In the last few months the world has witnessed a global pandemic due to severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection causing coronavirus disease 2019 (COVID-19). Obviously, ...this pandemic affected individuals differently, with a significant impact on populations considered to be at high-risk. One such population, was assumed to be patients with primary genetic defect involving components or pathways of the immune system. While human immunity against COVID-19 is not fully understood, it is, so far, well documented, that both adaptive and innate cells have a critical role in protection against SARS-CoV-2. Here, we aimed to summarize the clinical and laboratory data on primary immunodeficiency (PID) patients in Israel, who were tested positive for SARS-CoV-2, in order to estimate the impact of COVID-19 on such patients. Data was collected from mid-February to end-September. During this time Israel experienced two "waves" of COVID-19 diseases; the first, from mid-February to mid-May and the second from mid-June and still ongoing at the end of data collection. A total of 20 PID patients, aged 4 months to 60 years, were tested positive for SARS-CoV-2, all but one, were detected during the second wave. Fourteen of the patients were on routine monthly IVIG replacement therapy at the time of virus detection. None of the patients displayed severe illness and none required hospitalization; moreover, 7/20 patients were completely asymptomatic. Possible explanations for the minimal clinical impact of COVID-19 pandemic observed in our PID patients include high level of awareness, extra-precautions, and even self-isolation. It is also possible that only specific immune pathways (e.g. type I interferon signaling), may increase the risk for a more severe course of disease and these are not affected in many of the PID patients. In some cases, lack of an immune response actually may be a protective measure against the development of COVID-19 sequelae.
Pediatric morbidity due to unintentional poison exposure is a significant burden on public health. We prospectively characterize patterns of unintentional poison exposure in a single pediatric ...emergency department, using a detailed computerized questionnaire for all unintentional injuries admitted during 2009 to 2017. Out of 71,765 visits due to unintentional injuries, 252 children were admitted due to unintentional poison exposure. Most (198/252, 79%) were between 1 and 3 years of age. The majority of events (209/252, 82.9%) occurred at the patient’s home and 81% (205/255) were classified as exploratory ingestion. In 41/252 (14%) cases, exposure to more than one substance was reported. Most events 231/293 (79%) involved medications and 21% were due to domestic products. Four medications account for 45% of the events (Paracetamol, Salbutamol, Antihypertensive, and Antidepressants). Opioids were responsible for only 1.7%. By, collaboration between government, public health, educational institutions and commercial companies, can the burden of pediatric unintentional poison exposure be reduced.
Pediatrician, watch out for corona-phobia Rosenberg Danziger, Chen; Krause, Irit; Scheuerman, Oded ...
European journal of pediatrics,
01/2021, Letnik:
180, Številka:
1
Journal Article
Recenzirano
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The current outbreak of COVID-19 raging globally is taking a heavy toll on the adult population, with a rapidly growing number of newly infected and critically ill patients. However, to date, ...mortality rate among children is low as they mostly suffer from a mild disease. Yet, other more routinely encountered childhood diseases do not stand still and continue to be the main share of pediatricians’ everyday challenges. Here we describe a case series of routinely seen pediatric diseases with delayed diagnosis due to different aspects of what we call “Corona-phobia”. These cases were easily collected within a 1-week period which implies that this is a more widespread phenomenon.
In conclusion, this raises the possibility that measures taken to mitigate this pandemic may be more damaging to children overall than the virus itself. We believe that pediatricians as well as policy makers should take this important aspect into consideration.
What is Known:
• COVID-19 manifests as a mild disease in most children; however, children are an important reservoir and may become spreaders of the disease.
• Social distancing and isolation are important tools in mitigating COVID-19 transmission.
What is New:
• This case series describes 7 cases with delayed diagnosis of every-day pediatric diseases that were not caused by COVID-19 but were highly influenced by different aspects of “Corona-phobia”.
• Our objective is to highlight the possibility that measures taken to mitigate this pandemic may lead to a substantial delay in the diagnosis of other non-COVID-19 related diseases.
Severe combined immunodeficiency (SCID), the most severe form of T cell immunodeficiency, is detectable through quantification of T cell receptor excision circles (TRECs) in dried blood spots ...obtained at birth. Herein, we describe the results of the first year of the Israeli SCID newborn screening (NBS) program. This important, life-saving screening test is available at no cost for every newborn in Israel. Eight SCID patients were diagnosed through the NBS program in its first year, revealing an incidence of 1:22,500 births in the Israeli population. Consanguine marriages and Muslim ethnic origin were found to be a risk factor in affected newborns, and a founder effect was detected for both
and
deficiency SCID. Lymphocyte subset analysis and TREC quantification in the peripheral blood appear to be sufficient for confirmation of typical and leaky SCID and ruling out false positive (FP) results. Detection of secondary targets (infants with non-SCID lymphopenia) did not significantly affect the management or outcomes of these infants in our cohort. In the general, non-immunodeficient population, TREC rises along with gestational age and birth weight, and is significantly higher in females and the firstborn of twin pairs. Low TREC correlates with both gestational age and birth weight in extremely premature newborns. Additionally, the rate of TREC increase per week consistently accelerates with gestational age. Together, these findings mandate a lower cutoff or a more lenient screening algorithm for extremely premature infants, in order to reduce the high rate of FPs within this group. A significant surge in TREC values was observed between 28 and 30 weeks of gestation, where median TREC copy numbers rise by 50% over 2 weeks. These findings suggest a maturational step in T cell development around week 29 gestation, and imply moderate to late preterms should be screened with the same cutoff as term infants. The SCID NBS program is still in its infancy, but is already bearing fruit in the early detection and improved outcomes of children with SCID in Israel and other countries.
Defining combined immunodeficiency Roifman, Chaim M., MD; Somech, Raz, MD; Kavadas, Fotini, MD ...
Journal of allergy and clinical immunology,
07/2012, Letnik:
130, Številka:
1
Journal Article
Recenzirano
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Background Although the extreme condition of typical profound T-cell dysfunction (TD), severe combined immunodeficiency (SCID), has been carefully defined, we are currently in the process of better ...defining less typical T-cell deficiencies, which tend to present with autologous circulating T-cell combined immunodeficiency (CID). Because autologous cells might interfere with the outcome of bone marrow transplantation, protocols usually include conditioning regimens. Therefore it is important to define the numbers of autologous cells usually detected in patients with CID versus those with SCID. Objectives We sought to determine the number of circulating T cells in patients with SCID as opposed to those with CID, to study their function, and to evaluate their possible detection during newborn screening using T-cell receptor excision circle (TREC) analysis. Methods Numbers of circulating CD3+ T cells (as determined by means of flow cytometry), in vitro responses to PHA, and TREC levels, all measured at presentation, were compiled from the research charts of the entire cohort of patients followed prospectively for T-cell immunodeficiency at the Hospital for Sick Children. Clinical data were ascertained retrospectively from the patient's hospital charts. Results One hundred three patients had CD3+ determinations, and 80 of them had a genetic diagnosis. All patients considered to have typical SCID had CD3+ T-cell counts of fewer than 500 cells/μL. Some variability was observed among different genotypes. In vitro responses to PHA were recorded in 88 patients, of whom 68 had a genetic diagnosis. All patients with low CD3+ T-cell numbers (<500 cells/μL) also had markedly decreased responses to PHA (typical SCIDs). However, responses ranged widely in the groups of patients with TD who had more than 500 CD3+ autologous circulating T cells per microliter. Although patients with Omenn syndrome and ζ chain–associated protein, 70 kDa (ZAP70) , and purine nucleoside phosphorylase (PNP) deficiencies had low responses, patients with the p.R222C mutation in the IL-2 receptor γ (IL2RG) gene as well as IL-10 receptor and CD40 ligand deficiencies had normal or near-normal mitogen responses. Finally, 51 patients had TREC levels measured. All patients with typical SCID, Omenn syndrome, and ZAP70 deficiency had low TREC levels. In contrast, patients with mutations in forkhead box protein 3 (FOXP3) , CD40 ligand (CD40L) , and IL-10 receptor α (IL10RA) , as well as patients with the p.R222C mutation in the IL2RG gene, had normal TREC levels. Conclusion Patients with typical SCID can be defined as having fewer than 500 circulating CD3+ T cells. Most patients with autologous T cells still have profound TD, as defined by reduced in vitro function and thymus output. Some patients with conditions including TD have normal TREC levels and will therefore not be detected in a TREC-based newborn screening program.
The transition from milk protein–induced enterocolitis syndrome to IgE‐mediated milk allergy is uncommon. Herein, we describe three infants that suffered from recurrent vomiting and restlessness in ...response to cow’s milk formula with negative skin prick to milk and therefore diagnosed as milk protein–induced enterocolitis syndrome. After recovering and reintroducing cow’s milk formula, they developed disseminated urticaria and positive skin prick test to cow milk compatible with IgE‐mediated milk allergy.
Conclusion: An infant that recovers from cow milk food–induced enterocolitis syndrome might develop afterward IgE‐mediated cow milk allergy.
Background Kwasaki disease (KD) is the leading cause of acquired heart disease in children in most developed countries. The cause of KD remains unknown. The presumed theory is that KD occurs due to ...one or more infectious agents who evoke an abnormal immunological response in susceptible individuals. Epstein - Barr virus (EBV) infection has been considered as a suspected causative agent because of the potential effect on the immune system. Case presentation A previously healthy 19 month old boy presented with a 6 day history of fever accompanied by a diffuse macular erythematous rash that appeared 1 day after. The physical examination on admission revealed bilateral non-suppurative conjunctivitis, dry fissured and injected lips without "strawberry" tongue, diffuse macular rash on the trunk, face and limbs, swelling of the hands and feet, and right cervical lymphadenopathy (2 cm in diameter). Following fulfillment of all the clinical criteria, the diagnosis of KD was made and treatment with IVIG 2 g/Kg was administered along with oral aspirin (80 mg/ kg/day). However, despite the treatment, he remained febrile for an additional 2 days with persistent clinical manifestations. Therefore, he received a second 2 g/kg IVIG course with a favorable response. On the 14th day of illness the patient became febrile again and was readmitted. Blood examinations revealed remarkable leukocytosis up to 35.7 X 10.sup.9/L with 87.3% lymphocytes and the blood smear revealed atypical lymphocytes and monocytes. The liver enzymes were elevated. The serology for infectious mononucleosis from his first admission revealed: IgM CMV (+), IgG CMV (-); IgM VCA EBV (+) IgG VCA EBV (-), IgG EBNA (-). To confirm infectious mononucleosis following the administration of 2 doses of IVIG, serum EBV PCR was performed and was positive (1.6X 10.sup.3 cp/ml). Conclusions We describe here a case of KD with a concomitant primary EBV infection. To the best of our knowledge, this is the first case in western country that describes KD with acute EBV infection as confirmed by PCR. The case we described stands as a contribution in favor of the possible role of EBV in the development of KD. Keywords: Kawasaki disease, Epstein- Barr virus, Intravenous immunoglobulins
As of October 2021, SARS-CoV-2 infections were reported among 512,613 children and adolescents in Israel (~33% of all COVID-19 cases). The 5-11-year age group accounted for about 43% (223,850) of ...affected children and adolescents. In light of the availability of the Pfizer-BioNTech BNT162b2 vaccine against COVID-19 for children aged 5-11 years, we aimed to write a position paper for pediatricians, policymakers and families regarding the clinical aspects of COVID-19 and the vaccination of children against COVID-19. The first objective of this review was to describe the diverse facets of the burden of COVID-19 in children, including the direct effects of hospitalization during the acute phase of the disease, multisystem inflammatory syndrome in children, long COVID and the indirect effects of social isolation and interruption in education. In addition, we aimed to provide an update regarding the efficacy and safety of childhood mRNA COVID-19 vaccination and to instill confidence in pediatricians regarding the benefits of vaccinating children against COVID-19. We reviewed up-to-date Israeli and international epidemiological data and literature regarding COVID-19 morbidity and its sequelae in children, vaccine efficacy in reducing COVID-19-related morbidity and SARS-CoV-2 transmission and vaccine safety data. We conducted a risk-benefit analysis regarding the vaccination of children and adolescents. We concluded that vaccines are safe and effective and are recommended for all children aged 5 to 11 years to protect them from COVID-19 and its complications and to reduce community transmissions. Based on these data, after weighing the benefits of vaccination versus the harm, the Israeli Ministry of Health decided to recommend vaccination for children aged 5-11 years.
The widespread use of 7-valent pneumococcal conjugate vaccine (PCV7) has changed acute otitis media (AOM) bacteriology. Only scattered data with regard to this effect of PCV13 have been published so ...far.
We retrospectively identified children <6 years of age who presented to our hospital with AOM, and had middle ear fluid (MEF) cultures obtained during tympanocentesis or from spontaneous otorrhea during 2008-2013, when PCV7 (2009) and PCV13 (2010) were gradually introduced in the Israeli National Immunization Program. Data were extracted for demographics, clinical and microbiologic parameters, according to vaccination status.
Of the 295 eligible AOM episodes reported in 279 children, 224 (76%) had MEF cultures from tympanocentesis and 71 (24%) from spontaneous otorrhea. Boys and children <2 years of age contributed 178 (60%) and 219 (74%) AOM episodes, respectively. Acute mastoiditis complicated 58 (20%) of these episodes. None of the children were PCV immunized in 2008, but >90% had received ≥1 PCV dose(s) by 2011 or later. Of the 106 (36%) MEF cultures which tested positive for otopathogens, Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis and multiple bacteria grew in 60 (57%), 39 (37%), 2 (2%) and 5 (5%) episodes, respectively. S. pneumonia-positive MEF culture rate in unimmunized children (31, 69%) was significantly higher than in PCV7-immunized children (22, 59%) or PCV13-immunized children (12, 50%), P = 0.04 and P = 0.02, respectively.
PCV7 and PCV13 implementations in the Israeli National Immunization Program were associated with a rapid reduction of "severe" pneumococcal AOM episodes.
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