Reconstruction of the distal aspect of the radius with use of an osteoarticular allograft after excision of a skeletal tumor

Twenty-four patients had reconstruction of the distal aspect of the radius with use of an osteoarticular allograft, between 1974 and 1992, after excision of a giant-cell tumor (twenty patients), a desmoplastic fibroma (two patients), a chondrosarcoma (one patient), or an angiosarcoma (one patient). Nine giant-cell tumors were recurrent lesions, and eleven were extracompartmental primary lesions that had extended through the cortex or subchondral bone. The average age of the patients was 31.5 years (range, fifteen to sixty-one years); thirteen patients were female and eleven were male. Seventeen lesions involved the right wrist and seven involved the left wrist. The reconstruction was performed through a dorsoradial incision with use of a size-matched, preserved, fresh-frozen, distal radial allograft. All procedures included internal fixation and reconstruction of the radiocarpal ligaments. All patients were followed for a minimum of two years (average, 10.9 years; range, 2.1 to 22.3 years). At the time of follow-up, two patients -- one who had a giant-cell tumor and one who had a desmoplastic fibroma -- had a local recurrence. Eight patients needed a revision of the osteoarticular allograft, at an average of 8.1 years (range, 0.8 to 17.8 years) after the initial reconstruction. Seven of these patients had an arthrodesis and one had an amputation. The reason for the revision was a fracture of the allograft in four patients, recurrence of the tumor in one, pain in two, and volar dislocation of the carpus in one. There were fourteen other complications, including ulnocarpal impaction necessitating excision of the distal aspect of the ulna (four), painful hardware necessitating removal (four), rupture of the extensor pollicis longus tendon necessitating transfer of the extensor indicis proprius (two), fracture of the allograft necessitating open reduction and internal fixation (two), volar dislocation of the carpus necessitating closed reduction (one), and a ganglion of the dorsal aspect of the wrist necessitating excision (one). Of the sixteen patients in whom the osteoarticular allograft survived, three did not have pain, nine had pain in association with strenuous activities, and four had pain in association with moderate activities. Three patients reported no functional limitation, nine had limitation in the ability to perform strenuous activities, and four had limitation in the ability to perform moderate activities. The average range of motion of the wrist was 36 degrees of dorsiflexion, 21 degrees of volar flexion, 16 degrees of radial deviation, 15 degrees of ulnar deviation, 58 degrees of supination, and 72 degrees of pronation. Reconstruction of the distal aspect of the radius with use of an osteoarticular allograft was associated with a low rate of recurrence of the tumor, a moderately high rate of revision, little pain in association with common activities, good function, and a moderate range of motion. Osteoarticular allografts are an option for reconstruction of the distal aspect of the radius after excision of a malignant tumor or a recurrent or locally invasive benign lesion.     

HLA class I-specific inhibitory receptors in human T lymphocytes: interleukin 15-induced expression of CD94/NKG2A in superantigen- or alloantigen-activated CD8+ T cells

A fraction of human T lymphocytes, predominantly CD8+, express receptors for HLA class I molecules typical of natural killer cells (natural killer receptors or NKRs) that inhibit T cell receptor-mediated functions. Herein, we analyzed possible mechanism(s) leading to the expression of NKRs by T cells responding to superantigens or allogeneic cells in vitro. We show that, in the presence of interleukin 15 (IL-15), T cells (depleted of NKR+ cells) responding to toxic shock syndrome toxin 1 de novo express CD94, a molecule that is part of a heterodimeric NKR with a broad specificity for different HLA class I alleles. Maximal CD94 expression occurred when IL-15 was added shortly after the cells were placed into culture, and CD94 expression started 4-6 days after addition of IL-15. Although both CD4+ and CD8+ cells expressed CD94, the simultaneous expression of NKG2A (i.e., the other component of the CD94/NKG2A inhibitory NKR) was confined to CD8+ cells. Similar data were obtained in T cell populations activated in mixed lymphocyte cultures in the presence of IL-15. The expression of CD94/NKG2A led to an impairment of allo-specific cytolytic activity by mixed lymphocyte culture-derived T cell populations or clones. Remarkably, cytolysis could be restored by the addition of anti-CD94 mAb, i.e., by masking the inhibitory NKRs.     

Generation of splenic follicular structure and B cell movement in tumor necrosis factor-deficient mice

Secondary lymphoid tissue organogenesis requires tumor necrosis factor (TNF) and lymphotoxin alpha (LTalpha). The role of TNF in B cell positioning and formation of follicular structure was studied by comparing the location of newly produced naive recirculating and antigen-stimulated B cells in TNF-/- and TNF/LTalpha-/- mice. By creating radiation bone marrow chimeras from wild-type and TNF-/- mice, formation of normal splenic B cell follicles was shown to depend on TNF production by radiation-sensitive cells of hemopoietic origin. Reciprocal adoptive transfers of mature B cells between wild-type and knockout mice indicated that normal follicular tropism of recirculating naive B cells occurs independently of TNF derived from the recipient spleen. Moreover, soluble TNF receptor-IgG fusion protein administered in vivo failed to prevent B cell localization to the follicle or the germinal center reaction. Normal T zone tropism was observed when antigen-stimulated B cells were transferred into TNF-/- recipients, but not into TNF/LTalpha-/- recipients. This result appeared to account for the defect in isotype switching observed in intact TNF/LTalpha-/- mice because TNF/LTalpha-/- B cells, when stimulated in vitro, switched isotypes normally. Thus, TNF is necessary for creating the permissive environment for B cell movement and function, but is not itself responsible for these processes.     

Faster adjustment of O2 delivery does not affect V(O2) on-kinetics in isolated in situ canine muscle

The mechanism(s) limiting muscle O2 uptake (VO2) kinetics was investigated in isolated canine gastrocnemius muscles (n = 7) during transitions from rest to 3 min of electrically stimulated isometric tetanic contractions (200-ms trains, 50 Hz; 1 contraction/2 s; 60-70% of peak V(O2)). Two conditions were mainly compared: 1) spontaneous adjustment of blood flow (Q) [control, spontaneous Q (C Spont)]; and 2) pump-perfused Q, adjusted approximately 15 s before contractions at a constant level corresponding to the steady-state value during contractions in C Spont [faster adjustment of O2 delivery (Fast O2 Delivery)]. During Fast O2 Delivery, 1-2 ml/min of 10(-2) M adenosine were infused intra-arterially to prevent inordinate pressure increases with the elevated Q. The purpose of the study was to determine whether a faster adjustment of O2 delivery would affect V(O2) kinetics. Q was measured continuously; arterial (Ca(O2)) and popliteal venous (Cv(O2)) O2 contents were determined at rest and at 5- to 7-s intervals during contractions; O2 delivery was calculated as Q x Ca(O2), and V(O2) was calculated as Q x arteriovenous O2 content difference. Times to reach 63% of the difference between baseline and steady-state VO2 during contractions were 23.8 +/- 2.0 (SE) s in C Spont and 21.8 +/- 0.9 s in Fast O2 Delivery (not significant). In the present experimental model, elimination of any delay in O2 delivery during the rest-to-contraction transition did not affect muscle V(O2) kinetics, which suggests that this kinetics was mainly set by an intrinsic inertia of oxidative metabolism.     

Structural, functional, and genetic characterization of Gastrophilus hemoglobin

Hemoglobin of Gastrophilus intestinalis (Insecta, Diptera), was purified and characterized. At least two isoforms have been identified by isoelectrofocusing, mass spectrometry, and genomic Southern blotting. Functional studies show a high oxygen affinity due to a low ligand dissociation rate (koff = 2.4 s-1) and a relatively high autoxidation rate (t1/2 = 1.6/h). The globins were separated under denaturing conditions, and the sequence of Hb1 (Mr = 17,965 +/- 2) was determined at the protein and DNA level. The open reading frame codes for a polypeptide of 150 amino acids. Although the globin is distantly related to globins from other species, it has a low penalty score against globin templates. Freshly isolated hemoglobin was crystallized from polyethylene glycol. Crystals contain two hemoglobin molecules per asymmetric unit. Solution of the three-dimensional structure by molecular replacement could not be achieved, possibly due to the presence of three protein isoforms in the crystals. In order to determine its three-dimensional structure, G. intestinalis Hb1 was overexpressed in Escherichia coli, resulting in a fully functional molecule as confirmed by ligand binding affinity. The globin gene contains two introns at positions D7.0 and G7.0. The D7.0 intron is unprecedented, suggesting that globin gene evolution is much more complex than originally thought.     

Syphilitic labial leucokeratosis

BACKGROUND: Several diagnoses, including syphilis, can be entertained in patients with leukokeratosis of the buccal mucosa. We report a case of labial leucokeratosis which revealed latent syphilis. CASE REPORT: A 36-year-old man with a past history of genital syphilis chancre which have been treated 12 years earlier, developed buccal leucokeratosis with no other clinical manifestation. Histology showed dermal infiltration containing plasma cells, polynuclears and lymphocytes. Blood tests were positive for syphilis. Complementary examinations were unable to detect another localization. Leucokeratosis regressed completely after one injection of Extencilline. There has been no recurrence at one year. DISCUSSION: The clinical and histological presentations of syphilis can mimic different skin diseases. Serodiagnosis alone is significant. Isolated buccal lesions are rarely described in syphilis suggesting serodiagnosis should always be ordered. Whatever the clinical stage of the diseases, serological surveillance after treatment for syphilis is essential.     

Systematic screening for fragile X syndrome in a cohort of 574 mentally retarded children

The Sense of Coherence Scale is used frequently world-wide and is thought to be appropriate to any population. The purpose of this study was to highlight some of the potential limitations of the scale. Fifteen members of the Pentecostal Movement described several difficulties in completing the scale, especially items measuring manageability and comprehensibility. The following three statements indicate the nature of their interpretation of the items and the ambiguity of their answers as they argued that: (1) the concept of 'I' was interpreted as 'I and God', (2) it is not necessary to understand everything in life, because it is enough that God understands, and (3) life is meaningful in itself because of the salvation. According to this interpretation of the items, every proposed answer could be suitable and consequently, the scores can readily be misconstrued. Many of the respondents proposed additions or changes in the wording to make the scale more suitable. The strong ego-centred items seem to be inappropriate for the participants in this study and other populations might also be confronted with similar difficulties.     

Thrombotic thrombocytopenic purpura in 13 Dutch centres: treatment and longterm follow-up

OBJECTIVE: Analysis of the incidence, treatment modalities and disease course of thrombotic thrombocytopenic purpura (TTP) in the Netherlands. DESIGN: Retrospective follow-up study. SETTING: 13 centres in the Netherlands. METHODS: Regarding all patients admitted between 1-1-1979 and 1-1-1992 to one of 13 Dutch haematological centres, in whom the diagnosis of TTP was made for the first time, information was gathered from the medical records and from the patients own physicians on patient characteristics at presentation and the occurrence of relapse or death. The follow-up period tended on 1-4-1995. RESULTS: A total of 65 patients with newly diagnosed TTP were identified: 0.34 per 1,000,000 persons a year (95% confidence interval (95%-CI): 0.26-0.45), increasing to 0.83 in the last year of the study. Forty-six (95%) patients were treated with fresh frozen plasma: 18 (28%) by plasma infusion and 44 (68%) by plasma exchange; 48 (74%) (additionally) received corticosteroids. All 52 patients (80%) who survived the first four weeks after admission reached complete remission. Twelve patients with relapsing TTP underwent splenectomy in remission. The 5-year survival rate was 77% (95% CI: 66-87) and the 5-year relapse-free survival rate 38% (95% CI: 25-52). Cardiac symptoms, severe thrombocytopenia and a high serum LDH were risk factors for acute mortality, but no risk factors for relapse or late-occurring death could be identified. CONCLUSION: TTP is a rare disease which is increasingly being recognized. Plasma exchange and corticosteroids are the most frequently used therapies. The disease has a high mortality rate in the acute phase of the disease.