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Introduction
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Publications
Publications (33)
None:
Catathrenia, also known as sleep-related groaning, is a relatively rare sleep disorder with characteristics consistent with loud groaning on expiration during sleep. Organic causes of catathrenia are unknown and the decision of whether or not to treat is unclear. Limited research is available concerning appropriate treatment and none of the...
None:
Catathrenia, also known as sleep-related groaning, is a relatively rare sleep disorder with characteristics consistent with loud groaning on expiration during sleep. Organic causes of catathrenia are unknown and the decision of whether or not to treat is unclear. Limited research is available concerning appropriate treatment and none of the...
Background:
We describe the clinical characteristics and epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) in children with cystic fibrosis (CF) from the U.S. CF center with the highest MRSA prevalence.
Methods:
Medical records of children with CF were retrospectively reviewed from 1997-2009. MRSA clinical isolates from 2007-200...
To describe characteristics and surgical and clinical outcomes of obese children with obstructive sleep apnea (OSA).
At our institution from 2000 to 2010, 143 obese children with an overnight polysomnography (OPSG) diagnosis of OSA, excluding children with comorbidities, were identified. Relationships between demographics, clinical findings, and th...
Objective:
Recent advances in medicine have allowed children with chronic life-threatening disorders to survive longer than ever before with the use of complex medical device technology (e.g., mechanical ventilation, dialysis, etc.). The care of children with chronic pulmonary disorders and respiratory-technology dependence is often complex, invol...
Purpose of review:
Achieving an optimal nutritional status in patients with cystic fibrosis (CF) is important to maintain better pulmonary function, physical performance, and to prolong survival. So far, nutrition care in CF has mainly focused on fat intake and achieving energy balance, but there is increasing information on the clinical importanc...
Cystic fibrosis (CF) is a chronic disease requiring patients to have frequent specialty healthcare visits to delay progression of lung disease, prevent and treat failure to thrive and initiate early interventions to prevent acute illness and complications. The CF Foundation recommends that patients have visits with the CF care team at least every 3...
Obstructive sleep-disordered breathing (OSDB) is a condition that affects 1% to 3% of the pediatric population. These disorders are difficult to diagnosis and left untreated may be serious, including not only medical comorbidities but also cognitive, academic, behavioral, and emotional sequelae. This article is designed to bring awareness of the se...
To identify the characteristics of children with cystic fibrosis with low initial forced expiratory volume in 1 second (FEV1) % predicted and to investigate their outcome.
Patients were categorized into low or high initial FEV1 groups using cluster analysis. Comparisons of the demographic and clinical data were performed between the 2 groups.
From...
Adequate protein intake and digestion are necessary to prevent muscle wasting in cystic fibrosis (CF). Accurate and easy-to-use methodology to quantify protein maldigestion is lacking in CF.
To measure protein digestibility and the response to pancreatic enzyme intake in CF by using a new stable isotope methodology.
In 19 CF and 8 healthy subjects,...
Patients with cystic fibrosis (CF) have several risk factors for Clostridiumdifficile colonization such as frequent hospitalization and exposure to a broad array of antibiotics utilized for the control, eradication, and prophylaxis of respiratory pathogens. However, despite this high rate of colonization, the occurrence of C. difficile infection (C...
Background:
Many pediatric patients need positive airway pressure (PAP) for treatment of obstructive sleep-disordered breathing. Adherence to PAP (defined as percent of nights with PAP use of > 4 h) is often poor and not sustained long-term. With any chronic disease, education has been shown to help with patient outcomes. Education of patients and...
Objective:
To determine whether upregulated whole body de novo arginine synthesis and protein breakdown are present as a compensatory mechanism to meet the increased demand for arginine and nitric oxide (NO) production in pediatric patients with cystic fibrosis (CF) and nutritional failure.
Study design:
In 16 children with CF, studied at the en...
Background:
Current nutritional approaches have been partially successful in Cystic Fibrosis (CF). Essential amino acids mixtures with high Leucine levels (EAA) have anabolic properties in catabolic conditions, however data in CF are lacking.
Methods:
On two days according a randomized crossover design, 15 pediatric CF patients ingested 6.7 g EA...
Background and Aims
Bone mineral loss, reduced lung function and impaired nutritional status are frequently present in children with Cystic Fibrosis (CF). Blood concentrations and urinary excretion of hydroxyproline (OH-PRO) have been used as markers of bone mineral status and lung function in CF.
Objective
To examine whether whole body hydroxypro...
Background:
Cystic fibrosis (CF) affects approximately 30,000 people in the United States and is characterized by pulmonary disease and poor nutrition. Optimal nutrition is associated with better pulmonary function, and aggressive nutrition interventions have played a major role in increased survival. Even though the CF Foundation (CFF) has publis...
Background. Long-term outcomes for children who survive on tracheostomy and positive-pressure ventilation (TPPV) at home are not well known. Methods. A retrospective review of 20 years of clinical data at a single institution was performed. Outcome measures included 5-year survival, decannulation rate, and neurocognition. Results. A total of 91 chi...
Objectives:
Positive Airway Pressure (PAP) devices are being increasingly used in pediatric populations for problems including Obstructive Sleep Apnea. There is limited literature regarding pediatric adherence with PAP treatment or successful interventions that improve adherence. This paper describes a clinical program for pediatric PAP users whic...
Background & aims:
Nutritional failure in children with cystic fibrosis (CF) has a negative effect on their morbidity and survival. It is unknown if determination of fat-free mass is a better screening method for nutritional failure than the currently recommended body mass index (BMI) alone.
Methods:
This cross-sectional study in 77 children wit...
In adults with narcolepsy, periodic limb movements of sleep (PLMS) occur more frequently than in control population, and presence of increased PLMS is associated with greater sleep disruption and shorter mean sleep latency. This study was performed to determine whether PLMS are common in children with narcolepsy, and whether the presence of PLMS is...
Application of positive airway pressure is frequently indicated in pediatric patients with a diagnosis of obstructive sleep apnea. Adherence to equipment use is often less than optimal and can be more challenging when working with children with special needs. An in-patient protocol was designed utilizing various techniques and strategies from the m...
Adverse reactions to antibiotics in patients with cystic fibrosis (CF) are a growing concern. We report the case of a pediatric patient with CF with multiple comorbidities and a history of drug reactions, who developed life-threatening piperacillin-induced immune hemolytic anemia. We review drug-induced hemolytic anemia (DIIHA) in particular, and a...
Obstructive sleep-disordered breathing is associated with upper airway obstruction during sleep, which negatively affects sleep quality, ventilation, and/or oxygenation. The condition affects 2-11% of children. In this paper we discuss the epidemiology, pathophysiology, clinical features, diagnosis, and management of obstructive sleep-disordered br...
Cystic fibrosis (CF) is a life-threatening autosomal recessive disease and affects about 1 in 3500 newborns in the United States. In the last decade, advances to detect the disease include development of newborn screening. CF newborn screening is a complex process and diagnosing a newborn with CF sometimes can be challenging even for an expert. In...
Adenosine (Ado) regulates diverse cellular functions in the lung through its local production, release, metabolism, and subsequent stimulation of G-protein-coupled P1 purinergic receptors. The A(2B) adenosine receptor (A(2B)AR) is the predominant P1 purinergic receptor isoform expressed in surface airway epithelia, and Ado is an important regulator...