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249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis
OBJECTIVE: Identify risk factors associated to mortality in pediatric patients with hemophagocytic lymphohistiocytosis. METHODS: Retrospective cross-sectional study of medical records with discharge diagnosis of Hemophagocytic syndrome/Hemophagocytic lymphohistiocytosis (ICD-10; D76.1/D76.2) from Ja...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
World Allergy Organization Journal
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3512708/ http://dx.doi.org/10.1097/01.WOX.0000412006.76608.67 |
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author | Partida-Gaytan, Armando del Rio, Blanca Tercero-Quintanilla, Gabriela Rosas-Vargas, Miguel Angel |
author_facet | Partida-Gaytan, Armando del Rio, Blanca Tercero-Quintanilla, Gabriela Rosas-Vargas, Miguel Angel |
author_sort | Partida-Gaytan, Armando |
collection | PubMed |
description | OBJECTIVE: Identify risk factors associated to mortality in pediatric patients with hemophagocytic lymphohistiocytosis. METHODS: Retrospective cross-sectional study of medical records with discharge diagnosis of Hemophagocytic syndrome/Hemophagocytic lymphohistiocytosis (ICD-10; D76.1/D76.2) from Jan2004-May2011 in a pediatric-tertiary-care-center. Descriptive and risk analysis were made on SPSS Statistics V17.0. RESULTS: Thirty medical records were analyzed. Median-for-age: 2 years 8 months, (range: 2 months-to-15 years). Sex distribution: 14 girls (47%), 16 boys (53%). Median of symptoms duration: 1 month (range: 3 days-to-7 years). Reported symptoms and physical signs at hospital admission: fever n = 28 (93%), asthenia/adynamia n = 11 (37%), skin findings n = 10 (33%), epistaxis n = 5 (17%), gastrointestinal bleeding n = 4 (13%), hepatomegaly n = 27 (90%), splenomegaly n = 21 (70%), lymphadenopathies n = 14 (47%), paleness n = 14 (47%), purpura n = 5 (17%). Laboratory findings: anemia n = 29 (97%), LDH elevation n = 28 (93%), hypoalbuminemia n = 27 (90%), thrombocytopenia n = 26 (87%), hypertransaminasemia n = 25 (83%), haemophagocytosis n = 22 (73%), hypertrigliceridemia n = 21 (70%), hypofibrinogenemia n = 20 (67%), leucopenia n = 19 (63%), hyperferritinemia n = 15(50%). In 18 patients (60%) active infection was evident at hospital admission: pneumonia n = 9(50%), gastroenteritis n = 2 (11%), meningitis n = 1 (5%), others n = 6 (33%). Epstein-Barr virus infection was diagnosed in 7 patients (23%). All patients were treated according to HLH-2004 guidelines. Overall mortality 63% (n = 19), 9(47%) died from septic-shock, 7 (36%) haemorraghic-shock, and 1(5%) with acute liver failure. Differences between non-survivours and survivours by (x(2)): hypofibrinogenemia (53%versus 13%; P = 0.039), epistaxis (17% versus 0%; P = 0.023), evident clinical infection (47%versus 13%; P = 0.044), elevated LDH levels (63% versus 30%; P = 0.039), hemophagocytosis (57% versus 17%; P = 0.024). Risk factors associated to mortality: history of epistaxis (OR = 1.78, 95% CI, 1.26-2.52; P = 0.023), evident clinical infection at hospital admission (OR = 2.41, 95% CI, 1.08-5.8; P = 0.044). Normal levels of LDH showed diminished mortality risk (OR = 0.32, 95%, CI, 0.18-0.55; P = 0.039). CONCLUSIONS: The present study describes the most common clinical, physical and laboratory findings in patients with haemophagocytic lymphohistiocytosis attended in our hospital. We were able to identify risk factors associated to mortality, and 1 protective factor. |
format | Online Article Text |
id | pubmed-3512708 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | World Allergy Organization Journal |
record_format | MEDLINE/PubMed |
spelling | pubmed-35127082012-12-21 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis Partida-Gaytan, Armando del Rio, Blanca Tercero-Quintanilla, Gabriela Rosas-Vargas, Miguel Angel World Allergy Organ J Abstracts of the XXII World Allergy Congress OBJECTIVE: Identify risk factors associated to mortality in pediatric patients with hemophagocytic lymphohistiocytosis. METHODS: Retrospective cross-sectional study of medical records with discharge diagnosis of Hemophagocytic syndrome/Hemophagocytic lymphohistiocytosis (ICD-10; D76.1/D76.2) from Jan2004-May2011 in a pediatric-tertiary-care-center. Descriptive and risk analysis were made on SPSS Statistics V17.0. RESULTS: Thirty medical records were analyzed. Median-for-age: 2 years 8 months, (range: 2 months-to-15 years). Sex distribution: 14 girls (47%), 16 boys (53%). Median of symptoms duration: 1 month (range: 3 days-to-7 years). Reported symptoms and physical signs at hospital admission: fever n = 28 (93%), asthenia/adynamia n = 11 (37%), skin findings n = 10 (33%), epistaxis n = 5 (17%), gastrointestinal bleeding n = 4 (13%), hepatomegaly n = 27 (90%), splenomegaly n = 21 (70%), lymphadenopathies n = 14 (47%), paleness n = 14 (47%), purpura n = 5 (17%). Laboratory findings: anemia n = 29 (97%), LDH elevation n = 28 (93%), hypoalbuminemia n = 27 (90%), thrombocytopenia n = 26 (87%), hypertransaminasemia n = 25 (83%), haemophagocytosis n = 22 (73%), hypertrigliceridemia n = 21 (70%), hypofibrinogenemia n = 20 (67%), leucopenia n = 19 (63%), hyperferritinemia n = 15(50%). In 18 patients (60%) active infection was evident at hospital admission: pneumonia n = 9(50%), gastroenteritis n = 2 (11%), meningitis n = 1 (5%), others n = 6 (33%). Epstein-Barr virus infection was diagnosed in 7 patients (23%). All patients were treated according to HLH-2004 guidelines. Overall mortality 63% (n = 19), 9(47%) died from septic-shock, 7 (36%) haemorraghic-shock, and 1(5%) with acute liver failure. Differences between non-survivours and survivours by (x(2)): hypofibrinogenemia (53%versus 13%; P = 0.039), epistaxis (17% versus 0%; P = 0.023), evident clinical infection (47%versus 13%; P = 0.044), elevated LDH levels (63% versus 30%; P = 0.039), hemophagocytosis (57% versus 17%; P = 0.024). Risk factors associated to mortality: history of epistaxis (OR = 1.78, 95% CI, 1.26-2.52; P = 0.023), evident clinical infection at hospital admission (OR = 2.41, 95% CI, 1.08-5.8; P = 0.044). Normal levels of LDH showed diminished mortality risk (OR = 0.32, 95%, CI, 0.18-0.55; P = 0.039). CONCLUSIONS: The present study describes the most common clinical, physical and laboratory findings in patients with haemophagocytic lymphohistiocytosis attended in our hospital. We were able to identify risk factors associated to mortality, and 1 protective factor. World Allergy Organization Journal 2012-02-17 /pmc/articles/PMC3512708/ http://dx.doi.org/10.1097/01.WOX.0000412006.76608.67 Text en Copyright © 2012 by World Allergy Organization |
spellingShingle | Abstracts of the XXII World Allergy Congress Partida-Gaytan, Armando del Rio, Blanca Tercero-Quintanilla, Gabriela Rosas-Vargas, Miguel Angel 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title | 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title_full | 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title_fullStr | 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title_full_unstemmed | 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title_short | 249 Risk Factors Associated to Mortality in Pediatric Patients with Hemophagocytic Lymphohistiocytosis |
title_sort | 249 risk factors associated to mortality in pediatric patients with hemophagocytic lymphohistiocytosis |
topic | Abstracts of the XXII World Allergy Congress |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3512708/ http://dx.doi.org/10.1097/01.WOX.0000412006.76608.67 |
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