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1.
Eur Respir J ; 64(3)2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38901883

RESUMEN

BACKGROUND: We recently demonstrated that elexacaftor/tezacaftor/ivacaftor (ETI) improves the lung clearance index (LCI) and abnormalities in lung morphology detected by magnetic resonance imaging (MRI) in adolescent and adult patients with cystic fibrosis (CF). However, real-world data on the effect of ETI on these sensitive outcomes of lung structure and function in school-age children with CF have not been reported. The aim of this study was therefore to examine the effect of ETI on the LCI and the lung MRI score in children aged 6-11 years with CF and one or two F508del alleles. METHODS: This prospective, observational, multicentre, post-approval study assessed the longitudinal LCI up to 12 months and the lung MRI score before and 3 months after initiation of ETI. RESULTS: A total of 107 children with CF including 40 heterozygous for F508del and a minimal function mutation (F/MF) and 67 homozygous for F508del (F/F) were enrolled in this study. Treatment with ETI improved the median (interquartile range (IQR)) LCI in F/MF (-1.0 (-2.0- -0.1); p<0.01) and F/F children (-0.8 (-1.9- -0.2); p<0.001) from 3 months onwards. Further, ETI improved the median (IQR) MRI global score in F/MF (-4.0 (-9.0-0.0); p<0.01) and F/F children (-3.5 (-7.3- -0.8); p<0.001). CONCLUSIONS: ETI improves early abnormalities in lung ventilation and morphology in school-age children with CF and at least one F508del allele in a real-world setting. Our results support early initiation of ETI to reduce or even prevent lung disease progression in school-age children with CF.


Asunto(s)
Alelos , Aminofenoles , Benzodioxoles , Regulador de Conductancia de Transmembrana de Fibrosis Quística , Fibrosis Quística , Indoles , Pulmón , Imagen por Resonancia Magnética , Pirazoles , Quinolonas , Humanos , Niño , Fibrosis Quística/tratamiento farmacológico , Fibrosis Quística/genética , Fibrosis Quística/diagnóstico por imagen , Femenino , Masculino , Aminofenoles/uso terapéutico , Quinolonas/uso terapéutico , Estudios Prospectivos , Indoles/uso terapéutico , Benzodioxoles/uso terapéutico , Pulmón/diagnóstico por imagen , Pulmón/efectos de los fármacos , Pulmón/fisiopatología , Pirazoles/uso terapéutico , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Combinación de Medicamentos , Mutación , Piridinas/uso terapéutico , Pirrolidinas/uso terapéutico , Homocigoto
2.
Pediatr Radiol ; 54(2): 260-268, 2024 02.
Artículo en Inglés | MEDLINE | ID: mdl-37985493

RESUMEN

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.


Asunto(s)
Trasplante de Hígado , Radiología , Niño , Humanos , Ultrasonografía , Tomografía Computarizada por Rayos X , Imagen por Resonancia Magnética/métodos
3.
BMC Pulm Med ; 22(1): 424, 2022 Nov 17.
Artículo en Inglés | MEDLINE | ID: mdl-36397043

RESUMEN

BACKGROUND: Massive hemoptysis is a rare but potentially life-threatening condition of patients with cystic fibrosis (CF) and advanced pulmonary disease. Hypertrophied bronchial arteries are understood to cause massive hemoptysis when rupturing. Risk factors to predict massive hemoptysis are scarce and bronchial artery diameters are not part of any scoring system in follow-up of patients with CF. Aim of this study was to correlate bronchial artery diameter with massive hemoptysis in CF. METHODS: Bronchial artery and non-bronchial systemic artery diameters were measured in contrast enhanced computed tomography (CT) scans in patients with massive hemoptysis and compared to patients with end-stage CF and no history of hemoptysis. Demographic and clinical data and side of bronchial artery/non-bronchial systemic artery hypertrophy and coil embolization were documented. RESULTS: In this retrospective multicenter study 33 patients with massive hemoptysis were included for bronchial artery/non-bronchial systemic artery diameter measurements, (13 female, 20 male, median age 30 years (18-55)). Bronchial artery diameters were significantly larger in the case group than in the control group with median 4 mm (2.2-8.2 mm), and median 3 mm (1-7 mm), respectively (p = 0.002). Sensitivity of bronchial arteries ≥ 3.5 mm to be associated with hemoptysis was 0.76 and specificity 0.71 with ROC creating an area under the curve of 0.719. If non-bronchial systemic arteries were present, they were considered culprit and embolized in 92% of cases. CONCLUSION: Bronchial arteries ≥ 3.5 mm and presence of hypertrophied non-bronchial systemic arteries correlate with massive hemoptysis in patients with CF and might serve as risk predictor for massive hemoptysis. Therefore, in patients with advanced CF we propose CT scans to be carried out as CT angiography to search for bronchial arteries ≥ 3.5 mm and for hypertrophied non-bronchial systemic arteries as possible risk factors for massive hemoptysis.


Asunto(s)
Fibrosis Quística , Embolización Terapéutica , Humanos , Masculino , Femenino , Adulto , Arterias Bronquiales/diagnóstico por imagen , Fibrosis Quística/complicaciones , Embolización Terapéutica/métodos , Hemoptisis/etiología , Hemoptisis/terapia , Angiografía/efectos adversos , Angiografía/métodos
4.
Sci Rep ; 12(1): 18913, 2022 11 07.
Artículo en Inglés | MEDLINE | ID: mdl-36344592

RESUMEN

Up to now, there is only limited information available on a possible relationship between clinical characteristics and the mineralization of metacarpal bones and finger joint space distance (JSD) in patients with psoriatic arthritis (PsA). Computerized digital imaging techniques like digital X-ray radiogrammetry (DXR) and computer-aided joint space analysis (CAJSA) have significantly improved the structural analysis of hand radiographs and facilitate the recognition of radiographic damage. The objective of this study was to evaluate clinical features which potentially influence periarticular mineralization of the metacarpal bones and finger JSD in PsA-patients. 201 patients with PsA underwent computerized measurements of the metacarpal bone mineral density (BMD) with DXR and JSD of all finger joints by CAJSA. DXR-BMD and JSD were compared with clinical features such as age and sex, disease duration, C-reactive protein (CRP) as well as treatment with prednisone and disease-modifying antirheumatic drugs (DMARDs). A longer disease duration and an elevated CRP value were associated with a significant reduction of DXR-BMD, whereas JSD-parameters were not affected by both parameters. DXR-BMD was significantly reduced in the prednisone group (-0.0383 g/cm²), but prednisone showed no impact on finger JSD. Patients under the treatment with bDMARDs presented significant lower DXR-BMD (-0.380 g/cm²), JSDMCP (-0.0179 cm), and JSDPIP (-0.0121 cm) values. Metacarpal BMD was influenced by inflammatory activity, prednisone use, and DMARDs. In contrast, finger JSD showed only a change compared to baseline therapy. Therefore, metacarpal BMD as well as finger JSD represent radiographic destruction under different aspects.


Asunto(s)
Antirreumáticos , Artritis Psoriásica , Artritis Reumatoide , Humanos , Artritis Psoriásica/diagnóstico por imagen , Artritis Psoriásica/tratamiento farmacológico , Artritis Psoriásica/complicaciones , Artritis Reumatoide/tratamiento farmacológico , Prednisona/uso terapéutico , Antirreumáticos/uso terapéutico , Densidad Ósea , Absorciometría de Fotón
5.
Am J Respir Crit Care Med ; 206(3): 311-320, 2022 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-35536314

RESUMEN

Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in airway and intestinal epithelia to 40-50% of normal in patients with cystic fibrosis (CF) with one or two F508del alleles. In previous studies, this improvement of CFTR function was shown to improve clinical outcomes; however, effects on the lung clearance index (LCI) determined by multiple-breath washout and abnormalities in lung morphology and perfusion detected by magnetic resonance imaging (MRI) have not been studied. Objectives: To examine the effect of ELX/TEZ/IVA on LCI and lung MRI scores in patients with CF and one or two F508del alleles aged ⩾12 years. Methods: This prospective, observational, multicenter, postapproval study assessed LCI and lung MRI scores before and 8-16 weeks after initiation of ELX/TEZ/IVA. Measurements and Main Results: A total of 91 patients with CF, including 45 heterozygous for F508del and a minimal function mutation (MF) and 46 homozygous for F508del, were enrolled in this study. Treatment with ELX/TEZ/IVA improved LCI in F508del/MF (-2.4; interquartile range [IQR], -3.7 to -1.1; P < 0.001) and F508del homozygous (-1.4; IQR, -2.4 to -0.4; P < 0.001) patients. Furthermore, ELX/TEZ/IVA improved the MRI global score in F508del/MF (-6.0; IQR, -11.0 to -1.3; P < 0.001) and F508del homozygous (-6.5; IQR, -11.0 to -1.3; P < 0.001) patients. Conclusions: Our data demonstrate that improvement of CFTR function by ELX/TEZ/IVA improves lung ventilation and abnormalities in lung morphology, including airway mucus plugging and wall thickening, in adolescent and adult patients with CF and one or two F508del alleles in a real-world, postapproval setting. Clinical trial registered with www.clinicaltrials.gov (NCT04732910).


Asunto(s)
Regulador de Conductancia de Transmembrana de Fibrosis Quística , Fibrosis Quística , Adolescente , Adulto , Anciano , Alelos , Aminofenoles/uso terapéutico , Benzodioxoles/uso terapéutico , Fibrosis Quística/diagnóstico por imagen , Fibrosis Quística/tratamiento farmacológico , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/uso terapéutico , Humanos , Indoles , Pulmón/diagnóstico por imagen , Imagen por Resonancia Magnética , Mutación , Estudios Prospectivos , Pirazoles , Piridinas , Pirrolidinas , Quinolonas
6.
Eur Radiol ; 32(3): 1833-1842, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-34668994

RESUMEN

OBJECTIVES: To compare the diagnostic value of ultrashort echo time (UTE) magnetic resonance imaging (MRI) for the lung versus the gold standard computed tomography (CT) and two T1-weighted MRI sequences in children. METHODS: Twenty-three patients with proven oncologic disease (14 male, 9 female; mean age 9.0 + / - 5.4 years) received 35 low-dose CT and MRI examinations of the lung. The MRI protocol (1.5-T) included the following post-contrast sequences: two-dimensional (2D) incoherent gradient echo (GRE; acquisition with breath-hold), 3D volume interpolated GRE (breath-hold), and 3D high-resolution radial UTE sequences (performed during free-breathing). Images were evaluated by considering image quality as well as distinct diagnosis of pulmonary nodules and parenchymal areal opacities with consideration of sizes and characterisations. RESULTS: The UTE technique showed significantly higher overall image quality, better sharpness, and fewer artefacts than both other sequences. On CT, 110 pulmonary nodules with a mean diameter of 4.9 + / - 2.9 mm were detected. UTE imaging resulted in a significantly higher detection rate compared to both other sequences (p < 0.01): 76.4% (84 of 110 nodules) for UTE versus 60.9% (67 of 110) for incoherent GRE and 62.7% (69 of 110) for volume interpolated GRE sequences. The detection of parenchymal areal opacities by the UTE technique was also significantly higher with a rate of 93.3% (42 of 45 opacities) versus 77.8% (35 of 45) for 2D GRE and 80.0% (36 of 45) for 3D GRE sequences (p < 0.05). CONCLUSION: The UTE technique for lung MRI is favourable in children with generally high diagnostic performance compared to standard T1-weighted sequences as well as CT. Key Points • Due to the possible acquisition during free-breathing of the patients, the UTE MRI sequence for the lung is favourable in children. • The UTE technique reaches higher overall image quality, better sharpness, and lower artefacts, but not higher contrast compared to standard post-contrast T1-weighted sequences. • In comparison to the gold standard chest CT, the detection rate of small pulmonary nodules small nodules ≤ 4 mm and subtle parenchymal areal opacities is higher with the UTE imaging than standard T1-weighted sequences.


Asunto(s)
Imagenología Tridimensional , Imagen por Resonancia Magnética , Adolescente , Contencion de la Respiración , Niño , Preescolar , Femenino , Humanos , Pulmón/diagnóstico por imagen , Masculino , Tomografía Computarizada por Rayos X
7.
Med Ultrason ; 24(1): 27-32, 2022 Feb 16.
Artículo en Inglés | MEDLINE | ID: mdl-34508619

RESUMEN

AIMS: To evaluate the safety of the contrast enhanced voiding urosonography (ceVUS) and contrast enhanced ultrasound (CEUS) in children and adolescence and to receive data about parents' acceptance of intravesical and intravenous application of sulfur hexafluoride. MATERIAL AND METHODS: In this prospective, single centre study conducted over a 1 year study period, parents of 56 children (f/m=32/24; mean age 3.1 years; range 3 weeks - 15.9 years) with ceVUS and of 30 children (f/m=15/15; mean age 10.5 years; range 2 months - 17.7 years) with CEUS agreed to be included. A standardized telephone survey about the acceptance of the parents during and after the procedure as well as the adverse events (AE) were conducted within three days of the examination. RESULTS: The parents would agree with the use of both ceVUS and CEUS as a diagnostic tool again in 96% (54/56) or 100% (30/30) of the cases, respectively and 92.9% (52/56) would prefer ceVUS to voiding cystourethrography (VCUG). In addition, 83.3% (25/30) would prefer CEUS to CT and 73.3% (22/30) would prefer CEUS to MRI. AE were reported in 3.6% after ceVUS (2/56; skin rash, mild fever) and in 3.3% after CEUS (1/30; vomiting). AE were subacute and self­limited. CONCLUSIONS: The vast majority of parents prefer ceVUS and CEUS to VCUG, CT or MRI because of the safety profile of the contrast agent and diagnostic accuracy.


Asunto(s)
Medios de Contraste , Reflujo Vesicoureteral , Adolescente , Niño , Medios de Contraste/efectos adversos , Humanos , Recién Nacido , Padres , Estudios Prospectivos , Hexafluoruro de Azufre/efectos adversos , Ultrasonografía/métodos
8.
Arthritis Res Ther ; 22(1): 229, 2020 10 06.
Artículo en Inglés | MEDLINE | ID: mdl-33023661

RESUMEN

BACKGROUND: The reduction of finger joint space width (JSW) in patients with rheumatoid arthritis (RA) is strongly associated with joint destruction. Treatment with certolizumab pegol (CZP), a PEGylated anti-TNF, has been proven to be effective in RA patients. The computer-aided joint space analysis (CAJSA) provides the semiautomated measurement of joint space width at the metacarpal-phalangeal joints (MCP) based on hand radiographs. The aim of this post hoc analysis of the RAPID 1 trial was to quantify MCP joint space distance (JSD-MCP) measured by CAJSA between baseline and week 52 in RA patients treated with certolizumab pegol (CZP) plus methotrexate (MTX) compared with MTX/placebo. METHODS: Three hundred twenty-eight patients were included in the post hoc analysis and received placebo plus MTX, CZP 200 mg plus MTX and CZP 400 mg plus MTX. All patients underwent X-rays of the hand at baseline and week 52 as well as assessment of finger joint space narrowing of the MCP using CAJSA (Version 1.3.6; Sectra; Sweden). The joint space width (JSW) was expressed as mean joint space distance of the MCP joints I to V (JSD-MCPtotal). RESULTS: The MTX group showed a significant reduction of joint space of - 4.8% (JSD-MCPtotal), whereas in patients treated with CZP 200 mg/MTX and CZP 400 mg/MTX a non-significant change (JSD-MCPtotal + 0.6%) was observed. Over 52 weeks, participants with DAS28 remission (DAS28 ≤ 2.6) exhibited a significant joint space increase of + 3.3% (CZP 200 mg plus MTX) and + 3.9% (CZP pegol 400 mg plus MTX). CONCLUSION: CZP plus MTX did not reduce JSD-MCPtotal estimated by CAJSA compared with MTX/placebo. Furthermore, clinical remission (DAS28 ≤ 2.6) in patients treated with CZP plus MTX was associated with an increasing JSD, indicating radiographic remission in RA.


Asunto(s)
Antirreumáticos , Artritis Reumatoide , Antirreumáticos/uso terapéutico , Artritis Reumatoide/diagnóstico por imagen , Artritis Reumatoide/tratamiento farmacológico , Certolizumab Pegol/uso terapéutico , Computadores , Quimioterapia Combinada , Humanos , Metotrexato/uso terapéutico , Inducción de Remisión , Suecia , Resultado del Tratamiento , Inhibidores del Factor de Necrosis Tumoral
9.
Rheumatol Int ; 39(4): 637-645, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30569216

RESUMEN

The aim of this study, based on a post hoc analysis of the data set used in the RAPID 1 trial, focuses on the associations between metacarpal bone mineral density, as estimated by digital X-ray radiogrammetry (DXR), and clinical remission as well as ACR70-Response in rheumatoid arthritis (RA) patients treated with certolizumab pegol (CZP). The trial evaluates a total of 345 RA patients treated with methotrexate versus CZP 200 mg versus CZP 400 mg. All patients underwent X-rays of the hand at baseline and week 52 as well as computerized calculations of bone mineral density (BMD) by DXR. Clinical remission was defined as DAS28 < 2.6. ACR70-Response was also evaluated. The radiological assessment of disease progression was estimated using the modified total Sharp Score. The mean difference for DAS28 was observed for patients treated with CZP 400 mg (median: - 3.53, minimum: - 6.77; maximum: + 0.48) and CZP 200 mg (median: - 3.13, minimum: - 6.37; maximum: - 0.52) compared to the methotrexate group (median - 2.41, minimum: - 4.76; maximum: + 0.31). The DXR-BMD showed a minor bone loss for the treatment groups undergoing therapy with CZP 200 mg (median: - 0.009 g/cm2, minimum: - 0.059 g/cm2; maximum: + 0.095 g/cm2) and CZP 400 mg (median: - 0.008 g/cm2, minimum: - 0.064 g/cm2; maximum: + 0.080 g/cm2). The methotrexate group presented an advanced periarticular metacarpal bone loss as measured by DXR-BMD (median: - 0.024 g/cm2, minimum: - 0.102 g/cm2; maximum: + 0.057 g/cm2). In the case of clinical remission and ACR70-Response, no significant change of the DXR-BMD was observed for both CZP groups. The study highlights that patients treated with CZP show a less accentuated periarticular bone loss as estimated by DXR in comparison to patients with methotrexate plus placebo. In addition, patients with clinical remission and ACR70-Response revealed no periarticular demineralisation.


Asunto(s)
Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Densidad Ósea , Huesos del Metacarpo/diagnóstico por imagen , Inhibidores del Factor de Necrosis Tumoral/uso terapéutico , Adulto , Artritis Reumatoide/diagnóstico por imagen , Certolizumab Pegol/uso terapéutico , Femenino , Articulaciones de la Mano/diagnóstico por imagen , Humanos , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Intensificación de Imagen Radiográfica , Inducción de Remisión
10.
Acta Radiol ; 59(11): 1316-1325, 2018 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-29430936

RESUMEN

Background Endovascular aneurysm repair (EVAR) requires lifelong surveillance by computed tomography angiography (CTA). This is attended by a substantial accumulation of radiation exposure. Iterative reconstruction (IR) has been introduced to approach dose reduction. Purpose To evaluate adaptive statistical iterative reconstruction (ASIR) at different levels of tube voltage concerning image quality and dose reduction potential in follow-up post EVAR. Material and Methods One hundred CTAs in 67 patients with EVAR were examined using five protocols: protocol A (n = 40) as biphasic standard using filtered back projection (FBP) at 120 kV; protocols B (n = 40), C (n = 10), and D1 (n = 5) biphasic using ASIR at 120, 100, and 80 kV, respectively; and protocol D2 (n = 5) with a monophasic splitbolus ASIR protocol at 80 kV. Image quality was assessed quantitatively and qualitatively. Applied doses were determined. Results Applied doses in ASIR protocols were significantly lower than FBP standard (up to 75%). Compared to protocol A, signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) (e.g. arterial CNR intra-/extra-stent lumen: A = 35.4 ± 13.5, B = 34.2 ± 10.0, C = 29.6 ± 6.8, D1 = 32.1 ± 6.3, D2 = 40.8 ± 23.1) in protocol B were equal and in protocols C and D equal to partially inferior, however not decisive for diagnostic quality. Subjective image quality ratings in all protocols were good to excellent without impairments of diagnostic confidence (A-D2: 5), with high inter-rater agreement (60-100%). Conclusion ASIR contributes to significant dose reduction without decisive impairments of image quality and diagnostic confidence. We recommend an adapted follow-up introducing ASIR and combined low-kV in the long-term surveillance after EVAR.


Asunto(s)
Aneurisma de la Aorta Abdominal/diagnóstico por imagen , Aneurisma de la Aorta Abdominal/cirugía , Angiografía por Tomografía Computarizada/métodos , Procedimientos Endovasculares/métodos , Procesamiento de Imagen Asistido por Computador/métodos , Dosis de Radiación , Anciano , Aorta Abdominal/cirugía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Complicaciones Posoperatorias/diagnóstico por imagen , Estudios Prospectivos , Reproducibilidad de los Resultados , Resultado del Tratamiento
11.
Sci Rep ; 7(1): 17465, 2017 12 12.
Artículo en Inglés | MEDLINE | ID: mdl-29234058

RESUMEN

Abdominal symptoms are a hallmark of Cystic fibrosis (CF). Yet, their association with morphological abnormalities of different abdominal organs is still poorly understood. Aim was therefore to relate these symptoms, assessed with a questionnaire, to findings in abdominal ultrasound (US). In 114 CF patients of all ages, findings in US considering seventeen specific parameters were related to abdominal symptoms compiled with our novel CF-specific 26-modal symptom score (CFAbd-Score). US abnormalities were detected in 95% of the patients. Most frequent findings were pancreatic lipomatosis (88%), liver steatosis (37%), hepatomegaly (31%), and thickened bowel walls (23%). Highest burden of GI-symptoms was clearly associated with pancreatic lipomatosis (p = 0.036). In detail, patients revealing this pathology reported higher rates of abdominal pain (p = 0.018), flatulence (p = 0.006), heartburn (p = 0.04), and reflux of stomach content (p = 0.006). Patients with pancreatic sufficiency had less US-findings (p = 0.033), which in turn was associated with lower rates of abdominal symptoms. The majority of them were carriers of class IV-VI or G551D mutations. Our approach gives new insights regarding the underestimated multi-organ abdominal involvement in CF. The new score can be of high interest e.g. as a complementary tool to assess the gastrointestinal effects of promising novel CF therapeutics.


Asunto(s)
Abdomen/diagnóstico por imagen , Fibrosis Quística/diagnóstico , Ultrasonografía , Abdomen/fisiopatología , Adolescente , Niño , Preescolar , Fibrosis Quística/genética , Fibrosis Quística/fisiopatología , Fibrosis Quística/terapia , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Prospectivos , Índice de Severidad de la Enfermedad
12.
PLoS One ; 12(5): e0174463, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28472055

RESUMEN

BACKGROUND & AIMS: Abdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. METHODS: A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1.0) on abdominal pain and non-pain symptoms, disorders of appetite, eating, and bowel movements as well as symptom-related quality of life. Results were metrically dimensioned and related to abdominal manifestations, history of surgery, P. aeruginosa and S. aureus colonization, genotype, liver enzymes, antibiotic therapy, lung function, and nutritional status. RESULTS: AS during the preceding 3 months were reported by all of our patients. Most common were lack of appetite (130/131) and loss of taste (119/131) followed by abdominal pain (104/131), flatulence (102/131), and distention (83/131). Significantly increased AS were found in patients with history of rectal prolapse (p = 0.013), distal intestinal obstruction syndrome (p = 0.013), laparotomy (p = 0.022), meconium ileus (p = 0.037), pancreas insufficiency (p = 0.042), or small bowel resection (p = 0.048) as well as in patients who have been intermittently colonized with P. aeruginosa (p = 0.006) compared to patients without history of these events. In contrast, no statistically significant associations were found to CF-associated liver disease, chronic pathogen colonization, lung function, CF-related diabetes, and nutritional status. CONCLUSION: As the complex abdominal involvement in CF is still not fully understood, the assessment of the common AS is of major interest. In this regard, symptom questionnaires like the herein presented are meaningful and practical tools facilitating a wider understanding of the abdominal symptoms in CF. Furthermore, they render to evaluate possible abdominal effects of novel modulators of the underlying cystic fibrosis transmembrane (conductance) regulator (CFTR) defect.


Asunto(s)
Abdomen/fisiopatología , Fibrosis Quística/fisiopatología , Genotipo , Adolescente , Niño , Preescolar , Fibrosis Quística/genética , Fibrosis Quística/patología , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Femenino , Humanos , Lactante , Recién Nacido , Masculino
13.
Eur Radiol ; 27(7): 2752-2764, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-27896471

RESUMEN

OBJECTIVES: To compare the diagnostic performance of contrast-enhanced spectral mammography (CESM) to digital mammography (MG) and magnetic resonance imaging (MRI) in a prospective two-centre, multi-reader study. METHODS: One hundred seventy-eight women (mean age 53 years) with invasive breast cancer and/or DCIS were included after ethics board approval. MG, CESM and CESM + MG were evaluated by three blinded radiologists based on amended ACR BI-RADS criteria. MRI was assessed by another group of three readers. Receiver-operating characteristic (ROC) curves were compared. Size measurements for the 70 lesions detected by all readers in each modality were correlated with pathology. RESULTS: Reading results for 604 lesions were available (273 malignant, 4 high-risk, 327 benign). The area under the ROC curve was significantly larger for CESM alone (0.84) and CESM + MG (0.83) compared to MG (0.76) (largest advantage in dense breasts) while it was not significantly different from MRI (0.85). Pearson correlation coefficients for size comparison were 0.61 for MG, 0.69 for CESM, 0.70 for CESM + MG and 0.79 for MRI. CONCLUSIONS: This study showed that CESM, alone and in combination with MG, is as accurate as MRI but is superior to MG for lesion detection. Patients with dense breasts benefitted most from CESM with the smallest additional dose compared to MG. KEY POINTS: • CESM has comparable diagnostic performance (ROC-AUC) to MRI for breast cancer diagnostics. • CESM in combination with MG does not improve diagnostic performance. • CESM has lower sensitivity but higher specificity than MRI. • Sensitivity differences are more pronounced in dense and not significant in non-dense breasts. • CESM and MRI are significantly superior to MG, particularly in dense breasts.


Asunto(s)
Neoplasias de la Mama/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Mamografía/métodos , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias de la Mama/patología , Medios de Contraste/administración & dosificación , Femenino , Humanos , Imagen por Resonancia Magnética/normas , Mamografía/normas , Persona de Mediana Edad , Estudios Prospectivos , Curva ROC , Sensibilidad y Especificidad
14.
Arthritis Res Ther ; 18(1): 248, 2016 10 25.
Artículo en Inglés | MEDLINE | ID: mdl-27782850

RESUMEN

BACKGROUND: BoneXpert (BX) is a newly developed medical device based on digital X-ray radiogrammetry to measure human cortical bone thickness. The aim of this study was to quantify cortical bone loss of the metacarpals in patients with psoriatic arthritis (PsA) and compare these findings with other radiological scoring methods. METHODS: The study includes 104 patients with verified PsA. The BX method was used to measure the Metacarpal Index (MCI) at the metacarpal bones (II-IV). Additionally, the T-score of the MCI (T-scoreMCI) was calculated. Radiographic severity was determined by the Psoriatic Arthritis Ratingen Score (Proliferation Score and Destruction Score) as published by Wassenberg et al. and the Psoriatic Arthritis modified van der Heijde Sharp Score (Joint Space Narrowing Score and Erosion Score). RESULTS: For the total PsA study cohort, the T-scoreMCI was significantly reduced by -1.289 ± 1.313 SD. The MCI negatively correlated with the Proliferation Score (r = -0.732; p < 0.001) and the Destruction Score (r = -0.771; p < 0.001) of the Psoriatic Arthritis Ratingen Score. Lower coefficients of correlations were observed for the Psoriatic Arthritis modified van der Heijde Sharp Score. In this context, a severity-dependent and PsA-related periarticular demineralisation as measured by the MCI was quantified. The strongest reduction of -30.8 % (p < 0.01) was observed for the MCI in the Destruction Score. CONCLUSIONS: The BX MCI score showed periarticular demineralisation and severity-dependent bone loss in patients with PsA. The measurements of the BX technique were able to sensitively differentiate between the different stages of disease manifestation affecting bone integrity and thereby seem to achieve the potential to be a surrogate marker of radiographic progression in PsA.


Asunto(s)
Artritis Psoriásica/diagnóstico por imagen , Huesos del Metacarpo/diagnóstico por imagen , Intensificación de Imagen Radiográfica/métodos , Adulto , Anciano , Artritis Psoriásica/patología , Femenino , Humanos , Masculino , Huesos del Metacarpo/patología , Persona de Mediana Edad
15.
Invest Radiol ; 50(10): 733-42, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26154442

RESUMEN

OBJECTIVE: To evaluate whether magnetic resonance imaging (MRI) is effective as computed tomography (CT) in determining morphologic and functional pulmonary changes in patients with cystic fibrosis (CF) in association with multiple clinical parameters. MATERIALS AND METHODS: Institutional review board approval and patient written informed consent were obtained. In this prospective study, 30 patients with CF (17 men and 13 women; mean (SD) age, 30.2 (9.2) years; range, 19-52 years) were included. Chest CT was acquired by unenhanced low-dose technique for clinical purposes. Lung MRI (1.5 T) comprised T2- and T1-weighted sequences before and after the application of 0.1-mmol·kg gadobutrol, also considering lung perfusion imaging. All CT and MR images were visually evaluated by using 2 different scoring systems: the modified Helbich and the Eichinger scores. Signal intensity of the peribronchial walls and detected mucus on T2-weighted images as well as signal enhancement of the peribronchial walls on contrast-enhanced T1-weighted sequences were additionally assessed on MRI. For the clinical evaluation, the pulmonary exacerbation rate, laboratory, and pulmonary functional parameters were determined. RESULTS: The overall modified Helbich CT score had a mean (SD) of 15.3 (4.8) (range, 3-21) and median of 16.0 (interquartile range [IQR], 6.3). The overall modified Helbich MR score showed slightly, not significantly, lower values (Wilcoxon rank sum test and Student t test; P > 0.05): mean (SD) of 14.3 (4.7) (range, 3-20) and median of 15.0 (IQR, 7.3). Without assessment of perfusion, the overall Eichinger score resulted in the following values for CT vs MR examinations: mean (SD), 20.3 (7.2) (range, 4-31); and median, 21.0 (IQR, 9.5) vs mean (SD), 19.5 (7.1) (range, 4-33); and median, 20.0 (IQR, 9.0). All differences between CT and MR examinations were not significant (Wilcoxon rank sum tests and Student t tests; P > 0.05). In general, the correlations of the CT scores (overall and different imaging parameters) to the clinical parameters were slightly higher compared to the MRI scores. However, if all additional MRI parameters were integrated into the scoring systems, the correlations reached the values of the CT scores. The overall image quality was significantly higher for the CT examinations compared to the MRI sequences. CONCLUSIONS: One major diagnostic benefit of lung MRI in CF is the possible acquisition of several different morphologic and functional imaging features without the use of any radiation exposure. Lung MRI shows reliable associations with CT and clinical parameters, which suggests its implementation in CF for routine diagnosis, which would be particularly important in follow-up imaging over the long term.


Asunto(s)
Fibrosis Quística/diagnóstico por imagen , Fibrosis Quística/fisiopatología , Pulmón/diagnóstico por imagen , Pulmón/fisiopatología , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X , Adulto , Medios de Contraste , Femenino , Humanos , Aumento de la Imagen , Masculino , Persona de Mediana Edad , Compuestos Organometálicos , Estudios Prospectivos , Reproducibilidad de los Resultados , Adulto Joven
16.
Eur Radiol ; 25(3): 837-49, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25249313

RESUMEN

OBJECTIVES: To compare intraindividually two macrocyclic contrast agents - gadobutrol and gadoterate meglumine (Gd-DOTA) - for dynamic and quantitative assessment of relative enhancement (RE) in benign and malignant breast lesions. METHODS: This was an ethically approved, prospective, single-centre, randomized, crossover study in 52 women with suspected breast lesions referred for magnetic resonance imaging (MRI). Each patient underwent one examination with gadobutrol and one with Gd-DOTA (0.1 mmol/kg BW) on a 1.5 T system 1 - 7 days apart. Dynamic, T1-weighted, 3D gradient echo sequences were acquired under identical conditions. Quantitative evaluation with at least three regions of interest (ROI) per lesion was performed. Primary endpoint was RE during the initial postcontrast phase after the first and second dynamic acquisition, and peak RE. All lesions were histologically proven; differences between the examinations were evaluated. RESULTS: Forty-five patients with a total of 11 benign and 34 malignant lesions were assessed. Mean RE was significantly higher for gadobutrol than Gd-DOTA (p < 0.0001). Gadobutrol showed significantly less washout (64.4 %) than Gd-DOTA (75.4 %) in malignant lesions (p = 0.048) CONCLUSIONS: Gadobutrol has higher RE values compared with Gd-DOTA, whereas Gd-DOTA shows more marked washout in malignant lesions. This might improve the detection of breast lesions and influence the specificity of breast MRI-imaging.


Asunto(s)
Enfermedades de la Mama/patología , Medios de Contraste , Meglumina , Compuestos Organometálicos , Adolescente , Adulto , Anciano , Estudios Cruzados , Femenino , Compuestos Heterocíclicos , Humanos , Imagen por Resonancia Magnética/métodos , Persona de Mediana Edad , Estudios Prospectivos , Sensibilidad y Especificidad , Adulto Joven
17.
Int J Comput Assist Radiol Surg ; 10(5): 587-92, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-24989966

RESUMEN

OBJECTIVE: Digital X-ray radiogrammetry (DXR) is a computer-assisted technique used to quantify cortical bone density of the metacarpals. The influence of metacarpal bone rotation and type of cast material on bone mineral density (BMD) measurements using the DXR technique was tested. METHODS: The bone mineral density of the hand was measured by DXR, and rotation error (DXR-RE) as coefficients of variation were calculated, to verify reliability and reproducibility of this radiogeometric technique to assess in particular minor disease-related changes in the metacarpal bone mass. The reproducibility of the DXR measurements was also investigated using different cast materials (mull, elastic, and plastic). RESULTS: There were no significant changes in absolute values of DXR-BMD observed between 0 to [Formula: see text] angulation. The relative DXR-RE ranged between 0 % (degree 1) and 0.70 % (degrees 15 and 19) for DXR-BMD. Regarding the different cast materials, DXR-BMD revealed a coefficient of variation with 0.41 % (mull cast) and 0.21 % (elastic cast). For the plastic cast, the DXR technique was not able to perform an analysis of DXR-BMD. CONCLUSION: The study revealed no significant influence of metacarpal rotation on the measurements of metacarpal bone mineral density as estimated by DXR. DXR measurements are not optimal when cast material is used. DXR can accurately quantify periarticular cortical bone mass. This is significant especially for rheumatoid arthritis and related conditions where X-ray imaging of arthritic hands with varying degrees of deformity is performed.


Asunto(s)
Densidad Ósea/fisiología , Huesos del Metacarpo/diagnóstico por imagen , Intensificación de Imagen Radiográfica/métodos , Artritis Reumatoide/diagnóstico por imagen , Humanos , Reproducibilidad de los Resultados
18.
Breast Cancer Res Treat ; 146(2): 371-81, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24986697

RESUMEN

The purpose of this study was to compare contrast-enhanced spectral mammography (CESM) with mammography (MG) and combined CESM + MG in terms of detection and size estimation of histologically proven breast cancers in order to assess the potential to reduce radiation exposure. A total of 118 patients underwent MG and CESM and had final histological results. CESM was performed as a bilateral examination starting 2 min after injection of iodinated contrast medium. Three independent blinded radiologists read the CESM, MG, and CESM + MG images with an interval of at least 4 weeks to avoid case memorization. Sensitivity and size measurement correlation and differences were calculated, average glandular dose (AGD) levels were compared, and breast densities were reported. Fisher's exact and Wilcoxon tests were performed. A total of 107 imaging pairs were available for analysis. Densities were ACR1: 2, ACR2: 45, ACR3: 42, and ACR4: 18. Mean AGD was 1.89 mGy for CESM alone, 1.78 mGy for MG, and 3.67 mGy for the combination. In very dense breasts, AGD of CESM was significantly lower than MG. Sensitivity across readers was 77.9 % for MG alone, 94.7 % for CESM, and 95 % for CESM + MG. Average tumor size measurement error compared to postsurgical pathology was -0.6 mm for MG, +0.6 mm for CESM, and +4.5 mm for CESM + MG (p < 0.001 for CESM + MG vs. both modalities). CESM alone has the same sensitivity and better size assessment as CESM + MG and was significantly better than MG with only 6.2 % increase in AGD. The combination of CESM + MG led to systematic size overestimation. When a CESM examination is planned, additional MG can be avoided, with the possibility of saving up to 61 % of radiation dose, especially in patients with dense breasts.


Asunto(s)
Neoplasias de la Mama/diagnóstico , Medios de Contraste , Mamografía , Intensificación de Imagen Radiográfica , Absorciometría de Fotón , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias de la Mama/patología , Femenino , Humanos , Mamografía/métodos , Mamografía/normas , Persona de Mediana Edad , Estadificación de Neoplasias , Estudios Prospectivos , Dosis de Radiación , Intensificación de Imagen Radiográfica/métodos , Sensibilidad y Especificidad , Carga Tumoral
19.
Invest Radiol ; 49(7): 474-84, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24637587

RESUMEN

OBJECTIVE: In contrast to conventional breast imaging techniques, one major diagnostic benefit of breast magnetic resonance imaging (MRI) is the simultaneous acquisition of morphologic and dynamic enhancement characteristics, which are based on angiogenesis and therefore provide insights into tumor pathophysiology. The aim of this investigation was to intraindividually compare 2 macrocyclic MRI contrast agents, with low risk for nephrogenic systemic fibrosis, in the morphologic and dynamic characterization of histologically verified mass breast lesions, analyzed by blinded human evaluation and a fully automatic computer-assisted diagnosis (CAD) technique. MATERIALS AND METHODS: Institutional review board approval and patient informed consent were obtained. In this prospective, single-center study, 45 women with 51 histopathologically verified (41 malignant, 10 benign) mass lesions underwent 2 identical examinations at 1.5 T (mean time interval, 2.1 days) with 0.1-mmol kg doses of gadoteric acid and gadobutrol. All magnetic resonance images were visually evaluated by 2 experienced, blinded breast radiologists in consensus and by an automatic CAD system, whereas the morphologic and dynamic characterization as well as the final human classification of lesions were performed based on the categories of the Breast imaging reporting and data system MRI atlas. Lesions were also classified by defining their probability of malignancy (morpho-dynamic index; 0%-100%) by the CAD system. Imaging results were correlated with histopathology as gold standard. RESULTS: The CAD system coded 49 of 51 lesions with gadoteric acid and gadobutrol (detection rate, 96.1%); initial signal increase was significantly higher for gadobutrol than for gadoteric acid for all and the malignant coded lesions (P < 0.05). Gadoteric acid resulted in more postinitial washout curves and fewer continuous increases of all and the malignant lesions compared with gadobutrol (CAD hot spot regions, P < 0.05). Morphologically, the margins of the malignancies were different between the 2 agents, whereas gadobutrol demonstrated more spiculated and fewer smooth margins (P < 0.05). Lesion classifications by the human observers and by the morpho-dynamic index compared with the histopathologic results did not significantly differ between gadoteric acid and gadobutrol. CONCLUSIONS: Macrocyclic contrast media can be reliably used for breast dynamic contrast-enhanced MRI. However, gadoteric acid and gadobutrol differed in some dynamic and morphologic characterization of histologically verified breast lesions in an intraindividual, comparison. Besides the standardization of technical parameters and imaging evaluation of breast MRI, the standardization of the applied contrast medium seems to be important to receive best comparable MRI interpretation.


Asunto(s)
Algoritmos , Neoplasias de la Mama/patología , Compuestos Heterocíclicos , Aumento de la Imagen/métodos , Interpretación de Imagen Asistida por Computador/métodos , Imagen por Resonancia Magnética/métodos , Compuestos Organometálicos , Adulto , Anciano , Medios de Contraste , Femenino , Humanos , Persona de Mediana Edad , Reproducibilidad de los Resultados , Sensibilidad y Especificidad
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