ABSTRACT
Background: Immunoglobulin replacement has been revolutionized the treatment of inborn errors of immunity (IEI), ensuring greater survival and quality of life for these patients. Most IEI patients need to receive continuous and regular immunoglobulin replacement. In Brazil, unfortunately, there is no production of this biological or policies for plasma reuse and its importation becomes necessary. Immunoglobulin is distributed monthly by the Brazilian Public Health System (SUS), free of charge. Apart from the difficulty in importing, we had to face the worldwide decrease in blood donations related to COVID-19 pandemic. The aim of this study was to report the rate of lack of immunoglobulins and complications related to this event. Method: This is a cross-sectional, retrospective study, based on the analysis of patient's medical charts at an IEI reference center in Brazil, from January to December 2020. Results: During this period, 124 patients received intravenous immunoglobulin (IVIG) replacement, 62 patients are female (50%), 70 adults (18 to 84 years, median age: 38y) and 54 children (0 to 17 years, median 11y). The most prevalent diagnostic were Common Variable Immunodeficiency-CVID (28.2%), Hypogammaglobulinemia (19.3%), Specific Antibody Deficiency-SAD (11.2%), Ataxia-Telangiectasia (8.8%) and other diagnostics (32,5%: SCIDS, leaky SCID, XLP2, XHIGM, VEO-IBD). Indeed, 70 patients (56.4%) were affected by the lack of distribution of IVIG (56.4%) related to COVID-19 pandemic, remaining without IVIG from one to 12 months, 2.9 months on average. Of these, 48 patients (68.5%) had at least one infection (total 125), all of whom needed to use antibiotics for the treatment of infections and 12 patients required hospitalization. Conclusion: In 2020, 70 (56.4%) patients were affected by the lack of distribution of IVIG. It was possible to identify shortage of immunoglobulin replacement in Brazil due to COVID-19 pandemic led to more infections and increase of antibiotics use.
ABSTRACT
Background: Since 2019, we have experienced a terrible pandemic, COVID-19. Emerging countries, like Brazil, with logistical difficulties and lack of public policies, face a generalized collapse in health system. Rare Diseases Reference Centers are located distant from patients' houses. Thus, patients with lysosomal diseases, unable to travel and need to receive their recombinant enzyme replacement therapy (ERT) close to their homes. Infusion-related reactions (IRR) are uncommon;however, they can impair the treatment. Therefore, due to the impossibility of locomotion and unavailability of teams of allergists, RDD protocol were accomplished. The study aimed to describe remote points of training and protocols execution. Method: After appointments from treating lysosomal centers (TLC) diseases about adverse reactions, the following strategy was adopted: three online meetings between metabolic team and allergists to present the clinical case;lectures about adverse reactions to medications and RDD: video demonstrations off how to perform skin tests and nursing training for the use of. Two meetings were held with the families, terms of consent were applied, and a communication group was created on WhatsApp® with team leaders. Afterwards, the RDD was formulated and applied remotely, by Google Meet®. Finally, three infusions were followed up under the supervision of our center. Results:: Six patients presented immediate IRR to different recombinant enzymes: three patients with Fabry disease, one with MPS I, one with MPS II and one with MPS IV. The Allergy Center located in São Paulo, was composed of a team of allergology and health professionals with expertise in inborn errors of metabolism. The (TLC) were in the interior of São Paulo, Bahia, Pernambuco and Piauí, 300 to 1,800 miles apart. The protocols were carried out respecting the Standard 12-16 steps according to risk stratification. One of the patients, developed urticaria on the 11th step, despite the addition of premedication. Conclusion: The new Coronavirus' pandemic imposed a new reality, which include much more telecommunication. Barriers have been overcome, such as offering remote alternatives to the treatment of incurable diseases in countries with continental dimensions.
ABSTRACT
Background: Inborn Errors of Immunity (IEI) are a heterogeneous group of diseases which immune defect may be related to a complications by COVID 19. Although there are few studies on the evolution and the real clinical impact of infection by SARS-CoV 2 in this group of patients. The aim of the study was to describe the clinical evolution of COVID-19 in patients with different IEI, in a reference center in Brazil. Method: Retrospective and longitudinal study, with analysis of electronic medical charts of patients with IEI and diagnosis of suspected/ confirmed COVID-19 from March 2020 until now. Results: Eighteen patients (61% male), with a median age of 26.6 years (range 12,3-53,4 y), were included in the study. Four X-linked agammaglobulinemia (XLA), 7 common variable immunodeficiency (CVID), 1 specific antibody deficiency, one Good Syndrome-GS, 2 STAT1-GOF, 2 MSMD and one AT. Ten patients (55,5%) had mild flu syndrome and only one patient was asymptomatic. Hospitalization was necessary for 7 patients (38,8%) due to respiratory complications and 3 (16.6%) deceased (2 XLA and 1 GS). Two patients were reinfected (STAT1-GOF and MSMD), with no need for hospitalizations or long-term complications. One of the XLA patients remains hospitalized, with fever for more than 90 days. Five patients experience coughing and tiredness after more than three months of the disease, one also persists with anosmia. Conclusion: Almost 40% of our sample required hospitalization and 16% died. This rate is worrying and reveals how much immunological competence is required by SARS-CoV-2 as well as the fact that Brazil has increasing death rates from COVID-19. Post-acute COVID-19 syndrome (PASC) has presented in more than 25% of IEI patients infected with SARS-CoV-2.