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Author(s): Sonia M. Bramanti [sup.1] , Valerio Manippa [sup.1] [sup.2] , Alessandra Babore [sup.1] , Anna Dilillo [sup.3] , Alessia Marcellino [sup.3] , Vanessa Martucci [sup.3] , Saverio Mallardo [sup.3] [...], The COVID-19 pandemic could be a threat for the health status of children with a chronic condition. The present study aimed to explore parents' and children's psychological adjustment during the current pandemic, pursuing a triple objective: to compare the psychological adjustment of parents of children with pediatric rheumatic diseases (PRDs) and parents of healthy children; to analyze children's psychological symptoms (emotional problems and hyperactivity) before and during the COVID-19 pandemic, and with or without a PRDs diagnosis; to explore the associations of children's emotional problems and hyperactivity with parents' psychological adjustment, parent-child interactions and belonging or not to families with PRDs. This cross-sectional study involved 56 parents of children with PRDs and 53 parents of healthy children. Self-report questionnaires about parents' depression, anxiety, parenting stress, and children's emotional symptoms and hyperactivity-inattention were administered. No differences were detected on psychological adjustment between parents of children with PRDs and parents of healthy children. Parents of children with PRDs reported statistically significant higher levels of children's emotional problems and hyperactivity before the pandemic, compared to parents of healthy children; during COVID-19 pandemic, emotional symptoms increased for both groups, while hyperactivity-inattention symptoms increased only in the group of healthy children. Children's emotional difficulties were associated with higher levels of parental anxiety, worse parent-child interaction and having PRDs; children's hyperactivity symptoms were related to parent-child difficult interaction and higher levels of parental depression. Findings suggest the importance to target the children in relation to their parents, when approaching the psychological aspects of PRDs.

Tertiary hyperparathyroidism is characterized by hypercalcemia resulting from autonomous parathyroid hormone production and usually occurs after a prolonged period of secondary hyperparathyroidism. This condition can be a complication of X-linked hypophosphatemia (XLH), a rare genetic disease characterized by renal phosphate loss and consequent hypophosphatemia. Parathyroidectomy is considered the first-line therapy but surgical intervention can be complicated by hungry bone syndrome. A male Caucasian patient presented with XLH, diagnosed at the age of 3 years. At the age of 21, tertiary hyperparathyroidism occurred. Neck ultrasonography, neck magnetic resonance imaging, and 99Tc-sestamibi parathyroid scintigraphy revealed two hyperplastic parathyroid glands. To minimize the risk of hypercalcemia, calcimimetic therapy was initiated. After 6 months and preparation with 1,25-dihydroxy vitamin D, the patient underwent total parathyroidectomy with autotransplantation of half of a parathyroid gland into the sternocleidomastoid muscle. Histopathological examination revealed diffuse microscopical hyperplasia of the parathyroid glands. Despite oral supplementation with calcium carbonate and calcitriol, severe hypocalcemia developed on the second postoperative day, attributable to hungry bone syndrome. This finding was confirmed by an increase in bone turnover markers and a reduction in urinary calcium excretion. Hypocalcemia correction required continuous infusion of calcium gluconate for over 2 months. After approval, the patient began burosumab therapy with significant benefits. This case illustrates the complexity of treating tertiary hyperparathyroidism and mineral metabolism in patients with XLH. The hungry bone syndrome can complicate parathyroidectomy, exposing the patients to life-threatening risks. Burosumab therapy may reduce the risk of tertiary hyperparathyroidism developing in these patients. [ABSTRACT FROM AUTHOR]

Cesarean section is considered a possible trigger of atopy and gut dysbiosis in newborns. Bifidobacteria, and specifically B. bifidum, are thought to play a central role in reducing the risk of atopy and in favoring gut eubiosis in children. Nonetheless, no trial has ever prospectively investigated the role played by this single bacterial species in preventing atopic manifestations in children born by cesarean section, and all the results published so far refer to mixtures of probiotics. We have therefore evaluated the impact of 6 months of supplementation with B. bifidum PRL2010 on the incidence, in the first year of life, of atopy, respiratory tract infections, and dyspeptic syndromes in 164 children born by cesarean (versus 249 untreated controls). The results of our multicenter, randomized, and controlled trial have shown that the probiotic supplementation significantly reduced the incidence of atopic dermatitis, upper and lower respiratory tract infections, and signs and symptoms of dyspeptic syndromes. Concerning the gut microbiota, B. bifidum supplementation significantly increased α-biodiversity and the relative values of the phyla Bacteroidota and Actinomycetota, of the genus Bacteroides, Bifidobacterium and of the species B. bifidum and reduced the relative content of Escherichia/Shigella and Haemophilus. A 6-month supplementation with B. bifidum in children born by cesarean section reduces the risk of gut dysbiosis and has a positive clinical impact that remains observable in the following 6 months of follow-up. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) has been chiefly linked with substantial respiratory complications. However, emerging studies have brought attention to the occurrence of severe muscle inflammation (myositis) related to COVID-19, potentially leading to multi-organ failure and increased mortality. Myositis is generally characterized by heightened serum creatine kinase (CK) levels. Acute myositis is characterized by an infiltration of viruses into calf muscle fibers, which may cause a subsequent inflammatory response leading to calf muscle pain. Symptomatic and supportive management, along with explanation and reassurance, is all that is required in managing this condition. While the association between myositis and severe outcomes has been recognized in adults, it remains less understood in the pediatric population. The current retrospective study, conducted at Policlinico San Marco University Hospital in Catania, aimed to analyze clinical and laboratory factors associated with myositis in pediatric patients with SARS-CoV-2 infection. Between January 2022 and January 2023, ten pediatric patients diagnosed with myositis and SARS-CoV-2 infection were evaluated. The study highlighted clinical manifestations such as fever, calf muscle pain, and abnormal gait. Lab results showed elevated CK levels among other findings. All patients underwent treatment, with the majority recovering without complications. A notable correlation was observed between CK levels, blood urea nitrogen (BUN), and the urea/creatinine ratio (UCR). The study also discusses potential pathophysiological mechanisms behind SARS-CoV-2's impact on skeletal muscles, emphasizing an indirect inflammatory response. Our findings underscore that while myositis in children with SARS-CoV-2 infection appears to follow a benign and self-limiting trajectory, it is crucial to monitor specific markers for early intervention and management. Further research is warranted to elucidate the underlying mechanisms and improve clinical outcomes. [ABSTRACT FROM AUTHOR]

Background: Pediatric rheumatic diseases (PRDs) are a group of chronic disorders that start in childhood and are characterized by periodic exacerbations and remissions of symptoms, with limitations in family, school, and social activities. The aim of this study was to detect differences in parents' psychological adjustment and emotion regulation strategies, and parent-reported children's adjustments in families of children with active and inactive PRDs. Methods: Fifty-four parents (38 mothers and 16 fathers) of children with PRD were recruited from a pediatric unit. Disease activity was evaluated by their pediatric rheumatologist, while parents' depressive and anxiety symptoms, emotion regulation strategies, and children's emotional difficulties and hyperactivity–inattention symptoms were assessed through a web-based survey. Results: Parents of children with active PRDs reported higher levels of their child's emotional difficulties and hyperactivity–inattention symptoms. Linear regression analysis demonstrated that having a child in the active phases of PRD and lower use of cognitive reappraisal lead to higher children's emotional symptoms, while active disease, low use of cognitive reappraisal, and greater expressive suppression were associated with higher hyperactivity–inattention symptoms. Our study highlights that children with PRDs and their parents may be at increased risk for psychological problems, especially during the active disease phase, highlighting the importance of a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Psoriatic disease is a chronic, relapsing inflammatory disorder, characterized mostly by cutaneous erythematous scaly plaques sometimes associated with arthritis. Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease of the apocrine glands, characterized clinically by painful abscesses, sinus tracts and scars. It typically occurs after puberty, affecting mainly intertriginous areas of the body. There is a strong association between HS and psoriasis since they share the same pathogenic inflammatory pathway. The patient presented: low birthweight, microcephaly, facial dysmorphisms, lumbar hyperlordosis, walking difficulties, global psychomotor developmental delay and learning disabilities. A genetic evaluation revealed a 2.5 Mb de novo microduplication in the 17q21.31 chromosomal region. Dermatological examination revealed HS (Hurley stage II-HS) distributed in the genital area and inguinal folds, psoriatic plaques on the retroauricolar folds, on the elbows bilaterally and on the lateral aspect of the right ankle and psoriatic arthritis. The patient was treated with adalimumab, with a marked improvement of both conditions. To our best knowledge, we report the first case of coexisting Psoriatic Arthritis Disease and Hidradenitis Suppurativa in a child with chromosome 17q21.31 microduplication syndrome. We hypothesize that gene CRHR1 duplication included in the 17q21.31 chromosomal region might be involved in the pathogenesis of both diseases. [ABSTRACT FROM AUTHOR]

Point-of-care thoracic ultrasound at the patient's bedside has increased significantly recently, especially in pediatric settings. Its low cost, rapidity, simplicity, and repeatability make it a practical examination to guide diagnosis and treatment choices, especially in pediatric emergency departments. The fields of application of this innovative imaging method are many and include primarily the study of lungs but also that of the heart, diaphragm, and vessels. This manuscript aims to describe the most important evidence for using thoracic ultrasound in the pediatric emergency setting. [ABSTRACT FROM AUTHOR]

Background: infectious mononucleosis is very common during childhood and neurological manifestations are extremely rare. However, when they occur, an appropriate treatment must be undertaken to reduce morbidity and mortality as well as to ensure appropriate management. Methods: we describe the clinical and neurological records of a female patient with post-EBV acute cerebellar ataxia, whose symptoms rapidly resolved with intravenous immunoglobulin therapy. Afterwards, we compared our results with published data. Results: we reported the case of an adolescent female with a 5-day history of sudden asthenia, vomiting, dizziness, and dehydration, with a positive monospot test and hypertransaminasemia. In the following days, she developed acute ataxia, drowsiness, vertigo, and nystagmus with a positive EBV IgM titer, confirming acute infectious mononucleosis. The patient was clinically diagnosed with EBV-associated acute cerebellitis. A brain MRI showed no acute changes and a CT scan showed hepatosplenomegaly. She started therapy with acyclovir and dexamethasone. After a few days, because of her condition's deterioration, she received intravenous immunoglobulin and demonstrated a good clinical response. Conclusions: although there are no consensus guidelines for the treatment of post-infectious acute cerebellar ataxia, early intervention with intravenous immunoglobulin might prevent adverse outcomes, especially in cases that do not respond to high-dose steroid therapy. [ABSTRACT FROM AUTHOR]

The 4q deletion syndrome defines a disorder, which may involve patients affected by either the deletion of the interstitial region from the centromere to 4q31 or by the deletion of the terminal region from 4q31 to 4qter. Here, we describe clinical phenotypes of two unrelated children of the same age followed at the same time, with case 1 presenting with 4q interstitial and case 2 with terminal 4q deletion, and compare them each other and with those reported in the literature. Both children showed complex, heterogeneous clinical manifestations, including craniofacial features, pre-postnatal growth failure, speech and developmental delay. In case 2, thyroid and cholesterol dysfunction were also found. Analyzing these data, clinical differences between interstitial and terminal 4q deletions are scanty and no significant phenotype differences were found between the 4q regions deleted as observed in the comparison of the two children and the related cases of the literature. The term 4q deletion syndrome - inclusive for both the interstitial and terminal 4q regions deleted - seems to be appropriate. To note, the dysfunction of cholesterol metabolism and thyroid presented by case 2 may be clinically worthwhile, whether confirmed by other observations. [ABSTRACT FROM AUTHOR]

Objectives: We compared the number of accesses, causes, and instrumental evaluations of chest pain in children between the pre-COVID-19 era and the COVID-19 period and analyzed the assessment performed in children with chest pain, highlighting unnecessary examinations. Methods: We enrolled children with chest pain admitted to our emergency department between January 2019 and May 2021. We collected demographic and clinical characteristics and findings on physical examinations, laboratory tests, and diagnostic evaluations. Then, we compared the number of accesses, causes, and instrumental assessments of chest pain between the pre-COVID-19 era and the COVID-19 era. Results: A total of 111 patients enrolled (mean age: 119.8 ± 40.48 months; 62 males). The most frequent cause of chest pain was idiopathic (58.55%); we showed a cardiac origin in 4.5% of the cases. Troponin determination was performed in 107 patients, and the value was high only in one case; chest X-rays in 55 cases and echocardiograms in 25 cases showed pathological findings, respectively, in 10 and 5 cases. Chest pain accesses increased during the COVID-19 era (p < 0.0001), with no differences in the causes of chest pain between the two periods. Conclusions: The increase in accesses for chest pain during the COVID-19 pandemic confirms that this symptom generates anxiety among parents. Furthermore, our findings demonstrate that the evaluation of chest pain is still extensive, and new chest pain assessment protocols in the pediatric age group are needed. [ABSTRACT FROM AUTHOR]

Objective: Our aim was to evaluate the safety of COVID-19 vaccine in children resident in the Latina Local Health Authority. Methods: We conducted a telephone survey among children aged 5–11 years receiving BNT162b2 mRNA COVID-19 vaccine between December 15 and 21. The main outcomes included the presence of allergic reactions or anaphylaxis, adverse events after 24–48 h, 7 and 20 days of taking the first and second doses of medications, and documented SARS-CoV-2 infection after vaccination. The information obtained was automatically linked to a spreadsheet and analyzed. Results: 569 children were enrolled. The mean age was 114 ± 4.24 months; there were 251 males in the study. The vaccine showed a favorable safety profile; no anaphylaxis or serious adverse events were reported. The most common symptoms both after the first and second dose were injection site reactions, asthenia, and headache. Injection site reactions were more frequent after the first dose (p = 0.01), while systemic symptoms were more frequent after the second dose (p = 0.022). These symptoms were more frequent in patients with comorbidities (p = 0.0159). Conclusion: Our findings confirm the safety of COVID-19 vaccine in children younger 11 years and could be useful to promote its diffusion in pediatric ages in order to achieve "herd immunity" and prevent the virus's circulation. [ABSTRACT FROM AUTHOR]

Objective: To evaluate whether the use of a surgical and N95 mask for overweight and obese children was associated with respiratory distress. Methods: We enrolled 15 healthy and 14 overweight or obese children. We performed two sessions: one wearing a surgical, the other an N95 mask. We tracked changes in partial pressure of end-tidal carbon dioxide (PETCO2), oxygen saturation (SaO2), pulse rate (PR), and respiratory rate (RR) during a 72 min test: 30 min without a mask, 30 min wearing a mask, and then during a 12 min walking test. Results: In healthy children, there was no significant change in SaO2 and PETCO2 during the study; there was a significant increase in PR and RR after the walking test with both the masks. In overweight or obese children, there was no significant change in SaO2 during the study period; there was a significant increase in PETCO2 as fast as wearing the mask and an increase in PETCO2, PR, and RR after walking test. After the walking test, we showed a significant correlation between PETCO2 and body mass index. Conclusion: Overweight or Obese children who wear a mask are more prone to developing respiratory distress, which causes them to remove it frequently. In a crowded environment, they are at greater risk of infection. For this reason, it is desirable that they attend environments where everyone uses a mask. [ABSTRACT FROM AUTHOR]

At present, the vaccine authorized in children aged 5 years and older is the BNT162b2 messenger RNA COVID-19 vaccine. Unlike adults, there is limited data available in the pediatric age describing adverse events after vaccine. We report a case of adenomesenteritis in a young girl following the first dose of vaccine. [ABSTRACT FROM AUTHOR]