Browsing by Author "Borzutzky Schachter, Arturo"
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- ItemAsociación temporal entre la circulación de virus respiratorios y hospitalizaciones por enfermedad de Kawasaki(Sociedad Chilena de Infectologia, 2021) Aguirre, Daniela; Cerda, Jaime; Perret, Cecilia; Borzutzky Schachter, Arturo; Hoyos-Bachiloglu© 2021, Sociedad Chilena de Infectologia. All rights reserved.Background: The etiology of Kawasaki disease (KD) is unknown. It is believed that viral infections could trigger the disease in susceptible patients. Aim: To study the temporal association between the circulation of respiratory viruses and KD hospitalizations in the Metropolitan Region (MR), Chile, between 2010-2017. Methods: Ecologic study consisting of a review of KD cases in children under 18 years of age based on hospital discharges. The circulation of respiratory viruses was analyzed using the report of the metropolitan surveillance network. Moving averages for KD (MAKD) and respiratory viruses (MARV) were used. Results: 14,902 cases of respiratory virus infections were recorded between 2010-2017. A direct correlation was found between MARV-respiratory syncytial virus (RSV) of one month and year and MAKD of the subsequent month (correlation coefficient (ρ) = +0.441; p < 0.001). A similar association was found for MARV-influenza A (FLU A) (ρ = + 0.362; p < 0.001). MARV-influenza B (FLU B) and MARV-metapneumovirus (MPV) had direct correlations with MAKD (ρ = +0.443; p < 0.001 and ρ = +0.412; p < 0.001, respectively), being FLU B contemporary in month and year with KD, and MPV presenting a one-month lag. Conclusion: There is a direct temporal correlation between RSV, FLU A, FLU B and MPV circulation and KD in children from RM, Chile.
- ItemAssociation between obesity and atopic dermatitis in children: A case-control study in a high obesity prevalence population(2022) Iturriaga, Carolina; Bustos, María Francisca; Le Roy, Catalina; Rodríguez, Rocío; Cifuentes, Lorena; Silva-Valenzuela, Sergio; Vera-Kellet, Cristián; Cristi, Francisca; Pérez-Mateluna, Guillermo; Cabalín, Carolina; Hoyos-Bachiloglu, Rodrigo; Camargo Jr., Carlos A.; Borzutzky Schachter, ArturoBackground/Objective: Atopic dermatitis (AD) is a chronic inflammatory skin disease. Research suggests an association between obesity and AD, although evidence is lacking from Latin American populations. This study evaluated the association of obesity with AD in children from Chile, a country with high obesity prevalence. Methods: A case-control study was performed in children with active AD (cases) and healthy controls (HCs) from Santiago, Chile. Body mass index was evaluated by z-score (z-BMI), with overweight defined as z-BMI ≥+1 and <+2, and obesity as z-BMI ≥+2. Abdominal obesity was defined by a waist circumference-to-height ratio (WHR) ≥0.5. AD severity was evaluated by Scoring AD (SCORAD) index. Results: A total of 174 children with AD and 101 controls were included. AD patients had similar overweight (27% vs. 28%) and obesity (21% vs. 26%) rates as HCs (p = .65). Abdominal obesity rates were also comparable (64% vs. 62%, p = .81). In sex-specific analyses, girls with AD had higher abdominal obesity rates than HCs (71% vs. 53%, p < .05) while boys with AD had lower abdominal obesity rates than HCs (53% vs. 75%, p = .03). Among children with AD, higher z-BMI or WHR did not correlate with higher SCORAD, eosinophil counts or total IgE. Conclusion: In our study, Chilean children with AD had high but similar rates of obesity as HCs, but showed sex-specific associations of abdominal obesity and AD. Further research is needed to evaluate these associations and the roles that weight excess and weight loss could play in the pathogenesis and treatment of AD.
- ItemAtopic Dermatitis in Latin America: a Roadmap to Address Data Collection, Knowledge Gaps, and Challenges Ahead(2022) Borzutzky Schachter, Arturo; Larco, José Ignacio; Luna, Paula C.; McElwee, Elizabeth; Pires, Mario Cezar; Rico Restrepo, Mariana; Saez-de-Ocariz, Marimar; Sánchez, JorgeAtopic dermatitis (AD) is a systemic, multifactorial disease that causes significant morbidity and health care burden in Latin America (LA). Data on AD are scarce in LA. Lack of disease registries and non-standardized study methodologies, coupled with region-specific genetic, immunological, and environmental factors, hamper data collection. A panel of LA experts in AD was given a series of relevant questions to address before a conference. Each narrative was discussed and edited through numerous rounds of deliberation until achieving consensus. Identified knowledge gaps in AD research were updated prevalence, adult-disease epidemiology, local phenotypes and endotypes, severe-disease prevalence, specialist distribution, and AD public health policy. Underlying reasons for these gaps include limited funding for AD research, from epidemiology and public policy to clinical and translational studies. Regional heterogeneity requires that complex interactions between race, ethnicity, and environmental factors be further studied. Informed awareness, education, and decision making should be encouraged.
- ItemClinical characterization of Chilean patients with food protein-induced enterocolitis syndrome(ELSEVIER SCIENCE INC, 2021) Moya, Francisca; Cid, Barbara; Borzutzky Schachter, Arturo; Hoyos Bachiloglu, Rodrigo
- ItemClusters of Autoimmune Diseases in Children and the Role of PTPN22 C1858T Gene Polymorphism in Pediatric Polyautoimmunity(2014) Borzutzky Schachter, Arturo; Seiltgens, Cristián; Iruretagoyena B., Mirentxu; Cristi, Francisca; Ponce, María Jesús ; Melendez, Patricia; Martínez Aguayo, Alejandro; Hodgson Bunster, María Isabel; Talesnik Guendelman, Eduardo; Riera Cassorla, Francisca Paz; Méndez, Cecilia; Harris D., Paul R.; García Bruce, Hernán; Gana Ansaldo, Juan Cristóbal; Godoy, Claudia; Cattani Ortega, AndreínaBackground/Purpose:Autoimmune diseases (AIDs) have familial aggregation and frequently share a common genetic background, but few studies have evaluated autoimmune clusters in children with AIDs and their families. Children with more than one AID (pediatric polyautoimmunity) may have a stronger genetic component than children with a single AID. The objectives of this study were to identify clusters of AIDs in children and their first-degree relatives and to evaluate the association of PTPN22 C1858T gene polymorphism with pediatric polyautoimmunity.Methods:A cross-sectional study was performed in subjects with an AID of pediatric onset (<18 years)recruited at Pediatric Rheumatology, Endocrinology and Gastroenterology Clinics at the Health Network of the Pontificia Universidad Católica de Chile School of Medicine. Clusters of AIDs were identified by K-means cluster analysis. The PTPN22 C1858T gene polymorphism was determined by RT-PCR in subjects with pediatric polyautoimmunity and in subjects with three common AIDs: juvenile idiopathic arthritis (JIA), autoimmune thyroid disease (AITD), and type I diabetes (T1D).Results:191 subjects with pediatric AIDs were included, of which 45 (24%) had polyautoimmunity. Mean age was 12.1 years (range 1–19) and 68% were female. Most frequent AIDs were JIA (36%), AITD (25%), T1D (19%), uveitis (8%), celiac disease (6%), and vitiligo (6%). 59% of subjects with pediatric autoimmunity had first-degree relatives with an AID. Five clusters of AID were identified in families of children with autoimmunity (Table 1). Among the 45 subjects with pediatric polyautoimmunity, four clusters of AIDs were identified (Table 2). Genomic DNA from 128 subjects was evaluated for PTPN22 C1858T gene polymorphism revealing common homozygosity (C/C) in 85.2%, heterozygosity (C/T) in 13.3%, and rare homozygosity (T/T) in 1.6 %, in equilibrium with Hardy Weinberg equation (P = 0.4). 26% of polyautoimmune subjects had the T allele in contrast with 11% of monoautoimmune subjects (P = 0.04). No significant difference was found in the age of onset of autoimmunity between mono and polyautoimmune subjects (P = 0.44) or between subjects with C/C genotype vs. C/T and T/T genotypes (P = 0.81).
- ItemHealthcare disparities in atopic dermatitis in Latin America: A narrative review(2022) Sánchez, Jorge; Ale, Iris-Selva; Angles, María Valeria; Fogelbach, Guillermo Guidos; Janse, Angela Marie; Takaoka, Roberto; Borzutzky Schachter, ArturoIntroduction: Atopic dermatitis (AD) is a chronic, pruritic skin disease caused by a mixture of genetic, immunological, and environmental factors, characterized by periods of inflammation and remission. In Latin America (LA), the prevalence of AD ranges up to 25% in children and 1–3% in adults. The natural history of the disease for most patients is that AD goes into remission in adolescence and adult life. Only 10–30% of patients continue to have symptoms of the disease in adulthood. There are patients (3–4%) who have the onset of AD during adolescence or after adulthood. Those with limited access to healthcare services, such as diagnosis and treatment, have increased difficulties coping with AD. Healthcare disparities are a complex topic that include social, political, racial/ethnic, and geographical factors. Publications about healthcare disparities in AD in LA are scarce. As a result, recognizing and resolving healthcare inequalities is critical to improving the treatment and quality of life (QoL) of individuals with AD. Methods: A panel of Latin American experts in dermatology and allergies was provided with a series of relevant questions to address before a multiday conference. During this conference, the entire group discussed and edited each narrative through numerous drafts and rounds of discussion until they reached a consensus. Results: This paper examines the barriers to equal access to care and recommends realistic actions to overcome them. Inadequate disease knowledge, cultural and linguistic barriers, stigmatization, maldistribution of resources, absence of local clinical practice guidelines, arduous patient journey, and limited consultation time were identified as causes of health inequality. Conclusions: Among the suggested solutions are enhanced education for healthcare professionals, patients, and the general public, a focus on underprivileged communities, telemedicine and telementoring, translators, multidisciplinary teams, and local living clinical practice guidelines.
- ItemHertoghe’s sign in atopic dermatitis(2020) Borzutzky Schachter, Arturo; Tejos-Bravo, Macarena; Venegas, Luis F.; Iturriaga, Carolina
- ItemHigh prevalence of lupin allergy among patients with peanut allergy: identification of γ-conglutin as majo allergen(American College of Allergy, Asthma and Immunology, 2022) Aguilera-Insunza, Raquel; Iturriaga, Carolina; Mariñanco, Angélica; Venegas, Luis; Aravena, Gabriela; Pérez-Mateluna, Guillermo; Baptista-Dias, Nathalia; Borzutzky Schachter, Arturo; Wandersleben, TraudyBackground: Lupin is a protein-rich legume with a growing presence in the food market worldwide. With increased consumption, lupin allergy (LA) reports are also rising. Uncertainties exist on the cross-reactivity between peanut and lupin, the allergenic potential of different lupin species, and sensitization patterns among different populations. Objective: To evaluate the molecular basis of LA and to determine lupin allergens from 3 different species that may be involved in peanut allergy (PA) cross-reactivity. Methods: A total of 43 subjects with PA, those with LA, or controls without food allergy were evaluated with skin prick tests (SPTs) and specific IgEs (sIgEs). Lupin-sensitized subjects were offered a lupin oral food challenge (OFC). Immunoblots and enzyme-linked immunosorbent assays were performed on sera from lupin-sensitized subjects. Results: In this study, 44% of the PA subjects were confirmed to have LA by OFC. Anaphylaxis was the most frequent manifestation after lupin consumption, with a minimal eliciting dosage of 1 g lupin flour. There was no difference in lupin sIgE or SPT wheal size between lupin-sensitized and confirmed LA subjects or in the severity of symptoms among confirmed LA subjects. Sera from lupin-sensitized subjects uniformly reacted to all 3 different lupin species. Immunoblotting and enzyme-linked immunosorbent assays revealed immunoglobulin E binding to a- and g-conglutin in all analyzed sera, whereas a- and b-conglutin recognition was variable. Conclusion: Our findings reveal a high prevalence of LA among PA subjects, emphasizing lupin must be labeled as an allergen in foods. Owing to high variability in lupin-sIgE and lupin-SPT results, LA diagnosis may require OFC. In our population, g-conglutin is the major allergen of lupin
- ItemHypersensitivity reactions to measles-mumps-rubella vaccine in patients with IgE-mediated cow's milk allergy(American Academy of Allergy, Asthma and Immunology, 2020) Piñones, Mervin; Landaeta, María; Bustos, Paula; Toche, Paola; Morales, Pamela; Escobar, Carola; Borzutzky Schachter, Arturo; Hoyos-Bachiloglu, Rodrigo
- ItemICD-10 coded hospitalizations due to drug hypersensitivity: A nationwide study from Chile(ELSEVIER, 2020) Escobar, Juan J.; Aguilera Insunza, Raquel; Borzutzky Schachter, Arturo; Hoyos Bachiloglu, Rodrigo
- ItemIncidence of type 1 diabetes in Chilean children between 2006 and 2021: significant increase during the COVID-19 pandemic(Springer-Verlag Italia SRL, 2023) Tampe Araya, Ingeborg; Garfias, Carolina; Borzutzky Schachter, Arturo; Slaibe, Lissette; García Bruce, HernánAn increase in type 1 diabetes (T1D) incidence has been observed in several countries during the COVID-19 pandemic. The objective of this study is to determine T1D incidence trends in Chilean children between 2006 and 2021, and specifically evaluate the effect of the COVID-19 pandemic in this population.MethodsWe reviewed mandatory notifications of T1D in Chile's public and private health system in youth < 20 years between 2006 and 2021, and compared COVID-19 pre-pandemic and pandemic incidence.ResultsIn Chile, 9472 new T1D cases in children were confirmed between 2006 and 2021. The mean annual T1D incidence in the entire period was 12.7/100,000 inhabitants, with an incidence of 11.7/100,000 between 2006 and 2019 vs. 20.2/100,000 during 2020-2021 (& beta; = 0.691, [95%CI 0.479-0903], p < 0.001.) The highest incidence was observed in the 10-14 years age group, but a significant increasing incidence was observed in all age groups. The second year of the COVID-19 pandemic, 2021, had the highest incidence rate of the study period. While a 5% mean annual increase was observed between 2006 and 2019, in 2021 the T1D incidence jumped 28.5% compared with the two previous years. We found a higher T1D incidence in population with private insurance than public insurance (14.8 vs. 11.7/100.000, respectively, RR = 1.26 [95%CI 1.03-1.53], p < 0.027).ConclusionsT1D incidence rates in Chilean youth doubled between 2006 and 2018, subsequently presenting a striking increase during the COVID-19 pandemic.
- ItemInfantile Takayasu: clinical features and long-term outcome(2022) Miller-Barmak, Adi; Sztajnbok, Flavio; Balik, Zeynep; Borzutzky Schachter, Arturo; Fogel, Leslie A.; Goldzweig, Ofra; Ozen, Seza; Aviel, Yonatan ButbulObjectives: Takayasu arteritis (TAK) is a large-vessel vasculitis rarely reported in children and infants. Most articles on paediatric TAK have not focused on infants. We present the largest case series of infantile TAK, aiming to identify its demographic and clinical characteristics and compare them with existing data on older children. Methods: We conducted an international multicentre retrospective cohort study. Epidemiological and clinical data were collected from patients’ charts from six rheumatology centres. All patients met both the EULAR/PReS 2008 criteria and the 1990 ACR/EULAR criteria and were diagnosed with TAK at age <5 years. Results: Twelve patients were included (50% female). Median age of symptom onset was 11 months, with a diagnostic delay of 4 months. The most common symptoms at presentation were hypertension, blood pressure differences between limbs, and fever. The most commonly involved arteries were the abdominal aorta and renal artery. Medications included steroids, conventional and biologic DMARDs, and other immunosuppressive therapies. Half of the patients received biologic agents, of which infliximab had the highest complete remission rate (40%). Other medications resulting in complete remission were CYC (40%) and MTX (38%). Invasive procedures were required for 58% of patients. The most common complications were cardiac (50%), stroke (42%), and serious infections (33%). No patients died. Conclusion: This study presents the largest series of infantile TAK. Compared with other reported series on older children, infants with TAK have more severe disease and were more likely to receive biologic agents, develop complications, and require invasive interventions.
- ItemInmunodeficiencia combinada severa, reporte de pacientes chilenos diagnosticados durante el período 1999-2020(2020) Hoyos-Bachiloglu, Rodrigo; Rojas, Jorge; Borzutzky Schachter, Arturo; Hernández, Pamela; Vinet, Ana María; Bustos, Paula; Fernández, Fabiola; Lagos, Macarena; Strickler, Alexis; Marinovic, María Angélica; Casado, Cristina; Poli, María Cecilia; King, AlejandraLa inmunodeficiencia combinada severa (IDCS) corresponde a una de las formas más graves de inmunodeficiencia primaria, existiendo escasos datos nacionales sobre ésta. Objetivo: describir la epidemiología, complicaciones, pronóstico y uso de la vacuna BCG en pacientes chilenos con IDCS. Pacientes y Método: Estudio retrospectivo de pacientes diagnosticados con IDCS entre los años 1999 y 2020 por médicos inmunólogos a lo largo de Chile. El diagnóstico de IDCS se realizó conforme a los criterios propuestos por Shearer: linfocitos T (CD3+) < 300 células/µL y proliferación 10% del límite de normalidad en respuesta a fitohemaglutinina o presencia de linfocitos T de origen materno. Se obtuvieron de la ficha clínica los datos correspondientes a: sexo, edad al diagnóstico, consanguinidad, región de origen, subpoblaciones linfocitarias, diagnóstico genético, complicaciones infecciosas y no infecciosas, vacunación BCG y sus complicaciones, edad de derivación al centro de TPH y causa de mortalidad no relacionada al TPH. Resultados: se diagnosticaron 25 casos de IDCS en 22 familias entre los años 1999-2020. 78% varones, la edad media a la primera manifestación fue 2.3 meses (0-7), mientras que la edad media al diagnóstico fue de 3.4 meses (0- 7). Un 16% de los casos tenía un antecedente familiar de IDCS. Un 40% de los casos fueron diagnosticados en la Región Metropolitana. El inmunofenotipo más frecuente fue T-B-NK+ (48%). Se realizaron estudios genéticos en 69,5% de los casos, siendo los defectos genéticos en RAG2 (39%) la causa más frecuente. Un 88% de los casos recibió la vacuna Bacillus Calmette-Guerin (BCG) previo al diagnóstico, incluidos 2 pacientes con historia familiar positiva, 36% de los vacunados experimentó complicaciones de la BCG. La edad media a la derivación a trasplante fue de 7,4 meses (5-16). De los 25 pacientes, 11 fallecieron previo a la derivación a un centro de trasplante. Conclusión: En Chile existe un retraso clínicamente significativo entre las primeras manifestaciones y el diagnóstico de IDCS, así como un importante retraso en la derivación a centros de trasplante. La mayoría de los pacientes con IDCS reciben la vacuna BCG, pese a tener antecedentes familiares, y experimentan frecuentemente complicaciones de la vacuna.
- ItemMaternal H. pylori is associated with differential fecal microbiota in infants born by vaginal delivery(2020) Hernández, Caroll D.; Shin, Hakdong; Troncoso, Paula A.; Vera, Macarena H.; Villagran, Andrea A.; Rodríguez-Rivera, Selena M.; Ortiz, Marlene A.; Serrano, Carolina A.; Borzutzky Schachter, Arturo; Domínguez-Bello, María Gloria; Harris, Paul R.Helicobacter pylori colonization may affect the mucosal immune system through modification of microbiota composition and their interactions with the host. We hypothesized that maternal H. pylori status affects the maternal intestinal microbiota of both mother and newborn. In this study, we determine the structure of the fecal microbiota in mothers and neonates according to maternal H. pylori status and delivery mode. We included 22 mothers and H. pylori infection was determined by fecal antigen test. Eleven mothers (50%) were H. pylori-positive (7 delivering vaginally and 4 by C-section), and 11 were negative (6 delivering vaginally and 5 by C-section). Stool samples were obtained from mothers and infants and the fecal DNA was sequenced. The fecal microbiota from mothers and their babies differed by the maternal H. pylori status, only in vaginal birth, not in C-section delivery. All 22 infants tested negative for fecal H. pylori at 15 days of age, but those born vaginally –and not those by C-section- showed differences in the infant microbiota by maternal H. pylori status (PERMANOVA, p = 0.01), with higher abundance of Enterobacteriaceae and Veillonella, in those born to H. pylori-positive mothers. In conclusion, the structure of the infant fecal microbiota is affected by the maternal H. pylori status only in infants born vaginally, suggesting that the effect could be mediated by labor and birth exposures.
- ItemOpen-label phase 3 study of intravenous golimumab in patients with polyarticular juvenile idiopathic arthritis(Oxford University Press, 2021) Ruperto, Nicolino; Brunner, Hermine I.; Pacheco-Tena, César; Louw, Ingrid; Vega-Cornejo, Gabriel; Spindler, Alberto J.; Kingsbury, Daniel J.; Schmeling, Heinrike; Borzutzky Schachter, Arturo; Cuttica, RubénObjectives: To assess efficacy, pharmacokinetics (PK) and safety of intravenous (i.v.) golimumab in patients with polyarticular-course JIA (pc-JIA). Methods: Children aged 2 to <18 years with active pc-JIA despite MTX therapy for ≥2 months received 80 mg/m2 golimumab at weeks 0, 4, then every 8 weeks through week 52 plus MTX weekly through week 28. The primary and major secondary endpoints were PK exposure and model-predicted steady-state area under the curve (AUCss) over an 8-week dosing interval at weeks 28 and 52, respectively. JIA ACR response and safety were also assessed. Results: In total, 127 children were treated with i.v. golimumab. JIA ACR 30, 50, 70, and 90 response rates were 84%, 80%, 70% and 47%, respectively, at week 28 and were maintained through week 52. Golimumab serum concentrations and AUCss were 0.40 µg/ml and 399 µg ⋅ day/ml at week 28. PK exposure was maintained at week 52. Steady-state trough golimumab concentrations and AUCss were consistent across age categories and comparable to i.v. golimumab dosed 2 mg/kg in adults with rheumatoid arthritis. Golimumab antibodies and neutralizing antibodies were detected via a highly sensitive drug-tolerant assay in 31% (39/125) and 19% (24/125) of patients, respectively. Median trough golimumab concentration was lower in antibody-positive vs antibody-negative patients. Serious infections were reported in 6% of patients, including one death due to septic shock. Conclusion: Body surface area-based dosing of i.v. golimumab was well tolerated and provided adequate PK exposure for clinical efficacy in paediatric patients with active pc-JIA.
- ItemOral vitamin D modulates the epidermal expression of the vitamin D receptor and cathelicidin in children with atopic dermatitis(2022) Cabalín, Carolina; Pérez-Mateluna, Guillermo; Iturriaga, Carolina; Camargo Jr., Carlos A.; Borzutzky Schachter, ArturoAlthough vitamin D (VD) is known to have multiple effects on the skin and immunity, its effects on atopic dermatitis (AD) severity remain unclear. We investigated whether oral cholecalciferol (VD3) supplementation changes stratum corneum expression of the vitamin D receptor (vdr), and the epidermal alarmins Cathelicidin Antimicrobial Peptide (camp/LL-37) and Thymic Stromal Lymphopoietin (tslp) in children with AD. We conducted an open-label supplementation study with weekly oral VD3 for six weeks in children with AD. Serum 25-hydroxyvitamin D (25OHD), lesional Staphylococcus aureus colonization, and AD severity evaluated by SCORAD index were evaluated before and after supplementation. Tape stripping (TS) was performed on non-lesional and lesional skin to measure mRNA expression of vdr, camp, and tslp through RT-qPCR and LL-37 peptide by ELISA. Twenty-two children with moderate–severe AD received weekly oral VD3 for six weeks. Total serum 25OHD increased from 45.1 ± 23 to 93.5 ± 24.3 nmoL/L (p < 0.0001), while SCORAD decreased from 41.4 ± 13.5 to 31.5 ± 15.8 (p < 0.0001). After treatment, epidermal gene expression of camp increased significantly in non-lesional (p = 0.014) and lesional (p = 0.0007) tape stripping samples, while vdr only increased in lesional skin samples (p < 0.0001). LL-37 peptide increased significantly only in lesional skin samples (p = 0.008). Gene expression of tslp did not change after oral VD3 treatment. In children with AD, oral VD3 supplementation was associated with improved VD status and AD severity, as well as increased VDR and Cathelicidin expression in lesional skin, which provide mechanistic clues on its effects.
- ItemPediatric ANCA-associated vasculitis, a case seriesVasculitis asociadas a ANCA en pediatría, serie de casos clínicos(2021) Cid, Bárbara Javiera; Feuerhake, Teo; Méndez, Gonzalo P.; Talesnik, Eduardo; Borzutzky Schachter, ArturoLas vasculitis asociadas a ANCA (AAV) son enfermedades infrecuentes en la edad pediátrica. La literatura internacional en pediatría es escasa y la mayoría de las publicaciones se refieren a otras vasculitis de mayor incidencia en la infancia, como la vasculitis IgA y enfermedad de Kawasaki. Objetivo: Describir las características clínicas y de laboratorio de una serie de pacientes pediátricos con diagnóstico de AAV. Pacientes y Método: Estudio retrospectivo, descriptivo, de pacientes con diagnóstico de AAV atendidos en un centro terciario de salud en Santiago, Chile, entre los años 2000 y 2020. Se revisaron fichas electrónicas recolectando datos epidemiológicos, de laboratorio, imágenes y biopsias. Resultados: Se presentan cinco pacientes pediátricos con AAV de severidad variable, rango de edad al debut 5,5 a 13,5 años. Destaca una frecuencia elevada de compromiso renal en casos de poliangeítis microscópica (MPA) y el compromiso orbitario de tipo pseudotumor inflamatorio en pacientes con granulomatosis con poliangeítis (GPA); manifestación poco frecuente en series pediátricas internacionales. Los pacientes fueron tratados según recomendaciones extrapoladas de ensayos clínicos en población adulta con respuesta clínica satisfactoria; en su mayoría con corticoides sistémicos y ciclofosfamida o rituximab en etapa de inducción. Durante la mantención, la mayoría de pacientes se mantuvo estable con rituximab, azatioprina o metotrexato. Ningún paciente evolucionó con secuelas en órganos afectados y todos lograron suspender la terapia corticoidal. Conclusión: El presente reporte describe las características clínicas de una serie de pacientes pediátricos con AAV. En esta serie, la afectación renal fue frecuente en MPA y la afectación ocular por pseudotumor inflamatorio en GPA. La respuesta clínica con tratamiento según recomendaciones extrapoladas de población adulta fue favorable.
- ItemReacciones de hipersensibilidad a asparaginasa(2021) Hoyos Bachiloglu, Rodrigo Andrés; Concha, Sara; Borzutzky Schachter, Arturo; Aguilera Insunza, Raquel; Peralta, Teresa; Burnier, Loreto
- ItemRising hospitalization rates of Kawasaki disease in Chile between 2001 and 2007(2011) Borzutzky Schachter, Arturo; Hoyos Bachiloglu, Rodrigo Andrés; Cerda Lorca, Jaime Rodrigo; Talesnik Guendelman, Eduardo
- ItemScrotal Calcinosis in Juvenile Systemic Sclerosis(The Journal of Rheumatology, 2023) Borzutzky Schachter, Arturo; Valenzuela Vergara, Antonia María