LitCovid-sentences  

PMC:7289100 / 17647-30097 JSONTXT 12 Projects

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Id Subject Object Predicate Lexical cue
T113 0-46 Sentence denotes 7 Kawasaki like disease secondary to COVID-19
T114 47-146 Sentence denotes Kawasaki disease (KD) is a systemic vasculitis that usually affects children less than 5 years old.
T115 147-343 Sentence denotes Soon after the first description by Tomisaku Kawasaki in 1967, it was clear that the disease, although self-limiting, might cause coronary artery aneurism (CAA) in a high percentage of cases [61].
T116 344-457 Sentence denotes KD is nowadays considered as the first cause of acquired heart disease in children from developed countries [62].
T117 458-573 Sentence denotes The disease is far more common in eastern countries, and in children of eastern origin living in western countries.
T118 574-710 Sentence denotes For example, the 2010 annual incidence for KD was 222.9 cases/100000 children <5 y/o in Japan, compared to 20/100000 in the USA [63,64].
T119 711-855 Sentence denotes Since no specific test is available, diagnosis relies on the recognition of the cardinal clinical features and the exclusion of other mimickers.
T120 856-1061 Sentence denotes The mainstay of treatment is the administration of intravenous immunoglobulins (IVIG) at 2 g/kg of body weight, as soon as the diagnosis is established, and possibly within 10 days from the onset of fever.
T121 1062-1121 Sentence denotes IVIG have been demonstrated to reduce the risk of CAA [65].
T122 1122-1309 Sentence denotes A subgroup of patients, accounting for nearly 5% of those with KD, has been described to have a very severe disease onset with systolic hypotension or shock (Kawasaki Shock Syndrome-KSS).
T123 1310-1416 Sentence denotes The shock may be caused by a capillary leak syndrome or by poor perfusion due to inflammatory myocarditis.
T124 1417-1720 Sentence denotes KSS patients are more likely to be females and to have earlier onset, incomplete presentations, and more severe laboratory abnormalities (lower platelet counts, higher serum CRP levels, hyponatremia, increased transaminases, metabolic acidosis, consumptive coagulopathy, and lower serum albumin levels).
T125 1721-1899 Sentence denotes Patients with KSS are often resistant to IVIG treatment and have a higher incidence of coronary artery aneurysms, mitral regurgitation, and prolonged myocardial dysfunction [66].
T126 1900-2032 Sentence denotes Northern Italy was the first European region to be severely hit by the SARS-COV2 epidemic after the original outbreak in China [67].
T127 2033-2219 Sentence denotes Although the data on the clinical presentation of COVID-19 in children is still scarce, lower rates of infection and milder forms of disease seem to be typical of this age group [68,69].
T128 2220-2456 Sentence denotes Since the end of March 2020, we observed and treated or received information from all over Italy about an abnormally elevated number of critically ill patients with clinical characteristics consistent with Kawasaki Shock Syndrome (KSS).
T129 2457-2729 Sentence denotes The common features were: “middle-aged” children (6–9 y/o) with a history of persistent high spiking fever in the previous days, abdominal pain, diarrhea, skin rash, and rapidly deteriorating clinical conditions with the onset of shock, without clear signs of dehydration.
T130 2730-2834 Sentence denotes Other less common features were arthralgia, cough, meningism, conjunctivitis and reddened, cracked lips.
T131 2835-2957 Sentence denotes Labworks usually showed high inflammatory markers, low lymphocyte count, low sodium, and high troponin and pro-BNP levels.
T132 2958-3086 Sentence denotes Echocardiography was consistent with myocarditis in the majority of patients instead of classical coronary artery abnormalities.
T133 3087-3205 Sentence denotes Patients were diagnosed as Kawasaki disease (typical or incomplete) and treated accordingly with IVIG and/or steroids.
T134 3206-3295 Sentence denotes One patient refractory to such treatments responded successfully to intravenous anakinra.
T135 3296-3481 Sentence denotes Althoug all the patients had family contacts consistent with COVID-19, serology and nasopharyngeal swabs were inconsistently positive both in the single patients and among all children.
T136 3482-3560 Sentence denotes To date we are aware of at least 10 cases of KSS possibly related to COVID-19.
T137 3561-3693 Sentence denotes Given the unprecedented cluster of patients we decided to launch a nationwide alert for pediatricians and start a national registry.
T138 3694-3905 Sentence denotes Few days after the diagnosis of our first cases, news from all over Europe first, and Eastern USA thereafter, reported on the identification of patients with remarkably the same characteristics we have observed.
T139 3906-4122 Sentence denotes Some children with a less severe picture, without shock at the onset, but with clinical characteristics of systemic inflammation and some features of KD have also been seen by us and others (personal communications).
T140 4123-4217 Sentence denotes These observations lead to a variety of nationwide alerts in the respective countries [70,71].
T141 4218-4367 Sentence denotes On May 7, 2020, Riphagen et al. published on 8 patients treated at the South Thames Retrieval Service for a severe acute illness similar to KSS [72].
T142 4368-4483 Sentence denotes Age at presentation was between 4 and 14 years, 6 out of 8 children were of Afro-Caribbean descent and 5 were boys.
T143 4484-4617 Sentence denotes All the children had a similar disease onset with persistent fever, skin rash, conjunctivitis, peripheral oedema, and extremity pain.
T144 4618-4740 Sentence denotes Gastrointestinal symptoms were frequent and severe, all children developed shock requiring ICU admission and vasopressors.
T145 4741-4898 Sentence denotes Another key feature described in those patients was the cardiac involvement, as testified by very elevated cardiac enzymes, and echo-bright coronary vessels.
T146 4899-4947 Sentence denotes One patient developed a giant coronary aneurism.
T147 4948-5053 Sentence denotes Lab works typically showed high C-reactive protein, procalcitonin, ferritin, triglycerides, and D-dimers.
T148 5054-5138 Sentence denotes All children were treated with IVIG (2 g/kg) and 6 also received aspirin (50 mg/kg).
T149 5139-5307 Sentence denotes Broncho-alveolar lavage or nasopharyngeal aspirates were negative for SARS-COV-2 in all children; some of the patients had histories of familial contact with the virus.
T150 5308-5448 Sentence denotes The authors stated that, at the time they were writing their report, another ICU in London managed 20 patients with similar characteristics.
T151 5449-5647 Sentence denotes As per the Royal College of Pediatrics and Child Health (RCPCH) alert, some of these patients resulted to be positive for SARS-CoV-2 infection, by either nasopharyngeal swab, serology, or both [69].
T152 5648-5733 Sentence denotes The New York State Department of Health reported sixty-four cases as of May 5th 2020.
T153 5734-6010 Sentence denotes For the purpose of this review we will refer to this clinical entity as Pediatric COVID-19 Hyperinflammatory Syndrome (PeCOHS), while the English authorities refers to it as Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection (PIMS-TS).
T154 6011-6219 Sentence denotes Verdoni et al. published the first Italian experience on 10 patients admitted to their hospital for Kawasaki-like disease from February 18th to April 20th, 2020 (during the peak of COVID19 epidemic in Italy).
T155 6220-6367 Sentence denotes Very nicely, the authors compared these children (group 2) to the historical cohort of KD patients seen in the last 5 years (group 1, 19 patients).
T156 6368-6470 Sentence denotes Half of the children in group 2 presented with classic KD, while half were diagnosed as incomplete KD.
T157 6471-6629 Sentence denotes Half of the patients showed hypotension and signs of hypoperfusion at onset, and were interpreted as KSS, and half had signs of myocarditis at echocardiogram.
T158 6630-6682 Sentence denotes Pro-BNP was above normal values in all the patients.
T159 6683-6791 Sentence denotes Half of the patients satisfied the PRINTO laboratory criteria for Macrophage Activation Syndrome (MAS) [73].
T160 6792-6820 Sentence denotes Two patients developed CAAs.
T161 6821-7015 Sentence denotes Patients in group 2 displayed older age, a higher frequency of gastrointestinal and respiratory symptoms, meningeal signs, myocarditis, shock syndrome, and MAS criteria as compared with group 1.
T162 7016-7202 Sentence denotes Nasopharyngeal swab for SARS-CoV-2 was positive in 2 patients only, 8 patients had positive IgG while 3 patients had positive IgM (all of whom were positive also for IgG) for SARS-CoV-2.
T163 7203-7298 Sentence denotes All patients from group 2 were treated with IVIG and steroids, with good clinical outcome [74].
T164 7299-7491 Sentence denotes The occurrence of such cases with a stereotyped clinical picture, which is rarely seen in children, during the peak of SARS-CoV2 infection in highly epidemic areas, points to a causative link.
T165 7492-7594 Sentence denotes One hypothesis is that these children were suffering from actual KD triggered by the SARS-CoV-2 virus.
T166 7595-7675 Sentence denotes Indeed, a microbial etiology has been long advocated as a possible cause for KD.
T167 7676-7907 Sentence denotes Many viruses (HHV-6, Epstein Barr virus, Cytomegalovirus, Coronavirus other than SARS-CoV-2, Parvovirus B19 etc.) but also bacterial infections (such as staphylococci, streptococci, Bartonella, and Yersinia) have been linked to KD.
T168 7908-8084 Sentence denotes Interestingly enough, a case of classic KD has been recently reported in a 6 months-old child who tested positive for SARS-CoV-2, although this could be just coincidental [75].
T169 8085-8322 Sentence denotes The clinical and laboratory characteristics of the patients recently described are strikingly similar to the KSS typical picture, except for the older age, male predominance and relatively low frequency of coronary artery aneurysms [65].
T170 8323-8512 Sentence denotes Given these differences, it can be speculated that this COVID-19 Hyperinflammatory Syndrome is indeed a systemic vasculitis peculiar of the pediatric age and caused by SARS-CoV-2 infection.
T171 8513-8577 Sentence denotes How SARS-CoV-2 infection may cause a hyperinflammatory syndrome?
T172 8578-8792 Sentence denotes At this point we can only formulate hypotheses, based on the clinical picture of the observed patients and using other diseases with overlapping manifestations, such as KD and Toxic Shock Syndrome (TSS), as models.
T173 8793-9005 Sentence denotes The delay between the pandemic peak of COVID-19 and the occurrence of this hyperinflammatory syndrome in children does not point to a direct effect of the infection, but rather towards an immune-mediated disease.
T174 9006-9167 Sentence denotes This seems to be confirmed also by the observation of Verdoni et al. that the majority of their patients had positive SARS-CoV-2 IgG, but negative IgM and swabs.
T175 9168-9346 Sentence denotes Staphylococcal or Streptococcal TSS are the result of T cells induction by superantigens TSST-1, enterotoxins B or C (staphylococci), or pyrogenic exotoxin A or B (streptococci).
T176 9347-9546 Sentence denotes Superantigens can stimulate clonal T cell proliferation and this leads to the production of massive amounts of pro-inflammatory cytokines, as IL-1, IL-6, and IFN-γ in a so-called cytokine storm [76].
T177 9547-9808 Sentence denotes There is a body of evidence suggesting that KD may be caused by a superantigen response, and recent data demonstrated that KSS is characterized by a massive pro-inflammatory cytokine release that differentiate those patients from the “regular” KD patients [77].
T178 9809-9935 Sentence denotes This cytokine storm could be the underlying mechanism leading to the clinical picture similar of that of TSS, KSS, and PeCOHS.
T179 9936-10414 Sentence denotes Other possible mechanisms by which SARS-CoV-2 could induce a hyperinflammatory syndrome are: i) the autoantibodies formation through “molecular mimicry”, as in Acute Rheumatic Fever (although this mechanism would not justify the occurrence of shock); ii) a vascular damage secondary to the deposition of immune-complexes as in acute serum sickness; iii) an antibody-dependent enhancement (ADE) with IgG immune complexes that enhance virus infection in Fc-receptor-bearing cells.
T180 10415-10556 Sentence denotes Intriguingly, this phenomenon is the cause of Dengue vascular permeability syndrome, which, again, shares some similarities with PeCOHS [78].
T181 10557-10680 Sentence denotes ADE were demonstrated in SARS-CoV infection years ago, mediated by antibodies directed to the envelope spike proteins [79].
T182 10681-10750 Sentence denotes Children with SARS-CoV-2 infection usually have a benign course [67].
T183 10751-11040 Sentence denotes Differently from what we usually see in Kawasaki Disease, which has a predilection for asian countries, PeCOHS has not been described so far in those countries, suggesting that both the genetic predisposition of the host and differences in the viral genome may play a role in this disease.
T184 11041-11151 Sentence denotes To our knowledge, the majority of patients with PeCOHS respond to a variable combination of steroids and IVIG.
T185 11152-11381 Sentence denotes The discussion about the immunomodulatory effects of both steroids and IVIG is beyond the scope of this review, but it is interesting to highlight that one of the many effects of IVIG is the down modulation of superantigens [80].
T186 11382-11508 Sentence denotes Of note, IVIG is the therapy of choice in the capillary leak syndrome, whose main features are similar to KSS and PeCOHS [81].
T187 11509-11783 Sentence denotes Finally, the efficacy of anti-IL-1 treatment as “rescue therapy” for cases resistant to IVIG+steroids points out the relevance of this pro-inflammatory cytokine in the hyperinflammatory state, similar to what already shown in adults with COVID-19 and hyperinflammation [82].
T188 11784-12034 Sentence denotes In conclusion, although SARS-CoV-2 infection appears to occur as a oligo-symptomatic form in the majority of children, there is mounting evidence that it can cause a systemic hyperinflammatory syndrome that mimics KSS, but has some peculiar features.
T189 12035-12243 Sentence denotes The role of host and virus genetics and the exact immune mechanisms leading to the disease are far from been understood, and case collection would help us in better characterization of the clinical phenotype.
T190 12244-12450 Sentence denotes As light has been shed on the interaction between SARS-CoV-2 and the immune system, we will gain hints also at Kawasaki Disease pathogenesis, almost 50 years from its first description by Tomisaku Kawasaki.