Objective Considering the concerns regarding the coronavirus disease‐2019 (COVID‐19) vaccine safety among pediatric patients with inflammatory rheumatic diseases (IRD) due to a lack of data, an urgent need for studies evaluating safety profiles of vaccines emerged. Methods Among participants vaccinated by CoronaVac inactive SARS‐CoV‐2 or BNT162b2 messenger RNA (mRNA) COVID‐19 (Pfizer‐BioNTech) vaccine, healthy children under 18 and patients under 21 with an at least 1‐year follow‐up period in our department for a childhood‐onset rheumatic disease were included into this cross‐sectional study. Results Overall, 246 subjects (141 [57.3%] females) (biologic group: 43, non‐biologic group: 180, healthy control group: 23) were eligible for the study. The median age was 15.34 (12.02‐20.92) years. The most common adverse events were fatigue (n = 68, 27.6%), headache (n = 44, 17.9%), myalgia (n = 38, 15.4%), arthralgia (n = 38, 15.4%), and fever (n = 35, 14.2%). Only 3 subjects (2 patients with familial Mediterranean fever, and one healthy child) were considered to experienced serious adverse events, since they required hospitalization. Local reactions were seen in 20 (8.13%), and 27 patients (12.1%) had disease flares within 1 month after the vaccines. Although it was significantly higher in those who received the BNT162b2 mRNA vaccine (P < .001), there was no significant relationship between adverse event frequency and age, gender, the existing diseases, ongoing treatment regimens and pre‐vaccination COVID‐19 histories. Conclusion Although immunogenicity studies for efficacy of the vaccines and long‐term follow‐up studies for adverse events monitoring are required, our study indicates an acceptable safety profile of COVID‐19 vaccines and encourages children with IRD to be vaccinated.
Background: Since the COVID-19 pandemic became a serious health concern globally, patients with chronic diseases have required close attention with regard to general risks and individual treatment. We aimed to reveal the general health status of pediatric asthmatic patients during the pandemic, considering the role of household factors in parental attitudes. Methods: We asked 60 asthmatic patients and their parents to respond to a questionnaire, with the aim of revealing the current health status of the patients and the general approach of the family to asthma management during the pandemic. Results: A total of eight patients had had an asthma attack during the outbreak, but there was no confirmed correlation with COVID-19 infection. Most of the parents had never considered stopping their children's current medications. However, the majority of them reported concerns about the failure of the ambulatory care services and almost all saw their children as being at high risk for COVID-19 infection. There was no significant relationship between these concerns and their psychological status (P > 0.05). Conclusions: The crucial point regarding asthma management is to control patients' medical and psychological status to minimize the effects of the pandemic. Healthcare professionals should also pay attention to members of the patients' households because their adaptation to the "new normal" of pandemic may directly affect the patients' state of health.
Objectives: Primary ciliary dyskinesia (PCD) is a chronic genetic disease that affects the respiratory tract, characterized by different clinical and laboratory features. It has a very difficult diagnosis, and high morbidity. In recent years, with the advances in genetics, the rate of diagnosis has increased considerably. In this study, it was aimed to evaluate the relationship between PCD patients’ clinical, radiological and laboratory features and genetic analysis. Methods: The study included 14 children who were diagnosed with PCD between 2015-2019 and underwent exome analysis. Diagnostic ages, body mass indexes (BMI)- Z score, clinical and radiological findings, pulmonary function tests, sputum culture reproduction and gene analysis were evaluated and compared. Results: Six of the patients (43%) were girls and 8 (57%) were boys, and the median age at the time of diagnosis was 9 (min-max: 3-16) years. Genetic analysis revealed pathogenic mutations in DNAH5 (n=4, 29%), DNAH11 (n=2, 14%), RSPH4A (n=2, 14%), CCDC40 (n=2, 14%), DNAH9 (n=1, 7%), HYDIN (n=1, 7%), DNAH1 (n=1, 7%), and ARMC4 (n=1, 7%). Although not statistically significant, it was found that the diagnosis age was lower and the BMI Z-score was lower in CCDC40 mutations. Growth parametres were normal in DNAH5, DNAH11, RSPH4A and ARMC4 pathogenic variants. No significant correlation was found between genetic analysis and clinical features, culture reproduction and pulmonary function tests of the patients. Conclusion: It is thought that more detailed information about the possible clinical features and prognosis of the disease can be obtained by genetic examinations of PCD. However, clinical trials with higher patient numbers are still needed.
A limited number of cases of thrombotic microangiopathy (TMA) have previously been reported in association with COVID‐19. Our report describes two cases of TMA associated with COVID‐19, one of which was successfully treated with eculizumab. The first case was a 23‐month‐old girl, and the second case was a 9‐month‐old boy. PCR tests for SARS‐CoV‐2 were positive in both cases, and laboratory results showed microangiopathic haemolytic anaemia, thrombocytopenia, and acute kidney injury. No known aetiology for TMA was found in either case. Stool tests for Shigatoxin‐producing Escherichia coli were negative. Coagulation tests, ADAMTS13 activity, serum complement levels, and homocysteine levels were all within the normal range. No known genetic mutation was found, including mutations of complement, diacylglycerol kinase epsilon, and cobalamin C. In the first case, eculizumab was administered due to persistent haemolysis and prolonged anuria. In conclusion, TMA may be associated with COVID‐19 infection. Treatment with eculizumab may be beneficial in selected patients because of the potential activation of the complement system.
Background. Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation of teratoma in the form of carcinoma, rhabdomyosarcoma and leukemia have previously been reported on rare occasions. Case. A 19 month-old-girl was referred with a presacral mass of 29mm x 23mm x 24mm. She was diagnosed as Currarino syndrome. The presacral mass was surgically resected and pathological examination revealed a foci of primitive neurectodermal tumor. Conclusions. This is the first case of Currarino syndrome with a primitive neuroectodermal tumor (PNET) foci in the presacral mass. Considering the risk of malignant transformation, the accurate pathological examination is important for complete systemic evaluation and treatment plan in these children.
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