Hereditary spherocytosis before and after splenectomy and risk of hospitalization for infection.
Journal
Pediatric research
ISSN: 1530-0447
Titre abrégé: Pediatr Res
Pays: United States
ID NLM: 0100714
Informations de publication
Date de publication:
04 2023
04 2023
Historique:
received:
22
04
2022
accepted:
15
07
2022
revised:
12
07
2022
medline:
28
4
2023
pubmed:
2
8
2022
entrez:
1
8
2022
Statut:
ppublish
Résumé
The infectious burden in hereditary spherocytosis (HS) children before splenectomy has rarely been reported and the risk of severe postsplenectomy infection is controversial. We conducted a retrospective study of pediatric patients with HS to evaluate the risk of infection presplenectomy and postsplenectomy. The primary outcome was any bacterial, Mycoplasma, or fungal infection that required hospitalization. The secondary outcomes were sepsis and septic shock. Appendectomized children were matched on age at surgery and enrolled as controls. In all, 232 patients were included. Before splenectomy, the primary outcome was identified in 51 (22.0%) patients, and the secondary outcome was identified in 1 (0.4%) patient. After splenectomy, the primary and secondary outcomes were detected in 8 (4.1%) and 1 (0.5%) patients, respectively. The risk of infection was higher presplenectomy than postsplenectomy (OR, 6.6; 95% CI, 3.0-14.2). HS patients had a higher risk of infection than the controls before surgery (OR, 3.7; 95% CI, 2.3-5.9) but not after surgery (OR, 1.4; 95% CI, 0.6-3.6). HS patients who require splenectomy later in life had a high incidence of hospitalization for infections. In contrast, postsplenectomy risk of hospitalization involving infection or severe infection was low. Patients with hereditary spherocytosis who require splenectomy later in life have a high risk of hospital admission for infections, especially those with severe hereditary spherocytosis. With vaccines or postoperative antibiotics, splenectomy does not increase the risk of infection or severe infections. Splenectomy may reduce the risk of hospitalization for infections by alleviating the complications of hereditary spherocytosis. With vaccines, prophylaxis, or advanced antibiotics, the benefits of splenectomy in children with hereditary spherocytosis and a heavy disease burden may outweigh the risks.
Sections du résumé
BACKGROUND
The infectious burden in hereditary spherocytosis (HS) children before splenectomy has rarely been reported and the risk of severe postsplenectomy infection is controversial.
METHODS
We conducted a retrospective study of pediatric patients with HS to evaluate the risk of infection presplenectomy and postsplenectomy. The primary outcome was any bacterial, Mycoplasma, or fungal infection that required hospitalization. The secondary outcomes were sepsis and septic shock. Appendectomized children were matched on age at surgery and enrolled as controls.
RESULTS
In all, 232 patients were included. Before splenectomy, the primary outcome was identified in 51 (22.0%) patients, and the secondary outcome was identified in 1 (0.4%) patient. After splenectomy, the primary and secondary outcomes were detected in 8 (4.1%) and 1 (0.5%) patients, respectively. The risk of infection was higher presplenectomy than postsplenectomy (OR, 6.6; 95% CI, 3.0-14.2). HS patients had a higher risk of infection than the controls before surgery (OR, 3.7; 95% CI, 2.3-5.9) but not after surgery (OR, 1.4; 95% CI, 0.6-3.6).
CONCLUSIONS
HS patients who require splenectomy later in life had a high incidence of hospitalization for infections. In contrast, postsplenectomy risk of hospitalization involving infection or severe infection was low.
IMPACT
Patients with hereditary spherocytosis who require splenectomy later in life have a high risk of hospital admission for infections, especially those with severe hereditary spherocytosis. With vaccines or postoperative antibiotics, splenectomy does not increase the risk of infection or severe infections. Splenectomy may reduce the risk of hospitalization for infections by alleviating the complications of hereditary spherocytosis. With vaccines, prophylaxis, or advanced antibiotics, the benefits of splenectomy in children with hereditary spherocytosis and a heavy disease burden may outweigh the risks.
Identifiants
pubmed: 35915237
doi: 10.1038/s41390-022-02229-y
pii: 10.1038/s41390-022-02229-y
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1336-1341Informations de copyright
© 2022. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.
Références
Perrotta, S., Gallagher, P. G. & Mohandas, N. Hereditary spherocytosis. Lancet 372, 1411–1426 (2008).
doi: 10.1016/S0140-6736(08)61588-3
pubmed: 18940465
Tse, W. T. & Lux, S. E. Red blood cell membrane disorders. Br. J. Haematol. 104, 2–13 (1999).
doi: 10.1111/j.1365-2141.1999.01130.x
pubmed: 10027705
Yawata, Y. et al. Characteristic features of the genotype and phenotype of hereditary spherocytosis in the Japanese population. Int J. Hematol. 71, 118–135 (2000).
pubmed: 10745622
Delaunay, J. The molecular basis of hereditary red cell membrane disorders. Blood Rev. 21, 1–20 (2007).
doi: 10.1016/j.blre.2006.03.005
pubmed: 16730867
Mohandas, N. & Chasis, J. A. Red blood cell deformability, membrane material properties and shape: regulation by transmembrane, skeletal and cytosolic proteins and lipids. Semin. Hematol. 30, 171–192 (1993).
pubmed: 8211222
Baird, R. N., Macpherson, A. I. & Richmond, J. Red-blood-cell survival after splenectomy in congenital spherocytosis. Lancet 2, 1060–1061 (1971).
doi: 10.1016/S0140-6736(71)90380-1
pubmed: 4106913
Iolascon, A. et al. Recommendations regarding splenectomy in hereditary hemolytic anemias. Haematologica 102, 1304–1313 (2017).
doi: 10.3324/haematol.2016.161166
pubmed: 28550188
pmcid: 5541865
KING, H. & SHUMACKER, H. J. Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann. Surg. 136, 239–242 (1952).
doi: 10.1097/00000658-195208000-00006
pubmed: 14953147
pmcid: 1802258
Eraklis, A. J., Kevy, S. V., Diamond, L. K. & Gross, R. E. Hazard of overwhelming infection after splenectomy in childhood. N. Engl. J. Med. 276, 1225–1229 (1967).
doi: 10.1056/NEJM196706012762203
pubmed: 6024340
Bisharat, N., Omari, H., Lavi, I. & Raz, R. Risk of infection and death among post-splenectomy patients. J. Infect. 43, 182–186 (2001).
doi: 10.1053/jinf.2001.0904
pubmed: 11798256
Cullingford, G. L., Watkins, D. N., Watts, A. D. & Mallon, D. F. Severe late postsplenectomy infection. Br. J. Surg. 78, 716–721 (1991).
doi: 10.1002/bjs.1800780626
pubmed: 2070242
Thomsen, R. W. et al. Risk for hospital contact with infection in patients with splenectomy: a population-based cohort study. Ann. Intern. Med. 151, 546–555 (2009).
doi: 10.7326/0003-4819-151-8-200910200-00008
pubmed: 19841456
Yacobovich, J. et al. Splenectomy in childhood for non-malignant haematologic disorders - long-term follow-up shows minimal adverse effects. Br. J. Haematol. 190, 909–915 (2020).
doi: 10.1111/bjh.16657
pubmed: 32342506
Collaborators, G. U. M. Global, regional, and national progress towards Sustainable Development Goal 3.2 for neonatal and child health: all-cause and cause-specific mortality findings from the Global Burden of Disease Study 2019. Lancet 398, 870–905 (2021).
doi: 10.1016/S0140-6736(21)01207-1
Walker, C. et al. Global burden of childhood pneumonia and diarrhoea. Lancet 381, 1405–1416 (2013).
doi: 10.1016/S0140-6736(13)60222-6
pubmed: 23582727
pmcid: 7159282
Bolton-Maggs, P. H., Langer, J. C., Iolascon, A., Tittensor, P. & King, M. J. Guidelines for the diagnosis and management of hereditary spherocytosis–2011 update. Br. J. Haematol. 156, 37–49 (2012).
doi: 10.1111/j.1365-2141.2011.08921.x
pubmed: 22055020
Singer, M. et al. The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3). JAMA 315, 801–810 (2016).
doi: 10.1001/jama.2016.0287
pubmed: 26903338
pmcid: 4968574
Harris, A. M. et al. Air pollution and anemia as risk factors for pneumonia in Ecuadorian children: a retrospective cohort analysis. Environ. Health 10, 93 (2011).
doi: 10.1186/1476-069X-10-93
pubmed: 22050924
pmcid: 3223143
Kao, J. et al. Early childhood anemia in a birth cohort in coastal Kenya: links to infection and nutrition. Am. J. Trop. Med. Hyg. 101, 242–252 (2019).
doi: 10.4269/ajtmh.17-0688
pubmed: 31074407
pmcid: 6609201
Addiss, D. G., Shaffer, N., Fowler, B. S. & Tauxe, R. V. The epidemiology of appendicitis and appendectomy in the United States. Am. J. Epidemiol. 132, 910–925 (1990).
doi: 10.1093/oxfordjournals.aje.a115734
pubmed: 2239906
Dursun, I., Kiziltan, M. Y., Bozkaya, D., Aygun, A. & Gucuyener, K. Pneumococcal pneumonia preceding appendicitis in a child. Eur. J. Pediatr. 163, 500 (2004).
doi: 10.1007/s00431-004-1479-9
pubmed: 15168113
Pedersen, F. K. Postsplenectomy infections in Danish children splenectomized 1969-1978. Acta Paediatr. Scand. 72, 589–595 (1983).
doi: 10.1111/j.1651-2227.1983.tb09776.x
pubmed: 6624435
Luoto, T. T., Pakarinen, M. P. & Koivusalo, A. Long-term outcomes after pediatric splenectomy. Surgery 159, 1583–1590 (2016).
doi: 10.1016/j.surg.2015.12.014
pubmed: 26832988
Jain, S. et al. Community-acquired pneumonia requiring hospitalization among U.S. children. N. Engl. J. Med. 372, 835–845 (2015).
doi: 10.1056/NEJMoa1405870
pubmed: 25714161
pmcid: 4697461
Levy, A. et al. Anemia as a risk factor for infectious diseases in infants and toddlers: results from a prospective study. Eur. J. Epidemiol. 20, 277–284 (2005).
doi: 10.1007/s10654-004-6515-6
pubmed: 15921046
Martins, R. et al. Heme drives hemolysis-induced susceptibility to infection via disruption of phagocyte functions. Nat. Immunol. 17, 1361–1372 (2016).
doi: 10.1038/ni.3590
pubmed: 27798618
Ibrahim, M. K., Zambruni, M., Melby, C. L. & Melby, P. C. Impact of childhood malnutrition on host defense and infection. Clin. Microbiol. Rev. 30, 919–971 (2017).
doi: 10.1128/CMR.00119-16
pubmed: 28768707
pmcid: 5608884
Bhutta, Z. A. et al. Evidence-based interventions for improvement of maternal and child nutrition: what can be done and at what cost? Lancet 382, 452–477 (2013).
doi: 10.1016/S0140-6736(13)60996-4
pubmed: 23746776
Rubin, L. G. & Schaffner, W. Clinical practice. Care of the asplenic patient. N. Engl. J. Med. 371, 349–356 (2014).
doi: 10.1056/NEJMcp1314291
pubmed: 25054718
Kyaw, M. H. et al. Evaluation of severe infection and survival after splenectomy. Am. J. Med. 119, 271–276 (2006).
doi: 10.1016/j.amjmed.2005.07.044
Yu, Q. et al. The impact of childhood pneumococcal conjugate vaccine immunisation on all-cause pneumonia admissions in Hong Kong: a 14-year population-based interrupted time series analysis. Vaccine 39, 2628–2635 (2021).
doi: 10.1016/j.vaccine.2021.03.090
pubmed: 33858719
Dhiman, N. et al. Increased complications after appendectomy in patients with cerebral palsy: are special needs patients at risk for disparities in outcomes? Surgery 154, 479–485 (2013).
doi: 10.1016/j.surg.2013.05.038
pubmed: 23972654
Holdsworth, R. J., Irving, A. D. & Cuschieri, A. Postsplenectomy sepsis and its mortality rate: actual versus perceived risks. Br. J. Surg. 78, 1031–1038 (1991).
doi: 10.1002/bjs.1800780904
pubmed: 1933181
Jugenburg, M., Haddock, G., Freedman, M. H., Ford-Jones, L. & Ein, S. H. The morbidity and mortality of pediatric splenectomy: does prophylaxis make a difference? J. Pediatr. Surg. 34, 1064–1067 (1999).
doi: 10.1016/S0022-3468(99)90565-1
pubmed: 10442589
Ein, S. H. et al. The morbidity and mortality of splenectomy in childhood. Ann. Surg. 185, 307–310 (1977).
doi: 10.1097/00000658-197703000-00010
pubmed: 557312
pmcid: 1396609