Esophagogastric dissociation reduces the re-operation rate for persistent gastroesophageal reflux in severely neurologically impaired children

Purpose: In this study we want to demonstrate the effectiveness of the esophagogastric dissociation as a first level operation in treatment of the gastroesophageal reflux in severe neurologically impaired children, in term of a reduction of reoperation rate.Methods: We divided patients operated from 1998 to 2005 in a group A, composed by children treated with fundoplication, and in a group AR, composed by the patients of group A who had a recurrence of reflux and that was treated with esophagogastric dissociation. Patients operated from 2005 to 2013 were selected on the basis of the severity of the neurological impairment and were divided in a group B, treated with fundoplication, and in a group C of more severe impaired children, treated with esophagogastric dissociation. Data regarding the complications of the A and C groups were analyzed with Fisher’s test.Results: We evaluated 63 patients: 34 (54 %) in group A, 11 in group AR, 15 (23.6 %) in group B, 14 (22.4 %) in group C. The Fisher’s test showed a non significant difference with a p value of 0.2.Conclusion: Despite of statistic result we believe that TOGD is a useful procedure as the first choice of surgical management in severe neurological impaired children affected by gastroesophageal reflux

Cervical Lymphadenitis by Mycobacterium triplex in an Immunocompetent Child: Case Report and Review

Mycobacterium triplex was first described in 1996. This nontuberculous Mycobacterium causes a severe pulmonary disease in immunocompromised patients but it can involve also healthy patients. A literature search was made on the PubMed database and it produced only few cases of children with cervical lymphadenitis due to this Mycobacterium Triplex. We are describing a case of M. triplex cervical lymphadenitis in an immunocompetent child

Herlyn–Werner–Wunderlich syndrome: An “early” onset case report and review of Literature

AbstractHerlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn–Werner–Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3–8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9–14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis

HYPERPLASIA OF THYMIC GLAND: LEFT VIDEO-ASSISTED THORACOSCOPIC APPROACH

Hyperplasia of thymic gland is a rare benign entity that should be considered in the differential diagnosis of anterior mediastinal masses in children and young adolescents. We report a case of a patient with a thymic mass, diagnosed occasionally for respiratory symptoms and treated by video-assisted thoracoscopic surgery. A previously healthy 10 years-old boy presented to our hospital for retrosternal pain and dyspnea with restriction to daily activities from four months. Diagnostic imaging was performed, including a chest x-ray and a magnetic resonance imaging, showing a large homogeneous anterosuperior mediastinal mass, more extended on the left side. The additional laboratory analysis, considered essential for differential diagnosis with myasthenia gravis and lymphoma, resulted negative. In view of these findings, our patient underwent to video assisted thoracoscopy with left-sided approach for a total resection of thymus and perithymic fat. The patient made an excellent recovery without postoperative complications and was discharged from the hospital four days later. Histopathological examination showed a normal thymic architecture like a true thymic hyperplasia. At follow up, chest x-ray was normal in absence of pleural and parenchimal alterations. Thoracoscopic thymectomy is a safe technique that allows to achieve the goal of early thymectomy with the advantages of less invasive procedure

Discovery of a Novel Small Molecule Inhibitor Targeting the Frataxin/Ubiquitin Interaction via Structure-Based Virtual Screening and Bioassays

Friedreich ataxia (FRDA) is an autosomal recessive neuro- and cardiodegenerative disorder for which there are no proven effective treatments. FRDA is caused by decreased expression and/or function of the mitochondrial protein frataxin. Here, we report findings that frataxin is degraded via the ubiquitin-proteasomal pathway and that it is ubiquitinated at residue K147 in Calu-6 cells. A theoretical model of the frataxin-K147/Ub complex, constructed by combining bioinformatics interface predictions with information-driven docking, revealed a hitherto unnoticed, potential ubiquitin-binding domain in frataxin. Through structure-based virtual screening and cell-based assays, we discovered a novel small molecule (compound (+)-11) able to prevent frataxin ubiquitination and degradation. (+)-11 was synthesized and tested for specific binding to frataxin by an UF-LC/MS based ligand-binding assay. Follow-up scaffold-based searches resulted in the identification of a lead series with micromolar activity in disrupting the frataxin/Ub interaction. This study also suggests that frataxin could be a potential target for FRDA drug development

Preoperative distraction in children: hand-held videogames vs clown therapy.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish children's emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the child's hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the child's overall life quality

Caudal 'duplication' or 'split' syndrome: Is there a misnomer?

Abstract'Caudal duplication syndrome' was coined to describe the apparent duplication of organs derived from the hindgut, the neural tube and the adjacent mesoderm. Review of the anatomy suggests that the word 'duplication' may be a misnomer. This paper describes the management of 2 girls with caudal duplication syndrome who underwent multistage reconstructive surgery. Both had a large omphalocele and a severe diastasis of the pubic symphysis. The first patient also had an apparent duplication of the vulva, the perineum and the anus to either side of a wide midline. Each vulva contained a urethra, a hemi-clitoris with ipsilateral labium minor, and a hemi-vagina with hemi-uterus. The second child had an infrapubic sequestrated appendico-cecal duplication lying between two hemi-bladders each with ipsilateral ureter and urethra. The everted duplication split the single vulva longitudinally in the midline as far as the fourchette. To each side were a hemi-clitoris, and a hemi-vagina with hemi-uterus and ipsilateral fallopian tube. Analysis of our patients' anatomy and a literature review indicates for the most part 'hemi' organs on either side and suggests that the term 'duplication' is a misnomer such that caudal 'split' syndrome may be a more appropriate title

Synthesis and functionalization of casein nanoparticles with aptamers for triple-negative breast cancer targeting

This work shows the synthesis of a drug delivery system made of casein nanoparticles able to host hydrophobic molecules and be functionalized with aptamers targeting the epidermal growth factor receptor. In vitro cell viability and uptake analyses, performed on triple-negative breast cancer cells, confirmed the safety profile and the target selectivity

THORACOSCOPIC TREATMENT OF DIAPHRAMMATIC HERNIA: A CASE REPORT

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RENAL DUPLEX SYSTEM IN PEDIATRIC POPULATION: MANAGEMENT AND LONG-TERM FOLLOW-UP

Background. Duplex system is a duplication of renal parenchyma, pelvis and collecting system. It could be complete, if ureters lead to bladder separately or incomplete, if they joint before coming out. This study aims duplex system management evaluation, defining indications of conservative or demolitive therapy, and results. Materials and methods. At the section of pediatric surgery of University of Siena we have observed 27 patients with duplex system from January 1980 to May 2011: 7 male (26%) and 20 female (74%), 18 (67%) with complete duplicity, 9 (33%) with incomplete one. Patients were divided into 2 groups: the first one was composed by 12 children (44%), they had negative diagnostic exams for alterations of renal function and associated diseases and no symptoms;the second group had 15 children (56%) whose diagnostic-therapeutic iter was based on associated malformations and symptoms of each case. We found: 5 RVU (33%) with 1 Hutch diverticulum; 5 ureteroceles (33%); 3 ectopic ureters (20%); 4 megaureters (26%), 6 renal dysplasia and upper pole function <10% (40%). Results. Children belonging to second group were treated in different ways. 5 babies (33%) with RVU were approached with submeatal infiltration; 1 baby (7%) with ureterocele was treated with excision of the malformation and Cohen reimplatation; 2 ureteroceles (13%) were incised by transurethral approach and RVU appeared, 1 of them was then treated with eminephroureterectomy; in 1 case of ureterocele (7%)and 1 of ectopic ureter (7%) no treatment was undertaken and 1 baby with ureterocele (7%) needed eminephoureterectomy; 4 kidneys (26%) with upper pole impaired function required eminephroureterectomy. All second group patients, except 3, had a 2-16 years follow-up and they showed normal growth and no symptoms. First group children had negative exams and excellent clinic conditions. Conclusions. Our results suggest that management should be decided on patient’s age, clinic presentation and associated diseases