Periodic limb movement in sleep in children with Williams syndrome

Difficulty differentiating physiologic genu varum from early Blount's disease persists. Drennan's metaphyseal-diaphyseal (MD) angle remains the most consistently valuable radiographic parameter despite measurement error. Clinical risk factors also should be considered. All patients receiving orthoses for genu varum since 1985 were reviewed. The focus of the study was those patients with an MD angle of >16 degrees or between 9 and 16 degrees with a clinical risk factor for progression. Risk factors considered were ligamentous instability, obesity, asymmetry, and being female, black, or Hispanic. Thirty-eight patients with 60 tibiae were included. The success rate was 90%. Risk factors for failure (six cases) were instability, obesity, and delayed bracing. In cases with MD angles >16 degrees, the success rate was 86%. The results of orthotic treatment, restricted to patients meeting the stated parameters, represent improvement on the reported natural history.     

Angiotensin II signal transduction pathways

It has been 100 years since the discovery of renin by Tigerstedt and Bergman. Since that time, numerous discoveries have advanced our understanding of the renin-angiotensin system, including the observation that angiotensin II is the effector molecule of this system. A remarkable aspect of angiotensin II is the many different physiological responses this simple peptide induces in different cell types. Here, we focus on the signal transduction pathways that are activated as a consequence of angiotensin II binding to the AT1 receptor. Classical signaling pathways such as the activation of heterotrimeric G proteins by the AT1 receptor are discussed. In addition, recent work examining the role of tyrosine phosphorylation in angiotensin II-mediated signal transduction is also examined. Understanding how these distinct signaling pathways transduce signals from the cell surface will advance our understanding of how such a simple molecule elicits such a wide variety of specific cellular responses.     

Fever in intensive care: keep medications in mind at all times

In two patients, men aged 35 and 69 years admitted postoperatively to the intensive care unit, fever of unknown origin developed. One had been admitted because aspiration was suspected. He had been treated immediately with amoxicillin and clavulanic acid. The other had undergone oesophageal excision and gastric reconstruction because of oesophageal carcinoma and had been subjected to antibiotic decontamination (amphotericin B, norfloxacine en fungizone). No cause for the fever was detected, but it quickly subsided after discontinuation of the amoxicillin-clavulanic acid and the norfloxacine, respectively. When encountering fever of unknown origin in intensive care patients it is always important to think of drug fever.     

Rehabilitation outcomes of long-term survivors treated for head and neck cancer

BACKGROUND: Little is known about the rehabilitation outcomes of long-term survivors following treatment for head and neck cancer. There are, for example, no studies on physical and psychosocial rehabilitation outcomes of T1 glottic larynx carcinoma, despite the fact that these form the majority of head and neck cancer sites. Thus, this investigation afforded a unique opportunity for examining similarities and differences among T1 glottic larynx patients, laryngectomy patients, and those who had surgery for cancer of the oral cavity and/or oropharynx along a variety of physical and psychosocial dimensions. METHODS: To describe the impact of these three types of head and neck cancer and their treatment on the physical and psychosocial functioning of long-term survivors, a self-report questionnaire was completed by 110 patients treated between 2 and 6 years previously in a major cancer center. RESULTS: Data indicate that a higher percentage of patients treated with laryngectomy or commando procedures still experience severe psychosocial distress between 2 and 6 years after their last treatment than do patients treated with radiotherapy for a T1 carcinoma of the glottic larynx. Psychosocial and physical complaints are still reported by many laryngectomy patients, apparently the result of problems in effective communication with others. Many commando procedure patients experience problems with respect to food intake, and with disfigurement and its consequences. T1 larynx patients mainly experience a considerable number of physical complaints. The greater the time that had elapsed since treatment, the fewer the psychosocial problems associated with head and neck tumors. Open discussion of the illness in the family, social support, and perceptions of adequate information from the specialist are the most important predictors of positive rehabilitation outcomes. CONCLUSIONS: This study indicates that T1 larynx patients report many physical complaints even though several years had elapsed since treatment. Also, laryngectomy patients may need psychosocial guidance for a longer posttreatment period and that health care personnel must involve the partner as much as possible in all communications. Commando procedure patients in particular feel hindered by their disfigurement and its consequences. Future research with respect to validation of the specific head and neck modules is needed.     

A generalised fuzzy reasoning algorithm for an object-oriented expert system tool

Abstract: A fuzzy reasoning algorithm is presented in this paper. It is based on the concept of a generalised fuzzy production rule that adds new conjunctions such as ADD and REL to the usual AND and OR conjunctions for the linkage of premises in conventional rules. A definition is given for weights and related factors, to express and measure a relation between the premises. A formal representation for fuzzy premises is presented, together with the introduction of a fuzzy match method. Multiple thresholds are used to convert the uncertainty measures of reasoning results into linguistic variables. We have applied the fuzzy reasoning algorithm to a frame-based hybrid expert system tool. An object-oriented approach is used in implementing the system. Some results of the application are presented and discussed.     

The prevalence, structure and clinical problems of multiple sclerosis in the Transcarpathian area based on epidemiological study data

As many as 230 patients with disseminated sclerosis were examined in the Transcarpathian region. Sick male- and female populations were found out to be almost similar. There were more sick persons in the medium-range areas of the Carpathian Mountains, with a mean age being 35 +/- 5 years. It has been established that intake of spirulina makes for lengthening of remission in those patients with disseminated sclerosis.     

Preparation of the myristoylated and nonmyristoylated form of recombinant recoverin in E. coli cells and comparison of their functional activity]

Numerous data strongly suggest the involvement of cytokines and the matrix metalloproteinase collagenase (MMP-1) in the pathogenesis of periodontitis. Recently, we have demonstrated that, upon culturing under the influence of IL-1 alpha + EGF, a large amount of inactive procollagenase (MMP-1) is stored in the extracellular matrix of periosteal tissue. We now show that this endogenous reservoir of proenzyme can be operative after activation with plasmin and is able to induce a rapid and almost complete breakdown of the collagenous extracellular matrix. The level of collagen degradation following activation showed a strong correlation with the amount of proenzyme that was incorporated in the tissue. The highest level of degradation (70% of the total amount of collagenous proteins) was found with the IL-1 alpha + EGF-treated explants, followed by those treated with IL-1 alpha alone (35%). Explants cultured with EGF or in the absence of cytokines, containing only small amounts of procollagenase, showed little collagen breakdown following plasmin activation (7%). Inhibition of metalloproteinases by EDTA, or blockage of plasmin by PMSF, prevented the degradation in all explants irrespective of the amount of proenzyme present in the tissue. Our findings demonstrate that endogenous proenzyme stored in a native connective tissue matrix can be activated at a later time interval which results in a massive breakdown of the tissue. This study shows a possible pathway of collagenase-induced breakdown without recent de novo synthesis of the enzyme. Such a sequence may be operative in chronic inflammatory diseases, such as periodontitis, where production of procollagenase under the influence of cytokines spans a longer time period, whereas breakdown is often characterized by a cyclic behaviour.