Nodular fasciitis involving the palm.

Nodular fasciitis (NF) is a self-limiting fibrous neoplasm that can be mistaken for a soft tissue sarcoma. It is characterised by rapid growth, slight pain and local tenderness. Although it is frequently found in the forearm, a lesion distal to the wrist is quite rare. We present two unusual cases of NF involving the palm, supported by detecting ubiquitin specific protease 6 gene rearrangement. The first patient had non-intraneural NF presenting as peripheral neuropathy affecting the digital nerve while the second patient suffered from painless, non-tender NF in the palm, which had not regressed spontaneously during the five months prior to surgery.

Vascularised fibular grafts for reconstruction of extremity bone defects after resection of bone and soft-tissue tumours : a single institutional study of 49 patients.

AIMS: The aims of this study were to analyse the long-term outcome of vascularised fibular graft (VFG) reconstruction after tumour resection and to evaluate the usefulness of the method. PATIENTS AND METHODS: We retrospectively reviewed 49 patients who had undergone resection of a sarcoma and reconstruction using a VFG between 1988 and 2015. Their mean follow-up was 98 months (5 to 317). Reconstruction was with an osteochondral graft (n = 13), intercalary graft (n = 12), inlay graft (n = 4), or resection arthrodesis (n = 20). We analysed the oncological and functional outcome, and the rate of bony union and complications. RESULTS: Five- and ten-year overall survival rates were 89% and 86%, respectively. Local recurrence occurred in two patients. Eight patients developed pulmonary metastases. Bone union was achieved in 44 patients (90%). Fracture occurred in six patients (12%), infection in three (6%), and nonunion in five (10%). The mean Musculoskeletal Tumor Society (MSTS) scores were as follows: osteochondral graft 70%; intercalary graft 73%; inlay graft 89%; and resection arthrodesis 83%. CONCLUSION: Although associated with a relatively high rate of complications, each reconstruction method is useful, with a high rate of successful limb salvage and a good long-term functional outcome. Cite this article: Bone Joint J 2017;99-B:1237-43.

Hand metastasis from a sacral chordoma.

Chordomas are rare, low grade, malignant tumours derived from the ectopic remnants of the notochord that line the axial skeleton. They are characterised by their slow growth, long disease course and propensity for local relapse. Furthermore, up to 40% of non-cranial chordomas metastasise. We describe the first reported case of a hand metastasis arising from a conventional sacral chordoma after carbon ion radiotherapy. The common occurrence of distant metastasis with chordomas makes it important to perform a systemic examination, in part because their resection might improve patient prognosis.

Comparative roles of macrophages and NK cells in the host innate resistance of mice to Mycobacterium fortuitum infection.

OBJECTIVES: Profiles of host innate resistance to Mycobacterium fortuitum (MFT) infection in mice and the roles of macrophages (Mphis) and NK cells in host resistance to MFT infection were studied. METHODS: MFT-infected mice with or without the treatments to reduce Mphis and NK cells were examined for survival and the bacterial loads in the kidneys during the course of infection. RESULTS: A unique profile of strain difference was found in the innate resistance of mice to MFT. A/J, C3H/He and DBA/2 mice were susceptible, while BALB/c, B10A and C57BL/6 mice were resistant, in terms of survival after MFT infection. Such profiles of host resistance to MFT were essentially correlated with the ability of individual strain mice to prevent the bacterial growth in the early periods after infection. These profiles were different from the strain difference controlled by Bcg gene. Studies using carrageenan, anti-asialo GM1 antibody, and NK cell-deficient beige mice indicated the important roles of Mphis and NK cells in the host innate defense against MFT. CONCLUSIONS: These findings suggest that Bcg gene does not control the host resistance to MFT and that both Mphis and NK cells play crucial roles in the host innate resistance to MFT infection.

Evaluation of in vivo therapeutic efficacy of a new benzoxazinorifamycin, KRM-1648, in SCID mouse model for disseminated Mycobacterium avium complex infection.

In this study, profiles of infection due to Mycobacterium avium complex (MAC) in CB-17 SCID mice deficient in T and B cell functions were examined, when mice were given or not given a new benzoxazinorifamycin, KRM-1648 (KRM), during the course of infection. When mice were infected intravenously with MAC, the bacterial loads in their visceral organs were larger than those of their co-isogenic CB-17 counterparts. The incidence and the degree of gross lung lesions were less in SCID mice compared to CB-17 mice. Athymic BALB/c nude mice showed similar profiles of the infection. Beige mice showed more severe gross lesions and larger bacterial loads in the lungs than did SCID and athymic BALB/c nude mice. When MAC was infected subcutaneously into the hind footpads of mice, disseminated growth of organisms in the footpads, blood, and visceral organs was seen in SCID mice, but not in CB-17 or BALB/c mice. KRM exhibited the same level of therapeutic effect on SCID mice infected with MAC via the intravenous route in terms of inhibiting bacterial growth in the lungs and kidneys, as in cases of CB-17 and BALB/c mice with normal T-cell functions. In beige mice, the degree of growth inhibition of MAC due to KRM treatment was significantly greater than that achieved in SCID mice.

[Development of experimental model animals for disseminated Mycobacterium avium complex infections using immunodeficient mice and rats].

In order to establish an animal model for disseminated M. avium complex (MAC) infections frequently encountered in AIDS patients, we studied growth of M. intracellulare in visceral organs (lungs, livers, spleens, kidneys), in blood, and in footpads of mice with defined immunodeficiencies, such as SCID mice with T and B cell-defect, BALB/c athymic nude mice with matured T cell-defect, and beige mice with NK cell-defect. In addition, Sprague-Dawley rats with acquired immunodeficiency induced by cyclosporine-treatment were also examined. The following results were obtained. 1) SCID mice: First, SCID mice were infected sc with 6.1 x 10(6) CFU of M. intracellulare N-260 (virulent SmT colonial variant) into the hind footpad. The organisms grew in the footpad remarkably during the 12 weeks after infection in SCID mice, where the growth rate was much greater than that in CB-17 strain mice with the same genotype as SCID mice and in BALB/c mice with Bcgs genotype (CB-17 and BALB/c mice are MAC-susceptible). Furthermore, in SCID mice, bacteremia and dissemination of organisms to the visceral organs were observed but not in the two control strains of mice. Second, SCID mice were infected i.v. with 4.8 x 10(6) CFU. The bacterial loads in the viscera of SCID mice after infection were larger than those of CB-17 mice except for livers. However, the incidence and the degree of gross lung lesions were much less in SCID mice compared to CB-17 mice, presumably due to the defect in T cell-mediated immune reactions in SCID mice.(ABSTRACT TRUNCATED AT 250 WORDS)

Therapeutic efficacy of the benzoxazinorifamycin KRM-1648 against experimental Mycobacterium avium infection induced in rabbits.

The therapeutic efficacy of the benzoxazinorifamycin KRM-1648 was studied in an experimental rabbit infection system with avian Mycobacterium avium. The infected rabbits died from Yersin type infections, a peculiar type of experimental bovine tuberculosis characterized by a very rapid course, enlargement of the spleen and liver, and septic infection, 14 to 20 days after bacterial challenge, as evidenced by bacteremia and severe bacterial loads in the visceral organs. Histopathologic studies of the visceral organs of the infected rabbits revealed the development of numerous typical granulomatous lesions. This experimental rabbit infection system, features of which resemble certain features of disseminated M. avium complex infections in AIDS patients, was used to evaluate the therapeutic efficacy of KRM-1648, a newly synthesized benzoxazinorifamycin. KRM-1648 given orally at 25 and 50 mg/kg of body weight reduced the incidence and degree of bacteremia in infected rabbits and protected against subsequent death. Moreover, the drug allowed almost complete recovery of infected rabbits by week 7. KRM-1648 cleared infections in the lungs, liver, spleen, and kidneys and restored histopathologic features of healthy tissue in the visceral organs. KRM-1648 exhibited a more potent therapeutic effect against M. avium infection than rifampin and clarithromycin.

[Behaviour of gamma delta TCR+ T cells during the course of nontuberculous mycobacterial infections and proliferative response of host lymphocytes to 65kD heat shock protein].

In order to know the possibility that gamma delta TCR+ T cells induced by Mycobacterium avium complex (MAC) infections participate in the expression of host resistance and in the occurrence of Behçet disease, we examined the behaviour of them in MAC-infected host mice. In both BALB/c (Bcgs; MAC-susceptible) and CBA/JN (Bcgr; MAC-resistant) strain mice, a transient but appreciable increase in the number of gamma delta TCR+ T cells in the host peritoneal lymphocytes was noted around week 1 to 2 after M. intracellulare infection via ip. route. The degree of induction of gamma delta TCR+ T cells was somewhat higher in CBA/JN mice than in BALB/c mice. Therefore, gamma delta TCR+ T cells are partly responsible for the expression of host resistance against the MAC in the early phase of infection. However, the subsequent decrease in the level of gamma delta TCR+ T cells was observed by week 5. Thus, in the case of chronic state of MAC infection, the size of gamma delta TCR+ T cell-pool seems to be in normal level. This suggests that per cell activity of gamma delta TCR+ T cells rather than mobilizing number of them is important factor in the mechanisms for occurrence of allergic diseases including Behçet disease. Although, the early increase in gamma delta TCR+ T cells of peritoneal cells was also observed during the course of M. fortuitum infection, the degree of induction of gamma delta TCR+ T cells in A/J mice (M. fortuitum-susceptible) was in similar level as that in BALB/c mice (M. fortuitum-resistant).(ABSTRACT TRUNCATED AT 250 WORDS)

[Macrophage respiratory burst-inducing activity of Mycobacterium fortuitum: relationship with its virulence to mice].

Mycobacterium fortuitum strains F-3 and 126 were studied for their virulence to mice, by being given intravenously to BALB/c strain mice, in terms of incidence of spinning disease, degree of gross renal lesions and growth of organisms in kidneys. In three experiments separately carried out, strain 126 showed considerably higher virulence than strain F-3. In particular, in experiment 2, much higher incidence of spinning disease was seen in strain 126- infected mice than in strain F-3-infected ones. In experiment 3, the degree of gross renal lesions was significantly higher (P less than 0.025, chi 2-test) in the strain 126-infected animals than in the F-3-infected ones. Moreover, in experiment 2, the number of viable units in kidneys was significantly larger in the case of strain 126-induced infection than in the case of strain F-3-induced one (P less than 0.01, Student's t-test). Secondly the two M. fortuitum strains were studied for their activity to trigger chemiluminescence (a parameter for respiratory burst) of murine peritoneal macrophages, due to their contact with macrophages. In two of four experiments separately performed, strain 126 exhibited much lower activity of macrophage chemiluminescence-triggering than strain F-3. In the remaining two experiments, the triggering activities of the two strains were at almost the same level, although the activity of strain 126 was still somewhat lower than that of strain F-3.(ABSTRACT TRUNCATED AT 250 WORDS)

In vitro antimycobacterial activities of newly synthesized benzoxazinorifamycins.

Newly synthesized rifamycin derivatives, KRM-1648, KRM-1657, KRM-1668, KRM-1686, and KRM-1687, having the chemical structures of 3'-hydroxy-5'-(4-alkylpiperazinyl)-benzoxazinorifamycins (alkyl residues: isobutyl, propyl, sec-butyl, sec-butyl [R configuration], and sec-butyl [S configuration], respectively), were studied for their in vitro antimycobacterial activities. Representative (KRM-1648) MICs for 90% of the strains tested, determined by the agar dilution method on 7H11 medium, of various pathogenic mycobacteria (9 species, 174 strains) were as follows (in micrograms per milliliter): Mycobacterium tuberculosis (rifampin [RMP]-susceptible strains), less than or equal to 0.0125; M. tuberculosis (RMP-resistant strains), 12.5; M. kansasii, 0.05; M. marinum, less than or equal to 0.0125; M. scrofulaceum, 0.1; M. avium, 1.56; M. intracellulare, 0.1; M. fortuitum, greater than 100; and M. chelonae subsp. abscessus and M. chelonae subsp. chelonae, greater than 100. These values are more than 64 times lower than those of RMP, except for the values against RMP-resistant M. tuberculosis (8 times lower) and those against rapid growers, including M. fortuitum and M. chelonae (the same as those of RMP). The other derivatives had similar levels of in vitro activity against these mycobacteria. When murine peritoneal macrophages in which M. intracellulare was phagocytosed in vitro were cultured in the presence of the benzoxazinorifamycins (1 microgram/ml), much more rapid killing of the organisms ingested in the macrophages was seen compared with when the same amount of RMP was added to the medium. The addition of benzoxazinorifamycins at the concentration of 0.05 micrograms/ml caused more marked suppression of intracellular growth of the organisms compared with addition of RMP. KRM-1648 and KRM-1657 inhibited intracellular growth of M. tuberculosis, and their efficacies were much greater than that of RMP.

Recurrent mixed tumor of the right lacrimal gland causes acute contralateral visual loss.

A 23-year-old woman underwent excisional biopsy of a right palpebral mass: the histopathologic study showed a benign mixed tumor of the lacrimal gland. Thereafter, the tumor recurred repeatedly, and multiple excisions were done. Subsequently, right orbital exenteration was performed, and some orbital and frontal bones were removed. When the patient was 52 years old, the orbital tumor recurred. A histopathologic study revealed malignant transformation. At the age of 54 years, the patient suffered acute loss of vision in her left eye. Computed tomography disclosed invasion of the tumor into the posterior paranasal sinuses and brain.

Molecular cloning, nucleotide sequencing, and expression of the Bacillus subtilis (natto) IAM1212 alpha-amylase gene, which encodes an alpha-amylase structurally similar to but enzymatically distinct from that of B. subtilis 2633.

An alpha-amylase gene of Bacillus subtilis (natto) IAM1212 was cloned in a lambda EMBL3 bacteriophage vector, and the nucleotide sequence was determined. An open reading frame encoding the alpha-amylase (AMY1212) consists of 1,431 base pairs and contains 477 amino acid residues, which is the same in size as the alpha-amylase (AMY2633) of B. subtilis 2633, an alpha-amylase-hyperproducing strain, and smaller than that of B. subtilis 168, Marburg strain. The amino acid sequence of AMY1212 is different from that of AMY2633 at five residues. Enzymatic properties of these two alpha-amylases were examined by introducing the cloned genes into an alpha-amylase-deficient strain, B. subtilis M15. It was revealed that products of soluble starch hydrolyzed by AMY1212 are maltose and maltotriose, while those of AMY2633 are glucose and maltose. From the detailed analyses with oligosaccharides as substrates, it was concluded that the difference in hydrolysis products of the two similar alpha-amylases should be ascribed to the different activity hydrolyzing low-molecular-weight substrates, especially maltotriose; AMY1212 slowly hydrolyzes maltotetraose and cannot hydrolyze maltotriose, while AMY2633 efficiently hydrolyzes maltotetraose and maltotriose. Further analyses with chimeric alpha-amylase molecules constructed from the cloned genes revealed that only one amino acid substitution is responsible for the differences in hydrolysis products.

Isolation, purification, and structure of components from acidic polysaccharides of Pleurotus ostreatus (Fr.) Quél.

Isolation of an antitumor component from polysaccharide fraction A5 of some Basidiomyces was achieved by column chromatography on Sephadex G-200. A detection method based on the specific rotatory characteristics of the polysaccharide was applied to estimate components in effluent fractions from the chromatography, and it was confirmed that a series of eluates having similar specific rotation was made up of homogeneous polysaccharide. Three components (H51, H52, and H53) were isolated, in chromatographically pure state, from fraction A5. Component H51 consisted of a skeleton of beta-(1 leads to 3)-linked glucose residues, probably having branches of galactose and mannose residues, and also containing acidic sugars. Component H53 had a main structure similarly consisting of beta-(1 leads to 3)-linked glucose residues and a larger proportion of acidic sugar than H51. Component H52 was a heteropolysaccharide made up of alpha-linked galactose and mannose residues. Components H51 and H53 had a higher and a lower molecular weight, respectively, than H52. The only antitumor-active component was H51.