From Clinical Phenotype to Genotypic Modelling: Incidence and Prevalence of Recessive Dystrophic Epidermolysis Bullosa (RDEB) [Corrigendum]

Eichstadt S, Tang JY, Solis DC, et al. Clin Cosmet Investig Dermatol. 2019;12:933– 942. There is an error with Table 5 on page 940. The authors have advised due to an error that occurred inadvertently at the time of assembling the tables, Tables 4 and 5 were duplicated. The correct Table 5 is shown below. The authors apologize for this error.   Read the original articl

Multiple sclerosis registries in Europe – An updated mapping survey

There are significant geographical differences with the highest prevalence occurring in Northern Europe and North America (over 100/100,000) as compared to 2/100,000 in Japan . MS patients are typically diagnosed between the ages of 20 and 50 years and will in most cases be expected to live for a long time with MS. The value of MS registries for MS patients, health care providers and regulators is becoming increasingly recognised . The collection of patient data into a registry can benefit the patients by enabling the clinician to prospectively follow the individual patient concerning disease progression, treatment response and side effects, and other parameters including patient reported outcomes (PRO) measures . From a societal viewpoint there are also many advantages of using data from MS registries, especially within areas of epidemiology and treatment studies, such as post marketing effectiveness, comparative or post-authorisation safety studies . This was recognised by regulatory bodies in the EU resulting in the first EMA-Workshop on MS-Registries in July 2017 in London . Therefore it is important to improve and promote the collection of safety data by MS registries as this might provide an opportunity for the MS registries to replace the typical drug specific post-authorization safety studies

99mTc-2-Methoxy-Isobutyl-Isonitril in der Diagnostik der koronaren Herzkrankheit: Ergebnisse einer Multizenterstudie. [99mTc-2-methoxy-isobutyl-isonitrile in the diagnosis of coronary heart disease: results of a multicenter study]

A total of 226 patients was studied in a phase-III multicenter trial to compare the results of stress/rest 99mTc-MIBI with 201TI scintigraphy and/or coronary arteriography. The evaluation of planar and SPECT imaging was based on a semiquantitative visual scoring. A congruent diagnosis was generated from all planar 201TI and 99mTc-MIBI scintigrams performed in 36 patients. In 62 of 63 patients, SPECT-studies with 99mTc-MIBI and 201TI led to an identical diagnosis. In one patient a positive result was obtained with 201TI-SPECT whereas 99mTc-MIBI was negative. Segmental agreement for 1509 SPECT segments was 85.7%, for 554 planar segments it was 86.6%. In comparison to coronary arteriography, performed in 180 patients, the overall sensitivity for the correct diagnosis of coronary artery disease (CAD) was 86% for planar MIBI scintigraphy or 92% for MIBI-SPECT. Overall specificity was 100% for planar imaging and 57% for SPECT. The low specificity of MIBI-SPECT was probably due to high prevalence of CAD in this study population. In a subgroup of 43 patients, who underwent coronary angiography, identical results were found with 99mTc-MIBI and 201TI. Only one patient showed a positive scintigraphic result with both 99mTc-MIBI and 201TI without angiographically proven stenosis greater than 50% or prior myocardial infarction. Vessel sensitivities in stenosed coronary arteries (greater than 50% stenosis) were 54% for the LAD and 87% for the RCX/RCA areas with MIBI-SPECT, or 51% or 79% for planar scintigraphy, respectively. Vessel specificities for SPECT were 65% in the LAD or 52% in the RCX/RCA, or 90% or 83% in planar imaging.(ABSTRACT TRUNCATED AT 250 WORDS