Ascending aortic aneurysm after pediatric heart transplantation: case report of an unusual complication.

A 28-month-old boy, weighing 11 kg, with severe dilated cardiomyopathy, was transplanted on December 1995. Hypertension and supraventricular tachycardia were detected in the immediate post-operative period, with favorable outcome. After 5 months of clinically asymptomatic follow-up, a dilation in the ascending aorta was observed on routine echocardiogram. Nuclear magnetic resonance imaging (NMRI) confirmed an ascending aortic aneurysm, with a diameter of 38 mm. An operation was performed, a bovine pericardium patch was sutured with reconstruction of the aortic wall, excluding the aneurysm. Good recovery was obtained and the child was discharged on Day 7 postoperatively. A post-operative echocardiogram showed absence of the aortic aneurysm and good surgical results. Another NMRI was done 5 months later, showing an intact ascending aorta. After 64 months, the patients clinical condition was confirmed as normal by echocardiogram. Surgical treatment was successful and the positive results have been maintained.     

Long-term biocompatibility of implanted polymer-based intrafascicular electrodes

Polymer-based longitudinal intrafascicular electrodes (polyLIFEs) were chronically implanted into the sciatic nerve of white New Zealand rabbits (n=8) for a period of 6 months (hereafter referred to as the long-term group). The impact of the implantation procedure, as observed 6 months post surgery, was evaluated in a sham-treated control group (n=9). The contralateral sciatic nerve served as the control for each animal. Nerve-fiber counts, fiber diameters, and myelin thickness were estimated at the level of the implant site, 1.5 cm proximally, and 1.5 cm distally for both nerves in sham-treated and long-term groups. Implantation of polyLIFEs had no significant effect on fiber counts, nerve-fiber diameter, or myelin thickness. A slight increase in connective tissue in the vicinity of the implant site was evident in the long-term group, including a thin but dense capsule immediately surrounding the implanted electrode.     

Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team

We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginning of adult care (SP2). Retrospective data on metabolic control and number of clinic appointments were collected for each SP, and protein intakes were analysed. In SP2, three patients (6%) were lost to follow-up. There was a small but statistically significant increase in median number of annual blood spots from SP1 to SP2: 11 (7–15) vs. 14 (7–20); p = 0.002. Mean ± SD of median blood Phe remained stable (525 ± 248 µmol/L vs. 552 ± 225 µmol/L; p = 0.100); median % of blood Phe < 480 µmol/L decreased (51 (4–96)% vs. 37 (5–85)%; p = 0.041) and median number of clinic appointments increased from SP1 to SP2: (5 (4–6) vs. 11 (8–13); p < 0.001). No significant differences were found regarding any parameter of protein intake. Our results suggest that the implementation of an adult service was successful as impact on metabolic control was limited and attendance remained high. Continuous dietetic care likely contributed to these results by keeping patients in follow-up and committed to treatment.     

Inadequacy of combined 5-Fluorouracil, very-high-dose leucovorin, mitomycin-C and Cisplatin as 2nd-line therapy for advanced colon-cancer

Fourteen patients with metastatic colon cancer which had progressed under fluoro-folates chemotherapy were treated with MM-C (10 mg/m(2) on day 1), 5-FU (370 mg/m(2) for 5 consecutive days), l-LV (500 mg/m(2) for 5 consecutive days) and CDDP (70 mg/m(2) on day 4), every 29th day till further disease progression. Neither CR nor PR were observed; 3 patients only had stable disease, while the extant 11 patients progressed. Resulting toxicity was severe. Since no antitumor effects were evidenced in the first 14 patients, the study was discontinued according to commonly accepted criteria.     

A prospective study of obsessive‐compulsive and borderline personality traits,race and disordered eating

Most previous investigations of the relationship between personality and disordered eating have been cross‐sectional and have utilised relatively homogeneous samples. The current study sought to prospectively explore two specific putative personality risk factors for disordered eating in an ethnically diverse sample of women (n = 58). During the initial and 2‐year follow‐up assessments, semi‐structured diagnostic interviews were administered. Analyses revealed that borderline personality disorder symptoms, as well as an interaction between obsessive‐compulsive personality disorder symptoms and race, significantly predicted disordered eating over a 2‐year period. Obsessive‐compulsive personality pathology was most strongly predictive of disordered eating for the African American women, specifically. These data supported both borderline and obsessive‐compulsive personality pathology as predictors of the development of eating disorder symptoms among women over a 2‐year period in college. In addition, race may be an important consideration in understanding the role of personality in the aetiology of disordered eating. Copyright © 2007 John Wiley & Sons, Ltd and Eating Disorders Association.     

Sealability of MTA and calcium hydroxidecontaining sealers

Objectives

The aim of this study was to evaluate the apical sealability of Fillapex^®, Endo-CPM-Sealer^® and Sealapex^®.

Material and Methods

Ninety-four freshly extracted single-rooted teeth were selected and decoronated. All teeth were radiographed to confirm the existence of a single and straight root canal, which was prepared using Protaper Universal and 2.5% sodium hypochlorite. The teeth were randomly divided in groups of 10 specimens each according to the sealer, and the canals were filled using the single cone technique and one of the sealers. Four additional teeth were used as controls. The teeth were submitted to dye leakage with Rhodamine B for 24 h but using vacuum on the initial 15 min. Thereafter, they were cut longitudinally and the leakage was measured in a linear fashion from apex to crown. Data were analyzed by ANOVA and Tukey''s tests at 5% significance level.

Results

Fillapex^® and Sealapex^® showed significantly less dye leakage than Endo-CPM-Sealer^® (p<0.05).

Conclusions

It was concluded that Fillapex^® and Sealapex^® were able to prevent apical dye leakage differently from Endo-CPM-Sealer^®.     

Episodic ataxia and severe infantile phenotype in spinocerebellar ataxia type 14: expansion of the phenotype and novel mutations

Journal of Neurology - Spinocerebellar ataxia type 14 (SCA14) is a dominantly inherited neurological disorder characterized by slowly progressive cerebellar ataxia. SCA14 is caused by mutations in...     

POLR3A variants in hereditary spastic paraparesis and ataxia: clinical,genetic, and neuroradiological findings in a cohort of Italian patients

Neurological Sciences - Mutations in POLR3A are characterized by high phenotypic heterogeneity, with manifestations ranging from severe childhood-onset hypomyelinating leukodystrophic syndromes to...