Use of music and play in pediatric nursing care in the hospital context

Objective. To identify and analyze scientific publications on the use of music and play in pediatric nursing care in the hospital context. Method. In this bibliographic study, papers were sought that were published in Portuguese or English between 2004 and 2009 and included the descriptors: hospitalized child, childhood, child recreation, nursing team, nursing, pediatric nursing, alternatives therapies, music, music therapy, play and playthings, play therapy, playing. For the review, the bibliographic databases used were MEDLINE, ScIELO and LILACS. Results. Seventeen publications were obtained, among which: 59% adopted a quantitative method; mainly nursing developed the activities (88%); per type of article, reviews on the theme and assessments of clinical changes associated with the use of music and play were frequent (59% and 18%, respectively); and the utility of this kind of therapies in nursing care is acknowledged (94%). Conclusion. Play and music are useful therapies that can be used in nursing care for pediatric patients.

Are patients with encephalocraniocutaneous lipomatosis at increased risk of developing low-grade gliomas?

Cancer-prone genetic disorders are responsible for brain tumors in a considerable proportion of children. Additionally, rare genetic syndromes associated to cancer development may potentially disclose genetic mechanisms related to oncogenesis. We describe two pediatric patients with encephalocraniocutaneous lipomatosis (ECCL), a very rare genetic syndrome with around 60 reported cases, which developed low-grade astrocytoma at 3 and 12 years of age. Patients with ECCL seem to be at risk of benign forms of osseous tumors such as ossifying fibromas, odontomas, and osteomas. The association between brain tumor and ECCL was previously reported only once, in a pediatric case of a mixed neuronal-glial histology. Whether ECCL may be a genetic condition of predisposing brain tumor in children strongly needs to be addressed.

Impact of pediatric epilepsy surgery on intellectual efficiency

Introduction. Epilepsy surgery may be a promising alternative therapy for seizure control in patients with refractory seizures, resistant to medication. Cognitive outcome is another important factor in favor of the surgical decision. Aim. To investigate the correlation between seizure outcome and cognitive outcome after epilepsy surgery in a pediatric population. Patients and methods. A total of 59 pediatric patients were retrospectively assessed with the WISC-III (Full Scale, Verbal Scale and Performance Scale) before and, at least, 6 months after surgery. Patients were divided into two groups according whether or not improvement of seizure control after surgery. Data collected for each child included: epileptic syndrome, etiology, age at epilepsy onset, duration of epilepsy and seizure frequency. Results. Comparison using a MANOVA test revealed significant differences across pre-operative Full Scale, Verbal Scale and Performance Scale (p = 0.01) with seizure reduction group performing better than no seizure reduction group. Seizure improvement group achieved significant Performance Scale improvement (p = 0.01) and no seizure improvement group showed significant Verbal Scale worsened after surgery (p = 0.01). Conclusions. Our results suggest that the success of the epilepsy surgery in childhood when the seizure control is achieved may also provide an improvement in the Performance Scale whereas the seizure maintenance may worsen the Verbal Scale.

The evaluation of an instrument for pediatric oncology patient classification

The objectives of this study were to assess the interrater reproducibility of the instrument to classify pediatric patients with cancer; verify the adequacy of the patient classification instrument for pediatric patients with cancer; and make a proposal for changing the instrument, thus allowing for the necessary adjustments for pediatric oncology patients. A total of 34 pediatric inpatients of a Cancer Hospital were evaluated by the teams of physicians, nurses and nursing technicians. The Kappa coefficient was used to rate the agreement between the scores, which revealed a moderate to high value in the objective classifications, and a low value in the subjective. In conclusion, the instrument is reliable and reproducible, however, it is suggested that to classify pediatric oncology patients, some items should be complemented in order to reach an outcome that is more compatible with the reality of this specific population.

Nutritional risk and anthropometric evaluation in pediatric liver transplantation

OBJECTIVE: To analyze the nutritional status of pediatric patients after orthotopic liver transplantation and the relationship with short-term clinical outcome. METHOD: Anthropometric evaluations of 60 children and adolescents after orthotopic liver transplantation, during the first 24 hours in a tertiary pediatric intensive care unit. Nutritional status was determined from the Z score for the following indices: weight/age, height/age or length/age, weight/height or weight/length, body mass index/age, arm circumference/age and triceps skinfold/age. The severity of liver disease was evaluated using one of the two models which was adequated to the patients' age: 1. Pediatric End-stage Liver Disease, 2. Model for End-Stage Liver Disease. RESULTS: We found 50.0% undernutrition by height/age; 27.3% by weight/age; 11.1% by weight/height or weight/length; 10.0% by body mass index/age; 61.6% by arm circumference/age and 51.0% by triceps skinfold/age. There was no correlation between nutritional status and Pediatric End-stage Liver Disease or mortality. We found a negative correlation between arm circumference/age and length of hospitalization. CONCLUSION: Children with chronic liver diseases experience a significant degree of undernutrition, which makes nutritional support an important aspect of therapy. Despite the difficulties in assessment, anthropometric evaluation of the upper limbs is useful to evaluate nutritional status of children before or after liver transplantation.

Role of immunosuppressive and biologic therapy in pediatric IBD

We aimed to evaluate the role of anti-TNF-alpha therapy with infliximab and adalimumab in a cohort of pediatric patients followed by our Center from 2002 to 2012. The cohort of patients examined consisted of 40 patients: 34 with Crohn disease (85%), 5 with ulcerative colitis (12.5%), one with chronic pouchitis after IPAA for ulcerative colitis (2.5%). All patients were treated with the anti-TNF-α biologic agents infliximab and adalimumab. Thirty-six received infliximab therapy: 19/36 received only infliximab, 17/36 received infliximab and then adalimumab due to loss of response to infliximab and steroid dependency; 4 patients received only adalimumab (infliximab-naïve). Anti-TNF treatment was started before 18 years of age in 34 patients: 29 received infliximab and 5 started adalimumab during childhood. Medical charts were reviewed and safety and efficacy of anti-TNF-alpha have been determined in this population.

Budesonide is effective in adolescent and adult patients with active eosinophilic esophagitis

Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by dense tissue eosinophilia; it is refractory to proton pump inhibitor therapy. EoE affects all age groups but most frequently individuals between 20 and 50 years of age. Topical corticosteroids are effective in pediatric patients with EoE, but no controlled studies of corticosteroids have been reported in adult patients.

Gout in pediatric renal transplant recipients

Clinical gout has rarely been described after pediatric renal transplantation (RTx), although asymptomatic hyperuricemia is common in these patients. We describe three male pediatric patients who presented with gouty arthritis 7-8.5 years following RTx. Since receiving allopurinol, all patients had been free of gouty symptoms. To prevent severe bone marrow depletion, the dosage of azathioprine, an immunosupressant drug, was reduced by 50% to prevent interaction with allopurinol. Because atypical presentation of gout can occur, a high index of suspicion is needed to allow appropriate diagnosis of this disease in patients with skeletal pain after RTx.

Reduced atrial connexin43 expression after pediatric heart surgery

Myocardial dysfunction and arrhythmias may be induced by congenital heart defects, but also be the result of heart surgery with cardiopulmonary bypass (CPB), potentially caused by differential expression of connexin40 (Cx40) and connexin43 (Cx43). In 16 pediatric patients undergoing corrective heart surgery, connexin mRNA expression was studied in volume overloaded (VO group, n=8) and not overloaded (NO group, n=8) right atrial myocardium, excised before and after CPB. Additionally, in eight of these patients ventricular specimens were investigated. The atrial Cx43 expression decreased during CPB, which was restricted to the VO group (p=0.008). In contrast, atrial Cx40 mRNA did not change during CPB. In ventricular myocardium compared to atrial mRNA levels, Cx40 was lower (p=0.006) and Cx43 higher (p=0.017) expressed, without significant change during CPB. This study revealed a significant influence of CPB and the underlying heart defect on Cx43 expression.

Pediatric craniofacial trauma

PURPOSE: Maxillofacial and skull fractures occur with concomitant injuries in pediatric trauma patients. The aim of this study was to determine the causes and distributions of maxillofacial and skull fractures as well as concomitant injuries of pediatric patients in Switzerland. Results were compared with worldwide studies. MATERIALS AND METHODS: A retrospective review was conducted of 291 pediatric patients with maxillofacial and skull fractures presenting to a level-I trauma center over a 3-year span. Data concerning the mechanism of the accident and the topographic location of the injuries were analyzed. RESULTS: The most common causes were falls (64%), followed by traffic (22%) and sports-related accidents (9%). Fifty-four percent of the fractures occurred in the skull vault and 37% in the upper and middle facial third. One third of the patients (n = 95) suffered concomitant injuries, mostly cerebral concussions (n = 94). CONCLUSIONS: The spectrum of craniofacial injuries is related to the specific developmental stage of the craniofacial skeleton. It is probable that national prevention programs will have a positive effect on reducing the incidence of falls. Standardization of studies is needed for international comparison.

Prognosis in pediatric hematologic malignancies is associated with serum concentration of mannose-binding lectin-associated serine protease-2 (MASP-2)

BACKGROUND: Mannose-binding lectin (MBL) and MBL-associated serine protease-2 (MASP-2) are key components of the lectin pathway of complement activation. Their serum concentrations show a wide interindividual variability. This study investigated whether the concentration of MBL and MASP-2 is associated with prognosis in pediatric patients with cancer. METHODS: In this retrospective multicenter study, MBL and MASP-2 were measured by commercially available ELISA in frozen remnants of serum taken at diagnosis. Associations of overall survival (OS) and event-free survival (EFS) with MBL and MASP-2 were assessed by multivariate Cox regression accounting for prognostically relevant clinical variables. RESULTS: In the 372 patients studied, median serum concentration of MBL was 2,808 microg/L (range, 2-10,060) and 391 microg/L (46-2,771) for MASP-2. The estimated 4-year EFS was 0.60 (OS, 0.78). In the entire, heterogeneous sample, MBL and MASP-2 were not significantly associated with OS or EFS. In patients with hematologic malignancies, however, higher MASP-2 was associated with better EFS in a significant and clinically relevant way (hazard ratio per tenfold increase (HR), 0.22; 95% CI, 0.09-0.54; P = 0.001). This was due to patients with lymphoma (HR, 0.11; 95% CI, 0.03-0.47; P = 0.003), but less for those with acute leukemia (HR, 0.35; 95% CI, 0.11-1.15; P = 0.083). CONCLUSION: In this study, higher MASP-2 was associated with better EFS in pediatric patients with hematologic malignancies, especially lymphoma. Whether MASP-2 is an independent prognostic factor affecting risk stratification and anticancer therapy needs to be assessed in prospective, disease-specific studies.

Ultrasound of the pediatric female pelvis

Pelvic pain is a common indication for ultrasound examinations in female pediatric patients. Many pathological processes affect the female pelvis in childhood. Knowledge of the normal ultrasound appearance of the pelvic organs is the basis for the recognition of pathologic findings. Pelvic pain in children is a nonspecific clinical finding often prompting use of ultrasound. Other indications for pelvic ultrasound in female children include workup of cysts seen on fetal ultrasound, urogenital malformations in newborns, precocious puberty, vaginal discharge or abnormal bleeding, and amenorrhea. Knowledge of differential diagnosis for disease processes of the female pelvic organs is essential. Ultrasound is the imaging modality of choice for evaluating the pediatric female pelvis.

Gender modulates the expression of calcium-regulating proteins in pediatric atrial myocardium

A differential expression of sarcoplasmic reticulum calcium-ATPase (SERCA2a) and phospholamban (PLB) characterizes the remodeling process in heart failure and atrial arrhythmias in adult patients. Gender is known to modulate the course and prognosis of different forms of heart disease. We hypothesized that gender plays a role in molecular changes of myocardial calcium regulating components already in childhood. Moreover, we studied the influence of volume overloaded (VO) on SERCA2a and PLB in pediatric patients. Quantitative reverse transcription-polymerase chain reaction was used to measure mRNA expression of SERCA2a and PLB in atrial myocardium from 30 pediatric patients (12 girls, 18 boys). Eighteen patients had VO right atria, and 12 patients had not-overloaded atria (NO). Protein expression was studied by Western blot. In the entire population, SERCA2a and PLB expression was not different between girls and boys. If hemodynamic overload was taken into account, SERCA2a mRNA expression was significantly reduced in the VO group compared with the NO group (P = 0.021). The VO versus NO difference was restricted to boys, which corresponds to a highly significant interaction of gender versus VO status (P = 0.002). The PLB to SERCA2a protein ratio was significantly lower in girls (P = 0.028). The decrease in SERCA2a mRNA expression in VO atrial myocardium and the PLB to SERCA2a ratio of protein expression was modulated by gender in this pediatric population. To our knowledge, this study is the first to show the impact of gender on the differential expression of calcium-regulating components in pediatric cardiac patients.

Intramedullary nails for pediatric diaphyseal femur fractures in older, heavier children: early results

PURPOSE A common treatment for pediatric femur fractures is intramedullary nail (IMN) insertion. Elastic stable intramedullary nails (ESINs) are often used for these procedures in heavier patients, but the potential for complications and malunion is greater. We describe here a rigid IMN specifically designed for adolescents, the adolescent lateral entry femoral nail (ALFN). The purpose of this study was to compare the recovery and complications for patients treated with ESINs to those treated with the ALFN. METHODS Our study design was a retrospective cohort study. We performed a review of medical records of 22 children ages 10-17 requiring surgical fixation of a femur fracture for a 2½-year period. Patients selected for the study had traumatic diaphyseal femur fractures and were treated with ESINs without end-caps or ALFNs. Our analyses evaluated injury, surgical, and outcome information for all patients. RESULTS Twenty-two patients were eligible for inclusion and were divided into two groups according to their treatment: the ESIN group with 7 patients and the ALFN group with 15 patients. We then performed a comparison of complications and recovery for these patients. The mean time to full weight-bearing was significantly less for the ALFN group (4.1 weeks; SD, 2.2), than the ESIN group (9.4 weeks; SD 3.9). There was no statistical difference in the incidence of major or minor complications. CONCLUSIONS Older, heavier pediatric patients treated for femur fracture with ALFNs had a shorter recovery time than similar patients treated with ESINs. However, the outcomes for both groups were satisfactory.

Minimizing radiation injury and neoplastic effects during pediatric fluoroscopy: what should we know?

Radiation-induced injuries from fluoroscopic procedures in pediatric patients have occurred, and young patients are at greatest risk of many radiation-induced neoplasms. Some fluoroscopists have been injured from their use of fluoroscopy, and they are known to be at risk of radiation-induced neoplasm when radiation is not well-controlled. This article reviews the circumstances that lead to radiation injury and delineates some procedural methods to avoid injury and limit radiation exposure to both the patient and the fluoroscopist.