Cord Blood Transplantation for Very Early-Onset Inflammatory Bowel Disease Caused by Interleukin-10 Receptor Deficiency.

PURPOSE: Interleukin-10 receptor (IL-10R) deficiency can result in life-threatening very early-onset inflammatory bowel disease (VEO-IBD). Umbilical cord blood transplantation (UCBT) is a curative therapy for patients with IL-10R deficiency. This study aimed to investigate the efficacy of UCBT in treating IL-10R deficiency and develop a predictive model based on pre-transplant factors. METHODS: Eighty patients with IL-10R deficiency who underwent UCBT between July 2015 and April 2023 were retrospectively analyzed. Cox proportional hazards regression and random survival forest were used to develop a predictive model. RESULTS: Median age at transplant was 13.0 months (interquartile range [IQR], 8.8-25.3 months). With a median follow-up time of 29.4 months (IQR, 3.2-57.1 months), the overall survival (OS) rate was 65.0% (95% confidence interval [CI], 55.3%-76.3%). The engraftment rate was 85% (95% CI, 77%-93%). The cumulative incidences of acute and chronic graft-versus-host disease were 48.2% (95% CI, 37.1%-59.4%) and 12.2% (95% CI, 4.7%-19.8%), respectively. VEO-IBD-associated clinical symptoms were resolved in all survivors. The multivariate analysis showed that IL-6 and stool occult blood were independent prognostic risk factors. The multivariate Cox proportional hazards regression model with stool occult blood, length- or height-for-age Z-score, medical history of sepsis, and cord blood total nucleated cells showed good discrimination ability, with a bootstrap concordance index of 0.767-0.775 in predicting OS. CONCLUSION: Better inflammation control before transplantation and higher cord blood total nucleated cell levels can improve patient prognosis. The nomogram can successfully predict OS in patients with IL-10R deficiency undergoing UCBT.

Genotyping of oral Candida albicans and Candida tropicalis strains in patients with orofacial clefts undergoing surgical rehabilitation by MALDI-TOF MS: Case-series study.

Patients with orofacial clefts are more likely to develop oral fungal diseases due to anatomo-physiological changes and surgical rehabilitation treatment. This case-series study evaluated the genetic diversity and dynamics of oral colonization and spread of C. albicans and C. tropicalis in four patients with orofacial clefts, from the time of hospital admission, perioperative and outpatient follow-up, with specialized physician. Candida biotypes previously identified by CHROMagar Candida and PCR methods were studied by MALDI-TOF MS assays and clustering analyses. Possible correlations with pathogenicity characteristics were observed, including production of hydrolytic exoenzymes and the antifungal sensitivity profiles. Amphotericin B-sensitive and fluconazole-resistant (low frequency) C. tropicalis and C. albicans, including clinically compatible MIC of nystatin, were found in the oral cavity of these patients. Clusters of isolates revealed phenomena of (i) elimination in the operative phase, (ii) maintenance or (iii) acquisition of oral C. tropicalis in the perioperative period and specialized outpatient and medical follow-up. For C. albicans, these phenomena included (i) elimination in the operative phase, (ii) acquisition in the operative phase and propagation from the hospital environment, and (iii) maintenance during hospitalization and operative phase. Amphotericin B and nystatin were shown to be effective in cases of clinical treatment and/or prophylaxis, especially considering the pre-existence of fluconazole-resistant strains. This study confirmed the phenomena of septic maintenance, septic neocolonization and septic elimination involving the opportunistic pathogens. MALDI-TOF MS associated with clustering analysis may assist the monitoring of clinical isolates or groups of epidemiologically important microbial strains in the hospital setting.

Percutaneous Transluminal Angioplasty Alleviate Pediatric Postthrombotic Syndrome: A Case Report and Literature Review.

PURPOSE: 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. CASE REPORT: 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. CONCLUSIONS: 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. CLINICAL IMPACT: 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.

Rapid progression of orbital abscess requiring lateral canthotomy in a pediatric patient.

Subperiosteal abscess (SPA) is a rare complication of acute sinusitis in children that may develop rapidly. In this case report, we describe an 11 year-old boy who presented with a large SPA 2 days after being diagnosed with conjunctivitis. The patient required emergent lateral canthotomy and cantholysis (LCC), IV antibiotics, and emergent surgery. It is crucial that emergency physicians be able to identify and treat this vision-threatening complication.

The Impact of COVID-19 on Children and Adolescents with Chronic Illness.

Children and adolescents living with chronic illness may already be at a disadvantage when compared to their healthy peers in achieving developmental milestones as they are required to manage healthcare aspects of their condition in addition to the typical transitions associated with this developmental stage. The COVID-19 pandemic has significantly impacted on the physical and mental well-being of children and adolescents including those young people living with a chronic illness. Disruptions and delays of healthcare service provision, prolonged lockdowns, strict social distancing measures, and school closures are some of the pandemic implications that have affected daily routines imposing strains on young people themselves, but also on their caregivers. This chapter presents a critical elaboration on the available evidence documenting the unique impact of the pandemic at an individual-, family-, and system-level on children and adolescents aged up to 18 years old living with a non-communicable disease (e.g., cancer, diabetes, asthma).

The factors associated with the caregiving burden among family caregivers of pediatric patients with fractures: A descriptive cross-sectional study.

PURPOSE: To investigate the factors linked to the caregiving burden among family caregivers of pediatric patients with fractures. METHODS: This descriptive cross-sectional research used non-probabilistic sampling involving 200 pediatric patients (0-14 years) with fractures and their family caregivers. Data was collected between November 2019 and June 2020. The Chinese version of the Zarit Burden Interview (ZBI) and Simplified Coping Style Questionnaire (SCSQ) assessed caregiving burden and coping styles. We examined clinical data of patients and caregivers, identifying factors influencing caregiving burden by integrating findings from parallel studies, and conducted statistical analyses on these factors. RESULTS: Most family caregivers in this study were women (80.5%), with mothers comprising 56% of the total. The average ZBI score was 40.65 ± 17.27, and 81.5% of caregivers had moderate or severe burden. Factors associated with caregiving burden included caregiver gender, co-caregivers, positive and negative coping scores, and monthly income. The significant factors that were identified accounted for approximately 42.4% of the variability in the caregiving burden. CONCLUSIONS: This study concluded that family caregivers of pediatric patients with fractures often experience moderate to severe burdens, especially female caregivers, those without co-caregivers, using negative coping strategies, and from low-income families. Thus, it is essential to provide these families with professional information, policy support, and affordable, effective care services. IMPLICATIONS TO PRACTICE: Healthcare professionals should prioritize addressing the caregiving burden of family caregivers of pediatric patients with fractures. Nurses can actively improve the health of these pediatric patients and ease parental burden by providing information about social support systems.

Quality-of-Life Assessment in Children with Mild to Moderate Bronchial Asthma.

Background and Objectives: Assess the quality of life of children aged 2-10 with mild to moderate bronchial asthma. To evaluate the general health condition of children with mild and moderate severity bronchial asthma. To determine health changes in children with mild- and moderate-severity bronchial asthma as they grow older. To evaluate the impact of mild- and moderate-severity bronchial asthma on children's daily and social activities, physical health, emotional state, and general well-being. Materials and Methods: A comparative cross-sectional study was conducted in March-June 2020. Parents or guardians of 2-10-year-old children without bronchial asthma and children with mild to moderate bronchial asthma were interviewed after receiving their written informed consent. The questionnaire was based on the standardized quality-of-life quiz SF-36. A total of 248 questionnaires were collected-106 from the parents or guardians of children with bronchial asthma and 142 from parents/guardians of children without bronchial asthma. For further analysis, 106 children without bronchial asthma and with no chronic conditions were selected. Quantitative variables were compared using the Mann-Whitney U test and qualitative data using the chi-square (χ2) criteria. Quantitative data were described by giving means, medians, and standard deviations (SD); qualitative features by giving relative frequencies. Statistical data were analyzed using SPSS and Excel 2020. Results: Children with mild and moderate asthma exhibit poorer health compared to their healthy counterparts. Only 20.7% of respondents with asthma reported excellent or very good health, contrasting with 64.1% of healthy children (p < 0.001). As children with asthma age, their general condition improves, with 46.2% showing improvement in the past year, while 42.5% of healthy children had a stable condition (p < 0.05). In various activities, children with asthma face more constraints than healthy children (p < 0.05), including energetic activities (sick-59.5%; healthy-10.3%), moderate activities (sick-24.5%; healthy-4.7%), climbing stairs (sick-22.7%; healthy-3.8%), and walking over 100 m (sick-9.4%; healthy-0%). Children with asthma are more likely to experience exhaustion, anxiety, tiredness, lack of energy, and restraint in public activities (p < 0.05). Conclusions: Parents/caregivers of children with mild to moderate bronchial asthma rate their health worse than those of healthy children do. As children with mild to moderate bronchial asthma grow, the disease impact on their overall well-being decreases. Children with mild to moderate bronchial asthma, compared to healthy children, experience more limitations in vigorous or moderate activities; face more difficulties climbing stairs or walking more than 100 m; frequently feel exhaustion, anxiety, fatigue, or lack of energy; and encounter restrictions in social activities.

Displaced proximal humeral fractures in skeletally immature patients: functional outcomes of surgical treatment.

PURPOSE: To report the functional results of our experience and to describe intraoperative findings and complications due to the techniques used in our service. METHODS: From January 2018 to December 2022, 27 Pediatric patients aged from 8 to 16 years underwent surgery to treat proximal humerus fractures. Their demographic characteristics were evaluated, as well as their clinical characteristics on admission, type of reduction (closed/open), presence of interposition in open reductions, type of implant, complications, and functional range of movement according to two shoulder functional scores. RESULTS: Mean age was 11.2 years (8-15), there was a predominance of males, who accounted for 70% of the cases, and sports accidents were the cause of injury in 44% of the cases. Anatomical location was balanced between patients, including 55% of physeal fractures and 45% of metaphyseal ones. Overall, 81.5% of patients required open reduction to achieve axis correction, and that 55.5% of fractures were fixed with Kirschner wires, and 44, 4% with elastic titanium nails. Mean QuickDASH score was 0.58 (0-1.7), and Constant score was 9 (3-24). There were no major complications, but 27% of cases subjected to open reduction presented a hypertrophic scar. CONCLUSION: Surgical treatment of severely displaced proximal humerus fractures is successful, both with closed and open reduction; type of implant does not play a key role and should be selected based on the characteristics of each case. Surgical training should prevail at the time of decision-making. When these recommendations are followed, results can be excellent and sequelae are infrequent.

Disparity Analysis of Clinical Features and Prognostic Evaluation of Hemophagocytic Lymphohistiocytosis in Pediatric and Adult Patients: A Retrospective Multicenter Study of 525 Cases in HHLWG.

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare immune disorder with rapid progression and high mortality. There have been few large cohort study comparisons of pediatric and adult HLH until now. This study was designed to explore the disparity of clinical presentations and evaluate the prognosis in pediatric and adult HLH patients. METHODS: Totally, 525 newly diagnosed HLH patients were included and divided into 4 groups according to age: <6, 6-18, 18-60, and >60 years (geriatric patients). Mann-Whitney U test, Kruskal-Wallis test, χ2 test, and Bonferroni's adjustment were used to explore the difference between age groups. Overall survival (OS) was estimated by using Kaplan-Meier method. The Cox proportional hazard model was used to analyze the univariable and multivariable association between prognostic factors and OS. RESULTS: Geriatric patients had the lowest levels of hemoglobin, platelet, albumin, and the highest level of creatinine, while patients <6 years of age had the lowest values of fibrinogen, IgA, IgM and highest values of triglyceride. The trigger of HLH in patients <18 years of age was mainly EBV infection. However, lymphoma and non-EBV-driven infection were the more frequent drivers in patients aged 18-60 and >60 years, respectively. Geriatric patients were associated with highest mortality (58.8%), and 5-year OS was 43%. By contrast, 5-year OS of patients <6, 6-18, and 18-60 years was 86.1%, 74%, and 58.9%, respectively. Additionally, among patients with different etiologies (EBV, non-EBV-driven infection, and uncertain causes) and treatment regimens (HLH-04, HLH-94, and glucocorticoid regimen), geriatric patients showed lowest 5-year OS. Multivariate analysis revealed that creatinine and alanine aminotransferase were independent risk factors affecting the survival of patients aged 0-6 years, while albumin and IgG were independent factors affecting survival of geriatric patients. CONCLUSION: Our study showed a wide heterogeneity of clinical presentations, etiology distribution, prognostic factors, and survival outcomes in pediatric and adult HLH patients.

Hyperfibrinogenemia and hyponatremia as predictors of perforated appendicitis in children: A retrospective cohort study.

PURPOSE: The aim of this study was to investigate the predictive value of hyperfibrinogenemia and hyponatremia for perforated appendicitis in children. METHODS: A retrospective review of 521 pediatric patients (≤ 15 years) with acute appendicitis confirmed by histopathology from January 2017 to December 2020 was performed. Patients were divided in two groups, those with non-perforated (n = 379; 73%) and perforated appendicitis (n = 142; 27%). The serum values of sodium and fibrinogen were taken before surgery. We performed the receiver operating characteristic analysis for the two biochemical markers. The sensitivity, specificity, positive and negative predictive values for perforated appendicitis in the presence of hyponatremia and hyperfibrinogenemia were calculated. RESULTS: Hyperfibrinogenemia (≥ 4.0 g/L) was found in 58.45% of perforated appendicitis and 104 of 142 (73.34%) children with perforated appendicitis had hyponatremia (≤ 135 mmol/L). The perforated appendicitis group had a higher mean fibrinogen concentration (P = 0.001). There was a statistically significant difference in mean serum sodium levels between the perforated appendicitis and non-perforated appendicitis groups (P = 0.016). Receiver operating characteristic curve analysis for fibrinogen, sodium and combination of the both markers shown the combination had the largest area under the curve in identifying children with perforated acute appendicitis (0.858) (95% CI, 0.82-0.90) compared with fibrinogen (0.815) (95% CI, 0.77-0.86) and sodium 0.818 (95% CI, 0.78-0.86) alone. Furthermore, the combination of both markers had the best positive and negative predictive value for appendix perforation compared to fibrinogen and sodium. CONCLUSION: Hyponatremia and/or hyperfibrinogenemia are excellent markers for predicting perforated appendicitis in children. We propose that plasma sodium and/or fibrinogen concentrations be utilized as a supplementary to guide individual treatment decisions in children with appendicitis, such as surgery timing and nonoperative management options.

Views on Short Stature of Female vs Male Endocrine Pediatric Patients Undergoing Provocative Growth Hormone Testing and Their Parents.

OBJECTIVE: Boys outnumber girls in short stature evaluations and growth hormone treatment despite absence of gender differences in short stature prevalence. Family views on short stature influence medical management, but gender-based analysis of these views is lacking. This study explored endocrine patients' and their parents' perceptions of short stature and its impact on quality of life by patient gender. METHODS: Patients aged 8 to 14 years undergoing provocative growth hormone testing and 1 parent each completed semistructured interviews. Clinical data were extracted by chart review. RESULTS: Twenty-four patient-parent dyads (6 female patients, 22 mothers; predominantly non-Hispanic White) participated. Six major themes emerged: (1) patients' perceptions of their short stature were similar by gender, (2) physical experiences of short stature were similar by gender, (3) social experiences of short stature were both similar and different by gender, (4) parental perceptions of short stature as a factor limiting their child's functionality were similar by gender, (5) concern about societal stigma related to short stature arose for both genders, and (6) patients' perceptions of parental messaging about the import of their short stature were similar by gender. CONCLUSION: Our data reveal more similarities than differences between genders in patient perceptions and patient and parent-reported experiences of short stature. Worry about stature-related stigma was noted for patients of both genders. Parental messaging about short stature emerged as an important area to explore further by patient gender. Our findings suggest that clinicians should be wary of making gender or stigma-based assumptions when evaluating children with short stature.

Transition of Adolescents With Severe Asthma From Pediatric to Adult Care in Spain: The STAR Consensus.

OBJECTIVES: To assess the degree of consensus among a multidisciplinary expert panel on the transition of adolescents with severe asthma from pediatric to adult care. METHODS: A 61-item survey was developed based on guidelines for other chronic diseases, covering transition planning, preparation, effective transfer, and follow-up. A 2-round Delphi process assessed the degree of consensus among 98 experts (49 pediatricians, 24 allergists, and 25 pulmonologists). Consensus was established with ≥70% agreement. RESULTS: Consensus was reached for 42 items (70%). Panelists were unable to agree on an age range for initiation of transition. The main goal during the transition identified by the experts is for adolescents to gain autonomy in managing severe asthma and prescribed treatments. The panelists agreed on the importance of developing an individualized plan, promoting patient autonomy, and identifying factors associated with the home environment. They agreed that the adult health care team should have expertise in severe asthma, biologics, and management of adolescent patients. Pediatric and adult health care teams should share clinical information, agree on the criteria for maintaining biological therapy, and have an on-site joint visit with the patient before the effective transfer. Adult health care professionals should closely follow the patient after the effective transfer to ensure correct inhaler technique, adherence, and attendance at health care appointments. CONCLUSION: This consensus document provides the first roadmap for Spanish pediatric and adult teams to ensure that key aspects of the transition process in severe asthma are covered. The implementation of these recommendations will improve the quality of care offered to the patient.

Pharmacokinetics of intravenous daptomycin in Japanese pediatric patients: Pharmacokinetic comparisons supporting dosing recommendations in Japanese pediatric patients.

INTRODUCTION: The pharmacokinetics (PK) of daptomycin has not been previously characterized in Japanese pediatric patients with complicated skin and soft tissue infections (cSSTI) or bacteremia. An aim of the study includes evaluation of PK of daptomycin in Japanese pediatric patients and an appropriateness of the age-specific, weight-based dosing regimens in Japanese pediatric patients based on PK comparison with Japanese adult patients. METHODS: The phase 2 trial enrolled Japanese pediatric patients (age 1-17 years) with cSSTI (n = 14) or bacteremia (n = 4) caused by gram-positive cocci in order to evaluate safety, efficacy and PK. The Phase 3 trial in Japanese adult patients (SSTI n = 65, septicemia/right-sided infective endocarditis (RIE) n = 7) was referred to for PK comparison between adult and pediatric. Daptomycin concentrations in plasma were analyzed by reverse-phase high-performance liquid chromatography (HPLC). PK parameters were determined using non-compartmental analysis in Japanese pediatric and Japanese adult patients. The exposures in Japanese pediatric patients were graphically compared with those in Japanese adult patients. The relationship between daptomycin exposures and creatine phosphokinase (CPK) elevation was explored visually. RESULTS: Following administration of the age-specific, weight-based dosing regimens, daptomycin exposures were overlapping across age groups in pediatric patients with cSSTI with similar observations based on clearance. The distribution of individual exposure in Japanese pediatric patients was overlapping with that in Japanese adult patients. No apparent relationship between daptomycin exposures and CPK elevation in Japanese pediatric patients was observed. CONCLUSIONS: The results suggested that the age-specific, weight-based dosing regimens are considered to be appropriate in Japanese pediatric patients.

The Role of Blood Purification by HA330 as Adjunctive Treatment in Children with Septic Shock.

INTRODUCTION: Despite advances in supportive care for critically ill patients, sepsis remains an important cause of death worldwide in the PICU. One of the hallmarks of sepsis is hyperinflammation due to the excessive release of inflammatory mediators. Recently, new therapeutic approaches, such as immune modulation and blood purification, have been tried to improve outcomes in patients with septic shock. METHODS: This study is a prospective observational study composed of children with septic shock and the PELOD-2 score ≥10 or the PRISM-3 score ≥15. All received 2-4 h of HA330 treatment on 2 consecutive days, used as adjunctive therapy. The effectiveness of HA330 hemoperfusion was evaluated by improving the PELOD-2 and PRISM-3 scores, the vasoactive inotropic score (VIS), and inflammatory markers from baseline to 72 h after the use of HA330 hemoperfusion. RESULTS: Twelve patients hospitalized in the PICU and diagnosed with septic shock between July 2021 and May 2022 were included in this study and received hemoperfusion with HA330. The average PELOD-2 and PRISM-3 scores decreased significantly from 9.5 (IQR: 6.5-13.0) at baseline to 2.0 (IQR: 0-6.5) at 72 h (p = 0.002) and from 16.5 (IQR: 15.0-20.5) at baseline to 5.5 (IQR: 2.0-9.5) at 72 h (p = 0.002), respectively. The VIS decreased significantly from baseline to 72 h (p = 0.003). IL-6, procalcitonin, and lactate levels also decreased significantly from baseline to 72 h (p = 0.005, 0.03, and 0.03, respectively). Two of 12 patients expired due to their underlying condition (2/12, 16.7%). Device-related adverse events did not occur in this study. CONCLUSIONS: Our observational case series suggests a possible role for HA330 hemoperfusion as an adjunctive treatment of refractory septic shock in children with high severity scores in the context of rapid improvement in organ dysfunction, without serious adverse effects.

Efficacy of Incisional Ropivacaine Infiltration by Presternal Multi-Orifice Catheter for Post-sternotomy Pain Relief in Pediatric Patients Undergoing Cardiac Surgery: A Prospective, Randomized, Controlled Study.

OBJECTIVE: To evaluate the efficacy of incisional ropivacaine infiltration by presternal multi-orifice catheter to manage poststernotomy pain in pediatric cardiac surgery. DESIGN: A prospective, randomized, and double-blind comparative study. SETTING: At a single-institution tertiary referral cardiac center. PARTICIPANTS: The study comprised 200 children undergoing cardiac surgeries through a midline sternotomy. INTERVENTIONS: Children were allocated randomly to 1 of 3 groups. Group A (n = 65) and group B (n = 64) received 0.375% ropivacaine infusion and intermittent bolus, respectively, by presternal multi-orifice catheter, whereas Group C (n = 64) did not receive any local anesthetic (LA) drug. Postoperatively, intravenous paracetamol was used for multimodal analgesia, and fentanyl was given as rescue analgesia, respectively. MEASUREMENTS AND MAIN RESULTS: Pain was assessed by a Modified Objective Pain Score (MOPS) for 48 hours postextubation. Group B had significantly lower early MOPS at the first hour, but in the later period, the mean MOPS was lower in group A. The requirement of the first rescue analgesia was 3 ± 1.51, 6.1 ± 2.26, and 2.6 ± 0.87 hours for groups A (n = 60), B (n = 60), and C (n = 60), respectively. The 48-hour fentanyl consumption was significantly lower (p < 0.001) in group A (0.5 ± 0.68 µg/kg) and group B (0.7 ± 0.86 µg/kg) than the control group (3.4 ± 0.68 µg/kg). The length of intensive care unit stay was lower (p < 0.001) in groups A and B than in group C; however, the length of hospital stay was comparable (p = 0.07). CONCLUSION: LA bolus and infusion through presternal multi-orifice catheter provided effective analgesia postoperatively. However, the bolus was more efficacious in the early phase but equivalent in later periods. Therefore, bolus and LA infusion can be used for steady poststernotomy pain relief in children undergoing cardiac surgeries.

Antiseizure medication treatment outcomes in new-onset pediatric epilepsy.

BACKGROUND: Antiseizure medications (ASMs) are the primary treatment for epilepsy; however, some prospective cohort studies in adults suggested that the efficacy of the third and subsequent ASM treatments are poor. Thus, we aimed to assess the outcomes of ASM treatment in new-onset pediatric epilepsy. METHODS: We retrospectively studied 281 pediatric patients diagnosed with epilepsy, in which the first ASM was prescribed between July, 2015, and June, 2020, at Hiroshima City Funairi Citizens Hospital. We reviewed their clinical profiles and seizure outcomes at the end of the study in August, 2022. Seizure freedom was defined as having no seizures for the previous 12 months or longer. RESULTS: Age at the onset of epilepsy ranged from 22 days to 186 months (mean: 84 months). The most frequent classifications of the types and syndromes of epilepsy were focal epilepsy (n = 151, 53.7%), followed by generalized epilepsy (n = 30, 10.7%), and self-limited epilepsy with centrotemporal spikes (n = 20, 7.1%). During the first ASM regimen, 183 out of the 281 (65.1%) patients became seizure free. During the second ASM regimen, 47 out of the 92 (51.1%) patients became seizure free. Only 15 out of the 40 (37.5%) patients who tried the third and subsequent ASM regimen became seizure free, while none became seizure free after the sixth and subsequent ASM regimen. CONCLUSIONS: The efficacy of ASM treatment after the third and subsequent regimen was poor in children, as well as in adults. It is important to reconsider whether there are indications for treatments other than ASM.

Endoscopic transoral resection for an upper cervical chordoma in a pediatric patient.

BACKGROUND: Upper cervical chordoma (UCC) is a rare disease, and although transoral approaches are the methods of choice, minimally invasive techniques have not been established. METHOD: We report the successful use of endoscopic transoral surgery for upper cervical chordoma at the C1-3 levels in the midline epidural space in an 8-year-old girl who presented with neck pain and quadriplegia. Three months after occipitocervical posterior fixation, endoscopic transoral surgery was performed and the tumor was nearly totally removed. CONCLUSION: Endoscopic transoral surgery in pediatric patients with UCCs is a minimally invasive and safe technique.

Delayed Migration of Onyx Embolic Agent after Preoperative Embolization of an Arteriovenous Malformation in a Pediatric Patient: A Case Report and Review of the Literature.

INTRODUCTION: 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. CASE PRESENTATION: 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. CONCLUSION: 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.

Clinical usefulness of anaerobic blood culture in pediatric patients with bacteremia.

OBJECTIVES: The purpose of this study is to evaluate the usefulness of anaerobic blood culture in pediatric patients by comparing the detection rate and distribution of bacteria between aerobic and anaerobic blood culture bottles. METHODS: A retrospective analysis was conducted on 11,664 blood cultures obtained from children under the age of 14 between January 2013 and June 2020. The positive rate of total, aerobic, and anaerobic blood culture, as well as the species distribution of each blood culture bottle, were investigated. RESULTS: The positive rate of blood culture was 2.4 % (N = 281). Among them, 67 (23.8 %), 85 (30.3 %) and 129 (45.9 %) organisms were grown in only aerobic, only anaerobic, and both blood culture bottles, respectively. Gram-positive cocci were cultured on both, only aerobic, and only anaerobic blood culture bottles in proportions of 46.4 %, 23.4 %, and 30.2 %, respectively. Gram-negative bacilli were cultured on both, only aerobic, and only anaerobic blood culture bottles in proportions of 58.5 %, 12,3 %, and 29.2 %, respectively. Gram-positive bacilli grew best in aerobic bottle only. There were seven strains of obligate anaerobes. CONCLUSION: Because many facultative anaerobic bacteria are recognized primarily from anaerobic blood culture bottles, combining aerobic and anaerobic blood culture bottles might be beneficial in pediatric patients with suspected blood stream infection.

Is laparoscopic pyelolithotomy an alternative to percutaneous nephrolithotomy for treatment of kidney stones larger than 2.5 cm in pediatric patients?

PURPOSE: The aim was to compare laparoscopic pyelolithotomy and percutaneous nephrolithotomy in terms of efficacy and safety for the treatment of renal pelvis stones larger than 2.5 cm in pediatric patients. METHODS: Our study consisted of two groups. Group 1 included 33 patients who underwent laparoscopic pyelolithotomy (LPL) between January 2013 and March 2022, and group 2 included 39 patients who underwent percutaneous nephrolithotomy (PCNL). The basic clinical parameters of the patients were recorded. Mean operation time, stone size, estimated blood loss, blood transfusion rate, postoperative hospital stay, stone-free rate, postoperative analgesia requirements, intraoperative complications, and early and late postoperative complications were compared between the two groups. RESULTS: In our study, the mean age of the patients in groups 1 and 2 was 8.89 ± 1.58 years and 9.1 ± 1.85 years, respectively (p = 0.657). The mean stone size was 2.37 ± 0.38 cm in group 1 and 2.55 ± 0.45 cm in group 2 (p = 0.64). The mean operation time was 85.65 ± 20.55 min in group 1, while it was 76.11 ± 13.12 min in group 2 (p = 0.08). The stone-free rate was 100% in both groups. Intraoperative mean blood loss, need for postoperative analgesia, blood transfusion and intraoperative complication rates were significantly higher in the PCNL group (p < 0.01, p = 0.02, p < 0.01, p < 0.01, respectively). CONCLUSION: Our results show that laparoscopic pyelolithotomy is a safe and effective method for pediatric patients with large kidney stones.