Follow-up of primary snoring in children.

We reevaluated 13 children with primary snoring 3 years after their original polysomnograms were performed and compared them with healthy control subjects. As a group, the patients' snoring and polysomnographic findings did not change. There was no difference in any sleep-disordered breathing of the patients and control subjects. Only the youngest individual in the study developed frank obstructive sleep apnea. Children with primary snoring are not likely to develop polysomnography-confirmed obstructive sleep apnea, and it is safe to defer treatment.

The effect of epoch length and smoothing on infant sleep and waking state architecture for term infants at 42 to 46 weeks postconceptional age.

STUDY OBJECTIVES: Epoch lengths from 20 seconds to 1 minute, and smoothing strategies from zero to three minutes are encountered in the infant sleep and waking literature. The present study systematically examined the impact of various epoch lengths and smoothing strategies on infant sleep state architecture. DESIGN: Overnight polysomnographic recordings were visually assessed by epoch as wake or as each of four sleep state parameters: electroencephalographic patterns, respiration, body movement, and eye movement. From these findings, sleep and waking states were assigned for each of six combinations of epoch length (30-second or 1-minute) and smoothing window length (none, 3-epoch, or 5-epoch). SETTING: N/A. PARTICIPANTS: Subjects were 91 term infants, 42-46 weeks postconceptional age, from the Collaborative Home Infant Monitoring Evaluation (CHIME) study. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: A greater epoch length resulted in more active and less quiet sleep as a percentage of total study; however, the size of the smoothing window did not affect the percentage of sleep/waking states. In general, the greater the epoch length and the greater the smoothing window length, the fewer the number of, the greater the mean duration of, and the greater the longest continuous episode of sleep/waking states. Analysis of significant interactions indicated that a 1-minute epoch length relative to a 30-second epoch length resulted in increasingly longer episodes of quiet and especially active sleep with a greater smoothing window length. CONCLUSIONS: Smoothing strategy significantly altered sleep state architecture in infants and may explain part of the variability in infant sleep state findings between laboratories.

Comparison of apnea identified by respiratory inductance plethysmography with that detected by end-tidal CO(2) or thermistor. The CHIME Study Group.

As part of the Collaborative Home Infant Monitoring Evaluation (CHIME) we compared apnea identified by a customized home monitor using respiratory inductance plethysmography (RIP) with simultaneously recorded polysomnography-acquired nasal end-tidal CO(2) (PET(CO(2))) and nasal/oral thermistor in 422 infants during overnight laboratory recordings to determine concordance between techniques, sources of disagreement, and capacity of RIP to detect obstructed breaths within an apnea. Among 233 episodes of apnea identified by at least one method as >/= 16 s, 120 were observed by the CHIME monitor, 219 by PET(CO(2)), and 163 by thermistor. The positive predictive value of the CHIME-identified apnea was 89.2% (95% CI 83, 95) and 73% (95% CI 65, 81) for PET(CO(2)) and thermistor, respectively. However, the sensitivity of the CHIME monitor in identifying events detected by the other methods was only approximately 50%. Among 87 apnea events identified by all three techniques, no two methods showed high agreement in measurement of apnea duration: RIP and PET(CO(2)) (ICC = 0.54), RIP and thermistor (ICC = 0.13), PET(CO(2)) and nasal thermistor (ICC = 0.41). Among the 179 breaths identified by RIP as obstructed, 79.9% were judged to be obstructed on the PET(CO(2)) and 80.4% were judged to be obstructed on the thermistor channel. Among 238 breaths identified on PET(CO(2)) as obstructed, 54.2% were determined to be obstructed by RIP. Among 204 breaths identified on thermistor as obstructed, 55. 4% were determined to be obstructed by RIP. Reasons for discrepancies in apnea detection among channels included body movement, partial airway obstruction, and obstructed breaths. Despite these limitations the CHIME monitor provides an opportunity to record physiological data previously unavailable in the home.

Agreement among raters in assessment of physiologic waveforms recorded by a cardiorespiratory monitor for home use. Collaborative Home Infant Monitoring Evaluation (CHIME) Study Group.

There are numerous reports of cardiorespiratory patterns in infants on home monitors, but no data to determine whether "experts" agree on the description of these patterns. Therefore, we evaluated agreement among four experienced investigators and five trained technicians who assessed independently the same sample of physiologic waveforms recorded from infants enrolled in a multicenter study. The monitor used respiratory inductance plethysmography and recorded waveforms for apnea > or = 16 s or a heart rate < 80 beats/min for > or = 5 s. The investigators and technicians initially assessed 88 waveforms. After additional training, the technicians assessed another 113 additional waveforms. In categorizing waveforms as apnea present or absent, agreement among technicians improved considerably with additional training (kappa 0.65 to 0.85). For categorizing waveforms as having bradycardia present versus absent, the trends were the same. Agreement in measurement of apnea duration also improved considerably with additional training (intraclass correlation 0.33-0.83). Agreement in measurement of bradycardia duration was consistently excellent (intraclass correlation 0.86-0.99). Total agreement was achieved among technicians with additional training for measurement of the lowest heart rate during a bradycardia. When classifying apnea as including > or = 1, > or = 2, > or = 3, or > or = 4 out-of-phase breaths, agreement was initially low, but after additional training it improved, especially in categorization of apneas with > or = 3 or > or = 4 out-of-phase breaths (kappa 0.67 and 0.94, respectively). Although researchers and clinicians commonly describe events based on cardiorespiratory recordings, agreement amongst experienced individuals may be poor, which can confound interpretation. With clear guidelines and sufficient training raters can attain a high level of agreement in describing cardiorespiratory events.

Adenoid size is related to severity but not the number of episodes of obstructive apnea in children.

OBJECTIVE: The objective of this study was to determine the extent to which adenotonsillar hypertrophy contributes to the severity of obstructive sleep apnea (OSA) in children. STUDY DESIGN: Thirty-three consecutive children who were referred to a sleep disorders center for evaluation of suspected OSA had standard lateral neck roentgenography performed. Adenoid size was determined by measuring the adenoidal-nasopharyngeal (AN) ratio. Tonsil size was quantitated on physical examination. The severity of OSA was determined by full-night polysomnography in the sleep laboratory. RESULTS: All of the patients reported snoring with trouble breathing, apneas, or both problems witnessed by a parent. The patients' respiratory disturbance index ranged from 0 to 95.3 (mean +/- SD 12.5 +/- 9.1). The patients' AN ratio ranged from 0.48 to 0.98 (0.76 +/- 0.14); 30 (91%) of the 33 patients had AN ratios greater than published normal means, and 16 (48%) had AN ratios more than 2 standard deviations above published means. Although the AN ratio and tonsil size did not predict the number of apneas, a significant relationship was seen between the AN ratio and the duration of obstructive apneas (r = 0.48, p < 0.01). Obesity (percent ideal body weight) was the only independent predictor for the number of respiratory events per hour of sleep (r = 0.49, p < 0.01). Percent ideal body weight was also the major predictor of the lowest oxyhemoglobin saturation (r = -0.58, p < 0.0001), but the AN ratio also contributed to the variance in saturation, with a correlation coefficient (r) of 0.69 for the two factors (p < 0.0001). CONCLUSION: Lymphoid hyperplasia affects the severity of apnea more than the number of obstructive apneas. The pathophysiologic characteristics of OSA in children probably involve complex interactions between pharyngeal size and mechanics.

Infant polysomnography: reliability. Collaborative Home Infant Monitoring Evaluation (CHIME) Steering Committee.

Infant polysomnography (IPSG) is an increasingly important procedure for studying infants with sleep and breathing disorders. Since analyses of these IPSG data are subjective, an equally important issue is the reliability or strength of agreement among scorers (especially among experienced clinicians) of sleep parameters (SP) and sleep states (SS). One basic issue of this problem was examined by proposing and testing the hypothesis that infant SP and SS ratings can be reliably scored at substantial levels of agreement, that is, kappa (kappa) > or = 0.61. In light of the importance of IPSG reliability in the collaborative home infant monitoring evaluation (CHIME) study, a reliability training and evaluation process was developed and implemented. The bases for training on SP and SS scoring were CHIME criteria that were modifications and supplements to Anders, Emde, and Parmelee (10). The kappa statistic was adopted as the method for evaluating reliability between and among scorers. Scorers were three experienced investigators and four trainees. Inter- and intrarater reliabilities for SP codes and SSs were calculated for 408 randomly selected 30-second epochs of nocturnal IPSG recorded at five CHIME clinical sites from healthy full term (n = 5), preterm (n = 4), apnea of infancy (n = 2), and siblings of the sudden infant death syndrome (SIDS) (n = 4) enrolled subjects. Infant PSG data set 1 was scored by both experienced investigators and trained scorers and was used to assess initial interrater reliability. Infant PSG data set 2 was scored twice by the trained scorers and was used to reassess inter-rater reliability and to assess intrarater reliability. The kappa s for SS ranged from 0.45 to 0.58 for data set 1 and represented a moderate level of agreement. Therefore, rater disagreements were reviewed, and the scoring criteria were modified to clarify ambiguities. The kappa s and confidence intervals (CIs) computed for data set 2 yielded substantial inter-rater and intrarater agreements for the four trained scorers; for SS, the kappa = 0.68 and for SP the kappa s ranged from 0.62 to 0.76. Acceptance of the hypothesis supports the conclusion that the IPSG is a reliable source of clinical and research data when supported by significant kappa s and CIs. Reliability can be maximized with strictly detailed scoring guidelines and training.

Assessment of tidal volume over time in preterm infants using respiratory inductance plethysmography, The CHIME Study Group. Collaborative Home Infant Monitoring Evaluation.

Non-invasive techniques for monitoring ventilation in infants are widely used in short-term laboratory-studies but have not been evaluated in routine clinical settings. To determine whether respiratory inductance plethysmography (RIP) can provide reproducible measurements of tidal volume (VT) in premature infants over an extended period of time, we monitored respiration in eight healthy preterm infants over 4.9 +/- 1.0 hours (mean +/- SD). The algebraic sum (Sum) of rib cage (RC) and abdominal (AB) motion signals (obtained by RIP) was calculated and presented over the entire recording period as percent of an initial 5 minute calibration period. VT was simultaneously measured with a nasal mask pneumotachometer with infants in prone and supine positions during active and quiet sleep. Infants were studied in the morning (AM) and again in the afternoon (PM). Between these studies they were returned to the nursery wearing the RIP in a continuous record mode. For all patients there was a significant linear relationship between VT (in mL measured by pneumotachometer) and Sum (in % of calibration value, RIP). Neither the slope of the relationship (0.074 +/- 0.03 in AM vs. 0.071 +/- 0.02 in PM), nor its variability as measured by standard error of the estimate (SEE) (2.3 +/- 0.5 in AM vs. 2.5 +/- 0.8 in PM) changed significantly from AM to PM. The relationship between VT and Sum, as well as the variability of that relationship, was not altered by position, asynchrony of RC and AB, respiratory rate, or percent RC contribution to Sum. We conclude that RIP produces consistent measurements of respiratory effort over 5 hours in healthy preterm infants without need for recalibration and is not affected by routine care.

Normal oxyhemoglobin saturation during sleep. How low does it go?

STUDY OBJECTIVE: To determine oxyhemoglobin saturation (O2 Sat) in healthy humans. DESIGN: Retrospective review of all-night pulse oximetry data, carefully examined to exclude periods of motion artifact. The lowest saturation recorded during the night (Low Sat), the median saturation (Sat 50), and the saturation below which the patient spent 10% of the time (Sat 10) were tabulated. These data were compared to the O2 Sat in patients with obstructive sleep apnea (OSA) and patients with stable asthma. SETTING: Sleep laboratory in a tertiary care hospital. PATIENTS: Three hundred fifty people with normal results of overnight polysomnography. Patients with known craniofacial or neurologic abnormalities or any previously diagnosed pulmonary disease such as asthma or COPD were excluded. The healthy subjects ranged in age from 1 month to 85 years. There were 184 male and 166 female subjects. These were compared to 25 patients with OSA and 21 patients with asthma. RESULTS: For the healthy patients, the mean +/- SD Low Sat was 90.4% +/- 3.1%. The mean Sat 10 was 94.7% +/- 1.6%. The mean Sat 50 was 96.5% +/- 1.5%. There was no relationship between any of the O2 Sat measures and sex, race, or obesity as measured by body mass index. However, older subjects (> 60 years of age) had lower Sat 10 (92.8 +/- 2.3) and Sat 50 (95.1 +/- 2.0) than did younger subjects. The O2 Sat of the patients with asthma was not different from the healthy subjects, but the patients with OSA had a significantly lower Sat 50, Sat 10, and Low Sat. SUMMARY: We describe in detail O2 Sat in a large group of healthy people. Older subjects without known cardiorespiratory disease have lower O2 Sat than younger subjects.

Does sleep-disordered breathing contribute to the clinical severity of sickle cell anemia?

PURPOSE: This research was undertaken to determine whether obstructive sleep apnea (OSA) and/or nocturnal hemoglobin desaturations contribute to the clinical severity of sickle cell anemia (SS). PATIENTS AND METHODS: Eleven patients with severe SS (group S), defined by two or more hospitalizations in the previous year for painful crises, were compared to eight patients with mild SS (group M) who had not been hospitalized for painful crises in the past year. An additional cohort of nine patients with SS who had been referred to the Sleep Disorders Center because of a clinical suspicion of OSA were studied (group R). All patients underwent full overnight polysomnography and performed standard pulmonary function tests. RESULTS: There were no significant differences in the respiratory disturbance index (RDI; apneas plus hypopneas per hour of sleep) or hemoglobin desaturation between the mild and severe groups, and neither RDI nor hemoglobin saturation predicted the number of painful crises. Despite a suggestive clinical presentation, only 44% of the patients in group R had OSA confirmed polysomnographically. CONCLUSIONS: In this preliminary study, unsuspected nocturnal cardiopulmonary disease and hemoglobin desaturation did not explain the variability in the severity of SS disease. However, OSA can occur in patients with SS, and when clinically suspected, the diagnosis should be confirmed with overnight polysomnography so that appropriate treatment can be instituted.

Vulnerability of respiratory control in healthy preterm infants placed supine.

OBJECTIVE: We tested the hypothesis that healthy preterm infants have attenuated ventilatory responses to hypercapnia, associated with a decreased rib cage contribution to ventilation, in the supine versus prone position. STUDY DESIGN: We elicited hypercapnic ventilatory responses from 19 healthy preterm infants (postconceptional age 35 +/- 1 weeks) who were being prepared for hospital discharge. The O2 saturation was continuously monitored. Before and during CO2 rebreathing, ventilation was measured with a nasal mask pneumotachygraph and was derived from chest wall motion as determined by respiratory inductance plethysmograph. This measuring method allowed us to compare both ventilation and the percentage rib cage contribution to ventilation between supine and prone positions. Statistical analysis employed analysis of variance with repeated measures. RESULTS: The supine position was associated with a higher respiratory rate (p < 0.02) and lower O2 saturation (p < 0.007) than the prone position. The increase in ventilation in response to hypercapnia was lower in the supine than in the prone position. This was statistically significant for the respiratory inductance plethysmograph (p < 0.008) but not the pneumotachygraph (p = 0.077), and was associated with a smaller rib cage contribution to ventilation in the supine than in the prone position (p < 0.0001). CONCLUSION: Respiratory control may be vulnerable when healthy preterm infants are placed supine. Widespread avoidance of the prone position may not be appropriate for such patients.

Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea.

OBJECTIVE: To determine the safety and efficacy of nasal continuous positive airway pressure (CPAP) for obstructive sleep apnea (OSA) during childhood and the effects of growth and maturation on CPAP requirements. DESIGN: Retrospective study with use of a written questionnaire administered to pediatric practitioners treating sleep disorders. SETTING: Nine academic pediatric sleep disorders centers. RESULTS: Data were obtained for 94 patients. Three percent of patients receiving CPAP were less than 1 year, 29% were 1 to 5 years, 36% were 6 to 12 years, and 32% were 13 to 19 years of age; 64% were boys. The longest duration of CPAP use was 4 years. Indications for CPAP included OSA associated with obesity (27%), craniofacial anomalies (25%), idiopathic OSA persisting after adenoidectomy and tonsillectomy (17%), and trisomy 21 (13%). Continuous positive airway pressure was effective in 81 patients (86%), in one patient it was unsuccessful, and in 12 patients compliance was inadequate. The median pressure required was 8 cm H2O (range, 4 to 20 cm H2O); pressure requirements were independent of age or diagnosis. Twenty-two percent of patients eventually required a modification of CPAP levels. Complications of CPAP were minor. Sixty-four percent of centers reported difficulty in obtaining funding for CPAP. CONCLUSIONS: Continuous positive airway pressure is safe, effective, and well tolerated by children and adolescents with OSA. Experience in infants is limited. As pressure requirements change with patient growth, we recommend that CPAP requirements be regularly reevaluated over time. The marked center-to-center variability in CPAP use suggests that specific indications for this therapy require clarification.

Adenotonsillectomy for treatment of obstructive sleep apnea in children.

OBJECTIVES: To determine (1) the prevalence of obstructive sleep apnea (OSA) in children with a suggestive history; (2) the effectiveness of surgery in treating OSA in children; and (3) factors that may help the physician select patients who have physiologically significant OSA and are likely to respond to surgery. DESIGN: Prospective study. PATIENTS: Sixty-nine children aged 1 to 14 years who were referred to the otolaryngologist for evaluation of suspected OSA. INTERVENTIONS: Thirty children with a respiratory disturbance index (RDI) greater than 5 underwent adenotosillectomy. Twenty-six of the 30 children had follow-up polysomnography. MAIN OUTCOME MEASURES: Polysomnography after surgery. RESULTS: Thirty-five (51%) of 69 children had an RDI greater than 5 on polysomnography. Twenty-six of the 30 children who underwent adenotonsillectomy for OSA had follow-up polysomnography. All 26 children had a lower RDI after surgery, although four patients still had an RDI greater than 5. A preoperative RDI of 19.1 or less predicted a postoperative RDI of 5 or less. History and physical findings were not useful in predicting outcome. CONCLUSIONS: All patients improved with adenotonsillectomy, but patients with the most severe RDI often had many respiratory events after surgery. History and physical examination alone are not sufficient to assess the severity of OSA or the likelihood of an adequate response to surgical treatment.

Survival after liver transplantation for chronic hepatitis B using reduced immunosuppression.

BACKGROUND/AIMS: Recurrent hepatitis B virus infection after liver transplantation performed for chronic hepatitis B with cirrhosis is influenced by a number of factors, including coinfection with the hepatitis D virus, the level of HBV replication, and administration of hepatitis B immune globulin. Another potentially important factor in modulating HBV infection after liver transplantation is the degree of immunosuppression post-transplant. We reviewed an institutional experience with liver transplantation for chronic hepatitis B and analyzed the impact of using lower doses of corticosteroids on HBV reinfection, expression of recurrent HBV disease and patient survival. METHODS: Of 17 patients undergoing liver transplantation for chronic hepatitis B, 16 patients received variable doses of hepatitis B immune globulin for up to 6 months. RESULTS: Fifteen of the 16 patients remained HBsAg-negative during hepatitis B immune globulin therapy, but ultimately 13 of the 17 patients had HBV reinfection, including 3 of 4 patients with hepatitis D virus coinfection. Long-term survival (82%) of the 17 chronic hepatitis B patients was not different from the survival (75%) of 195 patients transplanted for other indications. Three of 13 patients who were reinfected died from chronic hepatitis B with liver failure. Reinfection did not appear to be related to the pretransplant degree of viral replication. Compared to an age- and sex-matched control group, patients undergoing liver transplantation for chronic hepatitis B received less cumulative intravenous methylprednisolone and oral prednisone, but did not experience a higher rate of graft rejection. CONCLUSIONS: We postulate that use of lower doses of corticosteroids after liver transplantation for chronic hepatitis B is safe and not associated with a higher incidence of graft rejection. Moreover, low-dose maintenance prednisone therapy may modify the course of post-transplant HBV reinfection by leading to less viral replication, milder HBV-related liver disease and better patient survival.

Congenital absence of the epiglottis and its potential role in obstructive sleep apnea.

A 3 month old girl with congenital absence of the epiglottis presents with inspiratory stridor. Over the next 8 years frank obstructive sleep apnea (OSA) develops, confirmed by polysomnography. She has no difficulty in swallowing or phonation as assessed clinically and with barium swallow. This suggests that the epiglottis may help stabilize the upper airway, and any role in swallowing or phonation may, in its absence, be compensated by other mechanisms.

Racemic versus l-epinephrine aerosol in the treatment of postextubation laryngeal edema: results from a prospective, randomized, double-blind study.

OBJECTIVE: To determine whether any advantage exists using racemic epinephrine instead of the more potent and less expensive levo(1)-epinephrine in the treatment of postextubation laryngeal edema. DESIGN: Prospective, double-blind, randomized study. SETTING: Pediatric intensive care unit in a university teaching hospital. PATIENTS: Twenty-eight patients with stridor during the immediate postextubation period. INTERVENTIONS: After extubation, patients demonstrating clinically important stridor were randomized in a double-blind fashion to receive an aerosol containing either 2.25% racemic or 1% l-epinephrine. MEASUREMENTS AND MAIN RESULTS: Heart rate, respiratory rate, blood pressure, and stridor score were determined at 20, 40, and 60 mins and 4 and 8 hrs after the initial aerosol administration. Patients in both groups demonstrated significant (p < .01) reductions in stridor score after aerosol administration. No significant differences were observed between treatment groups in improvement in stridor score or the number of subsequent aerosols required. Respiratory rate decreased significantly 40 and 60 mins after l-epinephrine but not after racemic epinephrine. No significant change in heart rate or blood pressure occurred after aerosol administration in either group. CONCLUSIONS: These data suggest that aerosolized l-epinephrine is as effective as aerosolized racemic epinephrine in the treatment of postextubation laryngeal edema without additional adverse side effects. When dosed appropriately, l-epinephrine is a less expensive and more widely available alternative to racemic epinephrine for the treatment of postextubation laryngeal edema.

Diagnosis and pathophysiology of obstructive sleep apnea in children.

Factors that decrease pharyngeal size or increase pharyngeal compliance increase an individual's risk for OSA. In children, these factors include craniofacial disorders, central nervous system problems, or simply adenotonsillar hypertrophy. Although children with OSA may present with nocturnal symptoms of snoring, respiratory pauses, secondary enuresis, and/or nightmares, their initial complaint may be poor school performance, hyperactivity, or other symptoms of sleep fragmentation. The diagnosis is confirmed by overnight polysomnography in a laboratory with expertise in the care of infants and children.

Treatment of otherwise normal children with obstructive sleep apnea.

There are many therapeutic approaches to children with OSA. Treatment should be considered only when the severity of the syndrome has been established by objective testing including overnight polysomnography. Anatomic abnormalities, including adenotonsillar hypertrophy, must be defined. Once the severity and underlying cause of OSA have been established, the most appropriate approach can be devised for the individual. Mild cases may simply be observed. Moderate or severe patients whose nasopharynx is obstructed by lymphoid hyperplasia may be treated with adenotonsillectomy. If surgery is declined or contraindicated, nasal CPAP is effective. CPAP is also useful as a temporary measure while weight loss is being effected.

Size and mechanical properties of the pharynx in healthy men and women.

Obstructive sleep apnea (OSA) has a marked male predominance. To determine whether there are differences in the mechanical properties of the pharynx of men and women that may contribute to the gender difference in disease incidence, we measured pharyngeal cross-sectional area during quiet breathing in 77 normal men and 98 normal women using the acoustic pulse technique. Standard pulmonary function tests were also performed. Pharyngeal mechanics were studied in 23 men and 34 women by measuring the change in pharyngeal area during a slow vital capacity maneuver. Gender was found to be the most important independent factor contributing to pharyngeal size. The men had a significantly larger pharynx than the women (3.63 +/- 0.10 versus 3.20 +/- 0.09 cm2, mean +/- SEM; p < 0.01). Pharyngeal mechanics were also different between men and women. The men had a larger change in pharyngeal area with changing lung volume than the women (0.60 +/- 0.14 versus 0.12 +/- 0.12 cm2, mean +/- SEM; p < 0.02). This difference persisted even after normalizing the data for pharyngeal size. We found that there are gender-related differences in the size and mechanical properties of the pharynx and speculate that the larger pharynx of men may be more than offset by greater changes in pharyngeal size with changing lung volume, contributing to the greater incidence of OSA in men.

Behavioral assessment and management of food refusal in children with cystic fibrosis.

Four children with cystic fibrosis, ranging in age from 10 to 40 months, were admitted to a specialized pediatric unit for evaluation and treatment of malnutrition. All were below the fifth percentile for weight despite appropriate pancreatic enzyme replacement and outpatient nutritional counseling. Dietary evaluation revealed oral intake of 48% to 62% of that required for growth. Standardized nursing and psychological assessments of feeding behaviors during meals indicated a low acceptance rate of foods and a high rate of maladaptive feeding behaviors. Treatment consisted of behavioral management using positive reinforcement of food acceptance, extinction of negative behaviors, and parent training. Mean percentage of caloric intake increased from 54% to 92% for the four patients. At long-term follow-up, the patients who continued the program demonstrated substantial and persistent catch-up growth. Behavioral feeding disorders may contribute to failure to thrive in patients with cystic fibrosis and must be considered when growth failure occurs despite correct medical management and apparently mild pulmonary and gastrointestinal involvement.