Mechanical testing of the tension band wire fixation in the proximal femur

The mechanical stability of proximal femoral osteotomies fixed by the tension band wire technique was studied in flexion-compression and torsion tests. The fixation consisted in crossing the section with two Kirschner wires and with a wire cerclage applied to the tension surface. The study was conducted in three steps. First, cyclinders of wood were cut either transversely or at 30° of inclination in relation to the long axis of the specimen, and fixed with two Kirschner wires and a wire cerclage. We concluded that the inclination of the plane of section significantly increased the stability of fixation. No significant difference was observed when oblique sections were made in the reverse orientation. Second, 30° subtrochanteric varus osteotomies were performed in dog femurs, so that the section plane was transverse in one group and oblique in another, after closing the osteotomy. In both groups the fixation was achieved by two Kirschner wires that crossed the osteotomy and a wire cerclage placed on the lateral cortex (tension surface). We concluded that inclination of the osteotomy plane increased the stability of osteosynthesis in bone specimens, as already seen with the wood pieces. Third, the stability of tension band wire fixation was compared with that provided by the AO/ASIF paediatric angled plate. Varus osteotomies (30°) were created at the subtrochanteric level of paired dog femurs. On one side, the femur was fixed with Kirschner wires and a wire cerclage as described previously. For the other femur, the osteotomy was fixed with the angled plate. We found that both types of fixation presented the same stability in flexion-compression tests. However, under torsion the tension band wire fixation was 30%–50% less stable than the plate fixation.     

Lambert-Eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigenic modulation

The Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease that can be transmitted from human to mouse with immunoglobulin G (IgG). Electrophysiological studies indicate that LEMS IgG acts on presynaptic voltage-sensitive calcium channels, probably reducing their number, and freeze-fracture electron microscopy demonstrates that LEMS IgG has an effect on the presynaptic active zone particles, which represent putative voltage-sensitive calcium channels. The active zone particles, normally arranged in double parallel rows, move closer together, form clusters, and are reduced in number. The morphological data suggest modulation of the active zone particles crosslinked by LEMS IgG. If this were the case, then only divalent LEMS IgG and F(ab')2 should alter the deployment of active zone particles and monovalent Fab should be without effect. To test this hypothesis, mouse diaphragms were exposed to control and LEMS IgG and IgG fragments in organ culture for 24 hours and then studied by quantitative freeze-fracture electron microscopy. Divalent LEMS IgG and F(ab')2 aggregated and depleted the active zone particles, whereas monovalent Fab had no effect. The findings reconfirm that the active zone particles are targets of LEMS IgG and are direct evidence for modulation of the particles by LEMS IgG. The findings are in harmony with parallel electrophysiological studies of the effects of LEMS IgG fragments on transmitter release in the same diaphragm muscles (Lang et al, J Physiol 1987;390:173P).     

Fatigue in neuroimmunological diseases

Journal of Neurology - Fatigue is a widespread symptom in numerous neuroimmunological diseases like multiple sclerosis (MS), myasthenia gravis, morbus Behcet, neurosarcoidosis, neuroborreliosis or...     

A new Le Fort I internal distraction device in the treatment of severe maxillary hypoplasia.

PURPOSE: The purpose of this pilot study was to test a new Le Fort I internal distraction device. PATIENTS AND METHODS: A new internal Le Fort I distraction device designed by 1 of the authors was used in 3 patients with cleft lip and palate and severe maxillary hypoplasia who needed maxillary advancements in excess of 12 mm. Presurgical planning used CASSOS (SoftEnable Technology, Ltd, Hong Kong SAR, China) prediction tracing software and a stereolithographic model to calculate the distraction vector. The distractors were pre-bent and installed on the stereolithographic model and activated to advance the maxilla. Surgery was performed in a conventional manner, and distraction was started after a 7-day latency phase at the rate of 1 mm/day and continued until the presurgical plan was achieved. The distractor was removed after a 3-month consolidation phase. Cephalometric radiographs were taken at the completion of each phase. RESULTS: This new Le Fort I internal distraction device successfully distracted the maxillae as planned in all 3 patients. At the end of the distraction phase, the maxillary advancement was measured at 15.8 mm, 15.8 mm, and 13.5 mm, respectively. In each patient, a clockwise rotation of the maxilla was observed with a tendency to a posterior open bite. Postoperative radiographs also showed that the actual distraction vectors differed from the planned vectors. After the consolidation phase, radiographs showed a relapse of 2.6 mm, 0 mm, and 5.0 mm, respectively. There was no further relapse on 3-month follow-up radiographs. Each case showed radiographic evidence of excellent new bone formation at the osteotomy sites. CONCLUSION: The new Le Fort I internal distraction device produced the necessary advancement in all 3 patients. The study also showed that the actual distraction vector differed from the planned vector. This discrepancy was caused by a clockwise rotation of the maxilla during the distraction. Finally, the study showed a variable relapse rate not previously reported in maxillary distraction.     

Frequent ongoing T-cell receptor rearrangements in childhood B- precursor acute lymphoblastic leukemia: implications for monitoring minimal residual disease

Crosslineage T-cell receptor delta (TCR delta) rearrangements are widely used as tumor markers for the follow up of minimal residual disease in childhood B-precursor acute lymphoblastic leukemia (ALL) by polymerase chain reaction (PCR). The major drawback of this approach is the risk of false-negative results due to clonal evolution. We investigated the stability of V delta 2D delta 3 rearrangements in a group of 56 childhood B-precursor ALL patients by PCR and Southern blot analysis. At the PCR level, V delta 2D delta 3-to-J alpha rearranged subclones (one pathway for secondary TCR delta recombination) were demonstrated in 85.2% of V delta 2D delta 3-positive patients tested, which showed that small subclones are present in the large majority of patients despite apparently monoclonal TCR delta Southern blot patterns. Sequence analysis of V delta 2D delta 3J alpha rearrangements showed a biased J alpha gene usage, with HAPO5 and J alpha F in 26 of 32 and 6 of 32 clones, respectively. Comparison of V delta 2D delta 3 rearrangement status between diagnosis and first relapse showed differences in seven of eight patients studied. In contrast, from first relapse onward, no clonal changes were observed in six patients studied. To investigate the occurrence of crosslineage TCR delta rearrangements in normal B and T cells, fluorescence-activated cell sorter-sorted peripheral blood CD19+/CD3- and CD19-/CD3+ cell populations from three healthy donors were analyzed. V delta 2D delta 3 rearrangements were detected at low frequencies in both B and T cells, which suggests that V delta 2-to-D delta 3 joining also occurs during normal B-cell differentiation. A model for crosslineage TCR delta rearrangements in B-precursor ALL is deduced that explains the observed clonal changes between diagnosis and relapse and is compatible with multistep leukemogenesis of B-precursor ALL.     

Picotamide inhibition of excess in vitro thromboxane B2 release by colorectal mucosa in inflammatory bowel disease.

BACKGROUND: Inflammatory bowel disease is associated with increased mucosal release of eicosanoids. Among these, thromboxane A2 has been proposed as a possible inflammatory mediator; its suppression may be a useful therapeutic option. METHODS: Using a tissue incubation technique, we compared release of immunoreactive thromboxane B2 by colonic biopsies from patients with ulcerative colitis, Crohn's disease and controls, and assessed the inhibitory effect of picotamide, a thromboxane synthesis inhibitor-receptor antagonist, which has been widely used in Italy for management of ischaemic heart and cerebrovascular disease. RESULTS: Increased amounts of thromboxane B2 were released from biopsies from patients with active ulcerative colitis (median 238 pg/20 min/mg wet weight (interquartile range 147- 325), n = 12) and active Crohn's disease (252 (174-450), 6) compared with those from patients with quiescent ulcerative colitis (95 (61- 140), 12) or Crohn's disease (105 (57-201), 13), or controls (136 (64- 206), 8). Incubation with picotamide at concentrations between 100 microM and 1 mM reduced thromboxane B2 release (IC50 890 microM). CONCLUSION: Since increased thromboxane A2 production may have pathogenetic importance, thromboxane synthesis inhibitor-receptor antagonists such as picotamide merit therapeutic trial in the management of inflammatory bowel disease.     

Consequence of long-term exposure to corticosterone or dexamethasone on ethanol consumption in the adrenalectomized rat,and the effect of type I and type II corticosteroid receptor antagonists

The daily fluid intake of male Wistar rats with simultaneous access to 6% ethanol and water was determined during a baseline period (1 week), following adrenalectomy (1 week) and for 3 weeks following SC implantation of hormone pellets containing corticosterone (CORT) or dexamethasone (DEX). Ethanol consumption dropped during the first week of adrenalectomy (ADX) but increased again in the absence of hormone replacement to reach preoperative levels during the ensuing weeks. The CORT treatment, which produced plasma hormone levels similar to the 24-h mean concentration of adrenally intact rats, not only reversed the effect of ADX on alcohol consumption but also enhanced it to levels above those observed in intact rats. Water intake was not affected by the CORT treatment. DEX implants stimulated water intake, but did not enhance the drinking of ethanol. SC injections of RU 28318 (type I corticosterone receptor antagonist; 10 mg/kg) or mifepristone (RU 38486; type II receptor antagonist; 25 mg/kg) at the beginning and halfway through three daily, 6-h tests failed to affect ethanol drinking in adrenally intact rats or in ADX rats bearing CORT implants. Similarly, there was no effect of giving the two antagonists in combination. These results suggest that exogenous CORT can induce excessive alcohol intake in genetically unselected rats and that this facilitatory effect may be mediated by non-genomic cellular mechanisms.     

Empty sella syndrome in childhood

The empty sella syndrome is common in middle-aged women, usually presenting with headache, and only occasionally associated with endocrine or visual abnormalities. It is rare in childhood. Childhood cases tend to present either with endocrine disturbances, visual symptoms, or with craniofacial syndromes. We present three cases of complete empty sella with childhood onset, each discovered unexpectedly during evaluation of endocrine or visual dysfunction.