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Background/Objectives: Risperidone-related metabolic side effects in children have been primarily linked to variations between guideline-recommended and clinical treatment procedures. We explored the metabolic effects of risperidone administration in pediatric patients diagnosed with neurological disorders, thus evaluating its influence on metabolic indicators. Methods: This prospective cohort study gathered data from electronic health records, medical databases, and clinical reports. These data included patient demographics (e.g., age, sex, and body mass index) and information on risperidone use, including dosage, dosing frequency, and treatment duration. Additionally, laboratory tests were conducted at baseline and during treatment, along with other pertinent clinical variables. Result: A total of 52 eligible children (male; 73.1%) with neurological disorders treated with risperidone were included. The mean age was 13.4 ± 2.2 years. Risperidone was administered to 32.7% of patients for <2 years, 40.4% for 2–5 years, and 26.9% for 6–9 years, with a mean duration of 3.6 years. Most (53.8%) of the children experienced at least one metabolic side effect, with hyperlipidemia being the most common (34.6%). The median prolactin level increased slightly from 448.5 ng/mL at baseline to 479 ng/mL after 6–8 weeks. No significant associations were found between age, sex, duration of treatment, dosage form, dosing frequency, and hemoglobin A1c levels. Conclusion: Monitoring metabolic and anthropometric parameters in children receiving risperidone for neurological disorders is cardinal. Clinicians should consider individualized treatment plans, closely monitor metabolic markers, and address potential risks associated with long-term risperidone use in this vulnerable population. [ABSTRACT FROM AUTHOR]

Background: Percutaneous CT-guided cryoablation is an emerging technique for treating bone tumors. However, experience with using this procedure for osteoid osteomas in pediatric patients remains limited. Our study aims to assess its technical feasibility, clinical efficacy, and safety in children treated under conscious anesthesia. Methods: We conducted a retrospective study of consecutive pediatric patients who underwent CT-guided percutaneous cryoablation for osteoid osteomas at our institution between September 2017 and March 2021. All patients received conscious anesthesia. Data on peri-procedural VAS scores, post-procedural VAS scores, imaging findings, and nonsteroidal anti-inflammatory drug (NSAID) usage rates were collected for each patient. Technical success was defined as proper cryoprobe placement at the nidus center, while clinical success referred to pain relief without NSAID use. Intra- and post-operative complications were also evaluated. Results: Nine patients underwent CT-guided percutaneous cryoablation for osteoid osteomas under conscious sedation, with a 100% overall success rate with low peri-procedural and median VAS scores (p < 0.01). No complications were observed during or after the procedure. Conclusions: CT-guided percutaneous cryoablation of pediatric osteoid osteomas is an effective and safe minimally invasive procedure feasible under conscious anesthesia, holding promise as a valuable treatment option. [ABSTRACT FROM AUTHOR]

Atopic dermatitis (AD) is a chronic inflammatory skin disease affecting up to 20% of children. Methotrexate (MTX) is used off-label as a systemic treatment for AD patients unresponsive to topical therapies, but limited data exist regarding its safety and efficacy in children, especially in those < 4 years old. To further investigate MTX in younger patients, we screened the medical records of three referral centers between 2016 and 2022 and identified 28 infants and toddlers < 4 years old with AD treated with MTX. Mean age upon MTX initiation was 2.7 ± 1.2 years and mean investigator global assessment (IGA) score was 3.78 ± 0.4. Median duration of MTX treatment was five months. Following 12 and 24 weeks of MTX treatment, the response rate was 50% and IGA 0/1 was achieved in 14.2% and 21.4% of patients, respectively. Most treatment cessations were attributed to a lack of efficacy or parental concern. Although adverse events were reported in 57.1% of patients, MTX was discontinued due to such adverse events only in two patients (7.1%). Taken together, MTX demonstrated a high safety profile in AD patients <4 years old. MTX efficacy was moderate and presumably underestimated by parents who opted for premature treatment cessation due to concerns associated with an immunomodulatory drug. [ABSTRACT FROM AUTHOR]

Children who sustain major injuries are at risk of receiving insufficient pain relief and sedation, which can have physical and psychological repercussions. Heightened emotional distress can increase the likelihood of developing symptoms of post-traumatic stress. Providing sufficient analgesia and sedation for children with major trauma presents specific challenges, given the potential for drug-related adverse events, particularly in non-intubated patients. The current literature suggests that a relatively low percentage of pediatric patients receive adequate analgesia in pre-hospital and emergency department settings following major trauma. There are only sparse data on the safety of the provision of analgesia and sedation in children with major trauma in the pre-hospital and ED settings. The few studies that examined sedation protocols in this context highlight the importance of physician training and competency in managing pediatric airways. There is a pressing need for prospective studies that focus upon pediatric major trauma in the pre-hospital and emergency department setting to evaluate the benefits and risks of administering analgesia and sedation to these patients. The aim of this narrative review was to offer an updated overview of analgesia and sedation management in children with major trauma in pre-hospital and ED settings. [ABSTRACT FROM AUTHOR]

(1) Background: Extravasation injuries in pediatric patients can lead to significant harm if they are not promptly diagnosed and treated. However, evidence-based standardization on extravasation management remains limited, particularly for extensive wound necrosis. This case report presents the management of an 8-week-old premature patient with an extensive extravasation injury involving the right forearm and dorsum of the hand. (2) Methods: The patient was evaluated by a multidisciplinary team in our Neonatal Intensive Care Unit. Surgical intervention involved the debridement of necrotic tissues, followed by temporary coverage with an acellular dermal matrix. Definitive coverage was achieved through Meek micrografting after three weeks. Physical therapy was provided with pre- and post-rehabilitation range of motion assessed using goniometric measurements. Scar quality was evaluated using the Vancouver Scar Scale. (3) Results: The engraftment rate of the Meek micrografts was 93%, with 16 out of 226 micrografts lost. The patient achieved a Vancouver Scar Scale score of 6, indicating a moderate degree of scarring. Significant improvements in elbow, wrist, and finger joint range of motion were observed at a 1-year follow-up. (4) Conclusions: Close observation and heightened awareness of extravasation risks by trained personnel are crucial. Meek micrografting combined with dermal substitute coverage represents an innovative approach to managing extravasation wounds in pediatric patients. [ABSTRACT FROM AUTHOR]

B-cell precursor acute lyphoblastic leukemia (ALL) is a common pediatric malignancy and patients may have significant benefits from monoclonal antibodies therapy with increased survival rates. Positive CD20 expression is identified in about half of these patients and its presence may serve as a prognostic factor in disease evolution. We performed a retrospective study including 114 patients diagnosed with B-ALL and evaluated the expression of CD20 through flow cytometry at diagnosis and on day 15. Additional immunophenotypic analyses as well as cytogenetic and molecular genetic analyses were also performed. We observed an increase in the mean fluorescence intensity (MFI) of CD20 between diagnosis—1.9 (1.2–3.26) and day 15: 6.17 (2.14–27.4), (p < 0.0001). Furthermore, we assessed that both diagnosis and day 15 CD20 MFI had an impact on RFS and OS, respectively, for cut-off values of >8.08 at diagnosis and >28.65 at day 15. In conclusion, CD20 expression appears to be a poor prognostic feature of B-ALL in pediatric patients. In this study, stratification of the outcome by the intensity of CD20 has implications concerning the allocation to rituximab-based chemotherapy and may offer new, potentially useful information for pediatric patients with B-ALL. [ABSTRACT FROM AUTHOR]

Objective: Robot-assisted laparoscopic ureteral reimplantation (RALUR) and trans-umbilical multiport laparoscopic ureteral reimplantation (TMLUR) are both minimally invasive procedures for benign distal ureteral stricture (DUS). However, TMLUR has rarely been reported in published research, thus the difference in mid-term outcome of these two procedures warrants investigation. Methods: Patients who underwent RALUR or TMLUR for pediatric DUS from April 2017 to November 2020 at our institution were retrospectively analyzed and 56 patients were included in this retrospective comparison. Demographic characteristics, perioperative data and follow-up results were collected and analyzed in RALUR and TALUR groups. Results: RALUR and TMLUR were successfully performed in children aged from 12.0 to 142.0 months, without conversion to open ureteral reimplantation. RALUR took shorter operative time than TMLUR (p = 0.005) with less blood loss (p = 0.001). Meanwhile, patients receiving RALUR encountered a greater financial burden (p < 0.001) with less cosmetic satisfaction than TMLUR. The mean mid-term follow-up time for RALUR and TMLUR was 18.29 months and 24.64 months, respectively. Mid-term follow-up data showed that DUS was relieved with improved renal function after surgery in both groups, with no significant difference. Conclusions: RALUR and TMLUR are both safe and efficient for DUS treatment and achieve comparable mid-term outcomes in children. RALUR can reduce operative time and operative blood loss benefiting from its prominent technical superiority, but may currently bring about greater financial burden, with cosmetic satisfaction remaining to be improved. [ABSTRACT FROM AUTHOR]

Chemotherapy, pelvic radiotherapy and ovarian surgery have known gonadotoxic effects that can lead to endocrine dysfunction, cessation of ovarian endocrine activity and early depletion of the ovarian reserve, causing a risk for future fertility problems, even in children. Important determinants of this risk are the patient's age and ovarian reserve, type of treatment and dose. When the risk of premature ovarian insufficiency is high, fertility preservation strategies must be offered to the patient. Furthermore, fertility preservation may sometimes be needed in conditions other than cancer, such as in non-malignant diseases or in patients seeking fertility preservation for personal reasons. Oocyte and/or embryo vitrification and ovarian tissue cryopreservation are the two methods currently endorsed by the American Society for Reproductive Medicine, yielding encouraging results in terms of pregnancy and live birth rates. The choice of one technique above the other depends mostly on the age and pubertal status of the patient, and personal and medical circumstances. This review focuses on the available fertility preservation techniques, their appropriateness according to patient age and their efficacy in terms of pregnancy and live birth rates. [ABSTRACT FROM AUTHOR]

Background: Total body irradiation (TBI) is a mandatory step for patients with acute lymphoblastic leukemia (ALL), undergoing allogeneic hematopoietic stem cell transplantation (HSCT). In the past, amylases have been reported to be a possible sign of TBI toxicity. We investigated the relationship between total amylases (TA) and transplant-related outcomes in pediatric recipients. Methods: We retrospectively analyzed the medical records of all the patients who underwent allogeneic HSCT between January 2000 and November 2019. The inclusion criteria were the following: recipient's age between 2 and 18, diagnosis of ALL, no previous transplantation, and use of TBI-based conditioning. The serum total amylase and pancreatic amylase were evaluated before, during, and after transplantation. Cytokines and chemokines assays were retrospectively performed. Results: 78 patients fulfilled the inclusion criteria. Fifty-seven patients were treated with fractionated TBI, and 21 with a single-dose regimen. The overall survival (OS) was 62.8%. Elevated values of TA were detected in 71 patients (91%). The TA were excellent in predicting the OS (AUC = 0.773; 95% CI = 0.66–0.86; p < 0.001). TA values below 374 U/L were correlated with a higher OS. The highest mean TA values (673 U/L) were associated with a high disease-progression mortality rate. The TA showed a high predictive performance for disease progression-related death (AUC = 0.865; 95% CI = 0.77–0.93; p < 0.0001). Elevated TA values were also connected with significantly higher levels of proinflammatory cytokines, such as TNF-α, IL-6, and RANTES (p < 0.001). Conclusions: this study shows that TA is a valuable predictor of post-transplant OS and increased risk of leukemia relapse. [ABSTRACT FROM AUTHOR]

Cardiac troponin I (cTnI) elevation is common in an acute episode of supraventricular tachycardia (SVT). However, there is limited evidence regarding the prognostic value of cTnI and the predictors of SVT recurrence in pediatric patients. We screened the electronic medical records of all pediatric patients presenting to the emergency departments at five Taiwanese hospitals from 1 January 2010 to 31 May 2021. Our primary outcomes were the occurrence of major adverse cardiac events (MACEs) during the follow-up period and 30-day SVT recurrence. A total of 112 patients were included in our study. Of these, 29 (25.9%) patients had positive cTnI values. Patients with cTnI elevation had significantly more complaints of dyspnea (27.6% vs. 7.2%, p = 0.008) and gastrointestinal discomfort (24.1% vs. 4.8%, p = 0.006). There were significantly more intensive care unit admissions (41.4% vs. 16.9%, p = 0.007) among the cTnI-positive group. One MACE was found in the cTnI-negative group. For 30-day SVT recurrence, the cTnI-positive group had a higher recurrence rate, without a statistically significant difference (20.7% vs. 7.2%, p = 0.075). Multivariable logistic regression analysis showed hypotension as an independent predictor of 30-day SVT recurrence (OR = 4.98; Cl 1.02–24.22; p = 0.047). Troponin had low value for predicting the outcomes of pediatric patients with SVT. The only significant predictor for recurrent SVT was initial hypotension. [ABSTRACT FROM AUTHOR]

Venous access devices (VADs) play an important role in different clinical contexts. In pediatric subjects, VAD placement is more complicated than in adults due to children's poor cooperativity and reduced vascular access. Adherence to guidelines for the placement of VADs could prevent the occurrence of complications, but data in the literature are general and not exhaustive, especially with regard to the pediatric population. The objective of this study was to assess adherence to guidelines for the placement of VADs in a pediatric setting. A retrospective observational study was conducted in the general ward of a pediatric hospital in the northern region of Italy. Data related to consecutive admissions in the period from 1 January to 31 December 2019 were collected according to the availability of clinical documentation. A cohort of 251 subjects was considered, yielding a total of 367 VADs. Device permanence in situ and the effective administration of intravenous therapy were associated with an increased risk of complications, while adherence to guidelines was an important protective factor. Adherence to guidelines for the placement of VADs is an independent and positive predictive factor for the prevention of complications due to the presence of a vascular device. [ABSTRACT FROM AUTHOR]

Acute graft-versus-host disease (GvHD) remains the second leading cause of death, after disease relapse, in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). The medical records of 112 pediatric patients who underwent allo-HSCT from matched unrelated and haploidentical donors were analyzed. Patients were divided into two groups, according to the GvHD prophylactic regimen used. In the control group, GvHD prophylaxis consisted of cyclosporine A (CsA) and methotrexate (MTX) or CsA and mycophenolate mofetil (MMF) at a standard daily dose of 30 mg/kg. All subjects in the study group received tacrolimus (FK506) and MMF. In this group, MMF was subjected to therapeutic drug monitoring (TDM) through mycophenolic acid (MPA) area under the curve AUC0–12. We found a statistically significant difference in both overall acute GvHD (p < 0.0001) and overall chronic GvHD (p < 0.05) incidence between the study and the control group. The initial daily MMF dose and the age at transplant in the study group proved to be inversely correlated (r = −0.523, p < 0.0001). The children under six years of age required a significantly higher daily MMF dose (p < 0.008). This study showed that pharmacological monitoring of MPA AUC0–12 concentration allowed a reduction in the incidence of acute and chronic GvHD. MMF showed age-dependent pharmacokinetics due to greater drug clearance in younger children. [ABSTRACT FROM AUTHOR]

Background: The aims of this study were to compare salivary cytokines and total protein between children with nephrotic syndrome (NS) and healthy children, and to examine whether saliva parameters can differentiate between steroid sensitivity and resistance and between disease remission and relapse. Methods: Twenty-seven children with nephrotic syndrome were classified according to steroid sensitivity and resistance, and disease remission and relapse. Twenty healthy children served as controls. Whole saliva samples were collected from all the participants. Urine and blood tests done on the same day as the saliva collection were recorded. Salivary total protein was quantified using bicinchoninic acid and IFNγ, IL-4, IL-8, IL-6, and IL1β levels using ELISA. Results: The mean ages of the nephrotic syndrome and control groups were 11.3 ± 2.4 and 9 ± 4.2, respectively. Compared to the control group, for the nephrotic syndrome group, total salivary protein was significantly lower, as were the levels of all the cytokines examined except IFNγ. Statistically significant differences were not found in any of the salivary markers examined between the children with nephrotic syndrome who were treatment sensitive (n = 19) and resistant (n = 8). Protein and IL-8 salivary levels were lower in the active (n = 7) than in the remission (n = 20) group. Conclusions: Salivary parameters distinguished children with nephrotic syndrome in relapse from healthy children. This may be due to decreased salivary protein excretion, which reflects decreased plasma levels, consequent to proteinuria. Accordingly, salivary markers may be developed as a diagnostic or screening tool for NS activity. [ABSTRACT FROM AUTHOR]

The 12-month mortality rate in patients undergoing hematopoietic stem cell transplantation (HSCT) remains high, especially with respect to transplant-related mortality (TRM), which includes mortality due to infection complications through the aplasia phase. The aim of this study was to determine whether the administration of Pentaglobin® could decrease TRM by lowering sepsis onset or weakening sepsis through the aplasia phase. One hundred and ninety-nine pediatric patients who had undergone HSCT were enrolled in our retrospective study. The patients were divided into two groups: the Pentaglobin group, which had received Pentaglobin® in addition to the standard antibiotic treatment protocol established for the aplasia phase, and the Control group, which received only the standard treatment. As compared to the control group outcome, Pentaglobin® led to a significant decrease in the days of temperature increase (p < 0.001) and a reduced infection-related mortality rate (p = 0.04). In addition, the number of antibiotics used to control infections, and the number of antibiotic therapy changes needed following first-line drug failure, were significantly lowered in the Pentaglobin group as compared to the control group (p < 0.0001). With respect to the onset of new infections following the primary infection detected, the Pentaglobin group showed a significant reduction for bacterial events, as compared to the control group (p < 0.03). Pentaglobin® use in patients undergoing HSCT seems to produce a significant decrease in infection-associated TRM rate. [ABSTRACT FROM AUTHOR]

The 12-month mortality rate in patients undergoing hematopoietic stem cell transplantation (HSCT) remains high, especially with respect to transplant-related mortality (TRM), which includes mortality due to infection complications through the aplasia phase. The aim of this study was to determine whether the administration of Pentaglobin ® could decrease TRM by lowering sepsis onset or weakening sepsis through the aplasia phase. One hundred and ninety-nine pediatric patients who had undergone HSCT were enrolled in our retrospective study. The patients were divided into two groups: the Pentaglobin group, which had received Pentaglobin ® in addition to the standard antibiotic treatment protocol established for the aplasia phase, and the Control group, which received only the standard treatment. As compared to the control group outcome, Pentaglobin ® led to a significant decrease in the days of temperature increase ( p < 0.001) and a reduced infection-related mortality rate ( p = 0.04). In addition, the number of antibiotics used to control infections, and the number of antibiotic therapy changes needed following first-line drug failure, were significantly lowered in the Pentaglobin group as compared to the control group ( p < 0.0001). With respect to the onset of new infections following the primary infection detected, the Pentaglobin group showed a significant reduction for bacterial events, as compared to the control group ( p < 0.03). Pentaglobin ® use in patients undergoing HSCT seems to produce a significant decrease in infection-associated TRM rate.

Supraglottic airway devices have been increasingly used because of their several advantages. Previous studies showed that the small-sized i-gel provides effective ventilation for young pediatric patients; however, few studies have reported the use of AuraGain in these patients. Herein, we compared the clinical performance of AuraGain and i-gel in young pediatric patients aged between 6 months and 6 years old and weighing 5–20 kg, who were scheduled to undergo extremity surgery under general anesthesia. In total, 68 patients were enrolled and randomly allocated into two groups: AuraGain group and i-gel group. The primary outcome was the requirement of additional airway maneuvers. We also analyzed insertion parameters, fiberoptic bronchoscopic view, oropharyngeal leak pressure, and peri-operative adverse effects. Compared with the AuraGain group, the i-gel group required more additional airway maneuvers during the placement of the device and maintenance of ventilation. The fiberoptic view was better in the AuraGain group than in the i-gel group. However, the oropharyngeal leak pressure was higher in the i-gel group. AuraGain might be a better choice over i-gel considering the requirement of additional airway maneuvers. However, when a higher oropharyngeal leak pressure is required, the i-gel is more beneficial than AuraGain. [ABSTRACT FROM AUTHOR]

Mitochondrial diseases (MD) are a group of genetic and acquired disorders which present significant diagnostic challenges. Here we report the disease characteristics of a large cohort of pediatric MD patients (n = 95) with a definitive genetic diagnosis, giving special emphasis on clinical muscle involvement, biochemical and histopathological features. Of the whole cohort, 51 patients harbored mutations in nuclear DNA (nDNA) genes and 44 patients had mutations in mitochondrial DNA (mtDNA) genes. The nDNA patients were more likely to have a reduction in muscle fiber succinate dehydrogenase (SDH) stains and in SDH-positive blood vessels, while a higher frequency of mtDNA patients had ragged red (RRF) and blue fibers. The presence of positive histopathological features was associated with ophthalmoplegia, myopathic facies, weakness and exercise intolerance. In 17 patients younger than two years of age, RRF and blue fibers were observed only in one case, six cases presented cytochrome c oxidase (COX) reduction/COX-fibers, SDH reduction was observed in five and all except one presented SDH-positive blood vessels. In conclusion, muscle involvement was a frequent finding in our series of MD patients, especially in those harboring mutations in mtDNA genes. [ABSTRACT FROM AUTHOR]