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Introduction: Inflammation is closely associated with tumor development and patient prognosis. The objective of this study is to assess the prognostic value of the preoperative inflammatory indexes in pediatric hepatoblastoma patients who receive neoadjuvant chemotherapy. Methods:: A retrospective analysis was performed on clinical and pathological data of 199 hepatoblastoma patients who underwent hepatectomy with preoperative neoadjuvant chemotherapy from January 2015 to June 2020. The receiver operating characteristic curve was used to evaluate the prognostic value of neutrophil-to-lymphocyte ratio (NLR), platelet-tolymphocyte ratio (PLR), systemic immune-inflammation index (SII), and systemic inflammation response index (SIRI) in predicting OS and EFS. Patients were grouped based on optimal cutoff values of preoperative inflammatory indexes. Survival rates were calculated using the Kaplan-Meier method, and survival outcomes were compared between groups using the log-rank test. Univariate and multivariate Cox proportional hazards regression models were used to identify independent prognostic factors, and a nomogram was constructed using R software to predict the probability of OS. Results: The receiver operating characteristic curve showed prognostic value for OS, not EFS, in preoperative inflammatory indexes. Patients were categorized into low/high groups: SII ≤ 266.70/higher, NLR ≤ 1.24/higher, PLR ≤ 85.25/higher, and SIRI ≤ 0.72/higher. High NLR, PLR, SII, and SIRI groups had significantly lower 5-year OS than their low counterparts (all p-value < 0.05). The Cox analysis identified four independent prognostic factors: SIRI (HR=2.997, 95% CI: 1.119-8.031), microvascular invasion (HR=2.556, 95% CI: 1.14-5.73), the post-treatment extent of disease (POSTTEXT) staging (IV vs. I: HR=244.204, 95% CI:11.306-5274.556), and alpha-fetoprotein (>100 ng/ml: HR=0.11, 95% CI: 0.032-0.381) for hepatoblastoma patients with neoadjuvant chemotherapy. High SIRI group had more patients with adverse NLR, SII, and POSTTEXT III (all p-value < 0.05). Independent prognostic factors led to an OS nomogram with a concordance index of 0.85 (95% CI: 0.78-0.91, p-value = 1.43e-27) and the calibration curve showed a good fit between the prediction curve and the true curve. Conclusions: SIRI is an independent prognostic factor of hepatoblastoma patients receiving neoadjuvant chemotherapy. The OS nomogram based on SIRI, POSTTEXT staging, MiVI, and AFP can be used to assess the prognosis of those patients. [ABSTRACT FROM AUTHOR]

Background: Thoracotomy is considered the standard surgical approach for the management of pulmonary metastases in osteosarcoma (OST). Several studies have identified the advantages of a thoracoscopic approach, however, the clinical significance of thoracotomy compared to thoracoscopy is yet to be evaluated in a randomized trial. Aims: The primary aim was to determine the survival outcomes in OST patients based on surgical approach for pulmonary metastasectomy (PM) and secondary aim was to assess the post‐operative morbidities of OST PM through various surgical approaches. Materials and Methods: We conducted a single institution retrospective study to compare survival outcomes and surgical morbidity according to the surgical approach of the management of pulmonary metastases in patients with OST. Results: Sixty‐one patients with OST underwent PM. Twenty‐one patients were metastatic at diagnosis and underwent PM during primary treatment; nine had thoracotomy, six thoracoscopy, and six combined thoracoscopy with thoracotomy (CTT). Forty‐three patients with first pulmonary relapse or progression underwent PM; 18 had thoracotomy, 16 thoracoscopy and nine CTT. There was no difference in survival between surgical approaches. There were significantly more postoperative morbidities associated with thoracotomy for initial PM (pain and postoperative chest tube placement), and for PM at first relapse (pneumothoraces, pain, Foley catheter use and prolonged hospitalizations). Conclusion: Our study demonstrates that patients with OST pulmonary metastases have comparable poor outcomes despite varying surgical approaches for PM. There were significantly more postoperative morbidities associated with thoracotomy for PM. Surgical bias and other competing risks could not be assessed given the limitations of a retrospective study and may be addressed in a prospective trial evaluating surgical approach for PM in OST. [ABSTRACT FROM AUTHOR]

Introduction: Abdominal surgery in children is known to contribute to Adhesive Small Bowel Obstruction (ASBO). No X-ray findings have been validated for predicting management in ASBO. Various imaging modalities often cannot predict complications or if a conservative line of management would be successful. Aim: To determine if a persistent prominently dilated small bowel loop in the Left Upper Quadrant (LUQ) on plain X-rays (Omega loop) would suggest early operative intervention. Materials and Methods: A retrospective cohort study was conducted in the Department of Paediatric Surgery, IMCH, Government Medical College, Kozhikode, Kerala, India from January 2016 to December 2018. Diagnosis was based on history, clinical features, and radiologic findings. Demographic, clinical, radiologic, and operative data were collected. After exclusions, the study population was divided into two groups: Group A - ASBO patients with the characteristic Omega loop, and Group B - patients without the Omega loop. Appropriate statistical methods were used to compare the groups, with significance defined as p<0.05. Results: Among the 72 cases of ASBO that met the inclusion criteria, 40 (55%) were successfully managed conservatively, and 32 (45%) required non urgent relaparotomy. The median age was 6.9 years (range: 2-12 years), and 55% were male. Group A comprised 16 patients (22.2%), and the remaining patients were in Group B 56 (77.8%). Group A showed higher rates of failure of conservative management (n=14, p=0.0002), with higher rates of single-band obstruction, bowel loss, perforation, matting of bowel loops, and difficult dissection. The Omega loop had a low sensitivity (43.7%) but high specificity (95%) and positive predictive value (87.5%) for the need for relaparotomy. Conclusion: The Omega loop suggests the possibility of more complications, and therefore, earlier operative intervention should be considered. [ABSTRACT FROM AUTHOR]

Background Lymphadenopathy (LN) affects as many as 90% of young children. A common cause of cervical LN is viral agents. The second most common cause is bacterial infections. While cancer is the most feared cause of generalized LN [2]. Objectives To define the etiology of localized versus generalized LN in children and adolescents in two tertiary centers (Ain Shams and Sultan Qaboos University Hospitals). Define the alarming signs for possible malignancy. Methods All children 2–16 years of both sexes presented to the two centers during the period 2018–2022, we divided them into patients with single lymph node enlargement and those with multiple LNs. Also with viral infection,either EBV or cytomegalovirus (CMV), and those with oncological diagnosis such as lymphoma and leukemia. Results Six hundred twenty-four children, 62% males aged 2–16 years (median, 6.5 years) over a period of 4 years,and acute both bacterial and granulomatous-related LNs were excluded (n=236). Meanwhile, 388 were enrolled from two centers, Ain Shams Children's hospital (90%) and Sultan Qaboos (10%). In Ain Shams, 40% were referred to the general pediatrics clinic (n=155), only 1.9% were malignant, while of those referred to the pediatric oncology clinic(n=233), 42 (mostly 18%) were malignant, either leukemia (n=21) or lymphoma (four Hodgkin and seven NHL), and 10 were IMLN. Viral infection was the commonest; two-thirds were EBV and one third were CMV infection. Affected children with leukemia or NHL were commonly younger than 5 years. Boys were affected more with either EB or CMV often (two-thirds). No difference in the frequency of high temperature and sore throat between examined children in both viruses, but stomachaches, eyelid swelling, and skin rash were more common in CMV, while the frequency of LN and hepatosplenomegaly was not different. Alarming signs for risk for leukemia were associated with fever for more than 2 weeks. Purpura, bone aches and LNs. Generalized, nonregressive, firm and nontender, and splenomegaly were more at risk for noninfectious. Delay in diagnosis was 9–56 days comparable in both localized and generalized LN(mean, 17±3.1 days). Conclusion EBV was the commonest viral cause of LN in young children. Alarming signs for more serious illness were bone aches, splenomegaly, and purpura. [ABSTRACT FROM AUTHOR]

Purpose: The initial management of primary spontaneous pneumothoraxes (PSP) in children remains controversial, particularly regarding the timing of operative intervention. This study aimed to identify factors associated with failure of non-operative management of PSP., Methods: A single-center, retrospective review was performed for patients presenting with PSP. Demographics and clinical predictors were collected. Patients successfully managed non-operatively were compared to failed non-operative management. Fischer exact and Mann-Whitney tests were used as appropriate., Results: Fifty-seven pediatric patients were identified as having PSP. Four patients underwent initial surgical intervention, 60% (n = 34) were successfully managed non-operatively, while 33% (n = 19) failed non-operative management and underwent video-assisted thoracic surgery (VATS). Those who failed were more likely to have PSP > 2 cm on initial X-ray (79% vs. 44%, p = 0.021) and have a persistent air leak for > 48 h (47% vs 6%, p ≤ 0.001). LOS was greater in the failure group (11.5 ± 5.1 vs 3.1 ± 2.5, p ≤ 0.001) as well as higher complication rates (21% vs 0%, p = 0.013)., Conclusion: Our findings suggest that patients presenting with PSP of > 2 cm or have a persistent air leak for > 48 h despite chest tube management are unlikely to be treated by chest tube alone and may benefit from earlier operative intervention., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Aim: The risk of organ loss is increased in children with testicular torsion or intestinal volvulus if surgical management is not expedient. The current retrospective study aims to review the time-course from first symptom to 'knife to skin' in these conditions, to determine where delays occur and facilitate a systems approach to better manage these children., Methods: One hundred consecutive paediatric cases of scrotal exploration for presumed testicular torsion, and 100 neonatal cases presenting with possible malrotation/volvulus were analysed to evaluate the exact time-course of events from admission to surgery., Results: (i) Scrotal exploration: the median time from onset of symptoms to presentation was 12 h (interquartile range (IQR): 5-48 h). In children over 5 years of age, 36% (33/93) were transferred from an external district service area. (ii) Malrotation/volvulus: the median duration of symptoms prior to arrival/assessment was 12 h (IQR: 4-24 h). The median cumulative in-hospital time was over 6 h (368 min, IQR: 247-634 min)., Conclusions: Time to presentation contributes significantly to testicular ischaemic time. This delay to timely surgical intervention is multi-factorial, and must be addressed at a public health level. Support and training in the management of testicular torsion should be provided to all adult surgeons/trainees that may care for these children. In general, this condition is best managed at the presenting hospital whenever appropriate expertise is available. Novel pathways that streamline care may improve efficiency at an institutional level. Addressing issues of access to specialised neonatal surgery is more vexed on account of the tyranny of distance, and the pre-requisite level of surgical expertise required., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Purpose: Extrahepatic portal vein obstruction (EHPVO) is a major cause of non-cirrhotic portal hypertension in children. Surgical procedures for EHPVO include portosystemic shunts (PSS) and meso-Rex bypass (MRB). We conducted a systematic review and meta-analysis to compare the effectiveness of MRB versus PSS in EHPVO patients., Methods: A systematic literature search was performed using four databases. Articles reporting EHPVO and comparing patients who received MRB and PSS were included in the analysis., Results: We retrieved 851 papers, of which five observational studies met the inclusion criteria. There was no difference in shunt complications, mortality, or gastrointestinal bleeding after surgery between MRB and PSS in the meta-analysis. MRB had increased shunt complications compared with PSS in the non-comparative studies. MRB had a potential advantage over PSS in long-term prognosis in one comparative study. Overall, the quality of the evidence was low., Conclusions: Based on available data, our meta-analysis indicates that MRB does not increase shunt complications, mortality, or gastrointestinal bleeding after surgery. The present study did not reveal superiority for either MRB or PSS. The paucity of well conducted trials in this area justifies future multicenter studies and studies that examine long-term outcomes., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Cancer treatments of the last decades improve the survival rate of children and adolescents. However, chemo- and radiotherapy result in gonadal damage, leading to acute ovarian failure and sterility. The preservation of fertility is now an integral part of care of children requiring gonadotoxic treatments. Ovarian tissue cryopreservation (OTC) is an effective fertility preservation option that allows long-term storage of primordial follicles, subsequent transplantation, and restoration of endocrine function and fertility. The efficacy of this technique is well-demonstrated in adults but the data are scarce for pediatric patients. Currently, OTC represents the only possibility of preserving the potential fertility in prepubertal girls. Procedure: This is a retrospective study of OTC practice of two French centers from January 2004 to May 2020. A total of 72 patients from pediatric units underwent cryopreservation of ovarian tissue before gonadotoxic therapy for malignant or non-malignant diseases. The ovarian cortex was cut into fragments and the number of follicles per square millimeter was evaluated histologically. The long-term follow-up includes survival rate and hormonal and fertility status. Results: The mean age of patients at OTC was 9.3 years [0.2-17] and 29.2% were postpubertal; 51 had malignant diseases and 21 had non-malignant diseases. The most frequent diagnoses included acute leukemia, hemoglobinopathies, and neuroblastoma. Indication for OTC was stem cell transplantation for 81.9% (n = 59) of the patients. A third of each ovary was collected for 62.5% (n = 45) of the patients, a whole ovary for 33.3% (n = 24) of the patients, and a third of one ovary for 4.2% (n = 3) of the patients. An average of 17 fragments [5-35] per patient was cryoconserved. A correlation was found between the age of the patients and the number of fragments (p < 0.001). More fragments were obtained from partial bilateral harvesting than from whole ovary harvesting (p < 0.05). Histological analysis of ovarian tissue showed a median of 6.0 primordial follicles/mm² [0.0-106.5] and no malignant cells were identified. A negative correlation was found between age and follicular density (p < 0.001). Median post-harvest follow-up was 92 months [1-188]. A total of 15 girls had died, 11 were still under treatment for their pathology, and 46 were in complete remission. Of all patients, 29 (40.2%) were subjected to a hormonal status evaluation and 26 were diagnosed with premature ovarian insufficiency (POI) (p < 0.001). One patient had undergone thawed ovarian tissue transplantation. Conclusion: OTC should be proposed to all girls with high risk of developing POI following gonadotoxic therapies in order to give them the possibility of fertility and endocrine restoration. [ABSTRACT FROM AUTHOR]

Background: As an emerging standard of care for portal vein cavernous transformation (PVCT), Meso-Rex bypass (MRB) has been complicated and variated. The study aim was to propose a new classification of PVCT to guide MRB operations. Methods: Demographic data, the extent of extrahepatic PVCT, surgical methods for visceral side revascularization, intraoperative blood loss, operating time, changes in visceral venous pressure before and after MRB, postoperative complications and the condition of bypass vessels after MRB were extracted retrospectively from the medical records of 19 patients. Results: The median age of the patients (13 males and 6 females) was 32.5 years, while two patients were underage. Causes of PVCT can be summarized as follows: thrombophilia such as dysfunction of antithrombin III or proteins C; secondary to abdominal surgeries; secondary to abdominal infection or traumatic intestinal obstruction, and unknown causes. Intraoperatively, the median operation time was 9.5 h (7–13 h), and the intraoperative blood loss was 300 mL (100-1,600 mL). Ten cases used autologous blood vessels while 10 used allogeneic blood vessels. The vascular anastomosis was divided into the following types according to the site and approach: Type (T) 1-PV pedicel type, T2-confluence type, T3-major visceral vascular type; and T4-collateral visceral vascular type. Furthermore, the visceral venous pressure before and after MRB dropped significantly from 36 cmH2O (28–44) to 24.5 cmH2O (15–31) (P < 0.01). Postoperatively, one patient had delayed wound healing, two developed biochemical pancreatic fistulae, one experienced lymphatic leakage, the former caused by heat damage of the pancreatic tissues, the latter by cutting lymphatic vessels in the mesentery or removing the local lymph nodes during the process of separating the superior mesenteric vein, and one was re-operated on for an intervening intestinal fistulae. Postoperative enhanced CT scans revealed a significant improvement in abdominal varix in the patients with patent bypass, and at the 1-year postoperative follow-up, enhanced CT scans of six patients showed that the long axis of the spleen was reduced by ≥ 2 cm. Conclusions: MRB can effectively reduce visceral venous pressure in patients with PVCT. It is feasible to determine the PVCT type according to the extent of involvement and to choose individualized visceral side revascularization performances. [ABSTRACT FROM AUTHOR]

Background: Embryonal rhabdomyosarcoma (ERMS) of the uterine cervix is rare, but the population affected is mostly underage females. The scope of surgery has now evolved from extensive to limited, and organ-preserving surgery combined with chemotherapy is recommended to preserve the patient's fertility. However, reports of birth outcomes are rare. Case: A minor woman with cervical ERMS who underwent only an outpatient biopsy of the lesion had no residual lesion on subsequent multipoint cervical biopsy and refused radical surgery or cervical conization, after which the patient received a nonclassical regimen of chemotherapy. The patient stopped the chemotherapy on her own, but the patient conceived spontaneously 16 years later with a good pregnancy outcome and no recurrence. Conclusions: This case suggests that preservation of reproductive function is often feasible in immature women with cervical ERMS, and the prognosis is usually good as long as the primary tumour can be surgically removed and the lesion is free of residual disease. We also look forward to reports of subsequent growth and pregnancy outcomes in other children with reproductive tract RMS. In cervical ERMS, accurate evaluation of the disease and development of an individualized treatment plan are crucial, and the protection of reproductive function and psychological well-being deserves special attention. [ABSTRACT FROM AUTHOR]

Background: According to standard medical practice, immediate orchiectomy is advised in the case of a non‐viable testis following testicular torsion. Because of the lack of objective criteria in the evaluation of testicular viability, the fate of the testis solely depends on the discretion and experience of the surgeon. Objective: In this study, we retrospectively reassess the management of patients with testicular torsion in our center, and we ask the question, from a retrospective point of view, of whether the decision to perform orchiectomy has always been correct. Materials and methods: We retrospectively reviewed all cases of patients with testicular torsion who underwent surgery in our center between 2001 and 2021. All orchiectomy specimens were reevaluated and graded by an experienced pathologist using the Mikuz grading system. Results: Immediate orchiectomy was performed in 48 of the 136 patients (35%). Five (10.4%) of the 48 orchiectomy specimens were categorized as "grade 1," and 17 (35.4%) were categorized as "grade 2." The time between the onset of symptoms and surgical exploration exceeded 12 h in three of the five patients with a "grade 1" testicular injury, and in one case, it even exceeded 24 h. Discussion: "Grade 1" testicular injury is potentially reversible, whereas the fate of a testis with a "grade 2" testicular injury remains unknown. Whether and to what extent partial testicular tissue salvage in a "grade 2" injury is still possible remain unclear. Conclusions: Our results indicate that at least 10% of the testicles removed in our center could, from a retrospective point of view, have been salvaged. Our study further demonstrated that the duration of symptoms is not an absolute indicator of testicular damage and that the decision of whether orchiectomy should be performed, based simply on the subjective macroscopic image of the affected testis, is not always correct. [ABSTRACT FROM AUTHOR]

Purpose of Review: Non-cirrhotic portal hypertension (NCPH) is primarily comprised of extrahepatic portal venous obstruction (EHPVO), followed by congenital hepatic fibrosis (CHF) and non-cirrhotic portal fibrosis (NCPF). Various complications besides variceal bleeding pose a management challenge. This review summarizes the best evidence-based management practices for children with NCPH. Recent Findings: Endotherapy in EHPVO eradicates esophageal varices but does not tackle problems like ectopic varices, portal biliopathy, massive splenomegaly, growth failure, etc. Meso-Rex shunt is a physiological shunt that ameliorates nearly all complications in EHPVO. In children with NCPF and isolated CHF, endotherapy remains the mainstay of management, with shunt surgery useful when indicated. Summary: Beyond variceal eradication, endotherapy has a limited role, and evaluation for meso-Rex shunt should be done at the beginning in EHPVO. Endotherapy constitutes the major therapeutic option in NCPF and CHF, except in a small subset who may require liver or a combined liver-kidney transplantation. [ABSTRACT FROM AUTHOR]

When children are discharged from the hospital after surgery, their caregivers often rely on subjective assessments (e.g., appetite, fatigue) to monitor postoperative recovery as objective assessment tools are scarce at home. Such imprecise and one-dimensional evaluations can result in unwarranted emergency department visits or delayed care. To address this gap in postoperative monitoring, we evaluated the ability of a consumer-grade wearable device, Fitbit, which records multimodal data about daily physical activity, heart rate, and sleep, in detecting abnormal recovery early in children recovering after appendectomy. One hundred and sixty-two children, ages 3–17 years old, who underwent an appendectomy (86 complicated and 76 simple cases of appendicitis) wore a Fitbit device on their wrist for 21 days postoperatively. Abnormal recovery events (i.e., abnormal symptoms or confirmed postoperative complications) that arose during this period were gathered from medical records and patient reports. Fitbit-derived measures, as well as demographic and clinical characteristics, were used to train machine learning models to retrospectively detect abnormal recovery in the two days leading up to the event for patients with complicated and simple appendicitis. A balanced random forest classifier accurately detected 83% of these abnormal recovery days in complicated appendicitis and 70% of abnormal recovery days in simple appendicitis prior to the true report of a symptom/complication. These results support the development of machine learning algorithms to predict onset of abnormal symptoms and complications in children undergoing surgery, and the use of consumer wearables as monitoring tools for early detection of postoperative events. [ABSTRACT FROM AUTHOR]

Background: The risks associated with deep vein thrombosis (DVT) have gained significant recognition over time. A prevalent form of distal DVT is isolated calf muscular venous thrombosis (ICMVT). Despite its common clinical occurrence, data on ICMVT subsequent to tibial plateau fracture (TPF) surgery are scarce. This study aimed to examine the epidemiological characteristics and associated risk factors (RFs) of ICMVT following TPF surgery. Methods: For this retrospective analysis, we included patients from our hospital, who underwent TPF surgery between March 2017 and March 2021. Patients' electronic medical records were reviewed, including admission details, fracture classification, surgical procedures, and laboratory biomarkers. The HSS (The American Hospital for Special Surgery) and Rasmussen scores were employed to evaluate the clinical effect. A Color Duplex Flow Imager (CDFI) was regularly used to detect pre- and postoperative venous thrombosis in the lower limbs. Finally, uni- and multivariate logistic regression analyses were used to identify independent RFs associated with ICMVT. Results: Overall, 481 participants were recruited for analysis. Postoperative ICMVT occurred in 47 patients. All ICMVTs occurred on the affected side. Four of the 47 ICMVT patients exhibited sudden postoperative swelling in the affected limb. The HSS and Rasmussen scores in the non-ICMVT cohort (87.6 ± 8.2, 16.0 ± 1.7) were markedly different from the ICMVT cohort (84.8 ± 8.2, 15.5 ± 1.6) (p = 0.014, p = 0.031). This study finally identified five postoperative ICMVT-related RFs, which were age (> 55 years old) (OR 3.06; 95% CI 1.47–6.37; p = 0.003), gender (female) (OR 2.67; 95% CI 1.37–5.22; p = 0.004), surgical duration (> 114 min) (OR 3.14; 95% CI 1.44–6.85; p = 0.004), elevated white blood cell content (OR 2.85; 95% CI 1.47–5.51; p = 0.002), and hyponatremia (OR 2.31; 95% CI 1.04–5.12; p = 0.040). Conclusion: The epidemiological findings of this study may help predict ICMVT risk after surgery thus facilitating the development of individualized clinical assessments and targeted prevention programs. [ABSTRACT FROM AUTHOR]

Background: Commonly, pediatric solid tumors occur independently. Only two patients with synchronous hepatoblastoma (HBL) and neuroblastoma (NBL) have been reported. Case reports: Two Chinese infants presented with abdominal mass at 10 and 8 months. Computed tomography (CT) scans in both revealed hepatic masses with additional mediastinal or adrenal masses. Pathology confirmed synchronous HBLs in the liver and NBLs in the mediastinum and adrenal. Next generation sequencing (NGS) found no remarkable germline mutations. Both patients received gross total resections with chemotherapy before or after surgery. They were followed up for 36 and 8 months, and recovered well. Conclusion: These two cases of synchronous HBL and NBL tumors lacked significant genetic alterations. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety and efficacy of percutaneous interventional treatment of portal vein stenosis in children. Material and methods: A retrospective analysis of all interventional treatments for portal vein stenosis in pediatric patients at a single institution from 2010 to 2021 was conducted. Platelet count, spleen size and portal vein flow velocity were assessed during the follow-up period. Primary and primary assisted patency time were determined. Results: A total of ten children (median age 28.5 months, interquartile range (IQR): 2.75–52.5 months) with portal vein stenosis after Mesorex-Shunt (n = 4), liver transplantation (n = 3) and other etiologies (n = 3) underwent 15 interventional procedures. There were five reinterventions and one discontinued intervention. The technical success rate was 93.3% (14/15) and clinical success of treated patients was 100% (14/14). Median follow-up was 18 months (IQR: 13.5–81 months). The median primary patency time for stent placement was 70 months (IQR: 13.5–127.25 months). For balloon angioplasty, the median primary patency time was 9 months (IQR 7.25–11.5 months), while the median assisted primary patency time was 14 months (IQR: 12 to 15 months). Platelet count, spleen size and portal vein flow velocity reliably corresponded to recurrence of portal vein stenosis in asymptomatic patients during follow-up. Conclusion: Interventional treatment is a safe and efficient method to treat portal vein stenosis with long patency times, regardless of etiology. Primary stent placement shows a higher primary patency time than balloon angioplasty. Implementation of stent placement as the primary interventional method may improve patency times and reduce the need for repeat reinterventions in pediatric patients. [ABSTRACT FROM AUTHOR]

The HALP score, which is a combined index composed of hemoglobin, albumin, lymphocyte, and platelet, is a new indicator showing both inflammation and nutritional status. This study aims to evaluate the relationship of this combined index consisting of simple laboratory values with the degree of appendicitis complication and postoperative results in patients operated on for acute appendicitis. The data of 684 patients operated for acute appendicitis between January 2017 and December 2022 and inclusion criteria were analyzed with a single-center retrospective cross-sectional study design. Using routine laboratory data, patients' HALP scores were divided into two groups as low and high. The cut-off value of the HALP score according to the presence of postoperative complications was determined as < 31.2 by ROC analysis and the ROC curve. Patients were grouped as HALP score cut-off value below (group 1) and above (group 2). Complicated appendicitis and postoperative outcomes were compared to the HALP score groups. According to the cut-off value of the HALP score, 113 (16.5%) of the patients were in Group 1, and 571 (83.5%) were in Group 2. Complications developed in 15 (26%) patients (p < 0.001). Low HALP scores were a significant risk factor for peri-appendicular abscess (OR 29.12 95% CI 12.39–68.43), appendicitis perforation (OR = 20.82 95% 12.67–34.19), gangrenous appendicitis (OR = 35, 54, 95% 13.33–94.77), and postoperative complications (OR = 15.29 95% 7.95–29.41) (p < 0.001). Besides clinical and radiological findings, the HALP score shows the degree of acute appendicitis complication. It can be used as a simple, inexpensive, and easily applicable diagnostic tool. [ABSTRACT FROM AUTHOR]

Background: Pediatric patients often undergo surgery during terminal admissions. However, the involvement and timing of palliative care consults in caring for these patients has not been readily described. Objective: To describe the presence and timing of palliative care consults for pediatric patients who undergo surgical procedures during terminal admissions. Design: Retrospective cohort study using data from the electronic health record. Setting and Participants: Pediatric patients who underwent at least one surgical procedure during a terminal admission at an urban, quaternary hospital in the United States from January 1, 2016 to December 31, 2021. Main Outcomes and Measures: Patients' medical, surgical, and admission-level characteristics were abstracted. Associations were evaluated between these characteristics and the occurrence and timing of a palliative care consult relative to surgery and death. Results: Of 134 patients, 84% received a palliative care consult during their terminal admission. Approximately 36% of consults occurred before surgery, and 12% were within one day of death. Children without a palliative care consult were more likely than children with a consult to die during surgery (19.1% vs. 2.7%, p = 0.02), have surgery within 24 hours of death (52.4% vs. 15.9%, p < 0.001), and undergo a full resuscitation attempt (47.6% vs. 12.4%, p = 0.002). Receipt of a palliative care consultation did not differ by patient sex, reported race and ethnicity, language, insurance, or income level. Conclusions and Relevance: Palliative care consults support high-quality end-of-life care for children and impact perioperative outcomes, including intensity of surgical care and resuscitation in the final hours of life. [ABSTRACT FROM AUTHOR]

Diagnostic reasoning is an important topic in General Practitioners' (GPs) vocational training. Interestingly, research has paid little attention to the content of the cases used in clinical reasoning education. Malpractice claims of diagnostic errors represent cases that impact patients and that reflect potential knowledge gaps and contextual factors. With this study, we aimed to identify and prioritize educational content from a malpractice claims database in order to improve clinical reasoning education in GP training. With input from various experts in clinical reasoning and diagnostic error, we defined five priority criteria that reflect educational relevance. Fifty unique medical conditions from a malpractice claims database were scored on those priority criteria by stakeholders in clinical reasoning education in 2021. Subsequently, we calculated the mean total priority score for each condition. Mean total priority score (min 5–max 25) for all fifty diagnoses was 17,11 with a range from 13,89 to 19,61. We identified and described the fifteen highest scoring diseases (with priority scores ranging from 18,17 to 19,61). The prioritized conditions involved complex common (e.g., cardiovascular diseases, renal insufficiency and cancer), complex rare (e.g., endocarditis, ectopic pregnancy, testicular torsion) and more straightforward common conditions (e.g., tendon rupture/injury, eye infection). The claim cases often demonstrated atypical presentations or complex contextual factors. Including those malpractice cases in GP vocational training could enrich the illness scripts of diseases that are at high risk of errors, which may reduce diagnostic error and related patient harm. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma is the most common soft tissue sarcoma in children, accounting for 4.5% of all cases of cancer in childhood. Although the head and neck are the most common sites of rhabdomyosarcoma, oral lesions are relatively rare and account for only 10% to 12% of head and neck rhabdomyosarcoma cases. This is a case report of a girl aged 2 years and 1 month who initially presented with an upper lip mass that invaded the oral mucosa, oral skin, and nostril skin, causing narrowing of the airway. Through our case, we show that rapidly growing small round cell malignancies, especially rhabdomyosarcoma, can be effectively diagnosed and treated at the same time using primary resection with intraoperative frozen section biopsy and that the time spent waiting for the results of preoperative biopsy can be saved in this way, particularly when the patient's symptoms are intensifying rapidly and require immediate operation. [ABSTRACT FROM AUTHOR]

Background: Cancer predisposition is most often studied in the context of single cancers. However, inherited cancer predispositions can also give rise to multiple primary cancers. Yet, there is a paucity of studies on genetic predisposition in multiple primary cancers, especially those outside of well-defined cancer predisposition syndromes. This study aimed to identify germline variants associated with dual primary cancers of the breast and lung. Methods: Exome sequencing was performed on germline DNA from 55 Singapore patients (52 [95%] never-smokers) with dual primaries in the breast and lung, confirmed by histopathology. Using two large control cohorts: the local SG10K_Health (n = 9770) and gnomAD non-cancer East Asians (n = 9626); and two additional local case cohorts of early-onset or familial breast cancer (n = 290), and lung cancer (n = 209), variants were assessed for pathogenicity in accordance with ACMG/AMP guidelines. In particular, comparisons were made with known pathogenic or likely pathogenic variants in the ClinVar database, pathogenicity predictions were obtained from in silico prediction software, and case–control association analyses were performed. Results: Altogether, we identified 19 pathogenic or likely pathogenic variants from 16 genes, detected in 17 of 55 (31%) patients. Six of the 19 variants were identified using ClinVar, while 13 variants were classified pathogenic or likely pathogenic using ACMG/AMP guidelines. The 16 genes include well-known cancer predisposition genes such as BRCA2, TP53, and RAD51D; but also lesser known cancer genes EXT2, WWOX, GATA2, and GPC3. Most of these genes are involved in DNA damage repair, reaffirming the role of impaired DNA repair mechanisms in the development of multiple malignancies. These variants warrant further investigations in additional populations. Conclusions: We have identified both known and novel variants significantly enriched in patients with primary breast and lung malignancies, expanding the body of known cancer predisposition variants for both breast and lung cancer. These variants are mostly from genes involved in DNA repair, affirming the role of impaired DNA repair in the predisposition and development of multiple cancers. [ABSTRACT FROM AUTHOR]

Surgery or radiation therapy is nearly universally applied for pediatric solid tumors. In many cases, in diverse tumor types, distant metastatic disease is present and evades surgery or radiation. The systemic host response to these local control modalities may lead to a suppression of antitumor immunity, with potential negative impact on the clinical outcomes for patients in this scenario. Emerging evidence suggests that the perioperative immune responses to surgery or radiation can be modulated therapeutically to preserve anti-tumor immunity, with the added benefit of preventing these local control approaches from serving as pro-tumorigenic stimuli. To realize the potential benefit of therapeutic modulation of the systemic response to surgery or radiation on distant disease that evades these modalities, a detailed knowledge of the tumor-specific immunology as well as the immune responses to surgery and radiation is imperative. In this Review we highlight the current understanding of the tumor immune microenvironment for the most common peripheral pediatric solid tumors, the immune responses to surgery and radiation, and current evidence that supports the potential use of immune activating agents in the perioperative window. Finally, we define existing knowledge gaps that limit the current translational potential of modulating perioperative immunity to achieve effective anti-tumor outcomes. [ABSTRACT FROM AUTHOR]

Background: Fertility preservation is an important healthcare focus in the paediatric and adolescent population when gonadotoxic treatments are required. Ovarian stimulation (OS) resulting in oocyte cryopreservation is a well-established fertility preservation option in the adult population. It's utility, however, is little known in young patients. The purpose of this review was to synthesise the available literature on OS in patients =18 years old, to identify gaps in current research and provide suggestions for future research directions. Methods: Using PRISMA guidelines, a systematic review of the literature was performed for all relevant full-text articles published in English in Medline, Embase, the Cochrane Library and Google Scholar databases. The search strategy used a combination of subject headings and generic terms related to the study topic and population. Two reviewers independently screened studies for eligibility, extracted data and assessed the risk of bias. Characteristics of the studies, objectives and key findings were extracted and summarised in a narrative synthesis. Results: Database search and manual review identified 922 studies, 899 were eliminated based on defined exclusion criteria. Twenty-three studies were included and comprised 468 participants aged =18 years who underwent OS (median 15.2, range 7-18 years old). Only three patients were premenarchal, and four patients were on treatment to suppress puberty. Patients had OS for a broad range of indications including oncology treatment, transgender care and Turner syndrome. A total of 488 cycles of OS were completed, with all but 18 of these cycles (96.3%) successfully resulting in cryopreserved mature oocytes (median 10 oocytes, range 0-35). Fifty-three cycles (9.8%) were cancelled. Complications were rare (<1%). One pregnancy was reported from a female who had OS aged 17 years old. Conclusion: This systematic review demonstrates that OS and oocyte cryopreservation is achievable in young females however there are only a few cases in the literature describing OS in premenarcheal children or those who have suppressed puberty. There is little proof that OS can lead to pregnancy in adolescents, and no proof that this can be achieved in premenarchal girls. Therefore it should be regarded as an innovative procedure for adolescents and experimental for premenarcheal girls. [ABSTRACT FROM AUTHOR]

Copyright of Revista Médica de Risaralda is the property of Universidad Tecnologica de Pereira and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi Dergisi is the property of Istanbul Tip Fakultesi Dergisi and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The arrest of neural crest-derived sympathoadrenal neuroblast differentiation contributes to neuroblastoma formation, and overriding this blocked differentiation is a clear strategy for treating high-risk neuroblastoma. A better understanding of neuroblast or neuroblastoma differentiation is essential for developing new therapeutic approaches. It has been proposed that Krueppel-like factor 7 (KLF7) is a neuroblastoma super-enhancer-associated transcription factor gene. Moreover, KLF7 was found to be intensely active in postmitotic neuroblasts of the developing nervous system during embryogenesis. However, the role of KLF7 in the differentiation of neuroblast or neuroblastoma is unknown. Here, we find a strong association between high KLF7 expression and favorable clinical outcomes in neuroblastoma. KLF7 induces differentiation of neuroblastoma cells independently of the retinoic acid (RA) pathway and acts cooperatively with RA to induce neuroblastoma differentiation. KLF7 alters the GTPase activity and multiple differentiation-related genes by binding directly to the promoters of neuroblast differentiation-associated protein (AHNAK and AHNAK2) and glycerophosphodiester phosphodiesterase domain-containing protein 5 (GDPD5) and regulating their expression. Furthermore, we also observe that silencing KLF7 in neuroblastoma cells promotes the adrenergic-to-mesenchymal transition accompanied by changes in enhancer-mediated gene expression. Our results reveal that KLF7 is an inducer of neuroblast or neuroblastoma differentiation with prognostic significance and potential therapeutic value., (© 2024 Federation of European Biochemical Societies.)

Key Points: Surgery in adults with head and neck rhabdomyosarcoma does not improve survival rates. Surgery should be performed deliberately and focused on the timing of combined treatment modality. Adult head and neck rhabdomyosarcoma benefits from salvage surgery following chemoradiotherapy., (© 2023 ARS‐AAOA, LLC.)

Background: Children with chronic IF require long-term home parenteral nutrition (HPN), administered through a central venous catheter. Catheter-related bloodstream infection (CRBSI) with Staphylococcus aureus is known to be a serious infection with a high mortality rate and risk of complications. A standardized protocol on the management of S aureus CRBSIs in children receiving HPN is lacking. The aim of this study is to evaluate the effectiveness and safety of the current management in an HPN expertise center in the Netherlands., Methods: We performed a retrospective descriptive cohort study between 2013 and 2022 on children 0-18 years of age with chronic IF requiring long-term HPN. Our primary outcomes were the incidence of S aureus CRBSI per 1000 catheter days, catheter salvage attempt rate, and successful catheter salvage rate. Our secondary outcomes included complications and mortality., Results: A total of 74 patients (39 male; 53%) were included, covering 327.8 catheter years. Twenty-eight patients (38%) had a total of 52 S aureus CRBSIs, with an incidence rate of 0.4 per 1000 catheter days. The catheter salvage attempt rate was 44% (23/52). The successful catheter salvage rate was 100%. No relapse occurred, and no removal was needed after catheter salvage. All complications that occurred were already present at admission before the decision to remove the catheter or not. No patients died because of an S aureus CRBSI., Conclusion: Catheter salvage in S aureus CRBSIs in children receiving HPN can be attempted after careful consideration by a multidisciplinary team in an HPN expertise center., (© 2024 The Authors. Journal of Parenteral and Enteral Nutrition published by Wiley Periodicals LLC on behalf of American Society for Parenteral and Enteral Nutrition.)

Objectives: This study aimed to establish a nomogram for predicting the probability of testicular salvage after testicular torsion in children., Methods: We retrospectively collected data of children with testicular torsion who were treated at Shenzhen Children's Hospital between September 2005 and August 2022. Of the training cohort, 113 patients who underwent orchiectomy and five with testicular atrophy after orchiopexy were included in the failed testicular salvage group. Additionally, 37 patients who underwent orchiopexy without postoperative testicular atrophy were included in the successful testicular salvage group. The predictive factors affecting testicular salvage were determined using univariate and multivariate logistic regression analyses; a nomogram was constructed. The nomogram was verified using data from the validation group., Results: Using multivariate logistic regression analysis, the independent risk factors of testicular salvage after testicular torsion were symptom duration (p = 0.034), intratesticular blood flow (p = 0.003), spermatic cord torsion degree (p = 0.037), and monocyte count (odds ratio: 0.012, p = 0.036). A nomogram was established based on these four risk factors. In the training cohort, the area under the receiver operating characteristic curve was 0.969. The area under the receiver operating characteristic curve of the verification cohort was 0.965, indicating good discrimination ability of the nomogram. Increased symptom duration without intratesticular blood flow increased the monocyte count and spermatic cord torsion degree and decreased the success rate of testicular salvage., Conclusion: This prediction model could obtain the corresponding probability of testicular salvage according to the clinical characteristics of different patients with testicular torsion, providing reference for clinicians and parents., (© 2024 The Authors. International Journal of Urology published by John Wiley & Sons Australia, Ltd on behalf of The Japanese Urological Association.)

Introduction: Historically, neuroblastoma has been diagnosed by surgical open biopsy (SB). In recent decades, core needle biopsy (CNB) has replaced surgical biopsy due to its safe and adequate method of obtaining tissue diagnosis., Aim: Our study aimed to assess the effectiveness of CNB in obtaining tissue diagnosis for neuroblastoma and evaluate its safety profile in terms of post-operative complications, in comparison to SB., Methods: A retrospective cohort study, including all patients younger than 18 years who were diagnosed with neuroblastoma from 2012 until 2022 in a single tertiary medical center. Patients' demographics, tumor size and location, pathological results, and clinical outcomes were collected., Results: 79 patients were included in our study: 35 biopsies were obtained using image-guided CNB and 44 using SB. Patients' and tumor characteristics including age, gender, tumor volume, and stage were similar in both groups. The biopsy adequacy rate in the CNB group was 91% and 3 patients in this group underwent repeated biopsy. The safety profile in the CNB group was similar to the SB group., Conclusions: CNB is a safe method and should be considered the first choice for obtaining tissue diagnosis when feasible due to its high adequacy in terms of tumor histopathological features., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: Patients with intestinal failure require central venous access which puts them at risk for central line-associated bloodstream infections (CLABSI). Maintaining vascular patency is critical for this population to receive nutrition support. When CLABSIs occur line salvage can help maintain vascular access. The aim of this study is to assess factors associated with safe and successful central venous catheter salvage., Methods: Retrospective cohort study of patients with intestinal failure at two tertiary care institutions between 2012 and 2020. The study examined the rates of attempted salvage, factors associated with successful salvage, and complications associated with salvage attempts., Results: Over the study period, 76 patients with intestinal failure were include while central venous access was in place. There were a total of 94 CLABSIs. Salvage was more likely to be attempted when patients were under the direct care of an intestinal rehabilitation service (95% vs. 68%, p = 0.04). The overall successful salvage rate was 91.6% (n = 77). Gram-positive, Gram-negative, and polymicrobial infections had successful salvage rates of 97%, 92%, and 94% respectively. The successful salvage rate for fungal infections was 40%. There was no difference in 30-day complication rates for hospital readmission, intensive care unit admission, and death between patients who underwent salvage attempt and those who did not., Conclusions: Central line salvage can be safely attempted for many infections in patients with intestinal failure, leading to vascular access preservation., (© 2024 The Authors. Journal of Pediatric Gastroenterology and Nutrition published by Wiley Periodicals LLC on behalf of European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Adhesions following abdominal surgery remain a common cause of bowel obstruction. The incidence is between 1 and 12.6% in children who have had previous abdominal surgery. While conservative management is usually trialled in all patients (including children) suspected of having ASBO, the majority will require surgical intervention. New materials such as Seprafilm ® have been studied in the paediatric population, with promising results of its use in index abdominal surgeries to prevent the formation of adhesions. In this article, we conducted a systematic review to present an overview of the current knowledge on the incidence, aetiology, pathophysiology, clinical presentation, and management of ASBO.

Purpose: To assess the long-term results after Rex bypass (RB) shunt and Rex transposition (RT) shunt and determine the optimal approach., Methods: Between 2010 and 2019, traditional RB shunt was performed in 24 patients, and modified RT shunt was performed in 23 children with extrahepatic portal hypertension (pHTN). A retrospective study was conducted based on comparative symptoms, platelet counts, color Doppler ultrasonography and computed tomographic portography of the portal system, and gastroscopic gastroesophageal varices postoperatively. The portal venous pressure was evaluated intraoperatively., Results: The operation in the RB group was notably more time-consuming than that in the RT group (P < 0.05). Compared to RT shunt, the reduction in gastroesophageal varix grading, the increases in platelets, and the caliber of the bypass were greater in the RB group (P < 0.05). Although not statistically significant, higher morbidity of surgical complications was found after RT shunt (17.4%) compared with RB shunt (8.3%) with patency rates of 82.6 and 91.7%, respectively. Additionally, patients exhibited a lower rate of rebleeding under the RB procedure (12.5%) than under the RT procedure (21.7%)., Conclusions: The RT procedure is an alternative option for the treatment of pediatric extrahepatic pHTN, and RB shunt is the preferred procedure in our center.

Purpose: Today, male and female adult and pediatric cancer patients, individuals transitioning between gender identities, and other individuals facing health extending but fertility limiting treatments can look forward to a fertile future. This is, in part, due to the work of members associated with the Oncofertility Consortium., Methods: The Oncofertility Consortium is an international, interdisciplinary initiative originally designed to explore the urgent unmet need associated with the reproductive future of cancer survivors. As the strategies for fertility management were invented, developed or applied, the individuals for who the program offered hope, similarly expanded. As a community of practice, Consortium participants share information in an open and rapid manner to addresses the complex health care and quality-of-life issues of cancer, transgender and other patients. To ensure that the organization remains contemporary to the needs of the community, the field designed a fully inclusive mechanism for strategic planning and here present the findings of this process., Results: This interprofessional network of medical specialists, scientists, and scholars in the law, medical ethics, religious studies and other disciplines associated with human interventions, explore the relationships between health, disease, survivorship, treatment, gender and reproductive longevity., Conclusion: The goals are to continually integrate the best science in the service of the needs of patients and build a community of care that is ready for the challenges of the field in the future.

Abstract: The reported incidence of pediatric pancreatitis is increasing. Noninvasive imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), play important roles in the diagnosis, staging, follow-up, and management of pancreatitis in children. In this position paper, generated by members of the Pancreas Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the Abdominal Imaging Committee of The Society for Pediatric Radiology (SPR), we review the roles of noninvasive imaging in pediatric acute, acute recurrent, and chronic pancreatitis. We discuss available evidence related to noninvasive imaging, highlighting evidence specific to pediatric populations, and we make joint recommendations for use of noninvasive imaging. Further, we highlight the need for research to define the performance and role of noninvasive imaging in pediatric pancreatitis., Competing Interests: A.T.T. has received funding from Canon Medical systems and in-kind research support from ChiRho Clin, Inc. S.Z.H., owns equity in Prevcon. The other authors report no conflicts of interest., (Copyright © 2020 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: This study aimed to investigate the protective effects of ellagic acid on testicular ischemia/reperfusion injury in rats. Materials and Methods: Twenty-one Sprague-Dawley rats were randomly divided into three groups: sham, ischemia/reperfusion (I/R), I/R + ellagic acid (EA). All animals underwent left scrotal exploration. In all groups except the sham group, the left testes were rotated 720 degrees clockwise for 3 h and 3 h reperfusion. 10 mg/kg ellagic acid was administered intraperitoneally to the I/R+E group before reperfusion. Then, the left orchiectomy was performed. The testes underwent biochemical and histological examination. Results: There was a significant difference between the sham and the I/R, I/R+EA groups according to the Cosentino system (p<0.001, p=0.036), and there was no difference between the I/R and IR+EA groups (p=0.319). A significant difference was found between sham and I/R groups according to Johnsen spermatogenesis score (p<0.001), but there was no significant difference between sham and I/R+EA groups (p=0.063). Superoxide dismutase, catalase, malondialdehyde, total oxidant status values were statistically different between I/R and I/R+EA groups (p=0.001, 0.002, 0.002, 0.001 respectively). Conclusion: Ellagic acid has a protective effect against testicular ischemia/reperfusion injury in rats. [ABSTRACT FROM AUTHOR]

Diagnosis and management of individuals who have differences of sex development (DSD) due to numerical or structural variations of sex chromosomes (NSVSC) remains challenging. Girls who have Turner syndrome (45X) may present with varying phenotypic features, from classical/severe to minor, and some remain undiagnosed. Boys and girls who have 45,X/46,XY chromosomal mosaicism may have Turner syndrome-like features and short stature; therefore, unexplained short stature during childhood requires karyotype analysis in both sexes, particularly if characteristic features or atypical genitalia are present. Many individuals with Klinefelter syndrome (47XXY) remain undiagnosed or are only diagnosed as adults due to fertility problems. Newborn screening by heel prick tests could potentially identify sex chromosome variations but would have ethical and financial implications, and in-depth cost-benefit analyses are needed before nationwide screening can be introduced. Most individuals who have NSVSC have lifelong co-morbidities and healthcare should be holistic, personalized and centralized, with a focus on information, psychosocial support and shared decision-making. Fertility potential should be assessed individually and discussed at an appropriate age. Oocyte or ovarian tissue cryopreservation is possible in some women who have Turner syndrome and live births have been reported following assisted reproductive technology (ART). Testicular sperm cell extraction (TESE) is possible in some men who have 45,X/46,XY mosaicism, but there is no established protocol and no reported fathering of children. Some men with Klinefelter syndrome can now father a child following TESE and ART, with multiple reports of healthy live births. Children who have NSVSC, their parents and DSD team members need to address possibilities and ethical questions relating to potential fertility preservation, with guidelines and international studies still needed. [ABSTRACT FROM AUTHOR]

Introduction: VACTERL is defined as 3 or more of the following congenital defects: vertebral, anorectal, cardiac, tracheoesophageal (TE), renal, and limb. The purpose of this study was to create an easy-to-use assessment tool to help providers counsel expecting families regarding the likelihood of additional anomalies and postnatal outcomes. Methods: Employing the Kids' Inpatient Database from 2003–2016, neonates (<29 days old) with VACTERL were identified using ICD-9-CM and ICD-10-CM codes. For each unique combination of VACTERL, multivariable logistic regression was used to estimate inpatient mortality, and Poisson regression was used to estimate length-of-stay during the initial hospitalization. Results: The assessment tool used in this study is available at https://choc-trauma.shinyapps.io/VACTERL. 1,886 of 11,813,782 (0.016%) neonates presented with VACTERL. 32% weighed <1,750 g, and 239 (12.7%) died prior to discharge. Associated with mortality were limb anomaly (1.8 [1.01–3.22], p < 0.05), prematurity (1.99 [1.14–3.47], p < 0.02), and weight <1,750 g (2.19 [1.25–3.82], p < 0.01). Median length-of-stay was 14 days (IQR: 7-32). Associated with increased length-of-stay were cardiac defect (1.47 [1.37–1.56], p < 0.001), vertebral anomaly (1.1 [1.05–1.14], p < 0.001), TE fistula (1.73 [1.66–1.81], p < 0.001), anorectal malformation (1.12 [1.07–1.16], p < 0.001), and weight <1,750 g (1.65 [1.57–1.73], p < 0.001). Conclusion: This novel assessment tool may help providers counsel families confronting a VACTERL diagnosis. [ABSTRACT FROM AUTHOR]

Copyright of Acta Pediatrica de Mexico is the property of Instituto Nacional de Pediatria (INP) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Nowadays, there is confusing evidence in the literature regarding the association between seasonal variations and events of testicular torsion (TT). We attempted to assess the correlation between seasonal variations, including season, ambient temperatures, and humidity levels to onset and laterality of testicular torsion. We conducted a retrospective review of patients diagnosed with testicular torsion and confirmed surgically between January 2009 and December 2019, at Hillel Yaffe Medical Center. Weather data was collected from meteorological observation stations near the hospital. TT incidents were stratified into five temperature categories (< 15 °C, 15–20 °C, 20–25 °C, 25–30 °C, > 30 °C) and into two categories of humidity (≤ 50%, > 50%). Potential associations between TT and seasonal variations were investigated. Of 235 patients diagnosed with TT, 156 (66%) were children and adolescents and 79 (34%) were adults. In both groups, rate of TT incidents increased in winter and fall months. Significant correlation between TT and temperatures below 15 °C was observed in both groups; OR 3.3 [95% IC 1.54–7.07], p = 0.002 in children and adolescents and 3.77 [1.79–7.94], p < 0.001) in adults. The correlation between TT and humidity was non-significant in both groups. Among children and adolescents left-sided TT was observed in most of the cases, with strong correlation to lower temperatures; OR 3.15 [1.34–7.40], p = 0.008. Higher rates of acute TT were observed in patients admitted to the emergency department (ED) during the cold seasons in Israel. Significant association was observed between left-side TT and temperatures below 15 °C in the children and adolescents' group. Our findings suggest that there might be a predilection for TT occurrence in cold weather, particularly left-side laterality among children and adolescents. [ABSTRACT FROM AUTHOR]

To evaluate the association of clinician demographics and practice patterns with delayed diagnosis of appendicitis. We included children with appendicitis at 13 regional emergency departments (EDs). We screened patients with a previous ED visit within 7 days for delayed diagnosis by chart review. We evaluated the association of clinician characteristics using logistic regression with random intercepts for site and clinician and delay as the outcome. Among 7,452 children with appendicitis, 105 (1.4%) had delayed diagnosis. Clinicians in the lowest quartile of obtaining blood in their general practice were more likely to have delayed diagnosis (odds ratio 4.9 compared to highest quartile, 95% confidence interval 1.8, 13.8). Clinicians' imaging rates, specialty, sex, and experience were not associated with delayed diagnosis. Clinicians who used more blood tests in their general practice had a lower risk of delayed diagnosis of appendicitis, possible evidence that lower risk tolerance has benefits. [ABSTRACT FROM AUTHOR]

Background: Adhesive small bowel obstruction is a significant cause of postoperative morbidity in children. There are limited studies on the epidemiology and scope of conservative management for adhesive small bowel obstruction in children. The purpose of this study was to determine the presentation, risk factors, and management, including the role of Gastrografin, for adhesive small bowel obstruction in children. Methods: Between June 2015 and June 2020, 90 patients with an adhesive small bowel obstruction presented to our pediatric surgery department. Out of these, 10 patients underwent upfront surgery and were excluded, while the rest were put on conservative treatment. Patients who failed to improve after 24 hours of conservative management in the absence of signs of strangulation were administered Gastrografin. Patients were evaluated clinically and radiologically to determine the resolution of the obstruction. Results: The mean age was 9.04 ± 3.89 with sex a ratio of 2.6:1. Vomiting was the most symptom (90% cases), followed by pain abdomen (87.5%). Appendectomy (50%) and enterotomy for worm obstruction (17.5%) were the most common previous surgical procedures leading to acute small bowel obstruction. Recurrent obstruction was seen in 7.5% of the cases. Thirty eight (47.5%) patients were successfully managed conservatively by standard decompression therapy. Oral administration of Gastrografin successfully resolved the obstruction in 43% patients who failed standard decompression therapy, whereas 24 (30%) patients with persistent obstruction required laparotomy. Conclusion: Adhesive small bowel obstruction is a common surgical emergency in children. It can occur at any age and after laparotomy for any surgical condition; however, history of appendectomy is associated with high incidence of adhesion obstruction. Vomiting and abdominal pain are constant symptoms. Conservative management with standard decompressive therapy followed by using water-soluble contrast agents (Gastrografin) is a safe and effective approach in managing adhesion obstruction in selected children without signs of bowel ischemia. [ABSTRACT FROM AUTHOR]

Background: Total colonic aganglionosis is an extremely rare variant of Hirschsprung's disease, which is predominant in males and can be seen in 1:50,000 live births. The presented case not only depicts a rare case, but also unusual clinical, laboratory, and instrumental data. Case presentation: A 2-day-old Caucasian female newborn was transferred to our hospital from maternity. The initial presentation was reverse peristalsis, abdominal distention, and inability to pass stool. Fever had started before the patient was transferred. Hirschsprung's disease was suspected, and tests such as contrast enema and rectal suction biopsy were done. Before enterostomy, the management of the disease included fluid resuscitation, colonic irrigation, antibiotic administration, enteral feeding, and supportive therapy. During ileostomy operation, no transition zone was visualized and full-thickness biopsy samples were retrieved from the rectum and descending colon. After surgical intervention, status significantly improved—defervescence and weight gain most importantly improved. Conclusion: It is well known that diagnosis of total colonic aganglionosis may be delayed for months or even years since the transition zone may not be visible and rectal suction biopsy, unlike full-thickness biopsy, is not always reliable. It might be more prudent not to be derailed because of negative radiography and rectal suction biopsy. Also, doctors should be more suspicious of the disease if signs and symptoms are starting to be consistent with Hirschsprung-associated enterocolitis, despite biopsy and radiology results. [ABSTRACT FROM AUTHOR]