Fatal pertussis infection - Swiss Society of Neonatology

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16 results in a hyperviscosity syndrome which can lead to impaired microcirculation and organ failure. In addition to hyperviscosity, obstruction of pulmonary arterioles by leukocyte thrombi may result in severe pulmonary hypertension as a prominent feature (5). Furthermore, pertussis toxin can mediate an increase of cAMP levels which contributes to pulmonary vaso constriction. Fi- nally, aggregation and activation of lymphocytes and neutrophils in the pulmonary vascular bed may aggra- vate acute lung injury. Postmortem studies have found cilia loss in airway mucosa, necrotizing bronchiolitis, and focal or diffuse bronchopneumonia (5). Aggre- gates of leukocytes in pulmonary arterioles, small ar- teries and venules and occlusive thrombi are frequent postmortem findings, as observed in the presented case. The management of severe pulmonary hypertension in pertussis patients is extremely challenging, since clas- sic approaches at reducing pulmonary vascular resi- stance such as iNO may fail due to hyperviscosity and vascular obstruction. The observation of a high mortality in infants presenting with hyperleukocytosis has therefore lead to strategies for leukoreduction (4). The GOSH protocol recommends leukofiltration in patients requiring ECMO for cardiorespiratory failure with WCC above 50 x10 9 /L. In patients that do not require ECMO, urgent double-volume blood exchange trans- fusion is advocated if the WCC is above 100 x10 9 /L, or above 70 x10 9 /L and cardiorespiratory failure or se-
17 vere pulmonary hypertension are present. Comparing a historical cohort of nine infants under 90 days of age with severe pertussis managed without leukoreduction versus a recent group of ten infants treated according to this protocol, the group at GOSH observed improved survival from 55% to 90%. To date, this small non-randomized study remains the only report on a significant treatment benefit for severe pertussis. ECMO is a valid treatment option in the setting of refractory pulmonary hypertension due to a poten- tially reversible cause (such as meconium aspiration syndrome or sepsis), and has therefore been used in patients with pertussis-induced cardiorespiratory failure (9). The Extracorporeal Life Support Organisati- on (ELSO) registry recorded 169 pertussis cases that were treated with ECMO between 1992 to 2009 (4). The prognosis of severe pertussis requiring ECMO was worse compared to other indications for respiratory ECMO (mortality of 70% compared to a mortality of 20% for respiratory syncytial virus bronchiolitis). The highest case fatality rate (84%) was observed in infants below 6 weeks of age. ECMO may be required for several weeks, which leads to a high risk of ECMO- related life-threatening complications such as sepsis, major bleeding or thromboembolic events. Recent serological follow-up data from vaccine cen- ters suggests that the protection after full immuniza- tion using the acellular pertussis vaccine wanes faster
Page 1 and 2: SWISS SOCIETY OF NEONATOLOGY Fatal
Page 3 and 4: 3 Before the widespread implementat
Page 5 and 6: 5 Over the next two hours, the cond
Page 7 and 8: 7 Chest X-ray after cannulation for
Page 9 and 10: 9 Chest X-ray on day 25 of ECMO: di
Page 11 and 12: 11 Twelve hours after admission to
Page 13 and 14: 13 Postmortem lung histology: acute
Page 15: 15 Bordatella pertussis, a gram-neg
Page 19 and 20: 19 1. Klein NP, Bartlett J, Rowhani
Page 22: SUPPORTED BY CONTACT Swiss Society

Fatal pertussis infection - Swiss Society of Neonatology

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