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Surfactant Abnormalities in ALTE and SIDS Arch Dis Child: First Published As 10.1136/Adc.71.6.501 on 1 December 1994

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Surfactant Abnormalities in ALTE and SIDS Arch Dis Child: First Published As 10.1136/Adc.71.6.501 on 1 December 1994 Archives ofDisease in Childhood 1994; 71: 501-505 501 Surfactant abnormalities in ALTE and SIDS Arch Dis Child: first published as 10.1136/adc.71.6.501 on 1 December 1994. Downloaded from I B Masters, J Vance, B A Hills Abstract mechanical stability of the distal airspaces.2 Abnormalities in the relative concentra- Reduced concentrations of surfactant, in tions ofthe components ofsurfactant have particular disaturated phosphatidylcholine been implicated in prolonged expiratory (DPPC) have been described in infants apnoea (PEA) and sudden infant death with PEA and SIDS.3-9 Southall et al have syndrome (SIDS). Controversy has, implicated low concentrations of DPPC and however, surrounded these findings, as postulated lung mechanic, neurosensory, and they may be secondary to terminal pulmonary vascular mechanisms for these life events. In this study the physical events.5 6 properties of surfactant were measured in Previously we have shown that both an children with recurrent apparent life infant and young child with prolonged threatening events (ALTEs), PEA, and expiratory apnoea had significant quantitative SIDS. Bronchial lavage samples were and qualitative abnormalities in their obtained from 21 children with recurrent surfactant.'I These findings were similar to the ALTEs, two SIDS victims, and 26 control low concentrations of DPPC found in other patients. Lipid components were immedi- studies.2-5 The reliability of such findings, ately elutriated from these samples however, is questionable in both those and with liquid chloroform. The physical our own observations, as the ability to extract properties of the extracted surfactant surfactant with lung washings in a standardised were studied on a Langmuir trough in fashion from the living subject's lung is which the area (A) of the monolayer was difficult. Despite this, there were clear cut cycled continuously as the surface tension abnormalities and, most importantly, the (-y) was measured by the Wilhelmy results were reproducible over time. This method using a platinum 'flag'. The suggests that there may be a sustained investigators performing these tests were abnormality and that it is not greatly unaware of the clinical diagnosis. Twenty influenced by intervening events, such as one of 23 patients displayed abnormal hypoxia. Our previous study also found greatly physical properties while seven of 26 reduced hysteresis in the relationship between controls displayed similar abnormalities. surface tension (y) and surface area (A), These abnormalities were partially with the normal clockwise -y:A loop actually http://adc.bmj.com/ inverted hysteresis (figure of eight) loops reversing and cycling anticlockwise for the and inverted (anticlockwise) loops that index cases. The reason for these changes is also generally exhibited less hysteresis. Of not clear although inverse hysteresis has the 26 controls 20 exhibited a wide hys- been reported for fatigued monolayers of teresis pattern that cycled in a normal surfactant,'1 while contact angle changes could (clockwise) direction. These differences also produce similar loops.'2 were significantly different. It is con- These initial findings clearly supported the cluded that children with recurrent previous hypotheses of Morley et al 3 and on September 23, 2021 by guest. Protected copyright. ALTEs have definable abnormalities in Southall et al5 6 in that abnormal surfactant the physical properties of surfactant and function may result in altered receptor- that these findings may provide a sensitive controller feedback regulation of functional means of identifying those at risk of residual capacity, thus promoting hypoxia and recurrent ALTEs and SIDS. possibly further disorganising respirations to (Arch Dis Child 1994; 71: 501-505) produce apnoea. While long term prospective studies are being planned, we present our findings from The cause or causes of sudden infant death infants presenting with recurrent or severe syndrome (SIDS) remain a major medical and ALTEs to assess further the likelihood of an scientific dilemma. The National Institutes of association of PEA (prolonged expiratory Health and Human Development cooperative apnoea), ALTEs, and SIDS on the basis of epidemiological study of risk factors suggests surfactant abnormalities. Respiratory Research that up to 7% of SIDS victims have had Unit, Mater prolonged apnoeic episodes or an apparent life Children's Hospital, Annerley Road, threatening event (ALTE) before death.1 Subjects and methods South Brisbane, While the clear inference is that ALTEs may INDEX CASES Queensland 4101, have a causative role in SIDS or represent part All patients were previously investigated by Australia I B Masters of a pathophysiological continuum with SIDS, consultant paediatricians before referral to the J Vance this association is low. respiratory unit for further assessment and B A Hills Anatomical pathology studies in children investigation. The index cases comprised Correspondence to: dying from SIDS implicate disturbance in 23 infants and children admitted to the Dr Masters. micromechanical processes and surfactant Mater Children's Hospital for investigation of Accepted 1 September 1994 function as the major factor responsible for recurrent or severe ALTEs from 1991-3. This 502502~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~Masters,Vance, Hills group represents approximately 30% of primary reason for an anaesthetic. Here the all ALTE patients referred for respiratory bronchoscopy was performed during the same Arch Dis Child: first published as 10.1136/adc.71.6.501 on 1 December 1994. Downloaded from assessment but would represent a much anaesthetic for procedures such as grommet smaller percentage of infants referred to the placement and replacement, cystoscopy, and Mater Children's Hospital for ALTEs over revision of tracheostomy stoma and the airway the same time period. All were well when before decannulation, or because of associated investigated with a bronchoscopic procedure tracheostomy problems such as granuloma. at an elective time at least two weeks after The bronchoscopy was carried out by one their admission for the ALTE. This did of the authors who is an experienced paediatric not include two infants who died from SIDS bronchoscopist. A halothane gaseous anaes- whose samples were obtained by deep thetic was used, the vocal cords sprayed with bronchial lavage through the endotracheal xylocaine and a Pentax 3-5 mm flexible scope tube during the resuscitation process. Four of was passed and wedged into a lower lobe the infants studied were siblings of SIDS bronchus where a bronchial lavage was victims and three of the four had had recurrent performed using 2 ml/kg warm normal saline ALTEs without a definable cause. Two instilled in two aliquots via the wedged had a tracheostomy in situ for 'dynamic' or bronchoscope in the lobar bronchus. Samples 'functional' (non-anatomical) upper airway of the lavage fluid recovered by suction were obstruction. No infant had an acute infective immediately shaken with liquid chloroform to process. There were 12 infants and children in elutriate lipid components of surfactant and whom it was logistically possible to undertake to prevent biochemical degradation. All polysomnographic sleep studies at the time of procedures were carried out with the same hospital investigation for their ALTE. instrument and by the same bronchoscopist. Each sample was assayed by scientists unaware of the diagnosis. DIAGNOSIS OF ALTE The diagnosis of ALTE was defined by a consultant paediatrician in accordance with a CONSENT generally accepted definition' and consisted of Informed parental consent was obtained for a convincing history of the sudden onset of the diagnostic bronchoscopy and broncho- colour change, tone change, and apnoea that alveolar lavage, as well as for the use of these required vigorous stimulation and, as such, investigatory results in the scientific forum. was frightening to the parent or caregiver. As Approval for this study was granted by the most infants were referred for investigation Mater Children's Hospital research and ethics after their initial ALTE, they usually had committee. apnoea alarms in use. Consequently the parental history ofALTE was often augmented those events defined an SURFACTANT ASSAYS: HYSTERESIS TESTS by by apnoea http://adc.bmj.com/ alarm. Other conditions were defined by The lipid and other hydrophobic components clinical assessments and usual investigatory of surfactant in the recovered sample were techniques. extracted from the lavage fluid with chloro- form and stored on ice. The aqueous layer was later decanted and the chloroform evaporated CONTROL CASES to dryness and redissolved in hexane/ethanol Twenty six children acted as controls. Twenty for deposition of the surfactant as a monolayer three had undergone diagnostic bronchoscopy on the saline pool of a Langmuir trough. The on September 23, 2021 by guest. Protected copyright. over the same period of time for disorders such evaluation of physical properties was carried as infantile larynx (laryngomalacia) or other out by a standard procedure whereby the structural abnormalities of the larynx or surface area (A) of the monolayer was cycled airway. Three children had a tracheostomy in over a ratio of 5:1 while its surface tension (-y) situ for severe upper airway obstruction. 507F- BRONCHOSCOPIC PROCEDURE cn 40 In no cases were the bronchoscopies 0) performed 'just to obtain surfactant'. V 30 Indeed all patients were referred from general C unit for 0 paediatricians to the
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