Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Anti- β2 glycoprotein antibodies in patients with antiphospholipid syndrome

RESUMEN: El objetivo de este estudio fue comparar la presencia de anticuerpos anti-β2glicoproteína I (anti-β2GPI) con las pruebas convencionales de laboratorio de anticuerpos anticardiolipina (aCL) y anticoagulante lúpico, y con las manifestaciones clínicas del síndrome antifosfolípido (SAF). Se incluyeron en el estudio 80 mujeres con SAF; 35 de ellas de la consulta de Reumatología y las otras 45 con historia de aborto recurrente espontáneo (ARE); 5 mujeres de la consulta de Reumatología sin SAF, 27 mujeres con ARE, sin SAF y un grupo control de 20 mujeres sanas en edad reproductiva. Se investigaron la presencia de anticuerpos IgG e IgM anticardiolipina (aCL) e IgG anti-β2GPI por la técnica de ELISA, y el anticoagulante lúpico por la determinación del tiempo parcial de tromboplastina activado. Adicionalmente, se registraron las manifestaciones clínicas asociadas al SAF. De las pacientes con SAF, 25.7% del grupo de Reumatología (9/35) y 4.4% de las pacientes con ARE (2/45) fueron positivas para anticuerpos anti- β2GPI, mientras que ninguna de las mujeres sin SAF, ni de las mujeres del grupo control, fue positiva. La asociación entre la presencia de anti-β2GPI y los anticuerpos IgG e IgM aCL mostró una diferencia significativa en los títulos de 3+ (altamente positivos) en contraste con los individuos negativos para anti-β2GPI. La positividad del anticoagulante lúpico también se correlacionó con la presencia de anticuerpos anti-β2GPI. No hubo diferencia significativa entre las diversas manifestaciones clínicas del SAF y la presencia de dichos anticuerpos. En conclusión, la determinación de anticuerpos anti-β2GPI tiene una alta especificidad en pacientes con SAF pero no se asoció con ninguna manifestación clínica en particular.ABSTRACT: The objective of this study was to compare the presence of anti-β2glycoprotein (anti-β2GPI) antibodies with the conventional laboratory tests of anticardiolipin antibodies (aCL) and lupus anticoagulant and with the clinical manifestations of the Antiphospholipid Syndrome (APS). In this study 80 women with APS (35 from the Rheumatology Service and 45 with a history of recurrent spontaneous abortion, RSA) were included, as well as 5 women with rheumatic diseases but no APS, 27 RSA-women without APS and 20 healthy women in their reproductive age. The presence of IgG and IgM anticardiolipin antibodies (aCL), anti-β2GPI antibodies by ELISA method and lupus anticoagulant by the test of activated partial thromboplastin time was investigated. Additionally the clinical manifestations associated to APS were registered. In the group of women with APS, 25.7% (9/35) of those with rheumatic diseases and 4.4% /2/45) of the ones with RSA were positive for anti-β2GPI while none of the women without APS or the controls had such positive reaction. There was a significant association at titers of 3+ (highly positive) between the presence of anti-β2GPI antibodies and IgG and IgM aCL in contrast to anti-β2GPI-negative individuals. The positivity of lupus anticoagulant also correlated with the presence of anti-β2GPI antibodies. There was no significant correlation between any specific clinical manifestation and the presence of anti-β2GPI antibodies. In conclusion, the determination of anti-β2GPI antibodies was highly specific in patients with APS but did not associate with any clinical manifestation of the syndrome

Mecanismos de modulación de la actividad de las células nkpormacrofagosen un modelo murino

IP 1115-05-11491Incluye anexos.PONENCIA(S) EN CONGRESO: Modulation of NK cell activity bysulfated polysaccharides : cytotoxicity and;cytokine production / J. Bueno and A. Cadavid. -- En: Annual Meeting of the Society for Natural Immunity and;International Natural Killer Cell Workshop (8 and 20 : 2004 Apr.24-28 : Noordwijkerhout, The Netherlands). --;Memorias. -- Noordwijkerhout : [s.n.], 2004. -- p. -- Modulaciondel triptofano sobre la actividad de las;celulas NK durante la gestacion en un modelo murino / J.C.Bueno... [et al.]. -- En: Reunion Bienal de la;Asociacion Latinoamericana de Investigadores en Reproduccion Humana (19 :2005 mayo 10-13 : Cartagena de;Indias, Colombia). -- [s.l.] : ALIRH, 2005. -- p. -- ARTICULO(S)EN REVISTA: Metabolismo del triptofano : ñun;elemento mas en el enigma de la tolerancia materna al feto? /Isabel Cristina Avila ... [et al]. -- En:;Revista de la Asociacion Colombiana de Alergia, Asma e Inmunologia. -- Vol. 11, no. 2 -- (jun. 2002); p.;41-46. -- ISSN 01236849 -- Isolation and characterizationof decidual macrophages in long term cultures /;Isabel C. Avila ... [et al.]. -- En: Clinical and investigativemedicine '=Medicine clinique et experimentale.; Vol. 27, no. 4 (2004); p. 141C-141C. -- ISSN 14882353 -'- induccion de la enzima triptofano 2.3 dioxigenasa;por glucocorticoides y su papel en la tolerancia materna al feto/ Lina Cadavid, Angela Cadavid. -- En:;Clinical and investigative medicine '= Medicine clinique etexperimentale.-- Vol. 27, no. 4. -- (Aug. 2004);p. 166-167. -- ISSN 14882353 -- Aislamiento y caracterizaciondemacrofagos deciduales en cultivos de largo;tiempo / Cristina Avila ... [et al]. -- En: Revista de laAsociacion Colombiana de Ciencias Biologicas. --;Vol. 16, no. 2. -- (jul.-dic. 2004); p. 194. -- ISSN 01204173

Determinación de anticuerpos anti-β2glicoproteína I en pacientes con síndrome antifosfolípido Anti- β2 glycoprotein antibodies in patients with antiphospholipid syndrome

EL objetivo de este estudio fue comparar la presencia de anticuerpos anti-β2glicoproteína I (anti- β2GPI) con las pruebas convencionales de laboratorio de anticuerpos anticardiolipina (aCL) y anticoagulante lúpico, y con las manifestaciones clínicas del síndrome antifosfolípido (SAF). Se incluyeron en el estudio 80 mujeres con SAF; 35 de ellas de la consulta de Reumatología y las otras 45 con historia de aborto recurrente espontáneo (ARE); 5 mujeres de la consulta de Reumatología sin SAF, 27 mujeres con ARE, sin SAF y un grupo control de 20 mujeres sanas en edad reproductiva. Se investigaron la presencia de anticuerpos IgG e IgM anticardiolipina (aCL) e IgG anti- β2GPI por la técnica de ELISA, y el anticoagulante lúpico por la determinación del tiempo parcial de tromboplastina activado. Adicionalmente, se registraron las manifestaciones clínicas asociadas al SAF. De las pacientes con SAF, 25.7% del grupo de Reumatología (9/35) y 4.4% de las pacientes con ARE (2/45) fueron positivas para anticuerpos anti-β2GPI, mientras que ninguna de las mujeres sin SAF, ni de las mujeres del grupo control, fue positiva. La asociación entre la presencia de anti- β2GPI y los anticuerpos IgG e IgM aCL mostró una diferencia significativa en los títulos de 3+ (altamente positivos) en contraste con los individuos negativos para anti- β2GPI. La positividad del anticoagulante lúpico también se correlacionó con la presencia de anticuerpos anti- β2GPI. No hubo diferencia significativa entre las diversas manifestaciones clínicas del SAF y la presencia de dichos anticuerpos. En conclusión, la determinación de anticuerpos anti- β2GPI tiene una alta especificidad en pacientes con SAF pero no se asoció con ninguna manifestación clínica en particular. The objective of this study was to compare the presence of anti-β2glycoprotein (anti- β2GPI) antibodies with the conventional laboratory tests of anticardiolipin antibodies (aCL) and lupus anticoagulant and with the clinical manifestations of the Antiphospholipid Syndrome (APS). In this study 80 women with APS (35 from the Rheumatology Service and 45 with a history of recurrent spontaneous abortion, RSA) were included, as well as 5 women with rheumatic diseases but no APS, 27 RSA-women without APS and 20 healthy women in their reproductive age. The presence of IgG and IgM anticardiolipin antibodies (aCL), anti- β2GPI antibodies by ELISA method and lupus anticoagulant by the test of activated partial thromboplastin time was investigated. Additionally the clinical manifestations associated to APS were registered. In the group of women with APS, 25.7% (9/35) of those with rheumatic diseases and 4.4% /2/45) of the ones with RSA were positive for anti- β2GPI while none of the women without APS or the controls had such positive reaction. There was a significant association at titers of 3+ (highly positive) between the presence of anti- β2GPI antibodies and IgG and IgM aCL in contrast to anti- β2GPI-negative individu als. The positivity of lupus anticoagulant also correlated with the presence of anti- β2GPI antibodies. There was no significant correlation between any specific clinical manifestation and the presence of anti- β2GPI antibodies. In conclusion, the determination of anti- β2GPI antibodies was highly specific in patients with APS but did not associate with any clinical manifestation of the syndrome