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Congenital anomalies are important causes of morbidity and mortality in children. Oxidative stress (OS) is involved in the physiopathology of pregnancy-related congenital malformations. This review summarizes the role of OS in the pathogenesis of congenital malformations; in particular, its purpose is to describe how OS influences the development of heart congenital malformations, oesophageal atresia, biliary atresia, diaphragmatic hernia, and autosomal dominant polycystic kidney disease.Systematic review of previous studies about the role of OS in pregnancy and its possible effects in developing of congenital malformations. One electronic database (PubMed) was searched and reference lists were checked.An imbalance between the production of reactive oxygen species (ROS) and antioxidant defense can occur early in pregnancy and continue in the postnatal life, producing OS. It may destroy the signaling pathways needed for a correct embryogenesis leading to birth defects. In fact, cell functions, especially during embryogenesis, needs specific signaling pathways to regulate the development. These pathways are sensitive to both endogenous and exogenous factors; therefore, they can produce structural alterations of the developing fetus.Because OS plays a significant role in pathogenesis of congenital malformations, studies should be developed in order to better define their OS mechanisms and the beneficial effects of supplemental therapeutic strategies.· Oxidative stress is involved in the pathogenesis of congenital malformations.. · Heart malformations, oesophageal atresia, biliary atresia, diaphragmatic hernia, and autosomal dominant polycystic kidney are analyzed.. · A knowledge of pathomechanism of OS-related congenital malformations could be useful to prevent them..

Acute scrotal pain is one of the most frequent symptoms in pediatric patients visited in the Emergency Department. Ultrasonography with color and power Doppler represents the first-line method that clinicians use to carry out the differential diagnosis between spermatic cord torsion and inflammation, but sensitivity and specificity are 63-100% and 97-100%, respectively; this variability may be related to operator's experience and testis vascular hemodynamics and also to machine performance and patient age. Recent technological innovations have made possible to create a new Doppler mode called ultrasound microvascular imaging. This technique exploits algorithms capable of separating low frequencies of static tissue artifacts from ones of very weak flows. It is known as MicroV (from Esaote) and Superb microvascular imaging (from Toshiba). It provides both macrocirculation vascular maps, as a typical Doppler feature, and microcirculation vascular maps. Furthermore, the use of background subtraction could improve the visibility of small vascular structures. We report a case of a pediatric patient suffering from acute scrotal pain assessed ultrasonographically with this innovative Doppler technique (MicroV) that may give more confidence in detecting testicular vascular signals if compared with traditional Doppler techniques.

The proto-oncogene MYCN is considered a transcription factor involved in the regulation of neuroblastoma (NB) cell biology. Since minimally invasive-surgery represents a debated treatment of NB, we investigated COSH-SY5Y and IMR-32 were exposed to COCOOur results suggest that CO

Based on the American (Bethesda, 2017) or Italian (SIAPEC 2014) cytological categories of thyroid nodules, the risk of malignancy and management vary. This risk is 5–10% or80% in females with hypoechoic nodules. For the malignant category, rate was always 100%. In conclusion, particular groups of nodules (based on gender, echotexture, and ICLT) within the cytologically benign through the suspiciously malignant category are at risk of malignancy substantially greater (even 100%) than the standard one. Accordingly, the suggested management cannot be standardized.

To systematically review literature and to assess the status of the ERAS protocol in pediatric populations undergoing gastrointestinal surgery. Literature research was carried out for papers comparing ERAS and traditional protocol in children undergoing gastrointestinal surgery. Data on complications, hospital readmission, length of hospital stay, intraoperative fluid volume, post-operative opioid usage, time to defecation, regular diet, intravenous fluid stop, and costs were collected and analyzed. Analyses were performed using OR and CI 95%. A p value

Background Transferring results obtained in cardiovascular outcome trials (CVOTs) to the real-world setting is challenging. We herein transposed CVOT results to the population of patients with type 2 diabetes (T2D) seen in routine clinical practice and who may receive the medications tested in CVOTs. Methods We implemented the post-stratification approach based on aggregate data of CVOTs and individual data of a target population of diabetic outpatients. We used stratum-specific estimates available from CVOTs to calculate expected effect size for the target population by weighting the average of the stratum-specific treatment effects according to proportions of a given characteristic in the target population. Data are presented as hazard ratio (HR) and 95% confidence intervals. Results Compared to the target population (n = 139,708), the CVOT population (n = 95,816) was younger and had a two to threefold greater prevalence of cardiovascular disease. EMPA-REG was the CVOT with the largest variety of details on stratum-specific effects, followed by TECOS, whereas DECLARE and PIONEER-6 had more limited stratum-specific information. The post-stratification HR estimate for 3 point major adverse cardiovascular event (MACE) based on EMPA-REG was 0.88 (0.74–1.03) in the target population, compared to 0.86 (0.74–0.99) in the trial. The HR estimate based on LEADER was 0.88 (0.77–0.99) in the target population compared to 0.87 (0.78–0.97) in the trial. Consistent results were obtained for SUSTAIN-6, EXSCEL, PIONEER-6 and DECLARE. The effect of DPP-4 inhibitors observed in CVOTs remained neutral in the target population. Conclusions Based on CVOT stratum-specific effects, cardiovascular protective actions of glucose lowering medications tested in CVOTs are transferrable to a much different real-world population of patients with T2D.

Total proctocolectomy with ileal J-pouch-anorectal anastomosis (IPAA) remains the preferred surgical treatment for ulcerative colitis (UC) in children. Considering the well-known advantages of minimally invasive approach, and its main application for the deep pelvis, robotic surgery may be used in UC reconstructive procedures. The aim of the study is to report our experience with Robotic IPAA in children. Single surgeon experience on Robotic IPAA were prospectively included. Data on patient demographics, surgical details, complications, and length of stay (LOS), were collected. Fifteen patients were included. Median age was 13.2 years, median body weight 45 kg. Median operative time was 240 min. Median LOS was 7 days and mean follow-up time 1 year. No intraoperative complication occurred. Five postoperative complications happened: 3 minors treated conservatively (CD I–II), 2 majors needing reintervention under anesthesia (CD IIIb). No mortality was observed. Our preliminary experience reveals that Robotic IPAA is a safe and feasible option for the surgical treatment of UC in children. A bigger patient sample and a long-term follow-up are needed to confirm our findings.

The aim was to retrospectively evaluate our experience in a large series of patients affected by isolated congenital ventral penile curvature, surgically treated using a previously described modified incisional corporoplasty. Two hundred and six patients with isolated congenital ventral penile curvature underwent a modified incisional corporoplasty. Mean age at surgery was 20.7 ± 5.5 years, and degree of ventral curvature was 60 ± 23°. After the point of maximum convexity identification, Buck's fascia was vertically opened along the deep dorsal vein, which was partially ligated, resected and removed. Tunica albuginea was then longitudinally incised and transversally closed. Post-operative follow-up examination was performed at 2 weeks, 6, 12 and 24 months and then annually. Surgical time was 79 ± 12 min. At follow-up, 189 out of 198 patients (95%) were completely satisfied, four patients (2%) complained a recurrence of penile curvature, and 6 (3%) complained about shortening of the penis. None of the patients had any interference with sexual activity. One patient (0.5%) showed erectile dysfunction 5 years after surgery, but there was no organic dysfunction during examination. The proposed technique allows correction of ventral congenital penile curvature without dorsal neurovascular bundle manipulation, resulting in minimum trauma of the erectile tissue, without injury to nerve fibres.

Goblet cell adenocarcinoma (ex-goblet cell appendiceal carcinoid) is a very unusual malignant neoplasm. We report herein a case of a 16-year-old female showing symptoms related to recurrent pleural effusions and abdominal masses. The present case suggests that the cytological approach reveals an intriguing cellular population composed by neoplastic small elements with scanty to moderate amount of cytoplasm with pseudo-glandular pattern intermingled with a second population represented by signet ring cells. The immunohistochemical analysis supports the final diagnosis of metastatic goblet cell adenocarcinoma (ex goblet cell carcinoid).

Background The aim of this study is to investigate the use of methylene blue in perioperative identification of the patent processus vaginalis in a group of boys presenting with congenital or recurrent hydrocele where surgery was performed by junior surgeons in training. Methods We retrospectively reviewed the notes of 22 boys with hydrocele, of which two recurrences, who were operated via a standard inguinal approach, by trainees. Methylene blue 0.3-0.5 mL was injected into the hydrocele fluid through the scrotal wall. A processus vaginalis was identified as a blue line. Results Methylene blue injection clearly identified a patent processus vaginalis in 91% of patients. In 9% (N.=2), of which one recurrence, methylene blue injection demonstrated a hydrocele with an obliterated processus vaginalis. There were no intraoperative complications. No testicular atrophy was recorded. Conclusions Injection of methylene blue into the hydrocele sac may be considered a useful aid for a clearer identification of a difficult patent processus vaginalis. In the present series, there were no complications, and thus we believe that this technique might be suitable and especially helpful, in cases of recurrent hydrocele, and for junior surgeons in training.

Objectives: The study aimed to investigate the expression of the integrin isoforms α7A and β1A, expressed by myogenic precursor cells, and α7B and β1D, expressed by mature muscle cells in the cremaster of patients affected by an undescended testis. Methods: Fifteen samples of cremaster were obtained from patients undergoing surgery for an undescended testis. Thirty control specimens of cremaster were harvested from patients with congenital hydrocele or inguinal hernia. Immunofluorescent analysis was carried out using anti-α7A, β1A, α7B, and β1D integrin antibodies. Sections were observed using confocal laser scanning microscopy. Results: As compared with controls, a significant loss of a α7B (p = 0.0355) and β1D (p = 0.0069) integrins and a higher expression of α7A (p = 0.0003) and β1A (p = 0.0150) was detected in the cremaster of patients affected by an undescended testis. Conclusions: Our data document a critical alteration of the cytoskeleton of cremasteric smooth muscle cells in patients with an undescended testis. This might explain the altered function in smooth muscle cells in cremaster implied during testicular descent. We therefore speculate that the postnatal splicing of α7A to α7B and of β1A to β1D integrins is delayed. This could account for the common clinical scenario of spontaneous descent of the testes in the first months of life.

Background The aim of this study was to investigate the prevalence of primary monosymptomatic and non-monosymptomatic nocturnal enuresis (PMNE and PNMNE) and associated factors in a major referral center for NE in Abu Dhabi. Methods Children referred to the Paediatric Continence Clinic, between 2014 and 2016, for PNE were included in the study. Exclusion criteria were neuropathic bladder, abnormality of the bladder and bowel, urethral stenosis, neurological and psychiatric problems, non-completion of the diagnostic protocol, and follow up

Transglutaminase 2 (TG2) is a multifunctional enzyme and two isoforms, TG2-L and TG2-S, exerting opposite effects in the regulation of cell death and survival, have been revealed in cancer tissues. Notably, in cancer cells a hypoxic environment may stimulate tumor growth, invasion and metastasis. Here we aimed to characterize the role of TG2 isoforms in neuroblastoma cell fate under hypoxic conditions. The mRNA levels of TG2 isoforms, hypoxia-inducible factor (HIF)-1&alpha, p16, cyclin D1 and B1, as well as markers of cell proliferation/death, DNA damage, and cell cycle were examined in SH-SY5Y (non-MYCN-amplified) and IMR-32 (MYCN-amplified) neuroblastoma cells in hypoxia/reoxygenation conditions. The exposure to hypoxia induced the up-regulation of HIF-1&alpha, in both cell lines. Hypoxic conditions caused the up-regulation of TG2-S and the reduction of cell viability/proliferation associated with DNA damage in SH-SY5Y cells, while in IMR-32 did not produce DNA damage, and increased the levels of both TG2 isoforms and proliferation markers. Different cell response to hypoxia can be mediated by TG2 isoforms in function of MYCN amplification status. A better understanding of the role of TG2 isoforms in neuroblastoma may open new venues in a diagnostic and therapeutic perspective.

Background: Lichen sclerosus (LS) is a disease of the skin of unclear etiology that can occur in the foreskin. Topical therapy with corticosteroids is recommended, but they can have side effects. Objectives: We aimed to compare the effects of ozonides with vitamin E acetate (OZOILE) versus topical corticosteroid in children undergoing circumcision. Method: Twenty children undergoing circumcision were treated before surgery: 10 children with OZOILE cream and 10 with 0.1% mometasone furoate once a day for 7 days. Ten age-matched patients with LS of the foreskin without any treatment were recruited as controls. Transcript levels of proinflammatory and anti-inflammatory cytokines and e-cadherin were evaluated in removed foreskins by qRT-PCR. Results: OZOILE and steroid topical treatment produced a similar reduction of TNF-α and IL-1β mRNA levels in foreskins from patients with LS when compared to untreated patients (p < 0.001). OZOILE and steroid treatment caused an increase in the transcript levels of IL-13 and e-cadherin in the foreskin of patients affected by LS in comparison to untreated foreskin (p < 0.001). Conclusions: On the basis of our biochemical data, a randomized clinical trial might be useful to verify the actual clinical effect of OZOILE as alternative treatment to corticosteroids in children affected by LS of the foreskin.

Respiratory distress is a common presenting feature among newborns. Thorough clinical assessment and prompt investigation are required to ensure correct treatment and improve outcomes. Pneumothorax (PTX) is the most common of the air-leak disorders in neonates and is more frequently observed in low birth weight infants. Although it is one of the few treatable causes of respiratory distress in neonatal age, it is life-threatening and is associated with an increased risk of mortality and morbidity. Incidence increases in patients with concurrent lung disease or requiring mechanical ventilation. Furthermore, neonatal PTX can develop due to high transpulmonary pressure, generated at the onset of respiration. Chylothorax is rarer, but it represents the most frequent cause of pleural effusion in the neonatal period. Clinical features depend on the amount of chyle collected in the pleural space, ranging from asymptomatic to life-threatening conditions, requiring urgent treatment. Prognosis is much better when chylothorax is diagnosed after birth than when discovered in utero, because during fetal life pleural effusion can act as a space-occupying mass resulting in pulmonary hypoplasia.

Objectives: In the last years, many surgical techniques of preputioplasty have aimed to preserve the foreskin in case of phimosis. These techniques are not reliable for patients affected by phimosis linked to balanitis xerotica obliterans (BXO) and scarred foreskin. We tried an original technique of resection of the pathological foreskin, removing the mucosal internal layer followed by reconstruction of the foreskin. The aim was to evaluate the outcome of paediatric patients who underwent modified partial circumcision for pathological phimosis. Patients and methods: In all, 360 patients with phimosis underwent modified partial circumcision at our institution. The mean age of the boys was 8.9 years, range 5–15 years. In 145 (40.3%) cases, indication for surgery was clinical suspicion of BXO, in 215 (59.7%) cases it was chronic inflammation of the foreskin. Results: In all cases, the postoperative period was uneventful. Cosmesis was considered by parents as excellent in 95.2% of patients. In these patients, the glans was almost completely covered by soft foreskin. Histopathological examination of the removed foreskin documented BXO in 162 (45%). Twelve (3.3%) patients complained of recurrences and five (1.4%) patients of smegmatic cysts. Conclusion: The described surgical technique of modified partial circumcision for the correction of pathological phimosis appears cosmetically well accepted, safe, and simple with low rate of late postoperative complications. Keywords: balanitis xerotica obliterans, circumcision, partial circumcision, phimosis

Cadmium (Cd) causes male infertility. There is the need to identify safe treatments counteracting this toxicity. Flavocoxid is a flavonoid that induces a balanced inhibition of cyclooxygenase (COX)-1 and COX-2 peroxidase moieties and of 5-lipoxygenase (LOX) and has efficacy in the male genitourinary system. We investigated flavocoxid effects on Cd-induced testicular toxicity in mice. Swiss mice were divided into 4 groups: 2 control groups received 0.9% NaCl (vehicle; 1 ml/kg/day) or flavocoxid (20 mg/kg/day ip); 2 groups were challenged with cadmium chloride (CdCl2; 2 mg/kg/day ip) and administered with vehicle or flavocoxid. The treatment lasted for 1 or 2 weeks. The testes were processed for biochemical and morphological studies. CdCl2 increased phosphorylated extracellular signal-regulated kinase (p-ERK) 1/2, tumor necrosis factor (TNF)-α, COX-2, 5-LOX, malondialdehyde (MDA), B-cell-lymphoma (Bcl)-2-associated X protein (Bax), follicle-stimulating hormone (FSH), luteinizing hormone (LH), transforming growth factor (TGF) -β3, decreased Bcl-2, testosterone, inhibin-B, occludin, N-Cadherin, induced structural damages in the testis and disrupted the blood-testis barrier. Many TUNEL-positive germ cells and changes in claudin-11, occludin, and N-cadherin localization were present. Flavocoxid administration reduced, in a time-dependent way, p-ERK 1/2, TNF-α, COX-2, 5-LOX, MDA, Bax, FSH, LH, TGF-β3, augmented Bcl-2, testosterone, inhibin B, occludin, N-Cadherin, and improved the structural organization of the testis and the blood-testis barrier. Few TUNEL-positive germ cells were present and a morphological retrieval of the intercellular junctions was observed. In conclusion, flavocoxid has a protective anti-inflammatory, antioxidant, and antiapoptotic function against Cd-induced toxicity in mice testis. We suggest that flavocoxid may play a relevant positive role against environmental levels of Cd, otherwise deleterious to gametogenesis and tubular integrity.

We report a case of congenital diaphragmatic hernia (CDH) with perinatal bowel strangulation requiring intestinal resection. Ten hours after birth, the newborn started to be lethargic and developed bilious emesis. X-ray documented distended loops of bowel with air fluid levels in the abdomen and a gasless, non-homogeneous opacity of the left hemithorax, a right mediastinal shift and loss of the sharp left hemidiaphram line. On gastrographin enema the left colon was above the adjacent left diaphragm. Emergency surgery was performed at 16 h of age. The entire small bowel appeared reddish and compromised. After 24 h, second-look laparotomy was performed and only 25 cm of small bowel were viable. The postoperative period was uneventful. Neonatal bowel strangulation in CDH should be taken into account when estimating postnatal morbidity and mortality and, even if CDH treatment is not an emergency procedure, if gastrointestinal symptoms prevail over respiratory symptoms, surgery should be carried out without delay.

Testicular torsion (TT) mainly affects boys under 18 years old. To avoid orchiectomy, TT requires an immediate operative management. The etiology of TT is still controversial. Observed familiar recurrence suggests the presence of a genetic involvement. The INSL3 gene consists of two exons, and it is specifically expressed in fetal and adult Leydig cells. In transgenic mice, deletion of this gene was observed an increased testicular mobility and testicular torsion. We have hypothesized the possible involvement of the INSL3 gene as a predisposing factor of human TT. We performed genetic analysis in 25 pediatric patients with unilateral and intravaginal TT (left, n = 13, 56%; right, n = 12, 48%). The age of the patients ranged from 1 to 16 years (median age n = 10.4 ± 5.46 years). In this study, we included two first male cousins affected by TT. Venous peripheral blood samples was obtained after parental written informed consent. The Thr60Ala polymorphism was detected in exon 1 of INSL3 gene and other 2 rarer variants (rs1047233 and rs1003887) were identified in the 3′ untranslated region. These variants are prevalent in patients with TT instead of healthy subjects. Additional studies in a larger population are needed to better understand the clinical consequence of the INSL 3 variations founded. This would allow in the future to identify the patients at risk of TT to improve clinical management.

The aim of the study was to retrospectively review the outcome of neonatal ureteropelvic junction obstruction with a good renal function and a poor drainage at a first diuretic renal scan, in cases where surgery was recommended on the basis of a loss of renal function, worsening of hydronephrosis or occurrence of clinical symptoms. Hydronephrosis was graded from 1 to 4 or as ureteral tract dilatation (UTD) P1 to UTD P3. During follow-up, 15 out of 38 patients (34.2%) required surgery while 25 out of 38 (65.8%) could have been managed conservatively. In patients with grade 2, 3, and 4 hydronephrosis, the ureteropelvic junction obstruction resolved or improved spontaneously in 100%, 63%, and 33% of cases (in 100% of UTD P1, 67% of UTD P2, and 50% of UTD P3), respectively. The median of follow-up was 14 years. Chi-square test showed a significant relationship between initial grade of hydronephrosis or UTD and the possibility of an efficient conservative management (p = 0.0088 and p = 0.0460).Conclusion: Conservative management can be safely achieved in ureteropelvic junction obstruction with poor drainage. Scheduled controls are needed for early discovery of functional renal deterioration. High-grade hydronephrosis is unlikely to resolve spontaneously and is often accompanied by a loss of renal function during the first years of life. What is Known: • There is controversy about which management should be adopted in infants with unilateral ureteropelvic junction obstruction with poor drainage but good differential renal function. What is New: • Long-term follow-up suggests that conservative management can be safely achieved also in unilateral ureteropelvic junction obstruction with poor drainage in more than 60% of cases, even if high-grade hydronephrosis is unlikely to resolve spontaneously and it is often accompanied by a loss of renal function during the first years of life. In our experience, surgical intervention was required in more than 50% of cases before 1 year of life and in all cases before 3 years of life.

Testicular torsion or torsion of the spermatic cord is one of the most serious urological conditions. It causes testicular injury, which potentially leads to male subfertility. The turning of the spermatic cord and spermatic structures around themselves results in biochemical and histological changes; however, following testicular detorsion, tissues undergo reperfusion that causes more severe damage than that induced by ischemia. Since the primary causes of testicular damage are reactive oxygen species production, an increase in intra-mitochondrial calcium concentration and an increased rate of cellular apoptosis, different medications may potentially be effective. It seems that several medications, experimentally and sometimes clinically, serve an adjuvant role in the cellular damage that occurs following ischemia-reperfusion. Antioxidants, calcium channel blockers, phytotherapeutical medicinals, anaesthetics, hormones and platelet inhibitors may potentially create a solid basis for an adjuvant restoring therapy and ameliorate testicular function following torsion. The current study aimed to review the relevant literature and discuss the actions of a number of molecules that may protect the testes during ischemia/reperfusion injury.