, adjusting for differences in disease and episode severity covariates. We stratified episodes by severity by using a propensity model. The primary outcome was the area under the curve (AUC) of total symptom scores among the more severe episodes.Two hundred fifteen participants from the Acute Intervention Management Strategies trial experienced 798 acute LRTIs, 112 of which were defined as severe based on propensity scores. The AUCs of total symptom scores did not differ between the episodes that were (n = 70) and were not (n = 42) treated with OCSs (P = .46) nor was there an OCS treatment effect on individual symptom scores. Similar analyses of the Maintenance Versus Intermittent Inhaled Corticosteroids in Wheezing Toddlers trial, involving 278 participants with 133 severe LRTIs, confirmed the above findings (P = .46 for AUC of total symptoms score comparison).In 2 separate cohorts of preschool children with episodic wheezing, OCS treatment during clinically significant LRTIs did not reduce symptom severity during acute LRTIs, despite asthma controller medication use during most episodes. These findings need to be confirmed in a prospective randomized controlled trial.Copyright Â© 2013 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.
Heterozygous 24-polyalanine repeats in the PHOX2B gene with different manifestations across three generations. - Pediatric pulmonology
Congenital central hypoventilation syndrome (CCHS) is an uncommon genetic disorder that is characterized by alveolar hypoventilation and autonomic dysregulation. More than 90% of the patients are heterozygous for polyalanine repeat expansion mutations in the paired-like homeobox 2b (PHOX2B) gene. The normal genotype has a 20-polyalanine sequence whereas expanded alleles are usually 25-33. Heterozygous 24-polyalanine repeat expansion mutation in the PHOX2B gene has rarely been reported. We report three consecutive generations harboring heterozygous 24-polyalanine repeats in the PHOX2B gene with manifestations ranging from apparently asymptomatic to alveolar hypoventilation and apnea requiring mechanical ventilation. The 3-year-old proband developed cor pulmonale and central hypoventilation following an upper respiratory tract infection. Our findings add to the accumulating evidence that the 24-polyalanine repeat in the PHOX2B is a disease-causing mutation. In addition, a high index of suspicion and careful monitoring after anesthesia, sedation, or respiratory illnesses should be exercised when evaluating asymptomatic family members with this genotype.Â© 2013 Wiley Periodicals, Inc.
Recurrent pleural effusion without intrathoracic migration of ventriculoperitoneal shunt catheter: a case report. - Pediatric pulmonology
Pleural effusion is a rare complication of ventriculoperitoneal (VP) shunting, usually due to the migration of the VP shunt catheter into the thorax. Herein we report a neurologically disadvantaged child with a lobar holoprosencephaly and hydrocephalus, initially treated with a VP shunt, who years later developed recurrent right-sided pleural effusion ultimately confirmed to be a cerebrospinal fluid (CSF) hydrothorax without intra-thoracic migration of the distal shunt catheter. Thoracentesis was compatible with a transudative effusion. Given the presence of a persistent pleural effusion, beta-2 transferrin concentrations were measured, which yielded a positive result. Plain radiographs and head computed tomography (CT) showed a normally positioned, functional VP shunt. A spine CT myelogram to look for a spinal dural-thoracic CSF fistula was negative. A radionuclide CSF shunt study demonstrated normal functioning VP shunt with radiotracer accumulation within the peritoneum, with subsequent tracer rapidly accumulating in the right hemithorax. Video-assisted thoracoscopic (VATS) exploration with drainage of the pleural effusion and pleurodesis was then performed. No diaphragmatic defect or shunt tubing within the thorax was found and the procedure failed to resolve the effusion. The patient's recurrent effusion was ultimately resolved with intracranial endoscopic choroid plexus coagulation to decrease CSF output.Copyright Â© 2011 Wiley Periodicals, Inc.
Detection and home management of worsening asthma symptoms. - Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology
Asthma guidelines recommend early home treatment of exacerbations. However, home treatment is often suboptimal and delayed.To describe antecedent symptoms and signs of asthma exacerbations noticed by parents and to learn when and how parents intensify asthma treatment.Parents of children 2 to 12 years old with asthma exacerbations that required urgent care in the past 12 months completed telephone questionnaires. Where multiple responses were possible, percentages may sum to more than 100%.One hundred one parents were enrolled and interviewed; 94% were the children's mothers. Seventy percent of the children were black, and 64% had Medicaid insurance. Parents reported multiple antecedent symptoms and signs (median number per child, 3; range, 1-6), including respiratory symptoms (79%), allergy or cold symptoms (43%), behavioral changes (24%), and other nonspecific symptoms (29%). Twenty-three parents reported late respiratory symptoms, such as gasping for breath and using accessory muscles to breath, as the earliest antecedent signs. Treatment was most often intensified when the parent noticed cough (55%), shortness of breath (54%), and wheeze (25%) and included adding albuterol (92%), an oral corticosteroid (17%), an inhaled corticosteroid (8%), or other nonasthma medications (16%).Although parents described antecedent symptoms and signs of impending asthma exacerbations that they consistently noticed in their children, many waited for lower respiratory signs to be present before intensifying treatment. Oral corticosteroids were used infrequently. Interventions to improve the ability of parents and children to accurately recognize worsening symptoms and initiate timely, effective treatment are needed.
Signs and symptoms that precede wheezing in children with a pattern of moderate-to-severe intermittent wheezing. - The Journal of pediatrics
To examine parent-reported signs and symptoms as antecedents of wheezing in preschool children with previous moderate to severe wheezing episodes, and to determine the predictive capacity of these symptom patterns for wheezing events.Parents (n = 238) of children age 12 to 59 months with moderate-to-severe intermittent wheezing enrolled in a year-long clinical trial completed surveys that captured signs and symptoms at the start of a respiratory tract illness (RTI). Sensitivity, specificity, negative predictive value, and positive predictive value (PPV) for each symptom leading to wheezing during that RTI were calculated.The most commonly reported first symptom categories during the first RTI were "nose symptoms" (41%), "significant cough" (29%), and "insignificant cough" (13%). The most reliable predictor of subsequent wheezing was significant cough, which had a specificity of 78% and a PPV of 74% for predicting wheezing.Significant cough is the most reliable antecedent of wheezing during an RTI. It may be useful to consider individualized symptom patterns as a component of management plans intended to minimize wheezing episodes.
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