Pediatric lung transplantation: Literature review 2005

This review summarizes important original articles in the field of pediatric lung transplantation that were published in 2005. The review is intended to be comprehensive, but not exhaustive.     

Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD)

Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD) have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE) may account for an alarming number of deficits in this population. In the current brief review, we critically evaluated the pediatric and adult literatures on the neurocognitive effects of SCD. We highlighted the studies that have been published on this topic and posit that early detection of CVE via neurocognitive testing, neuropsychiatric evaluations, and neuroimaging may significantly reduce adult cognitive and functional morbidities.     

EBV+ B Lymphoma Cell Lines from Patients with Post-Transplant Lymphoproliferative Disease Are Resistant to TRAIL-Induced Apoptosis

Lymphomas associated with post-transplant lymphoproliferative disease (PTLD) represent a significant complication of immunosuppression in transplant recipients. In immunocompetent individuals, EBV-specific cytotoxic T lymphocytes (CTL) prevent the outgrowth of activated B lymphoblasts through apoptosis induction. Soluble versions of TNF-related apoptosis-inducing ligand/Apo2 ligand (TRAIL) can induce apoptosis in numerous tumor cell types. Given the therapeutic potential of TRAIL, we examined the sensitivity of EBV⁺ spontaneous lymphoblastoid cell lines (SLCL) derived from patients with PTLD to treatment with soluble TRAIL. Despite abundant expression of TRAIL receptors (TRAIL-R), resistance to TRAIL-induced apoptosis was observed in all SLCL examined. This resistance could not be overcome by concomitant treatment with several pharmacological agents. Unlike BJAB positive control cells, for each SLCL tested, cleavage and activation of caspase 8 was inhibited due to failed recruitment of FADD and caspase 8 to TRAIL receptors upon stimulation. Further indicative of a proximal defect, TRAIL receptor aggregation could not be detected on the cell surface of SLCL following ligand engagement. These results suggest that the use of TRAIL for eliminating PTLD-associated tumors may be of limited clinical utility, and illustrate another mechanism by which EBV⁺ B lymphoma cells can evade tumor surveillance at the level of death receptor signaling.     

Long-term survival in children under 3 years of age with low-grade astrocytoma

Objective The purpose of this study is to analyze clinical aspects and disease-free survival (DFS) in children less than 3 years of age diagnosed with low-grade astrocytoma. Methods In a period of 24 years (1980–2004), a total of 43 (5.4%) children were registered with these characteristics. Twenty-three patients had pilocytic astrocytoma, 18 diffused, and 2 mixed. Thirty-one (72.1%) children had incomplete surgical tumor resection and 12 (27.9%) had a complete tumor resection. Twelve (27.9%) patients had cranial radiotherapy and 17 (39.5%) received chemotherapy. Overall survival was recorded in 23 (53%). DFS was 50% at 250 months of follow-up for the whole group. DFS for the supratentorial group was 60% at 250 months, whereas, for the infratentorial, it was 22% at 120 months (p = 0.008). Conclusion The only favorable prognostic pattern was the supratentorial presentation. Radiotherapy and chemotherapy did not alter the outcome.     

Effect of dietary fat content on the bioavailability of a sustained release quinidine gluconate tablet

Quinidine gluconate 324 mg sustained release tablets (Quinaglute) was administered as a single dose to 15 healthy male subjects following an overnight fast, immediately following a high fat (HF) breakfast or immediately following a low fat (LF) breakfast. Serum samples were obtained over a 48 h period and analyzed for quinidine content using a high performance liquid chromatographic assay. Under the conditions of the study, both the rate and extent of quinidine bioavailability was significantly affected by food. The extent of bioavailability was statistically significantly greater (p less than 0.05) following both the HF and LF meals as compared to that in the fasted state. Rate of bioavailability was significantly enhanced following the LF meal as compared to that of the other two treatment groups. Although peak concentrations were greater and time to peak concentrations somewhat later following the HF meal versus those under fasting conditions, these differences were not statistically significant. In addition, the characteristics of the serum concentration-time profile (as defined by the number, magnitude, and time of occurrence of the multiple absorption maxima) was unique for each of the three treatment groups. Possible mechanisms underlying these results are explored.     

Arylsulfatase A in urine of patients with urothelial tumors

A significant increase in urine arylsulfatase A activity (p less than 0.01) was found in patients with urothelial tumors. Arylsulfatase A activity was 1.36 +/- 1.10 U/24-h urine in control specimens, 1.90 +/- 1.66 U/24-h urine in various genitourinary tract disorders, and 3.90 +/- 1.98 U/24-h urine in transitional cell carcinoma specimens. Surgical treatment of the neoplastic patients lowered the arylsulfatase A activity found in urine to within reference values. The arylsulfatase A excreted by patients with these tumors was highly sensitive to thermal inactivation while the enzyme activity in the control urines was less affected by the heat treatment. The time course of the arylsulfatase A reaction with 4-nitrocatechol sulfate was not linear in normal individuals, while it was linear in 90% of patients with urothelial tumors. This difference in the kinetic pattern of the enzyme could be used to increase the diagnostic specificity of the determination.     

Barley diets with different fat sources have hypocholesterolemic effects in chicks.

Saturated fat is known to elevate serum cholesterol, whereas soluble dietary fiber has a hypocholesterolemic effect. The objective of this study was to compare the effects of barley and wheat diets supplemented with five fat sources on lipid metabolism in chicks. In two separate experiments, broiler chicks were fed isonitrogenous diets containing 23% protein, 11.4% dietary fiber and 10% dietary fat for 17 d. Diets contained 60% either hull-less barley or red spring wheat, with either palm oil, dehydrated egg yolk, butter, tallow or corn oil. Growth, feed efficiency, plasma lipids, liver cholesterol, and fecal fat and dry matter were measured. All chicks fed wheat grew faster and had greater food efficiency than those fed barley. All barley-fed chicks had lower (P less than 0.0001) total plasma cholesterol concentration (3.1 to 4.0 mmol/L) than those fed wheat (6.0 to 11.3 mmol/L). Chicks fed palm oil with wheat had the highest total cholesterol, 11.3 mmol/L. Liver cholesterol concentration was higher (P less than 0.0001) for all wheat-fed chicks (22.8-86.4 mmol/g) compared with those fed barley (6.7 to 12.2 mmol/g). Fecal crude fat was higher (P less than 0.05) for chicks fed barley, and excreta dry matter was lower for barley-fed chicks. Results indicate that the high soluble fiber content of this barley exerts a hypocholesterolemic effect in chicks regardless of dietary fat source, possibly mediated through lowered fat absorption.     

3D imaging and pathology of the base of the skull in children]

Three dimensional computed tomography was performed in 150 children suffering from a bone lesion of the base of the skull and face. These lesions were either malformative, neoplastic, infectious or traumatic. 3D CT provided an essential anatomical approach to the lesions facilitating the choice of the most appropriate treatment. 3D CT is also indicated in the follow-up of these operated children, particularly of the growth of the operated bone segment and its relations with adjacent structures.