To retrospectively evaluate the imaging and clinical findings of patients diagnosed with multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19.
The clinical, laboratory and ...radiological data of 110 patients (74 male and 36 female) diagnosed with COVID-19-related MIS-C between June 2020 and November 2021 were evaluated retrospectively. Cases with a diagnosis of MIS-C based on a positive real time polymerase chain reaction (RT-PCR) test or serology results according to the WHO criteria were included in the study. All the radiological data were evaluated by a pediatric radiologist with 14 years of radiology experience.
Peribronchial thickening and hyperinflation were the most common findings on chest X-ray, while atelectasis and pleural effusion were often present in thoracic CT. Cardiac involvement was detected in 30% of the patients, mainly with valve insufficiency and systolic dysfunction, and 7.2% of these patients had sequalae findings. The most common abdominal findings were hepatosplenomegaly, mesenteric inflammation, lymphadenomegaly, thickening of the intestinal walls and free fluid. 23 of the patients had comorbidities. Neurological radiological findings observed in a total of six patients were reversible splenial lesion syndrome, posterior reversible encephalopathy syndrome, meningitis, and cerebral edema. 37 patients were followed up in the intensive care unit and 2 of them died.
Radiological findings seen in MIS-C vary according to the affected system. There is no specific radiologic finding for this disease, but radiological findings can assist in the evaluation of affected systems and guide treatment.
Since there are few studies with a limited number of patients in the literature, data on this subject are limited. We aimed to contribute to the literature with our large patient data.
The COVID-19 pandemic has affected many aspects of life as well as hospital admissions. We hypothesized that many infectious diseases and hospitalizations in the pediatric age group might have ...decreased during the pandemic period.
Evaluate patients admitted to the general pediatric wards during the pandemic in comparison with the pre-pandemic period.
Retrospective cross-sectional SETTING: General pediatrics wards of a tertiary hospital in Istanbul PATIENTS AND METHODS: The study included patients aged 0-18 years who were followed up while hospitalized in the general pediatrics wards between 11 March 2019 and 11 March 2021. The hospitalizations were grouped as pre-pandemic and pandemic based on the date when COVID-19 was declared a pandemic (11 March 2020).
Hospital admissions, length of stay, diagnoses, gender, age.
4343 hospitalizations.
Of the total 4343 hospitalizations meeting the inclusion criteria, 2786 (64.1%) occurred before the pandemic and 1557 (35.9%) during the pandemic, a 44% decrease. The distribution of all hospitalization diagnoses during the two years was as follows: respiratory tract diseases, 1768 (40.7%); neurological diseases, 946 (21.8%); gastrointestinal diseases, 550 (12.7%); hematological and oncological diseases, 514 (11.8%); genitourinary system and nephrological diseases, 504 (11.6%); and soft tissue infections, 255 (5.9%). During two years, there were 1418 (32.7%) patients with lower respiratory tract infections, 316 (7.3%) with gastroenteritis, and 440 (10.1%) with urinary system infections. The median hospital stay was 6 days before the pandemic and 4 days during the pandemic (
<.0001). During the pandemic, the rate of respiratory diseases decreased from 48.7 to 26.5%, and that of lower respiratory tract infections decreased from 40.5 to 18.6% (
<.0001).
Both previous studies and our results indicate that many infectious diseases in the pediatric age group significantly decreased, especially in the first months of the COVID-19 pandemic.
Single-center study.
None.
We aimed to comprehensively analyse the available literature to identify the unmet requirements in transitional programs tailored specifically for patients diagnosed with JIA.
According to published ...guidance on narrative reviews, a systematic review of the literature on transitional care in rheumatology was conducted. Pertinent documents were collected from reputable databases, such as Web of Science, Scopus, and MEDLINE/PubMed. The search encompassed literature published from the inception of each database until January 2023.
In this study, a comprehensive analysis of the findings of 34 studies was conducted. Among these, 12 studies focused on assessing the readiness of adolescents and young adults diagnosed with JIA. Additionally, 18 studies examined the effectiveness of structured transition programs in terms of adherence and satisfaction. Finally, 4 studies investigated disease-related outcomes in this population.
The need for transitioning children with rheumatic diseases to adult rheumatology services for continued care is clearly evident. However, the absence of established best practice guidelines presents a challenge in facilitating this transition effectively. Although several scoring systems have been proposed to ensure organized and seamless transfers, a consensus has not yet been reached. Furthermore, the socio-economic and cultural variations across countries further complicate the development of universal guidelines for transitioning children with rheumatic diseases. To address these concerns, our objective in conducting this literature review was to emphasize the significance of this issue and identify the specific requirements based on the unmet needs in the transition process.
To investigate the rates of ocular complications and visual loss and their risk factors in patients with juvenile idiopathic arthritis (JIA) -associated uveitis
Medical records of 51 patients were ...reviewed.
The incidence of visual loss to the 20/50 or worse was found to be 0026/ eye-year (EY) in the present study. Cataract and ocular hypertension occurred during the follow-up period and were significantly associated with visual acuity loss to 20/50 or worse (p = .008, HR 11.932, 95% CI 1.915-74.355; p = .03, HR 7.323, 95% CI 1.216-44.110, respectively). Anti-TNF therapy was initiated in 88.2% of our cases and 93.3% of them achieved uveitis inactivity.
The risk of vision loss is higher in patients with JIA-associated uveitis who had complications at presentation. We attribute the lower complication rates and better visual outcomes in our study to the early and frequent use of biologic agents.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Background. Enthesitis is an extra-intestinal manifestation of inflammatory bowel disease (IBD) which often remains underdiagnosed in children. We aimed to evaluate the frequency of enthesitis in ...pediatric IBD patients using physical examination and ultrasound (US) assessment as the reference standard. Methods. 31 children, 7 -18 years of age, diagnosed with IBD were recruited for a cross-sectional study. All subjects completed a study questionnaire and underwent both physical and US examination for the presence of the enthesitis. Results. Of 31 subjects (17girls; median age 14(6) years) enrolled, 17 (55%) had ulcerative colitis, 11 (35%) had Crohn`s disease, and 3 (10%) had indeterminate colitis. The median time from IBD diagnosis was 1.2 years. At least one enthesitis (range 1-4) was identified in 14 (45%) patients of whom nine had more than one enthesitis with symmetric involvement in eight. The quadriceps femoris insertion at the superior portion of the patella was the frequently involved site (32%, 9 of 28 sites), followed by patellar ligament insertion at tibial tuberositas. The presence of enthesitis was associated with a higher intensity of the musculoskeletal pain (p=0.018), but physical activity remained unaffected (p=0.056). Conclusions. Enthesitis is a common underestimated extra-intestinal manifestation of IBD that may impact the musculoskeletal health of children. Future studies with more extensive cohorts are needed to evaluate enthesial involvement both with physical examination and US in order to predict the long-term outcomes of the enthesitis on children with IBD.
Background: Little is known about the long-term efficacy and safety of canakinumab in paediatric FMF patients.
Aim: To present the single centre experience of colchicine-resistant paediatric-onset ...FMF patients who were treated with canakinumab by off-label use since 2012.
Methods: The hospital files of 15 children who used canakinumab were retrospectively evaluated. Clinical and laboratory data of each visit were recorded. Drug-related adverse events were recorded. Complete remission was described as no attacks and normal acute phase reactants; partial remission was defined as decrease in severity and rate of attacks and/or elevated acute phase reactants with anti-IL-1 treatment.
Results: The average duration of canakinumab use was 23.9 months (min:12, max:58 months). Twelve patients were M694V homozygotes. Eleven patients achieved complete remission after the first dose at 2 months and 12 patients at 6 months. Canakinumab interval was shortened in 2 patients from 150 mg/8 weeks to 150 mg/4 weeks. Except one, 14 patients achieved complete remission by 12 months. Two patients had mild urinary tract infections. One patient had bronchopneumonia requiring hospitalization. Two patients had teeth abscess. There were no serious adverse events such as opportunistic infections, malignancies, or deaths. Besides, no significant laboratory abnormalities occurred in complete blood count parameters, liver and kidney function tests.
Conclusion: To the best of our knowledge, this is the longest outcome study about canakinumab use in paediatric FMF patients. This study suggested that canakinumab is safe and effective in children with FMF in the long term.
To develop a set of clinical criteria that identifies patients with a potential autoinflammatory IFNopathy.
Based on a literature review, a set of clinical criteria identifying genetically confirmed ...monogenic IFNopathies was selected. For validation, the clinical score was assessed in healthy controls (HCs) and 18 disease controls, including 2 known autoimmune IFNopathies, juvenile systemic lupus erythematosus (JSLE, n = 4) and dermatomyositis (JDM, n = 4); adenosine deaminase 2 deficiency (DADA2, n = 4); and oligoarticular juvenile idiopathic arthritis (oJIA, n = 6). We assessed an IFN score (IRG-S) in whole blood by NanoString using a previously published 28-gene-IRG-S and a reduced 6-gene-IRG-S.
The 12 patients with a possible IFNopathy had higher clinical scores (3-5) than the patients with sJLE, JDM, DADA2, and oJIA and in HCs. Both the 28-IRG-S and 6-IRG-S were significantly higher in the autoinflammatory IFNopathy patients compared to HCs and oJIA and DADA2 patients but not different from patients with JSLE and JDM. Subsequently, genetic analysis revealed mutations in genes previously reported in genes related to the IFN pathway in 9 of the 12 patients.
We developed a clinical score to identify patients with possible autoinflammatory IFNopathies. A clinical score was associated with a high IRG-S and may serve to identify patients with an autoinflammatory IFNopathy.
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