Significance of Serum Leucine-rich Alpha-2 Glycoprotein as a Diagnostic Marker in Pediatric Inflammatory Bowel Disease.

Serum leucine-rich alpha-2 glycoprotein (LRG) has been utilized for adult inflammatory bowel disease (IBD); however, its efficacy in pediatric IBD remains unknown. The aim of this study was to compare the diagnostic accuracy of serum LRG for pediatric IBD with that of current inflammatory markers, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). This retrospective case-control study included pediatric patients, aged <16 years, who underwent colonoscopy and/or esophagogastroduodenoscopy between April 2017 and March 2022. All eligible patients were divided into two groups: patients with IBD, diagnosed with ulcerative colitis and Crohn's disease, and non-IBD controls. The optimal cut-off value of serum LRG for IBD diagnosis was determined from receiver operating characteristic analysis, and diagnostic accuracy of serum LRG was compared to serum ESR and CRP. A total of 53 patients (24 with IBD and 29 non-IBD controls) met the inclusion criteria. The cut-off value of serum LRG for IBD diagnosis was determined to be 19.5 µg/ml. At this cut-off value, serum LRG had a positive predictive value (PPV) of 0.80 and negative predictive value (NPV) of 0.88. In contrast, PPV and NPV were 0.78 and 0.70 for serum ESR and 0.82 and 0.72 for serum CRP, respectively. Serum LRG can be a potential diagnostic marker for pediatric IBD, with higher diagnostic accuracy than that of the conventional serum markers ESR and CRP.

Endosurgery workshop to improve the confidence of novice pediatric surgeons in performing laparoscopic hepaticojejunostomy.

PURPOSE: This study assessed the efficacy of a high-impact, short-term workshop in honing the laparoscopic hepaticojejunostomy technical skills and self-confidence of novice pediatric surgeons, focusing on vertical needle driving and knot tying. METHODS: Lectures, hands-on sessions, pre- and post-workshop evaluations, and training using porcine models were conducted to refine basic and advanced skills. The "hepaticojejunostomy simulator" was used for comparative analysis of precision in pre- and post-workshop vertical needle driving and knot tying. Participants self-evaluated their skills and confidence on a 5-point scale. RESULTS: After the workshop, eight inexperienced pediatric surgeons demonstrated a significant improvement in hepaticojejunostomy suturing task completion rates and needle-driving precision at the jejunum and hepatic duct. However, the A-Lap Mini Endoscopic Surgery Skill Assessment System indicated no significant improvements in most assessed parameters, except for the full-layer closure score (p = 0.03). However, a significant increase in participants' confidence levels in performing laparoscopic hepaticojejunostomy was observed. CONCLUSION: The workshop augmented technical proficiency and confidence in young pediatric surgeons. The combination of lectures, practical exposure, and model training is an effective educational strategy in pediatric surgical instruction.

Peroral Endoscopic Myotomy in Pediatric Patients with Achalasia up to 12 Years of Age: A Pilot Study in a Single-Center Experience in Japan.

INTRODUCTION: Peroral endoscopic myotomy (POEM) is a minimally invasive endoscopic procedure for achalasia; its indication has expanded from adults to children. We aimed to evaluate the postoperative efficacy and antireflex status of POEM in young children with achalasia aged 12 years or younger. PATIENTS: AND METHODS: Pediatric patients with achalasia aged 18 years or younger who underwent POEM in our hospital between 2016 and 2021 were included and divided into two age groups: group A (≤ 12 years) and group B (13-18 years). The success rate (Eckardt score ≤ 3), endoscopic reflux findings, and antiacid use at 1 year postoperatively were compared between the groups. RESULTS: Ten patients (four boys and six girls; Chicago classification type I: five, type II: four, and unclassified: one) were included. Mean age and preoperative Eckardt scores in groups A (n = 4) and B (n = 6) were 9.2 ± 3.0 versus 15.6 ± 0.6 years (p = 0.001) and 5.5 ± 3.9 versus 7.2 ± 3.7 (p = 0.509), respectively, and mean operative time and myotomy length were 51.3 ± 16.6 versus 52.5 ± 13.2 minutes (p = 0.898) and 10.8 ± 4.6 versus 9.8 ± 3.2 cm (p = 0.720), respectively. The 1-year success rate was 100% in both groups. Mild esophagitis (Los Angeles classification B) was endoscopically found in one patient in each group (16.7 vs. 25.0%, p = 0.714), and antiacid use was required in three patients (group A, two; group B, one; 50.0 vs. 16.7%, p = 0.500). CONCLUSION: The success rate of POEM within 1 year in young children with achalasia aged 12 years or younger was equal to that in adolescent patients. However, young children tended to require antiacids 1 year postoperatively; therefore, long-term follow-up is necessary.

Criteria for Preserving Grossly Ischemic Torsed Testicles Using Indocyanine Green Fluorescence Imaging: A Single-Center Case Series.

Testicular torsion (TT) is a surgical emergency requiring early diagnosis and surgical intervention to avoid testicular loss. We report 3 pediatric TT cases with testicular salvage to visualize the blood flow by indocyanine green fluorescence imaging (ICG-FI) on the tunica albuginea of the testis. ICG-FI could not confirm blood flow in the testicular parenchyma; however, it could be detected in postoperative ultrasonography. Blood visualization of the tunica albuginea by ICG-FI may have potential criteria for testicular salvage. Long-term follow-up investigations after testicular salvage are needed to conclude the ICG-FI efficacy for TT.

Dienogest Use for Recurrent Pyosalpinx as a Long-Term Complication of Cloacal Exstrophy: A Case Report.

BACKGROUND: Cloacal exstrophy (CE) is a rare congenital disease that requires multiple surgeries for complex gastrointestinal and genitourinary anomalies. Long-term complications are not uncommon; however, they are poorly reported. Pyosalpinx is sometimes encountered during CE management in adolescents and young adults. CASE: A 28-year-old woman with a history of CE presented with fever, lower abdominal pain, and vomiting and was diagnosed with left pyosalpinx. Computed tomography-guided drainage and intravenous antibiotic administration were successful; however, she had 2 readmissions for recurrent pyosalpinx 1 week after discharge and again 4 months later. She was administered Dienogest, a synthetic progestin, to prevent recurrent pyosalpinx and had no recurrence for 8 months. SUMMARY AND CONCLUSION: Dienogest is a conservative treatment choice for preventing the recurrence of pyosalpinx for patients with CE.

Incidence of testicular microlithiasis after orchidopexy for cryptorchidism and its risk factors: A retrospective cohort study.

INTRODUCTION: Testicular microlithiasis (TML) is detected using scrotal ultrasound (US) and is characterized by hyperechoic non-shadowing foci 1-3 mm in diameter within the testicular parenchyma. Although a history of orchidopexy is a known risk factor for TML, and TML is a relatively common condition in postoperative cryptorchidism patients, no previous report has focused on risk factors for TML after orchidopexy. OBJECTIVE: The aims of this study were to evaluate the time-dependent incidence of TML after orchidopexy for cryptorchidism and to identify risk factors for TML. STUDY DESIGN: This study included patients who underwent primary orchidopexy for cryptorchidism between 2004 and 2018 and could be evaluated using scrotal US before and after the procedure. Patients with chromosomal abnormalities and those with preoperative TML were excluded. The Kaplan-Meier method was used to assess postoperative TML appearance. The Cox proportional hazard model was used to evaluate three potential risk factors for postoperative TML: higher testicular position (proximal to the external inguinal ring), delayed orchidopexy (after 18 months of corrected age), and bilateral cryptorchidism. RESULTS: A total of 214 testes from 163 patients were eligible for inclusion (operative age and follow-up time: 2.2 years ± 18 months and 6.3 years ± 46 months, respectively). TML was found in 14/163 patients (8.6%) and 17/214 testes (8.1%). No testicular tumors were discovered during follow-up. The time-dependent TML incidence was 6.0% at 5 years and 11.2% at 10 years after surgery (Summary figure). A higher testicular location proximal to the external inguinal ring was found to be an independent risk factor in the Cox proportional hazard model (hazard ratio 6.18, 95% confidence interval 1.37-27.9, Summary figure). DISCUSSION: Our findings show that the incidence of postoperative TML increases for approximately 10 years following orchidopexy and is associated with a higher testicular location proximal to the external inguinal ring at surgery. In the adult population, TML with a history of cryptorchidism is a known risk factor of testicular malignancy. Given that more than 20% of boys with a higher testicular position developed TML, US evaluation to detect TML during pubety is feasible for the patients with a history of orchidopexy. CONCLUSION: The incidence of postoperative TML in boys with cryptorchidism rises until puberty and is strongly associated with a higher testicular position at orchidopexy.

Possible pair-graphene structures govern the thermodynamic properties of arbitrarily stacked few-layer graphene.

The thermodynamic properties of few-layer graphene arbitrarily stacked on LiNbO3 crystal were characterized by measuring the parameters of a surface acoustic wave as it passed through the graphene/LiNbO3 interface. The parameters considered included the propagation velocity, frequency, and attenuation. Mono-, bi-, tri-, tetra-, and penta-layer graphene samples were prepared by transferring individual graphene layers onto LiNbO3 crystal surfaces at room temperature. Intra-layer lattice deformation was observed in all five samples. Further inter-layer lattice deformation was confirmed in samples with odd numbers of layers. The inter-layer lattice deformation caused stick-slip friction at the graphene/LiNbO3 interface near the temperature at which the layers were stacked. The thermal expansion coefficient of the deformed few-layer graphene transitioned from positive to negative as the number of layers increased. To explain the experimental results, we proposed a few-layer graphene even-odd layer number stacking order effect. A stable pair-graphene structure formed preferentially in the few-layer graphene. In even-layer graphene, the pair-graphene structure formed directly on the LiNbO3 substrate. Contrasting phenomena were noted with odd-layer graphene. Single-layer graphene was bound to the substrate after the stable pair-graphene structure was formed. The pair-graphene structure affected the stacking order and inter-layer lattice deformation of few-layer graphene substantially.

The clinical outcomes of pediatric inguinal hernia repairs operated by surgical residents.

BACKGROUND: Inguinal hernia is one of the most common surgical diseases, and surgical residents perform inguinal hernia repairs during pediatric surgery rotation. The aims of this study were to examine the clinical outcomes of pediatric inguinal herniorrhaphy performed by surgical residents and to assess the suitability for surgical training. METHODS: We extracted data on elective unilateral inguinal hernia repairs performed in children between 2014 and 2018. All eligible cases were divided into surgical resident and attending surgeon groups according to the operator. We reviewed medical records retrospectively and compared some clinical indicators between the 2 groups. RESULTS: Of 294 eligible cases, 163 were performed by 37 surgical residents and 131 were performed by 4 attending surgeons. The median operative times in the resident and attending surgeon groups were 30 and 19 min, respectively. Operative time was statistically longer in the resident group (p < 0.001). The complication and recurrence rates in the resident and attending surgeon groups were 11.0% and 11.5%, and 0% and 0.8%, respectively (p = 1.000 and p = 0.466). CONCLUSION: Inguinal hernia repairs could be properly performed by surgical trainees with the assistance of attending surgeons. Hernia operation might provide good opportunities for surgical residents to practice basic surgical skills.

The Outcomes of Portal Vein Reconstruction With Vein Graft Interposition in Pediatric Liver Transplantation for Small Children With Biliary Atresia.

BACKGROUND: Several technical modifications in portal vein (PV) reconstruction have shown excellent outcomes in pediatric liver transplantation (LT); however, which procedure is the best for PV reconstruction of the hypoplastic PV in pediatric LT remains unclear. METHODS: One hundred sixteen pediatric patients aged 1 or younger with biliary atresia (BA) undergoing living donor LT (LDLT) at our center were enrolled in the present study to investigate the outcomes of the different types of PV reconstruction. We compared the results between patients with and without vein graft (VG) interposition (VG group, n = 33; non-VG group, n = 83) to analyze the risk factors for PV complications (PVCs). The median follow-up period was 4.7 years. RESULTS: PVCs occurred in 10 cases (7.2%), including 5 cases in the non-VG group and 5 in the VG group. Stenosis and thrombosis occurred in 10 and 3 cases, respectively, and 3 cases suffered from both. Three patients were surgically treated for PVCs, and 2 underwent stent insertion to treat short-term recurrence after the initial treatment. The incidence of PVCs in the 2 groups did not differ to a statistically significant extent. Although retrograde PV flow was 1 of the significant risk factors in a univariate analysis, a multivariate analysis revealed that early transplant era was the only independent risk factor for PVCs. CONCLUSIONS: VG interposition for PV reconstruction in LDLT appears to be a feasible alternative option with acceptable outcomes for patients with BA. Achieving sufficient PV flow is essential to preventing PVCs after LDLT.

Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients.

BACKGROUND: Intrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS. PATIENTS AND METHODS: Of 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this study. The patients were divided into 3 groups, according to the shunt ratio, calculated by scintigraphy: mild (15%-20%, n = 26), moderate (20%-40%, n = 19), and severe (> 40%, n = 5). We compared the patients' characteristics before LT and the outcomes of LT between these groups. RESULTS: The major original disease resulting in LT in the mild and moderate groups was biliary atresia (73.1% and 52.6%, respectively), while that in the severe group was congenital portosystemic shunt (60%). The median ages at LT were 7.5, 6.1, and 8.3 years in the mild, moderate, and severe groups, respectively. All of the mild and moderate IPS patients lived; however, 3 patients with severe IPS (60.0%) died within 3 months. The shunt ratios of the mild and moderate IPS patients normalized within 2 years after LT, while the 2 surviving severe IPS patients showed a slight improvement. The autopsy findings of the lung in 1 deceased severe IPS patient showed medial hypertrophy and proliferation of intimal cells of the pulmonary arteries, suggesting a diagnosis of portopulmonary hypertension. CONCLUSIONS: LT can be safely performed for mild and moderate IPS patients; however, LT for severe IPS patients should be carefully indicated because concomitant portopulmonary hypertension may be masked by IPS.

Technical Considerations in Liver Transplantation for Biliary Atresia With Situs Inversus.

In liver transplantation (LT) for biliary atresia (BA) with situs inversus (SI), the surgical procedure is technically challenging due to multiple anatomical variations. We evaluated the surgical procedures and the outcomes in our patients and in the previously reported patients undergoing LT for BA with SI. Between November 2005 and October 2018, 235 children underwent LT with an overall 10-year graft survival of 94.7%. Of these, 6 (2.6%) patients received LT for BA with SI. Living donor liver transplantation (LDLT) was applied in all patients. Vascular anomalies at our center included the absence of the inferior vena cava (IVC; n = 5), a preduodenal portal vein (PV; n = 4), and an aberrant hepatic artery (HA; n = 1). Hepatic vein (HV) outflow obstruction occurred in 1 patient with an absent IVC and necessitated repositioning of the graft immediately after LDLT. PV stenosis occurred in 1 patient with a preduodenal PV at 5 months after LDLT. The overall survival of our patients was 80.0% at 3 years. In the literature, 49 patients with LT for BA with SI were reviewed. The survival rate was 91.8%, and the vascular complications included HV outflow obstruction (2.0%), PV complications (10.2%), and HA thrombosis (4.1%). All PV complications occurred in LDLT patients with a preduodenal PV (5/23; 21.7%). In conclusion, the detailed assessment of the surgical procedures and the systematic literature review demonstrated excellent outcomes after LT for BA with SI. Liver recipients with a preduodenal PV are exposed to a higher risk for vascular complications after a very challenging LT procedure with overall good outcomes.

MicroRNA-144 is regulated by activator protein-1 (AP-1) and decreases expression of Alzheimer disease-related a disintegrin and metalloprotease 10 (ADAM10).

BACKGROUND: MicroRNA (miR) dysregulation is found in Alzheimer disease (AD). A disintegrin and metalloprotease 10 (ADAM10) prevents generation of amyloid ß (Aß) and decrease AD pathology. RESULTS: miR-144 suppresses ADAM10 expression and is up-regulated by activator protein-1. CONCLUSION: miR-144 is a negative regulator of ADAM10 and may be involved in AD pathogenesis. SIGNIFICANCE: The first work to demonstrate the function of miRNA-144 and its regulation in the pathogenesis of AD. Amyloid ß-peptide (Aß) accumulating in the brain of Alzheimer disease (AD) patients is believed to be the main pathophysiologcal cause of the disease. Proteolytic processing of the amyloid precursor protein by α-secretase ADAM10 (a disintegrin and metalloprotease 10) protects the brain from the production of the Aß. Meanwhile, dysregulation or aberrant expression of microRNAs (miRNAs) has been widely documented in AD patients. In this study, we demonstrated that overexpression of miR-144, which was previously reported to be increased in elderly primate brains and AD patients, significantly decreased activity of the luciferase reporter containing the ADAM10 3'-untranslated region (3'-UTR) and suppressed the ADAM10 protein level, whereas the miR-144 inhibitor led to an increase of the luciferase activity. The negative regulation caused by miR-144 was strictly dependent on the binding of the miRNA to its recognition element in the ADAM10 3'-UTR. Moreover, we also showed that activator protein-1 regulates the transcription of miR-144 and the up-regulation of miR-144 at least partially induces the suppression of the ADAM10 protein in the presence of Aß. In addition, we found that miR-451, a miRNA processed from a single gene locus with miR-144, is also involved in the regulation of ADAM10 expression. Taken together, our data therefore demonstrate miR-144/451 is a negative regulator of the ADAM10 protein and suggest a mechanistic role for miR-144/451 in AD pathogenesis.