FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2912193148> ?p ?o ?g. }

• W2912193148 endingPage "AB113" @default.
• W2912193148 startingPage "AB113" @default.
• W2912193148 abstract "Ataxia-telangiectasia (AT) is a rare neurodegenerative disorder characterized by ataxia, telangiectasia, and immunodeficiency. We aimed to evaluate the multisystem involvement in AT by describing clinical features and outcomes of Latin American (LA) patients. cross-sectional and multicenter study. Referral centers from all over LA filled in a questionnaire with clinical and laboratory data based on patients’ records. 228 patients from 10 LA countries were evaluated. Mean ages at the time of symptom onset and diagnosis were 1.63±1.09 and 5.66±3.48 years, respectively. The most common immunodeficiency was IgA deficiency (60.8%), followed by IgG deficiency (28.6%). IgA and IgM showed a tendency to decrease as the patient grew older (p=0.001 and 0.048). IgA deficiency was associated with recurrent airway infections (p=0.038). 120 (79,5%) patients presented with low CD3+CD4+ count and 115 (92%) with low CD19+ count. Regarding nutritional status, as patients grow older there is an increase in severe thinness (p=0.016). Median survival was 23 years and Kaplan-Meier 20-year-survival rate was 52,6%. IgG deficiency and female gender were associated with a decrease in estimated survival function (p=0.02 and 0.049). There is a high prevalence of laboratory immunologic abnormalities and recurrent infections in AT patients. Knowledge of specific regional characteristics and variables which can be related with survival allows for suitable patient follow-up and may increase quality of life." @default.
• W2912193148 created "2019-02-21" @default.
• W2912193148 creator A5000953843 @default.
• W2912193148 creator A5003382594 @default.
• W2912193148 creator A5003953902 @default.
• W2912193148 creator A5005756889 @default.
• W2912193148 creator A5006218766 @default.
• W2912193148 creator A5006245490 @default.
• W2912193148 creator A5007855676 @default.
• W2912193148 creator A5009129781 @default.
• W2912193148 creator A5009434359 @default.
• W2912193148 creator A5009960859 @default.
• W2912193148 creator A5010951002 @default.
• W2912193148 creator A5011259618 @default.
• W2912193148 creator A5011696556 @default.
• W2912193148 creator A5011829364 @default.
• W2912193148 creator A5012848520 @default.
• W2912193148 creator A5013029706 @default.
• W2912193148 creator A5014340358 @default.
• W2912193148 creator A5016408770 @default.
• W2912193148 creator A5017545848 @default.
• W2912193148 creator A5017804933 @default.
• W2912193148 creator A5018431760 @default.
• W2912193148 creator A5019924843 @default.
• W2912193148 creator A5022486434 @default.
• W2912193148 creator A5024921525 @default.
• W2912193148 creator A5028152871 @default.
• W2912193148 creator A5029598034 @default.
• W2912193148 creator A5029847547 @default.
• W2912193148 creator A5030615672 @default.
• W2912193148 creator A5030886638 @default.
• W2912193148 creator A5033525325 @default.
• W2912193148 creator A5033934204 @default.
• W2912193148 creator A5034473926 @default.
• W2912193148 creator A5036291818 @default.
• W2912193148 creator A5036566074 @default.
• W2912193148 creator A5045433801 @default.
• W2912193148 creator A5049698621 @default.
• W2912193148 creator A5049922057 @default.
• W2912193148 creator A5051088234 @default.
• W2912193148 creator A5052489314 @default.
• W2912193148 creator A5052951233 @default.
• W2912193148 creator A5055739509 @default.
• W2912193148 creator A5056798189 @default.
• W2912193148 creator A5060120368 @default.
• W2912193148 creator A5062036598 @default.
• W2912193148 creator A5065883353 @default.
• W2912193148 creator A5066194508 @default.
• W2912193148 creator A5066257597 @default.
• W2912193148 creator A5066771691 @default.
• W2912193148 creator A5067307051 @default.
• W2912193148 creator A5070307330 @default.
• W2912193148 creator A5070949393 @default.
• W2912193148 creator A5072974990 @default.
• W2912193148 creator A5073904490 @default.
• W2912193148 creator A5074453125 @default.
• W2912193148 creator A5077651863 @default.
• W2912193148 creator A5083233160 @default.
• W2912193148 creator A5084230650 @default.
• W2912193148 creator A5084803157 @default.
• W2912193148 creator A5085052417 @default.
• W2912193148 creator A5085465333 @default.
• W2912193148 creator A5086219162 @default.
• W2912193148 creator A5089404754 @default.
• W2912193148 creator A5090580248 @default.
• W2912193148 date "2019-02-01" @default.
• W2912193148 modified "2023-10-03" @default.
• W2912193148 title "Ataxia-Telangiectasia: Epidemiological Survey in Latin America" @default.
• W2912193148 doi "https://doi.org/10.1016/j.jaci.2018.12.344" @default.
• W2912193148 hasPublicationYear "2019" @default.
• W2912193148 type Work @default.
• W2912193148 sameAs 2912193148 @default.
• W2912193148 citedByCount "0" @default.
• W2912193148 crossrefType "journal-article" @default.
• W2912193148 hasAuthorship W2912193148A5000953843 @default.
• W2912193148 hasAuthorship W2912193148A5003382594 @default.
• W2912193148 hasAuthorship W2912193148A5003953902 @default.
• W2912193148 hasAuthorship W2912193148A5005756889 @default.
• W2912193148 hasAuthorship W2912193148A5006218766 @default.
• W2912193148 hasAuthorship W2912193148A5006245490 @default.
• W2912193148 hasAuthorship W2912193148A5007855676 @default.
• W2912193148 hasAuthorship W2912193148A5009129781 @default.
• W2912193148 hasAuthorship W2912193148A5009434359 @default.
• W2912193148 hasAuthorship W2912193148A5009960859 @default.
• W2912193148 hasAuthorship W2912193148A5010951002 @default.
• W2912193148 hasAuthorship W2912193148A5011259618 @default.
• W2912193148 hasAuthorship W2912193148A5011696556 @default.
• W2912193148 hasAuthorship W2912193148A5011829364 @default.
• W2912193148 hasAuthorship W2912193148A5012848520 @default.
• W2912193148 hasAuthorship W2912193148A5013029706 @default.
• W2912193148 hasAuthorship W2912193148A5014340358 @default.
• W2912193148 hasAuthorship W2912193148A5016408770 @default.
• W2912193148 hasAuthorship W2912193148A5017545848 @default.
• W2912193148 hasAuthorship W2912193148A5017804933 @default.
• W2912193148 hasAuthorship W2912193148A5018431760 @default.
• W2912193148 hasAuthorship W2912193148A5019924843 @default.
• W2912193148 hasAuthorship W2912193148A5022486434 @default.
• W2912193148 hasAuthorship W2912193148A5024921525 @default.

• next