Relationships between non organ-specific autoantibody production and age-related modifications of mononuclear cell phenotype.

13 autoantibodies were sought in 23-26 elderly people and in 47-118 young controls. We found at least one autoantibody (mainly rheumatoid factor, RF, and anti-nuclear antibodies in 17/23 elderly (i.e., 17%) and in 7/45 young controls (i.e., 16%). Autologous rosette-forming cells and Leu-7 positive cells (Natural Killer Cells?) were higher, and OKT4 and OKT8 positive cells lower in the former than in the latter group. We show that elderly have a high proportion of CD5 positive B cells, compared with young controls, following phorbol myristic acetate activation in vitro. This abnormality might account for the discrepancy in production of RF compared to other autoantibodies.

Nonorgan-specific autoantibodies and immunoglobulin allotypes in relatives of patients with monoclonal gammopathy.

Polyclonal immunoglobulin increase, rheumatoid factor, antinuclear antibodies and cold lymphocytotoxins were detected 10, 8, 7 and 8 times, respectively, in a group of these informative families (22, 17 and 29 subjects tested, respectively). Each family included at least 1 subject with a monoclonal gammopathy in addition to that of the proband. No correlation could be shown between any of these abnormalities and Gm haplotypes. Nonetheless, it is worth noting that 6 out of 41 relatives under 30 years of age had cold lymphocytotoxins.

How and what kind of bone is made by metastatic cells of prostatic cancer.

In 14 patients with densifying bone metastases of prostatic cancer, the analysis of blood and urine P-Ca parameters, serum 25 OH D3, Ca infusion test, and histomorphometry with measure of calcification rate shows that: - hypocalcemia is common in such patients, sometimes associated to a lack of vitamin D with hypocalciuria and increased Ca retention at the infusion test, and sometimes unexplained; - from a histological point of view, this osteopathy is characterized by a hyperosteoidosis, often an important one, around and only around the metastasis foci, the malignant cells being indispensable to induce the osteoidosis. The entire mineralization of the osteoid matrix may happen but rarely because the hyperosteoidosis is most often increased by a vitamin D deficiency.

[Biological and histological profile of osteomalacia in condensing prostatic osteosis. Apropos of 4 cases]. / Profil biologique et histologique d'ostéomalacie dans l'ostéose condensante prostatique. A propos de 4 observations.

The authors report on 4 cases of condensating prostatic osteosis, whose biological picture and histomorphometric lesions in the involved area indicate osteomalacia. They discuss the individual nature of such a syndrome, the links between vitamin D deficiency and hyperosteoidosis at a very slow noted speed of calcification, and the usefulness of investigating and treating the syndrome.

[Osteosis deformans of thyroid origin. Radiological, histomorphometric and pathophysiological study (author's transl)]. / Ostése thyroöidienne déformante. Etude radiologique, histomorphométrique et physiopathologique.

A 73-year-old woman with severe, old standing thyrotoxicosis developed osteosis with deformities of the lower limbs and spine resembling osteomalacia. All the well-known radiological, and histomorphometric features of thyroid osteosis were caricaturally present in this patient who, in addition, exhibited curvatures of the lower limbs that are exceptionally described in hyperthyroidism. A study of standard phosphorus/calcium values, 25 OH D values, circulating parathyroid hormone levels and fractional absorption of 47Ca before and after thyroidectomy enables the authors to discuss the current pathophysiological concepts concerning phosphorus/calcium and bone metabolism in hyperthyroid conditions. A possible action of thyroid hormones on vitamin D metabolism or peripheral effects is envisaged.

[Juvenile and familial amyotrophic lateral sclerosis. 2 case reports (author's transl)]. / La sclérose latérale amyotrophique juvénile et familiale. Place nosologique, á propos de deux observations.

The case report of two brothers who evidenced neurological disorders with peripheral nerve syndrome, pyramidal deficit and bulbar symptoms is described. The diagnosis of amyotrophic lateral sclerosis was assumed upon the association of these clinical features. Seven and nine years respectively after the beginning of the disease, an increase of the clinical features was noticed in the 2 children, more pronounced in the second one. The possibility of true juvenile and familial amyotrophic lateral sclerosis is discussed in this study; different aspects of this disease are described. A final classification of these 2 cases will not be possible until histological features are available.

[Monocional immunoglobulins associated with bacterial endocarditis. Two case reports (author's transl)]. / Immunoglobulines monoclonales au cours d'endocardites bacteriennes. A propos de deux observations.

An IgGK monoclonal gammopathy occured in two patients with streptococcus viridans bacterial endocarditis. The gammopathy has been unchanged, respectively for 32 and 21 months following its detection. The alternate physiopathologic hypothesis are argued.

[Lipidic myopathy with severe cardiomyopathy caused by a generalized carnitine deficiency. Favourable course during carnitine hydrochloride treatment]. / Myopathie lipidique avec cardiomyopathie sévère par déficit généralisé en carnitine. Evolution favorable sous un traitement par chlorhydrate de carnitine.

The case of a girl who presented with gastrointestinal upsets with nausea, vomiting and occasional hypoglycaemic attacks during childhood is reported. At about 5 years of age generalised muscular weakness with severe amyotrophy, cardiomegaly with a cardiothoracic ratio of 0,63, left ventricular hypertrophy on electrocardiography and left ventricular dilatation with hypokinesis on echocardiography were observed. A few weeks later she developed severe cardiac failure. Muscle biopsy showed muscular dystrophy with lipid infiltration due to carnitine deficiency )serum carnitine 9 nmoles/ml, normal values: 46 +/- 6,9 nmoles/ml; muscle carnitine 0,27 nmoles/mg, normal values: 3,0 +/- 0,79 nmoles/mg fresh frozen weight). She improved rapidly with carnitine chlorhydrate and a diet low in lipids and high in medium chain triglycerides. Regression of muscular symptoms and cardiac failure was observed. After 13 months follow-up with no tonicardiac therapy she is much improved; the signs of heart failure have disappeared, the cardiothoracic ratio is now 0,55 and the electrocardiogramme and echocardiogramme are normal.

Familial occurrence of monoclonal gammapathies.

The purpose of this work was to evaluate the genetic propensity importance in the monoclonal gammapathies occurrence. Systematic serum-electrophoreses were performed on 172 relatives of 37 reported patients and on 10,784 controls over 30. The 4 families including one or more "paraproteinemia" other than the proband's were investigated. Experimental, epidemiologic, immunologic and genetic arguments are discussed.