Advantages of digital technology in the assessment of bone marrow involvement in Gaucher's disease.

Gaucher disease (GD) is a genetic lysosomal disorder characterized by high bone marrow (BM) involvement and skeletal complications. The pathophysiology of these complications is not fully elucidated. Magnetic resonance imaging (MRI) is the gold standard to evaluate BM. This study aimed to apply machine-learning techniques in a cohort of Spanish GD patients by a structured bone marrow MRI reporting model at diagnosis and follow-up to predict the evolution of the bone disease. In total, 441 digitalized MRI studies from 131 patients (M: 69, F:62) were reevaluated by a blinded expert radiologist who applied a structured report template. The studies were classified into categories carried out at different stages as follows: A: baseline; B: between 1 and 4 y of follow-up; C: between 5 and 9 y; and D: after 10 years of follow-up. Demographics, genetics, biomarkers, clinical data, and cumulative years of therapy were included in the model. At the baseline study, the mean age was 37.3 years (1-80), and the median Spanish MRI score (S-MRI) was 8.40 (male patients: 9.10 vs. female patients: 7.71) (p < 0.001). BM clearance was faster and deeper in women during follow-up. Genotypes that do not include the c.1226A>G variant have a higher degree of infiltration and complications (p = 0.017). A random forest machine-learning model identified that BM infiltration degree, age at the start of therapy, and femur infiltration were the most important factors to predict the risk and severity of the bone disease. In conclusion, a structured bone marrow MRI reporting in GD is useful to standardize the collected data and facilitate clinical management and academic collaboration. Artificial intelligence methods applied to these studies can help to predict bone disease complications.

Structured bone marrow report as an assessment tool in patients with hematopoietic disorders.

Background: There are multiple hematological and other entities (metastases, infections) that can affect the bone marrow (BM). The gold standard imaging technique for BM examination is magnetic resonance imaging (MRI). Technological advances have made it possible to digitalize image files and create applications that help to produce higher quality structured reports, facilitating the analysis of data and unifying the criteria collected, making it possible to fill an existing gap. The aim of this study is to present a structured report model applicable to BM studies by MRI. Methods: We have carried out a systematic review following the recommendations of the PRISMA checklist report to explore previous publications applying structured BM MRI reporting. Eligibility criteria: the selection of articles carried out by MeSH thesaurus. Original or review articles of BM pathology assessed by MRI. Our group with a wide experience in the evaluation of BM by MRI have designed a model for BM report using eight items: demographic data, diagnostic suspicion, technical data, type of exam initial or control, distribution and patterns involvement, complications and location, total assessment comments. Results: We have not found articles that reflect the existence of a structured report of BM examination by MRI. Only one descriptive article has been identified on guidelines for acquisition, interpretation and reporting which refers to a single entity. With the selected parameters, a software has been developed that allows to fill in the sections of the structured report with ease and immediacy and to send the result directly to the clinician. Discussion: Structured reports are the result of applying a logical structure to the radiological report, and the rules of elaboration comprise several criteria: (I) using a uniform language. The standardization of terminology avoids ambiguity in reporting and makes it easier to compare reports. (II) Accurately describe the radiological findings, following a prescribed order with review questions and answers. (III) Drafting using diagnostic screening tables. (IV) Respect the radiologists' workflow by facilitating the work and not hindering it. The final report of this work has been the product of the clinical-radiological collaboration in our working group.

Anatomical analysis of the pelvis to identify any predisposing anatomical factors for ischiofemoral space pathology: a retrospective study.

OBJECTIVE: Anatomical analysis of the hips and pelvis was performed using MRI to evaluate morphological characteristics and associations between them. We identified correlations between the ischiofemoral space (IFS), quadratus femoris space (QFS), femoral version angle (FVA) and cervicodiaphyseal angle (CDA). METHODS: This study involved a retrospective search of a database of consecutive reports of adult hip MRI examinations carried out between January and September 2016. Patients with a medical history likely to affect pelvic and hip morphometry were excluded. RESULTS: A total of 137 adult patients were enrolled in the study (45.3% males and 54.7% females), with a mean age of 50.16 ± 13.87 years. The mean IFS was 20.88 ± 5.96 mm, mean QFS was 15.2 ± 6.18 mm, mean FVA was 12.43 ± 6.98, and mean CDA was 121.27 ± 4.6°. The IFS measurements were significantly correlated with femoral measurements (p = 0.025). These visible differences showed a slight negative relationship (-0.191), and females had a smaller distance between these anatomical structures than males (p < 0.001). Females had a significantly smaller QFS than males (12.42 ± 5.94 vs 18.73 ± 4.48 mm, p = 0.000). There was a small but significant positive relationship between CDA and FVA (p = 0.022), with a correlation coefficient of 0.195. CONCLUSION: A higher FVA was correlated with a smaller IFS. Furthermore, an increase in the CDA appeared in tandem with an increase in the FVA. ADVANCES IN KNOWLEDGE: A single conventional MRI sequence can alert us to how anatomical factors could predispose individuals to a decrease in IFS.

Etravirina en primeras líneas de tratamiento / Etravirine in first-line therapy

Etravirina (ETR) es un inhibidor de la transcriptasa inversa no análogo de nucleósidos (ITINAN), con potente y amplia actividad in vitro frente al virus de la inmunodeficiencia humana 1 y a virus con resistencias a ITINAN, lo que permite el uso secuencial de esta familia. La potencia, eficacia y seguridad están demostradas en pacientes multitratados, pero se dispone de pocos datos en primeras líneas de tratamiento antirretroviral (TAR), sin estar definido su papel en fases iniciales. La presencia de resistencias a ITINAN primarias y adquiridas durante la primera línea de tratamiento es cada vez más frecuente. Gracias a su barrera genética y eficacia, ETR puede formar parte de un segundo régimen de TAR en pacientes que han fallado en un primer régimen. En fases iniciales, los efectos adversos siguen siendo el motivo principal por el que se modifica el TAR. ETR se ha mostrado segura y con buena tolerabilidad. No presenta efectos adversos en el sistema nervioso central y tiene un buen perfil hepático, lipídico y gastrointestinal. El efecto adverso más frecuente es el exantema, efecto adverso común al resto de ITINAN. Su buen perfil de tolerabilidad lo convierte en un fármaco que se debe considerar en la configuración de un nuevo tratamiento en cambios por tolerabilidad. Las características de ETR, posibilidad de administración 1 vez al día, posibilidad de administrar disuelta en agua y no interacción con metadona, hacen que sea un fármaco especialmente atractivo en primeras líneas de tratamiento y en pacientes a los que se les ha atribuido mala adherencia, como son los usuarios de drogas por vía parenteral en tratamiento con metadona

Etravirine (ETR) is a non-nucleoside reverse transcriptase inhibitor (NNRTI) with a potent and broad in vitro spectrum of activity against HIV-1 and viruses with NNRTI resistances, allowing sequential use of drugs of this family. The potency, efficacy and safety of etravirine have been demonstrated in multi-treated patients, but few data are available on first-line antiretroviral therapy (ART) and the role of this drug in initial treatment phases has not been defined. The presence of primary NNRTI resistances and those acquired during first-line therapy is increasingly frequent. Due to its genetic barrier and efficacy, ETR can form part of a second-line ART regimen in patients with failure to a first-line regimen. In the initial phases, adverse effects continue to be the main reason for modifying ART. ETR has demonstrated safety and tolerability, with no central nervous system adverse effects and a good liver, lipid and gastrointestinal safety profile. As with the other NNRTIs, the most common adverse effect is rash. Because of ETR good tolerability profile, this drug can be considered when a new treatment is required due to adverse effects. Because of the characteristics of ETR the possibility of once-daily administration and dissolution in water, as well as the absence of drug-drug interactions with methadone this drug is especially attractive as a firstline therapy and in patients with poor adherence, such as intravenous drug users receiving methadone treatment