Interleukin 4 counteracts the interleukin 2-induced proliferation of monoclonal B cells.

B cells from patients suffering from B-type chronic lymphocytic leukemia (B-CLL) are susceptible to the effects of several interleukins. Using the cells from 12 different patients we show that IL-4 does not synergize with anti-mu antibody for the enhancement of DNA synthesis. Moreover IL-4 profoundly (90%) suppresses the response to IL-2 in the 10 patient responders to this interleukin. This suppression occurs whether IL-2 is used alone, in costimulation with anti-mu antibody, or in synergy with IFN-gamma. In no instance did IL-4 induce terminal differentiation. This negative effect of IL-4 can take place in monoclonal B-CLL cells where IL-4 enhances the expression of CD23. IL-4 does not interfere with the upregulation of CD25 by IL-2. Thus, IL-4 may display inhibitory effects on the proliferative response of selected B cell populations. The antagonism between IL-4 and IL-2 has important implications for the potential use of cytokines in the management of B-CLL patients.

[Endovascular repair of iliocaval arteriovenous fistula complicating lumbar disc surgery]. / Traitement endovasculaire d'une fistule artério-veineuse ilio-cave compliquant une chirurgie discale lombaire.

Vascular complications of lumbar disc surgery are rare. Few cases have been reported. Arteriovenous fistulas are the most common. They are due to anatomical relationships between the last lumbar vertebrae, the corresponding discs, and the iliac vessels; degenerative lesions of the intervertebral discs facilitate instrumental vessel perforation, and operative difficulty. Computed tomography is particularly accurate for making the diagnosis. Treatment strategies consist in surgery or endovascular management. Percutaneous endovascular treatment using a stent-graft is a reasonable option for treating arteriovenous fistula. We describe the case of a 50-year-old patient who developed an iliocaval arteriovenous fistula following lumbar disc hernia surgery. The lesion was excluded by a stent-graft. The postoperative period was uneventful.

[Epithelioid hemangioendothelioma of bone complicated by femoral fracture]. / Hémangio-endothéliome épithélioïde osseux compliqué d'une fracture du fémur.

A 54-year-old man was seen with a fracture of the left femur. Plain radiographs revealed a 40-mm lytic centromedullary lesion. Magnetic resonance T1- and T2-weighted sequences showed high and low signals. After stabilization of the fracture, the tumor was removed followed by reconstruction with a vascularized fibula. The pathological examination demonstrated proliferation of non atypical CD34 and CD31 positive epithelioid cells with few lumens, accompanied by abundant fibrous stroma, sometimes masking tumor cells. Satisfactory motion was achieved with no recurrence at 20 months follow-up. Bone hemangioendothelioma can simulate metastasis and must be distinguished by immunohistochemistry. Prognosis is a subject of debate as the tumor is considered to exhibit intermediate malignancy by some authors while other consider it to be a malignant tumor.

[Lanthanides and microanalysis. Effects of oral administration of two lanthanides: ultrastructural and microanalytical study]. / Lanthanides et microanalyse. Effets de l'administration orale de deux lanthanides :etude ultrastructurale et microanalytique.

The subcellular localization of cerium and lanthanum in the intestinal mucosa was studied after oral administration of cerium chloride or lanthanum chloride or lanthanum chloride followed 30 minutes after of cerium chloride to young adults Wistar rats. Two methods of observation and microanalysis were used. The transmission electron microscopy revealed the presence of dense electron granulations in the lysosmes of the duodenum enterocyte, when these elements were administrated simultaneously. The ion mass microanalysis permits to detect the presence of La and Ce as bright points outlining the intestinal villi. These points correspond to the lysosomes containing the granulations previously described. These granulations are formed by the cerium and the lanthanum associated to the phosphor and forming probably insoluble salts of Ce/La phosphate.

Osteoblast-induced osteoclast apoptosis by fas ligand/FAS pathway is required for maintenance of bone mass.

The interplay between osteoblasts and osteoclasts has a crucial role in maintaining bone homeostasis. In this study, we reveal that osteoblasts are capable of inducing osteoclast apoptosis by FAS ligand (FASL)/FAS signaling. Conditional knockout of FASL in osteoblasts results in elevated osteoclast numbers and activity, along with reduced bone mass, suggesting that osteoblast-produced FASL is required to maintain physiological bone mass. More interestingly, we show that osteoblasts from ovariectomized (OVX) osteoporotic mice exhibit decreased FASL expression that results from the IFN-γ- and TNF-α-activated NF-κB pathway, leading to reduced osteoclast apoptosis and increased bone resorption. Systemic administration of either IFN-γ or TNF-α ameliorates the osteoporotic phenotype in OVX mice and rescues FASL expression in osteoblasts. In addition, ovariectomy induces more significant bone loss in FASL conditional knockout mice than in control group with increased osteoclast activity in which the levels of RANKL and OPG remain unchanged. Taken together, this study suggests that osteoblast-induced osteoclast apoptosis via FASL/FAS signaling is a previously unrecognized mechanism that has an important role in the maintenance of bone mass in both physiological conditions and OVX osteoporosis.

B8.7 antigen expression on B-CLL cells and its relationship to the LMW-BCGF responsiveness.

In this work, we studied the expression of B8.7 antigen on B lymphocytes from patients suffering from B type chronic lymphocytic leukemia (B-CLL) as well as on non Hodgkin lymphoma cells (NHL). B8.7 is an activation marker, which has been reported to be associated with the capacity of activated B cells to respond to LMW-BCGF. B lymphocytes of 11 out of 22 patients tested were B8.7 positive. With the exception of one case, LMW-BCGF is able to induce DNA synthesis by these cells in the absence of costimulation by anti-mu antibodies (anti-mu Ab). The LMW-BCGF dependent proliferation of these malignant cells is inhibited by the anti-B8.7 monoclonal antibody (anti-B8.7 MoAb), in the same line as that of normal B cells. These results obtained with monoclonal B cells confirm that the B8.7 molecule is involved in the signalling pathway of the LMW-BCGF.

Regulatory effects of IL-4 on human B-cell response to IL-2.

Interleukin-4 (IL-4) counteracts a number of the direct effects of interleukin-2 (IL-2) on B-cells. We here summarize and extend our results, obtained in two different experimental systems, on the antagonism between these two major interleukins. IL-4 inhibits the effect of IL-2 on the proliferation as well as the differentiation of B-type chronic lymphocytic leukemia (B-CLL) cells. When B-CLL cells are activated by anti-mu Ab in the presence of IL-4, this latter enhances the expression of the p55 as well as the p70/75 chain of the IL-2 receptor. In contrast IL-4 profoundly suppresses the number of high affinity binding sites for IL-2 on in vitro activated B-CLL cells. Such a discrepancy between the suppression of IL-2 binding sites and the enhancement of each component of the heterodimeric IL-2 receptor, is as far as we know, yet undescribed. The interaction of IL-4 with its own receptors might influence the state of p55-p70/75 complex association or act on a third subunit of the IL-2 receptor. When used alone, IL-4 enhances the expression of other activation molecules by B-CLL cells: CD23, DR antigen. Similarly IL-4 can concomitantly enhance the specific response of normal B-cells while suppressing the action of IL-2. When normal human B-cells are specifically stimulated by an insolubilized antigen, IL-4 alone induces an expansion of the number of specific antigen-binding cells. In contrast IL-4 profoundly suppresses the generation of antigen-induced IL-2-dependent specific IgM antibody forming cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Vertebral hydatidosis and paraplegia.

We report the management of two children and 11 adults with paraplegia secondary to vertebral hydatidosis. Destruction of pedicles, posterior vertebral elements and discs as well as the vertebral bodies was common and all six patients with thoracic disease had involvement of adjacent ribs. The 13 patients had a total of 42 major surgical procedures; two patients died from postoperative complications and four from complications of the disease and paraplegia. All eight patients initially treated by laminectomy or anterior decompression alone relapsed within two years and seven required further surgery. Circumferential decompression and grafting gave the best results, six of nine patients being in remission an average of three years and six months later. The prognosis for such patients is poor; remission is the aim, rather than cure. Anthelminthic drugs may improve the prognosis, but radical surgery is likely to remain the keystone of treatment in the foreseeable future.

[Primary chemotherapy with the Rosen T10 protocol before conservative surgery in limb primitive osteosarcomas: results about 56 cases]. / Chimiothérapie première par le protocole T10 de Rosen avant chirurgie conservatrice dans les ostéosarcomes primitifs des mebres: résultats à propos de 56 cas.

We report the results of a prospective Tunisian study using primary chemotherapy followed by conservative surgery in primitive limb osteosarcoma. From January 1988 to January 1998, 56 patients affected by limb osteosarcoma entered in a prospective study of neoadjuvant chemotherapy with the T10 protocol before surgery with a conservative intent. Initial work-up include: clinical exam with tumor measurements, chest and limb X-rays, limb CT-scan or MRI, chest CT-scan, bone scintigraphy and hematological and renal biological exams. Patients receive pre- and post-operative chemotherapy according to the T10 modified protocol. Fifty-six patients (33 M/23 F) with a mean age of 19 years (8 to 28) are included. Mean clinical and radiological tumor size is around 14 cm. Main histologic type is classic osteosarcoma (50% of cases) and 10 patients (9%) presented with initial metastasis; 42 patients on 56 receive the whole pre-operative protocol. Treatment is well tolerated excluding 18 episodes of mucositis, 29 of leucopenia (< grade 3), 7 of thrombopenia (< grade 3), 4 of cutaneous toxicity, 2 of pulmonary toxicity and 3 of nausea-vomiting. We observe 36% of good histological responders and 64% of bad responders to primary chemotherapy, 27 patients on 49 operated (53%) have a conservative surgery and 18 (47%) a radical surgery. With a median follow-up of 51 months (8 to 128), 29 patients remain alive free of disease (15/17 GR and 14/30 BR), 2 are alive with disease, 2 died by toxicity, 14 died by progressive disease and 9 are lost to follow-up with evolutive disease. Five year disease-free survival is 55% for the 46 non metastatic patients. In univariate analysis, seric alkaline phosphatase level (p = 0.0014) and histological response to chemotherapy (p = 0.0218) are significant factors for prognosis.

[Polyarthritic manifestations revealing Stickler syndrome]. / Manifestations poly-articulaires révélatrices d'un syndrome de Stickler.

Stickler syndrome, also called hereditary arthro-ophthalmopathy is a dominant disorder, with poly-epiphyseal dysplasia resulting in early osteoarthritis. It usually includes bilateral myopia, that changes little during the follow up period, but can lead to retinal detachment. Children with Stickler syndrome can show facial dysmorphism that may improve with age, but severe anomalies may exist such as Pierre Robin sequence. Our case, a woman, 20-year-old, presents chronic polyarthritis which is an unusual feature of this disease. The diagnosis of Stickler's syndrome is based on polyepiphyseal dysplasia in skeletal radiography and bilateral myopia. Otherwise, this patient has no facial dysmorphism.

[Fracture-luxation of the femur head. Apropos of 10 cases]. / Fracture-luxation de la tête fémorale. A propos de 10 cas.

The authors report 10 segmental fractures of the femoral head associated with a dislocation of the hip. Using PIPKIN's classification, they noted 1 type I, 7 type II and 2 type III fractures. The initial treatment of the hip dislocation was conservative in all cases. Failure of reduction was noted in 6 cases and was complicated by fracture of the femoral neck in 2 cases. The attitude regarding the head fragment was: conservative in 5 cases, the reduction was considered satisfactory in 4 cases and poor in 1 case; surgical in the other 5 cases: screw fixation in 1 case, excision of the fragment in 1 case and prosthetic replacement of the femoral head in 3 cases. After a mean follow-up of 5 years, functional results were satisfactory in the majority of cases. The radiological assessment showed malunion without osteoarthrosis in one case and avascular necrosis of the femoral head in three cases. The authors propose a treatment policy based upon their experience and a review of the literature.

[Consequences of tibiotalar arthrodesis on the foot. A retrospective study of 36 cases with 8.5 years of followup]. / Etude du retentissement de l'arthrodèse tibio-talienne sur le pied. A propos de 36 cas revus avec un recul moyen de 8 ans et demi.

The authors report the results of a retrospective study of 36 cases of tibiotalar arthrodesis performed in 22 men and 14 women with an average age of 32 years. All patients were reviewed with an average of 8.5 years follow-up. The predominating etiologies were ankle osteoarthritis (15 cases) and neurologic deformities of the foot (13 cases). Arthrodesis was performed using the Meary technique in 60% of cases, using the Charnley technique in 20% and the Crawford-Adams technique or with clamps in the other cases. Fusion was obtained in 97% of cases. Long-term results were assessed using Duquennoy et al.'s scoring system. They were very good or good in 58% of cases, fair in 31% and poor in 11%. The study of distal repercussions of tibiotalar arthrodesis shows progressive deterioration of the subtalar joint in 70% of cases and appearance or increase of degenerative changes in 75% of cases. The final results of the procedure depend on this deterioration; the latter is related with the arthrodesis position. Midtalar joint is a compensation joint showing hypermobility in 40% of cases. Degenerative changes were limited and asymptomatic in 80% of cases. Based on the findings in this study and on the literature, we conclude that the foot should be fixed at 90 degrees or with less than 5 degrees of equinus, with 5 degrees of valgus and 10 to 15 degrees of external rotation.

Extensive vertebral hydatidosis. A study.

An exceptional case of vertebral hydatidosis with involvement of the cervical, dorsal, lumbar and sacral segments of the spine is presented. Vertebral hydatidosis is a rare form of hydatid disease. It is found in less than 2% of all cases in echinococcosis. The initial parasite localization is usually univertebral. Extensive primary lesions were related to massive infestation by tapeworm ova at multivertebral levels. At this stage, the disease presents as a malignant tumor resistant to all surgical procedures. The prognosis is poor because of severe neurological complications and inexorable spinal destruction. The hope remains that in the future an effective medical parasiticidal treatment will be available.

[Giant cell tumors of the distal end of the radius treated by resection-arthrodesis]. / Tumeurs à cellules géantes de l'extrémité inférieure du radius traitées par résection-arthrodèse.

The distal end of the radius is one of the sites commonly affected by giant-cell tumor of bone; it poses problems of reconstruction after resection of the distal radius. The authors present a retrospective study of 5 giant cells tumors treated by resection-arthrodesis of the wrist. These tumors were seen in 3 men and 2 women. From the radiological point of view, all tumors were extensive and lytic lesions centered in the epiphysis and involving the metaphysis; the wrist joint was respected. From the histological point of view, the tumors were grade I in 3 cases and grade II in 2 cases. With a mean follow up of 8 years, the results were considered good in 3 cases, fair in 1 case and poor in 1 case. A high incidence of bone complications was noted: delayed union (up to 11 months) and fracture of the graft, which occurred in 3 patients.

[Intramuscular myxoma. Apropos of two cases]. / Myxome intra-musculaire. Apropos de deux localisations.

The authors report two cases of rare localization of intra muscular myxoma: one in the buttock, and the other in the lumbar area. The first case, a 58 years old woman had a four month history of a mass in the buttock. Computed tomography revealed the presence of a solid tumor. Diagnosis was based on histological examination. After surgical treatment no recurrence was observed after fourteen months. The second case was a 54 years old woman who presented a sciatica with back pain and neurological signs. The symptoms didn't response to medical treatment. Computed tomography showed an enlarged and-cystic lumbar lesion extending to the first sacral hole, with bone erosion of L3 and L4 articular apophysis and spinous process. Needle biopsy and surgical excision of the tumor were performed. Total neurological recovery was observed and no recurrence was observed after three years. The authors reminded the characteristics of this rare and benign soft tissue tumor and specified the place of magnetic resonance imaging (MRI) to establish the diagnosis which was still confirmed by histological examination of an open biopsy. Clinical follow-up revealed exceptional recurrence and no metastasis.

[A rare diaphyseal bone tumor: degenerative mucoid cyst]. / Une tumeur osseuse diaphysaire rare: le kyste mucoïde dégénératif.

The purpose of the study was to clarify the clinico pathological features of periosteal ganglion. The authors present a case of an unusual diaphyseal bony tumor in a 27 years old man. The diagnosis of diaphyseal periosteal ganglion cyst was made on roentgenographic appearance, presence of mucoid material and histologic evidence of a synovial type cellular lining. The patient was successfully treated with excision. The follow-up examination showed no recurrence one year after surgery. The radiological differential diagnosis were considered and possible pathogenes for a periosteal ganglion was discussed. Periosteal ganglia must be considered as a cause of cortical erosions. This lesion may radiologically mimic other periosteal lesions. Treatment with excision is successful in most cases.

[Use of Ilizarov fixator in the treatment of post-osteomyelitis pseudoarthroses in children]. / Utilisation du fixateur d'Ilizarov dans le traitement des pseudarthroses post-ostéomyélitiques de l'enfant.

PURPOSE OF THE STUDY: The aim of this study was to emphasize the importance of the distraction osteogenesis technique in the treatment of post-osteomyelitis nonunion. We report 12 cases. PATIENTS AND METHODS: Mean patient age was 9.5 years. There were 7 cases of nonunion of the femur and 5 involving the tibia. Nonunion was associated with 5.9 cm shortening in 10 cases, 8.4 cm circumferential bone defect in 1 case, shortening and bone defects in 6 cases, and shortening and joint deformities in 2 cases. RESULTS: Consolidation was obtained in 11 cases with an average delay of 11.6 months. Infection was eradicated in 75 per cent of the cases. Average residual shortening was 0.4 cm. Joint deformities and axial deviations were corrected at the same time in all cases. DISCUSSION: The advantages of the Ilizarov technique are well known for the treatment of such deformities and musculoskeletal system disorders subsequent to osteomyelitis: nonunion, infection, shortening, joint deformities and malunion.

[Valgus osteotomy in the treatment of pseudoarthrosis of the femoral neck: 41 cases]. / Ostéotomie de valgisation dans les pseudarthroses du col du fémur: à propos de 41 cas.

PURPOSE OF THE STUDY: Treating hip trauma victims who develop nonunion of the femoral neck remains a challenge in orthopedic surgery. We studied the clinical and radiological outcome after Pauwels osteotomy for valgisation. MATERIAL AND METHODS: This retrospective series included 41 patients (25 men and 16 women), mean age 44.5 years. Initial treatment was surgical in 21 cases and had been insufficient in 91%. Nonunion (subcapital in 53%, transcervical in 40% and basicervical in 7%) had persisted for 16 months. A tight nonunion was present in 65.9% of the cases and the femoral head appeared normal in 70%. Femoral osteotomy was performed for simple valgisation in 78% of the cases with a mean 31.5 degrees correction (range 15 degrees to 50 degrees ). A nail or screwed plate was used for fixation. Results were analyzed at a mean 76 months post-surgery. RESULTS: Bone healing was achieved in 98% of the cases. The only failure was related to inappropriate surgical technique. Necrosis was observed in 16 cases. The functional outcome was very good or good in 82% of the patients. CONCLUSION: Osteotomy for valgisation remains a very good salvage procedure for nonunion of the femoral neck following hip trauma. It can be used for elderly subjects even in the presence of early stage or partial necrosis.

[Extra-renal retroperitoneal angiomyolipoma]. / Angiomyolipome rétropéritonéal extra-rénal.

Angiomyolipoma is a benign kidney tumour. Its extrarenal, retroperitoneal site is exceptional. The authors report a case and emphasise the value of ultrasonography and computed tomography in the diagnosis of these tumours with a marked fatty component, without neglecting the contribution of arteriography which can be useful in the diagnosis of the origin of very large retroperitoneal tumours.

[A case of bladder pheochromocytoma]. / A propos d'une observation de phéochromocytome vésical.

Phaeochromocytoma is an endocrine neuroectodermal tumour usually located in the adrenal gland, but occasionally arising in other organs. The authors report a case of a vesical phaeochromocytoma presenting with haematuria. Radiological and endoscopic investigations revealed a right laterovesical submucosal tumour. Partial cystectomy was performed. The normality of the postoperative endocrine survey suggested disappearance of the tumour and absence of any other sites.