Obstructive sleep apnea is position dependent in young infants.

BACKGROUND: Obstructive sleep apnea in infants with Pierre Robin sequence is sleep-position dependent. The influence of sleep position on obstructive events is not established in other infants. METHODS: We re-evaluated ten-year pediatric sleep center data in infants aged less than six months, with polysomnography performed in different sleep positions. We excluded infants with syndromes, genetic defects, or structural anomalies. RESULTS: Comparison of breathing between supine and side sleeping positions was performed for 72 infants at the median corrected age of 4 weeks (interquartile range (IQR) 2-8 weeks). Of the infants, 74% were male, 35% were born prematurely, and 35% underwent study because of a life-threatening event or for being a SIDS sibling. Upper airway obstruction was more frequent (obstructive apnea-hypopnea index (OAHI), p < 0.001), 95th-percentile end-tidal carbon dioxide levels were higher (p = 0.004), and the work of breathing was heavier (p = 0.002) in the supine than in the side position. Median OAHI in the supine position was 8 h-1 (IQR 4-20 h-1), and in the side position was 4 h-1 (IQR 0-10 h-1). CONCLUSIONS: Obstructive upper airway events in young infants are more frequent when supine than when sleeping on the side. IMPACT: The effect of sleep position on obstructive sleep apnea is not well established in infants other than in those with Pierre Robin sequence. A tendency for upper airway obstruction is position dependent in most infants aged less than 6 months. Upper airway obstruction is more common, end-tidal carbon dioxide 95th-percentile values higher, and breathing more laborious in the supine than in the side-sleeping position. Upper airway obstruction and obstructive events have high REM sleep predominance. As part of obstructive sleep apnea treatment in young infants, side-sleeping positioning may prove useful.

Obstructive sleep apnea in young infants: Sleep position dependence and spontaneous improvement.

OBJECTIVES: The natural evolution of obstructive sleep apnea (OSA) in young infants is not established. METHODS: We re-evaluated 10-year pediatric sleep center infant polysomnography (PSG) data, excluding infants with syndromes, genetic defects, structural anomalies or periodic breathing > 5% of sleep time. RESULTS: Obstructive events > 1 h-1 were evident in 255 infants, of which 91 were eligible for the study. Of the 38 infants in a follow-up study, 30 (79%) were male, 15 (40%) were born prematurely, 25 (66%) had observed apneas, and 13 (33%) had experienced a brief, unexplained event or had a sibling of the infant died suddenly. The first PSG was performed at a median corrected age of 4 weeks (interquartile range [IQR] 2-7) and the second at 11 weeks (IQR 9-14). The obstructive apnea and hypopnea index (OAHI) was greater in the supine compared to side-sleeping position in both recordings (p < 0.001), whereas OAHI dropped from 10 h-1 (IQR 6-24) in the first PSG to 3 h-1 (IQR 1-9) in the second PSG (p < 0.001). OSA alleviation was also observable as a decrease in the number of oxygen desaturations (p < 0.001), as a decrease in transcutaneous (p = 0.001) and end-tidal carbon dioxide (p = 0.01) 95th percentile levels, and work of breathing (p = 0.002). Seven infants had a third PSG to verify a satisfactory improvement of OSA. CONCLUSIONS: OSA in young infants without a clear syndrome or structural anomaly is sleep position dependent and shows improvement during the following few months.

Lymphomas in cartilage-hair hypoplasia - A case series of 16 patients reveals advanced stage DLBCL as the most common form.

Background: Patients with cartilage-hair hypoplasia (CHH) have an increased risk of malignancy, particularly non-Hodgkin lymphoma and basal cell carcinoma. The characteristics, clinical course, response to therapy and outcome of lymphomas in CHH remains unexplored. Methods: We assessed clinical features of lymphoma cases among Finnish patients with CHH. Data were collected from the Finnish Cancer Registry, hospital records, the National Medical Databases and Cause-of-Death Registry of Statistics Finland. Results: Among the 160 CHH patients, 16 (6 men, 10 women) were diagnosed with lymphoma during 1953-2016. Lymphoma was diagnosed in young adulthood (median age 26.4 years, range from 6.4 to 69.5 years), mostly in advanced stage. The most common lymphoma type was diffuse large cell B-cell lymphoma (DLBCL) (6/16, 38%). Eight patients received chemotherapy (8/16, 50%), and two of them survived. Standard lymphoma chemotherapy regimens were administered in the majority of cases. Altogether, eleven CHH patients died due to lymphomas (11/16, 69%). In almost all surviving lymphoma patients, the diagnosis was made either during routine follow-up or after evaluation for non-specific mild symptoms. Search for CHH-related clinical predictors demonstrated higher prevalence of recurrent respiratory infections, in particular otitis media, and Hirschsprung disease in patients with lymphoma. However, three patients had no clinical signs of immunodeficiency prior to lymphoma diagnosis. Conclusion: DLBCL is the most common type of lymphoma in CHH. The outcome is poor probably due to advanced stage of lymphoma at the time of diagnosis. Other CHH-related manifestations poorly predicted lymphoma development, implying that all CHH patients should be regularly screened for malignancy.

Pierre Robin sequence causes position-dependent obstructive sleep apnoea in infants.

INTRODUCTION: Obstructive sleep apnoea (OSA) and feeding difficulties are key problems for Pierre Robin sequence (PRS) infants. OSA management varies between treatment centres. Sleep positioning represents the traditional OSA treatment, although its effectiveness remains insufficiently evaluated. DESIGN: To complete a polysomnographic (PSG) evaluation of effect of sleep position on OSA in PRS infants less than 3 months of age. We analysed a 10-year national reference centre dataset of 76 PRS infants. PSG was performed as daytime recordings for 67 in the supine, side and prone sleeping position when possible. In most cases, recording included one cycle of non-rapid eye movement (NREM) and rapid eye movement (REM) sleep in each position. RESULTS: One-third of infants (9/76, 12%) had severe OSA needing treatment intervention prior to PSG. During PSG, OSA with an obstructive apnoea and hypopnoea index (OAHI) >5 per hour was noted in 82% (55/67) of infants. OSA was most severe in the supine and mildest in the side or in the prone positions. The median OAHI in the supine, side and prone positions were 31, 16 and 19 per hour of sleep (p=0.003). For 68% (52/67) of the infants, either no treatment or positional treatment alone was considered sufficient. CONCLUSIONS: The incidence of OSA was 84% (64/76) including the nine infants with severe OSA diagnosed prior to PSG. For the most infants, the OSA was sleep position dependent. Our study results support the use of PSG in the evaluation of OSA and the use of sleep positioning as a part of OSA treatment.

Comparison of the severity and outcome of invasive pneumococcal infections in children and adults.

In a population-based observational study of 285 patients with positive blood culture for pneumococci, we found that the course and outcome of invasive pneumococcal infections differ considerably between adults and children. None of the children died, whereas the infection was fatal for 15% (35/229) of the adults (P<0.001). The differences are only partly explained by the underlying conditions.