Osteomesopyknosis: report of a new case with bone histology.

A new case of osteomesopyknosis, a rare autosomal dominant axial osteosclerosis is reported, with 4 affected members of the same family. Biochemical investigations, bone mineral content (BMC) measurement, 99mTc HMDP bone scan and microscopy of iliac crest bone and femoral head have been performed on 1 subject. A marked increase of BMC was found, without abnormality of biochemical data. Microscopy of bone showed an increase of trabecular thickness, and a low rate of bone turnover. No abnormality of mineralization was found on microradiographs.

Handedness, immune disorders and information bias.

In order to investigate the relations between handedness and migraine or immune disorders, we performed a case control study comparing the handedness of patients suffering from systemic lupus erythematosus (SLE), type I diabetes, Graves' disease, or migraine to that of a random sample of controls from the general population. A handedness index was measured from a 10-item questionnaire. No significant difference was observed. But when the controls who denied having ever suffered from migraine or any allergic disease were set apart from those who gave at least one positive answer to the same questions, the former were found more right-handed, i.e. with a lower handedness index than the latter (P less than 0.05) and than the SLE patients (P less than 0.05). More generally, the mean observed handedness index of controls giving a positive answer to any question about their health was found repeatedly higher than that of controls giving a negative answer: this was observed for 27 of the 32 questions. These results are highly suggestive of an information bias, the subjects saying they use the right hand for each of the 10 activities considered in the questionnaire being more likely to deny having suffered from a given disease or used, more or less recently, some drug or medical service. Our conviction is that previous observations dealing with the same topic are also more easily explained by the presence of an information bias than by Geschwind's theory. The implications for the design of further epidemiologic studies are discussed.

Management of male osteoporosis.

UNLABELLED: The objective of this study was to evaluate the efficacy of treatments for male osteoporosis selected based on the cause of the disease. METHODS: Sixty-three men with osteoporosis (T-score at the lumbar spine and/or femoral neck lower than -2.5) with a mean age of 53+/-11 years were studied. Forty-three (68.3%) had a history of fracturing without trauma (vertebral fractures, 37 patients, 57%). Treatments were as follows: idiopathic osteoporosis: calcium and vitamin D supplements (N = 10) or cyclical etidronate for 2 weeks followed by calcium and vitamin D supplements for 76 days (N = 29); moderate idiopathic phosphate diabetes: calcitriol and phosphate (N = 15); idiopathic hypercalciuria: hydrochlorothiazide (N = 6); and hypogonadism: testosterone (N = 3). RESULTS: Percentage change in bone mineral density (mean +/- standard error of the mean) after 18 months: calcium and vitamin D (lumbar spine: 0.6+/-2; femoral neck: 2.2+/-2.2); etidronate (lumbar spine: 3.6+/-1.4*; femoral neck: 0.5+/-1); calcitriol (lumbar spine: 7.0+/-3.5*; femoral neck: 0.0+/-1.4); thiazide diuretic (lumbar spine: 1+/-3.2; femoral neck: -2.3+/-3.7); and testosterone (lumbar spine: 6.8+/-6.4; femoral neck: 2.5+/-2.7), where *P < 0.05 versus baseline. Gastrointestinal side effects occurred in three patients (4.8%), including two on calcitriol-phosphate therapy and one on etidronate therapy. Of the six (9.5%) patients who experienced incident fractures, four were on etidronate, one on calcitriol-phosphate, and one on calcium-vitamin D. No patients discontinued their treatment because of side effects. CONCLUSION: Etidronate and the combination of calcitriol-phosphate produce a significant increase in lumbar spine bone mass in men with idiopathic osteoporosis or moderate idiopathic phosphate diabetes.

[Mastocytosis and bone manifestations. Results of the survey of the bone and phosphorus-calcium metabolism section of the French Society of Rheumatology]. / Mastocytose et manifestations osseuses. Résultat de l'enquête de la section os et métabolisme phospho-calcique de la Société Française de Rhumatologie.

Thirty two cases of the association of mastocytosis and bone lesions were collected in a multicentre study. Five cases involved osteocondensation forms. However, most often (27 cases), there was osteoporosis (OP). The diagnosis was made in the absence of obvious risk factors, and thus often in men (2/3 of patients), when there was the association of pigmented urticaria and an excess of mast cells in bone biopsies. Laboratory, radiological and isotope scan findings are often non-specific, being identical to those encountered in common OP. The histomorphometric profile involves an association of decreased cancellous bone volume, increased area of resorption and decreased bone formation parameters. Progression to malignant mastocytosis occurs essentially in diffuse osteocondensation forms and is rare in OP types. Emphasis must be placed on the importance of qualitative study of bone marrow, using specific stains, since the diagnosis may be missed in the absence of typical skin lesions. Conversely, since a simple increase in mast cell count is possible during common OP, a search for mast cell nodules is important in order to establish the diagnosis with certitude.

[Study of mastocytes in 1298 bone biopsies. Relationship between mastocytes and osteoporosis]. / Etude des mastocytes sur 1,298 biopsies osseuses. Relations entre mastocytes et ostéoporose.

The relationship between the bone damage in systemic mastocytosis and reactional mastocytosis is still poorly understood. The purpose of this study was to determine the incidence of excessive mastocytes in a series of bone biopsies and their significance in cases of osteoporosis. The mastocytes were routinely counted in 1,298 successive biopsies stained with May Grumwald Giemsa: 131 biopsies had more than 5 mastocytes/mm2, i.e., 10% of all samples for all diagnoses combined. In 11 patients (13 bone biopsies) with a large excess of mastocytes (more than 15/mm2) and osteoporosis, the biopsies were examined again to look for mastocytic nodules suggesting bone mastocytosis: mastocytic nodules of this type were found in only 4 cases. The mastocyte is an active cell which may play a role in bone metabolism through the intermediary of its mediators. In osteoporosis, the incidence and significance of excessive mastocytes is not yet understood; this excess of mastocytes appears to correspond to reactive mastocytosis rather than systemic mastocytosis.

Effects of glucocorticoids on skeletal growth in rabbits evaluated by dual-photon absorptiometry, microscopic connectivity and vertebral compressive strength.

The effects of corticosteroid on bone were examined in female growing rabbits treated with 0.7 mg/kg per day prednisolone for 5 months. The evolution of whole-body total bone mineral measured by dual-photon absorptiometry showed a significant difference between the prednisolone-treated group and the control group from the first to the fifth month. The histomorphometric profile of corticosteroid-induced osteoporosis was observed, in particular the lower bone volume and thinner and fewer trabecular plates. Mechanical tests are possible on rabbit vertebrae and showed a very significant difference in bone strength between the prednisolone-treated and control groups, and a good correlation between mechanical tests and histomorphometric or densitometric results. This bone corticosteroid model shows that vertebral compression tests are possible on rabbit lumbar vertebrae. It may contribute to a better evaluation of corticosteroid treatments.

[The school for patients with chronic backache. Retrospective study of 93 severely handicapped patients with backache and their socio-occupational status]. / L'école pour lombalgiques chroniques. Etude rétrospective de 93 lombalgiques lourdement invalidés et de leur devenir socio-professionnel.

A small percentage of low back pain sufferers are resistant to the various types of treatment tried and the problem becomes chronic. It is for this severely incapacitated group, threatened by socio-occupational misfortune, that we created: the School for Chronic Low Back Pain Sufferers. One hundred and five patients in whom treatment had failed were included after insurance programmes had agreed to provide cover. The system is of a multidisciplinary nature with hospitalisation for 5 days. The fate of 93 of these patients is known with a mean follow-up of 1 year. Quantified initial evaluation on an obstacle course confirmed that even severe low back pain sufferers were unaware of the basic rules for protection of the spine (mean score of 14/50 on admission). Socio-occupational fate analysed on the basis of a questionnaire showed: a high satisfaction index (90%) regardless of the subsequent outcome, and a correlation between follow-up time after the programme and the decrease in painful attacks (p less than 0.03, r = 0.23) or consumption of medications (p less than 0.01, r = 0.29). The relative simplicity of the system proposed, the situation of therapeutic impasse in which the patients for whom it is intended find themselves, the short time required and the results obtained confirm the importance of this multidisciplinary management approach being able to best envisage the possibilities of rehabilitation of the chronic low back pain sufferer, in particular when there are repeated periods off work.

[Study of bone repercussion in 33 cases with anorexia nervosa which 8 with fracture osteoporosis]. / Etude du retentissement osseux dans 33 cas d'anorexie mentale dont 8 avec une osteoporose fracturaire.

A national survey has regrouped 33 cases of anorexia nervosa (AN) with osseous investigations, in particular an absorptiometry of the lumbar spine. 2 groups have been defined: 25 patients without fracture (F-) and 8 patients with osteoporotic fractures (F+), including 5 cases with 12 vertebral fractures and 5 with non vertebral fractures: 31/33 have a low lumbar bone mineral content (BMC). The lumbar BMC of the F+ is significantly lower than the F-: -4.1 +/- 1.6 DS versus -2.2 +/- 1.2 DS (p less than 0.001). 5 F+ patients are under the fracture threshold (FT), (O F-), 2 F+ are on the same level as the FT, and the last F+ has a lumbar BMC 15% above the FT, but an osteoporotic wrist fracture and a very low femoral neck BMC (0.64 g/m2, normal 1.06 +/- 0.15). F+ are older (35.2 year old versus 23.6, p less than 0.001), owing the fact that the duration of the AN (+ 9.2 years, p less than 0.005) and of the amenorrhea (+ 9.8 years, p less than 0.001) are longer, although the AN began at the same age in the 2 groups. The minimum weight, the body mass index, the percentage of loss of weight are similar in the 2 groups. Phosphocalcic biological studies, which show rather high osteocalcinemia and hydroxyprolinuria (hyperremodeling), with rather high serum 1-25 OH vitamin D, do not differ between the 2 groups. Endocrinologic evaluations, with a constant hypooestradiolemia of hypothalamic origin, low somatomedinemia and normal cortisolemia, are not different between the 2 groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Labial salivary gland biopsy assessment in rheumatoid vasculitis.

OBJECTIVES: To assess the vascular involvement in labial salivary gland (LSG) from patients with rheumatoid vasculitis (RV). METHODS: Forty seven patients with rheumatoid arthritis (RA) took part in a prospective study. Among them, 12 had proven RV. LSG biopsy was performed after local anaesthesia. RESULTS: Histological appearance of inflammatory vascular damage was observed in all but one patient with proven RV (92%). Inflammatory vascular involvement was also identified in LSG biopsy of seven patients with RA (20%) and only one patient in the control group (8%). A second specimen of LSG was studied after a mean treatment period of six months and failed to show any feature of inflammatory vascular involvement in three of the five cases that were analysed. CONCLUSIONS: The study emphasises the high incidence of immunopathological features of microvascular damage in patients with RV. LSG biopsy is minimally invasive and may be a potential useful tool for the diagnosis of RV especially when skin lesions are absent or impossible to biopsy. The assessment of the predictive value of positive LSG biopsy in RA requires a long term prospective study.