Maffucci's syndrome. Case reports and literature review

Maffucci syndrome is a rare, congenital disease, which is associated with the appearance of multiple enchondromas (possibility of malignant transformation in 20 to 100%), soft tissue hemangiomas and other mesenchymatous injuries. Case 1 is a 33-year-old female who presented with multiple nodules predominantly in upper extremities. Upon examination, there was deformity in articulation and nodules on the hands, which were soft and moveable. There were bony shavings in the second and fourth fingers of the left hand (enchondromas and atypical cells associated with hemangioma esclerosante). Maffucci syndrome was diagnosed. Later, excision of subcutaneous nodules in superior extremities was performed along with excision of nodules in both hands and hypochondrium (enchondroma injuries of left hand and hypochondrium, hemangioma in right hand). There was dysarticulation of the second finger at the metacarpal level of the phalanges of the left hand (chondrosarcoma). The patient is being followed up currently. Case 2 is a 26-year-old female. The patient had a history of subcutaneous abdominal tumor, exostosis, nodules and nodule in right breast. Upon examination, a tumor was found in the right breast, exostosis of right tibia, injury to the right wrist and left thyroid nodule. A simple mastectomy and axillary dissection was performed (fibroadenoma to intracanalicular and 14 negative lymph nodes). Later, left thyroidectomy and lumpectomy in right wrist were performed (hyperplasia to nodular thyroid and hemangioma cavernous). There was injury in the carpus of the right hand and elbow (hemangioma cavernous and synovial tissue with fibrosis and enchondroma). A diagnosis was made of Marffucci syndrome associated with mesenchymatous tumors. The patient was in poor general health and did not survive this hospitalization.     

Developing a scale for measuring professional equity among Canadian physicians

This paper reports on progress made in defining and measuring the concept of professional equity through the development of a summative measure of professional equity and three of its components: financial, intrinsic and recognition equity. The study sample consisted of a stratified sample of 8375 Canadian physicians with usable responses from 2749 (32.8%). Following preliminary components analysis, items were grouped into constructs. Reliability of the constructs was then determined using Cronbach's alpha and total inter-item correlations followed by confirmatory factor analysis. A summary scale using all 15 equity items yielded a reliability: Cronbach's alpha=0.86. The sub-scales reliabilities were: financial equity (Cronbach's alpha=0.91); intrinsic equity (Cronbach's alpha=0.86); and recognition equity (Cronbach's alpha=0.70). The professional equity measures reported are therefore capable of assessing different aspects of equity and represent an advance over more general effort-reward scales or those that only measure the range of rewards.     

Comparison of standard and artificial neural network estimators of hemodialysis adequacy

The National Kidney Foundation and the European Renal Association recommend routine measurement of hemodialysis (HD) dose and have set standards for adequacy of treatment. We compare the results of five methods for HD dose estimation, classifying each result as adequate or inadequate on the basis of equilibrated (eq) Urea Reduction Ratio (URR(eq)) > or = 65% or Kt/V(eq) > or = 1.2, to assess the accuracy of each method as a diagnostic tool. Data from 113 patients from two different dialysis units were analyzed. Equilibrated postdialysis blood urea was measured 60 min after each hemodialysis session to calculate URR(eq) and Kt/V(eq), considered as gold standard indexes (GSI). URR and Kt/V were estimated by using the Smye formula, an artificial neural network (ANN), modified URR, the second generation Kt/V Daugirdas formula, and standard indexes based on postdialysis urea, then compared to the GSI. For URR, best estimator was ANN (error rate: ER% = 12.70), followed by modified URR (ER% = 17.46%), the Smye (ER% = 22.22), and standard URR (ER% = 23.81). For Kt/V, the Daugirdas equation and the ANN were similar (ER% = 9.52 and 11.11). The single-pool Kt/V (Kt/V(sp)) > or = 1.4 (ERA recommended) produced an ER% = 7.94 and a false positive rate (FPR%) equal to that shown by the ANN (FPR% = 3.17). According to the current threshold limits for HD dose adequacy, the ANN was a reliable and accurate tool for URR monitoring, better than the Smye and the modified URR methods. The use of the ANN urea estimation yields accurate results when used to calculate Kt/V. The Kt/V(sp) with an adequacy threshold of 1.4 is a superior approach for HD adequacy monitoring, suggesting that the current adequacy limits should be reviewed for both URR and Kt/V.     

Acoustic analysis of clear versus conversational speech in individuals with Parkinson disease

A number of studies have been devoted to the examination of clear versus conversational speech in non-impaired speakers. The purpose of these previous studies has been primarily to help increase speech intelligibility for the benefit of hearing-impaired listeners. The goal of the present study was to examine differences between conversational and clear speech in individuals with Parkinson disease (PD). Twelve individuals were recorded producing conversational and clear speech. Acoustic analysis revealed that individuals with PD used some of the same clear speech strategies used by non-impaired speakers. Specifically, clear speech in PD was characterized by decreased articulation rate, increased mean fundamental frequency (F(o)), and increased speaking F(o)S.D. compared to conversational speech. The discussion examines the possibility that individuals with PD may have been independently applying a clear speech strategy based on their habitually increased percent pause values. Discussion also focuses on implications of the present findings to management of individuals with PD, and research implications. LEARNING OUTCOMES: As a result of this activity, the participant will be able to (1) describe the characteristics of clear speech produced by non-disordered individuals, (2) describe the acoustic characteristics of clear and conversational speech produced by individuals with Parkinson disease; and (3) describe the strategies individuals with Parkinson disease use when asked to produce clear speech.     

Aripiprazole in psychosis associated with Parkinson's disease

Aripiprazole is an agent for treating schizophrenia. The authors assume that aripiprazole as a dopamine receptor partial agonist may be useful in treating PD patients. Three cases are presented in this study. All patients were evaluated by the same clinician, a neuropsychiatrist well trained in the use of Brief Psychiatric Rating Scale (BPRS) and Clinical Global Impression (CGI). Aripiprazole was effective for reducing psychosis in each case.     

Biological transmission of vesicular stomatitis virus (New Jersey serotype) by Simulium vittatum (Diptera: Simuliidae) to domestic swine (Sus scrofa)

The role of hematophagous arthropods in vesicular stomatitis virus (New Jersey serotype; VSV-NJ) transmission during epizootics has remained unclear for decades in part because it has never been shown that clinical or subclinical disease in a livestock host results from the bite of an infected insect. In this study, we investigated the ability of VSV-NJ-infected black flies (Simulium vittatum Zetterstedt) to transmit the virus to domestic swine, Sus scrofa L. Experimental evidence presented here clearly demonstrates that VSV-NJ was transmitted from black flies to the swine. Transmission was confirmed by seroconversion or by the presence of clinical vesicular stomatitis followed by seroconversion. Our results represent the first report of clinical vesicular stomatitis in a livestock host after virus transmission by an insect.     

Effects of algae on the efficacy of Bacillus thuringiensis var. israelensis against larval black flies

Personnel from several black fly control programs have reported that the efficacy of Bacillus thuringiensis var. israelesis (Bti) is reduced during periods when algal concentrations are high in the waterways. Although the reduction in Bti-induced mortality in black fly larvae is presumed to be related to the presence of algae, no scientific data support this theory. In this study, 4 genera of algae (Microcytis, Scenedesmus, Dictrosphaerium, and Chlorella) commonly detected in Pennsylvania rivers where Bti-induced mortality in black fly larvae has been reduced were assessed to determine their respective effects on Bti-induced mortality by using an orbital shaker bioassay with laboratory-reared black fly larvae (Simulium vittatum cytospecies IS-7). A significant reduction in Bti-induced mortality was observed when Scenedesmus was present in the flasks at concentrations > or = 16,000 cells/ml. The Bti-induced mortality of larvae was not significantly reduced when Chlorella, Dictyosphaerium, or Microcytis was present in the flasks, even at concentrations > or = 250,000 cells/ml. These results indicate that the presence of certain types of algae can reduce the mortality of black flies exposed to Bti. Although not clearly defined, the mechanisms involved may be related to algal morphology due to overall size and structures associated with certain types of algae, and possible interference with feeding.