Post Graduate Courses Internationaal Ped. Pulmo.Lisbon 2017.pdf

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2) Amaddeo A, Frapin A, Fauroux B. Long-term non-invasive

with the presence of SDB. Young children and children with

ventilation in children. Lancet Respir Med. 2016 Dec;4(12):999-1008.

underlying syndromes are especially at risk of severe OSA and its

3) Mehta S, Hill NS. Noninvasive ventilation, state of art. Am J Respir

possible complications. In the context of SDB symptoms and

Crit Care Med 2001;163:540-77.

underlying syndromes with an inherent risk of OSA, the presence

4) Racca F, Berta G, Sequi M, Bignamini E, Capello E, Cutrera R,
Ottonello G, Ranieri VM, Salvo I, Testa R, Wolfler A, Bonati M; LTV
Pediatric Italian Network. Long-term home ventilation of children in
Italy: a national survey. Pediatr Pulmonol. 2011 Jun;46(6):566-72.
5) Wallis C, Paton JY, Beaton S, Jardine E. Children on long-term
ventilatory support: 10 years of progress. Arch Dis Child. 2011 Nov;96

of failure to thrive and pulmonary hypertension are certainly
indicative for the presence of OSA. Polygraphy or polysomnography,
which is still the gold standard for the diagnosis of OSA, should be
performed to document the presence and severity of OSA.
Polygraphy and polysomnography provides us with the number of
obstructive events per hour of sleep (the obstructive apnea
hypopnea index, oAHI). Moderate-to-severe OSA is defined as an
oAHI>5. To date, there are no other screening tools that can

6) Kherani T, Sayal A, Al-Saleh S, Sayal P, Amin R. A comparison of

substitute polysomnography. However, some of these tools, for

invasive and noninvasive ventilation in children less than 1 year of

instance nocturnal oximetry, have their value considering their

age: A long-term follow-up study. Pediatr Pulmonol. 2016 Feb;51

inherent limitations.


Moderate-to-severe OSA is an indication for treatment

7) Chatwin M, Tan HL, Bush A, Rosenthal M, Simonds AK. Long Term

irrespective of the presence of morbidity. Especially in patients

Non-Invasive Ventilation in Children: Impact on Survival and Transi-

with underlying syndromes, treatment is a priority because these

tion to Adult Care. PLoS ONE 10(5):e0125839.

children have a higher risk of developing serious complications

8) Chau SK, Yung AW, Lee SL. Long-Term Management for VentilatorAssisted Children in Hong Kong: 2 Decades’ Experience. Respir Care.

including pulmonary hypertension. In the Task Force document, an
algorithm is presented guiding treatment from the least invasive
(pharmacological treatment) to the most invasive (tracheostomy).

2017 Jan;62(1):54-64.

Especially in children with underlying conditions, it is important to
9) Han YJ, Park JD, Lee B, Choi YH, Suh DI, Lim BC, Chae JH. Home
mechanical ventilation in childhood-onset hereditary neuromuscular
diseases: 13 years’ experience at a single center in Korea. PLoS One.
2015 Mar 30;10(3):e0122346.

identify the site(s) of upper airway obstruction. These children
might benefit from adenotonsillectomy, although residual disease
is highly prevalent with the need for additional treatment including
orthodontics, maxillofacial surgery and non-invasive ventilation.

10) Nathan AM, Loo HY, de Bruyne JA, Eg KP, Kee SY, Thavagnanam S,

Because of increasingly available devices and especially interfaces

Bouniu M, Wong JE, Gan CS, Lum LC. Thirteen Years of Invasive and

for non-invasive ventilation in children, this option is being

Noninvasive Home Ventilation for Children in a Developing Country: A

increasingly used in specialized centers. It is important after

Retrospective Study. Pediatr Pulmonol. 2016 Oct 6. doi: 10.1002/

each treatment and with increasing age to follow the child with


moderate-to-severe OSA to objectify if OSA is still present.
1. Kaditis A, Kheirandish-Gozal L, Gozal D. Algorithm for the diagnosis
and treatment of pediatric OSA: a proposal of two pediatric sleep

Management of Complex OSA in Children.

centers. Sleep medicine. 2012;13(3):217-227.

Stijn Verhulst

2. Katz ES, D’Ambrosio CM. Pathophysiology of pediatric obstructive sleep

Head of Department of Pediatrics, Pediatric Pulmonology and Sleep Medicine,
Antwerp University Hospital, Belgium

apnea. Proceedings of the American Thoracic Society. 2008;5(2):253-262.
3. American Thoracic Society. Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med.

Sleep-disordered breathing (SDB) is a prevalent disease in pediatrics. It


is not a distinct disease, but rather a syndrome of upper airway

4. Dayyat E, Kheirandish-Gozal L, Gozal D. Childhood Obstructive

dysfunction during sleep characterized by snoring and/or increased

Sleep Apnea: One or Two Distinct Disease Entities? Sleep Med Clin.

respiratory effort secondary to increased upper airway resistance and


pharyngeal collapsibility


. SDB includes a spectrum of clinical entities

5. Kaditis AG, Alonso Alvarez ML, Boudewyns A, et al. Obstructive

with variable severity of intermittent upper airway obstruction ranging

sleep disordered breathing in 2- to 18-year-old children: diagnosis and

from habitual snoring to severe obstructive sleep apnea (OSA)3,4. In

management. The European respiratory journal. 2016;47(1):69-94.

2016, the results of a European Respiratory Society Task Force on the
diagnosis and management of pediatric OSA were published5. The
main recommendations of this paper concerning severe OSA will be

PLTNIV in Children with Neuromuscular Diseases

presented in this summary.

Rosário Ferreira

In a first step, it is important to recognize the child with possible
severe OSA. Certain symptoms such as frequent loud snoring,
witnessed apneas, restless sleep and oral breathing are associated

Pediatric Respiratory Unit, Department of Pediatrics − Santa Maria HospitalCHLN, Academic Medical Center of Lisbon