Maxillary myxoma in children

Osseous myxomas are rare, slow growing, locally invasive, histologically benign, mesenchymal tumours that generally arise in the second and third decades of life. They are almost always seen in the facial skeleton, being less frequent in the maxilla than the mandible. These tumours are especially uncommon in children and present difficult diagnostic and therapeutic problems. The small amount of biopsy material may make diagnosis difficult, and if resection is conservative, the recurrence rate may be high. A literature review revealed only 20 reported cases of maxillary myxoma in patients aged 14 years or less. The purpose of this report is to add our case to the literature.     

Age-dependent decreases in mitogen-stimulation level and RNA content in peripheral blood mononuclear cells of Down syndrome patients

OBJECTIVE: The aim of the present study was to determine whether or not the phytohemagglutinin (PHA)-activated proliferation and average RNA content in peripheral blood mononuclear cells (PBMC) of Down syndrome (DS) patients change with age. METHOD: Stimulated portion of PBMC and total RNA levels in these cells after 72 h of PHA stimulation from 38 DS patients were compared with 28 age-matched healthy controls using flow cytometric measurement. RESULTS: Decreased ratio of PBMC from DS patients undergoes mitogenic stimulation with age (r = -0.84, P = 0.000). This decrease is not observed in the cells of control individuals (r = 0.03, P = 0.869). Stimulated PBMC in infants with DS have higher level of RNA contents compared to controls (Z = 2.227, P = 0.026). While RNA content in mitogen-stimulated PBMC of DS decreased progressively and significantly with age (r = -0.70, P = 0.000), no significant age-related change in RNA content was found among the cells of healthy individuals in the range of 0-27 year old (r = 0.275, P = 0.157, P > 0.05). CONCLUSION: Age-dependent decreases in mitogen-activated proliferation ratio and average RNA content of PBMC from DS patients appear as regular events. These results may contribute to the explanation of the immune deficiency seen in DS patients since the PHA-stimulated cells are principally T-lymphocytes. This is the first report on the decrease in PHA-stimulated proliferation ratio (stimulability) and RNA level in PBMC of DS patients in relation to age.     

Prevalence of angina, myocardial infarction and intermittent claudication assessed by Rose Questionnaire among patients with Behcet's syndrome

Objective. To determine the risk of clinical cardiovasculardisease in middle-aged patients with Behcet's syndrome (BS)compared with gender-matched non-BS subjects. Methods. The prevalence of angina, myocardial infarction (MI),doctor diagnosed ischaemic heart disease (IHD) and intermittentclaudication were sought by the Rose Angina Questionnaire in225 (141 M/84 F) BS patients (mean age: 52 ± 8) withBS and 117 (74 M/43 F) controls (mean age: 50 ± 5). Informationon atherosclerotic risk factors was also collected. Results. The prevalence of angina, MI and doctor-diagnosed IHDwere not different between BS patients and non-BS controls inthe whole study population and when males and females were separatelyanalysed. Angina tended to be more common among females comparedwith males among both patients and controls. Intermittent claudicationwas found to be significantly more common among BS patients,especially in males with venous disease. Conclusions. The findings in this cross-sectional clinical studyare in line with previous observations not indicating acceleratedatherosclerosis in BS. Intermittent claudication might not bea suitable tool for the detection of peripheral atheroscleroticdisease especially among BS patients having venous disease. KEY WORDS: Behcet's syndrome, Atherosclerosis, Cardiovascular disease, Angina, Myocardial infarction, Peripheral arterial disease, Claudication Submitted 30 September 2007; revised version accepted 20 December 2007.     

The management of Behçet's syndrome

Beh?et's syndrome (BS), originally described as a triad of oral aphthae, genital ulcerations and uveitis, is a systemic vasculitis that is prevalent in the Middle east, Far East and in the Mediterranean basin. It is characterized by a heightened state of inflammation although the main drive that initiates and sustains this is not yet elucidated. Suppression of this inflammatory state constitutes the major goal of treatment and therapy is tailored according to the specific manifestations observed. We now have considerable more insight on drug management of BS compared to 20 years ago. Particularly, within the recent past we have learned to use more rationally the agents that were already available to us. This is especially true for azathioprine, cyclosporin A, thalidomide and colchicine. Promising data are also being collected with alpha-interferon. With these agents, significant progress has been achieved in the management of uveitis and mucocutaneous symptoms but treatment issues related to thrombotic problems, major vessel involvement and neurological disease have not yet been resolved.     

Response to intradermal injection of monosodium urate crystals in Behçet's syndrome.

The cutaneous response to intradermal injection of monosodium urate crystals was investigated in 97 patients with Behçet's syndrome in Turkey and 14 in the United Kingdom, and in 82 healthy and 88 diseased controls. Urate crystals produced an increased erythematous response in patients compared with controls in both countries. This response was different from that of the pathergy test performed at the same time. The systemic acute phase response, studied only in Turkey, showed no differences between patients and controls.     

Purified protein derivative reaction is not augmented in Behçet's syndrome patients

SIR, In a recent position paper about the emerging role of TNF-antagonists in managing Behçet's syndrome (BS), the importanceof screening BS patients for latent tuberculosis before startingtreatment with these agents has been emphasized [1]. This isespecially important since in geographies where BS is endemic,the prevalence of tuberculosis is also relatively high. Guidelinesrecommend screening all patients with a tuberculin test andchest X-ray before starting treatment with TNF- antagonists[2]. Case reports suggest that