BACKGROUND: The imaging manifestations of oral and maxillofacial myofibroma/myofibromatosis can vary among patients. Although many clinical cases have been reported, a consensus on the clinicopathological features of and treatment principles for this disease is lacking.
OBJECTIVE: This study aimed to summarize the clinicopathological features of solitary myofibroma of the oral and maxillofacial regions in pediatric patients.
METHODS: The clinical data, histological features, and immunohistochemical characteristics of ten pediatric patients who underwent surgical removal and subsequent pathological diagnosis of myofibroma were collected and retrospectively and cross-sectionally analyzed.
RESULTS: Seven patients were male, and 3 were female, with ages ranging from 3 months to 6 years (mean: 2.6 years). The patients presented with solitary lesions involving the mandibular gingiva and adjacent mandible (4 patients), mandible (2 patients), oral floor and submandibular area and adjacent mandible (1 patient), gingiva (1 patient), maxilla (1 patient), and oropharynx (1 patient). Light microscopy revealed spindle-shaped tumor cells organized in bundles or vortex patterns, forming a hemangiopericytoma-like perivascular pattern, whereas immunohistochemical staining revealed diffuse smooth muscle actin (SMA) positivity. All patients underwent surgical resection, and none experienced recurrence over the 12- to 82-month follow-up.
CONCLUSIONS: Solitary myofibroma in the oral and maxillofacial regions is predominantly observed in infants and young children, with a higher incidence among males. The prognosis is favorable following localized lesion resection or curettage of jawbone lesions. Accurate recognition of the clinical, radiological, and pathological features of the disease will reduce the misdiagnosis rate.

UNASSIGNED: Postthrombotic syndrome (PTS) is one of the long-term sequelae of deep vein thrombosis (DVT), and effective symptom management in pediatric PTS remains a challenge, with interventional therapy rarely explored in this population. We present a successful case of interventional treatment pediatric PTS, resulting in a remarkable amelioration of her symptoms.
UNASSIGNED: This case features a 6-year-old girl diagnosed with hyperinsulinemia, leading to a hypoglycemic coma. Following a mini-pancreatic partial pancreatectomy, she required further intensive care in the pediatric intensive care unit. It was during this period that left lower extremity DVT was identified, prompting warfarin anticoagulation therapy. During the anticoagulation period, she had several bleeding events and was switched to anticoagulation with low molecular heparin. One month later, the left common iliac vein and external iliac vein was found to be completely occluded. Over time, she experienced a gradual onset of lower limb swelling and pain, which, after 6 months, was accompanied by perineal edema and venous claudication. As a result, she underwent successful percutaneous transluminal angioplasty. In addition, the anticoagulation regimen was adjusted to rivaroxaban. At the 8-month follow-up, we observed significantly improvement in her postoperative lower extremity swelling and symptoms related to venous occlusion had completely disappeared. Moreover, vascular imaging confirmed improvement in stenosis and uninterrupted blood flow.
UNASSIGNED: In our review of pediatric PTS studies, we observed limited options to alleviate symptoms, and interventional treatments have not been reported. Our case study, demonstrating the safe and effective use of percutaneous transluminal angioplasty, helps to illuminate this area and alleviate pediatric PTS symptoms.
CONCLUSIONS: This case validates the efficacy and safety of using percutaneous transluminal angioplasty (PTA) for the treatment of postthrombotic syndrome (PTS) in pediatric patients. This interventional approach offers significant symptomatic relief and improves quality of life, especially in cases where traditional anticoagulation therapies fail or lead to complications. The successful case presented emphasizes the necessity of considering endovascular interventions for children with moderate to severe PTS, particularly when conservative management is ineffective. This research underscores the potential for PTA to be adopted in clinical practice, offering a promising new approach for managing pediatric PTS.

Electrical impedance tomography (EIT) is a new method of monitoring non-invasive mechanical ventilation, at the bedside and useful in critically ill patients. It allows lung monitoring of ventilation and perfusion, obtaining images that provide information on lung function. It is based on the physical principle of impedanciometry or the body\'s ability to conduct an electrical current. Various studies have shown its usefulness both in adults and in pediatrics in respiratory distress syndrome, pneumonia and atelectasis in addition to pulmonary thromboembolism and pulmonary hypertension by also providing information on pulmonary perfusion, and may be very useful in perioperative medicine; especially in pediatrics avoiding repetitive imaging tests with ionizing radiation.

Granular cell tumor (GCT) is an uncommon benign neoplasm derived from Schwann cells, frequently affecting the oral cavity, skin, and gastrointestinal tract. On microscopy, pseudocarcinomatous squamous hyperplasia (PSH) and perineural involvement are potential diagnostic pitfalls. GCT should be differentiated from non-neural GCT (NN-GCT). A 13-year-old male patient was referred presenting a nodular lesion on the upper lip several months ago. After excisional biopsy, microscopy revealed GCT without PSH but presenting multifocal perineural involvement. By immunohistochemistry, ALK was negative, whereas Rb and INI1 expression was intact. Moreover, with few intraoral NN-GCTs being assessed, recent studies suggest that acquired dermal NN-GCT subgroup seems to correspond to ALK-rearranged variants of epithelioid fibrous histiocytoma. Accordingly, further research on this topic is strongly encouraged.

Medication errors during drug manipulations in pediatric care pose significant challenges to patient safety and optimal medication management. Epidemiological studies have revealed a high prevalenceof medication errors throughout the medication process. Due to the lack of age-appropriate dosage forms, medication manipulation is common in pediatric drug administration. The consequences of these manipulations on drug efficacy and safety could be devastating, highlighting the need for evidence-based guidelines and standardized compounding practices.
This review focuses on examining medication errors in pediatric care and delving into the manipulation of medicinal products.
The observed prevalence of medication errors and manipulations underscores the importance of addressing these issues to enhance patient safety and improve medication outcomes in pediatric care. Overall, the development of age-appropriate formulations and the dissemination of comprehensive clinical guidelines are essential steps toward improving medication safety and minimizing manipulations in pediatric healthcare settings.

The developing central nervous system is vulnerable to several stimuli, especially psychotropic drugs. Sedation procedures during the developmental period are frequent in pediatric intensive care units (PICUs), in which the use of the sedative agent is still a challenge for the PICU team. Ketamine has been indicated for sedation in critically ill children with hemodynamic and ventilatory instabilities, but the possible neurobehavioral consequences related to this use are still uncertain. Here, we performed a bibliometric analysis with conventional metrics and a critical review of clinical findings to reveal a gap in the literature that deserves further investigation. We revealed that only 56 articles corresponded to the inclusion criteria of the study. The United States of America emerges as the main country within the scope of this review. In addition, professional clinical societies play a key role in the publications of scientific clinical findings through the specialist journals, which encourages the sharing of research work. The co-occurrence of keywords evidenced that the terms \"sedation\", \"ketamine\", and \"pediatric\" were the most frequent. Case series and review articles were the most prevalent study design. In the critical evaluation, the scarce studies highlight the need of use and post-use monitoring, which reinforces the importance of additional robust clinical studies to characterize the possible adverse effects resulting from ketamine anesthetic protocol in critically ill children.

BACKGROUND: Brain arteriovenous malformations (AVMs) are increasingly being treated with Onyx liquid embolic agent (Onyx, Medtronic, Inc.). The phenomenon of delayed Onyx migration is not well documented in the literature. Moreover, the clinical presentation associated with Onyx migration is not well understood.
METHODS: A pediatric patient with a history of neonatal seizures was referred to our institution upon experiencing daily headaches with photophobia, phonophobia, and sleep disturbance. Cerebral angiography revealed an AVM of the medial left cerebellar hemisphere. Preoperative embolization with Onyx liquid embolic achieved 25% closure of the AVM nidus. Upon developing worsening headaches the following day, new perinidal parenchymal edema was revealed on MRI, and urgent angiography demonstrated delayed migration of Onyx into the venous drainage. The patient underwent emergency resection of the AVM due to the risk of hemorrhage resulting from venous outflow obstruction.
CONCLUSIONS: Our report and literature review demonstrate that while the delayed, unexpected migration of Onyx embolic material has been alluded to in a handful of papers, this phenomenon is not well documented. Future research is needed to understand the frequency of delayed Onyx migration from brain AVMs and the possible clinical presentations to look for. The sudden development of headaches and other signs of perilesional edema, in particular, should prompt repeat angiographic examination due to the possibility of delayed liquid embolic migration.

UNASSIGNED: Osteocalcin plays a role in glucose metabolism in mice, but its relevance in human energetic metabolism is controversial. Its relationship with markers of energetic metabolism in the pediatric population has not been systematically addressed in infants and adolescents.
UNASSIGNED: This study aims to assess the mean differences between tOC, ucOC, and cOC among healthy children and children with type 1 or type 2 diabetes (T1D or T2D) and the correlation of these bone molecules with metabolic markers.
UNASSIGNED: A systematic review and metanalysis were performed following PRISMA criteria to identify relevant observational studies published in English and Spanish using PubMed, Scopus, EBSCO, and Web of Science databases. The risk of bias was assessed using New Castle-Ottawa scale. Effect size measures comprised standardized mean difference (SMD) and Pearson correlations. Heterogeneity and meta-regressions were performed.
UNASSIGNED: The 20 studies included were of high quality and comprised 3,000 pediatric patients who underwent tOC, cOC, or ucOC measurements. Among healthy subjects, there was a positive correlation of ucOC with WC and weight, a positive correlation of tOC with FPG, HDL-c, WC, height, and weight, and a negative correlation between tOC and HbA1c. Among diabetic subjects, a negative correlation of ucOC with HbA1c and glycemia in both T1D and T2D was found and a negative correlation between tOC and HbA1c in T1D but not in T2D. The ucOC concentrations were lower in T2D, T1D, and patients with abnormal glucose status than among controls. The serum concentrations of tOC concentrations were lower among T1D than in controls. The patient\'s age, altitude, and HbA1c influenced the levels of serum tOC.
UNASSIGNED: Osteocalcin is involved in energy metabolism in pediatric subjects because it is consistently related to metabolic and anthropometric parameters.
UNASSIGNED: https://www.crd.york.ac.uk/prospero/, identifier: CRD42019138283.

Lichen sclerosus (LS) is defined as a chronic mucocutaneous inflammatory disease with a localization predominantly to the anus and genitals (vulvar sclerosus (VLS)). Pediatric lichen sclerosus (LS) is a chronic inflammatory skin condition with predilection for the anogenital area that if untreated can lead to scarring. Vulvar LS is characterized by two peaks in incidence: it occurs in prepubertal girls and in postmenopausal women. To date, several mechanisms and risk factors have been proposed in the pathogenesis of pediatric vulvar LS; however, the etiology of this condition is still not fully understood and constitutes a challenge for scientists and clinicians. The presented research aimed to systematically review the existing literature on the pathogenesis of pediatric LS and to identify possible underlying autoimmune mechanisms and molecular networks. The clinical presentation of pediatric lichen sclerosus and available treatment modalities are also presented to acquaint a broader audience with this underdiagnosed and undertreated condition. As a result of our review, we discuss several potential mechanisms, molecules, and pathways that have been recognized in this disease. The purpose of our review was also to summarize what we can induce in further studies, which will ultimately help to identify the mechanism responsible for the disease and aid in the development of new, more effective treatment strategies for diagnosis and treatment by clinicians and researchers.

BACKGROUND: Sjogren\'s syndrome (SS) is a rare chronic autoimmune disease involving exocrine glands presenting with sicca syndrome, recurrent parotitis and other extraglandular stigmata. SS is well characterized in the adult population with classification criteria; however, primary SS presenting in childhood is poorly defined and rare in males. Recurrent parotitis is the most common presenting symptom in children with primary SS; however, clinical phenotype in children appears more variable than in adults. The lungs are a common extraglandular location for manifestations of primary SS. However, interstitial lung disease (ILD) is rare in children with primary SS. There are only four published reports of ILD associated with primary SS in female children. Here, we present a very rare case of primary SS in a pediatric male with pulmonary manifestations and review of the literature on ILD in childhood-onset primary SS.
METHODS: A 14-year-old White male with a history of chronic severe asthma, recurrent parotitis and idiopathic intracranial hypertension was referred to pediatric rheumatology for evaluation of a positive ANA. In early childhood, he was diagnosed with persistent asthma recalcitrant to therapy. At age 8, he developed recurrent episodes of bilateral parotitis despite multiple treatments with sialoendoscopy. At age 14, respiratory symptoms significantly worsened prompting reevaluation. Lab workup was notable for positive ANA and Sjogren\'s Syndrome A and B antibodies. Pulmonary function tests showed only a mild obstructive process. Computed tomography of chest was significant for small airway disease, and lung biopsy was positive for mild interstitial lymphocytic inflammation presenting a conflicting picture for ILD. The constellation of findings led to the diagnosis of primary SS with associated pulmonary manifestations. He was treated with hydroxychloroquine, mycophenolate mofetil and oral corticosteroids with resolution of symptoms.
CONCLUSIONS: Primary SS is a rare disease in the pediatric population that is poorly characterized. This case is the very rare presentation of childhood-onset primary SS with pulmonary manifestations in a male patient. ILD associated with primary SS is also very rare with only four pediatric patients reported in the literature. Collaborative effort is needed to develop pediatric specific diagnostic and treatment guidelines in this rare condition.