Intramammary Angiomatoid Fibrous Histiocytoma, a Rare EWSR1 Rearranged Mesenchymal Neoplasm in a Previously Unreported Anatomic Location with Review of the Cleveland Clinic Experience.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that is most commonly reported to arise in the subcutaneous tissues of the upper extremities in adolescents and young adults. At present, the WHO classifies this neoplasm as a tumor of uncertain differentiation. AFH is most often clinically regarded as a tumor of intermediate risk due to low reported rates of recurrence and only rare occurrences of metastases. Its histomorphological hallmarks are a prominent lymphoid cuff surrounding a spindle cell neoplasm with syncytial-appearing cytoplasm. Several variant morphologies have been described. Genetically, the tumor is characterized by translocations involving the EWSR1 gene in over 90% of cases. A widening range of anatomical locations and morphological variants of AFH has been reported in the literature; however, neither anatomic location nor specific morphologic features have been shown to correlate with clinical/biological behavior. We report a unique case of AFH arising in the parenchyma of the breast. The neoplasm showed the typical histomorphology including a peripheral lymphoid cuff. The lesional cells in this case were found to be immunoreactive with desmin, and a positive EWSR1 result was confirmed by break-apart fluorescence in situ hybridization testing. To our knowledge, this is the first report of AFH arising in the breast parenchyma of a postmenopausal female.

Patient-reported outcomes after Silastic replacement of the trapezium for osteoarthritis.

This retrospective study evaluated 69 Swanson trapezium replacements performed between 1990 and 2009 for trapeziometacarpal osteoarthritis in 58 patients. Pain and function were assessed using the Michigan Hand Questionnaire and the Disability of the Arm, Shoulder and Hand questionnaire. Patients had a mean age of 62 years at the time of surgery, with a mean time of 7.7 years (range 9 months to 19 years) from surgery to follow-up interview. There was no association between outcome scores and the length of follow-up, suggesting that the results are maintained over time (Spearman's rank correlation test < ±0.2). Scores for activities of daily living and work-related activities were higher when surgery was on the dominant hand (p < 0.05). Silicone trapezium replacement remains a good option for patients with painful trapeziometacarpal osteoarthritis that has not responded to nonoperative management.

"S" Quattro external fixation for complex intra-articular thumb fractures.

PURPOSE: To report outcomes in 10 patients who underwent dynamic "S" Quattro external fixation for complex fractures of the base of the thumb. METHODS: Nine men and one woman aged 18 to 69 (mean, 31) years underwent "S" Quattro external fixation for complex fractures of the base of the thumb. The dominant hand was involved in 8 patients. Three patients had Bennett fractures, 5 had Rolando fractures, one had an open multi-fragmented fracture, and one had a fracture-subluxation. Four of them had had prior (failed) treatment with splints and/or Kirschner wires. The "S" Quattro external fixator was applied for a mean of 4.9 weeks. Patients were followed up in an out-patient setting for a mean of 10.7 months until bone union and removal of the external fixator. Finger flexor function was assessed based on total active movement (TAM). Functional outcomes were assessed using the Disability of Arm, Shoulder and Hand (DASH) questionnaire. RESULTS: No pin-site infection, malunion, or non-union was encountered. Mean loss of TAM was 7.5 degrees. Five patients lost 10 degrees or more, 2 of whom lost 20 degrees (one with an open comminuted fracture and one was elderly). Four patients regained full TAM and 6 attained more than 75% TAM compared to the contralateral thumb. At the 3-year follow-up, the mean DASH score was 3.4. Four patients reported no functional disability. Poorer outcomes were reported in the 2 patients who once had lost 20 degrees of TAM. CONCLUSION: The "S" Quattro external fixator is recommended as a primary and definitive treatment modality for complex intra-articular thumb fractures when conservative and other surgical interventions have failed.

Posterior compartment syndrome associated with clopidogrel therapy following trivial trauma.

Haematomata caused by blunt trauma may potentially induce a compartment syndrome by raising intra-compartmental pressure. We report a case of acute posterior compartment syndrome following minimal trauma to the leg of an elderly patient on the antiplatelet agent clopidogrel. This case highlights the high index of clinical suspicion required to detect compartment syndrome in those on long term antiplatelet therapy and prompt surgical decompression is recommended.

Outcome measures following metacarpophalangeal joint replacement.

We used the Jebsen-Taylor hand function assessment system to prospectively study the effect of metacarpophalangeal joint replacement on overall hand function in 29 hands. In addition pain relief, subjective improvement in hand function, appearance and overall patient satisfaction were assessed. There was modest improvement in the number of Jebsen-Taylor tasks performed (1.8 to 3. 1), and pain relief was good or excellent in 18 of 24 patients. Eleven patients felt their hand function had improved by more than 50%, and the majority of patients (22 of 24) were very satisfied with the procedure. This study demonstrates that despite limited improvements in objective outcome measures, this procedure is reliable in producing a very high rate of patient satisfaction.

Purified dengue 2 virus envelope glycoprotein aggregates produced by baculovirus are immunogenic in mice.

The full-length dengue 2 virus envelope glycoprotein (Egp) was expressed in insect cells by recombinant (r) baculovirus and found to form multimeric aggregates that were recovered in the void volume of gel filtration columns and by ultracentrifugation. An immunoblot confirmed that rEgp aggregrates disrupted with SDS sample buffer released a monomeric form that migrated with a molecular weight similar to native dengue 2 virus Egp on polyacrylamide gels. The rEgp aggregates reacted strongly with a panel of monoclonal antibodies specific for the native Egp and which identify critical structural and functional epitopes. The rEgp aggregates were purified by ultracentrifugation through 30% sucrose, and were shown to be the major protein band on a polyacrylamide gel and corresponding immunoblot. Purified rEgp aggregates in combination with aluminum hydroxide induced high titer neutralizing antibodies in adult mice. The generation of full-length dengue 2 rEgp aggregates in insect cells facilitated development of a simple, effective procedure for purification of the recombinant protein, and represents a good approach for producing highly immunogenic dengue 2 rEgp as a component of a subunit vaccine.

Use of the "S" Quattro dynamic external fixator in the treatment of difficult hand fractures.

Thirty-seven patients were treated over a 7-year period using the "S" Quattro dynamic external fixator. There were 30 intra-articular and nine extra-articular fractures. Patients were reviewed at an average of 22.5 months. The average total range of motion for the affected digit at follow-up was 232 degrees for intra-articular and 241 degrees for extra-articular fractures. We believe that this device is a simple, reliable technique in the treatment of these difficult fractures.

A comparative genetic analysis of the Irish greyhound population using multilocus DNA fingerprinting, canine single locus minisatellites and canine microsatellites.

Pairwise analysis of HinfI/33.6 DNA fingerprints from a total of one hundred and fifty-three Irish greyhounds of known pedigree were used to determine band-share estimates of unrelated, first-degree and second-degree relationships. Forty-eight unrelated Irish greyhounds were used to determine allele frequencies for three single-locus minisatellites, and following a preliminary screen, eight of the most polymorphic tetra-nucleotide microsatellites from a panel of 15. The results indicated that both band-share estimates by DNA fingerprinting and microsatellite allele frequencies are highly effective in resolving parentage in this greyhound population, while single-locus minisatellites showed limited polymorphism and could not be used alone for routine parentage testing in this breed. The present study also demonstrated that, to obtain optimal resolution of parentage, sample sets of known pedigree status are required to determine the band-share distribution and/or microsatellite allele frequencies.

Linear assessment of the thoroughbred horse: an approach to conformation evaluation.

A linear assessment trait evaluation system is proposed to allow quantitative description of the static conformation of the horse. Measurements were made on 27 selected traits. The system was tested initially for repeatability of measurements on 4 horses. Twenty-one of the selected traits were satisfactory and 6 proved unsatisfactory in terms of reproducibility. A population of 101, superior 2- and 3-year-old Thoroughbreds and 19 premium Thoroughbred stallions were similarly assessed. More than 65% of the traits exhibited large (CV > 10%) phenotypic variation within the sampled population. It is proposed that such a system of static conformation assessment, in conjunction with a similar system for dynamic linear assessment, would provide useful quantitative selection criteria in the description and breeding of horses.

Murine immunoglobulin G subclass responses following immunization with live dengue virus or a recombinant dengue envelope protein.

Murine immunoglobulin G (IgG) subclass responses to immunization are restricted to certain subclasses depending on the nature of the immunogen. Immunization with live viruses generally leads to a predominant IgG2a response, which may be the most effective at resisting future challenge due to the unique effector functions of IgG2a. Knowledge of subclass responses following immunization with dengue vaccine candidates may be helpful in determining which candidates are most efficacious. We measured the dengue-specific IgG subclass responses of BALB/c mice following immunization with live dengue-2 virus or with a partially purified recombinant dengue-2 envelope (E) protein. Subclass responses following immunization with live virus were IgG2a > IgG1 > IgG2b > IgG3, as opposed to IgG1 > IgG2a > IgG2b > IgG3 after immunization with recombinant protein. Responses of all subclasses except IgG1 were greater following immunization with live dengue than with the recombinant E protein. Neutralizing antibody titers were also higher after immunization with live virus than with E protein and were positively correlated with dengue-specific IgG2a responses in mice immunized with recombinant E protein. Following separation of the four IgG subclasses by chromatography, the IgG2a fraction exhibited the greatest neutralizing activity. The results seen after immunization with live dengue virus or recombinant E protein in this study are in concordance with studies involving other viruses and viral proteins and may have implications for the development of an effective vaccine for dengue.

Elbow fractures with carpal injuries.

We report four cases with six episodes of concurrent carpal and elbow fractures or dislocations. Few such combinations of injuries have been reported in the literature. We discuss the mechanism and management of such injuries and conclude that elbow injuries should be suspected in severe carpal injuries. Surgical treatment may be required in their management.

A new method of paternity testing for dogs, based on microsatellite sequences.

Microsatellite sequences, like minisatellites, belong to a class of polymorphic DNA that is commonly found in mammalian DNA. Although they vary significantly less in a population of animals than minisatellites, they have potential for use in paternity disputes. However, their inherently lower variability together with the more genetically homogeneous nature of pedigree dogs due to inbreeding (line breeding), raised doubts about their effectiveness for paternity tests. This paper demonstrates that canine microsatellites provide an adequate basis for assigning paternity in pedigree breeds. The system presented is more straightforward to perform and interpret than that based on canine minisatellites (DNA 'fingerprinting') and requires as little as 0.1 ml of blood.

Reversal of advanced liver fibrosis in rabbits with Schistosomiasis japonica.

Advanced liver fibrosis is generally considered to be irreversible. We studied the reversibility of marked liver fibrosis in rabbits infected with Schistosoma japonicum. We determined liver collagen content, collagen biosynthesis, and collagenase activity using serial biopsy specimens obtained 20, 40, and 60 weeks after infection. Reversibility of this process was investigated in rabbits cured of infection at 21 weeks; control rabbits not cured of infection were also studied. At 20 weeks, liver collagen content was 16-fold greater than normal, with accumulation of collagen types I, III, and V. Synthesis of collagen within fibrotic liver slices was 10-fold greater than normal. Liver collagenolytic activity for a type I substrate was 19-fold greater than normal. After parasitologic cure, a striking morphologic reversal of fibrosis occurred during the subsequent 40 weeks, with the return of liver collagen content to three-fold greater than normal and a 75% decrease in synthetic rates compared with those at 20 weeks (P < 0.01). Collagenolytic activity remained elevated to the same degree noted at 20 weeks. A similar but lesser resolution of fibrosis also occurred in untreated control rabbits, coincident with a spontaneous decrease in new egg deposition known to occur in this model system. We conclude that advanced liver fibrosis in S. japonicum-infected rabbits is slowly reversible after cure or senescence of the infection. A possible mechanism for this reversal is persistently increased collagenolysis as collagen synthesis diminishes.

Baclofen: effects on evoked field potentials and amino acid neurotransmitter release in the rat olfactory cortex slice.

A study has been made of the in vitro effects of (+/-)- and (-)-baclofen on the evoked field potentials and release of endogenous amino acid neurotransmitter candidates (aspartate, glutamate, GABA and possibly taurine) which accompany electrical stimulation of the excitatory input to the olfactory cortex slice, the lateral olfactory tract. Baclofen appears to reduce the excitatory input to the GABA-utilizing inhibitory interneurones; this action was manifest as a drug-induced abolition of the field potential known as the P-wave (IC50 for (-)-baclofen, 1.7 +/- 0.4 microM) together with a simultaneous reduction in the synaptically evoked release of aspartase and glutamate from the cut surface of slices. Both these actions of baclofen exhibited concentration dependence and stereospecificity and were not antagonized by picrotoxin (25 microM) thereby suggesting that they are directly related. The consequence of this action of baclofen was the abolition of GABA-mediated presynaptic and postsynaptic inhibition together with their respective field potential correlates, the late N- and I-waves. (+/-)-Baclofen (5 and 25 microM) also inhibited the potassium-evoked release of aspartate and glutamate from small cubes of tissue but, except at a high concentration (1 mM), had no effect on GABA release. Baclofen (up to 1 mM) did not affect transmission either at the lateral olfactory tract-superficial pyramidal cell synapse, a site where aspartate is the likely neurotransmitter, or at the superficial pyramidal cell collateral-deep pyramidal cell excitatory synapse. It is proposed that: (i) the actions of baclofen on the olfactory cortex are the result of inhibition of aspartate and glutamate release, probably from deep pyramidal cell collaterals; and (ii) not all neurones utilizing excitatory amino acids as their neurotransmitters are subject to the inhibitory action of baclofen.