Newborn screening for Cerebrotendinous Xanthomatosis: A retrospective biomarker study using both flow-injection and UPLC-MS/MS analysis in 20,000 newborns.

Background And Aims: Cerebrotendinous Xanthomatosis (CTX) is a treatable disorder of bile acid synthesis caused by deficiency of 27-sterol hydroxylase -encoded by CYP27A1- leading to gastrointestinal and progressive neuropsychiatric symptoms. Biochemically, CTX is characterized by accumulation of the bile alcohol cholestanetetrol glucuronide (GlcA-tetrol) and the deficiency of tauro-chenodeoxycholic acid (t-CDCA) and tauro-trihydroxycholestanoic acid (t-THCA).

Materials And Methods: To ascertain the feasibility of CTX newborn screening (NBS) we performed a study with deidentified Dutch dried blood spots using reagents and equipment that is frequently used in NBS laboratories.

Assessment of induction of secondary tumours due to various radiotherapy modalities.

One of the objectives of the European Sixth Framework integrated project MAESTRO is to perform an assessment of risk due to various radiotherapy modalities, regarding secondary tumour induction. Initially, the study will focus on cancer of the prostate and the present work represents the first step towards that goal. One of the intended tools, to be used in the assessment, is the Monte Carlo radiation transport code ORANGE.

ORANGE: a Monte Carlo dose engine for radiotherapy.

This study presents data for the verification of ORANGE, a fast MCNP-based dose engine for radiotherapy treatment planning. In order to verify the new algorithm, it has been benchmarked against DOSXYZ and against measurements. For the benchmarking, first calculations have been done using the ICCR-XIII benchmark.

Radiation synovectomy of the ankle with 75 MBq colloidal 186rhenium-sulfide: effect, leakage, and radiation considerations.

Objective: In a retrospective study we evaluated the effect, duration of effect, and safety of radiosynoviorthesis of the ankle in patients with persistent synovitis, refractory to disease modifying antirheumatic drugs (DMARD) and intraarticular glucocorticoid injections. We estimated leakage and dose to target and non-target organs.

Methods: Radiation synovectomy was performed by injection of 75 MBq 186rhenium colloid and 20 mg triamcinolone-hexacetonide mixed in a volume of about 1.

Scattered radiation from applicators in clinical electron beams.

In radiotherapy with high-energy (4-25 MeV) electron beams, scattered radiation from the electron applicator influences the dose distribution in the patient. In most currently available treatment planning systems for radiotherapy this component is not explicitly included and handled only by a slight change of the intensity of the primary beam. The scattered radiation from an applicator changes with the field size and distance from the applicator.