Intrahepatic bile duct dilation and gallbladder hydrops due to a cystic duct stenosis in a 2-month-old boy.

A 2-month-old boy presented with slight diffuse abdominal pain after an uncomplicated pregnancy and perinatal period. Height (62 cm) and weight (5.5 kg) were according to the age. Routine blood tests showed no abnormalities except for a minimal elevation of aspartate aminotransferase to 38 units/L (reference range 5–35 U/L). Primary abdominal ultrasound showed a dilation of the common bile duct and a gallbladder hydrops (figure 1). MR cholangiopancreatography (MRCP) was indicated to detect the cause for these incidental findings. Contrary to normal bile ducts in …

Benchmarking Wilms’ tumor in multisequence MRI data: why does current clinical practice fail? Which popular segmentation algorithms perform well?

Wilms' tumor is one of the most frequent malignant solid tumors in childhood. Accurate segmentation of tumor tissue is a key step during therapy and treatment planning. Since it is difficult to obtain a comprehensive set of tumor data of children, there is no benchmark so far allowing evaluation of the quality of human or computer-based segmentations. The contributions in our paper are threefold: (i) we present the first heterogeneous Wilms' tumor benchmark data set. It contains multisequence MRI data sets before and after chemotherapy, along with ground truth annotation, approximated based on the consensus of five human experts. (ii) We analyze human expert annotations and interrater varia…

Ultrasound Evaluation of Thyroid Gland Pathologies After Radiation Therapy and Chemotherapy to Treat Malignancy During Childhood.

The purpose of this study was to evaluate correlations between treatment of malignancy by radiation therapy during childhood and the occurrence of thyroid gland pathologies detected by ultrasonography in follow-up examinations.Reductions of thyroid gland volume below 2 standard deviations of the weight-specific mean value, occurrence of ultrasonographically detectable thyroid gland pathologies, and hypothyroidism were retrospectively assessed in 103 children and adolescents 7 months to 20 years of age (median: 7 years of age) at baseline (1997-2013) treated with chemoradiation therapy (with the thyroid gland dose assessable) or with chemotherapy alone and followed by ultrasonography and lab…

Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1

We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy.In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversi…

The Link Between Local Recurrence and Distant Metastases in Patients With Rectal Cancer.

BACKGROUND/AIM The relationships between local recurrence (LR), the development of distant metastases (DM) and prognosis in patients with rectal cancer remain unclear. PATIENTS AND METHODS In 606 patients who underwent curative resection, the role of LR was assessed retrospectively by time-dependent multivariate Cox models with inverse probability of treatment weighting taking into account competing risks. RESULTS Patients with LR had more DM than patients without LR (49/79, 62% vs. 86/524, 16.4%; p<0.001); 37% of LR-associated DM developed before or at LR, 63% after diagnosis of LR. Fifty-five percent of patients without DM at diagnosis of LR later developed DM. In these patients, the inci…

Tuberkulose 2019 – eine Herausforderung auch für die Radiologie

Evaluation of treatment response to enzyme replacement therapy with Velaglucerase alfa in patients with Gaucher disease using whole-body magnetic resonance imaging.

Abstract Objective This was a retrospective data analysis to evaluate the treatment response to enzyme replacement therapy (ERT) with Velaglucerase alfa using whole-body magnetic resonance imaging (MRI). Materials and methods A baseline and follow-up MRI were performed on 18 Gaucher Type 1 patients at an interval of 11.6 months. The MRI score systems determined the Bone-Marrow-Burden (BMB) score, the Dusseldorf-Gaucher score (DGS), and the Vertebra-Disc-Ratio (VDR). The Severity Score Index Type 1 (GD-DS3) was also assessed. Results The baseline MRI medians were: BMB, 7.00; DGS, 3.00; and VDR: 1.70; while, the follow-up MRI medians were: BMB, 7.00; DGS, 3.00; and VDR: 1.73. The baseline GD-…

Correlation between fecal incontinence and quality of life after low anterior resection for rectal cancer

SUMMARY Aim: The purpose of this study was to investigate the correlation between fecal incontinence and quality of life after low anterior resection and long-term follow-up. Methods: For 72 patients with coloanal or low colorectal anastomosis, the quality of life was determined by the European Organisation for Research and Treatment of Cancer (EORTC) questionnaires C-30 and CR-38. The Vaizey score was used for assessment of fecal incontinence. Results: The risk of fecal incontinence significantly increased with a lower level of anastomosis (p &lt; 0.001). The QLQ CR-38 did not discern differences between patients. The OLQ C-30 only found significantly worse social and role function in pat…

Permanent Stoma After Low Anterior Resection for Rectal Cancer

BACKGROUND AND OBJECTIVES: A low anterior resection procedure for removing a rectal tumor aims to preserve the sphincter and avoid a permanent stoma. Permanent stomas are primarily necessary in cases of poor anorectal function and local recurrence. The aim of this study was to clarify whether anastomosis-related complications and local recurrence influenced the rate of permanent stomas in a long-term follow-up. METHODS: Of 1032 consecutive patients with rectal cancer, 397 were treated by low anterior resection (R0 and R1 resections) between 1985 and 2007 at the Department of General and Abdominal Surgery of the University Hospital, Mainz (Germany). All patient data were collected prospectiv…

Acoustic radiation force impulse point shear wave elastography of the liver and spleen in patients with Gaucher disease type 1: Correlations with clinical data and markers of disease severity.

To evaluate the feasibility of acoustic radiation force impulse point shear wave elastography (ARFI-pSWE) of the liver and spleen in patients with Gaucher disease type 1 (GD1), and to assess correlations between organ stiffness and clinico-radiologic data, particularly the GD1 Severity Scoring System (GD-DS3).We retrospectively evaluated the results of ARFI-pSWE as measures of liver and spleen stiffness in 57 patients with GD1. The feasibility of the method was assessed. Correlations between elastography data and clinical data related to the metabolic syndrome, laboratory tests, and GD1-related clinico-radiologic data (bone marrow burden score, GD-DS3) were assessed.ARFI-pSWE provided relia…

Quantification of intramuscular fat in patients with late-onset Pompe disease by conventional magnetic resonance imaging for the long-term follow-up of enzyme replacement therapy

Objective The objective of this study was to evaluate a quantitative method based on conventional T1-weighted magnetic resonance (MR) imaging to assess fatty muscular degeneration in patients with late-onset Pompe disease and to compare it with semi-quantitative visual evaluation (the Mercuri score). In addition, a long-term retrospective data analysis was performed to evaluate treatment response to enzyme replacement therapy with alglucosidase alfa. Methods MR images of the lumbar spine were acquired in 41 patients diagnosed with late-onset Pompe disease from 2006 through 2015. Two independent readers retrospectively evaluated fatty degeneration of the psoas and paraspinal muscles by apply…

Rectal cancer: Dynamic contrast-enhanced MRI correlates with lymph node status and epidermal growth factor receptor expression

Purpose To evaluate correlations between dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) and clinicopathologic data as well as immunostaining of the markers of angiogenesis epidermal growth factor receptor (EGFR) and CXC-motif chemokine receptor 4 (CXCR4) in patients with rectal cancer. Materials and Methods Presurgical DCE-MRI was performed in 41 patients according to a standardized protocol. Two quantitative parameters (k21, A) were derived from a pharmacokinetic two-compartment model, and one semiquantitative parameter (TTP) was assessed. Standardized surgery and histopathologic examinations were performed in all patients. Immunostaining for EGFR and CXCR4 was performed an…

Evaluation of Bone Marrow Infiltration in Non-Neuropathic Gaucher Disease Patients with Use of Whole-Body MRI--A Retrospective Data Analysis.

Purpose: To evaluate whole-body magnetic resonance imaging (WB-MRI) for the assessment of bone marrow infiltration in patients with confirmed Gaucher disease type 1 under long-term enzyme replacement therapy (ERT). Materials and Methods: This retrospective data analysis included 38 patients in two subgroups. Group A: 10 females, 9 males, 15 – 29 years, mean age 22 years and Group B: 11 females, 8 males, 29 – 77 years, mean age 49 years, all treated with alglucerase or imiglucerase for at least 12.5 years. Whole-body MRI was carried out in all patients using a standard MRI protocol. Two radiologists assessed all MR images retrospectively with the use of three different MRI score systems: The…

Exploiting Gangliosides for the Therapy of Ewing’s Sarcoma and H3K27M-Mutant Diffuse Midline Glioma

Simple Summary Osteosarcoma, Ewing’s sarcoma, and H3K27M-mutant diffuse midline glioma are rare but aggressive malignancies occurring mainly in children. Due to their rareness and often fatal course, drug development is challenging. Here, we repurposed the existing drugs dinutuximab and eliglustat and investigated their potential to directly target or indirectly modulate the tumor cell-specific ganglioside GD2. Our data suggest that targeting and/or modulating tumor cell-specific GD2 may offer a new therapeutic strategy for the above mentioned tumor entities. Abstract The ganglioside GD2 is an important target in childhood cancer. Nevertheless, the only therapy targeting GD2 that is approve…

Vom Röntgen zum PET/MRT, und dann? – Zukunftsweisende Bildgebung in der Kinderradiologie

Die Kinderradiologie steht heute vor weitreichenden Veränderungen. Basismodalitäten wie Röntgen und Ultraschall werden zunehmend durch neuere, moderne Techniken ergänzt. Dieser Übersichtsartikel stellt Fortschritte in der Kinderradiologie sowie technische Innovationen vor, welche in Zukunft noch größere Bedeutung erlangen könnten. Hierzu werden CT-Dosisreduktionstechniken inklusive der Anwendung künstlicher Intelligenz sowie Fortschritte in den Gebieten der Magnetresonanztomografie und molekularen Bildgebung dargestellt. Kernaussagen 

Ganzkörper-MRT zur Evaluation der Knochenmanifestationen bei mittels Enzymersatztherapie langzeitbehandelten Patienten mit Morbus Gaucher (Typ 1)

ARFI Elastografie der Leber und Milz bei Patienten mit Morbus Gaucher (Typ 1) - Korrelationen mit klinischen Daten und Markern der Erkrankungsschwere

Rectal cancer: Assessment of response to neoadjuvant chemoradiation by dynamic contrast-enhanced MRI

Purpose To assess pretreatment functional and morphological tumor characteristics with magnetic resonance imaging (MRI) in advanced rectal carcinoma and to identify factors predicting response to neoadjuvant chemoradiation. Materials and Methods In a prospective study, 95 patients with rectal carcinoma underwent dynamic contrast-enhanced MRI before and after chemoradiation. Quantitative parameters were derived from a pharmacokinetic two-compartment model. Tumors were also characterized with regard to mucinous status at pretreatment high-resolution MRI as nonmucinous or mucinous. Response to treatment was defined as a downshift in the local tumor stage. Results The parameter k21 (contrast me…