Inverse correlation between tumor incidence and tissue histamine levels in W/WV, WV/+, and +/+ mice.

The influence of mast cells on tumor incidence and growth rate was studied in 2 grafted tumor models (fibrosarcoma MC-B6-1 and the Lewis lung carcinoma 3LL). Three kinds of WBB6F1 mice (a cross between WB/ReJ-W/+ and C57BL/6J-WV/+ mice) were used: W/WV (deeply mast cell depleted), WV/+ (partially mast cell depleted), and +/+ (normal mast cell number). The presumed resistance of F1 hybrids to tumor cells of parental origin was observed in 12 of 13 +/+ mice, but only in 11 of 22 WV/+ mice and in none of 39 W/WV mice. Tumor incidence and metastasis incidence were inversely correlated with tissue histamine levels and mast cell number. Growth rates of tumors were similar in W/WV and WV/+ mice, but the tumor growth rate was much slower in the only +/+ mouse in which the tumor grew. These results confirm the protective role of mast cells against tumors.

Endogenous superantigens in allogeneic bone marrow transplant recipients rapidly and selectively expand donor T cells which can produce IFN-gamma.

Despite the existence of many non-MHC disparities between MHC matched but non-MHC mismatched donors and recipients, graft-versus-host disease (GVHD) is not clinically apparent following a significant number of allogeneic bone marrow transplants (BMT) in experimental animals. The present studies examined V beta TcR expression and IFN-gamma production by donor T cells in a BMT model involving an MHC matched, allogeneic donor-recipient combination which included a unidirectional superantigen disparity (Mls). B10.D2-->BALB/c, but not BALB/c-->B10.D2 recipients develop GVHD and mortality ensues 8-12 weeks post-transplant. During the first 2 weeks post-transplant of B10.D2-->BALB/c, approximately 50% of all Thy1.2+ spleen and lymph node cells were found to express T cell receptors utilizing V beta 3. A similar rapid and selective expansion of V beta 3+ TcR bearing donor T cells was detected in two other H-2 matched superantigen disparate donor-recipient BMT combinations. An increased percentage of V beta 3+ T cells was noted among both the CD4+ and CD8+ populations. Thus, in these donor/recipient combinations, all TcR families were not equally expanded early following transplant. At 4-10 days post-transplant, IFN-gamma specific mRNA was readily detected in the spleens of B10.D2-->BALB/cBMT recipients containing large numbers of V beta 3+ T cells. Moreover, V beta 3+ donor T cells from these recipients contained IFN-gamma mRNA. Specific stimulation in vitro with immobilized anti-TcR moAbs demonstrated that V beta 3+ T cells secreted a large amount of the total IFN-gamma levels detected. The ability of endogenous superantigens to activate large numbers of T cells which can produce cytokines after BMT indicates that when present, such antigenic differences may contribute to events occurring during initial graft-versus-host reactions. Such antigens could therefore participate in the events influencing whether GVHD develops following BMT between certain donors and recipients.

Scavengers of free radical oxygen affect the generation of low molecular weight DNA in stimulated lymphocytes from patients with systemic lupus erythematosus.

Factors that potentially affect the generation of excess low molecular weight DNA (LMW-DNA) in cultured phytohemagglutinin (PHA)-stimulated lymphocytes of patients with systemic lupus erythematosus (SLE) were studied because this species of DNA is consistently found and this DNA may play a role in the pathogenesis of the disease. Superoxide dismutase (SOD; 0.05 mg/mL), a scavenger of free radical oxygen, decrease LMW-DNA formation in lymphocytes by 22%. Co-cultivation with cysteamine, a second scavenger of free radical oxygen and a sulfhydryl radioprotective agent, resulted in a 32% decrease in the generation of excess LMW-DNA at a concentration of 0.5 x 10(-3) mol/L and largely prevented its formation at 1.0 x 10(-3) mol/L. Other free radical scavengers (catalase, mannitol, vitamins C and E), cyclooxygenase inhibitors (ibuprofen and aspirin), a xanthine oxidase inhibitor (allopurinol), and an iron chelator (desferoxamine) did not affect excess LMW-DNA formation. Glutathione (1 x 10(-3) mol/L) had no effect and cysteine was toxic. Because scavengers of free radicals might be useful in the therapy of lupus, a trial of cysteamine (30 to 60 mg/kg/d) was administered to six acutely ill patients with SLE. A therapeutic benefit was not demonstrated, and some patients had exacerbation of disease. Lymphocyte cell growth from control and lupus subjects was stimulated when cysteamine, 1 x 10(-5) to 1 x 10(-4) mol/L was added to the media, but inhibited at concentrations of 2 x 10(-4) mol/L or greater. These studies suggest that the autooxidation and toxicity of high-dose cysteamine preclude its therapeutic use as a free radical scavenger.

Treatment of asthma with tulobuterol or albuterol in school-age children.

The subjects were 40 children aged 6 to 16 years with stable chronic asthma; 20 were randomly assigned to receive 40 micrograms/kg of tulobuterol twice daily and 20 received 100 micrograms/kg of albuterol three times daily for three months. Patients were assessed by spirometry after the morning dose of medication at 0, 2, 4, 8, and 12 weeks of treatment. After initial dosing, the mean percentage increases in forced expiratory volume in one second (FEV1) were significantly higher in the tulobuterol-treated patients than in the albuterol-treated patients: at 30 minutes after dosing, the mean increase was 17.2% in the tulobuterol group and 5% in the albuterol group; at one hour, 20.3% and 6.8%. Similar results were found at 12 weeks. Mean changes in forced vital capacity and peak expiratory flow rate were similar to the changes in FEV1. Treatment side effects were reported by seven tulobuterol-treated patients and by four albuterol-treated patients. Tulobuterol treatment was withdrawn in one patient because of severe vomiting and headache of unknown cause. No changes in cardiovascular function were found in any patient. It is concluded that tulobuterol taken twice daily was more effective than albuterol taken three times daily in the treatment of asthma in children.

Dissociation of symptom scores and bronchial hyperreactivity: study in asthmatic children on long-term treatment with inhaled beclomethasone dipropionate.

The aim of the study was to evaluate the effects of inhaled steroids (IS) on the improvement of clinical asthma symptoms and on the decrease in bronchial hyperreactivity (BHR). Twenty-four children with severe asthma were given 1,000 micrograms beclomethasone dipropionate (BDP) daily and compared with ten asthmatic control children. The study included the evaluation of daily clinical score, of exercise induced asthma, of bronchial obstruction (forced expiratory volume in 1 sec, FEV1), and of BHR at months 0, 1, 2-3, and 4-5 (M0, M1, M2-3, and M4-5). BHR was assessed by standardized inhaled carbachol provocation measuring plethysmographic specific airway resistance (SRaw). The carbachol dose causing a 40% decrease in specific conductance (SGaw) was determined (PD40 SGaw). Clinical scores decreased at M1 (P less than 0.01) and throughout the study. FEV1 increased at M1 (P less than 0.05), M2-3 (P less than 0.01), and M4-5 (P less than 0.05) compared to M0. PD40 SGaw only increased significantly at M1 and M2-3. No individual correlation was found between clinical scores and PD40 SGaw at any testing, or between the decrease of clinical scores and the decrease of BHR. We conclude that bronchoconstrictive challenge tests do not adequately assess the clinical efficacy of IS. In clinical practice non-specific BHR should be preferentially measured for diagnosing atypical forms of asthma.

Dermatitis due to orthopaedic implants. A review of the literature and report of three cases.

The cases of three patients who had dermatosis that was caused by an orthopaedic implant are reported. The main clinical pattern was localized or generalized eczema or urticaria. The diagnostic criteria that have been proposed by various authors are reviewed. Removal of the implant did not always result in rapid disappearance of the dermatosis, presumably because a few particles of metal remained in the area of the implant.

Congenital ciliary aplasia in two siblings. A primitive disregulation of ciliogenesis?

Congenital ciliary aplasia was demonstrated in two siblings with clinical history of primary ciliary dyskinesia. Ultrastructural histochemistry of successive bronchial biopsies revealed the predominance of immature mucous cells and the total absence of ciliated or preciliated cells in the respiratory epithelium. This original disorder may represent a unique variant of primary ciliary dyskinesia with primitive disregulation of ciliogenesis.

[Fabry's disease in ophthalmology (author's transl)]. / La maladie de Fabry et ses manifestations ophtalmologiques.

The authors report a new case of Fabry's disease and emphasize the easiness of diagnosis on simple slit lamp examination of the affected males and conductor females. This disease is transmitted as X linked so that both heterozygotes and hemizygotes show a corneal storage known as cornea verticillata. The clinical features are completed by a conjunctival biopsy and the electron microscopy study shows lamellar bodies in the lysozomes.

[Practical diagnostic approach to anomalies of the aortic arch. Apropos of 31 pediatric cases]. / Approche diagnostique pratique des anomalies des arcs aortiques. A propos de 31 cas pédiatriques.

This analysis of 31 cases of anomalies of the aortic arch has confirmed the predominance of subclavian retro-oesophageal arteries and double aortic arches (74% of cases). The age at which the first clinical signs presented was less than one year in 75% of cases. Respiratory signs predominated with recurrent bronchitis (16 cases) and bronchial congestion (9 cases). These signs were not specific for the type of aetiology and were characterised by their chronicity and recurrent nature. Inspiratory and expiratory stridor was found on 8 occasions and was very suggestive of the diagnosis. There was a frequent association of asthma and anomalies of the aortic arch in this series (13 cases) with 6 cases of atopic asthma in early childhood and 7 cases of asthma in infancy. The oesophago-gastroduodenal transit was the key diagnosis which enabled the vascular anomaly to be confirmed. There was, however, a failure on 5 occasions with 2 false negatives and 3 cases where the wrong type was suspected. Fibreoptic bronchoscopy enabled the topography to be established more precisely including the degree of compression (in 14 cases) and showed evidence of associated tracheomalacia in 7 cases. Nuclear magnetic resonance was very helpful, giving a better definition of the anatomical type and of the relationship of the vascular arch with the oesophago-tracheal axis. Broncho-fibroscopy and nuclear magnetic resonance make strong contributions towards the indications for operation.

[An unusual foreign body. Report of a case of subcutaneous emphysema during an asthma attack]. / Un corps étranger hors du commun. À propos d'un cas d'emphysème sous-cutané lors d'une crise d'asthme.

Pneumomediastinum is an uncommon complication after an asthma attack. The essential clinical sign is the presence of subcutaneous emphysema. In the great majority of cases, the treatment of pneumomediastinum corresponds to that of asthma, with resorption over several days. The situation is very different when the development of pneumomediastinum and subcutaneous emphysema is secondary to an aspiration syndrome.

[Allergic children]. / L'enfant allergique.

Epidemiologic data have shown an increased prevalence (and severity) of atopy related diseases (asthma, eczema and allergic rhinitis) during the post 15-20 years. Atopic respiratory diseases such as allergic rhinitis and asthma represent the effects of an immunological response to allergens, mediated through immunoglobulins E. Development of a clinically significant atopic reaction depends on environmental exposure. The majority of allergic children display positive skin tests to house dust mites, animal danders or pollens. Immediate hypersensitivity to food allergens starts early in life and is most often associated with atopic dermatitis. Allergic reactions to peanuts are generally acute and severe, with an increasing frequency. Parents must be aware of their child's problem and preventive measures must be undertaken very early in life, first at home and later also at school. Family history remains the best predictor of atopy in newborn babies.

[The asthmatic child]. / L'enfant asthmatique.

Asthma is the most frequent chronic pediatric disease. Management is based on assessment of severity and should normalize lifestyle as well as pulmonary function. Mild asthma is treated with intermittent beta-2-agonists. Moderate asthma should receive non-steroids prophylactic anti-inflammatory drugs. Immunotherapy should be considered. Severe asthma requires inhaled steroids. At each stage inhaled medications should be preferred. Therefore inhalation delivery system must be adapted for children of different ages and degree of dyspnea. The importance of environment control is well recognized. Establishing a partnership with the child and its family and with the medical staff and with the child's school is always necessary.