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Abstract Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles and elbows). This document is a review and consensus of the main genetic aspects of hemophilia, from the inheritance pattern to the concept of women carriers, physiopathology and classification of the disorder, the basic and confirmation studies when hemophilia is suspected, the various treatment regimens based on infusion of the deficient coagulation factor as well as innovative factor-free therapies and recommendations for the management of complications associated with treatment (development of inhibitors and/or transfusion transmitted infections) or secondary to articular hemorrhagic events (hemophilic arthropathy). Finally, relevant reviews of clinical and treatment aspects of hemorrhagic pathology charachterized by acquired deficiency of FVIII secondary to neutralized antibodies named acquired hemophilia.

OBJECTIVES: To document the clinical and epidemiological characteristics present in dengue patients served by a hospital in Culiacan, Sinaloa, Mexico. METHODS: A cross-sectional, observational, and analytical study was carried out at the Hospital General Dr. Bernardo J. Gastélum de Culiacan from 1 October to 2 December 2003. Associations between the independent variables (the patients' clinical and epidemiological characteristics) and the dependent variable (confirmed hemorrhagic dengue) were determined through simple regression analysis. The variables that were significantly associated (P < 0.05) were submitted to multifactorial logistic regression analysis. RESULTS: Of the only 241 cases that met the study's inclusion criteria (207 dengue and 34 hemorrhagic dengue), the mean age was 34.7 ± 15.1 years. According to the results of the multifactorial analysis (adjusted by age, sex, and the presence of dengue cases at the geographic location), the variables predictive of major complications of the disease were: the presence of ascites (odds ration [OR] = 22.12; 95% confidence interval [95%CI]: 5.00-97.87), gingivorrhagia (OR = 7.35; 95%CI: 2.11-25.61), hematemesis (OR = 7.40; 95%CI: 1.04-52.42), thrombocytopenia (platelets from 40001/mm³- 60000/mm³) (OR = 5.43; 95%CI: 1.58-18.72), conjunctival hyperemia (OR = 4.27; 95%CI: 1.37-13.28), persistent vomiting (OR = 3.04; 95%CI: 1.05-8.80), and the absence of nasal congestion (OR = 0.015; 95%CI: 0.0004-0.473). CONCLUSIONS: The presence of ascites, gingivorrhagia, hematemesis, thrombocytopenia (with platelet values from 40001/mm³-60000/mm³), and persistent vomiting were confirmed as warning signs of an imminent dengue attack. Platelet counts of > 100000/mm3 were confirmed in cases with acute clinical symptoms (capillary leak) that were not classified as hemorrhagic dengue due to falling short of the criteria established by WHO.

OBJETIVO: Documentar las características clínicas y epidemiológicas de los pacientes con dengue atendidos en un hospital de Culiacán, Sinaloa, México. MÉTODOS: Estudio transversal, observacional y analítico realizado en el Hospital General Dr. Bernardo J. Gastélum de Culiacán entre el 1 de octubre y el 2 de diciembre de 2003. Se evaluó la asociación entre las variables independientes (las características clínicas y epidemiológicas de los pacientes) y la variable dependiente (diagnóstico confirmado de dengue hemorrágico) mediante el análisis de regresión simple. Las variables que mostraron una relación significativa (P < 0,05) se incluyeron en el análisis de regresión logística multifactorial. RESULTADOS: En los 241 casos que cumplieron los criterios de inclusión para este estudio (207 de dengue y 34 de dengue hemorrágico), la edad promedio fue de 34,7 ± 15,1 años. Según los resultados del análisis multifactorial ajustado por la edad, el sexo y la presencia de casos de dengue en la localidad, las variables con valor predictivo de una mayor gravedad de la enfermedad fueron: la presencia de ascitis (OR = 22,12; IC95%: 5,00 a 97,87), la gingivorragia (OR = 7,35; IC95%: 2,11 a 25,61), la hematemesis (OR = 7,40; IC95%: 1,04 a 52,42), la trombocitopenia (plaquetas entre 40 001/mm³ y 60 000/mm³) (OR = 5,43; IC95%: 1,58 a 18,72), la hiperemia conjuntival (OR = 4,27; IC95%: 1,37 a 13,28), los vómitos persistentes (OR = 3,04; IC95%: 1,05 a 8,80) y la ausencia de congestión nasal (OR = 0,015; IC95%: 0,0004 a 0,473). CONCLUSIONES: Se confirmó el valor de la presencia de ascitis, gingivorragia, hematemesis, trombocitopenia (con valores de plaquetas entre 40 001/mm³ y 60 000/mm³) y vómitos persistentes como signos de alarma que anuncian la inminencia del choque por dengue. Se observaron conteos plaquetarios > 100 000/mm3 en casos con cuadros clínicos graves (fuga capilar) que no se clasificaron como dengue hemorrágico por no cumplir todos los criterios establecidos por la OMS.

Index code	Element
ti	article title
au	author
kw	article keywords
subject	subject (title words, abstract and keywords)
ab	abstract
ta	journal short title (e.g. Cad. Saúde Pública)
journal_title	journal full title (e.g. Cadernos de Saúde Pública)
la	publication language code (e.g. pt - Portuguese, es - Spanish)
type	document type
pid	publication identifier
publication_year	publication year of publication
sponsor	sponsor
aff_country	country code of the author's affiliation
aff_institution	author affiliation institution
volume	article volume
issue	article issue
elocation	elocation
doi	DOI number
issn	journal ISSN
in	SciELO colection code (e.g. scl - Brasil, col - Colômbia)
use_license	article usage license code

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