Adenosine as a Key Mediator of Neuronal Survival in Cerebral Ischemic Injury.

Several experimental studies have linked adenosine's neuroprotective role in cerebral ischemia. During ischemia, adenosine is formed due to intracellular ATP breakdown into ADP, further when phosphate is released from ADP, the adenosine monophosphate is formed. It acts via A1, A2, and A3 receptors found on neurons, blood vessels, glial cells, platelets, and leukocytes. It is related to various effector systems such as adenyl cyclase and membrane ion channels via G-proteins. Pharmacological manipulation of adenosine receptors by agonists (CCPA, ADAC, IB-MECA) increases ischemic brain damage in various in vivo and in vitro models of cerebral ischemia whereas, agonist can also be neuroprotective. Mainly, receptor antagonists (CGS15943, MRS1706) indicated neuroprotection. Later, various studies also revealed that the downregulation or upregulation of specific adenosine receptors is necessary during the recovery of cerebral ischemia by activating several downstream signaling pathways. In the current review, we elaborate on the dual roles of adenosine and its receptor subtypes A1, A2, and A3 and their involvement in the pathobiology of cerebral ischemic injury. Adenosine-based therapies have the potential to improve the outcomes of cerebral injury patients, thereby providing them with a more optimistic future.

Benign purely intraosseous meningioma of the skull: Diagnosis and surgical outcomes.

Primary intraosseous meningioma (PIM) is a rare subtype of extradural meningiomas that originates within bone. We aimed to characterize the clinical, radiographic, and pathologic features of PIM and the resulting outcomes following resection. Herein we examined a retrospective case series of all patients with a pathologically confirmed WHO grade I PIM that were managed at one of three tertiary care centers. Patients with tumors that demonstrated extraosseous extension or involvement of the dura mater were excluded. The main outcomes included surgical safety and duration of local tumor control. Nine patients were identified with benign PIMs, presenting with headaches or painless enlarging subcutaneous masses if involving the calvarium or with neurologic deficits if involving the skull base, or otherwise incidentally identified. Surgery was pursued for symptomatic relief and/or tissue diagnosis. Lesions were evaluated by radiographic imaging - including sensitive detection by plain X-ray films - and definitive diagnosis ascertained by histopathological examination. Maximal resection of both calvarial and skull base lesions was safely tolerated. PIM represents a rare benign skull lesion, whose identification depends on the integration of radiographic findings with intraoperative findings and histopathological confirmation; it should be considered in the differential for slow-growing expansile intraosseous lesions of the skull.

The long-term quality of life outcomes in adolescents with Hirschsprung disease.

BACKGROUND: Postoperative outcomes for Hirschsprung disease (HD) remain variable, with many patients affected by constipation and/or fecal incontinence. The long-term impact upon quality of life (QoL) for HD patients is unclear. We measured long-term QoL outcomes in adolescents with HD using validated questionnaires. METHODS: Patients with HD, managed at a large tertiary pediatric institution between 1997 and 2004, were identified. Patients and/or their proxy completed validated questionnaires. Results were compared with published healthy population controls. Two questionnaires assessed QoL: Pediatric Quality of Life Inventory (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FICQOL). Three measures assessed functional outcomes: Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Symptom Survey. RESULTS: Interviews were completed for 58 (70% response rate) patients [M:F, 49:9; median age, 14.5 years (11.1-18.7)]. No significant differences were found in general QoL scores between patients and healthy controls (84.84 versus 81.49, p = 0.28). Disease-specific questionnaires revealed reduced QoL in patients and families, with 17% of parents reporting the bowel dysfunction stopped their child from socializing and 47% of parents experiencing some degree of anxiety/depression regarding their child's bowel condition. Fecal incontinence (r = -0.59, p < 0.01), constipation (r = -0.36, p = 0.01), and dysfunctional elimination (r = -0.59, p < 0.01) all negatively correlated with QoL scores. CONCLUSIONS: In this study, generic QoL in the adolescent HD population was comparable to healthy populations. However, children with HD have ongoing bowel dysfunction which negatively impacts upon their QoL. LEVEL OF EVIDENCE: Prognosis study:- level II (prospective cohort study).

Spectrum of Syndromic Disorders Associated with Pediatric Tumors: Evolving Role of Practical Imaging Assessment.

Although pediatric tumors are largely sporadic in cause, continued advancements in science have elucidated a growing body of tumors that are associated with syndromes. Early identification of these syndromic disorders associated with developing tumors can alter the course of disease and potentially save lives. Medical imaging has a pivotal role in screening surveillance, diagnosis, and management of these tumors. Understanding characteristic manifestations of these syndromes is important to optimize image utilization. This article discusses clinically important syndromes associated with pediatric tumors with brief overview of the background, genetics, and clinical features. Diagnosis, imaging, management, and treatment are also reviewed.

The Effect of Dofetilide on the Heart Rate of GD11 and GD13 Rat Embryos, in vivo, Using Ultrasound.

BACKGROUND: There are a wide range of drugs including antidepressants, anticonvulsants and antipsychotics that cause embryonic bradycardia in vitro but it is unknown if they have a similar effect in vivo. One way to verify whether these in vitro findings are replicated in vivo is by the use of ultrasound examination of dosed pregnant rats. We tested this by examining the effect of dofetilide on embryonic heart rate (HR) in vivo using ultrasound. METHODS: Rats were dosed with dofetilide (4 or 2.5 mg/kg) on GD11 or (5 or 2.5 mg/kg) on GD13 and embryonic HR assessed by ultrasound, 2 and 24 hr later. Fetuses were examined for malformations on GD20. RESULTS: HR of control rat embryos showed a wide range at each gestational day. Dosing with dofetilide on GD11 caused severe bradycardia (∼ 60% reduction) 2 hours after dosing with recovery after 24 h of >60% of LD but death and slow HR among the HD embryos. At term, 32% of the LD surviving fetuses had hypoplastic upper lip while >90% of HD embryos had died. On GD13, embryonic HR was reduced in a dose-dependent manner with >85% of LD and HD recovered by 24 hr. At term, all LD fetuses were normal while 29% of HD fetuses had limb defects. CONCLUSIONS: Ultrasound is a useful technique to investigate the effect of maternally administered drugs on the embryonic HR in the rat. The results may provide more information about the safety of these drugs in pregnancy leading to better risk assessment for the human.

Variation in the documentation of findings in pediatric voiding cystourethrogram.

BACKGROUND: Few standards exist for reporting results of voiding cystourethrogram (VCUG). OBJECTIVE: To assess the variation in reporting of VCUG findings from different facilities using a standardized assessment tool. MATERIALS AND METHODS: VCUG reports were evaluated for demographic, technical, anatomical and functional information. Reports were categorized by age, gender, indication and vesicouretal reflux (VUR) status. Institutions were classified as a free-standing pediatric hospital (n = 3), pediatric hospital within a hospital (n = 11), or non-pediatric facility (n = 24) and reports were classified as having been read by a pediatric radiologist or not. Each category of outside reports (n = 152) was randomly matched with a twice-larger group of Hospital A reports from the same category (n = 304). Multivariate linear regression was used to analyze the association between the primary outcome (percentage of items described in dictated VCUG report) and the type of radiologist and institution. RESULTS: Of the 456 studies, 66% were in girls, 56% were in those <12 months old, and the indication was urinary tract infection (UTI) in 81%. The mean percentage of items reported was 67 ± 14% (74 ± 7% at free-standing pediatric hospitals, 61 ± 10% at pediatric hospitals within a hospital, and 48 ± 11% at non-pediatric facilities). In multivariate analysis, VCUG reports generated at non-pediatric facilities had 17% fewer items included (95% CI: 14.5-19.7%, P < 0.0001), and pediatric hospitals within a hospital had 9% fewer items included (5.9-12.5%, P < 0.0001) when compared to free-standing pediatric hospitals. Reports read by a pediatric radiologist had 12% more items included (9.1-15.3%, P < 0.0001) compared to those read by a non-pediatric radiologist. CONCLUSION: More complete VCUG reports were observed when generated at free-standing pediatric hospitals and when interpreted by a pediatric radiologist.

Previous PICC placement may be associated with catheter-related infections in hemodialysis patients.

BACKGROUND: Catheter-related infections (CRIs) are a significant source of morbidity and mortality in hemodialysis patients. The identification of novel, modifiable risk factors for CRIs may lead to improved outcomes in this population. Peripherally inserted central catheters (PICCs) have been hypothesized to compromise vascular access due to vascular damage and venous thrombosis, whereas venous thrombosis has been linked to the development of CRIs. Here we examine the association between PICC placement and CRIs. METHODS: A retrospective review was performed of all chronic hemodialysis catheter placements and exchanges performed at a large university hospital from September 2003 to September 2008. History of PICC line use was determined by examining hospital radiologic records from December 1993 to September 2008. Catheter-related complications were assessed and correlated with PICC line history. RESULTS: One hundred eighty-five patients with 713 chronic tunneled hemodialysis catheter placements were identified. Thirty-eight of those patients (20.5%) had a history of PICC placement; these patients were more likely to have CRIs (odds ratio = 2.46, 95% confidence interval = 1.71-3.53, p < .001) compared with patients without a history of PICC placement. There was no difference between the two groups in age or number of catheters placed. CONCLUSION: Previous PICC placement may be associated with catheter-related infections in hemodialysis patients.

Primary myxopapillary ependymoma of the medulla: case report.

OBJECTIVE: Myxopapillary ependymoma is a subclassification of ependymoma that is thought to be nearly exclusive to the conus medullaris or filum terminale. Primary intracerebral or brainstem myxopapillary ependymomas are rare. CLINICAL PRESENTATION: An 8-year-old child presented with a 5-month history of nausea and vomiting and a 1-week history of headache. Magnetic resonance imaging revealed a nodular mass in the medulla with an associated cyst extending into the fourth ventricle. INTERVENTION: A suboccipital craniotomy was performed, and a gross total resection of the lesion and cyst was achieved. Histological examination confirmed the diagnosis of myxopapillary ependymoma. A discussion of other reported cases of extraspinal myxopapillary ependymomas is presented. CONCLUSION: This is the first report of a case of myxopapillary ependymoma, confirmed by histology, in the medulla. Although rare, myxopapillary ependymomas outside of the filum terminale do exist.

Intracranial benign fibrous histiocytomas: a case report and review.

Fibrous histiocytomas are rare lesions, more commonly encountered in soft tissues and bones. They are uncommon as an intracranial lesion. Although there have been several reports about malignant fibrous histiocytomas, less is known about the benign variant of these intracranial tumors as they are often misclassified as other types of tumors. We describe a child who presented with seizure and was subsequently found to have a large temporal lesion. Pathology revealed benign fibrous histiocytoma. We also review other cases reported in the literature in an effort to provide further insight into the diagnosis and management of this rare tumor.

Gastric bypass: a risk factor for neural tube defects? Case report.

Gastric bypass surgery has become a safe and acceptable surgical weight loss treatment for individuals who suffer from morbid obesity. Patients who undergo this procedure are subject to vitamin deficiencies due to an iatrogenic malabsorptive state. Folate, a vitamin known for its role in the prevention of neural tube defects (NTDs), can be part of the deficiency spectrum resulting from this procedure. The authors describe the case of a woman who was nonadherent to multivitamin treatment after undergoing gastric bypass surgery. Her lack of understanding and appreciation of the relationship between gastric bypass surgery, folate deficiency, and NTDs may have contributed to her noncompliance with daily multivitamin consumption. As a result, her potential problems with folate absorption could have contributed to her subsequently giving birth to a child with a myelomeningocele. Thus, patient awareness and counseling along with aggressive vitamin supplementation among this particular population may help prevent the occurrence of NTDs after gastric bypass surgery.