Ameet S. Daftary

Effect of Long-term Steroids on Cough Efficiency and Respiratory Muscle Strength in Patients With Duchenne Muscular Dystrophy

OBJECTIVE. The objective of this study was to determine whether long-term steroid therapy is associated with increased peak cough flow in patients with Duchenne muscular dystrophy and to determine which pulmonary function test variable is most predictive of peak cough flow. METHODS. In this case-control study, the medical charts of patients who had Duchenne muscular dystrophy and had pulmonary function tests at our institution in the previous 2 years were examined. Steroid-treated patients were on therapy for at least 1 year. The measured pulmonary function tests included forced vital capacity, maximum expiratory pressure, maximum inspiratory pressure, maximum voluntary ventilation, and peak cough flow. Multiple linear regression analysis was used to determine which pulmonary function test measure was most predictive of peak cough flow and assess the influence of steroid treatment and patient age on peak cough flow. RESULTS. Ten steroid-treated and 25 untreated patients were analyzed. Peak cough flow and maximum expiratory pressure were significantly higher in the steroid-treated patients. Each of the pulmonary function test variables was significantly associated with peak cough flow. The linear model that had the highest adjusted r2 value included only 2 variables: maximum voluntary ventilation and steroid treatment, demonstrating that steroid-treated patients had peak cough flow values that were 27 L/min higher than the untreated patients. The interaction between maximum voluntary ventilation and steroid was not statistically significant, suggesting that the steroid-associated increase in peak cough flow was approximately constant over the observed range of maximum voluntary ventilation values. The effects of maximum voluntary ventilation and treatment group on peak cough flow were not confounded with the patient age. CONCLUSIONS. Long-term steroid therapy is associated with improved peak cough flow and respiratory muscle strength in patients with Duchenne muscular dystrophy. Maximum voluntary ventilation may be a useful predictor of lung function in Duchenne muscular dystrophy.

Inhalational Lung Injury Associated With Humidifier “White Dust”

Humidifiers are commonly used in the community to relieve symptoms associated with acute respiratory infections in young children; however, clear benefits of these devices have not been documented. The Environmental Protection Agency has not found any adverse health effects related to humidifier use. We report here the case of a young infant with significant accidental inhalational lung injury related to dispersal of mineral dust from an ultrasonic home-use humidifier. The clinical consequences included prolonged hypoxemia, tachypnea, and failure to thrive. Radiography revealed pneumonitis, and pulmonary-function testing showed a nonreversible mild obstructive ventilatory defect. Because of persistent symptoms, evolution of failure to thrive, and nonresponse to inhaled and short courses of systemic glucocorticoids, an aggressive management approach was successfully pursued with high-dose pulse steroid therapy, which could be a potential therapeutic approach for similar patients. In addition, this case raises important questions about the safety of exposing infants and young children to humidifiers and emphasizes the need for further study.

Intracardiac fungal masses in high‐risk neonates: clinical observations

Four cases of intracardiac fungal masses occurred over 2 y amongst 7 cases of systemic candidiasis in a neonatal referral unit. The gestations and birthweights were 25, 23, 24 and 30 wk and 805, 605, 640 and 1395 g, respectively. The pedunculated, solitary right atrial masses were detected 2—17 d after diagnosing candidemia in 3 cases, whereas it was the presenting feature in the 4th. All had indwelling right atrial catheters and received multiple courses of broad‐spectrum antibiotics. The masses were removed successfully in two cases fit for surgery. None survived despite antifungal therapy, including liposomal amphotericin B at 6 mg/kg/d. Early introduction of enteral feeds, minimization of prolonged exposure to broad‐spectrum antibiotics and judicious use of central catheters may reduce the incidence of systemic candidiasis in high‐risk neonates. Surveillance echocardiography and timely surgical intervention may reduce the mortality and/or morbidity related to intracardiac fungal masses. □Candidiasis, intracardiac mass, liposomal amphotericin B, neonate

Robin sequence: what the multidisciplinary approach can do

Robin sequence (RS) is a commonly encountered triad of micrognathia, glossoptosis, and airway obstruction, with or without a cleft palate. The management of airway obstruction is of paramount importance, and multiple reviews and retrospective series outline the diagnosis and treatment of RS. This article focuses on the multidisciplinary nature of RS and the specialists’ contributions and thought processes regarding the management of the RS child from birth to skeletal maturity. This review demonstrates that the care of these children extends far beyond the acute airway obstruction and that thorough monitoring and appropriate intervention are required to help them achieve optimal outcomes.

Association of pericardial effusion with gastroschisis.

Sir: If there is no doubt about the importance of psycho-neuro-rehabilitation in the therapy of comatose patients, there seems to be disagreement about when in the clinical course rehabilitation should begin. Some authors [1] suggest that comatose patients should be placed in stimulation-rich environments, even in the acute phase of therapy in intensive care units. This opinion raises many questions about both scientifictheoretical aspects and the feasibility of a precocious rehabilitation therapy for a patient whos life is at risk and who must be protected from the dangers deriving from exposure to external noxious agents. A simple kind of stimulation is the sound-musical one requiring only a cassette player. Listening to music, especially through earphones, by comatose patients seems to be a widespread practice nowadays, often requested by the families who search for a more active role in assisting their relative who is ataken awayo from their care by an often short visiting time and by high technology instruments necessary to monitor vital signs and maintain some physiological functions. This stimulation seems to be the object of scientific investigation [2] in a non-systematic way. Clinical research moves between two scientific parameters: the quantitative one and the qualitative one [3], both of them bringing forth important observations whose correlation is methodologically rather complex. According to Bruscia [4] basic aspects of research are documentation and communication. At the University of Turin, we began 3 years ago research concerning sound-musical stimulation of comatose patients in collaboration with the University of Buenos Aires (Professor R. O. Benenzon). We began by studying the theoretical basis of this research and by first approaching to healthy volunteers [5] to verify the feasibility of the method of investigation we elaborated. Our clinical study with comatose patients is just starting; the data we are collecting cannot be published yet because assumptions of inference between stimulation, on the one hand, and central nervous system (CNS) reactions and clinical outcome, on the other, need more documentation, but we would like to communicate the epistemological guidelines of our research program, given that little is known about the functioning of a comatose subjects mind. 1. Organic hypothesis. Psychic life coincides with brain activity. In this case the aim of the research is to establish a stimulus which can facilitate the aawakeningo of the CNS. The stimulation could facilitate the process of dendritic growth and could improve the synaptic connections [1]. In this case, a standard stimulus could function in a wide number of subjects. 2. Psychical hypothesis. According to this assumption, psychical activity still persists, but its connections with the external world are impaired. The lack of motor control and the impairment of perception do not mean an absence of psychical life, because this one cannot be reduced to consciousness. In this case, the purpose of the research is to establish a stimulus which, overcoming the perceptive barrier, can settle a communication path to the mind. A highly individualized stimulus could allow the subject to recall past experiences. By activating the mnesic processes, it is possible to maintain in the subject the past-present continuity and, then, the meaning of his or her own identity [2].

Unexplained Practice Variation in Primary Care Providers' Concern for Pediatric Obstructive Sleep Apnea

OBJECTIVE To examine primary care provider (PCP) screening practice for obstructive sleep apnea (OSA) and predictive factors for screening habits. A secondary objective was to describe the polysomnography completion proportion and outcome. We hypothesized that both provider and child health factors would predict PCP suspicion of OSA. METHODS A computer decision support system that automated screening for snoring was implemented in 5 urban primary care clinics in Indianapolis, Indiana. We studied 1086 snoring children aged 1 to 11 years seen by 26 PCPs. We used logistic regression to examine the association between PCP suspicion of OSA and child demographics, child health characteristics, provider characteristics, and clinic site. RESULTS PCPs suspected OSA in 20% of snoring children. Factors predicting PCP concern for OSA included clinic site (P < .01; odds ratio [OR] = 0.13), Spanish language (P < .01; OR = 0.53), provider training (P = .01; OR = 10.19), number of training years (P = .01; OR = 4.26) and child age (P < .01), with the youngest children least likely to elicit PCP concern for OSA (OR = 0.20). No patient health factors (eg, obesity) were significantly predictive. Proportions of OSA suspicion were variable between clinic sites (range, 6-28%) and between specific providers (range, 0-63%). Of children referred for polysomnography (n = 100), 61% completed the study. Of these, 67% had OSA. CONCLUSIONS Results suggest unexplained small area practice variation in PCP concern for OSA among snoring children. It is likely that many children at risk for OSA remain unidentified. An important next step is to evaluate interventions to support PCPs in evidence-based OSA identification.

NREM Sleep Parasomnia Associated with Chiari I Malformation

Parasomnias are common sleep disorders in children, and most cases resolve naturally by adolescence.(1) They represent arousal disorders beginning in NREM sleep and are generally non-concerning in children. The diagnosis can usually be made by clinical assessment, and testing with polysomnography is not routinely indicated.(2) However, in certain cases with atypical features, polysomnography and more extensive neurologic evaluation are medically indicated.

Treatment of childhood obstructive sleep apnea.

Opinion statementAn array of surgical, medical and orthodontic treatments is available for treating childhood obstructive sleep apnea. Adenotonsillectomy remains the first choice in treatment, with a need for subsequent clinical and polysomnographic reassessment in selected cases to determine residual sleep-disordered breathing. Residual obstructive sleep apnea is more likely in patients with craniofacial abnormalities or obesity. It may require the use of a positive airway pressure breathing device. Topical corticosteroids, leukotriene antagonists, weight reduction, and positional therapy also play a role in ameliorating childhood obstructive sleep apnea. The published evidence for the efficacy of various treatment modalities consists largely of case-controlled studies and case reports.

Neurodevelopmental Outcomes at Two Years of Age for Premature Infants Diagnosed With Neonatal Obstructive Sleep Apnea

STUDY OBJECTIVES Neurocognitive deficits have been shown in school-aged children with sleep apnea. The effect of obstructive sleep apnea (OSA) on the neurodevelopmental outcome of preterm infants is unknown. METHODS A retrospective chart review was performed for all preterm infants (< 37 weeks) who had neonatal polysomnography (PSG) and completed neurodevelopmental assessment with the Bayley Scales of Infant and Toddler Development, 3rd Edition, between 2006 to 2015 at Riley Hospital. Exclusion criteria included grade IV intraventricular hemorrhage, tracheostomy, cyanotic heart disease, severe retinopathy of prematurity, craniofacial anomalies, or central and mixed apnea on PSG. Sleep apnea was defined as an apnea-hypopnea index (AHI) > 1 event/h. Regression analyses were performed to find a relationship between PSG parameters and cognitive, language, and motor scores. RESULTS Fifteen patients (males: n = 10) were eligible for the study. Median postmenstrual age at the time of the PSG was 41 weeks (37-46). Median AHI for the cohort was 17.4 events/h (2.2-41.3). Median cognitive, language, and motor scores were 90 (65-125), 89 (65-121), and 91 (61-112), respectively. Mean end-tidal CO2 (median 47 mm Hg [25-60]) negatively correlated with cognitive scores (P = .01) but did not significantly correlate with language or motor scores. AHI was not associated with cognitive, language, or motor scores. CONCLUSIONS The median score for cognitive, language, and motor scores for preterm infants with neonatal OSA were within one standard deviation of the published norm. Mean end-tidal CO2, independent of AHI, may serve as a biomarker for predicting poor cognitive outcome in preterm infants with neonatal OSA. COMMENTARY A commentary on this article appears in this issue on page 1233.