Abstract
Introduction
This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the ...neonatal intensive care units (NICU) in Italy.
Methods
A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units.
Results
The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern <Northern<Central,
p
< 0.03). The most frequent clinical characteristics of neonates was preterm neonates with birthweight < 1200 g, with cardiorespiratory instability and/or ventilatory dependence. The most frequently selected indications to surgery were pneumothorax, pleural effusion, pericardial effusion, central venous catheter (CVC) positioning, intestinal perforation, patent ductus arteriosus ligation and congenital diaphragmatic hernia. More than 60% of respondents report no institutional recommendations and dedicated informed consent on bedside surgical procedures. The lack of dedicated areas and infrastructures is considered a relative contraindication to the performance of bedside surgery.
Conclusion
Bedside surgery is performed in the majority of the Italian pediatric surgery centers included in this census. The introduction of a national set of surgery guidelines would be widely welcomed.
Hirschsprung disease (HSCR) is a rare congenital anomaly characterized by the absence of enteric ganglia in the distal intestinal tract. While classified as a multigenic disorder, the altered ...function of the RET tyrosine kinase receptor is responsible for the majority of the pathogenesis of HSCR. Recent evidence demonstrate a strong association between RET and the homeostasis of immune system. Here, we utilize a unique cohort of fifty HSCR patients to fully characterize the expression of RET receptor on both innate (monocytes and Natural Killer lymphocytes) and adaptive (B and T lymphocytes) human peripheral blood mononuclear cells (PBMCs) and to explore the role of RET signaling in the immune system. We show that the increased expression of RET receptor on immune cell subsets from HSCR individuals correlates with the presence of loss-of-function RET mutations. Moreover, we demonstrate that the engagement of RET on PBMCs induces the modulation of several inflammatory genes. In particular, RET stimulation with glial-cell line derived neurotrophic factor family (GDNF) and glycosyl-phosphatidylinositol membrane anchored co-receptor α1 (GFRα1) trigger the up-modulation of genes encoding either for chemokines (CCL20, CCL2, CCL3, CCL4, CCL7, CXCL1) and cytokines (IL-1β, IL-6 and IL-8) and the down-regulation of chemokine/cytokine receptors (CCR2 and IL8-Rα). Although at different levels, the modulation of these "RET-dependent genes" occurs in both healthy donors and HSCR patients. We also describe another set of genes that, independently from RET stimulation, are differently regulated in healthy donors versus HSCR patients. Among these "RET-independent genes", there are CSF-1R, IL1-R1, IL1-R2 and TGFβ-1, whose levels of transcripts were lower in HSCR patients compared to healthy donors, thus suggesting aberrancies of inflammatory responses at mucosal level. Overall our results demonstrate that immune system actively participates in the physiopathology of HSCR disease by modulating inflammatory programs that are either dependent or independent from RET signaling.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Gastric volvulus is a significant, rare cause of non-bilious vomiting and consists of a pathological rotation of the stomach of more than 180° around the axis without obstruction of the ...gastrointestinal tract. A definitive diagnosis is made with upper radiological gastrointestinal studies. Treatment may be conservative or surgical with anterior and fundal gastropexy in patients with ingravescent symptoms. We describe the case of a 16-month-old female admitted to our hospital for recurrent and postprandial vomiting episodes which had started at 11 months of age. A history of gastroesophageal reflux was present until 1 year of age, in association with recurrent respiratory infections. The basic metabolic panel was normal. Barium study showed stomach rotation along a horizontal plane stomach. Esophagogastroduodenoscopy showed no mucosal alterations. The diagnosis was chronic organoaxial gastric volvulus. In our patient, the surgical procedure of gastropexy, both anterior and fundal, without fundoplication was performed. She showed good improvement after surgery, with resolution of symptoms and weight gain.
Marta Rusmini: Laboratory of Molecular Genetics, IRCCS, Giannina Gaslini Istitute, Genoa, Italy, Unit of Clinical and Experimental Immunology, Humanitas Clinical and Research Center, Rozzano, Milan, ...Italy Ivana Matera: Laboratory of Molecular Genetics, IRCCS, Giannina Gaslini Istitute, Genoa, Italy, Unit of Clinical and Experimental Immunology, Humanitas Clinical and Research Center, Rozzano, Milan, Italy Roberto Ravazzolo: Laboratory of Molecular Genetics, IRCCS, Giannina Gaslini Istitute, Genoa, Italy, DINOGMI Department, University of Genova, Genova, Italy Citation: Rusmini M, Griseri P, Lantieri F, Matera I, Hudspeth KL, Roberto A, et al. (2013) Correction: Induction of RET Dependent and Independent Pro-Inflammatory Programs in Human Peripheral Blood Mononuclear Cells from Hirschsprung Patients.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Mesenchymal stem cells (MSCs) may be of value in regeneration of renal tissue after damage; however, lack of biological knowledge and variability of results in animal models limit their utilization. ...We studied the effects of MSCs on podocytes in vitro and in vivo utilizing adriamycin (ADR) as a model of renal toxicity. The in vivo experimental approach was carried out in male Sprague-Dawley rats (overall 60 animals) treated with different ADR schemes to induce acute and chronic nephrosis. MSCs were given a) concomitantly to ADR in tail vein or b) in aorta and c) in tail vein 60 days after ADR. Homing was assessed with PKH26-MSCs. MSCs rescued podocytes from apoptosis induced by ADR in vitro. The maximal effect (80% rescue) was obtained with MSCs/podocytes coculture ratio of 1:1 for 72 h. All rats treated with ADR developed nephrosis. MSCs did not modify the clinical parameters (i.e., proteinuria, serum creatinine, lipids) but protected the kidney from severe glomerulosclerosis when given concomitantly to ADR. Rats given MSCs 60 days after ADR developed the same severe renal damage. Only a few MSCs were found in renal tubule-interstitial areas 1–24 h after injection and no MSCs were detected in glomeruli. MSCs reduced apoptosis of podocytes treated with ADR in vitro. Early and repeated MSCs infusion blunted glomerular damage in chronic ADR-induced nephropathy. MSCs did not modify proteinuria and progression to renal failure, which implies lack of regenerative potential in this model.
Abstract Introduction Neuroblastoma in the adolescent is characterized by indolent growth and poor outcome. Surgical resection of the tumor is an essential part of the multimodality treatment. ...Surgical complications depend on the presence of Image Defined Risk Factors (IDRFs). Methods We present an adolescent with pelvic neuroblastoma and epidural compression. To facilitate tumor resection, the patient underwent preoperative selective embolization. Results After selective embolization a subsequent complete resection etraspinal localisation was performed without complication with complete remission after 2 years. Conclusions Preoperative embolization is a safe and feasible technique that can help pediatric surgical oncologist to reduce complications IDRFs-related.
Bar dislocation has always been considered a fearsome complication of Minimally Invasive Repair of Pectus Excavatum (MIRPE), therefore multiple techniques and types of stabilization have been ...introduced. The aim of the study is to compare different stabilization techniques in a cohort of patients operated by the same first operator.
MIRPE was adopted at our institution in 2005. Data on MIRPE patients from January 2013 to December 2022 were collected prospectively and reviewed. Patients with a follow-up of at least 12 months were included. Throughout the years different ways of stabilization were used. Patients were divided in 3 groups according to the stabilization strategy adopted- Group A: no stabilizer; Group B: single bar fixation; Group C: bridge fixation. Dislocation was diagnosed if a bar rotated more than 30° or displaced laterally for more than 1.5 cm. We compared bar dislocation percentage of each group.
We positioned 733 bars in 468 patients. Group A included 113 bars (15.4%), Group B 415 bars (56.6%), Group C 205 bars (28%). No patients were lost at follow-up. Total dislocation rate was 4.1% (30 bars). Dislocation was observed in 10 bars of group A (8.8%), 20 bars of group B (4.8 %), 0 bars of group C (0%). Differences between groups were statistically significant.
The use of stabilizers reduced dislocation percentage. In particular, bridge fixation technique reduced to zero bar dislocation and is now our preferred technique of stabilization.
III.
•What is currently known about this topic?
Bar displacement is a common and fearsome complication after MIRPE, potentially hazardous and often requiring additional intervention. Different stabilization techniques have been described over the years.•What new information is contained in this article?
Comparing three different techniques in a large series of patients, we found bridge stabilization as the optimal technique for preventing bar dislocation.
Bicycle riding has multiple beneficial cardiovascular effects; however, it is a well-documented source of significant urologic injuries. Priapism is a rare condition in children, and occurs primarily ...because of congenital hematologic diseases or adverse drug reactions. A pediatric clinical case and literature review of a high-flow priapism secondary to cycling trauma is described here to highlight their etiopathologic correlation. Bicycle riding trauma is a rare but possible cause of high-flow priapism in children, and a high index of suspicion should ensure appropriate management.
This paper describes multiple intraluminal impedance (MII) in 50 children with typical and atypical gastroesophageal reflux (GER) symptoms and discusses the possible clinical significance of ...objective numeric data provided by MII computed analysis. Patients underwent 24-hr pH/MII monitoring. Reflux parameters were analyzed with relation to age and reported symptoms. Nonacidic MII events occurred as frequently as acidic ones. A Pathologic Bolus Exposure Index associated with a normal pH Reflux Index was detected in 26% of our series. Significant correlations were found regarding acid and bolus clearing times and their ratio. We conclude that the low rate of symptom occurrence in the pediatric population represents a limit on MII evaluation. Our study confirmed that nonacid GER is at least as frequent as acid GER. As MII provides interesting objective data that could be used in clinical practice, we suggest further research to define normal ranges in the pediatric population.
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