Setting up regional diagnostic reference levels for pediatric interventional cardiology in Latin America and the Caribbean countries: preliminary results and identified challenges.

The goal of the present study was to propose a set of preliminary regional diagnostic reference levels (DRLs) for pediatric interventional cardiology (IC) procedures in Latin America and the Caribbean countries, classified by age and weight groups. The study was conducted in the framework of the Optimization of Protection in Pediatric Interventional Radiology in Latin America and the Caribbean program coordinated by the World Health Organization and the Pan American Health Organization in cooperation with the International Atomic Energy Agency. The first step of the program was focused on pediatric IC. Dose data from diagnostic and therapeutic procedures were collected between December 2020 and December 2021. Regional DRLs were set as the third quartile of patient dose data (kerma area product) collected in 18 hospitals from 10 countries in an initial sample of 968 procedures. DRLs were set for four age bands and five weight ranges. The values obtained for the four age bands (<1 yr, 1 to <5 yr, 5 to <10 yr and 10 to <16 yr) were 2.9, 6.1, 8.8 and 14.4 Gy cm2for diagnostic procedures, and 4.0, 5.0, 10.0 and 38.1 Gy cm2for therapeutic procedures, respectively. The values obtained for the five weight bands (<5 kg, 5 to <15 kg, 15 to <30 kg, 30 to <50 kg and 50 to <80 kg) were 3.0, 4.5, 8.1, 9.2 and 26.8 Gy cm2for diagnostic procedures and 3.7, 4,3, 7.3, 16.1 and 53.4 Gy cm2for therapeutic procedures, respectively. While initial data were collected manually as patient dose management systems (DMSs) were not available in most of the hospitals involved in the program, a centralized automatic DMS for the collection and management of patient dose indicators has now been introduced and is envisaged to increase the sample size. The possibility of alerting on high dose values and introducing corrective actions will help in optimization.

High Blood Pressure States in Children, Adolescents, and Young Adults Associate Accelerated Vascular Aging, with a Higher Impact in Females' Arterial Properties.

The aims of the study were to determine (1) whether the presence of High blood pressure (HBP) states in the youth associate a steeper rate of age-related change in arterial geometrical and wall properties with respect to subjects with no previous cardiovascular risk factor (CRF) exposure, (2) in which parameters and in what magnitude, and (3) the existence of a gender-related difference in the impact of this condition on arterial properties. 300 individuals (mean/range: 15/4-29 years; 133 females) were included. Two groups were assembled: (1) Reference: nonprevious exposure to traditional CRF and (2) HBP: subjects with arterial hypertension and/or elevated blood pressure (BP) levels during the study. Additionally, HBP subjects were separated in BP-related subgroups. Measured parameters were (1) central (aortic) arterial BP and aortic pulse wave analysis parameters, (2) carotid and femoral artery local (pressure-strain elastic modulus) and regional (pulse wave velocity; PWV) stiffness, and (3) arterial diameters and carotid intima-media thickness (CIMT). Age-related changes in these parameters (absolute values and z-scores) were explored by obtaining simple linear regression models for each group. HBP presented a steeper rate of change (accelerated vascular aging; VA) for most of the parameters assessed, mainly in central (aortic) hemodynamics. VA increased as the HBP level got higher. Both males' and females' aging rates were affected by this condition, but females presented a more marked relative age-related increase with HBP exposure. HBP states in the youth gradually associate accelerated VA, with a progressive hemodynamic-structural-functional onset of damage, with females presenting a more marked relative HBP-associated arterial repercussion.

Ultrasound-guided Hartmann's solution enema: first-choice procedure for reducing idiopathic intussusception.

PURPOSE: This study was undertaken to compare the effectiveness of ultrasound-guided Hartmann's solution enema (US-E) and radiological liquid enema (RX-E) in reducing idiopathic ileocecocolic intussusceptions in relation to patient age and symptom duration. MATERIALS AND METHODS: The study group consisted of 42 patients with idiopathic ileocecocolic intussusception treated with US-E (20 patients) or RX-E (23 patients), with one patient undergoing both procedures owing to recurrence. Patients were divided into subgroups according to age (<6 months, 6-12 months, >12 months) and symptom duration (<12 h, 12-24 h, >24 h). RESULTS: Complete reduction was achieved in 15/20 patients treated with US-E (75%) and in 10/23 treated with RX-E (43.5%) (p=ns). Recurrence was observed in 1/20 US-E and 0/23 RX-E (p=ns) patients. No complications were encountered. US-E had a significantly higher success rate than RX-E in patients >12 months (p=0.0063) and with symptom duration >24 h (p=0.0361). No differences were found in the other subgroups (p=ns). CONCLUSIONS: US-E and RX-E are procedures of comparable value and safety in reducing idiopathic intussusception. US-E seems to be more effective in patients >12 months or with symptom duration >24 h. As US-E avoids radiation exposure, it should be considered the first-choice procedure for reducing idiopathic ileocecocolic intussusception, particularly in these two subgroups of patients.

An fMRI study on the neural correlates of social conformity to a sexual minority.

Social conformity refers to the tendency to align one's own behaviors, beliefs and values to those of others. Little is known about social influence coming from a minority group. To test whether social pressure from sexual minorities triggers avoidance-motivated behaviors, we explored how being influenced by the preferences of gay peers modifies the behavioral and neural reactivity of individuals defined as in- vs. out- groups on the basis of sexual orientation. To this aim, we combined fMRI with a social conformity paradigm in which heterosexual and gay/bisexual (hereafter non-exclusively heterosexual, NEH) individuals provided with male body attractiveness ratings by a fictitious group of gay students may or may not alter their previous rating and may or may not conform to the mean. Behaviorally, conformity to the minority preference was found in in-group NEH more than in out-group heterosexuals. Analysis of BOLD signal showed that social pressure brought about increased brain activity in frontal and parietal regions associated with the detection of social conflict. These results show that members of a sexual majority group display a smaller level of conformity when a sexual minority group exerts social influence. However, the neural correlates of this modulation are yet to be clarified.

Growing-Related Changes in Arterial Properties of Healthy Children, Adolescents, and Young Adults Nonexposed to Cardiovascular Risk Factors: Analysis of Gender-Related Differences.

The aims of our work were to determine normal aging rates for structural and functional arterial parameters in healthy children, adolescents, and young adults and to identify gender-related differences in these aging rates. Methods. 161 subjects (mean: 15 years (range: 4-28 years), 69 females) were studied. Subjects included had no congenital or chronic diseases, nor had they been previously exposed to traditional cardiovascular risk factors. Arterial parameters assessed were (1) central blood pressure (BP) and aortic pulse wave analysis, (2) arterial local (pressure-strain elastic modulus) and regional (pulse wave velocity, PWV) stiffness, and (3) arterial diameters and carotid intima-media thickness (CIMT). Simple linear regression models (age as the independent variable) were obtained for all the parameters and the resulting rates of change were compared between genders. Results. No gender-related differences were found in mean values of arterial structural and functional parameters in prepubertal ages (4-8 years), but they started to appear at ~15 years. Boys showed a greater rate of change for central systolic BP, central pulse pressure, CIMT, and carotid-femoral PWV. Conclusion. Gender-related differences in arterial characteristics of adults can be explained on the basis of different growing-related patterns between boys and girls, with no existing differences in prepubertal ages.

Endothelial perturbation in children and adolescents with type 1 diabetes: association with markers of the inflammatory reaction.

OBJECTIVE: The progression of diabetic angiopathy is, in most cases, unpredictable. The aim of this study was to investigate early events that could influence the development of diabetic angiopathy. RESEARCH DESIGN AND METHODS: Circulating levels of von Willebrand factor (vWF) and tissue-plasminogen activator (tPA), defining endothelial perturbation, were measured in 40 young patients with type 1 diabetes. Patients were divided into two groups according to the duration of diabetes (group A, <1 year; group B, >1 year) and compared with a control group of age- and sex-matched healthy individuals. Prothrombin fragment 1 and 2 (F(1+2)), tumor necrosis factor-alpha (TNF-alpha), and C-reactive protein (CRP) levels were also determined as markers of a prothrombotic state and inflammatory response. A total of 16 of the 20 children in group A were re-examined after 12 months. RESULTS: Compared with either normal subjects or patients in group B, children in group A showed increased levels of vWF, tPA, F(1+2), TNF-alpha, and CRP. Significant direct correlations between TNF-alpha or CRP and either vWF, tPA, or F(1+2) were observed. Endothelial perturbation was shown in 70% of group A and 20% of group B. After 1 year, 16 of the 20 patients in group A showed a significant reduction in vWF, tPA, F(1+2), TNF-alpha, and CRP levels, whereas endothelial perturbation was reversed in 5 of these patients. CONCLUSIONS: Endothelial perturbation represents an early and, in some cases, reversible event in the chronology of type 1 diabetes in children. A correlation might exist between the initial inflammatory reaction and the appearance of endothelial perturbation.

A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Endothelial perturbation in cystic fibrosis.

Cystic fibrosis (CF) is characterized by a persistent inflammatory state, which can be secondary to chronic pulmonary infection and may affect vascular endothelium. We measured circulating levels of von Willebrand factor (vWF), tissue-plasminogen activator (t-PA), and P-selectin in 20 CF patients and 20 healthy subjects. vWF, t-PA and P-selectin levels were significantly higher in CF patients. Endothelial perturbation (>2 SD increase in both vWF and t-PA) was present in 65% of CF patients. These patients displayed lower FEV1 values compared to individuals without endothelial perturbation and an inverse correlation between FEV1 and P-selectin levels was observed. Tumor necrosis factor-alpha (TNF-alpha) and interleukin (IL)-6 levels were also increased in CF patients and significant direct correlations were found between TNF-alpha and vWF, t-PA or P-selectin levels. These results indicate that CF patients exhibit signs of endothelial dysfunction/perturbation, which are likely to be related to a persistent inflammatory state due to chronic pulmonary infection, and may play a role in the progression of this disease.

Bifid sternum: neonatal surgical treatment.

The sternal cleft is a rare congenital anomaly resulting from a fusion failure of the sternum, generally observed at birth and asymptomatic. Surgery is indicated to protect the heart and major vessels from trauma, to improve respiratory dynamics, and for aesthetic reasons. We observed 2 neonates for a superior and medial thoracic mass. The defect involved the upper two thirds of the sternum. The surgical operation for both patients consisted in the primary closure of the defect.

Correlation between Down's syndrome and malformations of pediatric surgical interest.

PURPOSE: This is a collaborative study carried out by Pediatric Surgeons of the "G.D'Annunzio" University and the Regional Association of Down Children of Abruzzo (Italy). METHODS: Data were collected of malformations combined with Down Syndrome (DS) during a 10-year period in a population of defined age to look for a possible improvement of the patients' life conditions. Reportedly, 50% of these patients may reach an age of about 60 years. RESULTS: One hundred twenty-seven DS subjects from this region were evaluated, 54% of whom had associated malformations (13% cardiac, 41% extracardiac, and 13% both). Seventeen patients of 53 underwent surgery for extracardiac malformations, with gastrointestinal malformations prevailing. The largest number of DS babies were born from mothers under 30 years of age; this is attributed to the largest birth rate and the least prevention at this age. Mothers older than 38 years gave birth to DS babies with the lowest rate of associated malformations. CONCLUSION: The role of the pediatric surgeon in multidisciplinary assistance for DS patients is stressed.

Congenital giant megaureter: first neonatal case.

The authors report the first neonatal presentation of a congenital giant megaureter (CGM), a very rare unilateral urinary anomaly, which is defined as "a ureter whose lumen is congenitally, focally and segmentally dilated to more than 10 times the normal diameter, in presence of normal bladder volume and function." The definition of CGM is purely descriptive; it can be secondary to several different diseases, and in the series of 21 CGM (12 in duplex system) reported by Chen-Ju Huang, the morphology of the distal ureter varied from a near normal caliber to a ureteral stenosis, a ureterocele or a complete ureteral atresia. J Pediatr Surg 36:944-945.

Transumbilical laparoscopic-assisted appendectomy (TULAA): a safe and useful alternative for uncomplicated appendicitis.

AIM: To compare transumbilical laparoscopic-assisted appendectomy (TULAA) and open appendectomy (OA) in cases of acute uncomplicated appendicitis. METHODS: 58 TULAA and 65 OA in patients with a preoperative diagnosis of acute uncomplicated appendicitis are compared. Pneumoperitoneum was obtained with a transumbilical 10 mm trocar (telescope access) and a 5 mm operative channel introduced in the left iliac fossa. Appendectomy was performed outside the abdomen, after the exteriorisation of the appendix through the transumbilical incision. RESULTS: In the TULAA group, operative time and hospital stay were reduced. Conversion was necessary in one case (1.7 %), and in one case (1.7 %), an additional 5 mm operative channel was introduced. Neither intra- or postoperative complications were found in the TULAA group, with excellent cosmetic results. In the OA group we had a wound infection (1.5 %) and in 8 cases (12.3 %) an enlargement of the incision was necessary. CONCLUSIONS: TULAA is the best approach in uncomplicated appendicitis. It is less invasive and traumatic, permits a complete evaluation of the peritoneal cavity with superior cosmetic results, especially in obese patients and in cases of ectopic appendicitis.

Conservative treatment of neonatal primary megaureter.

The growing agreement toward the conservative treatment of primary megaureter (PM) is supported by the increasingly frequent reports in the literature of spontaneous resolution of this pathology after few years of attendance. If the PM is asymptomatic without the presence of parenchymatous damage, and the diuretic scintiscan does not show a definite obstruction of the uretero-vesical junction, the conservative treatment should be the choice for the neonatal forms of PM. We report our experience of 14 neonatal PM, conservatively managed out of a total of 22 PM observed between 1990 and 1996. All the patients have been controlled with serial ultrasonography and Tc99 DTPA scintigraphy. Three of them underwent a surgical operation because of persistence or impairment of the clinical and scintigraphic pictures after a 12-18 months' follow-up. Some of the remainders are completely recovered while others are going toward resolution. The conservative treatment of neonatal PM is therefore confirmed to be sure and effective, and in spite of the different attitudes expressed by reliable authors in the up-to-date literature we believe it should be undertaken for the asymptomatic forms in which there is no documented uretero-vesical obstruction.

Blue rubber bleb nevus syndrome and gastrointestinal haemorrhage: which treatment?

PURPOSE: To describe a paediatric case of "Blue Rubber Bleb Nevus Syndrome" (BRBNS) or Bean's syndrome, a rare systemic disorder characterised by cutaneous and gastrointestinal vascular malformations that often lead to overt life-threatening gastrointestinal bleeding or occult blood loss with severe anaemia and iron deficiency. CASE REPORT: A 6-year-old girl with multiple characteristic cutaneous vascular lesions was admitted for a massive rectal bleeding. A few months previously she was endoscopically treated for gastric angiomas which developed into melaena. Preoperative investigations revealed the recurrence of gastric lesions. At laparotomy, more than 25 angiomas of the GI tract were found. Multiple intestinal resections were carried out. RESULTS: No intraoperative or postoperative problems occurred and the girl is completely healthy without further bleeding after a follow-up period of three years. CONCLUSIONS: BRBNS belongs to the group of vascular venous malformations. Most of the time it occurs sporadically, but it can be inherited as an autosomal dominant trait. Recent analysis identified a locus on chromosome 9 responsible for venous malformations. BRBNS patients present typical skin lesions, with some lesions having a rubber-like nipple appearance; the number of skin and GI lesions and the severity of anaemia are correlated. Treatment is dependent on the extent of gut involvement and the severity of the clinical picture. In the absence of massive bleeding, a conservative treatment will be sufficient; otherwise resections are mandatory, but additional lesions may subsequently develop. Management with electrocautery or laser photocoagulation are usually not effective even if some reports recommend them. Pharmacological treatment is useless. Prognosis of BRBNS is unknown.

Multicystic kidney joined to the homolateral testis by an ectopic vas deferens: embryological hypothesis for a new description.

Ectopic location of the vas deferens is a rare congenital anomaly with multiple variations. The frequent association with ano-rectal abnormalities and hypospadias is well known. We report two cases of a newborn and a 3-year-old child with ectopic vas deferens coupled with a left multicystic kidney without any ureteral structure. Furthermore, the latter had a contralateral vesicoureteral reflux and anal agenesis with recto-bulbar fistula. Our attempt with this report is to explain the development of such anomalous deferential outlet into the multicystic kidney, according to the most qualified theory of the Wolffian duct embryology.

[Epidemiologic study on the prevalence of low back pain in health personnel exposed to manual handling tasks]. / Studio epidemiologico sulla prevalenza della lombalgia nel personale sanitario esposto a movimentazione manuale dei carichi.

An epidemiological study on the prevalence rate of low back pain among health care workers exposed to manual handling tasks and physical work load was performed. A representative sample (n. 1053) of nursing staff working at the San Matteo Hospital in Pavia was studied using a questionnaire with the purpose of evaluating the prevalence rate of low back pain and related risk factors. 86.4% of the subjects admitted having suffered from back pain at some stage in their life and 71.0% complained of back pain in the 3 months prior to answering the questionnaire. Multivariate logistic regression analysis revealed a significant correlation between low back pain, female gender and specific nursing activities. A significant correlation was found between smoking (> 20 cigarettes per day) and the presence of low back pain, due to intervertebral disk pathology. A statistically significant difference (p < 0.0001) was found in the average degree of disability between healthy nurses (1.3), those with low back pain taking analgesic drugs (5.9) and nurses suffering from low back pain without taking drugs (3.8).

[Surgical treatment of thoracic wall malformations]. / Tratamiento quirúrgico de las malformaciones de la pared torácica.

Anterior chest wall deformities in children are the expression of some congenital malformation such as Poland's syndrome and pectus excavatum. The surgical treatment of these malformations affecting children is aimed at correcting large wall defects and stabilizing the chest wall, assuring at the same time chest harmonious growth and postoperative functional assessment. We present the results of the treatment of 2 cases of Poland's syndrome, 20 cases of pectus excavatum and 2 cases of pectus carinatum, with a follow-up of 6 months--9 years.

[Modern trends in the treatment of esophageal atresia]. / Moderni orientamenti nel trattamento dell'atresia esofagea.

Results in the treatment of oesophageal atresia have greatly improved in the last 20 years. In an increasing number of patients it is possible to practice primary surgical correction at birth. From December '82 to December '95, 64 neonates with oesophageal atresia ranging in weight from 1.2 to 3.8 kg were observed. Fifty-eight neonates had type III atresia, four had type I atresia and two presented a rare membranous atresia with a long longitudinal intramural fistula. Fifty-four associated malformations were present in thirty-five neonates (55%). Congenital cardiopathies were the most frequent malformations followed by ano-rectal, skeletal and urinary malformations. According to the classification proposed by Waterston, 35% of the neonates were Class A, 33% Class B, and 32% Class C. Three neonates with polymalformations died before surgical treatment. Our trend is to attempt, when possible, primary correction using an extrapleural approach, even in the presence of a long gap. Oesophageal anastomosis was performed in 51 patients (80%). Gastrostomy was never performed routinely but only in selected cases. Early post-operative complications occurred in 22.5% of the cases. Gastroesophageal reflux, registered in 11 cases, was treated surgically in 4 cases. All 13 cases of post-anastomotic stenosis were treated with cycles of dilatations. The overall survival rate in 64 patients was 80% (96% in Class A; 86% in Class B, 55% in Class C). Of the 51 patients who underwent oesophageal anastomosis only 3 died, one because of the risk category according to Waterston. Survival is related more to the presence of severe multiple associated malformations, rather than bronchopneumonic complications of birth weight.

Case report: Inflammatory myofibroblastic tumor of pancreatic origin in a patient with down syndrome: The role of diagnostic ultrasound.

INTRODUCTION: Inflammatory myofibroblastic tumor (IMT) is a benign solid tumor of uncertain etiology. MATERIALS AND METHODS: We report a case of a 4-year-old Down syndrome affected child, who had a pancreatic mass identified by ultrasonography (US) and confirmed by computed tomography (CT). RESULTS: Monitoring of IMT was performed by serial US studies, and at follow-up after 4 years there was no relapse. DISCUSSION: As radical removal of the lesion was not possible, the patient was successfully treated with nonsteroidal anti-inflammatory drugs (NSAIDs). It was decided to monitor the lesion by serial US in order to reduce the number of CT examinations and thereby avoid excessive exposure to ionizing radiation. It is widely reported in the literature that repeated CT scans are associated with increased exposure to radiation which may cause cancer, a fact which should not be overlooked in children.

Anal canal duplication: a retrospective analysis of 12 cases from two European pediatric surgical departments.

Anal canal duplication (ACD) represents an extremely rare intestinal congenital anomaly of unknown origin. Usually evidenced within 2 years of age, nearly 45% of reported cases present associated malformations such as presacral mass, anorectal malformation (ARM) and genitourinary anomalies. The confirmative diagnosis is histopathological, with evidence of an anal mucosal lining (squamous +/- transitional epithelium), surrounded from a smooth muscle coat and anal glands. We review a conjoined experience from two European pediatric surgical departments. From 1970 to 2005, 12 patients were observed, seven in Pescara, Italy (1997-2005), five in Barcelona, Spain (1970-2004) - mean age at diagnosis 17.8 months, range 0-60; M:F = 1:11. Clinical presentation, diagnostic-surgical approach, and complications were reviewed. According to clinical presentation, patients could be divided in three age groups: asymptomatic (mean age 4.8 months, six patients - one with an associated complex genitourinary malformation, one with a presacral mature teratoma, one with ACD evidenced hysthologically on a retroanal mass removed during the correction of an ARM), mildly symptomatic - constipation, mucous discharge (mean age 29.2 months, four patients - one with associated presacral ependymoma and intestinal neuronal dysplasia type B, one with presacral mass) and complicated - perineal abscess, recurrent fistula (mean age 34 months, two patients). In 11 cases a perianal orifice was evident (ten posteriorly located). The pelvic-MRI was the preferred diagnostic tool in Pescara (5/7, with presacral mass in two patients), fistulography in Barcelona (5/5), where one presacral mass was discovered intraoperatively. Eleven patients underwent surgical removal of the ACD (five perineal approach, five posterior sagittal approach, and one PSARP). Histopathological findings confirmed the diagnosis in operated cases (11). The parents of the male patient denied the consent to surgical treatment. The only major post-operative complication was a sphincteric insufficiency (one case), surgically treated. When facing a perianal orifice, attention should be paid to ACD, particularly in female patients with coexistent genitourinary or intestinal malformations. Pelvic US and MRI are the gold standard to evidence the not rarely associated presacral mass. Surgical early removal (mucosectomy or perineal/posterior sagittal approach, depending on length of ACD and associated presacral mass) is warranted, also in asymptomatic patients, because of the risk of inflammatory complications and cancer (the latter reported in literature in adults).