Childrens Hospital Ricardo Gutierrez

Buenos Aires, Argentina

Childrens Hospital Ricardo Gutierrez

Buenos Aires, Argentina
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Dackiewicz N.,Pediatric Hospital Juan P Garrahan | Rodriguez S.,Pediatric Hospital Juan P Garrahan | Irazola V.,Institute for Clinical Effectiveness and Health Policy | Barani M.,Institute for Clinical Effectiveness and Health Policy | And 9 more authors.
International Journal for Quality in Health Care | Year: 2016

Objective: To create a hospital pediatric inpatient experience survey based on the Consumer Assessment of Healthcare Providers and Systems Hospital Survey (CAHPS® Hospital Survey). Design: Survey development based on: (i) Translation and back translation, (ii) Review by experts, (iii) Cultural adaptation: qualitative evaluation of dimensions with reformulation and adaptation of items, (iv) Local cognitive evaluation and (v) Final measurement of its psychometric properties. Inspection, content validity and reliability assessment through internal consistency (Cronbach's alpha coefficient) and inter-item correlation. Factor analysis matrix: extraction, selection and rotation. Setting: Two pediatric hospitals in Buenos Aires, Argentina: Hospital de Pediatría 'Garrahan' (HG) and Hospital de Niños 'Ricardo Gutiérrez' (HRG). Participants: Parents or caregivers of pediatric patients hospitalized for at least 24 h. Results: A feasible and easy to administer 21-item instrument was developed. One thousand and thirty-two surveys were analyzed, 630 (61%) in HG and 402 (39%) in HRG. Population: mothers of admitted children were interviewed 85% of the time, 61% (625) had completed minor schooling to high school education; 365 families (35%) had unsatisfied basic needs and 51% (529) did not have health insurance. Reliability: adequate Cronbach's alpha scores were found with correlation 0.7 or higher in most domains. Validity: a direct correlation was observed between overall positive opinion and quality of care perceived with the survey, and an indirect correlation (perceived low quality) with higher level of schooling and health insurance ownership. Conclusion: An instrument with adequate psychometric properties was adapted to evaluate patients and families' perceptions of quality of care received during children's hospitalization. © The Author 2016.


Conti F.,French Institute of Health and Medical Research | Conti F.,University of Paris Descartes | Conti F.,University of Rome Tor Vergata | Lugo-Reyes S.O.,French Institute of Health and Medical Research | And 89 more authors.
Journal of Allergy and Clinical Immunology | Year: 2016

Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by inborn errors of the phagocyte nicotinamide adenine dinucleotide phosphate oxidase complex. From the first year of life onward, most affected patients display multiple, severe, and recurrent infections caused by bacteria and fungi. Mycobacterial infections have also been reported in some patients. Objective: Our objective was to assess the effect of mycobacterial disease in patients with CGD. Methods: We analyzed retrospectively the clinical features of mycobacterial disease in 71 patients with CGD. Tuberculosis and BCG disease were diagnosed on the basis of microbiological, pathological, and/or clinical criteria. Results: Thirty-one (44%) patients had tuberculosis, and 53 (75%) presented with adverse effects of BCG vaccination; 13 (18%) had both tuberculosis and BCG infections. None of these patients displayed clinical disease caused by environmental mycobacteria, Mycobacterium leprae, or Mycobacterium ulcerans. Most patients (76%) also had other pyogenic and fungal infections, but 24% presented solely with mycobacterial disease. Most patients presented a single localized episode of mycobacterial disease (37%), but recurrence (18%), disseminated disease (27%), and even death (18%) were also observed. One common feature in these patients was an early age at presentation for BCG disease. Mycobacterial disease was the first clinical manifestation of CGD in 60% of these patients. Conclusion: Mycobacterial disease is relatively common in patients with CGD living in countries in which tuberculosis is endemic, BCG vaccine is mandatory, or both. Adverse reactions to BCG and severe forms of tuberculosis should lead to a suspicion of CGD. BCG vaccine is contraindicated in patients with CGD. © 2016 American Academy of Allergy, Asthma & Immunology.


Marciano B.E.,National Institute of Allergy and Infectious Diseases | Huang C.-Y.,National Institute of Allergy and Infectious Diseases | Joshi G.,National Institute of Allergy and Infectious Diseases | Rezaei N.,Tehran University of Medical Sciences | And 52 more authors.
Journal of Allergy and Clinical Immunology | Year: 2014

Background Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. Objectives We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. Methods An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. Results Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≥1 month) showed an increased prevalence of complications (P =.006) and death caused by BCG-associated complications (P <.0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P =.001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P<.0001). Conclusions BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications. © 2013 American Academy of Allergy, Asthma & Immunology.


PubMed | Childrens University Hospital, Tehran University of Medical Sciences, National Childrens Hospital Dr Carlos Saenz Herrera, Center for Pediatric Hematology and 30 more.
Type: Journal Article | Journal: The Journal of allergy and clinical immunology | Year: 2014

Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected.We sought to describe the complications and risks associated with BCG vaccination in patients with SCID.An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed.Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (1 month) showed an increased prevalence of complications (P= .006) and death caused by BCG-associated complications (P< .0001). The odds of experiencing complications among patients with T-cell numbers of 250/L or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P= .001) than among those with T-cell numbers of greater than 250/L. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160patients treated with antimycobacterial therapy for a symptomatic BCG infection (P< .0001).BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.


PubMed | Pediatric Hospital Juan P Garrahan, CEDES, Institute for Clinical Effectiveness and Health Policy and Childrens Hospital Ricardo Gutierrez
Type: | Journal: International journal for quality in health care : journal of the International Society for Quality in Health Care | Year: 2016

To create a hospital pediatric inpatient experience survey based on the Consumer Assessment of Healthcare Providers and Systems Hospital Survey (CAHPS Hospital Survey).Survey development based on: (i) Translation and back translation, (ii) Review by experts, (iii) Cultural adaptation: qualitative evaluation of dimensions with reformulation and adaptation of items, (iv) Local cognitive evaluation and (v) Final measurement of its psychometric properties. Inspection, content validity and reliability assessment through internal consistency (Cronbachs alpha coefficient) and inter-item correlation. Factor analysis matrix: extraction, selection and rotation.Two pediatric hospitals in Buenos Aires, Argentina: Hospital de Pediatra Garrahan (HG) and Hospital de Nios Ricardo Gutirrez (HRG).Parents or caregivers of pediatric patients hospitalized for at least 24 h.A feasible and easy to administer 21-item instrument was developed. One thousand and thirty-two surveys were analyzed, 630 (61%) in HG and 402 (39%) in HRG.mothers of admitted children were interviewed 85% of the time, 61% (625) had completed minor schooling to high school education; 365 families (35%) had unsatisfied basic needs and 51% (529) did not have health insurance. Reliability: adequate Cronbachs alpha scores were found with correlation 0.7 or higher in most domains.a direct correlation was observed between overall positive opinion and quality of care perceived with the survey, and an indirect correlation (perceived low quality) with higher level of schooling and health insurance ownership.An instrument with adequate psychometric properties was adapted to evaluate patients and families perceptions of quality of care received during childrens hospitalization.


Bezrodnik L.,Childrens Hospital Ricardo Gutierrez | Gaillard M.I.,Childrens Hospital Ricardo Gutierrez | Carelli D.,Childrens Hospital Ricardo Gutierrez
Journal of Pediatric Infectious Diseases | Year: 2011

We present the clinical and B cell immunophenotypical characterization of 94 patients with Common Variable immunodeficiency (CVID), selective IgA deficiency (SIgAD) and polysaccharide antibody deficiency syndrome (SAD). Study design: We retrospectively investigated clinical findings and B cell compartment in 31 patients with CVID, 35 with SIgAD and 28 with SAD. Regardless of underlying disease, a delay was observed between age at diagnosis and onset of first symptoms. The predominant clinical findings were upper and lower respiratory tract infections. Allergic symptoms were more frequent in SAD and SIgAD patients, hematological and autoimmune manifestations in CVID and celiac disease in SIgAD. B-cell Immunophenotype abnormalities were observed in SAD and CVID patients: both had reduced memory B cells (CD19{+} CD27{+}), and increased transitional B cells (CD24{ + + } CD38{+ +}) was found in SAD. We did not find any statistically significant abnormalities in any of differentiation stages of B cells in SIgAD. Defects of the B cell compartment were associated with bronchiectasis, splenomegaly, autoimmunity and/or malignancy in CVID and SAD patients.We conclude that flow cytometric evaluation of the B cell compartment could be a useful tool for the diagnosis and follow up of these patients. © 2011 - IOS Press and the authors. All rights reserved.

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