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1.
Pediatric Dermatology ; 40(Supplement 2):47, 2023.
Article in English | EMBASE | ID: covidwho-20244353

ABSTRACT

Objectives: Varicella is common infectious disease mainly in childhood, usually is a mild, self-limited illness and complications are usually rare. The incubation period for this disease is generally 14- 16 days but may vary from 7 to 21 days. Varicella in the adults with comorbidities or immunosuppressed children may be severe and prolonged with complications. Method(s): A case report of a 6-year-old girl hospitalized for new-onset manifestations of disseminated vesicular exanthema, the manifestations of which occurred mainly on the chest, back, capillitium, oral cavity, and genital area. The child was suffering from abdominal, knee and lumbosacral pain at that time. The patient's history revealed that 10 days prior to the cutaneous manifestations, she had influenza with bronchopneumonia requiring oxygen therapy, steroids and antibiotics. Result(s): The condition progressed within 48 h, complicated by the development of multi-organ failure, coagulopathy with the development of disseminated intravascular coagulopathy over the course of antiviral, antibiotic and antifungal therapy. Laboratory parameters included high elevation of C-reactive protein, il-6, leukocytosis, neutrophilia and highly elevated liver enzymes. Varicella infection was confirmed by detection of herpes zoster virus - polymerase chain reaction (PCR) from vesicles. The patient received intravenous immunoglobulin therapy at a dose of 2 g/L and fresh frozen plasma, thrombocyte concentrate. The girl was intubated with analogization. Laboratory parameters subsequently revealed high anti CoV-2 positivity, high CoV-2 IgG positivity and negative CoV-2 IgM. The patient's condition did not preclude the course of multisystem inflammatory syndrome in children (MIS-C) corticosteroids were added to the treatment at a dose of 1 mg/kg weight. Patient's condition stabilized after 1 month. Discussion(s): Our case report presents an example of fulminant complicated life-threatening course of varicella. Even in common respiratory infections, we must think about the risk and consequences of coinfections and post-infectious complications such as in our case especially influenza and COVID-19.

2.
Acta Medica Bulgarica ; 50(2):10-19, 2023.
Article in English | EMBASE | ID: covidwho-20244214

ABSTRACT

Compared to other respiratory viruses, the proportion of hospitalizations due to SARS-CoV-2 among children is relatively low. While severe illness is not common among children and young individuals, a particular type of severe condition called multisystem inflammatory syndrome in children (MIS-C) has been reported. The aim of this prospective cohort study, which followed a group of individuals under the age of 19, was to examine the characteristics of patients who had contracted SARS-CoV-2, including their coexisting medical conditions, clinical symptoms, laboratory findings, and outcomes. The study also aimed to investigate the features of children who met the WHO case definition of MIS-C, as well as those who required intensive care. A total of 270 patients were included between March 2020 and December 2021. The eligible criteria were individuals between 0-18 with a confirmed SARS-CoV-2 infection at the Infectious Disease Hospital "Prof. Ivan Kirov"in Sofia, Bulgaria. Nearly 76% of the patients were <= 12 years old. In our study, at least one comorbidity was reported in 28.1% of the cases, with obesity being the most common one (8.9%). Less than 5% of children were transferred to an intensive care unit. We observed a statistically significant difference in the age groups, with children between 5 and 12 years old having a higher likelihood of requiring intensive care compared to other age groups. The median values of PaO2 and SatO2 were higher among patients admitted to the standard ward, while the values of granulocytes and C-reactive protein were higher among those transferred to the intensive care unit. Additionally, we identified 26 children who met the WHO case definition for MIS-C. Our study data supports the evidence of milder COVID-19 in children and young individuals as compared to adults. Older age groups were associated with higher incidence of both MIS-C and ICU admissions.Copyright © 2023 P. Velikov et al., published by Sciendo.

3.
Retina-Vitreus ; 32(1):22-29, 2023.
Article in English | EMBASE | ID: covidwho-20243849

ABSTRACT

Purpose: The aim of this study was to evaluate how prevalent asymptomatic SARS-CoV-2 virus infection (COVID-19) is among patients undergoing ophthalmic surgery at two tertiary referral hospitals. Material(s) and Method(s): This retrospective study included patients without COVID-19 symptoms who underwent preoperative screening using reverse transcription-polymerase chain reaction (RT-PCR) before ophthalmic surgery at the Kocaeli University and Gaziantep University departments of ophthalmology [between September 1, 2020, and December 15, 2020 (group 1);between March 1, 2021, and May 30, 2021 (group 2)]. Patients scheduled for surgery and followed up in the retina, glaucoma, pediatric ophthalmology and strabismus, cataract and refractive surgery, and cornea departments were examined. Result(s): RT-PCR was positive for SARS-CoV-2 in 12 (1.4%) of 840 patients in group 1 and 7 (1.1%) out of 600 patients in group 2. None of the patients were symptomatic of COVID-19. The majority of the patients were scheduled for retina or cataract and refractive surgery in both groups (group 1;retina: 29.2%, cataract and refractive: 57.0%, group-2;retina: 31.3%, cataract and refractive: 54.5%). SARS-CoV-2 RT-PCR testing was positive for seven patients in group 1 (7/245, 2.9%) and five patients in group 2 (5/188, 2.6%) who were scheduled for retinal surgery. Conclusion(s): The necessity, availability, and practicality of COVID-19 RT-PCR testing prior to ophthalmic surgeries varies depending on the protocols of each institution. COVID-19 RT-PCR testing is suggested especially before vitreoretinal surgeries and general anesthesia procedures, because of the difficulty in managing postoperative complications.Copyright © 2023 Gazi Eye Foundation. All rights reserved.

4.
Archives of Pediatric Infectious Diseases ; 11(2) (no pagination), 2023.
Article in English | EMBASE | ID: covidwho-20242270

ABSTRACT

Introduction: Spontaneous pneumothorax is a rare complication of coronavirus disease 2019 (COVID-19), primarily reported in adults. Pediatric cases with bilateral pneumothorax are much less reported. Case Presentation: We presented the case of a five-year-old previously healthy boy who developed persistent fever, abdominal pain, generalized maculopapular rash, and dyspnea before admission. His chest computed tomography (CT) showed a viral involvement pattern of pneumonia suggestive of COVID-19. Subsequently, he was confirmed with multisystem inflammatory syndrome in children (MIS-C). While he responded well to the therapies, on the fifth day of admission, he developed respiratory distress again. A chest roentgenogram showed bilateral spontaneous pneumothorax. Bilateral chest tubes were inserted, and his condition improved sig-nificantly after five days of admission to the intensive care unit. Two weeks later, he was discharged in good condition. Conclusion(s): Children with MIS-C associated with COVID-19 may develop primary spontaneous pneumothorax. Owing to the clinical picture overlapping with MIS-C associated with COVID-19, the timely diagnosis of pneumothorax may be challenging in such patients.Copyright © 2022, Author(s).

5.
Pediatria Polska ; 98(1):79-82, 2023.
Article in English | EMBASE | ID: covidwho-20241151

ABSTRACT

The most common causes of acute hepatitis in children are hepatitis A and autoimmune hepatitis. Hepatitis in the course of Wilson's disease is sporadically registered in adolescents. An increase of activity of aminotransferases both in the course of multisystem inflammatory syndrome in children (MIS-C) and in the course of COVID-19 has been observed. Hepatitis is common in children with MIS-C and is associated with a more severe presentation and persistent elevation of liver function tests. To date, no cases of acute hepatitis in children due to COVID-19 have been reported. We present 2 cases of acute hepatitis in children where the only cause seems to be a previous asymptomatic SARS-CoV-2 infection.Copyright © 2023 Termedia Publishing House Ltd.. All rights reserved.

6.
Clinical Immunology ; Conference: 2023 Clinical Immunology Society Annual Meeting: Immune Deficiency and Dysregulation North American Conference. St. Louis United States. 250(Supplement) (no pagination), 2023.
Article in English | EMBASE | ID: covidwho-20239944

ABSTRACT

Introduction: Variants in PPP1R13L are associated with severe childhood-onset cardiomyopathy resulting in rapid progression to death or cardiac transplantation. PPP1R13L is proposed to encode a protein that limits the transcriptional activity of the NFkappaB pathway leading to elevated IL-1, IL-6, and TNF-alpha production in murine models. Optimal medical management for PPP1R13L-related cardiomyopathy is unknown. Here we report usage of a targeted anti-IL-1 immuno-modulatory therapy resulting in cardiac stabilization in a pediatric patient with congenital cardiomyopathy secondary to PPP1R13L variants. Case Report: A 4-year-old boy presented acutely with fever in the setting of persistent abdominal pain, vomiting, fatigue, and decreased appetite for two months following a mild COVID-19 related illness. Echocardiogram revealed severely depressed biventricular systolic function with an ejection fraction of 30%. Due to acute decompensated heart failure symptoms with hemodynamic instability, he was intubated and placed on continuous inotropic infusions with aggressive diuresis. Cardiac MRI demonstrated extensive subepicardial to near transmural fibrosis by late gadolinium enhancement in right and left ventricles. An implantable cardioverter-defibrillator (ICD) was placed due to frequent runs of polymorphic non-sustained ventricular tachycardia. Testing for viral pathogens was positive for rhino/enterovirus. Initial genetic testing was non-diagnostic (82-gene cardiomyopathy panel) but given the patient's significant presentation whole genome sequencing was pursued that showed two separate PPP1R13L variants in trans (c.2167A>C,p.T723P and c.2179_2183del,p. G727Hfs*25, NM_006663.4). Patient serum cytokine testing revealed elevations in IL-10 (4.7 pg/mL) and IL-1beta (20.9 pg/mL). Given the patient's tenuous circumstances and concern for continued progression of his cardiac disease, a trial of IL-1 inhibition via anakinra dosed at 3 mg/kg or 45 mg daily was initiated following hospital discharge. With approximately 6 months of therapy, the patient's cardiac function is stable with normalization of IL-10 and IL-1beta serum levels. Notably, the ventricular arrhythmia decreased after initiation of anakinra with no ICD shocks given. Therapy overall has been well tolerated without infectious concerns. Conclusion(s): In patients with PPP1R13L-related cardiomyopathy, immuno-modulatory therapies should be considered in an attempt to slow cardiac disease progression.Copyright © 2023 Elsevier Inc.

7.
JBMR Plus ; 5(Supplement 3):35, 2021.
Article in English | EMBASE | ID: covidwho-20239769

ABSTRACT

OBJECTIVE: To explore the experiences of a virtual group therapy programme for children with Osteogenesis Imperfecta (OI) who were unable to access face to face therapy due to a global pandemic. In a regional OI service up to 3 face to face 6-week therapy groups are offered for children during summer school holidays. Throughout the Covid-19 pandemic, OI children were at risk of deconditioning due to government restrictions in school attendance, exercise and socialisation. An alternative means of delivering therapy was required. METHOD(S): 30 families with school age children were invited to attend a virtual therapy group. Following initial interest from 21 children, 14 (age range 4-14 years) participated in a 5 week therapy group. Children were split into 3 age groups (4-7/8-12/13-14). Weekly 75 minute sessions comprised functional gross and fine motor activities. Educational elements with opportunities to explore thoughts and feelings were included. Each group were set a challenge to compete a virtual race from Bristol to Lands' End, recording their distance (walking, wheeling, cycling) throughout the week. Feedback was collected from participants and parents. RESULT(S): Feedback was received from 12/14 of attendees and parents and 4/7 who did not attend. Reasons for non-attendance included anxiety around video calls, other commitments and injuries. Of those that attended 100% (n=12) enjoyed the group and reported increased activity levels. Scavenger Hunt was cited as the favourite activity. 16.6% (n=2) children reported discussion on personal feelings uncomfortable. 16.6% (n=2) reported exercises were hard. 100% (n=9) of parents reported no significant difficulties with IT. Parent reported benefits included positive social interaction with other children with OI (66%, n=6), improving routine in preparation for school return (55%, n=5). Challenges for therapy team included identifying appropriate IT platform, choosing activities appropriate for various abilities and virtual instruction. Successes included time efficiency, full inclusion regardless of geographic location, increased activity levels of children with OI in preparation for school return. CONCLUSION(S): A safe, socially distanced method of delivering group therapy during a pandemic was achieved. Virtual therapy can be a useful adjunct, however this should not replace face to face therapy where possible.

8.
Clinical Immunology ; Conference: 2023 Clinical Immunology Society Annual Meeting: Immune Deficiency and Dysregulation North American Conference. St. Louis United States. 250(Supplement) (no pagination), 2023.
Article in English | EMBASE | ID: covidwho-20239149

ABSTRACT

Background: SAMD9L is a tumor suppressor involved in regulating the proliferation and maturation of cells, particularly those derived from the bone marrow, and appears to play an important role in cerebellar function. It can be activated in hematopoietic stem cells by type I and type II interferons. It has been hypothesized to act as a critical antiviral gatekeeper regulating interferon dependent demand driven hematopoiesis. Gain of function mutations can present with an immunodeficiency due to transient severe cytopenias during viral infection. Case presentation: We report a 3-year-old boy born full term with a history of severe thrombocytopenia requiring transfusions, developmental delay, ataxia, seizure disorder, and recurrent severe respiratory viral infections. His infectious history was significant for respiratory syncytial virus with shock requiring extracorporeal membrane oxygenation complicated by cerebral infarction and a group A streptococcus empyema, osteomyelitis requiring a left below the knee amputation, and infections with rhinovirus, COVID-19, and parainfluenza requiring hospitalizations for respiratory support. Initial immunologic evaluation was done during his hospitalization for parainfluenza. His full T cell subsets was significant for lymphopenia across all cell lines with CD3 934/microL, CD4 653/microL, CD8 227/microL, CD19 76/microL, and CD1656 61/microL. His mitogen stimulation assay to phytohemagglutinin and pokeweed was normal. Immunoglobulin panel showed a mildly decreased IgM of 25 mg/dL, but normal IgA and IgG. Vaccine titers demonstrated protective titers to 12/22 pneumococcus serotypes, varicella, diphtheria, mumps, rubella, and rubeola. Repeat full T cell subsets 6 weeks later revealed marked improvement in lymphocyte counts with CD3 3083/microL, CD4 2101/microL, CD8 839/microL, CD19 225/microL, and CD1656/microL. A primary immunodeficiency genetic panel was ordered and positive for a heterozygous SAMD9L c.1549T>C (p.Trp517Arg) mutation classified as a variant of unknown significance. Discussion(s): This patient's history of severe viral infections, ataxia, thrombocytopenia, and severe transient lymphopenia during infection is suggestive of a SAM9DL gain of function mutation. Protein modeling done by the laboratory suggests this missense mutation would affect protein structure. The mutation found has been observed in individuals with thrombocytopenia. This case highlights the importance of immunophenotyping both during acute illness and once recovered.Copyright © 2023 Elsevier Inc.

9.
Journal of Pediatric Infectious Diseases ; 2022.
Article in English | EMBASE | ID: covidwho-20237646

ABSTRACT

Objective: Acute respiratory tract infections are one of the leading causes of morbidity and mortality in children. Although human bocavirus (HBoV) infections are not as common as other seasonal respiratory viruses, children who are infected with HBoV are more likely to suffer from a variety of respiratory conditions, including the common cold, acute otitis media, asthma exacerbations, bronchiolitis pneumonia, some of the affected children require pediatric intensive care unit stay. Here, we aimed to evaluate pediatric bocavirus (HBoV) cases presenting with severe respiratory tract symptoms during the coronavirus disease 2019 (COVID-19) pandemic. Method(s): This retrospective study evaluated the medical records of children diagnosed with respiratory infections, followed up at the Faculty of Medicine, Eskisehir Osmangazi University between September 2021 and March 2022. In this study, patients with HBoV identified using nasopharyngeal polymerase chain reaction (PCR) were considered positive. Cases were analyzed retrospectively for their clinical characteristics. Result(s): This study included 54 children (29 girls and 25 boys) with HBoV in nasopharyngeal PCR samples. The cases ranged in age from 1 month to 72 months (median 25 months). At the time of presentation, cough, fever, and respiratory distress were the most prevalent symptoms. Hyperinflation (48%), pneumonic consolidation (42%), and pneumothorax-pneumomediastinum (7%) were observed on the chest X-ray;54% of the children required intensive care unit stay. The median length of hospitalization was 6 days. Bacterial coinfection was detected in 7 (17%) children, while HBoV and other viruses were present in 20 (37%) children;57% of children received supplemental oxygen by mask, 24% high-flow nasal oxygen, 7% continuous positive airway pressure, and 9% invasive mechanical ventilation support. Antibiotics were given to 34 (63%) cases, and systemic steroid treatment was given to 41 (76%) cases. Chest tubes were inserted in three out of the four cases with pneumothorax-pneumomediastinum. All patients were recovered and were discharged from the hospital. Conclusion(s): The COVID-19 pandemic changed the epidemiology of seasonal respiratory viruses and the clinical course of the diseases. Although it usually causes mild symptoms, severe respiratory symptoms can lead to life-threatening illnesses requiring intensive care admission.Copyright © 2023. The Author(s).

10.
British Journal of Haematology ; 201(Supplement 1):124, 2023.
Article in English | EMBASE | ID: covidwho-20237251

ABSTRACT

Autoimmune haemolytic anaemia (AIHA) is rare but described after the SARS-CoV- 2 Pfizer-BioNTech vaccine. We present a case of severe refractory warm AIHA after this vaccine, managed with emergency splenectomy and complement inhibition with eculizumab. A male in his teens with a history of liver transplant for biliary atresia (aged 2 years) and AIHA (aged 6 years) presented to his district general hospital with jaundice, dark urine, fatigue and chest discomfort 48 h after the first dose of SARS-CoV- 2 Pfizer-BioNTech vaccine (BNT162b2 mRNA). Investigations revealed haemoglobin (Hb) of 70 g/L and bilirubin of 98 mumol/L, which was treated as AIHA. The patient initially responded to prednisolone (1 mg/kg, 60 mg) but subsequently deteriorated and failed to respond to second-line rituximab (375 mg/m2) and two units of packed red blood cells (PRBC). By day 29 the patient had developed life-threatening anaemia culminating in a Hb of 35 g/L (after transfusion), lactate dehydrogenase (LD) of 1293 units/L and bilirubin of 228 mumol/L. This necessitated an immediate transfer to our tertiary centre for specialist support. Further investigations revealed a haptoglobin <0.1 g/L and direct antiglobulin test (DAT) strongly positive for IgG (4+) and negative for C3d. The peripheral blood film showed severe anaemia, nucleated red cells, anisocytosis and spherocytes with no autoagglutination, schistocytes or platelet clumps. Thrombocytopaenia (platelets 49 +/- 109/L) was present. Differentials were ruled out, such as paroxysmal nocturnal haemoglobinuria and heparin-induced thrombocytopaenia. HIV and hepatitis serology were negative, as were adenovirus, cytomegalovirus and Epstein-Barr virus PCR assays. A CT showed splenomegaly of 15.5 cm. Urinalysis found urobilinogen and bilirubin at high concentrations and negative urinary haemosiderin. Together, the investigations were consistent with warm AIHA. On day 29, four units of PRBC were transfused alongside 100 mg methylprednisolone and 1 g/kg IVIG. On day 30 the patient deteriorated despite the escalated treatment: Hb had only increased to 54 g/L, bilirubin was 200 mumol/L and LD was rising. Considering this life-threatening fulminant haemolysis, an emergency splenectomy was performed. This slowed haemolysis but did not completely ameliorate it: by day 33 the patient had received 15 units of PRBC. Thus, eculizumab, a terminal complement pathway inhibitor, was trialled to arrest intravascular haemolysis, alongside rituximab, repeat IVIG 1 g/kg, prednisolone 40 mg and tacrolimus 2 mg. This showed a favourable response, requiring less frequent transfusions and settling haemolysis. This case highlights the rare complication of warm AIHA with the SARS-CoV- 2 Pfizer-BioNTech vaccine, the use of emergency splenectomy for disease control, and the potential of eculizumab for refractory cases.

11.
Modern Pediatrics ; Ukraine.(1):72-86, 2023.
Article in Ukrainian | EMBASE | ID: covidwho-20235001

ABSTRACT

Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells. The prognosis varies depending on the form of the disease and organ damage. Any organs and systems can be involved in the pathological process in various combinations. A poor response to standard therapy and an unfavorable prognosis are characteristic of patients with a multisystem form of LCH and involvement of organs at risk. Skin lesions are a classic sign of LCH. Purpose - to describe the complexity and duration of diagnosis of LCH with multisystem damage in a boy aged 2 years and 2 months, infected with poliomyelitis and coronavirus. Clinical case. The first clinical manifestations of LCH in the child debuted with an eczematous-seborrheic rash on the scalp with spread to the limbs and trunk. The child was treated for toxicoderma, hemorrhagic vasculitis at the place of residence for 6 months. The boy lost 1.5 kg of body weight in 1 month. At the time of hospitalization, seborrheic-eczematous rashes on the skin with a hemorrhagic component, trophic-inflammatory changes in the nails of the hands, signs of protein-energy deficiency, stomatitis, gingivitis, hepatosplenomegaly, polyserositis, diabetes insipidus, osteolytic foci of the frontal bones were found. Results of the tests: anemia, thrombocytopenia, hypoproteinemia and hypoalbuminemia, coagulation disorders. The patient had the onset of lower flaccid paraparesis, muscle hypotonia. The boy was diagnosed with a number of infectious complications, including poliomyelitis (a derivative of vaccine poliovirus type 2), COVID-19. The child received LCH-III cytostatic therapy with a positive effect. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies.Copyright © 2023 Institute of Physics of the Russian Academy of Sciences. All rights reserved.

12.
Clinical Immunology ; Conference: 2023 Clinical Immunology Society Annual Meeting: Immune Deficiency and Dysregulation North American Conference. St. Louis United States. 250(Supplement) (no pagination), 2023.
Article in English | EMBASE | ID: covidwho-20234193

ABSTRACT

Background: Lymphoproliferation is the persistent proliferation of lymphoid cells and it's incidence in inborn errors of immunity varies from 0.7 to 18%. Material(s) and Method(s): This is a retrospective analysis of patients referred to the department of Immunology, B. J. Wadia Hospital for Children, Mumbai between March 2017 to December 2022. Inclusion criteria consisted of 3 months duration of significant lymphadenopathy and/or splenomegaly or history of lymphoma. The clinical characteristics, laboratory and molecular findings of the included patients were analyzed. Result(s): A total of 66 patients were included. There was a male preponderance with male:female ratio of 25:8. Median age of onset of lymphoproliferation was 4.75 years(Range 1 year to 60 years). Splenomegaly was seen in 75%. Infections included recurrent pneumonia (14/66), recurrent ear infections(5/66), COVID(4/66), one episode of pneumonia(6/66), herpes zoster(3/66), recurrent subcutaneous abscess (3/66), abdominal koch(3/66), chronic sinusitis(2/66), dermatophytosis(2/66), esophageal candidiasis(2/66), recurrent malaria(1/66), recurrent varicella(1/66), cryptococcal meningitis(1/66), gram negative sepsis(1/66), BCG adenitis(1/66), pseudomonas osteomyelitis(1/66), impetigo (1/66), pseudomonas urinary tract infection (1/66), chicken pox(1/66), herpes keratitis(1/66), dengue(1/66), Other manifestations included Evans plus phenotype(10/66), Evans phenotype(8/66), Autoimmune hemolytic anemia(5/66), bronchiectasis(5/66), Type 1 diabetes(3/66), hyper reactive airway disease(2/66), inflammatory bowel disease(4/66), autoimmune thrombocytopenia(2/66), stroke(3/66), hemophagocytic lymphohistiocytosis(2/66), hypertriglyceridemia(2/66), hypothyroidism(2/66), celiac disease(1/66), Type 2 diabetes(1/66), autoimmune encephalitis(1/66), autoimmune hepatitis(2/66), anti-parietal cell antibody(1/66), arthritis(1/66), autoimmune enteropathy(1/66), systemic lupus erythromatosus(1/66), primary biliary cirrhosis requiring liver transplant(1/66), nephrotic syndrome(1/66), lymphoedema(1/66), hypersplenism(1/66), recurrent oral ulcers(1/66), gout(1/66), dermatitis(1/66), ovarian teratoma(1/66), alopecia areata(1/66). Hodgkin's lymphoma(HL) was the most common malignancy(9/66), followed by non Hodgkin lymphoma(NHL)(6/66), transformation from NHL to HL(1/66), Burkitt to T-cell lymphoma(1/66), HL to DLBCL(1/66), HL to anaplastic T-cell lymphoma(1/66). EBV driven lymphoproliferation was seen in biopsy of21/66. Genetic testing showed mutations in LRBA(11/66), PIK3CD(5/66), CTLA4(3/66), TET2(2/66), IL2RA (1/66), IL12RB1(1/66), BACH2(1/66), PRKCD(1/66), TNFSFR13B(1/66), TNFAIP3(1/66), FAS(2/66), FASL(1/66), Caspase8(1/66), CARD11(1/66), RTEL1(1/66), AICD(1/66), PIK3R1(1/66), IKBKB(1/66). Treatment included IVIG, chemotherapy, rituximab, sirolimus, abatacept, HSCT. Conclusion(s): All children with persistent lymphoproliferation, with or without autoimmunity and/or infections should be worked up for an underlying monogenic disorder of immune dysregulation. Lymphomas presenting at abnormal site and/or age, relapse and EBV driven lymphomas require further evaluation. Presence of monogenic cause helps in providing targeted therapy.Copyright © 2023 Elsevier Inc.

13.
International Eye Science ; 23(5):873-877, 2023.
Article in Chinese | EMBASE | ID: covidwho-20234057

ABSTRACT

AIM: To explore the characteristics of astigmatism in preschool children before, during and after the COVID-19 epidemic, so as to provide a reference for further prevention and control of children's vision. *METHODS: In the consecutive four years from January 2018 to December 2021, a retrospective analysis of vision data was conducted on 2 273 preschool children (4546 eyes) younger than 4 years old who participated in children' s vision screening test in Baiyun district, Guangzhou. They were divided into 1 - year old group (ages<1-year old, 420 cases), 2-year old group (1-year <= ages < 2- year, 543 cases), 3- year old group (2- year <=ages <3-year, 614 cases), and 4 - year old group (3 - year <= ages< 4- year, 696 cases) according to ages. The analysis included astigmatic degrees of children's eyes as well as their conditions of astigmatism. *RESULTS: In 2018, the astigmatic degrees of the both eyes of 1-year-old group were higher than those of other groups (P<0.05). The binocular astigmatic degrees of the preschool children in four groups were obviously higher in 2020 than 2019 (P < 0. 05), while they were significantly decreased in 2021 when compared with 2020 (P < 0. 05). From 2019 to 2020, the increase of astigmatic degrees of the right eye is more considerable than the left eye of preschool children in those four groups (P < 0. 001). Furthermore, the morbidity of astigmatism basically echoes with the changing tendency of astigmatic degrees from 2018 to 2021. *CONCLUSIONS: Preschool children in Baiyun district, Guangzhou, have the highest degree of astigmatism and the fastest progression rate within 1 year old. Before COVID - 19 epidemic, the changes in astigmatism and prevalence were relatively stable;during COVID - 19 epidemic, the astigmatism and prevalence increased significantly and the astigmatic degrees of right eye increased more than that of the left eye;after the normalization of epidemic prevention and control, the astigmatism and prevalence decreased significantly.Copyright © 2023 International Journal of Ophthalmology (c/o Editorial Office). All rights reserved.

14.
Journal of Clinical Rheumatology ; 29(4 Supplement 1):S70-S71, 2023.
Article in English | EMBASE | ID: covidwho-2322254

ABSTRACT

Objectives: As of March 5th, 2022, around 1.585 cases of MIS-C and 98 deaths (6,4%) were reported in Brazil. The state of Rio de Janeiro State (RJ) having 94 cases (5,9%) and 4 deaths (4,2%)1.Our aim was to evaluate clinical and laboratory features, and management of MIS-C in seven pediatric hospitals in RJ, Brazil. Method(s): Multicenter, observational, ambidirectional cohort study in seven tertiary hospitals in RJ(Brazil), assessing medical charts of pediatric inpatients (0-18 years) diagnosed with MIS-C according to WHO/CDC criteria, from August, 2020 to February, 2022. Descriptive statistics were used to analyze distributions of continuous variables, frequencies, and proportions. Result(s): A total of 112 cases of MIS-C were enrolled. The mean age was 4.2 years and thre was male predominance (59,8%). All cases had a SARS-CoV-2 contact (29.5% close contact;31.3%:positive PCR;serology:43.8%).Only 12.5% had comorbidities. Length of stay (LOS) was 7 days.Median duration of fever was 8 days. Most common symptoms were: rash(67%);gastrointestinal (67%);conjunctivitis (42%);neurological(39.6%);cardiovascular(37.5%);cervical lymphadenopathy (36.6%), and shock/hypotension(28.6%).Co-infection occurred in 3 patients. Forty-four patients fulfilled criteria for Kawasaki disease. Most patients were admitted to PICU(12;62,5%) for amedian of 2 days. Respiratory distress was seen in 18,7%;hypotension:28,6%, and shock in 23,2%. Main laboratory findings were: high C-reactive protein in 95%;D-dimer:77%, anemia:77%, thrombocytosis:63%;transaminitis:43.8%, lymphopenia:38%;hypoalbuminemia:34%;thrombocytopenia: 29%;hypertriglyceridemia:28%, and high pro-BNP in 27%. Echocardiogram was performed in 91/112 patients;abnormal in 70,3%;exhibiting myocardial dysfunction( 25%);pericardial effusion(21%);coronary dilation/aneurysms(11%) and, valvulitis (14.5%). IVIG+corticosteroids (CTC) were administered in 59.8%(67/ 112);18.6%(18/112) IVIG only;10.7%(12/112) CTC only;3.4%(4/112)biologics, and 15(13.3%) received no treatment. ASA low dose in 77.7% (87/112) and moderate/high dose in 34.8%. Oxygen support was needed in 27,7%;vasoactive amines:18,7%;dialysis:5,3%, and transfusion:18,7%.One patient died from a cytokine storm syndrome. Conclusion(s): Our study reports a higher number of MIS-C cases in RJ than the number reported to Brazilian authorities, highlighting underreporting. Our patients were younger, had fewer comorbidities, cardiovascular/gastrointestinal/renal involvement, shortest LOS in ICU, and a higher frequency of myopericarditis.

15.
Infectious Diseases: News, Opinions, Training ; 10(2):47-53, 2021.
Article in Russian | EMBASE | ID: covidwho-2325957

ABSTRACT

Acute respiratory viral infections (ARVI) play an important role in morbidity formation among children. At the same time, studies about the ARVI etiological structure are not enough. The article presents the results of structure analyses of ARVI in children with severe and moderate degrees of disease hospitalized in the children's clinical hospital of Novosibirsk for the period 2015-2018. This research aimed to analyze the morbidity of acute respiratory viral infections with the estimation of a causal virus in children admitted to the hospital for the period 2015-2018. Material and methods. In this study, 1137 children aged between 0 and 15 years were examined. In order to determine the etiological factor in children with damage of the upper or lower respiratory tract, by using the method of RT-PCR (AmpliSensARVI-screen-FL test systems (InterLabService, Russia), mucus from the nose and throat was examined for the presence of genetic material of viruses that cause ARVI (influenza A and B viruses, parainfluenza viruses of types 1-4, respiratory syncytial virus, metapneumovirus, four types of human coronavirus, rhinovirus, adenovirus, and bocavirus). Results. The research found that the most frequently detected pathogens are respiratory syncytial virus (23.52%), influenza A and B viruses (19.73%) and rhinovirus (19.21%). Observe the dynamics some fluctuations in the detection of mentioned viral agents and increasing of mixed infections were detected. In addition, the importance of respiratory and gastrointestinal tract combined lesions, particularly for infants and preschool - age children has been noted. Conclusion. The distribution of respiratory viruses in children with severe ARVI who required hospitalization was assessed. It was shown the significance of the respiratory syncytial infection virus, influenza virus and rhinovirus in the etiological structure of hospitalized children of different ages that damage not only the respiratory tract, but also to the gastrointestinal tract. This is an important factor in optimizing the diagnosis, treatment and prevention of viral infections in children.Copyright © Infectious Diseases: News, Opinions, Training 2021.

16.
Heart Rhythm ; 20(5 Supplement):S582, 2023.
Article in English | EMBASE | ID: covidwho-2325582

ABSTRACT

Background: Stereotactic radiotherapy (SBRT) is a new therapeutic option for patients with scar related ventricular tachycardia (VT). Objective(s): To describe our experience with the use of SBRT for the treatment of recurrent VT in patients with Chagas Cardiomyopathy (CCM) in whom catheter ablation is not an option. Method(s): We selected patients with Chagas Disease that underwent SBRT for recurrent VT treatment. The target sites of SBRT were planned based on CMR and CT reconstruction on ADAS software, bipolar voltage maps from previous CA procedures and VT morphology induced on a electrophysiologic study performed SBRT planning. Target sites were decided together by electrophysiology and radiation oncology group. Result(s): We performed SBRT in six CCM patients July 2021 to July 2022. Most patients were male (66.7%), mean age 62.3+/-5.7 years-old and EF 28.5% (Q1: 20 Q3:42.7). One patient (16.7%) had two prior catheter ablation, four (66.7%) had one and one patient had no prior ablation, but had severe pulmonary fibrosis after COVID and was O2 dependent. The mean PTV (planning target volume) was 85+/-14 mL and the ITV (internal target volume) was 29+/-4 ml, with safe constraints regarding the esophagus and stomach. In a mean FU of 244+/-173days, 3 (50%) patients presented VT recurrence after blanking period. Two patients died 86 and 50 days after SBRT. The median number of VT episodes reduced from 13 (6.25;44.75) to 7.5 (3;7.5) (P = 0.093). All alive patients stop presenting VT in a median period of 174 (Q1: 44.75: Q3: 199) days, being at the end of the follow-up in a median of 196 (Q1: 137;Q3: 246) days without new VT episodes. Conclusion(s): SBRT presents a high rate of early recurrence in Chagas disease patients that improves during timeCopyright © 2023

17.
International Journal of Infectious Diseases ; 130(Supplement 2):S58, 2023.
Article in English | EMBASE | ID: covidwho-2325450

ABSTRACT

Intro: COVID-19 Vaccination has proven to be very effective in preventing infection and progression to severity and death. However, there were concerns about very rare but potentially fatal adverse reactions after vaccination;myocarditis/pericarditis, TTS/VITT et al. It suggested that the evaluation of the two values of personal safety and public benefit is necessary. Method(s): The benefit of vaccination was measured by the number of critically ill patients prevented from vaccination. The number of critically ill patients predicted in the future was measured through two Methods: based on a fixed scenario, and using a mathematical model. Damage through vaccination was calculated as the occurrence of TTS/VITT, Myocarditis/Pericarditis, and of severe cases. Finding(s): The evaluation results on vaccine safety and effectiveness were made in the form of age restrictions for vaccination by each vaccine platform. As a result of the evaluation, the AstraZeneca vaccine was limited to those under the age of 30 but there was no restriction on the age of mRNA vaccination. In addition, the risks and benefits of vaccination for children aged 5-11 years and 12-17 years of age were evaluated respectively, and it was confirmed that the benefits of vaccination outweigh the potential harm in children and adolescents. Conclusion(s): Our nation has the own policy for COVID 19 vaccination from the results. The pandemic situation has presented a new approach to the benefits and risks of large-scale vaccination. In particular, the method of comparing the risks and benefits of vaccination was considered as a useful method for health communication.Copyright © 2023

18.
International Journal of Infectious Diseases ; 130(Supplement 2):S132, 2023.
Article in English | EMBASE | ID: covidwho-2325221

ABSTRACT

Intro: Children and Young people were the last group in England be offered COVID-19 vaccination (from September 2021), thus were the largest susceptible group when SARS-CoV-2 Omicron variants emerged. We monitored vaccine and naturally-derived antibodies in schools between November 2021 and March 2022. Method(s): We conducted three large surveys (November 2021, January and March 2022) in a nationally representative random sample of primary and secondary schools, stratified by regions. Oral fluid samples were tested for IgG antibodies against SARS-CoV-2 nucleocapsid (anti-NP) and spike (anti-S1) proteins using novel validated ELISAs;vaccines used in England elicit anti-S1 antibodies only. We calculated weighted prevalences for each survey, and used multilevel logistic regression to investigate associations with socio-demographic factors. Finding(s): Overall 11311 students contributed 22478 biological samples (respectively 4840, 7549 and 10089 in rounds 1, 2 and 3, with similar socio- demographic characteristics). In 4-11 year olds, not eligible for vaccination, anti- S1 and anti-NP antibody prevalences were 31.3% and 26.6%, 46.2% and 43.8%, and 53.4% and 58.7% respectively over the three rounds. The corresponding estimates in 12 to 18 year olds) were 70.7% and 34.6%, 85.6% and 45.9%, 89.0 and 53.9%. In November 2021 (before Omicron dominance), higher anti-S1 antibody positivity was associated with older age and Black ethnicity, and non- eligibility for free school meals in 4-11 year olds. In 12-18 years it was associated with non-eligibility for free school meals. In March 2022 when Omicron dominated, these associations remained, together with urban location in 4-11 years old. Conclusion(s): The steep increase in 4-11 year olds in both sets of antibodies reflected the emergence and spread of highly infectious Omicron variants whilst high and increasing anti-S1 prevalence in secondary students was consistent with greater vaccine uptake. Socioeconomically deprived 12-18 year olds had lower odds of anti-S1, suggesting lower vaccine uptake or access.Copyright © 2023

19.
Paediatr Child Health ; 28(3): 184-202, 2023 Jun.
Article in English, French | MEDLINE | ID: covidwho-2325613

ABSTRACT

COVID-19 transformed the family media environment and spurred research on the effects of screen media exposure and use on young children. This update of a 2017 CPS statement re-examines the potential benefits and risks of screen media in children younger than 5 years, with focus on developmental, psychosocial, and physical health. Four evidence-based principles-minimizing, mitigating, mindfully using, and modelling healthy use of screens-continue to guide children's early experience with a rapidly changing media landscape. Knowing how young children learn and develop informs best practice for health care providers and early years professionals (e.g., early childhood educators, child care providers). Anticipatory guidance should now include child and family screen use in (and beyond) pandemic conditions.

20.
Pediatric Hematology Oncology Journal ; 7(2):52-53, 2022.
Article in English | Scopus | ID: covidwho-2320584
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