Papers by Enrique Faugier
Circulation, 2015
Background: Little is known about the burden of Kawasaki disease (KD) in Latin America (LA). The ... more Background: Little is known about the burden of Kawasaki disease (KD) in Latin America (LA). The LA KD network (REKAMLATINA) was established in 2013 and has 120 physicians in over 85 hospitals in 20 countries. It’s goal is to characterize the epidemiology of KD in LA children, to understand the disease impact, and plan for studies that address the specific needs of LA children with KD. Methods: Retrospective review of 437 children 1 echo, worst CA status was evaluated for either the RCA or LAD. IVIG therapy response was classified as resistant (fever ≥38C more than 36 hrs after completion of IVIG), responsive or unknown. Conclusions: This is the first multicenter report describing the clinical course and treatment of KD in LA. Differences in the care of KD patients were observed among participating countries, specially regarding obtaining echos at baseline and follow up. Also, KD could be an important cause of antibiotic misuse in LA. More research is needed to describe how KD care ...
Circulation, 2015
Case Report 8 months old male, who presented with 7 days fever, hematuria, conjunctival injection... more Case Report 8 months old male, who presented with 7 days fever, hematuria, conjunctival injection and limb edema; treated as a post infectious glomerulonephritis. Twenty days later, desquamation at the tip of the fingers and in palms and soles plus thrombocytosis were found. Considering Kawasaki disease (KD), an echocardiogram was performed, finding a 5mm aneurysm in right coronary artery and 4mm ectasia in the left coronary artery. The patient was on the subacute therefore IVIG was not administered; aspirin however in antiplatelet dose range was begun. He continued on surveillance by Cardiology Department without signs of cardiac malfunction. At two years follow up a new echocardiogram was performed, showing a left coronary aneurism of 6mm and a right coronary artery of 2.0mm which was difficult to identify. Left ventricular function was preserved with an ejection fraction of 72%. Four years later, posterior myocardial necrosis, without acute ischemia on electrocardiography was fou...
The Journal of Rheumatology, 2021
Objective.To assess the relationship between infection risk and abatacept (ABA) exposure levels i... more Objective.To assess the relationship between infection risk and abatacept (ABA) exposure levels in patients with polyarticular-course juvenile idiopathic arthritis (pJIA) following treatment with subcutaneous (SC) and intravenous (IV) ABA.Methods.Data from 2 published studies (ClinicalTrials.gov: NCT01844518, NCT00095173) of ABA treatment in pediatric patients were analyzed. One study treated patients aged 2–17 years with SC ABA and the other treated patients aged 6–17 years with IV ABA. Association between serum ABA exposure measures and infection was evaluated using Kaplan-Meier plots of probability of first infection vs time on treatment by ABA exposure quartiles and log-rank tests. Number of infections by ABA exposure quartiles was investigated.Results.Overall, 343 patients were included in this analysis: 219 patients received SC ABA and 124 patients received IV ABA. Overall, 237/343 (69.1%) patients had ≥ 1 infection over 24 months. No significant difference in time to first in...
Paediatric rheumatology, 2018
(PANLAR). Results: The response rate of web-based survey by LAPR was 170/288 (59%) and the majori... more (PANLAR). Results: The response rate of web-based survey by LAPR was 170/288 (59%) and the majority worked in University Hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging and biopsies were generally available (>75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (>75%), except for belimumab (11%). Endemic illnesses were reported by LAPR in at least one cSLE patient during the previous year: tuberculosis (16%) and Hansen disease (2%). Emerging mosquito-borne diseases were also reported: dengue (20%), Chikungunya (11%) and Zika (8%). Groups were further divided in two, according to the number of cSLE patients followed by LAPR in the last year: group A (3 25 patients) and group B (<25 patients). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (69% vs. 48%, p=0.01). The frequencies of reported pregnancy (50% vs. 16%, p<0.001) and non-adherence to therapy were significantly higher in group A (100% vs. 93%, p=0.023). Alcohol intake (42% vs. 21%, p=0.004) and illicit drug use (19% vs. 5%, p=0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. Conclusions: This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of University Hospitals. Adherence to therapy, pregnancy and substance abuse were identified as major challenges in this population, particularly in larger centres.
Pediatric rheumatology online journal, Jan 17, 2018
Musculoskeletal Ultrasonography (MSUS) is an important tool for the clinical assessment in Juveni... more Musculoskeletal Ultrasonography (MSUS) is an important tool for the clinical assessment in Juvenile Idiopathic Arthritis (JIA). The objective of this study was to evaluate the reliability of MSUS to detect elementary lesions: synovitis, tenosynovitis, cartilage damage and bone erosions in the wrist and metacarpal (MCP) joints of patients with JIA. Thirty children in various subgroups of JIA according to ILAR criteria, were included in this cross-sectional study. Clinical data including painful, swollen and limited joints were recorded. Five rheumatologist ultrasonographers, blinded to the clinical evaluation, evaluated the presence of elementary lesions in the wrist and MCP 2 and 3 joints bilaterally. The synovitis was graded in B-Mode and Power Doppler (PD). In addition to descriptive statistics intra- and inter-observer reliability was calculated using Cohen's kappa according to Landis and Koch. US detected more synovitis than the clinical examination (62% vs 28%, 30% vs 23% a...
Pediatric Rheumatology, 2017
Introduction: In 2015 the historic Jones criteria for the diagnosis of Acute Rheumatic Fever (ARF... more Introduction: In 2015 the historic Jones criteria for the diagnosis of Acute Rheumatic Fever (ARF) were revised introducing two different sets of criteria for low-risk and for moderate/high-risk populations (according to ARF incidence). In Italy the exact ARF incidence is unknown but small regional or local reports suggest an incidence of 2-5/100.000 per year, suggesting that our population might be considered at moderate risk for ARF. Objectives: To evaluate the performance of the revised Jones criteria in a retrospective population and to compare it with the performance of the previous version of Jones criteria. Methods: We conducted a retrospective study on 288 patients with ARF (108 female; median age 8.5 years, IQR 7.1-10.3) diagnosed from 2001 to 2015 in a Pediatric Rheumatology Division by pediatric rheumatologists, discharged with an ICD 9 code consistent with ARF. We retrospectively applied the two sets (for low-risk and for moderate/high-risk) of the 2015 revised Jones criteria and the 1992 version of the Jones criteria. Results: Of 288 patients, 253 (87.8%) met the 1992 version of the Jones criteria, 237 (82.3%) met the revised criteria for low-risk populations and 259 (89.9%) for moderate/high-risk populations. None of these differences was significant. Prevalence of major and minor criteria is shown in Table. With the exception of difference in arthritis, the 1992 version and the 2015 revised version did not show major differences. Of the 288 patients with a clinical diagnosis of ARF 29 did not meet any version of the Jones criteria. Patients in this group presented with isolated chorea or silent carditis without other manifestations. Prevalence of the clinical characteristics and comparison among the 1992 version of Jones criteria and the 2015 revised Jones criteria (low risk and moderate-high risk populations): Values are expressed in Number (percentage). *p value (Fisher Exact test) Conclusion: The revised Jones criteria for low-risk populations are slightly more sensitive than the 1992 version of Jones criteria, while the revised Jones criteria for moderate/high populations are slightly less sensitive than the 1992 version. In this population, the revised criteria did not substantially modify the diagnosis of ARF. Approximately 10% of patients presented with isolated chorea or silent carditis.
Pediatric Rheumatology, 2017
Introduction: Behçet's disease is a complex multisystemic disease with variable vasculitis. The d... more Introduction: Behçet's disease is a complex multisystemic disease with variable vasculitis. The disease is most prevalent in the Eastern Mediterranean, the Middle East, and East Asia, along the ancient Silk Road. The typical triad is oral ulcers, genital ulcers and ocular inflammation. But other clinical manifestations due to vasculitis and thrombosis, involvement of the gastrointestinal system, nervous system and musculoskeletal system can be seen variably. Although it is a disease of young adulthood, one-forth of cases are diagnosed during childhood. There is a long interval before onset of enough manifestations to satisfy adult Behçet's disease criteria, so a new subset of consensus classification criteria was put forward by an international Behçet's expert group. Objectives: To evaluate the demographic data, clinical features and treatment modalities of children with Behçet's disease and to display the relationship of HLA B51 genotype with mild and severe system involvements of pediatric Behçet's disease patients. Methods: Two pediatric rheumatology centers; one from West and one from East of Turkey collected the data of patients with pediatric Behçet's disease retrospectively between January 2010 and May 2016. The children were enrolled to the study if they fulfilled the Internationational classifications criteria for Behçet's disease. Children diagnosed ≤16 years of age were included to the study. The demographic, clinical, laboratory and medication reports were collected from data sheets. Results: Fifty-four patients were included; 23 male (42,5%), 31 female (57.5%). Family history of Behçet's disease in a first-degree relative was present in 17 (31.5%) of cases. Of them, 11 (31,4%) were HLA B51 positive. The mean age of the patients was 15,1 (8-21) years. The mean age of onset was 11,1 (5-15,6) years. The mean age at diagnosis was 12,9 (6-16) years. All the patients had oral ulceration. Genital ulcers were present at 34 (62,9%) patients. Skin findings were present in 35 (64,8%) patients. Ten patients (18,5%) had uveitis during the course of disease. Seven patients (13%) had thrombosis. Gastrointestinal involvement was reported in 15 patients (27,8%) and arthralgia/arthritis was present at 29 patients (53,7%). Five patients (9,3%) had neurological signs and symptoms. ANA positivity was seen at 9 (17%) patients. HLA B51 positivity was detected at 35 (64,8%) children of whom 15 (42,8%) had a mild course of disease while 20 (57,2%) had a severe course with major organ involvement. All the patients were under colchicine treatment, 16 of them had aditional DMARD (azathiopurine or methotrexate) and 2 of them had biologic treatment. Steroids were used by 15 patients. The age that the first symptom ensues was significantly lower in males (9,9 vs 12,8). No relationship with the family history and age of onset of the disease, severity of the disease was shown. The age of onset, the clinical features, treatment modalities reported from two centers from different regions of country were very similar to each other. Conclusion: This is a retrospective evaluation of a large series of children with Behçet's disease from a country with high prevalance. HLA-B51 positivity does not seem to help to predict the course of the disease. Disclosure of Interest None Declared.
European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery, 2017
The decision whether or not to proceed with surgical intervention of a patient with a ruptured ab... more The decision whether or not to proceed with surgical intervention of a patient with a ruptured abdominal aortic aneurysm (rAAA) is very difficult in daily practice. The primary objective of the present study was to develop and to externally validate a new prediction model: the Dutch Aneurysm Score (DAS). With a prospective cohort of 10 hospitals (n = 508) the DAS was developed using a multivariate logistic regression model. Two retrospective cohorts with rAAA patients from two hospitals (n = 373) were used for external validation. The primary outcome was the combined 30 day and in-hospital death rate. Discrimination (AUC), calibration plots, and the ability to identify high risk patients were compared with the more commonly used Glasgow Aneurysm Score (GAS). After multivariate logistic regression, four pre-operative variables were identified: age, lowest in hospital systolic blood pressure, cardiopulmonary resuscitation, and haemoglobin level. The area under the receiver operating c...
Annals of the Rheumatic Diseases, 2016
group the presence of periodontal disease was 71.74%, tender joints (19.57%), swollen joints (6.5... more group the presence of periodontal disease was 71.74%, tender joints (19.57%), swollen joints (6.52%). Disease activity measures were not representative. Only a woman in the group of family related RA patients was positive for RA 33 (49 years old, without treatment, painful and swollen joints and the presence of periodontal disease and Pg, ESR and CRP positives but RF and ACPAs negative). No patient with RA of less than two years of diagnosis was positive for RA 33. Conclusions: The diagnostic value of anti RA33 was low in this population, with regard of worst prognosis markers and higher disease severity. Ra33 should be more follow-up clinical and paraclinical to subjects with risk factors who could have positive markers, without symptoms. References: [1] Fritsch R, Eselböck D.Characterization of Autoreactive T Cells to the Autoantigens Heterogeneous Nuclear Ribonucleoprotein A2 (RA33) and Filaggrin in Patients with Rheumatoid Arthritis.
La presencia de neumoencéfalo es común en neurocirugía y sus causas incluyen trauma, infecciones,... more La presencia de neumoencéfalo es común en neurocirugía y sus causas incluyen trauma, infecciones, tumores y cirugías. Generalmente se encuentra como hallazgo casual en estudios de neuroimagen. Reportamos el caso de un paciente masculino de 13 años con antecedente de quiste temporal izquierdo Galeasi III, post-operado de una derivación cistoperitoneal que 37 días después presentó datos clínicos de hipertensión intracraneal. La tomografía axial computarizada (TAC) de cráneo mostró un neumoencéfalo masivo con efecto de masa. La válvula de derivación se encontró siempre normofuncional y no se evidenció lesión en la base de cráneo. Fue necesario drenaje por punción en una ocasión y no se utilizaron antibióticos ya que nunca presentó fiebre o foco infeccioso. Este caso ilustra una presentación rara de neumoencéfalo espontáneo sin causa evidente. The presence of pneumocephalus is common in neurosurgery and its causes include trauma, infections, tumors and surgery. It is usually found accid...
The Israel Medical Association journal : IMAJ, 2012
Data on pediatric antiphospholipid syndrome (APS) are very sparse. To describe the main clinical ... more Data on pediatric antiphospholipid syndrome (APS) are very sparse. To describe the main clinical characteristics, laboratory data and complications of pediatric APS patients, and to analyze the differences between primary APS and APS associated with systemic lupus erythematosus (SLE). We retrospectively reviewed clinical and laboratory data of 32 children at the Federico Gomez children's hospital in Mexico. Nineteen patients had SLE, 12 (37.5%) had primary APS and 1 (3%) had immune thrombocytopenic purpura. We collected information on sociodemographic variables, vaccinations, age at onset, and family history of rheumatic disease, hematological disorders, skin disorders and non-thrombotic neurological disorders. Immunological features included immunoglobulin (Ig) G and IgM anticardiolipin antibodies, IgG and IgM anti-beta2 glycoprotein I antibodies, lupus anticoagulant, and anti-dsDNA and antinuclear antibodies. The patients included 24 females and 8 males. The most common thromb...
Pediatric Rheumatology, 2014
Pediatric Rheumatology, 2014
The Journal of Rheumatology, 2014
Objective.To evaluate factors associated with mortality and infections in patients with systemic ... more Objective.To evaluate factors associated with mortality and infections in patients with systemic lupus erythematosus (SLE) and diffuse alveolar hemorrhage (DAH).Methods.A retrospective chart review was carried out for medical admissions of patients with a diagnosis of SLE and DAH in 9 hospitals. Clinical and laboratory data were recorded for each patient at DAH diagnosis.Results.We included 57 episodes of DAH of 50 patients (7 recurrences), 49 women (86%), 14 juvenile SLE (24.6%); 24 had died (42.1%). In the chart review we detected infection in 22 episodes (38.6%): 8 invasive fungal infections, 16 bacterial infections, and 2 patients had both types. In the bivariate analysis, factors associated with mortality were high Acute Physiology and Chronic Health Evaluation II scores, requirement of mechanical ventilation (OR 15.0, 95% CI 1.9 to 662.2), infections (fungal or bacterial; OR 3.2, CI 0.9 to 11.1), renal failure (OR 4.9, CI 1.4 to 18.0), and thrombocytopenia (OR 4.3, CI 1.2 to 1...
Annals of the Rheumatic Diseases, 2013
Methods: Retrospective cohort. Children and adolescents with SLE admitted to the intensive care u... more Methods: Retrospective cohort. Children and adolescents with SLE admitted to the intensive care unit (ICU) of a single pediatric center in Mexico City from January 1999 to December 2008 were included. We reviewed the medical records of these patients, including age, sex, disease evolution, diagnostic criteria, disease activity (MEX-SLEDAI), neurological and kidney disease, nephropathy (histological classification), treatment at admission, reason for ICU admission, days in pediatric ICU, requirement of catecholamines, invasive procedures, complications, reason of death and PRISM score. Statistical analysis: Categorical variables were analyzed with X² test or Fisher`s exact test. Continuous variables were analyzed with Student t-test. Results: Fifty-six patients were included: thirty-two died and 24 survived. Forty-four were women (78.6%) and 12 men (21.4%). Table 1 shows the main clinical characteristics evaluated in our cohort. Table 1. Clinical findings in our cohort. All (n=56) Alive (n=24) Deceased (n=32) p-value Age*
Pediatric Rheumatology, 2014
Background: Rheumatic diseases in children are associated with significant morbidity and poor hea... more Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY©) to Simple Measure of Impact of Illness in Youngsters (SMILY©-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY©-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n=15) and 17 parents participated. The mean age was 12±4 years, with median disease duration of 21 months (1-172 months). We translated SMILY©-Illness into the following 28 languages:
Reumatología Clínica (English Edition), 2011
Kawasaki disease is of relevance in pediatric practice because it is a systemic vasculitis of unk... more Kawasaki disease is of relevance in pediatric practice because it is a systemic vasculitis of unknown origin and the most common cause of acquired heart disease in young patients. Its main complication is the formation of a coronary aneurism in 25% of the patients, unless they receive timely medical treatment. We report the case of a 4-month-old male child with Kawasaki's disease, who received treatment with gammaglobulin and acetyl-salicylic acid, in which the initial echocardiogram showed aneurisms. Admitted to our hospital with cardiogenic shock, we documented, by echocardiography, the presence of coronary aneurisms with intrasaccular thrombus and acute myocardial infarction. He received fibrinolytic therapy, with an adequate response: the size of aneurisms decreased, as did the intrasaccular thrombus. Currently the patient is asymptomatic and receiving treatment with warfarin and acetylsalicylic acid. The diagnosis and treatment, as well as identification of risk factors can prevent fatal complications at the cardiovascular level. The treatment in the first 10 days of illness with gammaglobulin and acetylsalicylic acid reduced cardiac complications from 4.7% to 25%.
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Papers by Enrique Faugier