[Crow-Fukase syndrome (POEMS syndrome) and osseous mastocytosis secondary to Castleman's angiofollicular lymphoid hyperplasia]. / Syndrome de Crow-Fukase ("P.O.E.M.S. syndrome") et mastocytose osseuse secondaires à une hyperplasie lymphoïde angiofolliculaire de Castleman.

In a case of Crow-Fukase (POEMS) syndrome there was a chronic, progressive, and eventually lethal polyradiculoneuropathy. In addition, adenomegaly, oedema and pleural effusions, gonadic atrophy, serum monoclonal IgA, and skin pigmentation were present. Plain x-rays and CT scan of the pelvis and lower vertebrae showed multiple poorly defined lesions. At postmortem there was no myeloma and a bone mastocytosis was found. In addition, next to T11, there was an abdominal nodule, 2 cm in diameter, with histological characteristics of Castleman's angiofollicular lymphoid hyperplasia. Immunohistochemical studies showed that plasmocytes of this lesion secreted polyclonal immunoglobulins with a high prevalence of IgA. Thus, the primary interest of this case lies in the association of bone mastocytosis with a POEMS syndrome. Also, one single localisation of Castleman angiofollicular lymphoid hyperplasia was found, assumed to be in this case the cause of the POEMS syndrome. Therefore, a minute and benign hyperplasic lesion, which was only discovered at autopsy, secreted the protein responsible for the symptoms and signs, and eventually the patient's death.

[Chronic osteitis and arthritis of palmoplantar pustulosis. A familial form of B-27 negative spondylarthropathy]. / Les ostéites et arthrites chroniques de la pustulose palmo-plantaire. Une forme familiale de spondylarthropathie B27 négative.

A 35-year old woman presented with pustulosis palmaris et plantaris evolving in acute episodes parallel to those of a rheumatic disease consisting of bilateral sacro-iliac arthritis, manubriosternal fusion and sterno-clavicular arthritis. Her brother had spondylitis compatible with the diagnosis of pustular osteo-arthritis though without pustulosis. He presented with bilateral sacro-iliac arthritis, manubriosternal fusion, sterno-clavicular arthritis and an ossified fragment detached from the antero-inferior angle of C6. The two patients had the same HLA phenotype: A1, A9, B5, B41, CW4, DR2, DR4, absence of B27. In a specimen from the sacro-iliac joint the authors found active bone remodelling and inflammatory osteitis with fibrosis and infiltrates rich in mononucleate cells. An electron microscopic study--to the authors' knowledge, the first to be performed in such cases--demonstrated intracytoplasmic filamentous inclusions in two macrophages of the synovial fringe. Only one other case of the familial form has been published so far. Like the rheumatism of acne conglobata, the spondylitis of pustulosis palmaris et plantaris is one of the causes of sterno-costo-clavicular hyperostosis.

[Trichorhinophalangeal syndrome. Apropos of 4 cases discovered in adults]. / Syndrome tricho-rhino-phalangien. A propos de quatre cas découverts chez l'adulte.

Of four adult patients with the trichorhinophalangeal syndrome, 3 were from one family, one had associated major femoral trochlear dysplasia and bilateral mandibular condyle dysplasia, and another a bilateral coxa plana.

[Osteoarticular lesions in Whipple's disease. Apropos of a destructive form and review of the literature]. / Lésions ostéo-articulaires de la maladie de Whipple. A propos d'une forme destructrice et revue de la littérature.

A patient with Whipple's disease was considered as cured after treatment for two years, but relapse occurred as the neurological form. Radiographic imaging showed destructive osteoarticular lesions, with few or no symptoms, affecting the peripheral skeleton but also the sacroiliac joints. The published literature is reviewed and the different osteoarticular radiologic forms of the affection described.

[Bilateral nephrocalcinosis during the course of a Butler-Albright syndrome, explored by ultrasonography. A case report (author's transl)]. / Néphrocalcinose bilatérale explorée par échotomographie, au cours d'un syndrome de Butler-Albright. A propos d'un cas.

Ultrasonography was employed to explore bilateral nephrocalcinosis in a patient with chronic renal tubular acidosis. Ultrasonographic findings in this affection include multiple intraparenchymatous dense echos in the papillomedullary region with a posterior acoustic shadow cone. Technical artefacts employed to establish diagnosis are discussed.

[A study in dogs of micropellets for use in angiographic therapeutic procedures (author's transl)]. / Expérimentation chez le chien de micro-bille utilisables en angiographie thérapeutique.

A study was conducted in dogs to evaluate the effect on the organism of a new embolisation material: carnauba wax micropellets. The ultimate objective was to study the possible use of micropellets containing an active product for therapy in situ. Renal embolisation was performed by selective or highly selective arterial catheterisation in dogs. Pathological examination demonstrated that only mild foreign body reactions occurred at the embolisation site. Overall tolerance was good. The micropellets were shown to possess some biodegradation properties and to be susceptible to deformation. An attempt is made to define the ideal micropellet from the point of view of size, kinetic features, and biodegradation properties.

[Comparative study of Hexabrix and Lipiodol UF in sialography]. / Etude comparative de l'Hexabrix et du Lipiodol UF en sialographie.

Many new lipid- and water-soluble contrast media can be used in sialography, the three main criteria of choice being the cost, the quality of filling, particularly of the parenchyma, and its evacuation and clinical tolerance. Duroliopaque possesses very similar physicochemical properties to those of Lipiodol UF, but a sufficiently satisfactory filling of the parenchyma is not obtained. Comparison of Lipiodol UF and Hexabrix in parotid and submaxillary contrast imaging showed good clinical tolerance for both products, but improved parenchymatous filling with Lipiodol UF. However, the more effective glandular evacuation with Hexabrix makes this the product most suitable at the present time for morphologic exploration of salivary glands.

Chemoembolization: principles and perspectives.

Chemoembolization is the injection of active-principle-bearing microparticles via selective catheterization. The authors discuss the advantages and the possibilities of this method in relation to selective catheterization and to the various time periods required for microparticle biodegradation and the kinetics of drug release. Particular attention is paid to intra-arterial chemoperfusion, chemoembolization and chemoinfusion with microparticles.

Biodegradable cross-linked albumin microcapsules for embolization.

The interfacial polymerization process used in the present study produced individual cross-linked albumin microcapsules, the particle size of which depended on the emulsification stirring rate. The variation in cross-linking agent concentration altered the microcapsule wall properties. As shown by SEM observations, microcapsules prepared with low acyl chloride concentrations presented rippled surfaces, while the surfaces of the microcapsules prepared with high acyl chloride concentrations were smooth. It was then suggested that the membranes of the weakly cross-linked microcapsules consisted of cross-linked and denatured albumin. Embolization experiments in the dog kidney showed that the weakly cross-linked microcapsules were biodegradable within 7 days, whereas the highly cross-linked microcapsules were degraded over more than 14 days and led to prolonged ischaemia. The results of the histological experiments carried out on a further eight dogs supported the previous findings yielded by the angiographical experiments. Moreover, they indicated that no foreign body reaction occurred. The lack of this inflammatory granuloma should be attributed to the rapid biodegradation of the microcapsules which produced reversible ischaemic lesions.