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Lymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities that can depict central lymphatic anatomy and flow pattern. Our objective was to describe the technical aspects and our imaging findings of central lymphatic abnormalities and their impact on patient management and outcomes: We conducted a retrospective review of 26 children with congenital heart disease who presented for lymphatic imaging between 2015 and 2020 at our institution. Eleven had postoperative chylothorax, six had plastic bronchitis, seven had protein-losing enteropathy and three had Noonan syndrome. Our lymphatic imaging demonstrated severely abnormal lymphatic flow in all of the children, but only minor abnormalities in protein-losing enteropathy. No major procedure-related complication occurred. Lymphatic interventions were performed in six patients, thoracic duct decompression in two patients and chylothorax revision in three patients. This led to symptomatic improvements in all of the patients: Lymphatic imaging is safe and essential for the diagnosis of lymphatic flow disorders and therapy planning. Our intranodal lymphangiography depicts an abnormal lymphatic flow pattern from the central lymphatics but failed to demonstrate an abnormal lymphatic flow in protein-losing enteropathy. These imaging techniques are the basis for selective lymphatic interventions, which are promising to treat lymphatic flow disorders.

Aims: As in vivo real-life data are still scarce, we conducted a study to assess the safety and feasibility of cardiac magnetic resonance imaging (MRI) in patients with a leadless pacemaker system., Methods and Results: In this prospective non-randomized interventional trial, we enrolled 15 patients with an MRI conditional Micra® leadless pacemaker system to undergo either a 1.5 T or 3.0 T cardiac MRI scan. Clinical adverse events as well as device parameters such as pacing threshold, sensing, impedance, and battery life were assessed at baseline as well as 1 and 3 months after the scan. Device parameter changes between different time points were tested for statistical significance and compared with pre-set cut-off values. Fourteen patients underwent the cardiac MRI scan according to the protocol as well as the scheduled follow-up visits. One participant was excluded from analysis, as the MRI scan was not possible because of severe claustrophobia. Other clinical events did not occur during the scan and the follow-up period. Device parameters stayed stable and changes during the observational period were statistically not significant (changes vs. baseline: pacing threshold: 0.01 ± 0.05 V, P = 0.308, 0.01 ± 0.07 V, P = 0.419, sensing: -0.15 ± 1.11 mV, P = 0.658, -0.19 ± 1.17 mV, P = 0.800, impedance: -7.86 ± 30.7 Ohm, P = 0.447, -7.86 ± 25.77 Ohm, P = 0.183, at 1 and 3 months follow-up, respectively). Parameter changes were not statistically different between patients who underwent imaging at 1.5 T (n = 7) or 3.0 T (n = 7)., Conclusion: In our set of patients with a Micra® leadless pacemaker, cardiac magnetic resonance imaging at either 1.5 T or 3.0 T proved feasible and safe with no relevant changes in device parameters within 3 months of follow-up.

Background: There are limited data on patients with leadless cardiac pacemakers (LCP) undergoing magnetic resonance imaging. The aim of this prospective, single-center, observational study was to evaluate artefacts on cardiovascular magnetic resonance (CMR) images in patients with LCP., Methods: Fifteen patients with Micra™ LCP, implanted at least 6 weeks prior to CMR scan, were enrolled and underwent either 1.5 Tesla or 3 Tesla CMR imaging. Artefacts were categorized into grade 1 (excellent image quality), grade 2 (good), grade 3 (poor) and grade 4 (non-diagnostic) for each myocardial segment. One patient was excluded because of an incomplete CMR investigation due to claustrophobia., Results: LCP caused an arc-shaped artefact (0.99 ± 0.16 cm 2 ) at the right ventricular (RV) apex. Of 224 analyzed myocardial segments of the left ventricle (LV) 158 (70.5%) were affected by grade 1, 27 (12.1%) by grade 2, 17 (7.6%) by grade 3 and 22 (9.8%) by grade 4 artefacts. The artefact burden of grade 3 and 4 artefacts was significantly higher in the 3 Tesla group (3 Tesla vs 1.5 Tesla: 3.7 ± 1.6 vs 1.9 ± 1.4 myocardial segments per patient, p = 0.03). A high artefact burden was particularly observed in the mid anteroseptal, inferoseptal and apical septal myocardial segments of the LV and in the mid and apical segments of the RV. Quantification of LV function and assessment of valves were feasible in all patients. We did not observe any clinical or device-related adverse events., Conclusion: CMR imaging in patients with LCP is feasible with excellent to good image quality in the majority of LV segments. The artefact burden is comparable small allowing an accurate evaluation of LV function, cardiac structures and valves. However, artefacts in the mid anteroseptal, inferoseptal and apical septal myocardial segments of the LV due to the LCP may impair or even exclude diagnostic evaluation of these segments. Artefacts on CMR images may be reduced by the use of 1.5 Tesla CMR scanners.

Background: Patients undergoing transcatheter aortic valve implantation (TAVI) are at increased risk for post-interventional conduction disturbances leading to pacemaker (PM) implantation. We analyzed the association between implantation depth within the left ventricular outflow tract (LVOT), measured by 64-slice computed tomography (CT), and 'index electrocardiographic (ECG) changes' (new onset atrioventricular-block grade II or III or left bundle branch block with PR interval prolongation >200ms)., Methods: We evaluated patients who underwent TAVI with the Core Valve(®) revalving system (Medtronic, Minneapolis, MN, USA) for treatment of severe aortic stenosis at our department. Patients with a prior PM implantation and patients for whom no CT scan was available after 3 months were excluded from analysis. We assessed implantation depth of the prosthesis within the LVOT as possible risk factors for the development of post-interventional 'index ECG changes' resulting in PM implantation and compared it with individual patient data as well as echocardiographic and electrocardiographic parameters., Results: The final study cohort comprised 53 patients for whom a 64-slice CT scan was available (mean age 81.7±5.1 years, 36% male). Twenty-eight of these finally underwent PM implantation due to 'index ECG changes' within the first 48hours after TAVI. Univariate logistic regression analysis could identify implantation depth of the prosthesis as the only significantly correlated risk factor for PM need in our cohort (OR 1.27, 95% CI: 1.08-1.51, p=0.004). A cut-off value of 6mm predicted this need with a sensitivity of 89% and specificity of 40%., Conclusion: Implantation depth of the Core Valve(®) into the LVOT was associated with post-procedural PM requirement. Thereby, a cut-off value of 6mm, as measured by 64-slice CT, proved useful to define patients at risk for PM requirement., (Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background and Purpose: In a very limited number of cases, acute migrainous aura may mimic acute brain infarction. The aim of this study was to recognize patterns of MR perfusion abnormalities in this presentation., Materials and Methods: One thousand eight hundred fifty MR imaging studies performed for the suspicion of acute brain infarction were analyzed retrospectively to detect patients with acute migrainous aura not from stroke. All patients were examined clinically by 2 neurologists and underwent a standard stroke MR imaging protocol, including PWI. Two radiologists reviewed the perfusion maps visually and quantitatively for the presence, distribution, and grade of perfusion abnormalities., Results: Among 1850 MR imaging studies, 20 (1.08%) patients were found to have acute migrainous aura. Hypoperfusion was found in 14/20 patients (70%) with delayed rMTT and TTP, decreased rCBF, and minimal decrease in rCBV. In contrast to the typical pattern in stroke, perfusion abnormalities were not limited to a single vascular territory but extended to >1. Bilateral hypoperfusion was seen in 3/14 cases. In 11/14 cases, hypoperfusion with a posterior predominance was found. TTP and rMTT were the best maps to depict perfusion changes at visual assessment, but also rCBF maps demonstrated significant hypoperfusion in quantitative analysis. In all patients, clinical and imaging follow-up findings were negative for stroke., Conclusions: Acute migrainous aura is rare but important in the differential diagnosis among patients with the suspicion of acute brain infarction. Atypical stroke perfusion abnormalities can be seen in these patients.

OBJECTIVE: Owing to the widespread use of abdominal imaging studies the detection rate of solid renal masses has increased, and an accurate characterisation of imaging features of renal masses has become more essential for case management. METHOD AND RESULTS: MR imaging (MRI) and computed tomography (CT) are frequently used modalities for detection and differentiation of renal masses. This article gives a review of imaging characteristics of benign and malignant renal masses, discussing their appearance in CT and MR imaging. Advanced MR techniques like diffusion-weighted imaging and apparent diffusion coefficient (ADC) mapping, which have shown promising results in the differentiation between benign and malignant renal lesions, will be introduced. CONCLUSION: MRI and CT are useful in the characterisation and estimation of the prognosis for renal masses.

Background and Purpose: Image quality and diagnostic reliability of T2-weighted MR images of the cervical spine are often impaired by several kinds of artifacts, even in cooperative patients. The aim of this study was to evaluate if BLADE sequences might solve these problems in a routine patient collective., Materials and Methods: TSE and BLADE sequences were compared in 60 patients for T2-weighted sagittal imaging of the cervical spine. Image sharpness, motion artifacts, truncation artifacts, metal artifacts, CSF flow phenomena, contrast of anatomic structures (vertebral body/disk, spinal cord/CSF), and diagnostic reliability of spinal cord depiction were evaluated by 2 independent readers. Another 2 readers selected the sequence they would prefer for diagnostic purposes. Statistical evaluations were performed by using the Wilcoxon and the chi(2) test; differences with P < .05 were regarded as statistically significant., Results: BLADE was significantly superior to TSE regarding image sharpness, image contrast, diagnostic reliability of spinal cord depiction, motion artifacts, CSF flow phenomena, and truncation artifacts; for metal artifacts no significant improvements were found. In 50 of 60 patients, BLADE was preferred for diagnostic purposes, and TSE was favored in 3 patients. The number of examinations that were nondiagnostic due to impaired spinal cord depiction was reduced from 12 in TSE to 3 in BLADE, and nondiagnostic examinations due to overall motion artifacts were reduced from 2 to 1., Conclusions: Using the BLADE sequence for sagittal T2-weighted imaging of the cervical spine proved to be advantageous to reduce various kinds of artifacts.

Background and Purpose: We prospectively evaluated the influence of different imaging techniques (time-of-flight MR angiography [TOF-MRA], contrast-enhanced MR angiography [CE-MRA], multisection CT angiography [CTA]) and postprocessing methods (maximum intensity projection [MIP], multiplanar reformation [MPR]) on carotid artery stenosis grading., Materials and Methods: Fifty patients (34 men, 16 women) with symptomatic stenosis of the internal carotid artery were examined with a 16-section spiral CT and a 1.5T MR unit. Two MRA techniques were applied: 3D-TOF and CE-MRA. MPR was used for postprocessing with all modalities; MIP was used only with MRA. Four readers measured and calculated the percentage diameter stenosis independently according to NASCET criteria. The Wilcoxon test was used to measure interobserver variability, and the Friedman test was used to test the null-hypothesis of equality of the modalities., Results: The hypothesis for global equality was rejected (P < .001). TOF-MRA and CTA assessed with MPR showed the highest concordance (difference, 0.6%; confidence interval [CI], -3.0, 4.3%), and CE-MRA with MIP and CTA showed the lowest concordance in stenosis grading (difference, 7.0%; CI, 3.4, 10.6%). MPR resulted in lower degrees of stenosis than MIP for both MRA sequences, although not statistically significant (CE, -3.0%; CI, -6.6, 0.6%; TOF, -2.2%; CI, -5.8, 1.4%). When only studies with good or excellent image quality were considered, the differences decreased, but the trends remained., Conclusion: Stenosis grading is dependent on the examination method and postprocessing technique. CTA and TOF-MRA evaluated with MPR revealed highest concordance.

Objectives: The aim of this study was to compare methods of quantifying calcification of the internal carotid artery., Materials and Methods: We examined 92 internal carotid artery endarterectomy specimens. Grey scale median (GSM) values were calculated from optimized B-mode scans. The degree of calcification was assessed using radiographic calcification grading. Plaques were processed histologically, and classified into: (1) calcium-rich hard plaques, (2) lipid-rich soft plaques, and (3) combined plaques. The specimens were scanned in CT-scanner. The calcium score was determined as described by Agatston., Results: Histopathology and GSM results concurred in 39 out of 92 cases (kappa=0.088). There was no significant correlation between the calcium score and the median GSM value (R=0.005; P=0.959). Histology and CT morphology showed a significant concordance (P<0.001). Also CT and radiomorphological classification showed close agreement (R=0.628, P<0.001)., Conclusions: Calcium scores calculated using CT morphology enable precise in vitro evaluation of the calcium content of plaques in the internal carotid. In contrast, grey scale median values do not adequately reflect calcification of plaques.

Background and Purpose: A close correlation between B-mode sonography and the histopathology and surface structure of plaque is rarely seen in vivo with high-grade stenoses of the extracranial carotid artery. The goal of this study was to determine whether normalized gray scale analysis and surface analysis of the plaque are capable of characterizing the attributes correctly., Methods: Optimized B-mode images of 107 carotid endarterectomy specimens were captured, and the gray scale median (GSM) was calculated. The specimens were classified histologically into 3 groups: (1) calcium-rich hard plaques, (2) lipid-rich soft plaques, and (3) combined plaques. The surfaces of the plaques were classified as smooth or rough on the basis of standardized reference samples. The endoluminal surface was digitally documented in vitro by videoendoscopy and again classified into the same categories. All stages of the investigation were performed by 2 observers at 2 different times., Results: Evaluation of the GSM showed close interobserver and intraobserver correlation (P<0.01, R>0.8). However, there was only 46% agreement between the GSM and the histopathological findings. In both in vitro angioscopy (kappa=0.936, P<0.001) and sonographic evaluation (kappa=0.842, P<0.001), there was a high correlation between the observers with regard to the evaluable specimens. In 73%, agreement was observed between the sonographic image and angioscopy., Conclusions: Normalized gray scale analysis and evaluation of the plaque surface in an in vitro study make possible observer-independent evaluation. The composition of the plaque cannot be visualized with sufficient accuracy by sonographic GSM analysis. This also applies to the correlation between sonography and actual surface composition of the plaque.

Purpose: To determine the value of the gradient- and spin-echo (GRASE) technique as compared with the fast spin-echo and conventional spin-echo techniques in MR imaging of the brain., Methods: Sixty-six patients with ischemic and neoplastic brain lesions were examined with T2-weighted spin-echo, fast spin-echo, and GRASE sequences. Three independent observers evaluated the contrast characteristics of anatomic and pathologic structures and of artifacts. Quantitative image analysis included region-of-interest measurements of anatomic structures and lesions., Results: The contrast of anatomic structures was superior in images obtained with conventional and fast spin-echo techniques as compared with those obtained with the GRASE technique. Extended lesions, such as tumors and territorial infarcts, were identified equally with all techniques. For delineation of small ischemic lesions, GRASE was slightly inferior to fast and conventional spin-echo sequences. Flow artifacts were considerably reduced with fast spin-echo and GRASE sequences. Chemical-shift artifacts were significantly reduced, but ringing artifacts were more pronounced with GRASE., Conclusion: Fast spin-echo remains the standard technique in MR imaging of the brain. However, GRASE might be useful in special cases, such as with uncooperative patients whose conventional or fast spin-echo images show severe motion artifacts.

Based on the sequence of cDNA encoding the intracellular domain of the insulin receptor beta-subunit, we recently defined a heterozygous point mutation causing a Ser for Trp substitution at position 1200 in the tyrosine kinase domain of a patient (BI-2) with the type A syndrome of insulin resistance. We have now sequenced the remainder of BI-2's insulin receptor cDNA-coding region and find no additional alterations in the encoded proreceptor protein. The nucleotide sequence of cDNA encoding the portion of the beta-subunit which includes Trp1200 was normal in BI-2's unaffected mother. Hybridization of a mutant allele-specific oligonucleotide to polymerase chain reaction-amplified cDNA confirmed the presence of the mutant allele in the proband and excluded it in her unaffected sister and mother, 18 normal control subjects, and six other subjects with insulin resistance. To determine whether this mutation had functional consequences for receptor signalling, we reconstructed it into a full-length insulin receptor cDNA expression vector. Chinese hamster ovary cells were transfected with mutant cDNA, and the expressed insulin receptors were compared to receptors expressed by cells transfected with wild-type receptor cDNA. Both mutant and wild-type receptors were properly processed into receptor alpha- and beta-subunits, were expressed on the cell surface, and displayed similar insulin-binding affinity. In contrast, insulin-stimulated autophosphorylation of the mutant receptors was severely impaired, whether assessed in intact cells or with a partially purified receptor preparation.(ABSTRACT TRUNCATED AT 250 WORDS)

A 10-year longitudinal study was conducted on 16 prepubescent youngsters who had undergone successful weight reduction. Their ages ranged from 2 to 10 years when the study began. In all subjects, weight reduction proceeded only by a decrease in adipose cell size (from 0.62 +/- 0.02 to 0.46 +/- 0.02 micrograms lipid per cell) and resulted in a corresponding 33% decrease (from 177 +/- 6 to 144 +/- 5%) in percent ideal body weights. Cell numbers did not change appreciably during the period of weight loss (29.4 +/- 2.6 versus 28.7 +/- 2.3 x 10(9) total adipocytes). Three years after the start of the study, 14 of 20 youngsters had maintained their reduced percent ideal body weights, including eight who remained below 130% ideal body weight. Ten years later, only four remained below 130% ideal body weight. All four children had total adipose cell numbers below 20 x 10(9) total adipocytes at the start of the weight reduction program, a value below the lower limit for adult normal weight subjects. Thirteen other children have maintained or decreased their initial percent ideal weights. The remaining nine youngsters have further increased their percent ideal body weights. In vitro metabolic studies of the patient's adipocytes revealed a greater than 50% depression of epinephrine-stimulated lipolysis pre- and immediately postweight reduction; this decrease persisted for the entire period of study, irrespective of the maintenance of a normal percent ideal body weight. At the same time, normal 150% increases in the in vitro production of 14CO2 from [1-14C]glucose in the presence of insulin occurred.

A de nova bistaellited derivative of chromosome 15, inv dup (15) (pter leads to q11 or 12::p11 or q11 or 12 leads to pter), was identified by multiple banding techniques in a patient with Prader-Willi syndrome. A comparison of familial no. 15 short arm polymorphisms indicated that the extra chromosome was the result of a non-sister chromatid exchange between the paternal no. 15 homologs prior to or during meiosis.

An increased incidence of insulin-dependent diabetes mellitus (IDDM) has been reported in patients with congenital rubella syndrome (CRS). Thus, studies of children with CRS would be of great importance in following the development of IDDM in a susceptible population. A total of 242 children with CRS, 30 of whom already have diabetes (mean age, 17.4 +/- 0.3 years) have been evaluated. In this latter group, the frequency of HLA DR3 is significantly increased and that of HLA DR2 significantly decreased. While pancreatic islet cell cytotoxic or surface antibodies (ICSA) are found in 20.2% of the total population of patients with CRS, they are present in 50%-80% of patients with glucose abnormalities. In all but five of the ICSA-positive patients, glucose abnormalities are currently present. In addition, glucose intolerance is found in greater than 50% of the DR3-positive nondiabetic patients with CRS evaluated to date. The data demonstrate that patients with CRS at risk for IDDM have the same genetic and immunologic features seen in classic IDDM, namely the presence of HLA DR3 and the absence of HLA DR2 and the high prevalence of ICSA before decompensation.

Three different published sets of HLA-typed families of juvenile diabetes mellitus (JDM) patients have been analyzed. There was no significant genetic heterogeneity between them according to the criterion of Morton, and the total material was analyzed on the assumption of a single recessive (JDM-P) gene with incomplete penetrance. The analysis, carried out with the NYLIP program modified to account for penetrance less than 1 and for selection bias, yields highly significant lod scores for linkage between HLA and JDM-P, with a maximum value of 7.40 at theta = .05 +/- .03. The segregation of HLA and GLO in five affected sib pairs, in which one of the sibs carries an HLA/GLO recombinant, places JDM-P closer to HLA than the GLO locus: four of these five pairs are HLA-identical and GLO-different, in agreement with the conclusions of the formal linkage analysis. The data from these three independent sets of families are therefore consistent with our earlier claim that JDM is inherited as a recessive trait closely linked to HLA with reduced penetrance, and its analysis does not require more complicated genetic models.

Residual beta cell secretory capacity was assessed in short term (2 months to 2 years) and long term (5 to 8 years) insulin-dependent diabetics by measurement of serum C-peptide immunoreactivity during three provocative tests: glucose, tolbutamide, and glucagon. Minimal C-peptide secretion could be detected in only one out of seven long term diabetics by the stimulatory tests. All seven short-term diabetics responded to at least one provocative test of beta cell reserve, although these responses were blunted. The greatest C-peptide responses occurred after glucagon administration (mean increase 0.62 pmol/ml) in short-term responders. Patients who responded to one test did not necessarily respond to another stimulus. There was no correlation between basal C-peptide levels and the ability to provoke further C-peptide secretion by any of the three tests. C-peptide responses did not correlate with % Haemoglobin A1c, mean fasting blood glucose levels, or mean blood glucose concentrations during an oral glucose tolerance test. The data indicate that stimulation tests are only useful in assessing endogenous beta cell reserve in patients with diabetes of less than 5 years duration. In diabetics of longer duration there is little insulin reserve above basal levels.