Dedifferentiated liposarcoma of the gallbladder: first reported case

Background

Liposarcoma of the gallbladder is an extremely rare sarcoma, with only five cases reported in the literature according to our knowledge.

Case presentation

A 71-year-old woman was referred to the Surgical Oncology Division of Napoleão Laureano Hospital (João Pessoa, PB, Brazil) due to a solid mass at the right side of the abdomen and fever, with no signs of jaundice. Abdominal ultrasonography and computed tomography (CT) evidenced an extensive gallbladder lobular formation adhered to the inferior border of the right hepatic lobe and cholelithiasis. The CT report suggested gallbladder liposarcoma. A cholecystectomy associated with resection of segments IV-B and V of the liver were performed. Intraoperative frozen sections were compatible with gallbladder sarcoma. Anatomopathological examination and immunohistochemistry confirmed dedifferentiated liposarcoma with foci of heterologous leiomyosarcomatous differentiation and undifferentiated fusocellular areas of high histological grade.

Conclusion

This is the first case of dedifferentiated liposarcoma of the gallbladder to be reported.

     

Incidence and outcome of prosthetic valve endocarditis after transcatheter aortic valve replacement in the Netherlands

Background

Transcatheter aortic valve replacement (TAVR) is increasingly being used as an alternative to conventional surgical valve replacement. Prosthetic valve endocarditis (PVE) is a rare but feared complication after TAVR, with reported first-year incidences varying from 0.57 to 3.1%. This study was performed to gain insight into the incidence and outcome of PVE after TAVR in the Netherlands.

Methods

A multicentre retrospective registry study was performed. All patients who underwent TAVR in the period 2010–2017 were screened for the diagnosis of infective endocarditis in the insurance database and checked for the presence of PVE before analysis of general characteristics, PVE parameters and outcome.

Results

A total of 3968 patients who underwent TAVR were screened for PVE. During a median follow-up of 33.5 months (interquartile range (IQR) 22.8–45.8), 16 patients suffered from PVE (0.4%), with a median time to onset of 177 days (IQR 67.8–721.3). First-year incidence was 0.24%, and the overall incidence rate was 0.14 events per 1000 person-years. Overall mortality during follow-up in our study was 31%, of which 25% occurred in hospital. All patients were treated conservatively with intravenous antibiotics alone, and none underwent a re-intervention. Other complications of PVE occurred in 5 patients (31%) and included aortic abscess (2), decompensated heart failure (2) and cerebral embolisation (1).

Conclusion

PVE in patients receiving TAVR is a relatively rare complication and has a high mortality rate.

     

A case of colonization of a prosthetic mitral valve by Acremonium strictum

A case of colonization of a prosthetic mitral valve in a 73-year-old Spanish male by the fungus Acremonium strictum W. Gams is described. The valve was replaced due to paravalvular leak and severe insufficiency and the patient died of multiorgan failure. The identity of the fungus was determined by morphological studies and it was confirmed by the analysis of the ITS region sequence analysis. Molecular studies seem to demonstrate that A. strictum is a species complex. The case emphasizes the potential high risk of fungal infection for patients with prosthetic valves.     

Intracardiac echocardiographic guidance for hemodynamic assessment in a patient with congenital abnormalities and a prosthetic aortic valve

In this case report, we present the use of intracardiac echocardiography (ICE) for guiding the cardiac catheterization and subsequent hemodynamic investigation in an unusual patient case with multiple congenital abnormalities (bicuspid aortic valve, left cervical aortic arch, two aortic coarctations) and two aortic valve replacement operations in the past. The ICE catheter (AcuNav) permitted us to accurately and safely puncture the interatrial septum and place the Swan-Ganz catheter in the left ventricle; additionally, visualization of the aortic coarctation in the ascending aorta was also achieved.     

Chimney sweeper's disease revisited: first case reported in a black. Case report.

We present a case of occupation-related carcinoma of the scrotum (chimney sweeper's disease) in a black man. The history of this disease is outlined.     

Undiagnosed hypertrophic obstructive cardiomyopathy during transcatheter aortic valve replacement: a case report

Background

Transcatheter aortic valve replacement is indicated for severe symptomatic aortic stenosis in patients who have a very high or prohibitive surgical risk as assessed pre-procedurally by the Society of Thoracic Surgery Risk Score, EuroSCORE (II), frailty testing, and other predictors. When combined with another left ventricular outflow tract obstruction, careful consideration must be taken prior to proceeding with transcatheter aortic valve replacement because an additional masked left ventricular outflow tract pathology can lead to challenging hemodynamics in the peri-deployment phase, as reported in this case.

Case presentation

A 56-year-old Caucasian man with multiple comorbidities and severe aortic stenosis underwent transcatheter aortic valve replacement under monitored anesthesia care. During the deployment phase, he developed dyspnea that progressed to pulmonary edema requiring emergent conversion to general anesthesia, orotracheal intubation, acute respiratory distress syndrome-type ventilation, and vasopressor medications. Intraoperative transesophageal echocardiography was performed and hypertrophic obstructive cardiomyopathy with systolic anterior motion of the mitral valve was discovered as an underlying pathology, undetected on preoperative imaging. After treatment with beta blockers, fluid resuscitation, and alpha-1 agonists, he stabilized and was eventually discharged from our hospital without any lasting sequelae.

Conclusions

Patients with aortic stenosis most often develop symmetric hypertrophy; however, a small subset has asymmetric septal hypertrophy leading to left ventricular outflow tract obstruction. In cases of severe aortic stenosis, however, evidence of left ventricular outflow tract obstruction via both symptoms and echocardiographic findings may be minimized due to extremely high afterload on the left ventricle. Diagnosing a left ventricular outflow tract obstruction as the cause of hemodynamic instability during transcatheter aortic valve replacement, in the absence of abnormal findings on echocardiogram preoperatively, requires a high index of clinical suspicion. The management of acute onset left ventricular outflow tract obstruction intraoperatively consists primarily of medical therapy, including rate control, adequate volume resuscitation, and avoidance of inotropes. With persistently elevated gradients, interventional treatments may be considered.

     

Disseminated lymphoma in a rhesus monkey: a case report.

A rhesus monkey was inoculated with rhesus cytomegalovirus. A leukocyte-associated herpes virus, unrelated to cytomegalovirus, was later isolated from the same monkey. Four years after the virus inoculation, the monkey developed a disseminated lymphoma.     

Syngamosis,an unusual cause of asthma: the first reported case in Canada.

The first case of syngamosis in a human in Canada is reported. The patient, a traveller to the Caribbean islands, presented with a chronic dry cough. The nematode Syngamus laryngeus is found in wild and domestic birds and mammals in the tropics and subtropics. Humans are only accidental hosts. The diagnosis of syngamosis is usually made by fibreoptic bronchoscopic examination, which reveals the Y-shaped worms in the bronchi or the characteristic eggs in the sputum or feces. Physicians must be alerted to the possibility of syngamosis in patients with symptoms of asthma who have recently travelled to South America or the Caribbean islands.     

A new family of type III polyketide synthases in Mycobacterium tuberculosis

The Mycobacterium tuberculosis genome has revealed a remarkable array of polyketide synthases (PKSs); however, no polyketide product has been isolated thus far. Most of the PKS genes have been implicated in the biosynthesis of complex lipids. We report here the characterization of two novel type III PKSs from M. tuberculosis that are involved in the biosynthesis of long-chain alpha-pyrones. Measurement of steady-state kinetic parameters demonstrated that the catalytic efficiency of PKS18 protein was severalfold higher for long-chain acyl-coenzyme A substrates as compared with the small-chain precursors. The specificity of PKS18 and PKS11 proteins toward long-chain aliphatic acyl-coenzyme A (C12 to C20) substrates is unprecedented in the chalcone synthase (CHS) family of condensing enzymes. Based on comparative modeling studies, we propose that these proteins might have evolved by fusing the catalytic machinery of CHS and beta-ketoacyl synthases, the two evolutionarily related members with conserved thiolase fold. The mechanistic and structural importance of several active site residues, as predicted by our structural model, was investigated by performing site-directed mutagenesis. The functional identification of diverse catalytic activity in mycobacterial type III PKSs provide a fascinating example of metabolite divergence in CHS-like proteins.     

Pollination of Specklinia by nectar-feeding Drosophila: the first reported case of a deceptive syndrome employing aggregation pheromones in Orchidaceae

Background and Aims The first documented observation of pollination in Pleurothallidinae was that of Endrés, who noticed that the ‘viscid sepals’ of Specklinia endotrachys were visited by a ‘small fly’. Chase would later identify the visiting flies as being members of the genus Drosophila. This study documents and describes how species of the S. endotrachys complex are pollinated by different Drosophila species.Methods Specimens of Specklinia and Drosophila were collected in the field in Costa Rica and preserved in the JBL and L herbaria. Flies were photographed, filmed and observed for several days during a 2-year period and were identified by a combination of non-invasive DNA barcoding and anatomical surveys. Tissue samples of the sepals, petals and labellum of Specklinia species were observed and documented by SEM, LM and TEM. Electroantennogram experiments were carried out on Drosophila hydei using the known aggregation pheromones ethyl tiglate, methyl tiglate and isopropyl tiglate. Floral compounds were analysed by gas chromatography–mass spectometry using those same pheromones as standards.Key Results Flowers of S. endotrachys, S. pfavii, S. remotiflora and S. spectabilis are visited and pollinated by several different but closely related Drosophila species. The flies are arrested by aggregation pheromones, including ethyl tiglate, methyl tiglate and isopropyl tiglate, released by the flowers, and to which at least D. hydei is very sensitive. Visible nectar drops on the adaxial surface of sepals are secreted by nectar-secreting stomata, encouraging male and female Drosophila to linger on the flowers for several hours at a time. The flies frequently show courtship behaviour, occasionally copulating. Several different Drosophila species can be found on a single Specklinia species.Conclusions Species of the S. endotrachys group share a similar pollination syndrome. There seem to be no species-specific relationships between the orchids and the flies. It is not expected that Specklinia species will hybridize naturally as their populations do not overlap geographically. The combination of pheromone attraction and nectar feeding is likely to be a generalized pollination syndrome in Pleurothallidinae.     

Identification of type III protein kinase C in bovine aortic tissue

We identified a subtype of protein kinase C in bovine aortic tissue. In Western blots, both the soluble and the particulate fractions from the aorta reacted only with MC-3a. In the case of hydroxylapatite column chromatography, a single activity peak of protein kinase C from the soluble and the particulate fractions was obtained with about 140 mM of potassium phosphate, a finding similar to that with the Type III protein kinase C from rabbit brain. The sandwich-type enzyme immunoassay for protein kinase C, with which the contents of each protein kinase C isozyme can be determined in the crude extracts, revealed that the Type III bovine aortic protein kinase C included 25.9 ng/mg protein. These results strongly suggest that it is the Type III protein kinase C which is mainly expressed in aortic tissue. Kinetic parameters of the Type III protein kinase C of the soluble and the particulate fractions, with respect to the Km for ATP, were 33 and 15 microM and the Km values for myosin light chain from chicken gizzard were 6.3 and 4.6 microM, respectively.     

In vitro flow dynamics of four prosthetic aortic valves: a comparative analysis

The velocity fields downstream of four prosthetic heart valves were mapped in vitro over the entire cross-section of a model aortic root using laser Doppler anemometry. THe Bj?rk-Shiley 60 degrees convexo-concave tilting disc valve, the Smeloff-Cutter caged ball valve, the St. Jude Medical bileaflet valve, and the Ionescu-Shiley standard bioprosthesis were examined under both steady and pulsatile flows. Velocity profiles under steady flow conditions were a good approximation for pulsatile profiles only during midsystole. The pulsatile flow characteristics of the four valves showed variation in large scale flow structures. Comparison of the valves according to pressure drop, shear stress and maximum velocities are also provided.     

Definition of the surface antigens of Mycobacterium malmoense and use in studying the etiology of a form of mycobacteriosis

Mycobacterium malmoense is the latest of a roster of atypical mycobacteria implicated in pulmonary infections. Yet it lacks recognizable phenotypic features to allow its ready identification. Some 23 clinical isolates of M. malmoense were examined for homologous seroagglutination reactions and characteristic surface antigens. One group showed concordant agglutination interreactions and an identical spectrum of glycolipids and are regarded as M. malmoense sensu stricto. The glycolipids are of the newly found, trehalose-containing lipooligosaccharide class. De-O-acylation followed by high-pressure liquid chromatography revealed one major and several minor oligosaccharides. Partial acidic cleavage to release glycosidically linked trehalose, alpha-mannosidase digestion to demonstrate the presence of a non-reducing-end mannobiose, perdeuteriomethylation, partial acid hydrolysis, reduction, and O ethylation, combined with 1H nuclear magnetic resonance and electron impact and fast-atom bombardment mass spectrometry revealed the structure of the major oligosaccharide as alpha-D-Manp-(1----3) -alpha-D-Manp-(1----[2-alpha-L-Rhap-(1--]4--3)-alpha-L-Rh ap- (1----3)-alpha-D-Glcp-(1----1)-alpha-D-Glcp, in which two of the 2-alpha-L-Rhap residues are O methylated at C-3. (Man, mannose; Rha, rhamnose; Glc, glucose; p, pyranosyl). The structures of the minor oligosaccharides were also determined; they differ at the distal nonreducing end. The dominant oligosaccharide was acylated by octanoate, 2-methyleicosanoate, and 2,4-dimethylpentacosanoate to yield the major species-specific surface antigen of M. malmoense, which we regard as the most characteristic feature of the pathogen.     

Yersinia yopQ mRNA encodes a bipartite type III secretion signal in the first 15 codons

The type III machinery of Yersinia transports Yop proteins across the bacterial envelope. The minimal secretion signal of yopQ is located in codons 1-10 that, when fused in frame to the neomycin phosphotransferase gene, is sufficient to promote type III secretion of YopQ(1-10)-Npt. Frame-shift mutations, generated by nucleotide insertions or deletions following the AUG start and suppressed at the fusion site with npt, abrogate signalling of yopQ(1-10) but not of yopQ(1-15). By generating transversions of every single nucleotide in yopQ(1-10), we identified 10 nucleotide positions in codons 2, 3, 5, 7, 9 and 10 that were each required for substrate recognition. One transversion that abolishes secretion, uridyl 9 to adenyl (U9A), is a synonymous codon 3 mutation that retains the original amino acid as confirmed by Edman degradation analysis, suggesting that the mRNA but not the amino acid sequence of yopQ(1-10) is involved in secretion signalling. Although transversion of U8A abrogates signalling of yopQ(1-10), fusion of yopQ codons 11-15 restores secretion. The nucleotides that are required for this suppression by yopQ(11-15) were identified and revealed both synonymous and non-synonymous mutations. Frame-shift mutations introduced into just this suppressor region (codons 11-15) did not abrogate its ability to suppress mutations in the minimal secretion signal (codons 1-10). Thus, elements downstream of the minimal secretion signal of YopQ increase the efficiency of YopQ secretion and suppress mutations elsewhere in the secretion signal.     

Risk evaluation of adverse aortic events in patients with non-circular aortic annulus after transcatheter aortic valve implantation: a numerical study

Biomechanics and Modeling in Mechanobiology - Transcatheter aortic valve implantation (TAVI) is a micro-invasive surgery used to treat patients with aortic stenosis (AS) efficiently. However, the...     

宏基因组学诊断播散型组织胞浆菌病1例

播散型组织胞浆菌病是一种进行性肺外疾病好发于免疫缺陷者。该病诊断的应结合组织胞浆菌病的高危因素(免疫抑制剂、高龄、高风险的暴露等)与临床表现。本文报道一例播散型组织胞浆菌病但免疫功能健全的病例,其表现为亚急性起病,后期病程进展迅速,骨髓涂片及宏基因测序诊断为播散型组织胞浆菌病。予以两性霉素B去氧胆酸盐及伊曲康唑治疗后病情明显好转。