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Çocuklarda enfeksiyon ilişkili hemofagositik sendrom: 15 vakanın geriye dönük analizi

Year 2018, Volume: 11 Issue: 1, 57 - 63, 01.01.2018

Abstract

GİRİŞ ve AMAÇ: Hemofagositik sendrom (HFS) aşırı immün aktivasyona bağlı hızla ilerleyen ve hayatı tehdit eden bir sendromdur. HFS ile ilişkili klinik tablo ve laboratuvar anormalliklerinin erken tanınması ve tedaviye hemen başlanması hayat kurtarıcı olabilmektedir. Bu çalışmada enfeksiyon ilişkili hemofagositik sendrom (EİHFS) tanısıyla izlediğimiz çocuk hastalarda; altta yatan enfeksiyöz etkenlerin, klinik, laboratuvar bulguların ve tedavi sonuçlarının incelenmesi amaçlanmıştır.YÖNTEM ve GEREÇLER: Aralık 2012 ile Ocak 2016 arasında EİHFS tanısı konan çocuk hastaların kayıtları geriye dönük olarak incelendi.BULGULAR: Çalışmaya yaşları ortanca 6 yaş (2-16 yaş) olan toplam 15 hasta (8 erkek, 7 kız) dahil edildi. HFS’yi tetikleyen en sık enfeksiyöz etken olarak Brusella (8 hasta, %53) saptandı. Diğer altta yatan enfeksiyöz etkenler; 3 hastada Leishmania, 1 hastada Salmonella Typhi, 1 hastada Mycobacterium tuberculosis, 1 hastada parvovirüs B19 ve 1 hastada influenza A (H3N2) idi. Ateş, bi/pansitopeni, hiperferritinemi ve serum transaminaz düzeylerinde yükseklik tüm hastalarda (%100), splenomegali 12 hastada (%80) mevcuttu. Tüm hastaların kemik iliği aspirasyon incelemesinde hemofagositoz görüldü. Tüm hastalara altta yatan enfeksiyonuna yönelik antimikrobiyal tedavi; uzamış ateş ve klinik durumunda giderek kötüleşme olan 8 hastaya intravenöz immünglobülin (IVIG), 2 hastaya IVIG+steroid tedavileri verildi. Olguların %93’ünde (14 hasta) iyileşme kaydedildi. İnfluenza A (H3N2) pnömonisi olan sadece 1 hastada (%6) ölüm görüldü. TARTIŞMA ve SONUÇ: Hekimler, enfeksiyon nedeniyle izlenen hastalarda uzamış ateş, splenomegali ve sitopeninin varlığında HFS gelişimi konusunda farkındalığa sahip olmalıdır.

Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases

Year 2018, Volume: 11 Issue: 1, 57 - 63, 01.01.2018

Abstract

INTRODUCTION: Haemophagocytic syndrome (HPS) is a rapidly progressive, life-threatening syndrome of excessive immune activation. Early recognition of the clinical presentation and laboratory abnormalities associated with HPS and prompt initiation of treatment can be life saving. The aim of this study was to investigate the infectious etiologies, clinical and laboratory characteristics, and treatment outcomes of pediatric patients with infection associated hemophagocytic syndrome (IAHPS).METHODS: This is a retrospective record review of pediatric patients diagnosed to have IAHFS between December 2012 and January 2016.RESULTS: A total of 15 pediatric patients with IAHPS (8 boys / 7 girls; median age: 6 years, range 2-16 years) were enrolled in this study. Brucella (8 patients, 53%) was the most frequent infectious trigger of HFS. Other infectious triggers were Leishmania (3 patients), Salmonella Typhi (1 patient), Mycobacterium tuberculosis (1 patient), parvovirus B19 (1 patient), and influenza A (H3N2) (1 patient). Fever, bi/pansitopenia, hyperferritinemia, and elevated transaminases were present in all patients (100%), and splenomegaly in 12 patients (80%). All of the patients underwent bone marrow aspirations that confirmed hemophagocytosis. All patients received antimicrobial treatment for the underlying infections; 8 patients received intravenous immunoglobulin (IVIG) and 2 patients received IVIG+steroids due to persistent fever and progressively worsening clinical conditions. Recovery was noted in 14 (93%) patients. Mortality was only seen in 1 patient (6%) with influenza A (H3N2) pneumonia.DISCUSSION AND CONCLUSION: Physicians should be aware of the occurrence of HFS in patients with prolonged fever, splenomegaly, and cytopenias in the setting of an infectious process.

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Details

Other ID JA33BF45FG
Journal Section Research Article
Authors

Meltem Polat This is me

Fatma Burcu Belen This is me

Publication Date January 1, 2018
Submission Date January 1, 2018
Published in Issue Year 2018 Volume: 11 Issue: 1

Cite

APA Polat, M., & Belen, F. B. (2018). Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases. Pamukkale Medical Journal, 11(1), 57-63.
AMA Polat M, Belen FB. Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases. Pam Med J. January 2018;11(1):57-63.
Chicago Polat, Meltem, and Fatma Burcu Belen. “Infection Associated Hemophagocytic Syndrome in Children: A Retrospective Analysis of 15 Cases”. Pamukkale Medical Journal 11, no. 1 (January 2018): 57-63.
EndNote Polat M, Belen FB (January 1, 2018) Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases. Pamukkale Medical Journal 11 1 57–63.
IEEE M. Polat and F. B. Belen, “Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases”, Pam Med J, vol. 11, no. 1, pp. 57–63, 2018.
ISNAD Polat, Meltem - Belen, Fatma Burcu. “Infection Associated Hemophagocytic Syndrome in Children: A Retrospective Analysis of 15 Cases”. Pamukkale Medical Journal 11/1 (January 2018), 57-63.
JAMA Polat M, Belen FB. Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases. Pam Med J. 2018;11:57–63.
MLA Polat, Meltem and Fatma Burcu Belen. “Infection Associated Hemophagocytic Syndrome in Children: A Retrospective Analysis of 15 Cases”. Pamukkale Medical Journal, vol. 11, no. 1, 2018, pp. 57-63.
Vancouver Polat M, Belen FB. Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases. Pam Med J. 2018;11(1):57-63.

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