{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T15:13:17Z","timestamp":1789398797537,"version":"build-2803163510"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,4,26]],"date-time":"2022-04-26T00:00:00Z","timestamp":1650931200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Neonatal hemophagocytic lymphohistiocytosis (HLH) is a rare entity. The objective of the study was to describe the prevalence, clinical characteristics, interventions and outcomes of neonates diagnosed with HLH in the United States.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective analysis of 2009, 2012, and 2016 Kids' Inpatient Database was performed. Neonates discharged\/died with a diagnosis of HLH were identified and analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 11,130,055 discharges, 76 neonates had a diagnosis of HLH. Fifty-two percent (95% CI: 38.6\u201363.6) were males and 54% (95% CI: 39.7\u201368.5) were white. Herpes simplex infection was present in 16% (95% CI: 9.2\u201328.1). 24.4% (95% CI: 14.5\u201337.9) received chemotherapy, 11.5% (95% CI: 5.2\u201323.6) IVIG and 3.6% (95% CI: 0.8\u201314.4) allogenic hemopoietic stem cell transplantation. Organ dysfunction was commonly seen and severe sepsis was documented in 26.6% (95% CI: 16.4\u201339.9). Median LOS was 16 (IQR 7\u201354) days. The mortality was 42% (95% CI: 30.8\u201355).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>HLH is a rare diagnosis and carries a high mortality in neonates. Herpes simplex virus is the most common infection associated with neonatal HLH. HLH should be considered in the differential diagnosis in neonates presenting with multi-organ dysfunction or sepsis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2022.848004","type":"journal-article","created":{"date-parts":[[2022,4,26]],"date-time":"2022-04-26T09:00:25Z","timestamp":1650963625000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":11,"title":["Epidemiology and Outcomes of Neonatal Hemophagocytic Lymphohistiocytosis"],"prefix":"10.3389","volume":"10","author":[{"given":"Niveditha","family":"Balakumar","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prithvi","family":"Sendi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Balagangadhar R.","family":"Totapally","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,4,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1007\/s00431-006-0258-1","article-title":"Familial and acquired hemophagocytic lymphohistiocytosis","volume":"166","author":"Janka","year":"2007","journal-title":"Eur J pediatr."},{"key":"B2","doi-asserted-by":"publisher","first-page":"2367","DOI":"10.1182\/blood-2002-01-0172","article-title":"Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation","volume":"100","author":"Henter","year":"2002","journal-title":"Blood."},{"key":"B3","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer."},{"key":"B4","doi-asserted-by":"publisher","first-page":"e27929","DOI":"10.1002\/pbc.27929","article-title":"Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO)","volume":"66","author":"Jordan","year":"2019","journal-title":"Pediatr Blood Cancer."},{"key":"B5","doi-asserted-by":"publisher","first-page":"e316","DOI":"10.1542\/neo.20-6-e316","article-title":"Neonatal hemophagocytic lymphohistiocytosis","volume":"20","author":"McLean","year":"2019","journal-title":"Neoreviews."},{"key":"B6","doi-asserted-by":"publisher","first-page":"S82","DOI":"10.1016\/j.bbmt.2009.11.014","article-title":"Histiocytic disorders: recent insights into pathophysiology and practical guidelines","volume":"16","author":"Filipovich","year":"2010","journal-title":"Biol Blood Marrow Transplant"},{"key":"B7","first-page":"197","article-title":"Hemophagocytic lymphohistiocytosis. 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