Child abuse experience, training, knowledge, and attitude of healthcare professionals in sixty hospitals in Greece.

OBJECTIVE: This study aims to record the overall perception of healthcare professionals on child abuse and identify potential affecting factors in a nationwide scale in Greece as well as to provide information that might be useful for future educational actions. MATERIALS AND METHODS: A total of 1,185 healthcare professionals in 60 hospitals with pediatric departments across Greece participated in this cross-sectional study. Participants included pediatricians, pediatric surgeons, residents, nurses, psychiatrists, psychologists, and social workers. Sections under investigation involved experience and training in child abuse, knowledge of formal and judicial issues, clinical knowledge, and self-assessment. RESULTS: Although more than half of the participants had confronted child abuse (n=712, 60.08%), only 273 (38.34% of them) submitted reports. One third of participants reported that they had received some training (n=440, 37.13%), mainly of postgraduate nature and based on personal initiative. Of those who reported child abuse, 175 (64.10%) had been trained. Each professional category was aware of topics regarding its own interest, without adequate knowledge of other disciplines. One third of psychiatrists, psychologists, and social workers felt confident in discussing with children and parents. Relevant scores were lower in the other categories. The lower scores were recorded among nurses and residents. The training deficit and reluctance to engage with judicial issues were the main causes of avoidance to deal with child abuse. CONCLUSIONS: Focused and organized training in child abuse is crucial to create reliable professionals in the field. The internet is a considerably helpful tool. Professionalism must characterize knowledge and practice in child abuse at the same level as in other medical topics. Motivation to engage should be early inspired and developed during the graduate years.

Liver Transplantation for Wilson's Disease in Non-adult Patients: A Systematic Review.

INTRODUCTION: Wilson's disease (WD) is a rare autosomal recessive disorder transmitted through a gene located on chromosome 13. Liver transplantation (LT) provides a therapeutic option for patients with WD presenting fulminant liver failure or drug resistance. LT in patients with WD has a twofold aim: to save the patient's life when the disorder has progressed to hepatic (or other organ) failure and to cure the underlying metabolic defect. The aim of our study was to investigate the indications, aspects and post-operative outcomes in pediatric patients (< 18 years old) with WD who underwent LT. METHODS: A meticulous search of the literature since 1971 was performed. A retrospective analysis of all the studies, presenting cases of LT in children due to WD, was conducted. Studies that did not report patients' characteristics, transplantation indications, post-operative outcomes, and complications, as well as those with small study populations (< 10 patients), were excluded. RESULTS: Six studies were included in the present review, which involved 290 children. The main indications for LT included chronic liver failure and fulminant liver failure. The average 1-year survival rate was 91.9%, while the average 5-year survival rate was 88.2%. Retransplantation was performed in 16 patients due to transplant rejection. In general, patients transplanted for WD displayed an excellent quality of life after LT. CONCLUSION: LT is a safe and efficient procedure in selected pediatric patients with WD, demonstrating excellent long-term outcomes and quality of life.

An alternative management of a Littré hernia case: food for thought.

AIM: We present a novel approach to a Littré hernia case. CASE REPORT: A 62-year old male presented at our department with a painless mass in the inguinal area and was successfully treated for an inguinal Littré hernia. A Lichtenstein tension-free mesh repair was used without performing simultaneous diverticulectomy. DISCUSSION: Resection of an asymptomatic Meckel's diverticulum remains a controversial issue. In adult population, leaving an accidentally found silent Meckel's diverticula in situ could reduce the risk of postoperative complications without increasing late complications. Mesh-based techniques provide lower recurrence rates compared to non-mesh techniques. CONCLUSIONS: Management of asymptomatic Littré hernias presents a challenge for the operating surgeon. Treatment guidelines should be developed for the optimal management of these patients.

Cystic hygroma of the neck: single center experience and literature review.

OBJECTIVE: Malformations of the lymphatic system are recognized as benign congenital tumors that affect infant and children in the perinatal era. In children, these abnormalities usually found in the neck and the axillary region, but they can present in other parts of the body such as mediastinum, pelvis, retroperitoneum as well as in solid organs (e.g., adrenal glands, pancreas, stomach). Our aim is to report our experience on cystic hygromas via two cases and review the literature. MATERIALS AND METHODS: Herein we present two cases of cystic hygroma, the first of female children and the second of a female adult patient respectively. Both of these patients underwent surgical excision of the masses. RESULTS: After the procedure, both patients have recovered well, and no recurrence of the lesion has been noted during the follow-up period. CONCLUSIONS: Surgical treatment remains the gold-standard treatment for these tumors, while other modalities have been used with mixed results.

Pheochromocytoma, diagnosis and treatment: Review of the literature.

OBJECTIVE: We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO). METHODS: Pub Med and Google Scholar databases were searched systematically for studies concerning pheochromocytomas (intra-adrenal paragangliomas) from 1980 until 2016. Bibliographies were searched to find additional articles. RESULTS: More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. The patient needs pre-treatment with α1-blockers at least 10-14 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/or ß-Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered. CONCLUSIONS: The biggest problem for pheochromocytoma is to suspect it in the first place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.

Radiology of the adrenal incidentalomas. Review of the literature.

The term "adrenal incidentaloma" is a radiological term. Adrenal incidentalomas are adrenal tumors discovered in an imaging study that has been obtained for indications exclusive to adrenal conditions (Udelsman 2001; Linos 2003; Bulow et al. 2006; Anagnostis et al. 2009). This definition excludes patients undergoing imaging testing as part of staging and work-up for cancer (Grumbach et al. 2003; Anagnostis et al. 2009). Papierska et al. (2013) have added the prerequisite that the size of a tumor must be "greater than 1cm in diameter", in order to be called incidentaloma. Although in the most cases these masses are non-hypersecreting and benign, they still represent an important clinical concern because of the risk of malignancy or hormone hyperfunction (Barzon et al. 2003). Th e adrenal tumors belong to the commonest incidental findings having been discovered (Kanagarajah et al. 2012).

Infantile perianal pyramidal protrusion: a report of 8 new cases and a review of the literature.

Infantile perianal pyramidal protrusion (IPPP) is characterized by a protrusion located most often anterior to the anus. Three types of IPPPs are recognized: (a) constitutional, (b) acquired (mainly due to constipation), and (c) IPPPs due to lichen sclerosus et atrophicus. The aim of this study was to make a brief review on IPPPs from articles retrieved from PubMed, and to present our experience in this field. We conclude that awareness of pediatric surgeons and pediatricians of the condition is essential for the correct diagnosis of IPPP, and could help to avoid erroneous investigation of other lesions in this area.

T cell therapy for nasopharyngeal carcinoma.

Among the novel biologic therapeutics that will increase our ability to cure human cancer in the years to come, T cell therapy is one of the most promising approaches. However, with the possible exception of tumor-infiltrating lymphocytes therapy for melanoma, clinical trials of adoptive T-cell therapy for solid tumors have so far provided only clear proofs-of-principle to build on with further development. Epstein-Barr virus (EBV)-associated malignancies offer a unique model to develop T cell-based immune therapies, targeting viral antigens expressed on tumor cells. In the last two decades, EBV-specific cytotoxic T-lymphocytes (CTL) have been successfully employed for the prophylaxis and treatment of EBV-related lymphoproliferative disorders in immunocompromised hosts. More recently, this therapeutic approach has been applied to the setting of EBV-related solid tumors, such as nasopharyngeal carcinoma. The results are encouraging, although further improvements to the clinical protocols are clearly necessary to increase anti-tumor activity. Promising implementations are underway, including harnessing the therapeutic potential of CTLs specific for subdominant EBV latent cycle epitopes, and delineating strategies aimed at targeting immune evasion mechanisms exerted by tumor cells.

Inflammatory myofibroblastic tumor of the liver due to Mycobacterium tuberculosis in an immunocompetent girl.

The case of a 9-year-old girl with a Mycobacterium tuberculosis inflammatory myofibroblastic tumor (IMT) of the left lobe of the liver is reported. The tumor was surgically excised and had histological features diagnostic of IMT, a positive Ziehl-Nielsen staining for acid-fast bacilli and a positive polymerase chain reaction for Mycobacterium tuberculosis. Surgical excision of the tumor followed by anti-tuberculosis treatment for 9 months resulted in full recovery. The patient had no apparent immune disorder, and there was no evidence of extrahepatic tuberculosis. These findings make this case exceptional because IMTs, due mostly to atypical mycobacteria, have been described only in immunocompromised patients.

Muesothelial cyst of the spermatic cord as a cause of acquired cryptorchidism.

Mesothelial cysts of the spermatic cord (MCSC) are a rare entity. A 2-year-old cryptorchid boy with an inguinal mass was found to have a MCSC which was confused with undescended testis. The testis was readily placed into the hemiscrotum after excision of the cyst. MCSC may present as undescended testis and may be an unusual cause of acquired cryptorchidism. It should be excised and the inguinal canal should be explored for the existence of an undescended testis.

Congenital patent urachus associated with incomplete urethral duplication: a rare association.

Congenital patent urachus (CPU) is an uncommon anomaly. Blind incomplete urethral duplication is encountered more frequently than other types of urethral duplication (UD) in clinical practice. We describe a child with CPU in association with distal blind incomplete UD. A possible pathogenesis of this extremely rare coexistence is discussed.

Strangulated low Spigelian hernia in children: report of two cases.

Low Spigelian hernia (LSH) in children is considered an extreme surgical rarity. This clinically deceptive hernia is difficult to diagnose preoperatively and has a real risk of strangulation. Strangulated LSH may be misdiagnosed as strangulated inguinal hernia. Early recognition and timely surgical repair are important to avoid strangulation.

Irreducible inguinal hernia due to crossed testicular ectopia in an infant.

The usual presentation of crossed testicular ectopia (CTE) is that of inguinal hernia with contralateral absent testis. We report on a 10-month-old infant with CTE, which presented as irreducible inguinal hernia. Diagnosis was made during surgery, as the child underwent an emergency operation for repair of his irreducible right inguinal hernia. A normal-sized and normal-shaped testis was found in the hernial sac with its blood vessels and vas deferens. A herniotomy with fixation of the ectopic gonad to the opposite hemiscrotum was done. The child remained asymptomatic 1 year postoperatively. Crossed testicular ectopia in infancy may present as irreducible hernia, requiring urgent surgery.

Influence of an aggressive early enteral nutrition protocol on nitrogen balance in critically ill children.

The objective was to determine stress related factors and nutritional indices affecting the nitrogen balance (NB) and the creatinine height index (CHI) in critically ill children on early enteral nutrition (EEN). Seventy-one consecutively enrolled critically ill children aged 2 to 204 months, requiring prolonged mechanical ventilation, were studied. All patients were on early intragastric nutrition (Nutrison Pediatric or Standard) from day 1 (energy intake equal to 1/2, 1, 5/4, 6/4 and 6/4 of the predicted basal metabolic rate on days 1-5, respectively). Nitrogen balance and CHI changes determined efficacy. Study patients had severe depletion of somatic protein status on stress day 1 (CHI <60%) but they reached the normal range of somatic protein status at the end of the EEN, on post-stress day 5 (CHI >80%, p <.004). On day 1, none of the patients had positive NB but after 5 days of EEN, 44 (62%) had positive NB and only 27 (38%) had negative NB (p <.0001). Multivariate stepwise regression analysis showed that only the difference of daily given-recommended dietary allowances protein and the total repleted energy were positively correlated (r(2) =.47, p <.001 and r(2) = 34, p =.003, respectively) and multiple organ system failure negatively correlated with the NB (r(2) = -.24, p <.03) on the 5th day of the EEN protocol. Our data suggest that achievement of positive protein and energy balance in relation to the basic metabolic rate using an aggressive EEN protocol improves NB during the acute phase of stress in 2/3 of critically ill children.

Myocardial injury in meningococcus-induced purpura fulminans in children.

OBJECTIVES: To assess the incidence of myocardial ischemia in meningococcus-induced purpura fulminans in pediatric patients, to compare troponin I (cTnI) levels with changes in electrocardiogram (ECG) and to evaluate whether cTnI is related to myocardial function and contractility, to severe acquired anticoagulant deficiency and to the severity of disease. METHODS: Twenty-two patients with acute meningococcemia, supported with inotropes or vasoactive agents, were studied, Blood samples for the determination of serum cTnI and conventional myocardial ischemia and coagulopathy markers were drawn daily. Measurements of cardiac index (CI), ejection (EF) and shortening fractions (SF) and ECGs were performed daily. RESULTS: The Leclerc score, the Neisseria sepsis index (NESI) and the pediatric risk of mortality (PRISM) score predicted a mean mortality rate of 34%, 27% and 23%, respectively. Four patients died (18%). Five patients (23 %) presented with myocardial ischemia. Their ECG ischemic changes were associated with pathologically high cTnI levels (1.93 +/- 0.13 vs 0.18 +/- 0.08 ng/ml, p < 0.001 for patients with or without ischemic changes) and depressed myocardial contractility (mean difference +/- SE -14 +/- 5%, p = 0.01, for the EF and -7.4 +/- 3, p < 0.02, for the SF). High cTnI values were significantly correlated to low protein C (PC) (p < 0.0001), factor VIII (p < 0.04) and antithrombin III (AIII, p = 0.01) levels, but not to the PRISM, Leclerc or the NESI scores. Means of AIII, VII, and especially of VIII, and PC, were significantly lower in ischemic than in non-ischemic patients, although severity scoring systems and inotropic support did not differ between the two groups. Survivors tended to significantly higher PC (p < 0.01) and factor VIII levels (p = 0.001) than non-survivors and, also, to lower levels of cTnI (p = 0.05) and CPK-MB (p < 0.05), while in meningococcal shock. CONCLUSIONS: The incidence of myocardial ischemia is increased in acute meningococcemia in pediatric patients and correlates with myocardial dysfunction. High cTnI is associated with severe coagulopathy, but not with clinical prognostic scores or inotropic support. Early recognition of myocardial injury, myocardial support and early replacement therapy with PC, AIII, factor VIII or fibrinogen might improve outcome in acute meningococcemia in children.

Malnutrition, nutritional indices, and early enteral feeding in critically ill children.

We measured the incidences of protein and fat depletions and the frequencies of acute and chronic protein-energy malnutrition during stress states in children and investigated the influence of early enteral feeding on nutrition indices and acute-phase proteins. Seventy-one, consecutively enrolled, critically ill children received early enteral feeding (energy intakes equal to 0.50, 1, 1.25, 1.5, and 1.5 of the predicted basal metabolic rates on days 1 through 5, respectively) through nasogastric tubes. On the first day of the study, 16.7% of the patients already were depleted of protein and 31% of fat stores. Overall, 16.9% were at risk for chronic protein-energy malnutrition and 21.1% for acute protein-energy malnutrition, whereas 4.2% and 5.6% already had chronic and acute, respectively protein-energy malnutrition. Only 22.7% of patients without protein deficiencies versus 37% of those at risk or already deficient developed multiple-organ system failure. Transferrin and prealbumin levels improved at the end of the period of early enteral feeding (187 +/- 6.6 versus 233 +/- 7 mg/dL, P < 0.0001; 15.1 +/- 2 versus 21.9 +/- 2.9 mg/dL, P < 0.0001; respectively); survivors had higher prealbumin levels than non-survivors (22.3 versus 15.5 mg/dL). With logistic regression analysis, only repleted energy, not anthropometric or nutrition indices, was independently associated with survival (P = 0.05). These results reinforce the observation that critically ill children are at risk for fat or protein depletion and development of malnutrition, which is associated with increased morbidity and mortality. We conclude that early enteral nutrition improves nutrition indices and outcomes.

Effectiveness and safety of a protocol for promotion of early intragastric feeding in critically ill children.

OBJECTIVES: To investigate the feasibility, adequacy, and efficacy of early poststress intragastric feeding (EPIGF) in critically ill children. DESIGN: A prospective clinical study. SETTING: Pediatric intensive care unit in a tertiary care children's hospital. PATIENTS: Seventy-one consecutively enrolled critically ill children requiring prolonged mechanical ventilation. INTERVENTIONS: Full-strength intragastric tube feedings (Nutrison Pediatric, Standard) were initiated within 12 hrs of the study-entry event. Enteral feedings were advanced to a target volume of energy intake = 1/2, 1, 5/4, 6/4, and 6/4 of the predicted basal metabolic rate (PBMR) on days 1-5, respectively. MEASUREMENTS AND MAIN RESULTS: Nutritional status by the caloric intake, recommended dietary allowances, PBMR, predicted energy expenditure (PEE), anthropometry, and clinical indices were evaluated on days 1 and 5. Safety was assessed by the clinical course of disease, laboratory findings, and occurrence of complications. Success was determined by accomplishment of the PEE target. The early success rate was 94.4% and predicted late enteral feeding success accurately (p =.0001). Caloric intake approached PBMR the second day (43 +/- 1.7 kcal/kg/day vs. 43.2 +/- 1.1 kcal/kg/day) and PEE the fifth day (66.2 +/- 2.7 kcal/kg/day vs. 67.7 +/- 6.4 kcal/kg/day). Multivariate stepwise regression analysis showed that poor outcome and a high Therapeutic Intervention Scoring System score correlated with failure of EPIGF (p <.0001). Patients who succeeded EPIGF had significantly higher myocardial ejection (65% vs. 43%; p <.0001) or shortening fractions (34% vs. 20%; p =.0001) on day 1 than those who failed. Patients tolerated EPIGF well; 9.9% developed nosocomial pneumonia, 5.6% developed diarrhea, and 8.5% needed treatment with cisapride because of a delay of gastric emptying. The mortality rate (5.6%) was different between initial and final success and failure groups (p <.0001) and was lower than predicted by the admission severity scores (12% +/- 2%). CONCLUSIONS: This study showed that increases of caloric intake during the acute phase of a critical illness are well tolerated and may approach PBMR by the second day and PEE by the fourth day in critically ill children. Caloric intake lower than PBMR is associated with higher mortality and morbidity rates.

Cardiac troponin I in fulminant adenovirus myocarditis treated with a 24-hour infusion of high-dose intravenous immunoglobulin.

We report a successful outcome on an acute adenovirus myocarditis treated with a 24-hour high-dose intravenous immunoglobulin (24-HDIVIG) in a 4.5-year-old girl. A postviral etiology of acute myocarditis was assessed on the basis of the polymerase chain reaction technique. Among other early markers of cardiac injury, cardiac isoform of troponin-I (cTnI) was significantly correlated to the left ventricular ejection fraction (r = -0.86, p < 0.0001). Follow-up of cTnI, which might also be correlated to the short-term outcome, allows fast, easy, and noninvasive estimation of response to the aggressive treatment with 24-HDIVIG in acute adenovirus myocarditis in children.