Rare variation of hepatic blood supply with accessory right and left hepatic artery / A máj ritka vérellátási variációja járulékos arteria hepatica dextra és sinistra jelenlétével

In recent decades, multiple studies have examined the various manifestations of the hepatic blood supply using anatomical preparation and imaging procedures (angiography, CT). Based on these observations, several classifications have emerged to determine blood supply types and their incidence rates. Our aim is to present a rare variation of the arterial blood supply of the liver through an anatomical preparation. Postmortem native CT scans of the abdominal organs of the described cadaver were performed, followed by three-dimensional reconstruction. After imaging, the abdominal block was fixed with formalin, then the vessels were visualized by layered anatomical preparation. The liver is supplied not only from the bifurcated proper hepatic artery, but also receives accessory branches from the left gastric artery and the superior mesenteric artery. This variation represents the widely used Michels' classification type VII, found in 0.2­0.73% of the cases. Vascular variations are not only remarkable from an anatomical point of view, but their preoperative mapping also plays a significant role in performing surgical interventions without any complications.

Human Cadaveric Artificial Lung Tumor-Mimic Training Model.

Introduction: An important phase in surgical training is gaining experience in real human anatomical situations. When a cadaver is available it may complement the various artificial practice models. However, it is often necessary to supplement the characteristics of the cadavers with a simulation of a tumor. Our objective was to develop an easy-to-create, realistic artificial tumor-mimic model for peripheral lung tumor resection practice. Methods: In our work we injected barium sulphate enriched silicone suspension into 10 isolated, non-fixed lungs of human cadavers, through the puncture of the visceral pleura. Four lesions-apical, hilar and two peripheral-were created in each of ten specimens. After fixation CT scans were obtained and analyzed. The implanted tumor-mimics were examined after anatomical preparation and slicing. Also performed CT-guided percutaneous puncture was also performed to create the lesions in situ in two lungs of human cadavers. Results: Analyzing the CT data of 10 isolated lungs, out of 40 lesions, 34 were nodular (85.0%) and in the nodular group five were spiculated (12.5%). Satellite lesions were formed in two cases (5.0%). Relevant outflow into vessels or airway occurred in five lesions (12.5%). Reaching the surface of the lung occured in 11 lesions (27.5%). The tumor-mimics were elastic and adhered well to the surrounding tissue. The two lesions, implanted via percutaneous puncture, both were nodular and one also showed lobulated features. Conclusion: Our artificial tumor-mimics were easy to create, varied in shape and size, and with percutaneous implantation the lesions provide a model for teaching every step of a surgical procedure.

Osteoarcheological and biomolecular evidence of leprosy from an 11-13th century CE Muslim cemetery in Europe (Orosháza, Southeast Hungary).

Orosháza site no. 10 (Southeast Hungary) contains the partially excavated archaeological remains of an 11-13th century CE Muslim merchant village and its cemetery located in close proximity to Christian villages of the same era. The skeleton of a young woman (grave no. 16) from the last phase of the cemetery use was identified with rhinomaxillary lesions associated with lepromatous leprosy. The right parietal bone also exhibited signs of cranial trauma, possibly caused by symbolic trepanation, a well-known ritual practice in the 9-11th century CE Carpathian Basin. The retrospective diagnosis of the disease was supported by ancient DNA analysis, as the samples were positive for Mycobacterium leprae aDNA, shown to be of genotype 3. Contrary to the general practice of the era, the body of the young female with severe signs of leprosy was interred among the regular graves of the Muslim cemetery in Orosháza, which may reflect the unique cultural background of the community.

Thallium Labeled Citrate-Coated Prussian Blue Nanoparticles as Potential Imaging Agent.

Background: The aim of this study was to develop and characterize a nanoparticle-based image-contrast platform which is biocompatible, chemically stable, and accessible for radiolabeling with 201Tl. We explored whether this nanoparticle enhanced the T1 signal which might make it an MRI contrast agent as well. Methods: The physical properties of citrate-coated Prussian blue nanoparticles (PBNPs) (iron(II);iron(III);octadecacyanide) doped with 201Tl isotope were characterized with atomic force microscopy, dynamic light scattering, and zeta potential measurement. PBNP biodistribution was determined by using SPECT and MRI following intravenous administration into C57BL6 mice. Activity concentrations (MBq/cm3) were calculated from the SPECT scans for each dedicated volume of interest (VOI) of liver, kidneys, salivary glands, heart, lungs, and brain. Results: PBNP accumulation peaked at 2 hours after injection predominantly in the kidneys and the liver followed by a gradual decrease in activity in later time points. Conclusion: We synthetized, characterized, and radiolabeled a Prussian blue-based nanoparticle platform for contrast material applications. Its in vivo radiochemical stability and biodistribution open up the way for further diagnostic applications.

Complement system activation in obstructive sleep apnea.

The complement system may play a role in the systemic inflammation characterising obstructive sleep apnea; however, this has not been investigated before. We aimed to study the involvement of effector complement elements in obstructive sleep apnea, namely C3a, C5a and SC5b-9. Venous blood was collected in 50 patients with obstructive sleep apnea and 26 control subjects in the evening and the following morning. Plasma complement proteins were analysed with ELISA. Complement factor levels were compared between the two groups and correlated with clinical variables. Plasma C3a concentration was elevated in obstructive sleep apnea both in the evening (84.1 [0-338.5] ng ml-1 ) and in the morning (85.5 [0-247.8] ng ml-1 ) compared with controls (30.3 [0-176.8] ng ml-1 and 36.3 [0-167.1] ng ml-1 , evening and morning, respectively, both p < 0.05). On the contrary, C5a and SC5b-9 levels were comparable between patients and controls at each time point (p > 0.05). There was no change in complement factors from evening to morning in either group (p > 0.05), except for C5a that decreased from evening to morning in obstructive sleep apnea (from 11.6 [1.6-47.4] ng ml-1 to 9.3 [0-46.4] ng ml-1 , p = 0.01). Elevated C3a levels were directly related to obstructive sleep apnea severity, and were significantly associated with male gender, weight, body mass index, hypertension, high C-reactive protein and low high-density lipoprotein cholesterol (p < 0.05). The complement system is activated in obstructive sleep apnea, which is correlated with disease severity. Our findings highlight the potential role of complement system in the pathophysiology of obstructive sleep apnea, thus facilitating further research.

Pancreaticoduodenal arterial arcades: Their dominance and variations-their potential clinical relevance.

The complex arterial system makes pancreatic interventions technically challenging for surgeons, and interventional radiologists. The arterial variants may alter tumor resecability, and cause complications in arterial embolization. International data on pancreatic blood supply are variable; therefore, we aimed to determine the frequency of variants of pancreaticoduodenal (PD) arterial arcades. Arteries of human abdominal organ complexes (50) were injected with resin mixture, and then corroded. CT scans and three-dimensional reconstructions were made; diameters of arcades were measured. Two PD arcades were found in 58%, three arcades in 30%, one arcade in three cases, four arcade in one specimen, and five arcades in two cases. In the casts with two arches the average diameter was 1.472 ± 0.432 mm on the anterior and 1.383 ± 0.343 mm on the posterior arch. Assessing the dominance of the PD arcades, the anterior arch proved to be dominant in 52% of the cases, and in 35% the posterior one by the criterion of 25% cross-section difference. It is advised to analyze the variations and dominance on a CT/MR-angiography before local chemotherapy. Furthermore, to reveal the arterial variations, a preoperative CT/MR-angiography would provide faster recuperation and better postoperative life quality. Clin. Anat. 31:544-550, 2018. © 2018 Wiley Periodicals, Inc.

Environmental Factors Responsible for Variability of Hepatic Vein Flow: A Doppler Assessment in Healthy Twins.

Doppler interrogation studies of the liver blood flow indicate altered hepatic vein waveforms in association with impaired hepatocellular function. However, little is known about the mechanisms responsible for variations of these parameters in the absence of disease. We aimed to investigate the contribution of heritable and environmental factors to the physiological variability of hepatic vein flow in a twin cohort. Two hundred twenty-eight healthy adult Hungarian twins (69 monozygotic, 45 same-sex dizygotic pairs) underwent Doppler sonography of the hepatic vein. Age- and sex-adjusted heritability of the highest velocity (amplitude of S wave) of hepatic vein flow was negligible. Shared environment contributed to 33% (95% CI, 16%-51%), and unshared environment was responsible for the largest portion (67%; 95% CI, 49%-84%) of the variance. Duration of sports activities was significantly (P < 0.05) related to the magnitude of hepatic vein flow, while other risk factors and lifestyle characteristics had no significant influence. The data suggest that genetic factors have little impact on the parameters of hepatic venous blood flow. The variability observed in healthy twins by the Doppler interrogation can be explained by the effect of unshared environmental components primarily related to regular physical activity. These findings underscore the importance of unique environments in physiological variations of hepatic venous blood flow.

Radiomics-based differentiation of lung disease models generated by polluted air based on X-ray computed tomography data.

BACKGROUND: Lung diseases (resulting from air pollution) require a widely accessible method for risk estimation and early diagnosis to ensure proper and responsive treatment. Radiomics-based fractal dimension analysis of X-ray computed tomography attenuation patterns in chest voxels of mice exposed to different air polluting agents was performed to model early stages of disease and establish differential diagnosis. METHODS: To model different types of air pollution, BALBc/ByJ mouse groups were exposed to cigarette smoke combined with ozone, sulphur dioxide gas and a control group was established. Two weeks after exposure, the frequency distributions of image voxel attenuation data were evaluated. Specific cut-off ranges were defined to group voxels by attenuation. Cut-off ranges were binarized and their spatial pattern was associated with calculated fractal dimension, then abstracted by the fractal dimension -- cut-off range mathematical function. Nonparametric Kruskal-Wallis (KW) and Mann-Whitney post hoc (MWph) tests were used. RESULTS: Each cut-off range versus fractal dimension function plot was found to contain two distinctive Gaussian curves. The ratios of the Gaussian curve parameters are considerably significant and are statistically distinguishable within the three exposure groups. CONCLUSIONS: A new radiomics evaluation method was established based on analysis of the fractal dimension of chest X-ray computed tomography data segments. The specific attenuation patterns calculated utilizing our method may diagnose and monitor certain lung diseases, such as chronic obstructive pulmonary disease (COPD), asthma, tuberculosis or lung carcinomas.

Genetic and Environmental Effects on the Abdominal Aortic Diameter Development / Efeitos Genéticos e Ambientais no Desenvolvimento do Diâmetro da Aorta Abdominal

Abstract Background: Configuration of the abdominal aorta is related to healthy aging and a variety of disorders. Objectives: We aimed to assess heritable and environmental effects on the abdominal aortic diameter. Methods: 114 adult (69 monozygotic, 45 same-sex dizygotic) twin pairs (mean age 43.6 ± 16.3 years) underwent abdominal ultrasound with Esaote MyLab 70X ultrasound machine to visualize the abdominal aorta below the level of the origin of the renal arteries and 1-3 cm above the bifurcation. Results: Age- and sex-adjusted heritability of the abdominal aortic diameter below the level of the origin of the renal arteries was 40% [95% confidence interval (CI), 14 to 67%] and 55% above the aortic bifurcation (95% CI, 45 to 70%). None of the aortic diameters showed common environmental effects, but unshared environmental effects were responsible for 60% and 45% of the traits, respectively. Conclusions: Our analysis documents the moderate heritability and its segment-specific difference of the abdominal aortic diameter. The moderate part of variance was explained by unshared environmental components, emphasizing the importance of lifestyle factors in primary prevention. Further studies in this field may guide future gene-mapping efforts and investigate specific lifestyle factors to prevent abdominal aortic dilatation and its complications.

Resumo Fundamento: A configuração da aorta abdominal relaciona-se com o envelhecimento saudável e uma série de distúrbios. Objetivos: Avaliar efeitos herdáveis e ambientais no diâmetro da aorta abdominal. Métodos: 114 pares de gêmeos adultos (69 monozigóticos e 45 dizigóticos do mesmo sexo), com idade média de 43,6 ± 16,3 anos, foram submetidos a ultrassonografia abdominal com o aparelho Esaote MyLab 70X para visualização da aorta abdominal abaixo da origem das artérias renais e 1-3 cm acima da bifurcação aórtica. Resultados: A herdabilidade ajustada para idade e sexo do diâmetro da aorta abdominal abaixo da origem das artérias renais foi 40% [intervalo de confiança (IC) 95%, 14 – 67%] e acima da bifurcação, 55% (IC 95%, 45 – 70%). Nenhum dos diâmetros aórticos apresentou efeitos ambientais comuns, mas os efeitos ambientais não compartilhados foram responsáveis por 60% e 45% dos traços, respectivamente. Conclusões: Nossa análise mostrou herdabilidade moderada e diferença do diâmetro da aorta abdominal com especificidade de segmento. A parte moderada da variância foi explicada pelo componente ambiental não compartilhado, enfatizando a importância do estilo de vida na prevenção primária. Estudos adicionais nesse campo poderão guiar futuros esforços de mapeamento genético e investigar fatores específicos de estilo de vida para prevenir dilatação da aorta abdominal e suas complicações.

Genetic and Environmental Effects on the Abdominal Aortic Diameter Development.

BACKGROUND: Configuration of the abdominal aorta is related to healthy aging and a variety of disorders. OBJECTIVES: We aimed to assess heritable and environmental effects on the abdominal aortic diameter. METHODS: 114 adult (69 monozygotic, 45 same-sex dizygotic) twin pairs (mean age 43.6 ± 16.3 years) underwent abdominal ultrasound with Esaote MyLab 70X ultrasound machine to visualize the abdominal aorta below the level of the origin of the renal arteries and 1-3 cm above the bifurcation. RESULTS: Age- and sex-adjusted heritability of the abdominal aortic diameter below the level of the origin of the renal arteries was 40% [95% confidence interval (CI), 14 to 67%] and 55% above the aortic bifurcation (95% CI, 45 to 70%). None of the aortic diameters showed common environmental effects, but unshared environmental effects were responsible for 60% and 45% of the traits, respectively. CONCLUSIONS: Our analysis documents the moderate heritability and its segment-specific difference of the abdominal aortic diameter. The moderate part of variance was explained by unshared environmental components, emphasizing the importance of lifestyle factors in primary prevention. Further studies in this field may guide future gene-mapping efforts and investigate specific lifestyle factors to prevent abdominal aortic dilatation and its complications.

Genetic and environmental influence on thyroid gland volume and thickness of thyroid isthmus: a twin study

Objectives Decreased thyroid volume has been related to increased prevalence of thyroid cancer. Subjects and methods One hundred and fourteen Hungarian adult twin pairs (69 monozygotic, 45 dizygotic) with or without known thyroid disorders underwent thyroid ultrasound. Thickness of the thyroid isthmus was measured at the thickest portion of the gland in the midline using electronic calipers at the time of scanning. Volume of the thyroid lobe was computed according to the following formula: thyroid height*width*depth*correction factor (0.63). Results Age-, sex-, body mass index- and smoking-adjusted heritability of the thickness of thyroid isthmus was 50% (95% confidence interval [CI], 35 to 66%). Neither left nor right thyroid volume showed additive genetic effects, but shared environments were 68% (95% CI, 48 to 80%) and 79% (95% CI, 72 to 87%), respectively. Magnitudes of monozygotic and dizygotic co-twin correlations were not substantially impacted by the correction of covariates of body mass index and smoking. Unshared environmental effects showed a moderate influence on dependent parameters (24-50%). Conclusions Our analysis support that familial factors are important for thyroid measures in a general twin population. A larger sample size is needed to show whether this is because of common environmental (e.g. intrauterine effects, regional nutrition habits, iodine supply) or genetic effects.

Genetic and environmental influence on thyroid gland volume and thickness of thyroid isthmus: a twin study.

OBJECTIVES: Decreased thyroid volume has been related to increased prevalence of thyroid cancer. SUBJECTS AND METHODS: One hundred and fourteen Hungarian adult twin pairs (69 monozygotic, 45 dizygotic) with or without known thyroid disorders underwent thyroid ultrasound. Thickness of the thyroid isthmus was measured at the thickest portion of the gland in the midline using electronic calipers at the time of scanning. Volume of the thyroid lobe was computed according to the following formula: thyroid height*width*depth*correction factor (0.63). RESULTS: Age-, sex-, body mass index- and smoking-adjusted heritability of the thickness of thyroid isthmus was 50% (95% confidence interval [CI], 35 to 66%). Neither left nor right thyroid volume showed additive genetic effects, but shared environments were 68% (95% CI, 48 to 80%) and 79% (95% CI, 72 to 87%), respectively. Magnitudes of monozygotic and dizygotic co-twin correlations were not substantially impacted by the correction of covariates of body mass index and smoking. Unshared environmental effects showed a moderate influence on dependent parameters (24-50%). CONCLUSIONS: Our analysis support that familial factors are important for thyroid measures in a general twin population. A larger sample size is needed to show whether this is because of common environmental (e.g. intrauterine effects, regional nutrition habits, iodine supply) or genetic effects.

Modest genetic influence on bronchodilator response: a study in healthy twins.

AIM: To determine the reasons for large standard deviation of bronchodilator response (BDR) and establish whether there is a potential heritable component in healthy subjects. METHODS: 67 monozygotic and 42 dizygotic adult twin pairs were assessed for bronchodilator response (% change in FEV1 after inhaling 400 µg salbutamol). Univariate quantitative genetic modeling was performed. RESULTS: Multiple regression modeling showed a significant association between BDR and sex and baseline FEV1 (P<0.05), while no association was found with smoking habits, body mass index, or age. Within pair correlation in monozygotic twins was modest (0.332), but higher than in dizygotic twins (0.258). Age-, sex-, and baseline FEV1-adjusted genetic effect accounted for 14.9% (95% confidence interval, CI 0%-53.1%) of the variance of BDR, shared environmental effect for 18.4% (95% CI 0%-46.8%), and unshared environmental effect for 66.8% (95% CI 46.8%-88.7%). CONCLUSION: Our twin study showed that individual differences in BDR can be mostly explained by unshared environmental effects. In addition, it is the first study to show low, insignificant hereditary influences, independently from sex, age, and baseline FEV1.

Two positive tuberculosis cases in the late Nigrovits family, 18th century, Vác, Hungary.

Two mummies of the Hungarian mummy collection from Vác were the subjects of anthropological, paleopathological, radiological, paleomicrobiological, paleohistological and paleoproteomic studies. Both individuals belonged to the same family. The father, József Nigrovits (No 29), died at the age of 55 on the 11th of November 1793; his son, Antal Nigrovits (No 54), died on the 16th of July 1803, at the age of 22. They lived in the 18th century in Vác, a small town in northern Hungary. The macroscopic examination of the son showed a severely deformed neck and back region; the father has no visible mark of any illnesses. As earlier researches showed that tuberculosis was widespread in the community, the etiology of these deformities was examined. The paleomicrobiological results found that both individuals were infected with tuberculosis. Although they suffered from TB, the CT scan data of the bodies and their 3D reconstructions showed no skeletal evidence of tuberculosis. The deformity of the son turned to be a developmental abnormality of unknown origin, but no Pott's gibbus was present.

Clinical value of whole-body magnetic resonance imaging in health screening of general adult population.

BACKGROUND: Whole-body magnetic resonance imaging (WB-MRI) and angiography (WB-MRA) has become increasingly popular in population-based research. We evaluated retrospectively the frequency of potentially relevant incidental findings throughout the body. MATERIALS AND METHODS: 22 highly health-conscious managers (18 men, mean age 47±9 years) underwent WB-MRI and WB-MRA between March 2012 and September 2013 on a Discovery MR750w wide bore 3 Tesla device (GE Healthcare) using T1 weighted, short tau inversion recovery (STIR) and diffusion weighted imaging (DWI) acquisitions according to a standardized protocol. RESULTS: A suspicious (pararectal) malignancy was detected in one patient which was confirmed by an endorectal sonography. Incidental findings were described in 20 subjects, including hydrocele (11 patients), benign bony lesion (7 patients) and non-specific lymph nodes (5 patients). Further investigations were recommended in 68% (ultrasound: 36%, computed tomography: 28%, mammography: 9%, additional MRI: 9%). WB-MRA were negative in 16 subjects. Vascular normal variations were reported in 23%, and a 40% left proximal common carotid artery stenosis were described in one subject. CONCLUSIONS: WB-MRI and MRA lead to the detection of clinically relevant diseases and unexpected findings in a cohort of healthy adults that require further imaging or surveillance in 68%. WB-MR imaging may play a paramount role in health screening, especially in the future generation of (epi)genetic based screening of malignant and atherosclerotic disorders. Our study is the first which involved a highly selected patient group using a high field 3-T wide bore magnet system with T1, STIR, MRA and whole-body DWI acquisitions as well.

Three-dimensional imaging of past skeletal TB: From lesion to process.

3D imaging has become an essential tool in the field of biological anthropology, notably for human evolution purposes. High resolution virtual 3D reconstructions of original specimens contribute to their preservation and broaden the ability for research, teaching and exchanges. Paleopathology can get substantial benefit from these methods, among others for reconstructing infectious pathological processes on ancient bones. Tuberculosis is frequently diagnosed on ancient human remains; however, some osseous expressions are difficult to interpret using classical methods. We illustrate here the interest of 3D methods for reconstructing processes involved in pathological bone changes due to Mycobacterium tuberculosis infection. Four paleopathological specimens attributed to this infection, dating from different time periods and concerning diverse parts of the skeleton have been analyzed using a specific 3D digital chain we have previously developed. These 3D analyses allow to virtually reconstruct the initial location and aspect of the infectious process, its extension as well as its possible diffusion to the surrounding soft tissues. This possible virtual follow-up of the disease leads to the concept of processual paleopathology that we would like to introduce in the field. The 3D methodology can help to improve our knowledge of natural history and evolution of ancient human infections such as tuberculosis.

Transmission of second-hand smoke sensitivity and smoking attitude in a family.

INTRODUCTION AND OBJECTIVE: The role of genetic factors in nicotine dependence is well understood, but no information is available on the inheritability of second-hand smoke (SHS) exposure sensitivity and their co-variance. MATERIALS AND METHODS: 186 adult same-gender pairs of twin (146 monozygotic, 40 dizygotic; 44±17 years±SD) completed a questionnaire. RESULTS: The model showed a significant role of unshared environmental factors influencing the co-variance between smoking habit and SHS sensitivity (re=-0.191, 95% CI, -0.316 to -0.056, or the total phenotypic correlation of rph=-0.406, p<0.001) without evidence for genetic covariation. Age, gender and country-adjusted analysis indicated 51.5% heritability for smoking habit (95% confidence interval/CI/, 6.2 to 89.8%), 49.7% for SHS sensitivity (95%CI, 19.1-72.0%), 35.5% for general opinions on SHS exposure in restaurants/cafés (95%CI, 10.7-58.6%), and 16.9% in pubs/bars (95%CI, 0.0-49.0%). CONCLUSIONS: The co-variance between SHS sensitivity and smoking habits is driven mainly by the unshared environment. SHS sensitivity is moderately inheritable. The considerable influence of environmental factors on general opinions on SHS exposure in designated indoor public venues emphasizes the importance of smoking bans and health behaviour interventions at the individual level in developing an anti-smoking attitude.

Bosniak category III cysts are more likely to be malignant than we expected in the era of multidetector computed tomography technology.

BACKGROUND: Complex indeterminate Bosniak category III renal cystic masses are traditionally considered to be malignant in 50%. Our aim was to retrospectively evaluate the attenuation characteristics in multiphase computed tomography (CT) and to determinate the incidence of malignancy based on histological findings on all Bosniak category III renal cystic masses investigated in our department between April 3, 2007 and November 21, 2013. MATERIALS AND METHODS: QUADRIPHASIC MULTIDETECTOR CT IMAGES OF NINETEEN PATIENTS (MEAN AGE: 56.5 ± 16.5 years) with radiologically detected Bosniak category III lesions were reviewed retrospectively. All lesions were surgically removed, and the incidence of malignancy, based on pathological results was determined. RESULTS: Calcification was present in four lesions (21%). The mean largest diameter was 48.7 ± 28.8 mm. All lesions were multilobulated and septated. Of the 19 removed lesions, 16 (84%) were malignant, and 3 (16%) were benign (one inflammatory cyst including a nephrolith, one cystic nephroma and one atypical angiomyolipoma). CT and histological findings of 19 Bosniak III cysts were correlated. CONCLUSION: Our study demonstrated much higher prevalence of malignancy (84%) in radiologically detected Bosniak category III cysts than it has been described before. It may due to the era of modern multidetector CT technology and multiphase protocol.

Leukoencephalopathy, cerebral calcifications and cysts: a family study.

We present a clinical, neuro-radiological and genetic study on a family with members suffering from an autosomal dominantly inherited syndrome characterised by epilepsy, cerebral calcifications and cysts, bone abnormalities; progressive neuro-cognitive deterioration and paranasal sinusitis. This syndrome shares several features with leukoencephalopathy with calcifications and cysts also called Labrune syndrome and the condition of cerebroretinal microangiopathy with calcifications and cysts (CRMCC; Coats plus syndrome). Genetic studies in this family did not reveal mutations in the CTC1 gene defected in CRMCC. We interpret our results as those supporting recent findings that despite clinical similarities, late-onset Labrune and Coats plus syndrome might be distinct entities. This family may have Labrune syndrome or a yet unclassified entity; exploration of similar cases could help classifying this one, and related conditions.

The utility of the combination of the corrosion cast method and post mortem MSCT scans.

A careful post-mortem investigation is needed to determine the cause of death of patients bearing coronary stents and to describe complications of stent implantation. The main purpose of this study was to combine post mortem methods of CT angiography and corrosion cast preparation for the visualization of coronary stenoses, coronary stents, instent restenosis, and stent occlusion. Injection-corrosion method was combined with post-mortem MSCT angiography to characterize the pathomorphological changes after stent implantation in 6 male cadaver hearts. Multi-slice computed tomography was employed to visualize the coronary artery system. For image post processing, multiplanar reconstructions, maximal intensity projections and three dimensional reconstructions were used. This study was assessing the feasibility of post mortem MSCT for intracoronary stent evaluation. We described a method for characterization of the coronary side branch stenosis caused by stent implantation. Post mortem CT imaging proved to be a feasible and highly reproducible technique for the characterization of pathological changes in the coronary system.