Sample records for replace atypical responses

  1. Atypical clinical response patterns to ipilimumab. (United States)

    Ledezma, Blanca; Binder, Sandra; Hamid, Omid


    Patients with advanced melanoma have few treatment options, and survival is poor. However, improved understanding of how the immune system interacts with cancer has led to the development of novel therapies. Ipilimumab is a monoclonal antibody that inhibits cytotoxic T-lymphocyte antigen-4 (CTLA-4), a key negative regulator of host T-cell responses. This article presents cases of patients receiving ipilimumab in clinical trials along with a discussion of their significance and relevance to nursing practice. The patients showed different response patterns to ipilimumab and also had various typical immune-related adverse events (irAEs), which were managed successfully. The atypical response patterns produced by ipilimumab likely reflect its mechanism of action, which requires time for the immune system to mount an effective antitumor response. Meanwhile, lesions may appear to enlarge as a consequence of enhanced T-cell infiltration, although this may not necessarily be true disease progression. Patients receiving ipilimumab may respond very differently compared to how they might react to chemotherapy. Responses can take weeks or months to develop; therefore, clinicians should not terminate treatment prematurely, providing the patient's condition allows for continuation. Early recognition of irAEs combined with prompt management will ensure that events are more likely to resolve without serious consequences.

  2. Relationships among Sensory Responsiveness, Anxiety, and Ritual Behaviors in Children with and without Atypical Sensory Responsiveness. (United States)

    Bart, Orit; Bar-Shalita, Tami; Mansour, Hanin; Dar, Reuven


    To explore relationships between sensory responsiveness, anxiety, and ritual behaviors in boys with typical and atypical sensory responsiveness. Forty-eight boys, ages 5-9 participated in the study (28 boys with atypical sensory responsiveness and 20 controls). Atypical sensory responsiveness was defined as a score of ≤154 on the Short Sensory Profile. Parents completed the Sensory Profile, the Screen for Child Anxiety Related Emotional Disorders, and the Childhood Routines Inventory. Children with atypical sensory responsiveness had significantly higher levels of anxiety and a higher frequency of ritual behaviors than controls. Atypical sensory responsiveness was significantly related to both anxiety and ritual behaviors, with anxiety mediating the relationship between sensory modulation and ritual behaviors. The findings elucidate the potential consequences of atypical sensory responsiveness and could support the notion that ritual behaviors develop as a coping mechanism in response to anxiety stemming from primary difficulty in modulating sensory input.

  3. Mediastinal vacuum phenomenon: atypical pneumomediastinum caused by gas replacement of diminished fat. (United States)

    Hagiwara, Hiroaki; Torii, Ikuo


    We report a case involving an 83-year-old man with interstitial lung disease who developed atypical pneumomediastinum caused by gas replacement of diminished fat. The patient presented with a complaint of worsening symptoms of respiratory difficulty since a diagnosis of chronic eosinophilic pneumonia 5 months back. He had been under observation with no particular treatment for 5 months. Computed tomography performed on admission revealed pneumomediastinum. When the current scan was compared with that obtained 5 months ago, it was evident that the fat surrounding the mediastinum had been replaced by gas density. There was no mediastinal enlargement, pneumothorax, or pneumopericardium. Because the patient was elderly, home oxygen therapy was initiated for the interstitial pneumonia with no steroid therapy. Computed tomography performed 10 months after discharge showed the reappearance of mediastinal fat and no evidence of gas density. This case is unique because the pneumomediastinum was distinct from spontaneous pneumomediastinum caused by alveolar air leaks and resembled the vacuum phenomenon caused by intervertebral disc degeneration.

  4. Atypical radiation response of SCID cells (United States)

    Chawapun, Nisa

    Murine SCID (severe combined immune deficiency) cells are well known for their defect in DNA double-strand break repair and in variable(diversity)joining [V(D)J] recombination due to a mutation in a catalytic subunit of DNA-dependent protein kinase (DNA-PKcs). As a consequence, scid cells are hypersensitive to ionizing radiation. The present study showed that asynchronous populations of scid cells were about two-fold more sensitive than Balb/c with respect to cell killing and the defect in scid cells was corrected by complementation with human chromosome 8. Analysis of the survival of synchronized populations as a function of the cell cycle revealed that while scid cells were hypersensitive in all cell cycle phases compared to wild-type cells, this hypersensitivity is even more pronounced in G1 phase. The hypersensitivity reduced as the cells progressed into S phase suggested that homologous recombination repair plays a role. The results imply that there are at least two pathways for the repair of DSB DNA, consistent with a model previously proposed by others. The scid cells were also more sensitive to UVC light (254 nm) killing as compared to wild type cells by clonogenic survival. Using a host cell reactivation (HCR) assay to study the nucleotide excision repair (NER) which is the major repair pathway for UV-photoproducts, the results showed that NER in scid cells was not as efficient as CB- 17. This suggests that DNA-PK is involved in NER as well as non-homologous end-joining (NHEJ) DSB repair which is responsible for ionizing radiation sensitivity in scid cells. Repair in scid cells was not totally absent as shown by low dose rate sparing of cell killing after exposure to 137Cs γ-rays at dose rate of 0.6 cGy/h, 1.36 cGy/h, 6 cGy/h as compared to high dose rate at 171 cGy/min, although this phenomenon could be explained partly by proliferation. However, for radiation induced transformation, no significant dose rate effect was seen. A plot of transformation

  5. Atypical depressive symptoms as a predictor of treatment response to exercise in Major Depressive Disorder. (United States)

    Rethorst, Chad D; Tu, Jian; Carmody, Thomas J; Greer, Tracy L; Trivedi, Madhukar H


    Effective treatment of Major Depressive Disorder (MDD) will require the development of alternative treatments and the ability for clinicians to match patients with the treatment likely to produce the greatest effect. We examined atypical depression subtype as a predictor of treatment response to aerobic exercise augmentation in persons with non-remitted MDD. Our results revealed a small-to-moderate effect, particularly in a group assigned to high-dose exercise (semi-partial eta-squared =0.0335, p=0.0735), indicating that those with atypical depression tended to have larger treatment response to exercise. Through this hypothesis-generating analysis, we indicate the need for research to examine depression subtype, along with other demographic, clinical and biological factors as predictors of treatment response to exercise.

  6. Preparation and cellular response of porous A-type carbonated hydroxyapatite nanoceramics

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    Li Bo, E-mail: [Institute of Biomaterials and Living Cell Imaging Technology, School of Metallurgy and Materials Engineering, Chongqing University of Science and Technology, Chongqing 401331 (China) and National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Liao Xiaoling [Institute of Biomaterials and Living Cell Imaging Technology, School of Metallurgy and Materials Engineering, Chongqing University of Science and Technology, Chongqing 401331 (China); Zheng Li [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); He Huawei [Department of Prosthodontics, Beijing Stomatological Hospital, Capital Medical University, Beijing, 100050 (China); Wang Hong [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Fan Hongsong, E-mail: [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China); Zhang Xingdong [National Engineering Research Center for Biomaterials, Sichuan University, Chengdu 610064 (China)


    Microwave sintering using the activated carbon as embedding material was applied in preparation of porous A-type carbonated hydroxyapatite ceramics with nano(nCHA) and submicron (mCHA) structure. By examining the linear shrinkages and the compressive strengths of samples at different temperatures, a suitable microwave sintering temperature was achieved. The microwave sintering method was successfully used to prepare A-type CHA with nano or submicron structure, and the mechanism of the formation of A-type carbonate groups was discussed also. Compared with the samples prepared by the conventional sintering method (mHA), the nCHA bioceramics synthesized by the microwave sintering approach had smaller grain size and more uniform microstructure, and showed a compressive strength similar to the conventional samples. In vitro dissolution test proved that nCHA exhibits better degradation property in comparison to pure HA. Rat osteoblasts were cultured with nCHA, mCHA and mHA to evaluate their biocompatibility, and nCHA showed significant enhancement of cells in attachment, proliferation and differentiation. In conclusion, carbonate groups can be easily introduced to HA crystal structure using the activated carbon as embedding material, and microwave sintering is an effective and simple method in preparing A-type CHA with a nanostructure. Results from this in vitro biological study suggest that porous A-type carbonated hydroxyapatite nanoceramics may be a much better candidate for clinical use in terms of bioactivity. - Highlights: Black-Right-Pointing-Pointer We prepared porous A-type carbonated hydroxyapatite nanoceramics with microwave sintering. Black-Right-Pointing-Pointer We examined physico-chemical characterization and osteoblast response. Black-Right-Pointing-Pointer The nanoceramics have a comparable compressive strength to samples with conventional sintering method. Black-Right-Pointing-Pointer The nanoceramics enhance degradation property, osteoblast

  7. Chronic Cluster Headache with an Atypical Presentation and Treatment Response

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    Telma Santos


    Full Text Available The management of cluster headache (CH may be challenging. We report a 50-year-old male with recurrent attacks of dull and severe unilateral periorbital pain, lasting 30–45 minutes, twice a day, exclusively during sleep, and accompanied by ipsilateral rhinorrhea and lacrimation. The pain switched sides within every attack. CH treatment was initiated but the patient maintained recurrence rates compatible with chronic CH, even after increasing verapamil to 460 mg/day. Afterwards we decided to add lithium (800 mg/day. With this treatment the severity and recurrence of CH substantially decreased, despite the patient’s autonomous decision to take lithium only during the acute phase of the cluster. The exclusively alternating location and the excellent response to short cycles of lithium represent two unique features of CH.

  8. Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab

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    Alexander G. Raufi


    Full Text Available Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS. Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A lupus nephritis and hypocomplementemia.

  9. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab (United States)

    Raufi, Alexander G.; Scott, Shruti; Darwish, Omar; Harley, Kevin; Kahlon, Kanwarpal; Desai, Sheetal; Lu, Yuxin; Tran, Minh-Ha


    Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.

  10. Replacement

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    S. Radhakrishnan


    Full Text Available The fishmeal replaced with Spirulina platensis, Chlorella vulgaris and Azolla pinnata and the formulated diet fed to Macrobrachium rosenbergii postlarvae to assess the enhancement ability of non-enzymatic antioxidants (vitamin C and E, enzymatic antioxidants (superoxide dismutase (SOD and catalase (CAT and lipid peroxidation (LPx were analysed. In the present study, the S. platensis, C. vulgaris and A. pinnata inclusion diet fed groups had significant (P < 0.05 improvement in the levels of vitamins C and E in the hepatopancreas and muscle tissue. Among all the diets, the replacement materials in 50% incorporated feed fed groups showed better performance when compared with the control group in non-enzymatic antioxidant activity. The 50% fishmeal replacement (best performance diet fed groups taken for enzymatic antioxidant study, in SOD, CAT and LPx showed no significant increases when compared with the control group. Hence, the present results revealed that the formulated feed enhanced the vitamins C and E, the result of decreased level of enzymatic antioxidants (SOD, CAT and LPx revealed that these feeds are non-toxic and do not produce any stress to postlarvae. These ingredients can be used as an alternative protein source for sustainable Macrobrachium culture.

  11. Phosphorylation-independent activity of atypical response regulators of Helicobacter pylori. (United States)

    Schär, Jennifer; Sickmann, Albert; Beier, Dagmar


    The genome of the gastric pathogen Helicobacter pylori harbors a remarkably low number of regulatory genes, including three and five open reading frames encoding two-component histidine kinases and response regulators, respectively, which are putatively involved in transcriptional regulation. Two of the response regulator genes, hp1043 and hp166, proved to be essential for cell growth, and inactivation of the response regulator gene hp1021 resulted in a severe growth defect, as indicated by a small-colony phenotype. The sequences of the receiver domains of response regulators HP1043 and HP1021 differ from the consensus sequence of the acidic pocket of the receiver domain which is involved in the phosphotransfer reaction from the histidine kinase to the response regulator. Using a genetic complementation system, we demonstrated that the function of response regulator HP166, which is essential for cell growth, can be provided by a mutated derivative carrying a D52N substitution at the site of phosphorylation. We found that the atypical receiver sequences of HP1043 and HP1021 are not crucial for the function of these response regulators. Phosphorylation of the receiver domains of HP1043 and HP1021 is not needed for response regulator function and may not occur at all. Thus, the phosphorylation-independent action of these regulators differs from the well-established two-component paradigm.

  12. Atypical Chemokine Receptors and Their Roles in the Resolution of the Inflammatory Response (United States)

    Bonecchi, Raffaella; Graham, Gerard J.


    Chemokines and their receptors are key mediators of the inflammatory process regulating leukocyte extravasation and directional migration into inflamed and infected tissues. The control of chemokine availability within inflamed tissues is necessary to attain a resolving environment and when this fails chronic inflammation ensues. Accordingly, vertebrates have adopted a number of mechanisms for removing chemokines from inflamed sites to help precipitate resolution. Over the past 15 years, it has become apparent that essential players in this process are the members of the atypical chemokine receptor (ACKR) family. Broadly speaking, this family is expressed on stromal cell types and scavenges chemokines to either limit their spatial availability or to remove them from in vivo sites. Here, we provide a brief review of these ACKRs and discuss their involvement in the resolution of inflammatory responses and the therapeutic implications of our current knowledge. PMID:27375622

  13. Gender Atypicality and Anxiety Response to Social Interaction Stress in Homosexual and Heterosexual Men. (United States)

    Jacobson, Roi; Cohen, Hagit; Diamond, Gary M


    Gender non-conforming behavior and a homosexual sexual orientation have both been linked to higher levels of anxiety. This study examined the independent and interactive effects of gender atypicality and sexual orientation on levels of state anxiety immediately following a stressful social interaction task among a sample of homosexual and heterosexual Israeli men (n = 36). Gender atypicality was measured via both self-report and observer ratings. State anxiety was measured via both self-report immediately subsequent to the stressful social interaction task and pre- to post task changes in salivary cortisol. Results showed that self-reported gender atypicality and heterosexual sexual orientation predicted higher levels of self-reported social interaction anxiety, but not changes in cortisol. There were no sexual orientation by gender behavior interactions and there were no significant effects for observer rated gender atypicality. These findings suggest that gender atypicality, not homosexuality, place individuals at risk for increased anxiety.

  14. The Atypical Response Regulator AtvR Is a New Player in Pseudomonas aeruginosa Response to Hypoxia and Virulence. (United States)

    Kaihami, Gilberto Hideo; Breda, Leandro Carvalho Dantas; de Almeida, José Roberto Fogaça; de Oliveira Pereira, Thays; Nicastro, Gianlucca Gonçalves; Boechat, Ana Laura; de Almeida, Sandro Rogério; Baldini, Regina Lúcia


    Two-component systems are widespread in bacteria, allowing adaptation to environmental changes. The classical pathway is composed of a histidine kinase that phosphorylates an aspartate residue in the cognate response regulator (RR). RRs lacking the phosphorylatable aspartate also occur, but their function and contribution during host-pathogen interactions are poorly characterized. AtvR (PA14_26570) is the only atypical response regulator with a DNA-binding domain in the opportunistic pathogen Pseudomonas aeruginosa Macrophage infection with the atvR mutant strain resulted in higher levels of tumor necrosis factor alpha secretion as well as increased bacterial clearance compared to those for macrophages infected with the wild-type strain. In an acute pneumonia model, mice infected with the atvR mutant presented increased amounts of proinflammatory cytokines, increased neutrophil recruitment to the lungs, reductions in bacterial burdens, and higher survival rates in comparison with the findings for mice infected with the wild-type strain. Further, several genes involved in hypoxia/anoxia adaptation were upregulated upon atvR overexpression, as seen by high-throughput transcriptome sequencing (RNA-Seq) analysis. In addition, atvR was more expressed in hypoxia in the presence of nitrate and required for full expression of nitrate reductase genes, promoting bacterial growth under this condition. Thus, AtvR would be crucial for successful infection, aiding P. aeruginosa survival under conditions of low oxygen tension in the host. Taken together, our data demonstrate that the atypical response regulator AtvR is part of the repertoire of transcriptional regulators involved in the lifestyle switch from aerobic to anaerobic conditions. This finding increases the complexity of regulation of one of the central metabolic pathways that contributes to Pseudomonas ubiquity and versatility. Copyright © 2017 American Society for Microbiology.

  15. Atypical signaling and functional desensitization response of MAS receptor to peptide ligands. (United States)

    Tirupula, Kalyan C; Desnoyer, Russell; Speth, Robert C; Karnik, Sadashiva S


    MAS is a G protein-coupled receptor (GPCR) implicated in multiple physiological processes. Several physiological peptide ligands such as angiotensin-(1-7), angiotensin fragments and neuropeptide FF (NPFF) are reported to act on MAS. Studies of conventional G protein signaling and receptor desensitization upon stimulation of MAS with the peptide ligands are limited so far. Therefore, we systematically analyzed G protein signals activated by the peptide ligands. MAS-selective non-peptide ligands that were previously shown to activate G proteins were used as controls for comparison on a common cell based assay platform. Activation of MAS by the non-peptide agonist (1) increased intracellular calcium and D-myo-inositol-1-phosphate (IP1) levels which are indicative of the activation of classical Gαq-phospholipase C signaling pathways, (2) decreased Gαi mediated cAMP levels and (3) stimulated Gα12-dependent expression of luciferase reporter. In all these assays, MAS exhibited strong constitutive activity that was inhibited by the non-peptide inverse agonist. Further, in the calcium response assay, MAS was resistant to stimulation by a second dose of the non-peptide agonist after the first activation has waned suggesting functional desensitization. In contrast, activation of MAS by the peptide ligand NPFF initiated a rapid rise in intracellular calcium with very weak IP1 accumulation which is unlike classical Gαq-phospholipase C signaling pathway. NPFF only weakly stimulated MAS-mediated activation of Gα12 and Gαi signaling pathways. Furthermore, unlike non-peptide agonist-activated MAS, NPFF-activated MAS could be readily re-stimulated the second time by the agonists. Functional assays with key ligand binding MAS mutants suggest that NPFF and non-peptide ligands bind to overlapping regions. Angiotensin-(1-7) and other angiotensin fragments weakly potentiated an NPFF-like calcium response at non-physiological concentrations (≥100 µM). Overall, our data suggest

  16. Neuroinvasive West Nile Infection Elicits Elevated and Atypically Polarized T Cell Responses That Promote a Pathogenic Outcome.

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    Eddie A James


    Full Text Available Most West Nile virus (WNV infections are asymptomatic, but some lead to neuroinvasive disease with symptoms ranging from disorientation to paralysis and death. Evidence from animal models suggests that neuroinvasive infections may arise as a consequence of impaired immune protection. However, other data suggest that neurologic symptoms may arise as a consequence of immune mediated damage. We demonstrate that elevated immune responses are present in neuroinvasive disease by directly characterizing WNV-specific T cells in subjects with laboratory documented infections using human histocompatibility leukocyte antigen (HLA class II tetramers. Subjects with neuroinvasive infections had higher overall numbers of WNV-specific T cells than those with asymptomatic infections. Independent of this, we also observed age related increases in WNV-specific T cell responses. Further analysis revealed that WNV-specific T cell responses included a population of atypically polarized CXCR3+CCR4+CCR6- T cells, whose presence was highly correlated with neuroinvasive disease. Moreover, a higher proportion of WNV-specific T cells in these subjects co-produced interferon-γ and interleukin 4 than those from asymptomatic subjects. More globally, subjects with neuroinvasive infections had reduced numbers of CD4+FoxP3+ Tregs that were CTLA4 positive and exhibited a distinct upregulated transcript profile that was absent in subjects with asymptomatic infections. Thus, subjects with neuroinvasive WNV infections exhibited elevated, dysregulated, and atypically polarized responses, suggesting that immune mediated damage may indeed contribute to pathogenic outcomes.

  17. Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern (United States)

    Peralta Roselló, Carmen; Baltar Martín, José María; Castillo Eraso, Lorena; de Álvaro Moreno, Fernando; Martínez Vea, Alberto; Visus-Fernández de Manzanos, María Teresa


    In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14 ± 2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol. PMID:28025630

  18. Children's responses to social atypicality among group members - advantages of a contextualized social developmental account. (United States)

    Abrams, Dominic; Rutland, Adam; Palmer, Sally B; Purewal, Kiran


    Abrams, Rutland, Palmer, Ferrell, and Pelletier (2014) showed that better second-order mental state understanding facilitates 6-7-year-olds' ability to link a partially disloyal child's atypicality to inclusive or exclusive reactions by in-group or outgroup members. This finding is interpreted in terms of predictions from the developmental subjective group dynamics model. We respond to thoughtful commentaries by Rhodes and Chalik, Patterson, and Rakoczy. Children face a significant developmental challenge in becoming able to recognize and interpret social atypicality in intergroup contexts. Researching that ability to contextualize judgements raises new questions about the nature of peer inclusion and exclusion, about children's social cognition, and about the way that social cognitive development and social experience combine. Rather than individual-focused cognition taking priority over category-based cognition, we argue the two become more systematically integrated during development. We note that loyalty is but one example of typicality, and we also consider the role of more advanced perspective taking among older children, and the role of multiple classification skill among younger children, as well as potential implications for intervention to reduce peer victimization and prejudice.


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    Vijay Vinay


    Full Text Available Antipsychotics are a group of drugs commonly but not exclusively used to treat psychosis. Antipsychotic agents are grouped in two categories: Typical and Atypical antipsychotics. The first antipsychotic was chlorpromazine, which was developed as a surgical anesthetic. The first atypical anti-psychotic medication, clozapine, was discovered in the 1950s, and introduced in clinical practice in the 1970s. Both typical and atypical antipsychotics are effective in reducing positive and negative symptoms of schizophrenia. Blockade of D2 receptor in mesolimbic pathway is responsible for antipsychotic action. Typical antipsychotics are not particularly selective and also block Dopamine receptors in the mesocortical pathway, tuberoinfundibular pathway, and the nigrostriatal pathway. Blocking D2 receptors in these other pathways is thought to produce some of the unwanted side effects. Atypical antipsychotics differ from typical psychotics in their "limbic-specific" dopamine type 2 (D2-receptor binding and high ratio of serotonin type 2 (5-HT2-receptor binding to D2. Atypical antipsychotics are associated with a decreased capacity to cause EPSs, TD, narcoleptic malignant syndrome, and hyperprolactinemia. Atypical antipsychotic agents were developed in response to problems with typical agents, including lack of efficacy in some patients, lack of improvement in negative symptoms, and troublesome adverse effects, especially extrapyramidal symptoms (EPSs and tardive dyskinesia (TD.

  20. Atypical Depression (United States)

    ... satisfaction and control in your life Help ease depression symptoms such as hopelessness and anger As part of your treatment, it's important to also address other conditions that often accompany atypical depression, in particular anxiety and drug or alcohol use, ...

  1. Atypical Moles (United States)

    ... 2013 Annual Meeting 2013 OMED Schedule Needs Assessments Speaker Bios Meeting Feedback 2014 Midyear Meeting Attestation 2014 ... not an atypical mole. Still, there is potentially great benefit in identifying persons at increased risk of ...

  2. Life-threatening pregnancy-associated atypical haemolytic uraemic syndrome and its response to eculizumab. (United States)

    Gately, Ryan; San, Aye; Kurtkoti, Jagadeesh; Parnham, Alan


    Pregnancy-associated atypical haemolytic uraemic syndrome (P-aHUS) is a rare, potentially lethal condition that can complicate pregnancy in up to 1 in 25 000 cases. Without prompt diagnosis and initiation of appropriate treatment, this condition can lead to disastrous consequences for both mother and child. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is often difficult to establish the correct diagnosis in a timely manner. Recently, the terminal complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy HUS; however, its use in P-aHUS is limited to isolated case reports. Here, we present a case of fulminant P-aHUS in the postpartum period that was successfully treated with eculizumab resulting in significant recovery of renal function.

  3. Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern

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    Camino García Monteavaro


    Full Text Available In patients with atypical hemolytic uremic syndrome (aHUS, complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14±2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol.

  4. Some Direct and Generalized Effects of Replacing an Autistic Man's Echolalia with Correct Responses to Questions. (United States)

    McMorrow, Martin J.; Foxx, R. M.


    The use of operant procedures was extended to decrease immediate echolalia and increase appropriate responding to questions of a 21-year-old autistic man. Multiple baseline designs demonstrated that echolalia was rapidly replaced with correct stimulus-specific responses. A variety of generalized improvements were observed in verbal responses to…

  5. Oxidative stress and antioxidant response in fibroblasts from Werner and atypical Werner syndromes. (United States)

    Seco-Cervera, Marta; Spis, Marta; García-Giménez, José Luis; Ibañez-Cabellos, José Santiago; Velázquez-Ledesma, Ana; Esmorís, Isabel; Bañuls, Sergio; Pérez-Machado, Giselle; Pallardó, Federico V


    Werner Syndrome (WS, ICD-10 E34.8, ORPHA902) and Atypical Werner Syndrome (AWS, ICD-10 E34.8, ORPHA79474) are very rare inherited syndromes characterized by premature aging. While approximately 90% of WS individuals have any of a range of mutations in theWRN gene, there exists a clinical subgroup in which the mutation occurs in the LMNA/C gene in heterozygosity. Although both syndromes exhibit an age-related pleiotropic phenotype, AWS manifests the onset of the disease during childhood, while major symptoms in WS appear between the ages of 20 and 30. To study the molecular mechanisms of progeroid diseases provides a useful insight into the normal aging process. Main changes found were the decrease in Cu/Zn and Mn SOD activities in the three cell lines. In AWS, both mRNA SOD and protein levels were also decreased. Catalase and glutathione peroxidases decrease, mainly in AWS. Glutaredoxin (Grx) and thioredoxin (Trx) protein expression was lower in the three progeroid cell lines. Grx and Trx were subjected to post-transcriptional regulation, because protein expression was reduced although mRNA levels were not greatly affected in WS. Low antioxidant defense and oxidative stress occur simultaneously in these rare genetic instability disorders at the onset of progeroid disease.

  6. Atypical Cities (United States)

    DiJulio, Betsy


    In this creative challenge, Surrealism and one-point perspective combine to produce images that not only go "beyond the real" but also beyond the ubiquitous "imaginary city" assignment often used to teach one-point perspective. Perhaps the difference is that in the "atypical cities challenge," an understanding of one-point perspective is a means…

  7. Atypical Optic Neuritis. (United States)

    Gaier, Eric D; Boudreault, Katherine; Rizzo, Joseph F; Falardeau, Julie; Cestari, Dean M


    Classic demyelinative optic neuritis is associated with multiple sclerosis and typically carries a good prognosis for visual recovery. This disorder is well characterized with respect to its presentation and clinical features by baseline data obtained through the optic neuritis treatment trial and numerous other studies. Atypical optic neuritis entails clinical manifestations that deviate from this classic pattern of features. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features to consider include lack of pain, simultaneous or near-simultaneous onset, lack of response to or relapse upon tapering from corticosteroids, or optic nerve head or peripapillary hemorrhages. The most important alternative etiologies to consider and the steps towards their respective diagnostic evaluations are suggested for these atypical features.

  8. Treatment of simple and complex endometrial non-atypical hyperplasia with natural progesterone: response rate to different doses. (United States)

    Marra, Chiara; Penati, Cristina; Ferrari, Luisa; Cantù, Maria Grazia; Bargossi, Lorena; Fruscio, Robert


    The aim of this study is to evaluate the response rate to natural progesterone in non-atypical endometrial hyperplasia and to identify the lowest effective dose. A total of 197 patients of childbearing age with simple or complex hyperplasia were retrospectively identified. The women were treated with a cyclic administration of progesterone at different dosages (100 versus 200 versus 300 mg daily). Endometrial biopsies were performed at 6, 12, 18 months. In comparing progesterone to a regimen of no therapy, a significantly higher remission rate was observed in the progesterone group than in the latter (95 versus 75%, p = 0.05 for simple hyperplasia; 89 versus 35%, p hyperplasia). Out of 60 women with simple hyperplasia, remission was observed in 9/11 (81.8%), 40/41 (97.5%) and 8/8 (100%) patients treated, respectively, with progesterone 100, 200 and 300 mg daily. Out of 72 women with complex hyperplasia, remission was observed in 3/5 (60%), 49/53 (92.4%) and 12/14 (85.7%) patients treated with progesterone 100, 200 and 300 mg daily, respectively. There was no statistically significant difference in the response rate in the two groups, neither with simple nor with complex hyperplasia. In conclusion, progesterone increased the regression rate of both simple and complex hyperplasia.

  9. Immune response to Mycoplasma pneumoniae P1 and P116 in patients with atypical pneumonia analyzed by ELISA

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    Birkelund Svend


    Full Text Available Abstract Background Serology is often used for the diagnosis of Mycoplasma pneumoniae. It is important to identify specific antigens that can distinguish between the presence or absence of antibodies against M. pneumoniae. The two proteins, P116 and P1, are found to be immunogenic. By using these in ELISA it is possible to identify an immune response against M. pneumoniae in serum samples. Results A recombinant protein derived from the P116 protein and one from the P1 protein were used in two ELISA tests, rP116-ELISA and rP1-ELISA. Human serum samples from patients with atypical pneumonia were tested and compared to the results of the complement fixation test. There was a good agreement between the two tests but the rP1-ELISA showed the best discrimination between positive and negative samples. Conclusion Two ELISA tests based on recombinant proteins have been analysed and compared to the complement fixation test results. The two ELISA tests were found suitable for use in serodiagnostics of M. pneumoniae infections. The use of specific antigens eliminates the risk of cross reaction to an immune response against other bacteria.

  10. The role of the atypical kinases ABC1K7 and ABC1K8 in abscisic acid responses

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    Anna eManara


    Full Text Available The ABC1K family of atypical kinases (activity of bc1 complex kinase is represented in bacteria, archaea and eukaryotes. In plants they regulate diverse physiological processes in the chloroplasts and mitochondria, but their precise functions are poorly defined. ABC1K7 and ABC1K8 are probably involved in oxidative stress responses, isoprenyl lipid synthesis and distribution of iron within chloroplasts. Because reactive oxygen species take part in abscisic acid (ABA-mediated processes, we investigated the functions of ABC1K7 and ABC1K8 during germination, stomatal movement and leaf senescence. Both genes were upregulated by ABA treatment and some ABA-responsive physiological processes were affected in abc1k7 and abc1k8 mutants. Germination was more severely affected by ABA, osmotic stress and salt stress in the single and double mutants; the stomatal aperture was smaller in the mutants under standard growth conditions and was not further reduced by exogenous ABA application; ABA-induced senescence symptoms were more severe in the leaves of the single and double mutants compared to wild type leaves. Taken together, our results suggest that ABC1K7 and ABC1K8 might be involved in the cross-talk between ABA and ROS signaling.

  11. Comparative responsiveness of measures of pain and function after total hip replacement

    DEFF Research Database (Denmark)

    Nilsdotter, A K; Roos, Ewa M.; Westerlund, J P


    To compare the responsiveness of the Functional Assessment System (FAS), the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), and the Medical Outcomes Study 36-item Short Form (SF-36) in patients with osteoarthritis (OA) scheduled for total hip replacement....

  12. The Atypical Response Regulator Protein ChxR Has Structural Characteristics and Dimer Interface Interactions That Are Unique within the OmpR/PhoB Subfamily

    Energy Technology Data Exchange (ETDEWEB)

    Hickey, John M.; Lovell, Scott; Battaile, Kevin P.; Hu, Lei; Middaugh, C. Russell; Hefty, P. Scott (Kansas); (HWMRI)


    Typically as a result of phosphorylation, OmpR/PhoB response regulators form homodimers through a receiver domain as an integral step in transcriptional activation. Phosphorylation stabilizes the ionic and hydrophobic interactions between monomers. Recent studies have shown that some response regulators retain functional activity in the absence of phosphorylation and are termed atypical response regulators. The two currently available receiver domain structures of atypical response regulators are very similar to their phospho-accepting homologs, and their propensity to form homodimers is generally retained. An atypical response regulator, ChxR, from Chlamydia trachomatis, was previously reported to form homodimers; however, the residues critical to this interaction have not been elucidated. We hypothesize that the intra- and intermolecular interactions involved in forming a transcriptionally competent ChxR are distinct from the canonical phosphorylation (activation) paradigm in the OmpR/PhoB response regulator subfamily. To test this hypothesis, structural and functional studies were performed on the receiver domain of ChxR. Two crystal structures of the receiver domain were solved with the recently developed method using triiodo compound I3C. These structures revealed many characteristics unique to OmpR/PhoB subfamily members: typical or atypical. Included was the absence of two {alpha}-helices present in all other OmpR/PhoB response regulators. Functional studies on various dimer interface residues demonstrated that ChxR forms relatively stable homodimers through hydrophobic interactions, and disruption of these can be accomplished with the introduction of a charged residue within the dimer interface. A gel shift study with monomeric ChxR supports that dimerization through the receiver domain is critical for interaction with DNA.

  13. Evidence for Atypical Auditory Brainstem Responses in Young Children with Suspected Autism Spectrum Disorders (United States)

    Roth, Daphne Ari-Even; Muchnik, Chava; Shabtai, Esther; Hildesheimer, Minka; Henkin, Yael


    Aim: The aim of this study was to characterize the auditory brainstem responses (ABRs) of young children with suspected autism spectrum disorders (ASDs) and compare them with the ABRs of children with language delay and with clinical norms. Method: The ABRs of 26 children with suspected ASDs (21 males, five females; mean age 32.5 mo) and an age-…

  14. Atypical Brain Responses to Sounds in Children with Specific Language and Reading Impairments (United States)

    McArthur, Genevieve; Atkinson, Carmen; Ellis, Danielle


    This study tested if children with specific language impairment (SLI) or children with specific reading disability (SRD) have abnormal brain responses to sounds. We tested 6- to 12-year-old children with SLI (N = 19), children with SRD (N = 55), and age-matched controls (N = 36) for their passive auditory event-related potentials (ERPs) to tones,…

  15. Association between Ghrelin gene (GHRL polymorphisms and clinical response to atypical antipsychotic drugs in Han Chinese schizophrenia patients

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    Yang Yongfeng


    Full Text Available Abstract Background Ghrelin (GHRL is a pivotal peptide regulator of food intake, energy balance, and body mass. Weight gain (WG is a common side effect of the atypical antipsychotics (AAPs used to treat schizophrenia (SZ. Ghrelin polymorphisms have been associated with pathogenic variations in plasma lipid concentrations, blood pressure, plasma glucose, and body mass index (BMI. However, it is unclear whether GHRL polymorphisms are associated with WG due to AAPs. Furthermore, there is no evidence of an association between GHRL polymorphisms and SZ or the therapeutic response to AAPs. We explored these potential associations by genotyping GHRL alleles in SZ patients and controls. We also examined the relation between these SNPs and changes in metabolic indices during AAP treatment in SZ subgroups distinguished by high or low therapeutic response. Methods Four SNPs (Leu72Met, -501A/C, -604 G/A, and -1062 G > C were genotyped in 634 schizophrenia patients and 606 control subjects. Results There were no significant differences in allele frequencies, genotype distributions, or the distributions of two SNP haplotypes between SZ patients and healthy controls (P > 0.05. There was also no significant difference in symptom reduction between genotypes after 8 weeks of AAP treatment as measured by positive and negative symptom scale scores (PANSS. However, the -604 G/A polymorphism was associated with a greater BMI increase in response to AAP administration in both APP responders and non-responders as distinguished by PANSS score reduction (P P Conclusions These four GHRL gene SNPs were not associated with SZ in this Chinese Han population. The -604 G/A polymorphism was associated with significant BW and BMI increases during AAP treatment. Patients exhibiting higher WG showed greater improvements in positive and negative symptoms than patients exhibiting lower weight gain or weight loss.

  16. Atypical Cutaneous Sporotrichosis in an Immunocompetent Adult: Response to Potassium Iodide. (United States)

    Gandhi, Nikita; Chander, Ram; Jain, Arpita; Sanke, Sarita; Garg, Taru


    Cutaneous sporotrichosis, also known as "Rose Gardener's disease," caused by dimorphic fungus Sporothrix schenkii, is usually characterized by indolent nodular or nodulo-ulcerative lesions arranged in a linear pattern. We report bizarre nonlinear presentation of Sporotrichosis, in an immunocompetent adult occurring after a visit to Amazon rain forest, speculating infection with more virulent species of Sporothrix. The diagnosis was reached with the help of periodic acid-Schiff positive yeast cells and cigar shaped bodies seen in skin biopsy along with the therapeutic response to potassium iodide.

  17. Atypical cutaneous sporotrichosis in an immunocompetent adult: Response to potassium iodide

    Directory of Open Access Journals (Sweden)

    Nikita Gandhi


    Full Text Available Cutaneous sporotrichosis, also known as “Rose Gardener's disease,” caused by dimorphic fungus Sporothrix schenkii, is usually characterized by indolent nodular or nodulo-ulcerative lesions arranged in a linear pattern. We report bizarre nonlinear presentation of Sporotrichosis, in an immunocompetent adult occurring after a visit to Amazon rain forest, speculating infection with more virulent species of Sporothrix. The diagnosis was reached with the help of periodic acid-Schiff positive yeast cells and cigar shaped bodies seen in skin biopsy along with the therapeutic response to potassium iodide.

  18. A Case Report on Juvenile Neuromyelitis Optica: Early Onset, Long Remission Period, and Atypical Treatment Response. (United States)

    Elpers, Christiane; Gross, Catharina C; Fiedler, Barbara; Meuth, Sven G; Kurlemann, Gerhard


    Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease of the central nervous system and preferentially targets the optic nerves and spinal cord. NMO is rare in children and clinical course of the disease is highly variable as described in studies. Here, we present a case report of a young girl presenting with a rare course of pediatric NMO with an early disease onset at the age of 12 years, a relapse free interval of 4 years, evidence of NMO immunoglobulin G (IgG) and an unusual response against immunosuppressive therapy. The aim of this report is to highlight the potentially long remission period between relapses complicating proper diagnosis despite well defined diagnostic criteria. In addition, we want to encourage the use of rituximab in pediatric NMO, although larger cohorts are warranted to establish B cell depleting therapies in juvenile NMO.

  19. Atypically high insulin responses to some foods relate to sugars and satiety. (United States)

    Trout, David L; Hallfrisch, Judith; Behall, Kay M


    Much research has focused on how the glycemic index (GI) of the diets of healthy people relates to long-term risk for coronary heart disease, stroke, and non-insulin dependent diabetes. Low-GI diets appear to produce some of their beneficial effects largely by moderating insulinemic responses to meals. Wolever and Bolognesi (1996) have derived a formula for predicting the insulinemic index (II) from the GI for starchy foods. Using data from Holt et al. (1995, 1997) on a wide variety of common foods, we have examined differences between the observed II and GI-based estimates of the II. These differences were found to correlate negatively with satiety index ratings and positively with contents of total sugars. We suggest that the aforementioned method of measuring and expressing the relation between the GI and the II may prove useful in exploring how various components and sensory properties of food may affect hunger and energy intake.

  20. Prevalence of hematological abnormalities in patients with Sheehan's syndrome: response to replacement of glucocorticoids and thyroxine. (United States)

    Laway, Bashir Ahmad; Mir, Shahnaz Ahmad; Bashir, Mir Iftikhar; Bhat, Javid Rasool; Samoon, Jeelani; Zargar, Abdul Hamid


    Anemia and other hematological abnormalities are common in patients with Sheehan's syndrome. The response of these abnormalities to replacement of thyroxine and glucocorticoids is not clear. The aim of the present study was to document the profile of hematological abnormalities and response to treatment in patients with Sheehan's syndrome. Forty patients of Sheehan's syndrome and an equal number of age and parity matched healthy controls were studied for prevalence of hematological abnormalities. Hemoglobin concentration, hematocrit, red cell, white cell and platelet count were significantly decreased in patients with Sheehan's syndrome compared to controls. Frequency of anemia, leucopenia, thrombocytopenia and pancytopenia was significantly higher in these patients compared to controls. After achieving euthyroid and eucortisol state, there was a complete recovery of these hematological abnormalities. We conclude that anemia and other cytopenias are common in patients with Sheehan's syndrome and replacement with thyroxine and glucocorticoids results in complete recovery of these abnormalities.

  1. Complement factor H-associated atypical hemolytic uremic syndrome in monozygotic twins: Concordant presentation, discordant response to treatment

    NARCIS (Netherlands)

    J.C. Davin; K.H. Olie; R. Verlaak; F. Horuz; S. Florquin; J.J. Weening; J.W. Groothoff; L. Strain; T.H.J. Goodship


    Hemolytic uremic syndrome not associated with diarrhea (diarrhea negative, atypical) is less common than the diarrhea-positive typical form, but frequently results In end-stage renal failure. Although there are anecdotal cases of successful treatment with fresh frozen plasma alone, the value of this

  2. Endoplasmic reticulum stress in complex atypical hyperplasia as a possible predictor of occult carcinoma and progestin response. (United States)

    Tierney, Katherine E; Ji, Lingyun; Dralla, Shannon S; Yoo, Eunjeong; Yessaian, Annie; Pham, Huyen Q; Roman, Lynda; Sposto, Richard; Mhawech-Fauceglia, Paulette; Lin, Yvonne G


    Glucose-regulated protein (GRP)-78, the key regulator of endoplasmic reticulum (ER) stress, is associated with endometrial cancer (EC) development and progression. However, its role in the continuum from complex atypical hyperplasia (CAH) to EC is unknown and the focus of this study.

  3. Breakfast replacement with a low-glycaemic response liquid formula in patients with type 2 diabetes : a randomised clinical trial

    NARCIS (Netherlands)

    Stenvers, Dirk J; Schouten, Lydia J; Jurgens, Jordy; Endert, Erik; Kalsbeek, A.; Fliers, Eric; Bisschop, Peter H


    Low-glycaemic index diets reduce glycated Hb (HbA1c) in patients with type 2 diabetes, but require intensive dietary support. Using a liquid meal replacement with a low glycaemic response (GR) may be an alternative dietary approach. In the present study, we investigated whether breakfast replacement

  4. Short communication: Lactational responses to palmitic acid supplementation when replacing soyhulls or dry ground corn. (United States)

    de Souza, J; Preseault, C L; Lock, A L


    The objective of this study was to evaluate the response of mid-lactation dairy cows to a palmitic acid (C16:0)-enriched fatty acid supplement when replacing soyhulls or dry ground corn in the diet. Twenty-four multiparous Holstein cows (182 ± 60 d in milk; mean ± SD) were blocked by preliminary 3.5% fat-corrected milk and randomly assigned to treatment sequence in a replicated 3 × 3 Latin square design with 21-d periods. Treatments consisted of a control diet containing no supplemental fat (CON), and 2 C16:0-enriched fatty acid-supplemented treatments (PA; BergaFat F100, Berg & Schmidt, Hanover, Germany) as a replacement for either soyhulls (PA-SH) or dry ground corn (PA-CG). The C16:0-enriched supplement was fed at 1.5% of diet dry matter. The PA treatments did not affect dry matter intake, but PA-SH increased dry matter intake by 1.4 kg/d compared with PA-CG. The PA treatments did not affect milk yield; however, PA-SH increased milk yield by 2.4 kg/d compared with PA-CG. The PA treatments tended to decrease milk protein content (3.12 vs. 3.15%). In contrast, PA-SH increased milk protein content (3.14 vs. 3.10%) and milk protein yield (1.27 vs. 1.19 kg/d) compared with PA-CG. The PA treatments increased milk fat concentration (3.68 vs. 3.55%) and milk fat yield (1.46 vs. 1.38 kg/d). The increase in milk fat yield with PA treatments was due to the increase in the yield of 16-carbon fatty acid in milk fat. Furthermore, PA-SH tended to increase yield of de novo fatty acids and yield of 16-carbon fatty acids compared with PA-CG. The PA treatments tended to increase feed efficiency (3.5% fat-corrected milk/dry matter intake) compared with CON (1.51 vs. 1.46). The PA-SH treatment tended to increase insulin concentration compared with PA-CG (1.58 vs. 1.49 μg/L) and PA treatments increased nonesterified fatty acids compared with CON (110 vs. 99 μEq/L). Overall, PA treatments improved feed efficiency and increased milk fat yield and the response to the C16:0-enriched

  5. Histological characterization of periprosthetic tissue responses for metal-on-metal hip replacement. (United States)

    Phillips, Eual A; Klein, Gregg R; Cates, Harold E; Kurtz, Steven M; Steinbeck, Marla


    The histology of periprosthetic tissue from metal-on-metal (MOM) hip devices has been characterized using a variety of methods. The purpose of this study was to compare and evaluate the suitability of two previously developed aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL) scoring systems for periprosthetic hip tissue responses retrieved from MOM total hip replacement (THR) systems revised for loosening. Two ALVAL scoring systems (Campbell and Oxford) were used to perform histological analyses of soft tissues from 17 failed MOM THRs. The predominant reactions for this patient cohort were macrophage infiltration and necrosis, with less than half of the patients (41%) showing a significant lymphocytic response or a high ALVAL reaction (6%). Other morphological changes varied among patients and included hemosiderin accumulation, cartilage formation, and heterotopic ossification. Both scoring systems are useful for correlating macrophage and lymphocyte responses and for comparison with the other; however, given the diversity and variability of the current responses, the Oxford-ALVAL system is more suitable for scoring tissues from MOM THR patients revised for loosening. It is important that standardized methods of scoring MOM tissue responses be used consistently so multiple study results can be compared and a consensus can be generated.

  6. Technical Approach Determines Inflammatory Response after Surgical and Transcatheter Aortic Valve Replacement.

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    Gabor Erdoes

    Full Text Available To investigate the periprocedural inflammatory response in patients with isolated aortic valve stenosis undergoing surgical aortic valve replacement (SAVR or transcatheter aortic valve implantation (TAVI with different technical approaches.Patients were prospectively allocated to one of the following treatments: SAVR using conventional extracorporeal circulation (CECC, n = 47 or minimized extracorporeal circulation (MECC, n = 15, or TAVI using either transapical (TA, n = 15 or transfemoral (TF, n = 24 access. Exclusion criteria included infection, pre-procedural immunosuppressive or antibiotic drug therapy and emergency indications. We investigated interleukin (IL-6, IL-8, IL-10, human leukocyte antigen (HLA-DR, white blood cell count, high-sensitivity C-reactive protein (hs-CRP and soluble L-selectin (sCD62L levels before the procedure and at 4, 24, and 48 h after aortic valve replacement. Data are presented for group interaction (p-values for inter-group comparison as determined by the Greenhouse-Geisser correction.SAVR on CECC was associated with the highest levels of IL-8 and hs-CRP (p<0.017, and 0.007, respectively. SAVR on MECC showed the highest descent in levels of HLA-DR and sCD62L (both p<0.001 in the perioperative period. TA-TAVI showed increased intraprocedural concentration and the highest peak of IL-6 (p = 0.017. Significantly smaller changes in the inflammatory markers were observed in TF-TAVI.Surgical and interventional approaches to aortic valve replacement result in inflammatory modulation which differs according to the invasiveness of the procedure. As expected, extracorporeal circulation is associated with the most marked pro-inflammatory activation, whereas TF-TAVI emerges as the approach with the most attenuated inflammatory response. Factors such as the pre-treatment patient condition and the extent of myocardial injury also significantly affect inflammatory biomarker patterns. Accordingly, TA-TAVI is to be classified not

  7. Apolipoprotein E-specific innate immune response in astrocytes from targeted replacement mice

    Directory of Open Access Journals (Sweden)

    Montine Thomas J


    Full Text Available Abstract Background Inheritance of the three different alleles of the human apolipoprotein (apo E gene (APOE are associated with varying risk or clinical outcome from a variety of neurologic diseases. ApoE isoform-specific modulation of several pathogenic processes, in addition to amyloid β metabolism in Alzheimer's disease, have been proposed: one of these is innate immune response by glia. Previously we have shown that primary microglia cultures from targeted replacement (TR APOE mice have apoE isoform-dependent innate immune activation and paracrine damage to neurons that is greatest with TR by the ε4 allele (TR APOE4 and that derives from p38 mitogen-activated protein kinase (p38MAPK activity. Methods Primary cultures of TR APOE2, TR APOE3 and TR APOE4 astrocytes were stimulated with lipopolysaccharide (LPS. ApoE secretion, cytokine production, and nuclear factor-kappa B (NF-κB subunit activity were measured and compared. Results Here we showed that activation of primary astrocytes from TR APOE mice with LPS led to TR APOE-dependent differences in cytokine secretion that were greatest in TR APOE2 and that were associated with differences in NF-κB subunit activity. Conclusion Our results suggest that LPS activation of innate immune response in TR APOE glia results in opposing outcomes from microglia and astrocytes as a result of TR APOE-dependent activation of p38MAPK or NF-κB signaling in these two cell types.

  8. Improved Dynamic Postural Task Performance without Improvements in Postural Responses: The Blessing and the Curse of Dopamine Replacement

    Directory of Open Access Journals (Sweden)

    K. B. Foreman


    Full Text Available Introduction. Dopamine-replacement medications may improve mobility while not improving responses to postural challenges and could therefore increase fall risk. The purpose of this study was to measure reactive postural responses and gait-related mobility of patients with PD during ON and OFF medication conditions. Methods. Reactive postural responses to the Pull Test and performance of the Functional Gait Assessment (FGA were recorded from 15 persons with PD during ON and OFF medication conditions. Results. Persons with PD demonstrated no significant difference in the reactive postural responses between medication conditions but demonstrated significantly better performance on the FGA when ON medications compared to OFF. Discussion/Conclusion. Dopamine-replacement medications alone may improve gait-related mobility without improvements in reactive postural responses and therefore could result in iatrogenic increases in fall risk. Rehabilitation providers should be aware of the side effects and limitations of medication treatment and implement interventions to improve postural responses.

  9. Atypical manifestations of leptospirosis. (United States)

    Rajapakse, Senaka; Rodrigo, Chaturaka; Balaji, Krishan; Fernando, Sumadhya Deepika


    Leptospirosis is an illness with a wide spectrum of clinical manifestations and severe illness affects nearly all organ systems. Serious and potentially life-threatening clinical manifestations of acute leptospirosis are caused by both direct tissue invasion by spirochaetes and by the host immune responses. In its severe form, leptospirosis can cause multi-organ dysfunction and death in a matter of days. Therefore it is critical to suspect and recognize the disease early, in order to initiate timely treatment. While the classical presentation of the disease is easily recognized by experienced clinicians practising in endemic regions, rarer manifestations can be easily missed. In this systematic review, we summarize the atypical manifestations reported in literature in patients with confirmed leptospirosis. Awareness of these unusual manifestations would hopefully guide clinicians towards early diagnosis.

  10. Atypical Mucocutaneous Leishmaniasis Caused by Leishmania braziliensis in an Acquired Immunodeficiency Syndrome Patient: T-cell Responses and Remission of Lesions Associated with Antigen Immunotherapy

    Directory of Open Access Journals (Sweden)

    Da-Cruz Alda M


    Full Text Available An atypical case of acquired immunodeficiency syndrome-associated mucocutaneous lesions due to Leishmania braziliensis is described. Many vacuolated macrophages laden with amastigote forms of the parasite were found in the lesions. Leishmanin skin test and serology for leishmaniasis were both negative. The patient was resistant to therapy with conventional drugs (antimonial and amphotericin B. Interestingly, remission of lesions was achieved after an alternative combined therapy of antimonial associated with immunotherapy (whole promastigote antigens. Peripheral blood mononuclear cells were separated and stimulated in vitro with Leishmania antigens to test the lymphoproliferative responses (LPR. Before the combined immunochemotherapy, the LPR to leishmanial antigens was negligible (stimulation index - SI=1.4. After the first course of combined therapy it became positive (SI=4.17. The antigen responding cells were predominantly T-cells (47.5% most of them with CD8+ phenotype (33%. Very low CD4+ cells (2.2% percentages were detected. The increased T-cell responsiveness to leishmanial antigens after combined therapy was accompanied by interferon-g (IFN-g production as observed in the cell culture supernatants. In this patient, healing of the leishmaniasis lesions was associated with the induction of a specific T-cell immune response, characterized by the production of IFN-g and the predominance of the CD8+ phenotype among the Leishmania-reactive T-cells.


    Ristovski, Ljiljana; Milankov, Olgica; Vislavski, Melanija; Savić, Radojica; Bjelica, Milena


    Kawasaki disease is an acute vasculitis which occurs primarily in children under the age of 5. The etiology of the disease is still unknown. Diagnostic criteria for Kawasaki disease are fever and at least four of the five additional clinical signs. Incomplete Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. The diagnosis of incomplete Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients. CASE REPORT. In this report we present a case of atypical Kawasaki disease in a 3.5-month-old infant. As soon as the diagnosis was made, the patient received high doses of intravenous immunoglobulin, with the initial introduction of ibuprofen, then aspirin with a good clinical response. Due to the presence of aneurysm of coronary arteries, further therapy involved aspirin and clopidogrel over the following 3 months, and then only aspirin for 2 years. There was a gradual regression of the changes in the coronary blood vessels to the normalization of the echocardiographic findings after 2 years. Kawasaki disease is the second most common vasculitis of childhood, so it should be included in the differential diagnosis for any child with a prolonged unexplained fever. Atypical Kawasaki disease should be taken into consideration in cases when not all clinical criteria are present but coronary abnormalities are documented.

  12. Concordance between Responses to Questionnaire Scenarios and Actual Treatment to Repair or Replace Dental Restorations in the National Dental PBRN (United States)

    Heaven, Tim J.; Gordan, Valeria V.; Litaker, Mark S.; Fellows, Jeffrey L.; Rindal, D. Brad; Gilbert, Gregg H.


    Objective To quantify the agreement between treatment recommended during hypothetical clinical scenarios and actual treatment provided in comparable clinical circumstances. Methods A total of 193 practitioners in the National Dental Practice-Based Research Network participated in both a questionnaire and a clinical study. The questionnaire included three hypothetical scenarios about treatment of existing restorations. Clinicians then participated in a clinical study about repair or replacement of existing restorations. We quantified the overall concordance between their questionnaire responses and what they did in actual clinical treatment. Results Practitioners who recommended repair (instead of replacement) of more scenario restorations also had higher repair percentages in clinical practice. Additionally, for each of the three hypothetical scenario restorations, practitioners who recommended repair had higher repair percentages in clinical practice. Conclusions The questionnaire scenarios were a valid measure of clinicians’ tendency to repair or replace restorations in actual clinical practice. Clinical implications Although there was substantial variation in practitioners’ tendency to repair or replace restorations, responses to questionnaire scenarios by individual practitioners were concordant with what they did in actual clinical practice. PMID:25998565

  13. Predictors of Insulin Like Growth Factor-I responses to Growth Hormone replacement in young adults with Growth Hormone deficiency


    Thankamony, Ajay; Capalbo, Donatella; Jonsson, Peter J.; Simpson, Helen L.; Dunger, David B.


    This is the author accepted manuscript. It is currently under an indefinite embargo pending publication by Karger Publishers. Background/Aims: Physiological growth hormone (GH) secretion and IGF-I levels are greater in young compared to older adults. We evaluated IGF-I levels and predictors of IGF-I responses in young adults on GH replacement. Design: From KIMS database, 310 young adults (age, 15 26 years) with the severe GH deficiency related to childhood-onset disease, and commenced ...

  14. [Dementia with Lewy bodies; 2 patients with exacerbation due to an atypical antipsychotic, but with a favorable response to the cholinesterase inhibitor rivastigmine

    NARCIS (Netherlands)

    Scheepmaker, A.J.T.M.; Horstink, M.W.I.M.; Hoefnagels, W.H.L.; Strijks, F.E.


    In two patients, men aged 80 and 75 years with cognitive deterioration, hallucinations and parkinsonism, the clinical diagnosis 'dementia with Lewy bodies' was established. Treatment with an atypical antipsychotic, risperidone and olanzapine respectively, resulted in an exacerbation of the parkinson

  15. Protein-energy malnutrition developing after global brain ischemia induces an atypical acute-phase response and hinders expression of GAP-43.

    Directory of Open Access Journals (Sweden)

    Shari E Smith

    Full Text Available Protein-energy malnutrition (PEM is a common post-stroke problem. PEM can independently induce a systemic acute-phase response, and pre-existing malnutrition can exacerbate neuroinflammation induced by brain ischemia. In contrast, the effects of PEM developing in the post-ischemic period have not been studied. Since excessive inflammation can impede brain remodeling, we investigated the effects of post-ischemic malnutrition on neuroinflammation, the acute-phase reaction, and neuroplasticity-related proteins. Male, Sprague-Dawley rats were exposed to global forebrain ischemia using the 2-vessel occlusion model or sham surgery. The sham rats were assigned to control diet (18% protein on day 3 after surgery, whereas the rats exposed to global ischemia were assigned to either control diet or a low protein (PEM, 2% protein diet. Post-ischemic PEM decreased growth associated protein-43, synaptophysin and synaptosomal-associated protein-25 immunofluorescence within the hippocampal CA3 mossy fiber terminals on day 21, whereas the glial response in the hippocampal CA1 and CA3 subregions was unaltered by PEM. No systemic acute-phase reaction attributable to global ischemia was detected in control diet-fed rats, as reflected by serum concentrations of alpha-2-macroglobulin, alpha-1-acid glycoprotein, haptoglobin, and albumin. Acute exposure to the PEM regimen after global brain ischemia caused an atypical acute-phase response. PEM decreased the serum concentrations of albumin and haptoglobin on day 5, with the decreases sustained to day 21. Serum alpha-2-macroglobulin concentrations were significantly higher in malnourished rats on day 21. This provides the first direct evidence that PEM developing after brain ischemia exerts wide-ranging effects on mechanisms important to stroke recovery.

  16. Protein-energy malnutrition developing after global brain ischemia induces an atypical acute-phase response and hinders expression of GAP-43. (United States)

    Smith, Shari E; Figley, Sarah A; Schreyer, David J; Paterson, Phyllis G


    Protein-energy malnutrition (PEM) is a common post-stroke problem. PEM can independently induce a systemic acute-phase response, and pre-existing malnutrition can exacerbate neuroinflammation induced by brain ischemia. In contrast, the effects of PEM developing in the post-ischemic period have not been studied. Since excessive inflammation can impede brain remodeling, we investigated the effects of post-ischemic malnutrition on neuroinflammation, the acute-phase reaction, and neuroplasticity-related proteins. Male, Sprague-Dawley rats were exposed to global forebrain ischemia using the 2-vessel occlusion model or sham surgery. The sham rats were assigned to control diet (18% protein) on day 3 after surgery, whereas the rats exposed to global ischemia were assigned to either control diet or a low protein (PEM, 2% protein) diet. Post-ischemic PEM decreased growth associated protein-43, synaptophysin and synaptosomal-associated protein-25 immunofluorescence within the hippocampal CA3 mossy fiber terminals on day 21, whereas the glial response in the hippocampal CA1 and CA3 subregions was unaltered by PEM. No systemic acute-phase reaction attributable to global ischemia was detected in control diet-fed rats, as reflected by serum concentrations of alpha-2-macroglobulin, alpha-1-acid glycoprotein, haptoglobin, and albumin. Acute exposure to the PEM regimen after global brain ischemia caused an atypical acute-phase response. PEM decreased the serum concentrations of albumin and haptoglobin on day 5, with the decreases sustained to day 21. Serum alpha-2-macroglobulin concentrations were significantly higher in malnourished rats on day 21. This provides the first direct evidence that PEM developing after brain ischemia exerts wide-ranging effects on mechanisms important to stroke recovery.

  17. Atypical charles bonnet syndrome. (United States)

    Arun, Priti; Jain, Rajan; Tripathi, Vaibhav


    Charles Bonnet syndrome (CBS) is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision.

  18. Optimal Fluxes, Reaction Replaceability, and Response to Enzymopathies in the Human Red Blood Cell

    Directory of Open Access Journals (Sweden)

    A. De Martino


    most harmful reaction knockouts. The integration of combinatorial methods with sampling techniques to explore the space of viable flux states may provide crucial insights on this issue. We assess the replaceability of every metabolic conversion in the human red blood cell by enumerating the alternative paths from substrate to product, obtaining a complete map of he potential damage of single enzymopathies. Sampling the space of optimal steady state fluxes in the healthy and in the mutated cell reveals both correlations and complementarity between topologic and dynamical aspects.

  19. PapR6, a putative atypical response regulator, functions as a pathway-specific activator of pristinamycin II biosynthesis in Streptomyces pristinaespiralis. (United States)

    Dun, Junling; Zhao, Yawei; Zheng, Guosong; Zhu, Hong; Ruan, Lijun; Wang, Wenfang; Ge, Mei; Jiang, Weihong; Lu, Yinhua


    There are up to seven regulatory genes in the pristinamycin biosynthetic gene cluster of Streptomyces pristinaespiralis, which infers a complicated regulation mechanism for pristinamycin production. In this study, we revealed that PapR6, a putative atypical response regulator, acts as a pathway-specific activator of pristinamycin II (PII) biosynthesis. Deletion of the papR6 gene resulted in significantly reduced PII production, and its overexpression led to increased PII formation, compared to that of the parental strain HCCB 10218. However, either papR6 deletion or overexpression had very little effect on pristinamycin I (PI) biosynthesis. Electrophoretic mobility shift assays (EMSAs) demonstrated that PapR6 bound specifically to the upstream region of snaF, the first gene of the snaFE1E2GHIJK operon, which is likely responsible for providing the precursor isobutyryl-coenzyme A (isobutyryl-CoA) and the intermediate C11 αβ-unsaturated thioester for PII biosynthesis. A signature PapR6-binding motif comprising two 4-nucleotide (nt) inverted repeat sequences (5'-GAGG-4 nt-CCTC-3') was identified. Transcriptional analysis showed that inactivation of the papR6 gene led to markedly decreased expression of snaFE1E2GHIJK. Furthermore, we found that a mutant (snaFmu) with base substitutions in the identified PapR6-binding sequence in the genome exhibited the same phenotype as that of the ΔpapR6 strain. Therefore, it may be concluded that pathway-specific regulation of PapR6 in PII biosynthesis is possibly exerted via controlling the provision of isobutyryl-CoA as well as the intermediate C11 αβ-unsaturated thioester.

  20. Genome-Wide Association Studies Identify Two Novel BMP15 Mutations Responsible for an Atypical Hyperprolificacy Phenotype in Sheep (United States)

    Demars, Julie; Fabre, Stéphane; Sarry, Julien; Rossetti, Raffaella; Gilbert, Hélène; Persani, Luca; Tosser-Klopp, Gwenola; Mulsant, Philippe; Nowak, Zuzanna; Drobik, Wioleta; Martyniuk, Elzbieta; Bodin, Loys


    Some sheep breeds are naturally prolific, and they are very informative for the studies of reproductive genetics and physiology. Major genes increasing litter size (LS) and ovulation rate (OR) were suspected in the French Grivette and the Polish Olkuska sheep populations, respectively. To identify genetic variants responsible for the highly prolific phenotype in these two breeds, genome-wide association studies (GWAS) followed by complementary genetic and functional analyses were performed. Highly prolific ewes (cases) and normal prolific ewes (controls) from each breed were genotyped using the Illumina OvineSNP50 Genotyping Beadchip. In both populations, an X chromosome region, close to the BMP15 gene, harbored clusters of markers with suggestive evidence of association at significance levels between 1E−05 and 1E−07. The BMP15 candidate gene was then sequenced, and two novel non-conservative mutations called FecXGr and FecXO were identified in the Grivette and Olkuska breeds, respectively. The two mutations were associated with the highly prolific phenotype (pFecXGr = 5.98E−06 and pFecXO = 2.55E−08). Homozygous ewes for the mutated allele showed a significantly increased prolificacy (FecXGr/FecXGr, LS = 2.50±0.65 versus FecX+/FecXGr, LS = 1.93±0.42, p<1E−03 and FecXO/FecXO, OR = 3.28±0.85 versus FecX+/FecXO, OR = 2.02±0.47, p<1E−03). Both mutations are located in very well conserved motifs of the protein and altered the BMP15 signaling activity in vitro using a BMP-responsive luciferase test in COV434 granulosa cells. Thus, we have identified two novel mutations in the BMP15 gene associated with increased LS and OR. Notably, homozygous FecXGr/FecXGr Grivette and homozygous FecXO/FecXO Olkuska ewes are hyperprolific in striking contrast with the sterility exhibited by all other known homozygous BMP15 mutations. Our results bring new insights into the key role played by the BMP15 protein in ovarian function and could

  1. Effects of music on psychophysiological responses and opioid dosage in patients undergoing total knee replacement surgery. (United States)

    Chen, Hsin-Ji; Chen, Tsung-Ying; Huang, Chiung-Yu; Hsieh, Yuan-Mei; Lai, Hui-Ling


    The present authors examined the effects of listening to music on psychophysiological parameters (blood pressure, heart rate, and respiratory rate) during preoperative and postoperative days and determined whether listening to music could lower pain intensity and opioid dosage during postoperative days in patients who underwent total knee replacements. This was a two group repeated measures design for 30 subjects aged 53-85 years who were scheduled for total knee replacement. Subjects were randomly assigned to either a music group or a control group. Psychophysiological parameters were obtained from patients' monitors. A visual analog scale was used to assess postoperative pain. Opioid dosage was recorded and converted to standardized units. Mann-Whitney U-test and generalized estimating equation analysis were used to compare groups. Respiratory rates while in the surgical waiting area were lower for the music group than for the control group (P = 0.02). There was no significant difference between these groups for blood pressure, heart rate, pain intensity, or opioid dosage. However, a within-group comparison showed that systolic blood pressure in the music group was significantly and consistently decreased during postoperative recovery (Wald = 9.21, P = 0.007). These results suggest that listening to music stabilized systolic blood pressure in patients during postoperative recovery. However, the effects of music on psychophysiological parameters, pain intensity, and opioid dosage in a surgical setting require further research. © 2015 The Authors. Japan Journal of Nursing Science © 2015 Japan Academy of Nursing Science.

  2. Characterization of the atypical lymphocytes in African swine fever

    Directory of Open Access Journals (Sweden)

    Z. A. Karalyan


    Full Text Available Aim: Atypical lymphocytes usually described as lymphocytes with altered shape, increased DNA amount, and larger size. For analysis of cause of genesis and source of atypical lymphocytes during African swine fever virus (ASFV infection, bone marrow, peripheral blood, and in vitro model were investigated. Materials and Methods: Atypical lymphocytes under the influence of ASFV were studied for morphologic, cytophotometric, and membrane surface marker characteristics and were used in vivo and in vitro models. Results: This study indicated the increased size, high metabolic activity, and the presence of additional DNA amount in atypical lymphocytes caused by ASFV infection. Furthermore, in atypical lymphocytes, nuclear-cytoplasmic ratio usually decreased, compared to normal lymphocytes. In morphology, they looking like lymphocytes transformed into blasts by exposure to mitogens or antigens in vitro. They vary in morphologic detail, but most of them are CD2 positive. Conclusions: Our data suggest that atypical lymphocytes may represent an unusual and specific cellular response to ASFV infection.

  3. Characterization of the atypical lymphocytes in African swine fever (United States)

    Karalyan, Z. A.; Ter-Pogossyan, Z. R.; Abroyan, L. O.; Hakobyan, L. H.; Avetisyan, A. S.; Karalyan, N. Yu; Karalova, E. M.


    Aim: Atypical lymphocytes usually described as lymphocytes with altered shape, increased DNA amount, and larger size. For analysis of cause of genesis and source of atypical lymphocytes during African swine fever virus (ASFV) infection, bone marrow, peripheral blood, and in vitro model were investigated. Materials and Methods: Atypical lymphocytes under the influence of ASFV were studied for morphologic, cytophotometric, and membrane surface marker characteristics and were used in vivo and in vitro models. Results: This study indicated the increased size, high metabolic activity, and the presence of additional DNA amount in atypical lymphocytes caused by ASFV infection. Furthermore, in atypical lymphocytes, nuclear-cytoplasmic ratio usually decreased, compared to normal lymphocytes. In morphology, they looking like lymphocytes transformed into blasts by exposure to mitogens or antigens in vitro. They vary in morphologic detail, but most of them are CD2 positive. Conclusions: Our data suggest that atypical lymphocytes may represent an unusual and specific cellular response to ASFV infection. PMID:27536044

  4. Level of response to telematic questionnaires on Health Related Quality of Life on total knee replacement. (United States)

    Besalduch-Balaguer, M; Aguilera-Roig, X; Urrútia-Cuchí, G; Puntonet-Bruch, A; Jordan-Sales, M; González-Osuna, A; Celaya-Ibáñez, F; Colomina-Morales, J


    Questionnaires measuring health-related quality of life are difficult to perform and obtain for patients and professionals. Computerised tools are now available to collect this information. The objective of this study was to assess the ability of patients undergoing total knee replacement to fill in health-related quality-of-life questionnaires using a telematic platform. Ninety eight consecutive patients undergoing total knee arthroplasty were included. Participants were given an access code to enter the website where they had to respond to 2 questionnaires (SF8 and the reduced WOMAC), and 3 additional questions about the difficulty in completing the questionnaires. A total of 98 patients agreed to participate: 45 males and 53 females (mean age 72.7 years). Fourteen did not agree to participate due to lack of internet access. Of the final 84 participants, 50% entered the website, and only 36 answered all questions correctly. Of the patients who answered the questionnaire, 80% were helped by a relative or friend, and 22% reported difficulty accessing internet. The use of telematic systems to respond to health-related quality of life questionnaires should be used cautiously, especially in elderly population. It is likely that the population they are directed at is not prepared to use this type of technology. Therefore, before designing telematics questionnaires it must be ensured that they are completed properly. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  5. CALiPER Benchmark Report: Performance of Incandescent A Type and Decorative Lamps and LED Replacements

    Energy Technology Data Exchange (ETDEWEB)

    Lingard, R. D. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Myer, M. A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Paget, M. L. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)


    This benchmark report addresses common omnidirectional incandescent lamps - A-type and small decorative, candelabra-type lamps - and their commercially available light-emitting diode (LED) replacements.

  6. Hip disability and osteoarthritis outcome score (HOOS)--validity and responsiveness in total hip replacement

    DEFF Research Database (Denmark)

    Nilsdotter, Anna K; Lohmander, L Stefan; Klässbo, Maria


    The aim of the study was to evaluate if physical functions usually associated with a younger population were of importance for an older population, and to construct an outcome measure for hip osteoarthritis with improved responsiveness compared to the Western Ontario McMaster osteoarthritis score...

  7. The Foundations of Corporate Social Responsibility (replaced by CentER DP 2014-069)

    NARCIS (Netherlands)

    Liang, H.; Renneboog, L.D.R.


    Abstract: We investigate the roles of legal origins and political institutions – believed to be the fundamental determinants of economic outcomes – in corporate social responsibility (CSR). We argue that CSR is an essential path to economic sustainability, and document strong correlations between co

  8. Accumulation of VH Replacement Products in IgH Genes Derived from Autoimmune Diseases and Anti-Viral Responses in Human. (United States)

    Lange, Miles D; Huang, Lin; Yu, Yangsheng; Li, Song; Liao, Hongyan; Zemlin, Michael; Su, Kaihong; Zhang, Zhixin


    VH replacement refers to RAG-mediated secondary recombination of the IgH genes, which renews almost the entire VH gene coding region but retains a short stretch of nucleotides as a VH replacement footprint at the newly generated VH-DH junction. To explore the biological significance of VH replacement to the antibody repertoire, we developed a Java-based VH replacement footprint analyzer program and analyzed the distribution of VH replacement products in 61,851 human IgH gene sequences downloaded from the NCBI database. The initial assignment of the VH, DH, and JH gene segments provided a comprehensive view of the human IgH repertoire. To our interest, the overall frequency of VH replacement products is 12.1%; the frequencies of VH replacement products in IgH genes using different VH germline genes vary significantly. Importantly, the frequencies of VH replacement products are significantly elevated in IgH genes derived from different autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and allergic rhinitis, and in IgH genes encoding various autoantibodies or anti-viral antibodies. The identified VH replacement footprints preferentially encoded charged amino acids to elongate IgH CDR3 regions, which may contribute to their autoreactivities or anti-viral functions. Analyses of the mutation status of the identified VH replacement products suggested that they had been actively involved in immune responses. These results provide a global view of the distribution of VH replacement products in human IgH genes, especially in IgH genes derived from autoimmune diseases and anti-viral immune responses.

  9. Effects of bovine somatotropin administration on growth, physiological, and reproductive responses of replacement beef heifers. (United States)

    Cooke, R F; Bohnert, D W; Francisco, C L; Marques, R S; Mueller, C J; Keisler, D H


    proportion of BST heifers reached puberty during the experiment compared with control cohorts, despite lesser plasma leptin concentrations, backfat thickness, and similar ADG. Hence, circulating IGF-I was positively associated with hastened puberty attainment independently of growth rate, circulating leptin concentrations, and body fat content of replacement beef heifers.

  10. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo;


    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  11. Atypical Odontalgia (Phantom Tooth Pain) (United States)

    ... Odontalgia Atypical odontalgia, also known as atypical facial pain, phantom tooth pain, or neuropathic orofacial pain, is characterized by chronic pain in a tooth or teeth, or in a site where teeth ...

  12. Atypical charles bonnet syndrome

    Directory of Open Access Journals (Sweden)

    Priti Arun


    Full Text Available Charles Bonnet syndrome (CBS is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision.

  13. The stress response and anesthetic potency of unilateral spinal anesthesia for total Hip Replacement in geriatric patients. (United States)

    Zhu, Li; Tian, Chun; Li, Min; Peng, Ming-Qing; Ma, Kun-Long; Wang, Zhong-Lin; Ding, Jia-Hui; Cai, Yi


    Recently, some scholars suggested that it is important to keep a stablehemodynamic state and prevent the stress responses in geriatric patients undergoing total hip replacement (THR). We conducted this randomized prospective study to observe anesthetic potency of unilateral spinal anesthesia and stress response to it in geriatric patients during THR. We compared the effect of unilateral spinal and bilateral spinal on inhibition of stress response through measuring Norepinephrine (NE), epinephrine (E) and cortisol (CORT). Plasma concentrations of NE, E and CORT were determined in blood samples using ELISA (enzyme-linked immunosorbent assays) at three time points: To (prior to anesthesia) T1 (at the time point of skin closure), T2 (twenty-four hours after the operation). Sixty patients were randomly divided into two groups: group A (unilateral spinal anesthesia) and group B (conventional bilateral spinal anesthesia). 7.5tymg of hypobaric bupivacaine were injected into subarachnoid cavity at group A and 12mg hypobaric bupivacaine were given at group B. The onset time of sensory and motor block, loss of pinprick sensation, degree of motor block, regression of sensory and motor blocks and hemodynamic changes were also recorded. These data were used to evaluate anesthetic potency of spinal anesthesia. The results of this experiment show that unilateral spinal anesthesia can provide restriction of sensory and motor block, minimize the incidence of hypotension and prevent the stress responses undergoing THR. It is optimal anesthesia procedure for geriatric patients by rapid subarachnoid injection of small doses of bupivacaine.

  14. Replacing The Responsibility to Protect: The Equitable Theory of Humanitarian Intervention

    Directory of Open Access Journals (Sweden)

    Ciarán Burke


    Full Text Available In this article, Ciarán J. Burke argues that the ‘Responsibility to Protect’ initiative has failed. Burke presents a series of fundamental flaws, both with the doctrine advanced by the ICISS, and with the subsequent attempts to incorporate it into the international legal framework. Burke opines that equity, as a source of international law, should instead be used to shed fresh light on the debate, keeping the discourse within the law and away from subjective ethics, and drafting a novel framework which he dubs ‘equitable humanitarian intervention’.

  15. That Was Then, This Is Now: Replacing the Mobile-Optimized Site with Responsive Design

    Directory of Open Access Journals (Sweden)

    Laurie M. Bridges


    Full Text Available As mobile technologies continue to evolve, libraries seek sustainable ways to keep up with these changes and to best serve our users.  Previous library mobile usability research has examined tasks users predict they might be likely to perform, but little is known about what users actually do on a mobile-optimized library site.  This research used a combination of survey methodology and web analytics to examine what tasks users actually carry out on a library mobile site.  The results indicate that users perform an array of passive and active tasks and do not want content choices to be limited on mobile devices.  Responsive design is described as a long-term solution for addressing both designers and users’ needs.

  16. Atypical prefrontal cortical responses to joint/non-joint attention in children with autism spectrum disorder (ASD): A functional near-infrared spectroscopy study (United States)

    Zhu, Huilin; Li, Jun; Fan, Yuebo; Li, Xinge; Huang, Dan; He, Sailing


    Autism spectrum disorder (ASD) is a neuro-developmental disorder, characterized by impairments in one’s capacity for joint attention. In this study, functional near-infrared spectroscopy (fNIRS) was applied to study the differences in activation and functional connectivity in the prefrontal cortex between children with autism spectrum disorder (ASD) and typically developing (TD) children. 21 ASD and 20 TD children were recruited to perform joint and non-joint attention tasks. Compared with TD children, children with ASD showed reduced activation and atypical functional connectivity pattern in the prefrontal cortex during joint attention. The atypical development of left prefrontal cortex might play an important role in social cognition defects of children with ASD. PMID:25798296

  17. Predictions replaced by facts: a keystone species' behavioural responses to declining arctic sea-ice. (United States)

    Hamilton, Charmain D; Lydersen, Christian; Ims, Rolf A; Kovacs, Kit M


    Since the first documentation of climate-warming induced declines in arctic sea-ice, predictions have been made regarding the expected negative consequences for endemic marine mammals. But, several decades later, little hard evidence exists regarding the responses of these animals to the ongoing environmental changes. Herein, we report the first empirical evidence of a dramatic shift in movement patterns and foraging behaviour of the arctic endemic ringed seal (Pusa hispida), before and after a major collapse in sea-ice in Svalbard, Norway. Among other changes to the ice-regime, this collapse shifted the summer position of the marginal ice zone from over the continental shelf, northward to the deep Arctic Ocean Basin. Following this change, which is thought to be a 'tipping point', subadult ringed seals swam greater distances, showed less area-restricted search behaviour, dived for longer periods, exhibited shorter surface intervals, rested less on sea-ice and did less diving directly beneath the ice during post-moulting foraging excursions. In combination, these behavioural changes suggest increased foraging effort and thus also likely increases in the energetic costs of finding food. Continued declines in sea-ice are likely to result in distributional changes, range reductions and population declines in this keystone arctic species.

  18. Human antibody responses to the polyclonal Dryvax vaccine for smallpox prevention can be distinguished from responses to the monoclonal replacement vaccine ACAM2000. (United States)

    Pugh, Christine; Keasey, Sarah; Korman, Lawrence; Pittman, Phillip R; Ulrich, Robert G


    Dryvax (Wyeth Laboratories, Inc., Marietta, PA) is representative of the vaccinia virus preparations that were previously used for preventing smallpox. While Dryvax was highly effective, the national supply stocks were depleted, and there were manufacturing concerns regarding sterility and the clonal heterogeneity of the vaccine. ACAM2000 (Acambis, Inc./Sanofi-Pasteur Biologics Co., Cambridge, MA), a single-plaque-purified vaccinia virus derivative of Dryvax, recently replaced the polyclonal smallpox vaccine for use in the United States. A substantial amount of sequence heterogeneity exists within the polyclonal proteome of Dryvax, including proteins that are missing from ACAM2000. Reasoning that a detailed comparison of antibody responses to the polyclonal and monoclonal vaccines may be useful for identifying unique properties of each antibody response, we utilized a protein microarray comprised of approximately 94% of the vaccinia poxvirus proteome (245 proteins) to measure protein-specific antibody responses of 71 individuals receiving a single vaccination with ACAM2000 or Dryvax. We observed robust antibody responses to 21 poxvirus proteins in vaccinated individuals, including 11 proteins that distinguished Dryvax responses from ACAM2000. Analysis of protein sequences from Dryvax clones revealed amino acid level differences in these 11 antigenic proteins and suggested that sequence variation and clonal heterogeneity may contribute to the observed differences between Dryvax and ACAM2000 antibody responses.

  19. Ankle replacement (United States)

    Ankle arthroplasty - total; Total ankle arthroplasty; Endoprosthetic ankle replacement; Ankle surgery ... You may not be able to have a total ankle replacement if you have had ankle joint infections in ...

  20. Knee Replacement (United States)

    Knee replacement is surgery for people with severe knee damage. Knee replacement can relieve pain and allow you to ... Your doctor may recommend it if you have knee pain and medicine and other treatments are not ...

  1. Case Report: An atypical case of systemic lupus erythematosus ...

    African Journals Online (AJOL)

    Results: She had a favorable clinical response and continues to be followed up as an outpatient.Conclusion: Systemic lupus erythematosus can be difficult diagnosis to make as it may present with atypical features.

  2. Dose-response analysis of testosterone replacement therapy in patients with female to male gender identity disorder. (United States)

    Nakamura, Aya; Watanabe, Masami; Sugimoto, Morito; Sako, Tomoko; Mahmood, Sabina; Kaku, Haruki; Nasu, Yasutomo; Ishii, Kazushi; Nagai, Atsushi; Kumon, Hiromi


    Gender identity disorder (GID) is a conflict between a person's actual physical gender and the one they identify him or herself with. Testosterone is the key agent in the medical treatment of female to male GID patients. We conducted a dose-response analysis of testosterone replacement therapy (TRT) in 138 patients to determine the onset of the therapeutic effects. The TRT consisted of intramuscular injection of testosterone enanthate and patients were divided into three groups; 250 mg every two weeks, 250 mg every three weeks and 125 mg every two weeks. The onset of deepening of voice, increase in facial hair and cessation of menses was evaluated in each group. At one month after the start of TRT, the onset of these physical changes was more prevalent in the group receiving the higher dose of testosterone, and there were dose-dependent effects observed between the three treatment groups. On the other hand, at six months after the start of TRT, most of the patients had achieved treatment responses and there were no dose-dependent effects with regard to the percentage of patients with therapeutic effects. No significant side effects were observed in any of the treatment groups. We demonstrated that the early onset of the treatment effects of TRT is dose-dependent, but within six months of starting TRT, all three doses were highly effective. Current study provides useful information to determine the initial dose of TRT and to suggest possible changes that should be made in the continuous dosage for long term TRT.

  3. [Atypical presentation of preeclampsia]. (United States)

    Ditisheim, A; Boulvain, M; Irion, O; Pechère-Bertschi, A


    Preeclampsia is a pregnancy-related syndrome, which still represents one of the major causes of maternal-fetal mortality and morbidity. Diagnosis can be made difficult due to the complexity of the disorder and its wide spectrum of clinical manifestations. In order to provide an efficient diagnostic tool to the clinician, medical societies regularly rethink the definition criteria. However, there are still clinical presentations of preeclampsia that escape the frame of the definition. The present review will address atypical forms of preeclampsia, such as preeclampsia without proteinuria, normotensive preeclampsia, preeclampsia before 20 weeks of gestation and post-partum preeclampsia.

  4. Critical appraisal of eculizumab for atypical hemolytic uremic syndrome. (United States)

    Palma, Lilian M Pereira; Langman, Craig B


    The biology of atypical hemolytic uremic syndrome has been shown to involve inability to limit activation of the alternative complement pathway, with subsequent damage to systemic endothelial beds and the vasculature, resulting in the prototypic findings of a thrombotic microangiopathy. Central to this process is the formation of the terminal membrane attack complex C5b-9. Recently, application of a monoclonal antibody that specifically binds to C5, eculizumab, became available to treat patients with atypical hemolytic uremic syndrome, replacing plasma exchange or infusion as primary therapy. This review focuses on the evidence, based on published clinical trials, case series, and case reports, on the efficacy and safety of this approach.

  5. Response Blocking with Guided Compliance and Reinforcement for a Habilitative Replacement Behavior: Effects on Public Masturbation and On-Task Behavior (United States)

    Dufrene, Brad A.; Watson, T. Steuart; Weaver, Adam


    There is limited empirical research regarding effective treatment for public masturbation. In the current study, the relative and combined effects of reinforcement of an incompatible habilitative replacement behavior and response blocking with guided compliance on masturbation and on-task behavior were evaluated for a seven year-old…

  6. Directed Replacement

    CERN Document Server

    Karttunen, L


    This paper introduces to the finite-state calculus a family of directed replace operators. In contrast to the simple replace expression, UPPER -> LOWER, defined in Karttunen (ACL-95), the new directed version, UPPER @-> LOWER, yields an unambiguous transducer if the lower language consists of a single string. It transduces the input string from left to right, making only the longest possible replacement at each point. A new type of replacement expression, UPPER @-> PREFIX ... SUFFIX, yields a transducer that inserts text around strings that are instances of UPPER. The symbol ... denotes the matching part of the input which itself remains unchanged. PREFIX and SUFFIX are regular expressions describing the insertions. Expressions of the type UPPER @-> PREFIX ... SUFFIX may be used to compose a deterministic parser for a ``local grammar'' in the sense of Gross (1989). Other useful applications of directed replacement include tokenization and filtering of text streams.

  7. Increasing intake of essential fatty acids from milk replacer benefits performance, immune responses, and health of preweaned Holstein calves. (United States)

    Garcia, M; Shin, J H; Schlaefli, A; Greco, L F; Maunsell, F P; Thatcher, W W; Santos, J E P; Staples, C R


    The objective was to evaluate the effect of feeding increasing amounts of essential fatty acids (FA) in milk replacer (MR) during the first 60 d of life on growth, health, and immunity of Holstein calves. Calves were born from dams fed low concentrations of total and essential FA during the lasT2 mo of pregnancy. Newborn calves were blocked by sex and parity of the dam and assigned randomly to receive 1 of 4 MR treatments (T). Hydrogenated coconut oil and soybean oil were mixed with emulsifier and commercial MR powder to prepare the following 4 MR containing 0.119 and 0.007 (T1), 0.187 and 0.017 (T2), 0.321 and 0.036 (T3), and 0.593 and 0.076 (T4) g of intake per kg of metabolic body weight (BW(0.75)) of linoleic acid and α-linolenic acid, respectively. At 30 d of life, concentrations of essential FA (linoleic acid and α-linolenic acid) in liver increased, whereas concentrations of C12:0, C14:0, C16:0, and C20:3n-9 decreased linearly with increasing intake of essential FA. Body weight gain and feed efficiency were optimized when male calves consumed T2, whereas gain by female calves tended to increase linearly with increasing intake of essential FA during the first 30 d of age. However, these responses to treatment were not maintained after initiation of concentrate feeding at 31 d of life. Over the 60-d preweaning period, wither and hip heights were improved in both sexes as intake of essential FA increased up to T3. Some measures of health and immunity were affected by replacing some coconut oil with soybean oil. Severity of diarrhea tended to decrease linearly; plasma concentrations of haptoglobin during diarrhea were lower in T2, T3, and T4; phagocytosis by blood neutrophils tended to peak for calves fed T2; in vitro proliferation of stimulated blood lymphocytes was greater for calves fed T2; in vitro stimulated blood cells produced more IFN-γ (up to T3 for males and T2 for females), concentrations of serum IgG against ovalbumin injections were increased in

  8. Atypical retroperitoneal extension of iliopsoas bursitis

    Energy Technology Data Exchange (ETDEWEB)

    Coulier, B.; Cloots, V. [Department of Diagnostic Imaging, Cliniques St. Luc, Rue St Luc 8, 5004, Bouge, Namur (Belgium)


    We report two rare cases of iliopsoas bursitis extending into the retroperitoneal space. The first lesion contained much gas, mimicking a retroperitoneal abscess, and the second was responsible for atypical inguinal pain. The diagnosis was made by contrast-enhanced CT in both cases and arthrography in the first case. Iliopsoas bursitis in these two patients, it is hypothesized, extended into the retroperitoneum, at least in part, by way of intraneural or perineural structures. (orig.)

  9. Paleocommunity Replacements of Benthic Brachiopod in the Middle-Upper Devonian in the Longmenshan Area, Southwestern China: Responses to Sea Level Fluctuations

    Institute of Scientific and Technical Information of China (English)

    CHEN Yuanren; LI Xianghui


    Twelve paleocommunities dominated by benthic brachiopod are recognized in the Givetian-Frasnian stages of the Devonian in the Longmenshan area, southwestern China, in which two kinds of brachiopod community replacement are classified. One is the abrupt replacement, represented by abrupt alternation between the Leiorhynchus community and Zhonghuacoelia-Striatopugnax community in the Frasnian Tuqiaozi Formation. The other is the gradual one, developed in the Givetian Guanwushan Formation, which had been completed by the shift of the Independatrypa lemma-Uncinulus heterocostellis-Emanuella takwanensis community via the Sinospongophyllum irregulare-Pseudomicroplasma fongi community to the Clathrocoilona spissa-Hexagonaria composite reef community. According to analyses of the paleocommunities, either the abrupt or gradual paleocommunity replacement of the Middle-Upper Devonian in the Longmenshan area is suggested as a response to the 5th-order sea level fluctuation due to the replacements of the paleocommunities in a tracts-system of depositional sequence. It is supposed that changes of paleocommunity diversity,one of the results of paleocommunity replacement, are depended on the range and magnitude of sea level fluctuation, but there is not a linear relationship between them. Furthermore, a suggestion is proposed that the concept of paleocommunity succession seem to be abandoned in the paleocommunity analysis because it almost never be practiced to recover the information of community succession in the geological record at present.

  10. Knee Replacement (United States)

    ... need knee replacement surgery usually have problems walking, climbing stairs, and getting in and out of chairs. Some ... a total living space on one floor since climbing stairs can be difficult. Install safety bars or a ...

  11. Replacing penalties

    Directory of Open Access Journals (Sweden)

    Vitaly Stepashin


    Full Text Available УДК 343.24The subject. The article deals with the problem of the use of "substitute" penalties.The purpose of the article is to identify criminal and legal criteria for: selecting the replacement punishment; proportionality replacement leave punishment to others (the formalization of replacement; actually increasing the punishment (worsening of legal situation of the convicted.Methodology.The author uses the method of analysis and synthesis, formal legal method.Results. Replacing the punishment more severe as a result of malicious evasion from serving accused designated penalty requires the optimization of the following areas: 1 the selection of a substitute punishment; 2 replacement of proportionality is serving a sentence other (formalization of replacement; 3 ensuring the actual toughening penalties (deterioration of the legal status of the convict. It is important that the first two requirements pro-vide savings of repression in the implementation of the replacement of one form of punishment to others.Replacement of punishment on their own do not have any specifics. However, it is necessary to compare them with the contents of the punishment, which the convict from serving maliciously evaded. First, substitute the punishment should assume a more significant range of restrictions and deprivation of certain rights of the convict. Second, the perfor-mance characteristics of order substitute the punishment should assume guarantee imple-mentation of the new measures.With regard to replacing all forms of punishment are set significant limitations in the application that, in some cases, eliminates the possibility of replacement of the sentence, from serving where there has been willful evasion, a stricter measure of state coercion. It is important in the context of the topic and the possibility of a sentence of imprisonment as a substitute punishment in cases where the original purpose of the strict measures excluded. It is noteworthy that the

  12. In vitro Induction of primary antibody responses to particulate and soluble protein antigens in T cell—replaced murine spleen cell cultures

    Institute of Scientific and Technical Information of China (English)



    Specific antibody responses could be induced in serumfree condition.Specific anti-SRBC or anti-SRBC ghost antibody were induced from anti-Thy treated (T-depleted) murine spleen cells in serum-free culture in the presence of Con A conditioned medium.This induction system may facilitate the study of lymphokine functions on antigen triggered B cells. In T cell-replaced cultures,the antibody responses of B cells could be successfully induced when soluble SRBC membrane proteins were used as antigens.It thus indicates that antigen together with lymphokines are sufficient to drive B cells to become antibody secreting cells in the absence of T cells.The T cell-replaced system provides a more stable way for in vitro immunization and may be applied to monoclonal antibody production when in vivo immunization is difficult to be carried out.

  13. Esophageal replacement. (United States)

    Kunisaki, Shaun M; Coran, Arnold G


    This article focuses on esophageal replacement as a surgical option for pediatric patients with end-stage esophageal disease. While it is obvious that the patient׳s own esophagus is the best esophagus, persisting with attempts to retain a native esophagus with no function and at all costs are futile and usually detrimental to the overall well-being of the child. In such cases, the esophagus should be abandoned, and the appropriate esophageal replacement is chosen for definitive reconstruction. We review the various types of conduits used for esophageal replacement and discuss the unique advantages and disadvantages that are relevant for clinical decision-making. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. PAPP2C Interacts with the Atypical Disease Resistance Protein RPW8.2 and Negatively Regulates Salicylic Acid-Dependent Defense Responses in Arabidopsis

    Institute of Scientific and Technical Information of China (English)

    Wen-Ming Wang; Xian-Feng Ma; Yi Zhang; Ming-Cheng Luo; Guo-Liang Wang; Maria Bellizzi; Xing-Yao Xiong; Shun-Yuan Xiao


    Many fungal and oomycete pathogens differentiate a feeding structure named the haustorium to extract nutrition from the plant epidermal cell.The atypical resistance (R) protein RPW8.2 activates salicylic acid (SA)-dependent,haustorium-targeted defenses against Golovinomyces spp.,the causal agents of powdery mildew diseases on multiple plant species.How RPW8.2 activates defense remains uncharacterized.Here,we report that RPW8.2 interacts with the phytochrome-associated protein phosphatase type 2C (PAPP2C) in yeast and in planta as evidenced by coimmunoprecipitation and bimolecular fluorescence complementation assays.Down-regulation of PAPP2C by RNA interference (RNAi) in Col-0 plants lacking RPW8.2 leads to leaf spontaneous cell death and enhanced disease resistance to powdery mildew via the SA-dependent signaling pathway.Moreover,down-regulation of PAPP2C by RNAi in the RPW8.2 background results in strong HR-like cell death,which correlates with elevated RPW8.2 expression.We further demonstrate that hemagglutinin (HA)-tagged PAPP2C prepared from tobacco leaf cells transiently transformed with HA-PAPP2C possesses phosphatase activity.In addition,silencing a rice gene (Os04g0452000) homologous to PAPP2C also results in spontaneous cell death in rice.Combined,our results suggest that RPW8.2 is functionally connected with PAPP2C and that PAPP2C negatively regulates SA-dependent basal defense against powdery mildew in Arabidopsis.

  15. Atypical GTPases as drug targets. (United States)

    Soundararajan, Meera; Eswaran, Jeyanthy


    The Ras GTPases are the founding members of large Ras superfamily, which constitutes more than 150 of these important class of enzymes. These GTPases function as GDP-GTP-regulated binary switches that control many fundamental cellular processes. There are a number of GTPases that have been identified recently, which do not confine to this prototype termed as "atypical GTPases" but have proved to play a remarkable role in vital cellular functions. In this review, we provide an overview of the crucial physiological functions mediated by RGK and Centaurin class of multi domain atypical GTPases. Moreover, the recently available atypical GTPase structures of the two families, regulation, physiological functions and their critical roles in various diseases will be discussed. In summary, this review will highlight the emerging atypical GTPase family which allows us to understand novel regulatory mechanisms and thus providing new avenues for drug discovery programs.

  16. [Atypical hemolytic uremic syndrome]. (United States)

    Blasco Pelicano, Miquel; Rodríguez de Córdoba, Santiago; Campistol Plana, Josep M


    The hemolytic uremic syndrome (HUS) is a clinical entity characterized by thrombocytopenia, non-immune hemolytic anemia and renal impairment. Kidney pathology shows thrombotic microangiopathy (TMA) with endothelial cell injury leading to thrombotic occlusion of arterioles and capillaries. Traditionally, HUS was classified in 2 forms: Typical HUS, most frequently occurring in children and caused by Shiga-toxin-producing bacteria, and atypical HUS (aHUS). aHUS is associated with mutations in complement genes in 50-60% of patients and has worse prognosis, with the majority of patients developing end stage renal disease. After kidney transplantation HUS may develop as a recurrence of aHUS or as de novo disease. Over the last years, many studies have demonstrated that complement dysregulation underlies the endothelial damage that triggers the development of TMA in most of these patients. Advances in our understanding of the pathogenic mechanisms of aHUS, together with the availability of novel therapeutic options, will enable better strategies for the early diagnosis and etiological treatment, which are changing the natural history of aHUS. This review summarizes the aHUS clinical entity and describes the role of complement dysregulation in the pathogenesis of aHUS. Finally, we review the differential diagnosis and the therapeutic options available to patients with aHUS. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  17. Atypical Light Curves

    CERN Document Server

    Steenwyk, Steven D; Molnar, Lawrence A


    We have identified some two-hundred new variable stars in a systematic study of a data archive obtained with the Calvin-Rehoboth observatory. Of these, we present five close binaries showing behaviors presumably due to star spots or other magnetic activity. For context, we first present two new RS CVn systems whose behavior can be readily attribute to star spots. Then we present three new close binary systems that are rather atypical, with light curves that are changing over time in ways not easily understood in terms of star spot activity generally associated with magnetically active binary systems called RS CVn systems. Two of these three are contact binaries that exhibit gradual changes in average brightness without noticeable changes in light curve shape. A third system has shown such large changes in light curve morphology that we speculate this may be a rare instance of a system that transitions back and forth between contact and noncontact configurations, perhaps driven by magnetic cycles in at least o...

  18. A Rare Case: Atypical Measles


    Ümmü Sena Sarı; Figen Kaptan


    Atypical measles has been described in persons who were exposed to wild measles virus several years after they were immunized with killed measles vaccine. Occasionally, it can be caused by live measles vaccines also. It is a clinical picture different from typical measles. In this report, an adult patient with a history of immunization, who presented with high fever, maculopapular rash starting at the palms and soles, and pneumonia, is presented. Atypical measles that was ...

  19. Optimization of fat-reduced ice cream formulation employing inulin as fat replacer via response surface methodology. (United States)

    Pintor, Aurora; Severiano-Pérez, Patricia; Totosaus, Alfonso


    The use of new ingredients like inulin for fat replacement is of wide application in the food industry. The aim of the present work was to reduce the fat content on ice cream formulations. It was possible to reduce up to 25% of butyric and vegetable fats with 3% of inulin, with good textural and sensory characteristics of the final product. The substitution of fat with inulin increased the ice cream mix viscosity, improved air incorporation, and produced ice cream with soft and homogeneous textures. Color characteristics were not affected by the replacement. Hedonic sensory analysis showed that optimized fat-reduced inulin ice cream was not perceived different to commercial vanilla ice cream.

  20. Tomato Growth and Organic Acid Changes in Response to Partial Replacement of NO-3-N by NH+4-N

    Institute of Scientific and Technical Information of China (English)

    DONG Cai-Xia; SHEN Qi-Rong; WANG Ge


    A hydroponic experiment was conducted to study the effect of partial replacement of NO-3-N by NH4+-N on the seedling growth and organic acid content of tomato (Lycopersicon esculentum Mill.). A completely randomized design was established with three replications and five treatments, i.e., NO-3-N/NH4+-N of 100/0, 75/25, 50/50, 25/75 and 0/100. Results showed that 25% replacement of NO3--N by NH4+-N significantly (P = 0.05) improved fresh and dry weight, revealing that a proper percentage of NH4+-N was important for tomato nitrogen nutrition. This could increase the plant growth even though tomato was a crop that preferred nitrate nutrition. Also an increase in the proportion of NH4+-N in the nutrient solution led to a significant decrease (P = 0.05) in malate, citrate and fumarate. However, the 25% NH4+-N plus 75% NO3--N treatment had no significant effect (P = 0.05) on the 2-ketoglutarate, succinate or oxalic acid content, showing that only some organic acids in tomato plants were affected. Only pyruvate increased significantly (P = 0.05), and it only increased for 25% and 50% replacement of NO3--N by NH4+-N. Metabolism of these organic acids, especially malate, citrate and fumarate, should be further studied at the molecular level in vegetables applied with different nitrogen forms.

  1. Nano-calciumphosphate scaffold generation for bone repair/replacement: elucidating the signalling response and cell cycle

    CSIR Research Space (South Africa)

    Wepnener, I


    Full Text Available applications: A picture for the next 20 years. Journal of the european ceramic society, 29(7):1245-55. 4. dorozhkin, s.V. 2007. Bioceramics based on calcium orthophosphates (review). Glass and ceramics, 64(11):442-7. 5. schilling A... requirements for bone repair and replacement due to some characteristics such as: • Lack of collagen fibres2-4 • Very brittle, therefore not prone for use in load-bearing circumstances • General bioactivity needs improvement • Most applications...

  2. Atypical antipsychotics in bipolar disorder: systematic review of randomised trials

    Directory of Open Access Journals (Sweden)

    Moore R Andrew


    Full Text Available Abstract Background Atypical antipsychotics are increasingly used for treatment of mental illnesses like schizophrenia and bipolar disorder, and considered to have fewer extrapyramidal effects than older antipsychotics. Methods We examined efficacy in randomised trials of bipolar disorder where the presenting episode was either depression, or manic/mixed, comparing atypical antipsychotic with placebo or active comparator, examined withdrawals for any cause, or due to lack of efficacy or adverse events, and combined all phases for adverse event analysis. Studies were found through systematic search (PubMed, EMBASE, Cochrane Library, and data combined for analysis where there was clinical homogeneity, with especial reference to trial duration. Results In five trials (2,206 patients participants presented with a depressive episode, and in 25 trials (6,174 patients the presenting episode was manic or mixed. In 8-week studies presenting with depression, quetiapine and olanzapine produced significantly better rates of response and symptomatic remission than placebo, with NNTs of 5–6, but more adverse event withdrawals (NNH 12. With mania or mixed presentation atypical antipsychotics produced significantly better rates of response and symptomatic remission than placebo, with NNTs of about 5 up to six weeks, and 4 at 6–12 weeks, but more adverse event withdrawals (NNH of about 22 in studies of 6–12 weeks. In comparisons with established treatments, atypical antipsychotics had similar efficacy, but significantly fewer adverse event withdrawals (NNT to prevent one withdrawal about 10. In maintenance trials atypical antipsychotics had significantly fewer relapses to depression or mania than placebo or active comparator. In placebo-controlled trials, atypical antipsychotics were associated with higher rates of weight gain of ≥7% (mainly olanzapine trials, somnolence, and extrapyramidal symptoms. In active controlled trials, atypical antipsychotics

  3. High-Dose Estradiol-Replacement Therapy Enhances the Renal Vascular Response to Angiotensin II via an AT2-Receptor Dependent Mechanism

    Directory of Open Access Journals (Sweden)

    Tahereh Safari


    Full Text Available Physiological levels of estrogen appear to enhance angiotensin type 2 receptor- (AT2R- mediated vasodilatation. However, the effects of supraphysiological levels of estrogen, analogous to those achieved with high-dose estrogen replacement therapy in postmenopausal women, remain unknown. Therefore, we pretreated ovariectomized rats with a relatively high dose of estrogen (0.5 mg/kg/week for two weeks. Subsequently, renal hemodynamic responses to intravenous angiotensin II (Ang II, 30–300 ng/kg/min were tested under anesthesia, while renal perfusion pressure was held constant. The role of AT2R was examined by pretreating groups of rats with PD123319 or its vehicle. Renal blood flow (RBF decreased in a dose-related manner in response to Ang II. Responses to Ang II were enhanced by pretreatment with estradiol. For example, at 300 ng kg−1 min−1, Ang II reduced RBF by 45.7±1.9% in estradiol-treated rats but only by 27.3±5.1% in vehicle-treated rats. Pretreatment with PD123319 blunted the response of RBF to Ang II in estradiol-treated rats, so that reductions in RBF were similar to those in rats not treated with estradiol. We conclude that supraphysiological levels of estrogen promote AT2R-mediated renal vasoconstriction. This mechanism could potentially contribute to the increased risk of cardiovascular disease associated with hormone replacement therapy using high-dose estrogen.

  4. Improved response of growth hormone to growth hormone-releasing hormone and reversible chronic thyroiditis after hydrocortisone replacement in isolated adrenocorticotropic hormone deficiency. (United States)

    Inagaki, Miho; Sato, Haruhiro; Miyamoto, Yoshiyasu; Hirukawa, Takashi; Sawaya, Asako; Miyakogawa, Takayo; Tatsumi, Ryoko; Kakuta, Takatoshi


    We report a 44-year-old Japanese man who showed a reversible blunted response of growth hormone (GH) to GH-releasing hormone (GRH) stimulation test and reversible chronic thyroiditis accompanied by isolated ACTH deficiency. He was admitted to our hospital because of severe general malaise, hypotension, and hypoglycemia. He showed repeated attacks of hypoglycemia, and his serum sodium level gradually decreased. Finally, he was referred to the endocrinology division, where his adrenocorticotropic hormone (ACTH) and cortisol values were found to be low, and his GH level was slightly elevated. An increased value of thyroid stimulating hormone (TSH) and decreased values of free triidothyronine and free thyroxine were observed along with anti-thyroglobulin antibody, suggesting chronic thyroiditis. Pituitary stimulation tests revealed a blunted response of ACTH and cortisol to corticotropin-releasing hormone, and a blunted response of GH to GRH. Hydrocortisone replacement was then started, and this improved the patient's general condition. His hypothyroid state gradually ameliorated and his titer of anti-thyroglobulin antibody decreased to the normal range. Pituitary function was re-evaluated with GRH stimulation test under a maintenance dose of 20 mg/day hydrocortisone and showed a normal response of GH to GRH. It is suggested that re-evaluation of pituitary and thyroid function is useful for diagnosing isolated ACTH deficiency after starting a maintenance dose of hydrocortisone in order to avoid unnecessary replacement of thyroid hormone.

  5. Protein-Energy Malnutrition Developing after Global Brain Ischemia Induces an Atypical Acute-Phase Response and Hinders Expression of GAP-43: e107570

    National Research Council Canada - National Science Library

    Shari E Smith; Sarah A Figley; David J Schreyer; Phyllis G Paterson


      Protein-energy malnutrition (PEM) is a common post-stroke problem. PEM can independently induce a systemic acute-phase response, and pre-existing malnutrition can exacerbate neuroinflammation induced by brain ischemia...

  6. Protein-energy malnutrition developing after global brain ischemia induces an atypical acute-phase response and hinders expression of GAP-43

    National Research Council Canada - National Science Library

    Smith, Shari E; Figley, Sarah A; Schreyer, David J; Paterson, Phyllis G


    Protein-energy malnutrition (PEM) is a common post-stroke problem. PEM can independently induce a systemic acute-phase response, and pre-existing malnutrition can exacerbate neuroinflammation induced by brain ischemia...

  7. Atypical Cutaneous Manifestations in Syphilis. (United States)

    Ivars Lleó, M; Clavo Escribano, P; Menéndez Prieto, B


    Although the diversity of the clinical manifestations of syphilis is well-known, atypical presentations can also occur. Such atypical presentations are associated with a high risk of transmission as a result of diagnostic confusion and treatment delays owing to the disease's ability to mimic other common skin diseases, deviate from classic clinical presentations, and adopt unique forms. Cases of atypical syphilis have been described most frequently in patients with concomitant human immunodeficiency virus (HIV) infection. Because the incidence of syphilis has been growing over recent years -particularly in patients with HIV co-infection- dermatologists need to be familiar with the less well-known clinical presentations of this venereal disease. Copyright © 2015 AEDV. Published by Elsevier España, S.L.U. All rights reserved.

  8. [Therapy for atypical facial pain]. (United States)

    Ishida, Satoshi; Kimura, Hiroko


    Atypical facial pain is a pain in the head, neck and the face, without organic causes. It is treated at departments of physical medicine, such as dental, oral and maxillofacial surgery, otolaryngology, cerebral surgery, or head and neck surgery. In primary care, it is considered to be a medically unexplained symptom (MUS), or a somatoform disorder, such as somatization caused by a functional somatic syndrome (FSS) by psychiatrists. Usually, patients consult departments of physical medicine complaining of physical pain. Therefore physicians in these departments should examine the patients from the holistic perspective, and identify organic diseases. As atypical facial pain becomes chronic, other complications, including psychiatric complaints other than physical pain, such as depression may develop. Moreover, physical, psychological, and social factors affect the symptoms by interacting with one another. Therefore, in examining atypical facial pain, doctors specializing in dental, oral and maxillofacial medicine are required to provide psychosomatic treatment that is based on integrated knowledge.

  9. Behavioral Activation for the Treatment of Atypical Depression: A Pilot Open Trial (United States)

    Weinstock, Lauren M.; Munroe, Mary K.; Miller, Ivan W.


    Psychosocial interventions for atypical depression (AD) have been relatively ignored in the clinical research literature, despite evidence that the atypical subtype of major depression is marked by earlier age of onset, longer duration of mood episode, greater symptom severity, and poorer response to pharmacologic treatment. Given the symptom…

  10. Effect of estrogen receptor-alpha (ESR1 gene polymorphism on high density lipoprotein levels in response to hormone replacement therapy

    Directory of Open Access Journals (Sweden)

    N.C. Nogueira-de-Souza


    Full Text Available Studies have shown that estrogen replacement therapy and estrogen plus progestin replacement therapy alter serum levels of total, LDL and HDL cholesterol levels. However, HDL cholesterol levels in women vary considerably in response to hormone replacement therapy (HRT. A significant portion of the variability of these levels has been attributed to genetic factors. Therefore, we investigated the influence of estrogen receptor-alpha (ESR1 gene polymorphisms on HDL levels in response to postmenopausal HRT. We performed a prospective cohort study on 54 postmenopausal women who had not used HRT before the study and had no significant general medical illness. HRT consisted of conjugated equine estrogen and medroxyprogesterone acetate continuously for 1 year. The lipoprotein levels were measured from blood samples taken before the start of therapy and after 1 year of HRT. ESR1 polymorphism (MspI C>T, HaeIII C>T, PvuII C>T, and XbaI A>G frequencies were assayed by restriction fragment length polymorphism. A general linear model was used to describe the relationships between HDL levels and genotypes after adjusting for age. A significant increase in HDL levels was observed after HRT (P = 0.029. Women with the ESR1 PvuII TT genotype showed a statistically significant increase in HDL levels after HRT (P = 0.032. No association was found between other ESR1 polymorphisms and HDL levels. According to our results, the ESR1 PvuII TT genotype was associated with increased levels of HDL after 1 year of HRT.

  11. Atypical moles: diagnosis and management. (United States)

    Perkins, Allen; Duffy, R Lamar


    Atypical moles are benign pigmented lesions. Although they are benign, they exhibit some of the clinical and histologic features of malignant melanoma. They are more common in fair-skinned individuals and in those with high sun exposure. Atypical moles are characterized by size of 6 mm or more at the greatest dimension, color variegation, border irregularity, and pebbled texture. They are associated with an increased risk of melanoma, warranting enhanced surveillance, especially in patients with more than 50 moles and a family history of melanoma. Because an individual lesion is unlikely to display malignant transformation, biopsy of all atypical moles is neither clinically beneficial nor cost-effective. The ABCDE (asymmetry, border irregularity, color unevenness, diameter of 6 mm or more, evolution) mnemonic is a valuable tool for clinicians and patients to identify lesions that could be melanoma. Also, according to the "ugly duckling" concept, benign moles tend to have a similar appearance, whereas an outlier with a different appearance is more likely to be undergoing malignant change. Atypical moles with changes suggestive of malignant melanoma should be biopsied, using an excisional method, if possible.

  12. Hematological Side Effects of Atypical Antipsychotic Drugs

    Directory of Open Access Journals (Sweden)

    Serap Erdogan


    Full Text Available Atypical antipsychotics cause less frequently extrapyramidal system symptoms, neuroleptic malignant syndrome and hyperprolactinemia than typical antipsychotics. However hematological side effects such as leukopenia and neutropenia could occur during treatment with atypical antipsychotics. These side effects could lead to life threatening situations and the mortality rate due to drug related agranulocytosis is about 5-10%. There are several hypothesis describing the mechanisms underlying drug induced leukopenia and/or neutropenia such as direct toxic effects of these drugs upon the bone marrow or myeloid precursors, immunologic destruction of the granulocytes or supression of the granulopoiesis. Clozapine is the antipsychotic agent which has been most commonly associated with agranulocytosis. A nitrenium ion which is formed by the bioactivation of clozapine is thought to have an important role in the pathophysiogy of this adverse effect. Aside from clozapine, there are several case reports reporting an association between olanzapine, quetiapine, risperidone, ziprasidone, aripiprazole and leukopenia. We did not find any study or case report presenting amisulpride or sulpride related hematological side effects in our literature search. Patients who had hematological side effects during their previous antipsychotic drug treatments and who had lower baseline blood leukocyte counts, have higher risk to develop leukopenia or neutropenia during their current antipsychotic treatment. Once leukopenia and neutropenia develops, drugs thought to be responsible for this side effect should be discontinued or dosages should be lowered. In some cases iniatition of lithium or G-CSF (granulocyte colony-stimulating factor therapy may be helpful in normalizing blood cell counts. Clinicans should avoid any combination of drugs known to cause hematological side effects. Besides during antipsychotic treatment, infection symptoms such as fever, cough, sore throat or

  13. Atypical pyoderma gangrenosum in a patient with osteomyelofibrosis

    Directory of Open Access Journals (Sweden)

    Živanović Dubravka


    Full Text Available Background. Atypical forms of pyoderma gangrenosum generally appear on the upper extremities; most frequently they are associated with myeloproliferative disorders, including osteomyelofibrosis. A response to systemic steroids is more pronounced than in classical form. Sometimes it may be the first sign of an underlying malignancy. Case report. We reported a patient with atypical pyoderma gangrenosum developed during the course of a myeloid malignancy - osteomyelofibrosis. The lesions occurred after a minor trauma. Painful blistering plaques, with an elevated, bluish-gray border were located on the dorsal aspect of hands. No skin malignancy was found. The lesions resolved rapidly to systemic steroids. Conclusion. Considering the unusual clinical presentation which makes the diagnosis difficult, as well as the fact that atypical forms of pyoderma gangrenosum can be the first sign of malignancies, especially myeloproliferative ones, recognizing this entity enables timely guiding future investigations toward their prompt detection.

  14. Gender-Atypical Mental Illness as Male Gender Threat. (United States)

    Michniewicz, Kenneth S; Bosson, Jennifer K; Lenes, Joshua G; Chen, Jason I


    The present study examined whether men view gender-atypical (i.e., feminine) psychological disorders as threats to their gender status. Men and women (N = 355) rated their expectations of gender status loss, feelings of distress, and help-seeking intentions in response to 10 different stereotypically masculine and feminine psychological disorders. Men as compared to women expected greater gender status loss for, and reported more distress to, gender-atypical versus gender-typical disorders. Expectations of gender status loss partially mediated the link between participant gender and distress at the thought of gender-atypical disorders. These findings suggest that feminine disorders pose more powerful gender status threats for men than masculine disorders do and that men's expectations of gender status loss for feminine disorders drive their negative reactions to these mental illnesses. The discussion emphasizes the importance of considering the gender-typicality of disorders, and the implications of these findings for clinical interventions.

  15. Atypical chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS), primary angiitis of the CNS mimicking CLIPPERS or overlap syndrome? A case report. (United States)

    Buttmann, Mathias; Metz, Imke; Brecht, Isabel; Brück, Wolfgang; Warmuth-Metz, Monika


    A novel type of encephalomyelitis was first described as chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in 2010 and few additional patients were reported since then. Partially due to its unknown aetiology and a lack of pathognomonic features some have suggested that CLIPPERS may not represent a distinct disease, but rather a syndrome with different underlying aetiologies. Here we report a 49-year-old German female who presented with a number of clinical and paraclinical features described as typical for CLIPPERS, while additionally showing symptoms and findings compatible with primary angiitis of the CNS (PACNS). This case may establish a previously unnoted link between two poorly understood autoimmune conditions of the CNS.

  16. An atypical CD8 T-cell response to Chlamydia muridarum genital tract infections includes T cells that produce interleukin-13. (United States)

    Johnson, Raymond M; Kerr, Micah S; Slaven, James E


    Chlamydia trachomatis urogenital serovars D-K are intracellular bacterial pathogens that replicate almost exclusively in human reproductive tract epithelium. In the C. muridarum mouse model for human Chlamydia genital tract infections CD4 T helper type 1 cell responses mediate protective immunity while CD8 T-cell responses have been associated with scarring and infertility. Scarring mediated by CD8 T cells requires production of tumour necrosis factor-α (TNF-α); however, TNF-α is associated with protective immunity mediated by CD4 T cells. The latter result suggests that TNF-α in-and-of itself may not be the sole determining factor in immunopathology. CD8 T cells mediating immunopathology presumably do something in addition to producing TNF-α that is detrimental during resolution of genital tract infections. To investigate the mechanism underlying CD8 immunopathology we attempted to isolate Chlamydia-specific CD8 T-cell clones from mice that self-cleared genital tract infections. They could not be derived with antigen-pulsed irradiated naive splenocytes; instead derivation required use of irradiated immune splenocyte antigen-presenting cells. The Chlamydia-specific CD8 T-cell clones had relatively low cell surface CD8 levels and the majority were not restricted by MHC class Ia molecules. They did not express Plac8, and had varying abilities to terminate Chlamydia replication in epithelial cells. Two of the five CD8 clones produced interleukin-13 (IL-13) in addition to IL-2, TNF-α, IL-10 and interferon-γ. IL-13-producing Chlamydia-specific CD8 T cells may contribute to immunopathology during C. muridarum genital tract infections based on known roles of TNF-α and IL-13 in scar formation.

  17. Atypical Manifestation of LPS-Responsive beige- like anchor (LRBA Deficiency Syndrome as an Autoimmune Endocrine Disorder without Enteropathy and Immunodeficiency.

    Directory of Open Access Journals (Sweden)

    Shahrzad Bakhtiar


    Full Text Available Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type1 diabetes mellitus, hypothyroidism, adrenal insufficiency and vitiligo. LPS-responsive beige-like anchor protein (LRBA deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation. The third child of consanguineous Egyptian parents (Patient 1 presented at six months of age with intractable enteropathy and failure to thrive. Later on he developed symptoms of adrenal insufficiency, autoimmune haemolytic anaemia, thrombocytopenia, and infectious complications due to immunosuppressive treatment. The severe enteropathy was non-responsive to the standard treatment and led to death at the age of 22 years. His younger sister (Patient 2 presented at the age of 12 to the endocrinology department with decompensated hypothyroidism, perioral vitiligo, delayed pubertal development, and growth failure without enteropathy and immunodeficiency.Using whole-exome sequencing (WES we identified a homozygous frameshift mutation (c.6862delT, p.Y2288MfsX29 in the LRBA gene in both siblings. To our knowledge our patient (patient 2 is the first case of LRBA deficiency described with predominant endocrine phenotype without immunodeficiency and enteropathy. LRBA deficiency should be considered as underlying disease in pediatric patients presenting with autoimmune endocrine symptoms. The same genetic mutation can manifest with a broad phenotypic spectrum without genotype-phenotype correlation. The awareness for disease symptoms among non-immunologists might be a key to early diagnosis. Further functional studies in LRBA deficiency are necessary to

  18. Atypical yeasts identified as Saccharomyces cerevisiae by MALDI-TOF MS and gene sequencing are the main responsible of fermentation of chicha, a traditional beverage from Peru. (United States)

    Vallejo, Juan Andrés; Miranda, Patricia; Flores-Félix, José David; Sánchez-Juanes, Fernando; Ageitos, José M; González-Buitrago, José Manuel; Velázquez, Encarna; Villa, Tomás G


    Chicha is a drink prepared in several Andean countries from Inca's times by maize fermentation. Currently this fermentation is carried out in familiar artesanal "chicherías" that make one of the most known types of chicha, the "chicha de jora". In this study we isolate and identify the yeasts mainly responsible of the fermentation process in this type of chicha in 10 traditional "chicherías" in Cusco region in Peru. We applied by first time MALDI-TOF MS analysis for the identification of yeast of non-clinic origin and the results showed that all of yeast strains isolated belong to the species Saccharomyces cerevisiae. These results agree with those obtained after the analysis of the D1/D2 and 5.8S-ITS regions. However the chicha strains have a phenotypic profile that differed in more than 40% as compared to that of current S. cerevisiae strains. To the best of our knowledge this is the first report concerning the yeasts involved in chicha fermentation.

  19. [Estrogen replacement]. (United States)

    Søgaard, A J; Berntsen, G K; Magnus, J H; Tollan, A


    Recent research on long-term postmenopausal hormone replacement therapy (HRT) indicates a positive effect on both total mortality and morbidity. This has raised the question of widespread preventive long-term use of HRT. Possible side-effects and ideological issues related to preventive HRT have led to debate and uncertainty among health professionals, in the media, and in the population at large. In order to evaluate the level of knowledge about and attitudes towards HRT, a randomly selected group of 737 Norwegian women aged 16-79 was interviewed by the Central Bureau of Statistics. One in three women had received information about HRT in the last two years, mainly through weekly magazines and physicians. The proportion who answered the questions on knowledge correctly varied from 36% to 47%. Those who had been given information by a physician possessed accurate knowledge, had more positive attitudes towards HRT and were more willing to use HRT than women who had reviewed information through other channels. Women with a higher level of education were better informed and more knowledgeable than others, but were nevertheless more reluctant to use HRT than those who were less educated. The limited number of women who actually receive information on HRT, the low level of knowledge and the ambivalent attitudes toward HRT are a major challenge to the public health service.

  20. Atypical inheritance: new horizons for neurology. (United States)

    Wilson, G N


    Rediscovery of Mendel's laws produced an enthusiastic new discipline at the turn of this century. The eugenics movement had many disciples in the United States, and it should be noted that the term "final solution" was first used by the National Association of Charities and Corrections in the 1920s. American advocates of eugenics accomplished mass sterilization of retarded individuals and the prohibition of Jewish immigration from Germany during World War II. It is interesting that the close of this century has produced a similar revolution in genetics. These newer genetic mechanisms expose the major fallacy of eugenics: traits may be genetic without showing obvious familial transmission. Sanctions against reproduction or immigration thus will have little effect on the gene pool. The clinical implications of atypical inheritance are enormous. Almost every medical disorder must be reinvestigated for evidence of subtle chromosome changes, for worsening in progressive generations, and for influence of parental origin. The classical Mendelian model taught that extreme and rare phenotypes shed light on more frequent ones, hence the definition of genes responsible for hypercholesterolemia, for Alzheimer disease, and for amyotrophic lateral sclerosis. Atypical inheritance mechanisms further enhance this approach, bringing all of neurology under the light of genetic technology. The lure for the practitioner, then, is not the hyperbole of molecular biology; it is the need for a seasoned hand so emphasized by Huntington's disease and the duty to protect the next century from disasters of the current one.

  1. Changes in adipocyte cell size, gene expression of lipid metabolism markers, and lipolytic responses induced by dietary fish oil replacement in gilthead sea bream (Sparus aurata L.). (United States)

    Cruz-Garcia, Lourdes; Sánchez-Gurmaches, Joan; Bouraoui, Lamia; Saera-Vila, Alfonso; Pérez-Sánchez, Jaume; Gutiérrez, Joaquim; Navarro, Isabel


    The effects of fish oil (FO) substitution by 66% vegetable oils in a diet with already 75% vegetable protein (66VO) on adipose tissue lipid metabolism of gilthead sea bream were analysed after a 14-month feeding trial. In the last 3 months of the experiment, a FO diet was administrated to a 66VO group (group 66VO/FO) as a finishing diet. Hormone-sensitive lipase (HSL) activity was measured in adipose tissue and adipocyte size, and HSL, lipoprotein lipase and liver X receptor gene expression in isolated adipocytes, on which lipolysis and glucose uptake experiments were also performed. Lipolysis was measured after incubation with tumour necrosis factor-α (TNFα), linoleic acid, and two conjugated linoleic acid isomers. Glucose uptake was analysed after TNFα or insulin administration. Our results show that FO replacement increased lipolytic activity and adipocyte cell size. The higher proportion of large cells observed in the 66VO group could be involved in their observed lower response to fatty acid treatments and lower insulin sensitivity. The 66VO/FO group showed a moderate return to the FO conditions. Therefore, FO replacement can affect the morphology and metabolism of gilthead sea bream adipocytes which could potentially affect other organs such as the liver.

  2. Effects of residual feed intake and dam body weight on replacement heifer intake, efficiency, performance, and metabolic response. (United States)

    Walker, R S; Martin, R M; Buttrey, B


    70 combined T4 (r = 0.32), respectively. Heifers out of dams from the HEV BW group were heavier and a positive correlation existed between dam BW and heifer BW, gain, DMI, and FS, which can impact selection goals for replacement heifers.

  3. The GnRH receptor and the response of gonadotrope cells to GnRH pulse frequency code. A story of an atypical adaptation of cell function relying on a lack of receptor homologous desensitization.

    Directory of Open Access Journals (Sweden)

    Christian Bleux


    Full Text Available Brain control of the reproductive system is mediated through hypothalamic gonadotropin-releasing hormone (GnRH which activates specific receptors (GnRHR present at the surface of the pituitary gonadotropes to trigger secretion of the two gonadotropins LH and FSH. A unique feature of this system is the high dependence on the secretion mode of GnRH, which is basically pulsatile but undergoes considerable fluctuations in pulse frequency pattern in response to endogenous or external factors. How the physiological fluctuations of GnRH secretion that orchestrate normal reproduction are decoded by the gonadotrope cell machinery to ultimately control gonadotropin release and/or subunit gene transcription has been the subject of intensive studies during the past decades. Surprisingly, the mammalian GnRHR is unique among G protein-coupled receptor family as it lacks the carboxy-terminal tail usually involved in classical endocytotic process. Accordingly, it does not desensitize properly and internalizes very poorly. Both this atypical intrinsic property and post-receptor events may thus contribute to decode the GnRH signal. This includes the participation of a network of signaling pathways that differently respond to GnRH together with a growing amount of genes differentially sensitive to pulse frequency. Among these are two pairs of genes, the transcription factors EGR-1 and NAB, and the regulatory factors activin and follistatin, that function as intracellular autoregulatory feedback loops controlling respectively LHbeta and FSHbeta gene expression and hence, LH and FSH synthesis. Pituitary gonadotropes thus represent a unique model of cells functionally adapted to respond to a considerably fluctuating neuroendocrine stimulation, from short individual pulses to sustained GnRH as observed at the proestrus of ovarian cycle. Altogether, the data emphasize the adaptative reciprocal complementarity of hypothalamic GnRH neurones and pituitary gonadotropes to

  4. Atypical Steatocystoma Multiplex with Calcification (United States)

    Rahman, Muhammad Hasibur; Islam, Muhammad Saiful; Ansari, Nazma Parvin


    A 60-year-old male reported to us with an atypical case of giant steatocystoma multiplex in the scrotum with calcification. There was no family history of similar lesions. Yellowish, creamy material was expressed from a nodule during punch biopsy. The diagnosis was based on clinical as well as histological findings. Successful surgical excision was done to cure the case without any complications. PMID:22363850

  5. Atypical Centrioles During Sexual Reproduction

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    Tomer eAvidor-Reiss


    Full Text Available Centrioles are conserved, self-replicating, microtubule-based 9-fold symmetric subcellular organelles that are essential for proper cell division and function. Most cells have two centrioles and maintaining this number of centrioles is important for animal development and physiology. However, how animals gain their first two centrioles during reproduction is only partially understood. It is well established that in most animals, the centrioles are contributed to the zygote by the sperm. However, in humans and many animals, the sperm centrioles are modified in their structure and protein composition, or they appear to be missing altogether. In these animals, the origin of the first centrioles is not clear. Here, we review various hypotheses on how centrioles are gained during reproduction and describe specialized functions of the zygotic centrioles. In particular, we discuss a new and atypical centriole found in sperm and zygote, the proximal centriole-like structure (PCL. We also discuss another type of atypical centriole, the zombie centriole, which is degenerated but functional. Together, the presence of centrioles, PCL, and zombie centrioles suggests a universal mechanism of centriole inheritance among animals and new causes of infertility. Since the atypical centrioles of sperm and zygote share similar functions with typical centrioles in somatic cells, they can provide unmatched insight into centriole biology.

  6. Atypical Imaging Appearances of Meningioma

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    Ahmad Soltani shirazi


    Full Text Available "nIntroduction: Meningiomas are the commonest primary non-glial intracranial tumors. The diagnosis is usually correctly established on characteristic imaging appearances. Atypical meningiomas may be difficult to diagnose because of their similarity to other brain tumors. This paper presents one case of atypical meningioma, misdiagnosed primarily as glioblastoma multiforms (GBM by radiological techniques. "nCase report: A 15-year-old girl presented with a severe intermittent generalized headache that on occasion localized to retro-orbital and vertex. Other manifestations were blurred vision, photophobia, diplopia, weakness and clumsiness of the right hand. The result of systemic and neurological examinations was normal, except for a positive right hand drift test. MRI showed a large lobulated mass with peripheral edema, central necrosis and a heterogenous enhancement at the central part of the parietal lobe inducing to subfalcian herniation. Glioblastoma multiforms (GBM was misdiagnosed for the patient on the basis of MRI appearance. Pathology evaluation was compatible with meningioma (WHO grade I to II. The patient was operated and discharged with minimal right hand weakness. Physiotherapy was recommended to improve the remaining problems. "nConclusion: Atypical meningiomas may mimic other intracranical brain lesions and may cause misdiagnosis. It is important to be aware of these features in order to avoid misdiagnosis. "n"n  

  7. High Spinal Anesthesia Enhances Anti-Inflammatory Responses in Patients Undergoing Coronary Artery Bypass Graft Surgery and Aortic Valve Replacement: Randomized Pilot Study. (United States)

    Lee, Trevor W R; Kowalski, Stephen; Falk, Kelsey; Maguire, Doug; Freed, Darren H; HayGlass, Kent T


    Cardiac surgery induces many physiologic changes including major inflammatory and sympathetic nervous system responses. Here, we conducted a single-centre pilot study to generate hypotheses on the potential immune impact of adding high spinal anaesthesia to general anaesthesia during cardiac surgery in adults. We hypothesized that this strategy, previously shown to blunt the sympathetic response and improve pain management, could reduce the undesirable systemic inflammatory responses caused by cardiac surgery. This prospective randomized unblinded pilot study was conducted on 14 patients undergoing cardiac surgery for coronary artery bypass grafting and/or aortic valve replacement secondary to severe aortic stenosis. The primary outcome measures examined longitudinally were serum pro-inflammatory (IL-6, IL-1b, CCL2), anti-inflammatory (IL-10, TNF-RII, IL-1Ra), acute phase protein (CRP, PTX3) and cardiovascular risk (sST2) biomarkers. The kinetics of pro- and anti-inflammatory biomarker was determined following surgery. All pro-inflammatory and acute phase reactant biomarker responses induced by surgical stress were indistinguishable in intensity and duration between control groups and those who also received high spinal anaesthesia. Conversely, IL-10 levels were markedly elevated in both intensity and duration in the group receiving high spinal anesthesia (p = 0.005). This hypothesis generating pilot study suggests that high spinal anesthesia can alter the net inflammatory response that results from cardiac surgery. In appropriately selected populations, this may add incremental benefit by dampening the net systemic inflammatory response during the week following surgery. Larger population studies, powered to assess immune, physiologic and clinical outcomes in both acute and longer term settings, will be required to better assess potential benefits of incorporating high spinal anesthesia. NCT00348920.

  8. A family of congenital hepatic fibrosis and atypical retinitis pigmentosa

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    Sunil Pawar


    Full Text Available Congenital hepatic fibrosis is a rare cause of portal hypertension and esophageal varices in children. We report cases of siblings with biopsy proven congenital hepatic fibrosis and with atypical retinitis pigmentosa. They presented with repeated episodes of jaundice along with progressive decrease of vision in night. They had hepatosplenomegaly and portal hypertension with esophageal varices. One of the siblings had a large regenerating nodule replacing the entire right lobe of the liver and other one developed repeated hematemesis. This constellation of diagnosis belongs to the ciliopathy group of disorders. The spectrum of ciliopathy disorders has been evolving, and it varies from mild to severe manifestations.

  9. Combined treatment of ulinastatin and tranexamic acid provides beneficial effects by inhibiting inflammatory and fibrinolytic response in patients undergoing heart valve replacement surgery. (United States)

    Chen, Ting-Ting; Jiandong-Liu; Wang, Gang; Jiang, Sheng-Li; Li, Li-Bing; Gao, Chang-Qing


    To investigate the effect of ulinastatin and tranexamic acid administered alone or in combination on inflammatory cytokines and fibrinolytic system in patients undergoing heart valve replacement surgery during cardiopulmonary bypass (CPB). CPB-induced fibrinolytic hyperfunction and systemic inflammatory response syndrome (SIRS) are the leading causes responsible for the occurrence of postsurgical complications such as postsurgical cardiac insufficiency and lung injury, which may lead to an increase in postsurgical bleeding, prolongation of hospital stay, and increased costs. One hundred twenty patients undergoing heart valve replacement surgery during CPB were randomly assigned into 4 groups of 30 patients each: blank control group (Group C), tranexamic acid group (Group T), ulinastatin group (Group U), and tranexamic acid-ulinastatin combination group (Group D). Physiological saline, tranexamic acid, ulinastatin, and a combination of tranexamic acid and ulinastatin were given to each group, respectively. Arterial blood was collected from the radial artery at 4 time points: after induction of anesthesia (T1), unclamping the ascending aorta (T2), and at 1 hour (T3) and 24 hours (T4) after CPB. The levels of plasma tumor necrosis factor alpha (TNF-α), interleukin 6 (IL-6), neutrophil elastase (NE), and the concentrations of tissue plasminogen activator (t-PA) and α2-antiplasmin (α2-AP) were detected. The changes in the volume of pericardial mediastinal drainage after surgery were observed and recorded. The plasma TNF-α, IL-6, and NE levels significantly increased in patients from all 4 groups at time points of T2, T3, and T4 in comparison to those before CPB (P surgery (P surgery.

  10. Radical treatment strategies improve the long-term outcome of recurrent atypical meningiomas

    Institute of Scientific and Technical Information of China (English)

    LI Fei; LAI Zhao-pan; LIN Jiang-kai; ZHU Gang; FENG Hua


    Background Atypical meningioma is one of the rare subtypes of meningioma, which is lacking of optimal consensus on treatment strategies. This study aimed to investigate the radical treatment strategies to improve the long-term outcome of recurrent atypical meningiomas. Methods The prognostic factors including the age and gender of patients; the location, histology, recurrence pattern and mitotic cell rate of the tumors; and the resection extents, surgical strategies and adjuvant therapies of 15 cases of recurrent atypical meningiomas were analyzed retrospectively. Results The age and gender of patients were not associated with tumor recurrence. However, high recurrence rates and poor prognosis for atypical meningiomas were associated with the high mitotic cell rate, failure to achieve Simpson gradeⅠ-Ⅱ resection, and without the dura and bone flap replacement intraoperatively. Post-operative radiotherapy improved the outcomes of tumors in patients after the second surgery. Conclusion Radical treatment strategies such as dura and bone flap replacements and radiotherapy should be considered in patients diagnosed with atypical meningiomas.

  11. Atypical Bacteria and Macrolides in Asthma

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    Xepapadaki Paraskevi


    Full Text Available Chlamydophila pneumoniae and Mycoplasma pneumoniae are common pathogens causing acute illness in both the upper and lower airways. Several observations are supportive of a possible causative role of these pathogens in asthma; however, more evidence is required before this becomes meaningful in clinical practice. Atypical bacteria can enhance airway hyperresponsiveness and inflammation, both of which have been associated with exacerbations in patients with preexisting asthma. It is less clear whether the above mechanisms might also be responsible for the development of asthma. Difficulties in accurately diagnosing these infections contribute to such uncertainty. In the present report, evidence of the involvement of Chlamydophila and Mycoplasma infection in the development and the progression of asthma are reviewed.

  12. Treatment options for atypical optic neuritis

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    Amina Malik


    Full Text Available Context: Optic neuritis (ON is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or "typical" ON, usually associated with multiple sclerosis. This is in contrast to "atypical" causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision. Aims: The aim was to describe the clinical presentations of atypical ON and their treatments. Settings and Design: Review article. Materials and Methods: Literature review. Results: Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression. Conclusions: Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

  13. Atypical disease phenotypes in pediatric ulcerative colitis

    DEFF Research Database (Denmark)

    Levine, Arie; de Bie, Charlotte I; Turner, Dan


    Definitive diagnosis of pediatric ulcerative colitis (UC) may be particularly challenging since isolated colitis with overlapping features is common in pediatric Crohn's disease (CD), while atypical phenotypes of UC are not uncommon. The Paris classification allows more accurate phenotyping...... of atypical inflammatory bowel disease (IBD) patients. Our aim was to identify the prevalence of atypical disease patterns in new-onset pediatric UC using the Paris classification....

  14. Using a retrospective pretest instead of a conventional pretest is replacing biases: a qualitative study of cognitive processes underlying responses to thentest items. (United States)

    Taminiau-Bloem, Elsbeth F; Schwartz, Carolyn E; van Zuuren, Florence J; Koeneman, Margot A; Visser, Mechteld R M; Tishelman, Carol; Koning, Caro C E; Sprangers, Mirjam A G


    The thentest design aims to detect and control for recalibration response shift. This design assumes (1) more consistency in the content of the cognitive processes underlying patients' quality of life (QoL) between posttest and thentest assessments than between posttest and pretest assessments; and (2) consistency in the time frame and description of functioning referenced at pretest and thentest. Our objective is to utilize cognitive interviewing to qualitatively examine both assumptions. We conducted think-aloud interviews with 24 patients with cancer prior to and after radiotherapy to elicit cognitive processes underlying their assessment of seven EORTC QLQ-C30 items at pretest, posttest and thentest. We used an analytic scheme based on the cognitive process models of Tourangeau et al. and Rapkin and Schwartz that yielded five cognitive processes. We subsequently used this input for quantitative analysis of count data. Contrary to expectation, the number of dissimilar cognitive processes between posttest and thentest was generally larger than between pretest and posttest across patients. Further, patients considered a range of time frames when answering the thentest questions. Moreover, patients' description at the thentest of their pretest functioning was often not similar to that which was noted at pretest. Items referring to trouble taking a short walk, overall health and QoL were most often violating the assumptions. Both assumptions underlying the thentest design appear not to be supported by the patients' cognitive processes. Replacing the conventional pretest-posttest design with the thentest design may simply be replacing one set of biases with another.

  15. Atypical Manifestation of Vestibular Schwannoma

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    Webster, Guilherme


    Full Text Available Introduction: Vestibular schwannoma (also known as acoustic neuroma is a benign tumor whose cells are derived from Schwann sheaths, which commonly occurs from the vestibular portion of the eighth cranial nerve. Furthermore, vestibular schwannomas account for ∼8% of intracranial tumors in adults and 80 to 90% of tumors of the cerebellopontine angle. Its symptoms are varied, but what stands out most is a unilateral sensorineural hearing loss, with a low index of speech recognition. Objective: Describe an atypical manifestation of vestibular schwannoma. Case Report: The 46-year-old woman had vertigo and binaural hearing loss and fullness, with ear, nose, and throat examination suggestive of cochlear injury. After 6 months, the patient developed worsening of symptoms and onset of right unilateral tinnitus. In further exams the signs of cochlear damage remained, except for the vestibular test (hyporeflexia. Magnetic resonance imaging showed an expansive lesion in the right cerebellopontine angle. Discussion: This report warns about the atypical manifestations of vestibular schwannoma, which must always be remembered in investigating and diagnosing hearing loss.

  16. A case of atypical progressive supranuclear palsy

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    Spaccavento S


    Full Text Available Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy Background: Progressive supranuclear palsy (PSP is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS and aphasia. Aim: We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments. Methods: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results: Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20, low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall, and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion: Our case highlights the heterogeneity of the clinical features in

  17. A Study of the Familial and Career Attitudes of College Women Enrolled in Typical and Atypical Programs. (United States)

    Wilson, Marian L.

    A study was conducted to determine if a disparity exists between the familial and occupational attitudes of women in typical and atypical careers. Questionnaire responses of 225 undergraduate women in three typical careers (home economics, nursing, and elementary education) and three atypical careers (engineering, pharmacy, and agriculture)…

  18. Ulnar head replacement. (United States)

    Herbert, Timothy J; van Schoonhoven, Joerg


    Recent years have seen an increasing awareness of the anatomical and biomechanical significance of the distal radioulnar joint (DRUJ). With this has come a more critical approach to surgical management of DRUJ disorders and a realization that all forms of "excision arthroplasty" can only restore forearm rotation at the expense of forearm stability. This, in turn, has led to renewed interest in prosthetic replacement of the ulnar head, a procedure that had previously fallen into disrepute because of material failures with early implants, in particular, the Swanson silicone ulnar head replacement. In response to these early failures, a new prosthesis was developed in the early 1990s, using materials designed to withstand the loads across the DRUJ associated with normal functional use of the upper limb. Released onto the market in 1995 (Herbert ulnar head prosthesis), clinical experience during the last 10 years has shown that this prosthesis is able to restore forearm function after ulnar head excision and that the materials (ceramic head and noncemented titanium stem), even with normal use of the limb, are showing no signs of failure in the medium to long term. As experience with the use of an ulnar head prosthesis grows, so does its acceptance as a viable and attractive alternative to more traditional operations, such as the Darrach and Sauve-Kapandji procedures. This article discusses the current indications and contraindications for ulnar head replacement and details the surgical procedure, rehabilitation, and likely outcomes.

  19. Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

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    Y. T. Udaka


    Full Text Available Atypical Teratoid Rhabdoid Tumor (ATRT is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.


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    E. A. Roslavtseva


    Full Text Available Celiac disease has traditionally been associated with severe malabsorption syndrome. Recent years it was shown that among children of preschool and school-age mild cases with atypical clinical picture were dominated that leads to diagnostic difficulties. Here we are citing an example of an atypical clinical/latent celiac disease course in a child aged 4.5 years.

  1. Constrictive Pericarditis Associated with Atypical Antipsychotics

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    Kuan-chin Jean Chen


    Full Text Available We report the successful surgical intervention in a case of constrictive pericarditis after long-term use of atypical antipsychotics. Pericarditis developed in our patient with a longstanding history of schizophrenia treated with atypical antipsychotics. Pericardiectomy was undertaken, and the patient's presenting symptom of shortness of breath resolved subsequently with an uneventful postoperative course.

  2. Assessment of yeast cell wall as replacements for antibiotic growth promoters in broiler diets: effects on performance, intestinal histo-morphology and humoral immune responses. (United States)

    Ghosh, T K; Haldar, S; Bedford, M R; Muthusami, N; Samanta, I


    The study compared the effects of an antibiotic growth promoter (AGP), yeast (Saccharomyces cerevisiae) and yeast cell wall (YCW) on performance, microbiology and histo-morphology of the small intestine and humoral immune responses in Ross 308 broilers. The treatments (eight replicates/treatment, n = 12/replicate) were negative control (NC, without AGP), positive control (PC, supplemented with bacitracin methylene disalicylate, 400 mg/kg), Y and YCW (supplemented with yeast and YCW, respectively, 1000 mg/kg). Live weight at 42 days improved (p = 0.086) in the PC, Y and YCW groups. Feed conversion ratio was better (p = 0.039) in the YCW group compared with the other groups. Antibiotic growth promoter in the PC group shortened the villi in duodenum (p = 0.044). Mucosal Escherichia coli number was higher in the PC group (p Yeast cell wall -treated birds exhibited better (p yeast and the yeast cell wall may have effects identical to BMD on performance of broilers and thus may constitute an effective replacement strategy in the dietary regimens for broiler chickens.

  3. Atypical moral judgment following traumatic brain injury

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    Angelica Muresan


    Full Text Available Previous research has shown an association between emotions, particularly social emotions, and moral judgments. Some studies suggested an association between blunted emotion and the utilitarian moral judgments observed in patients with prefrontal lesions. In order to investigate how prefrontal brain damage affects moral judgment, we asked a sample of 29 TBI patients (12 females and 17 males and 41 healthy participants (16 females and 25 males to judge 22 hypothetical dilemmas split into three different categories (non-moral, impersonal and personal moral. The TBI group presented a higher proportion of affirmative (utilitarian responses for personal moral dilemmas when compared to controls, suggesting an atypical pattern of utilitarian judgements. We also found a negative association between the performance on recognition of social emotions and the proportion of affirmative responses on personal moral dilemmas. These results suggested that the preference for utilitarian responses in this type of dilemmas is accompanied by difficulties in social emotion recognition. Overall, our findings suggest that deontological moral judgments are associated with normal social emotion processing and that frontal lobe plays an important role in both emotion and moral judgment.

  4. Infective endocarditis following transcatheter aortic valve replacement-

    DEFF Research Database (Denmark)

    Loh, Poay Huan; Bundgaard, Henning; S�ndergaard, Lars


    Transcatheter aortic valve replacement (TAVR) can improve the symptoms and prognosis of patients with severe aortic stenosis who, due to a high expected operative risk, would not have otherwise been treated surgically. If these patients develop prosthetic valve endocarditis, their presentations may...... be atypical causing a delay in the diagnosis and treatment. The management is also complicated by their comorbidities, and surgical treatment may not be feasible leading to a significant morbidity and mortality. We describe a case of an 85-year-old man with TAVI prosthetic valve endocarditis successfully...

  5. Atypical presentations of celiac disease

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    Balasa Adriana Luminita


    Full Text Available In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016 in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

  6. Effects of changing milk replacer feedings from twice to once daily on Holstein calf innate immune responses before and after weaning. (United States)

    Hulbert, L E; Cobb, C J; Carroll, J A; Ballou, M A


    The objectives of this study were to determine the effects of switching Holstein calves to once-daily feeding during the fourth week of life (24 ± 2.3 d of age; once-fed n=22; twice-fed n=22) on innate immune responses, and to evaluate whether carry-over effects occurred when the calves were weaned during the seventh week of life. Peripheral blood samples were taken immediately before the change in feeding strategy (24 d of age) and at 27, 31, 45, 48, 52, and 66 d of age and were analyzed for circulating cortisol, haptoglobin, total leukocyte counts, neutrophil:mononuclear cells, and hematocrit percentage. Heparinized whole blood was also stimulated with lipopolysaccharide (LPS) for 24h and the concentration of tumor necrosis factor-α (TNF-α) in the supernatant was analyzed. Neutrophil L-selectin and β(2)-integrin expression were analyzed by flow cytometry. Simultaneous neutrophil phagocytic and oxidative burst responses to a heat-killed Escherichia coli were quantified by dual-color flow-cytometry. Treatment (once-daily or twice daily feeding) had no effect on pre- or postweaning performance. Once-fed calves tended to have more circulating neutrophils at 27 d of age, greater expression of L-selectin on neutrophils at 31 and 45 d of age, and greater intensity of phagocytosis at 45 d of age. Once-fed calves secreted less TNF-α in LPS-stimulated whole blood cultures at 45 d of age compared with twice-fed calves and this tended to persist through the immediate postweaning period. None of the other immune parameters differed after weaning between the preweaning feeding strategies. Consolidating calf milk replacer into one feeding during the fourth week of life was likely a mild and acute stressor, as evidenced by transient neutrophilia in the absence of suppressed functional capacities of neutrophils. Future research should address the mechanism and immunological significance of the persistent decreased TNF-α response in once-fed calves.

  7. Atypical Takotsubo syndrome during anagrelide therapy. (United States)

    Proietti, Riccardo; Rognoni, Andrea; Ardizzone, Fabio; Maccio, Sergio; Santagostino, Alberto; Rognoni, Giorgio


    Anagrelide is a phosphodiesterase III inhibitor utilized in the treatment of essential thrombocythemia. Anagrelide can be responsible for positive inotropic and chonotropic activity of the cardiovascular system. Moreover, it can induce vasospam directly on the epicardial coronary arteries. In the literature, it is well reported that this inhibitor can determine serious cardiovascular side effects, including congestive heart failure, arrhythmia and acute coronary syndrome. We describe the case of a 75-year-old woman who developed a mid-ventricular Takotsubo syndrome while on anagrelide therapy. Takotsubo cardiomyopathy, also known as left ventricular ballooning syndrome, is characterized by a reversible ventricular contractile dysfunction with akinesis and expansion of apical segments and hyperkinesis of the basal segments. Recently, atypical cases with akinesia and dilation of mid-ventricular segment and hypercontraction of the apical segments, also called mid-ventricular and inverted Takotsubo syndrome, have been described. Even though the pathogenesis of Takotsubo syndrome is poorly understood, several mechanisms have been proposed, including catecholamine-induced myocardial stunning, and ischemia-mediated stunning due to multivessel epicardial or microvascular spasm. We think that in our case, the adverse response of anagrelide therapy was determined, by accumulated dosage of the drug, through an intensive inotropic stimulation and a sympathetic hyperactivation in a vulnerable myocardium. To our knowledge, this is one of the first reports of an association between anagrelide therapy and Takotsubo cardiomyopathy.

  8. Pseudoarthrosis in atypical femoral fracture: case report. (United States)

    Giannotti, S; Bottai, V; Dell'Osso, G; De Paola, G; Ghilardi, M; Guido, G


    Atypical femoral fractures can be subsequent to a long-term biphosphonates treatment; they have a high frequency of delayed healing. The authors describe a femoral pseudoarthrosis of an atypical fracture treated with intramedullary nailing in a female after prolonged alendronate therapy. Atypical femoral fractures can be subsequent to a long-term biphosphonates treatment even if, in the literature, there is no clarity on the exact pathogenetic mechanism. The Task Force of the American Society for Bone and Mineral Research described the major and minor features to define atypical fractures and recommends that all the five major features must be present while minor features are not necessary. Another controversial aspect regarding the atypical femoral fractures is the higher frequency of the delayed healing that can be probably related to a suppressed bone turnover caused by a prolonged period of bisphosphonates treatment. This concept could be corroborated by the Spet Tc exam. In the case of a pseudoarthrosis, there is not a standardization of the treatment. In this report, the authors describe a femoral pseudoarthrosis of an atypical fracture treated with intramedullary nailing in a female after prolonged alendronate therapy; the patient was studied with clinical, bioumoral end SPECT-Tc exam of both femurs. Many studies show the relationship between bisphosphonates and the presence of atypical fractures. These fractures should be monitored more closely due to the risk of nonunion and they require considering an initial treatment with pharmacological augmentation to reduce the complications for the patient and the health care costs.

  9. Atypicalities in cortical structure, handedness, and functional lateralization for language in autism spectrum disorders. (United States)

    Lindell, Annukka K; Hudry, Kristelle


    Language is typically a highly lateralized function, with atypically reduced or reversed lateralization linked to language impairments. Given the diagnostic and prognostic role of impaired language for autism spectrum disorders (ASDs), this paper reviews the growing body of literature that examines patterns of lateralization in individuals with ASDs. Including research from structural and functional imaging paradigms, and behavioral evidence from investigations of handedness, the review confirms that atypical lateralization is common in people with ASDs. The evidence indicates reduced structural asymmetry in fronto-temporal language regions, attenuated functional activation in response to language and pre-linguistic stimuli, and more ambiguous (mixed) hand preferences, in individuals with ASDs. Critically, the evidence emphasizes an intimate relationship between atypical lateralization and language impairment, with more atypical asymmetries linked to more substantive language impairment. Such evidence highlights opportunities for the identification of structural and functional biomarkers of ASDs, affording the potential for earlier diagnosis and intervention implementation.

  10. Relations between A-type granites and copper mineralization as exemplified by the Machangqing Cu deposit

    Institute of Scientific and Technical Information of China (English)

    毕献武; 胡瑞忠; 叶造军; 邵树勋


    This paper deals with the relations between the Machangqing rockbody which corresponds to the A-type granites and porphyry copper mineralization in terms of petrochemistry, trace element geochemistry, fluid inclusion geochemistry and isotope geochemistry. The results show that the Machangqing porphyry copper deposit was formed from the fluid predominated by mag-matic fluid. This kind of ore-forming fluid was just differentiated from the magma responsible for the A-type granites. Therefore, as viewed from whether they contain water or not, the A-type granites can, at least, be divided into two types: water-bearing and water-free. The water-bearing A-type granites can serve as the host of porphyry copper deposits under certain geological conditions.

  11. Relations between A-type granites and copper mineralization as exemplified by the Machangqing Cu deposit

    Institute of Scientific and Technical Information of China (English)


    This paper deals with the relations between the Machangqing rockbody which corresponds to the A-type granites and porphyry copper mineralization in terms of petrochemistry,trace element geochemistry,fluid inclusion geochemistry and isotope geochemistry.The results show that the Machangqing porphyry copper deposit was formed from the fluid predominated by magmatic fluid.This kind of ore-forming fluid was just differentiated from the magma responsible for the A-type granites.Therefore,as viewed from whether they contain water or not,the A-type granites can,at least,be divided into two types:water-bearing and water-free.The water-bearing A-type granites can serve as the host of porphyry copper deposits under certain geological conditions.

  12. Bisphosphonate-associated atypical subtrochanteric femur fracture. (United States)

    Wolin, Ely A; Banks, Kevin P; Vroman, Penny J


    Bisphosphonates help prevent progressive bone mineralization loss and subsequent osteoporotic fractures. However, long-term bisphosphonate therapy paradoxically increases the risk of a unique injury called an atypical subtrochanteric femur fracture. Despite this, the benefits of bisphosphonates outweigh the risks, because far more pathologic fractures are prevented than induced. The early identification of atypical subtrochanteric femur fractures is important as there is high associated morbidity and mortality. We describe a case of a 76-y-old woman with a completed bisphosphonate-associated atypical subtrochanteric femur fracture.

  13. Atypical CT findings in bacterial meningoencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Fink, I.J.; Dillon, W.P.; Brant-Zawadzki, M.; Danziger, A.; Rechthand, E.


    Computed tomography has become a valuable imaging modality in the evaluation and management of most intracerebral infections. We report two cases of intracranial infections with atypical CT findings, and attempt to correlate these findings with the pathophysiology.

  14. Atypical imaging appearances of intracranial meningiomas

    Energy Technology Data Exchange (ETDEWEB)

    O' Leary, S. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Adams, W.M. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Parrish, R.W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Mukonoweshuro, W. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom)]. E-mail:


    Meningiomas are the commonest primary, non-glial intracranial tumours. The diagnosis is often correctly predicted from characteristic imaging appearances. This paper presents some examples of atypical imaging appearances that may cause diagnostic confusion.

  15. Molecular insights into the evolutionary pathway of Vibrio cholerae O1 atypical El Tor variants. (United States)

    Kim, Eun Jin; Lee, Dokyung; Moon, Se Hoon; Lee, Chan Hee; Kim, Sang Jun; Lee, Jae Hyun; Kim, Jae Ouk; Song, Manki; Das, Bhabatosh; Clemens, John D; Pape, Jean William; Nair, G Balakrish; Kim, Dong Wook


    Pandemic V. cholerae strains in the O1 serogroup have 2 biotypes: classical and El Tor. The classical biotype strains of the sixth pandemic, which encode the classical type cholera toxin (CT), have been replaced by El Tor biotype strains of the seventh pandemic. The prototype El Tor strains that produce biotype-specific cholera toxin are being replaced by atypical El Tor variants that harbor classical cholera toxin. Atypical El Tor strains are categorized into 2 groups, Wave 2 and Wave 3 strains, based on genomic variations and the CTX phage that they harbor. Whole-genome analysis of V. cholerae strains in the seventh cholera pandemic has demonstrated gradual changes in the genome of prototype and atypical El Tor strains, indicating that atypical strains arose from the prototype strains by replacing the CTX phages. We examined the molecular mechanisms that effected the emergence of El Tor strains with classical cholera toxin-carrying phage. We isolated an intermediary V. cholerae strain that carried two different CTX phages that encode El Tor and classical cholera toxin, respectively. We show here that the intermediary strain can be converted into various Wave 2 strains and can act as the source of the novel mosaic CTX phages. These results imply that the Wave 2 and Wave 3 strains may have been generated from such intermediary strains in nature. Prototype El Tor strains can become Wave 3 strains by excision of CTX-1 and re-equipping with the new CTX phages. Our data suggest that inter-chromosomal recombination between 2 types of CTX phages is possible when a host bacterial cell is infected by multiple CTX phages. Our study also provides molecular insights into population changes in V. cholerae in the absence of significant changes to the genome but by replacement of the CTX prophage that they harbor.

  16. Atypical presentations of Wolframs syndrome

    Directory of Open Access Journals (Sweden)

    S Saran


    Full Text Available Background: Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. Case 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. Case 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. Case 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome. Conclusion: Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

  17. Atypical Neurological Manifestations Of Hypokalemia

    Directory of Open Access Journals (Sweden)

    pal P K


    Full Text Available A part from the well-established syndrome of motor paralysis, hypokalemia may present with atypical neurological manifestations, which are not well documented in literature. Methods: We treated 30 patients of hypokalemia whose neurological manifestations improved after corrections of hypokalemia. A retrospective chart review of the clinical profile was done with emphasis on the evolution of symptoms and occurrence of unusual manifestations. Results: Twenty-eight patients had subacute quadriparesis with duration of symptoms varying from 10hrs to 7 days and two had slowly progressive quadriparesis. Fifty percent of patients had more than one attack of paralysis. Early asymmetric weakness (11, stiffness and abnormal posture of hands (7, predominant bibrachial weakness (4, distal paresthesias (4, hemiparesthesia (1, hyperreflexia(4, early severe weakness of neck muscles (3, chorea (1, trismus (1,and, retention of urine (1 were the unusual features observed. The means level of serum potassium on admission was 2.1+0.6mEq/L.and the serum creatine kinase was elevated in 14 out of 17 patients. All patients except two had complete recovery.

  18. Atypical presentations of neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    Douglas Sato


    Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4 has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.


    Directory of Open Access Journals (Sweden)

    Ashish Chauhan*, Amit Mittal, Pradeep Kumar Arora


    Full Text Available Mental illness constitutes the second-largest disease burden in the United States. Psychosis is one of the most common and severe mental illnesses. It is an extremely devastating condition characterised by delusions, hallucinations, distortion of thoughts and deteriorating social functioning experiences. Psychosis in all human societies has approximately same incidence of occurrence as in accordance to “anthropo-parity principle.” It has large economic impact on various aspects of cognition, health, and quality of life which has devastated effects on its sufferers and facing them large economic burden. Psychosis (Schizophrenia is associated with an imbalance of the dopaminergic system, entailing hyper-stimulation of dopamine function in the brain, particularly in the mesolimbic pathway. Consequences of antipsychotic treatment are far reaching and expensive. Detrimental extrapyramidal side effects associated with conventional antipsychotics and non-compliance among patients limits long term treatment with conventional antipsychotics. It gives rise to a new class, atypical antipsychotics owning low propensity to cause EPS, efficacy against refractory cases and better control over negative symptoms, better tolerance and compliance along with lower relapse rate and safer adverse effect profile. Atypical antipsychotics have revolutionized the treatment of psychosis, now being the treatment of choice for patients with psychosis. The positive therapeutic experience with the atypical antipsychotics in the treatment of psychosis and their favourable effects outweighs their unfavourable adverse effects. Though atypical antipsychotics are widely prescribed in the treatment of schizophrenia, however not a single atypical antipsychotic drug having any exceptional efficacy and safety profile. Thus, there is still a lot of research needed to be carried out in the development of novel atypical antipsychotics. This review is comprehensive appraisal about

  20. Critical appraisal of eculizumab for atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Palma LMP


    Full Text Available Lilian M Pereira Palma,1 Craig B Langman2  1Pediatric Nephrology, State University of Campinas (UNICAMP, Campinas, São Paulo, Brazil; 2The Feinberg School of Medicine, Northwestern University, and the Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA Abstract: The biology of atypical hemolytic uremic syndrome has been shown to involve inability to limit activation of the alternative complement pathway, with subsequent damage to systemic endothelial beds and the vasculature, resulting in the prototypic findings of a thrombotic microangiopathy. Central to this process is the formation of the terminal membrane attack complex C5b-9. Recently, application of a monoclonal antibody that specifically binds to C5, eculizumab, became available to treat patients with atypical hemolytic uremic syndrome, replacing plasma exchange or infusion as primary therapy. This review focuses on the evidence, based on published clinical trials, case series, and case reports, on the efficacy and safety of this approach. Keywords: acute kidney injury, ESRD, thrombotic microangiopathy, kidney, alternative complement pathway, complement blockade

  1. Atypical relapse of hemolytic uremic syndrome after transplantation. (United States)

    Olie, Karolien H; Florquin, Sandrine; Groothoff, Jaap W; Verlaak, René; Strain, Lisa; Goodship, Timothy H J; Weening, Jan J; Davin, Jean-Claude


    Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of immediate recurrent HUS. Prophylactic plasma exchange before and after transplantation was used. Two months after transplantation, concomitant with a reduction in plasma exchange frequency, the plasma creatinine increased from 70 micro mol/l to 194 micro mol/l in 2 weeks without thrombocytopenia or signs of hemolytic anemia. The patient had minimal clinical symptoms and a presumptive diagnosis of graft rejection was made. Despite treatment with six daily pulses of methylprednisolone, plasma creatinine continued to increase and a graft biopsy was therefore undertaken. This showed the typical appearance of a thrombotic microangiopathy without any evidence of rejection. Despite daily plasmapheresis and replacement of cyclosporine with tacrolimus, there was no improvement and transplant nephrectomy was undertaken. This patient demonstrates that HUS can recur in a kidney transplant without the diagnostic hematological features and emphasizes the need for early transplant biopsy in such patients showing a decline in transplant function.

  2. Atypical mitochondrial inheritance patterns in eukaryotes. (United States)

    Breton, Sophie; Stewart, Donald T


    Mitochondrial DNA (mtDNA) is predominantly maternally inherited in eukaryotes. Diverse molecular mechanisms underlying the phenomenon of strict maternal inheritance (SMI) of mtDNA have been described, but the evolutionary forces responsible for its predominance in eukaryotes remain to be elucidated. Exceptions to SMI have been reported in diverse eukaryotic taxa, leading to the prediction that several distinct molecular mechanisms controlling mtDNA transmission are present among the eukaryotes. We propose that these mechanisms will be better understood by studying the deviations from the predominating pattern of SMI. This minireview summarizes studies on eukaryote species with unusual or rare mitochondrial inheritance patterns, i.e., other than the predominant SMI pattern, such as maternal inheritance of stable heteroplasmy, paternal leakage of mtDNA, biparental and strictly paternal inheritance, and doubly uniparental inheritance of mtDNA. The potential genes and mechanisms involved in controlling mitochondrial inheritance in these organisms are discussed. The linkage between mitochondrial inheritance and sex determination is also discussed, given that the atypical systems of mtDNA inheritance examined in this minireview are frequently found in organisms with uncommon sexual systems such as gynodioecy, monoecy, or andromonoecy. The potential of deviations from SMI for facilitating a better understanding of a number of fundamental questions in biology, such as the evolution of mtDNA inheritance, the coevolution of nuclear and mitochondrial genomes, and, perhaps, the role of mitochondria in sex determination, is considerable.

  3. Persistent consequences of atypical early number concepts

    Directory of Open Access Journals (Sweden)

    Michèle M. M. Mazzocco


    Full Text Available How does symbolic number knowledge performance help identify young children at risk for poor mathematics achievement outcomes? In research and practice, classification of mathematics learning disability (MLD, or dyscalculia is typically based on composite scores from broad measures of mathematics achievement. These scores do predict later math achievement levels, but do not specify the nature of math difficulties likely to emerge among students at greatest risk for long-term mathematics failure. Here we report that gaps in 2nd and 3rd graders’ number knowledge predict specific types of errors made on math assessments at Grade 8. Specifically, we show that early whole number misconceptions predict slower and less accurate performance, and atypical computational errors, on Grade 8 arithmetic tests. We demonstrate that basic number misconceptions can be detected by idiosyncratic responses to number knowledge items, and that when such misconceptions are evident during primary school they persist throughout the school age years, with variable manifestation throughout development. We conclude that including specific qualitative assessments of symbolic number knowledge in primary school may provide greater specificity of the types of difficulties likely to emerge among students at risk for poor mathematics outcomes.

  4. Letter to Editor: Carpal tunnel syndrome due to an atypical deep soft tissue leiomyoma: The risk of misdiagnosis and mismanagement

    Directory of Open Access Journals (Sweden)

    Caliandro Pietro


    Full Text Available Abstract A response to Chalidis et al: Carpal tunnel syndrome due to an atypical deep soft tissue leiomyoma: The risk of misdiagnosis and mismanagement. World J Surg Oncol 2007, 5:92.

  5. Shoulder Joint Replacement (United States)

    ... Shoulder Replacement Options Shoulder replacement surgery is highly technical. It should be performed by a surgical team ... area and will meet a doctor from the anesthesia department. You, your anesthesiologist, and your surgeon will ...

  6. Partial knee replacement - slideshow (United States)

    ... page: // Partial knee replacement - series—Normal anatomy To use the sharing ... A.M. Editorial team. Related MedlinePlus Health Topics Knee Replacement A.D.A.M., Inc. is accredited ...

  7. How do we choose between atypical antipsychotics? The advantages of amisulpride. (United States)

    Mortimer, Ann M


    Clinician choice of an atypical antipsychotic may depend on a number of factors such as perceived efficacy, tolerability and cost. It is also important that the choice of treatment takes into consideration the previous response to treatment, experience of side-effects and personal clinical characteristics. The receptor-affinity profiles of the atypical antipsychotics differ; with the exception of amisulpride, a selective D2/D3 antagonist, all the atypical antipsychotics exhibit a greater affinity for the serotonin-2A receptors than dopamine receptors. However, there is no evidence that the variation in receptor affinities is relevant to efficacy. Indeed, the crucial factor may be fast dissociation from low affinity for the D2 receptor. Tolerability also varies between the atypical antipsychotics and the side-effect profile may be related to the receptor-affinity profile of the individual drugs. Extrapyramidal side-effects are generally less of a problem with most atypical drugs than with conventional drugs, but weight gain, loss of glycaemic control, sedation and hyperprolactinaemia remain problematic in some patients. Amisulpride is effective for the treatment of both positive and negative symptoms, and is well tolerated with regard to weight gain, glucose tolerance and sedation. In two clinical trials, the AMIRIS and SOLIANOL studies, amisulpride demonstrated clear advantages over some other atypical antipsychotics with respect to negative symptoms, depressive symptoms and weight gain.

  8. Can training normalize atypical passive auditory ERPs in children with SRD or SLI? (United States)

    McArthur, Genevieve M; Atkinson, Carmen M; Ellis, Danielle


    This study tested if training can normalize atypical passive auditory event-related potentials in the N1-P2 time window in children with specific reading disability (SRD) or specific language impairment (SLI). Children with SRD or SLI and untrained controls were tested for their behavioral responses and N1-P2 windows to tones, backward-masked tones, vowels, and consonant-vowels. Children with SRD or SLI with poor behavioral responses to one of these sounds trained to discriminate that sound for 30 minutes a day, 4 days a week, for 6 weeks. Post-training measures revealed that training normalized atypical behavioral responses but not atypical N1-P2 windows.

  9. HDL cholesterol response to GH replacement is associated with common cholesteryl ester transfer protein gene variation (-629C > A) and modified by glucocorticoid treatment

    NARCIS (Netherlands)

    Dullaart, Robin P. F.; van den Berg, Gerrit; van der Knaap, Aafke M.; Dijck-Brouwer, Janneke; Dallinga-Thie, Geesje M.; Zelissen, Peter M. J.; Sluiter, Wim J.; van Beek, Andre P.


    Objective: GH replacement lowers total cholesterol and low-density lipoprotein cholesterol (LDL-C) in GH-deficient adults, but effects on high-density lipoprotein (HDL) cholesterol (HDL-C) are variable. Both GH and glucocorticoids decrease cholesteryl ester transfer protein (CETP) activity, which is

  10. Clinical Presentation of Atypical Genital Herpes

    Institute of Scientific and Technical Information of China (English)

    李俊杰; 梁沛杨; 罗北京


    Objective: To make a clinical analysis on the basis of 36cases of atypical genital herpes (GH) patients. Methods: Thirty-six cases of atypical GH were diagnosedclinically, and their case histories, symptoms and signs wererecorded in detail and followed up. Polymerase chain reaction(PCR) was adopted for testing HSV2-DNA with cotton-tippedswabs. Enzyme-linked immuno sorbent assay (ELISA) forserum anti-HSV2-IgM was done to establish a definfiivediagnosis. Other diagnoses were excluded at the same time bytesting for related pathogens including fungi, Chlamydia,Mycoplasma, Treponema pallidum, gonococci, Trichomonas,etc. Results: The main clinical manifestations of atypical GHwere: (1) small genital ulcers; (2) inflammation of urethralmeatus; (3) nonspecific genital erythema; (4) papuloid noduleson the glands; (5) nonspecific vaginitis. Twenty-three cases(64%) tested by PCR were HSV2-DNA sera-positive, and 36cases (100 %) anti-HSV2-IgM sera-positive by ELISA. Conclusion: atypical HSV is difficult to be diagnosed. Butthe combination of PCR and ELIAS will be helpful to thediagnosis of atypical HSV.

  11. Atypical aging in Down syndrome. (United States)

    Zigman, Warren B


    divided into two sections. The first section will review typical and atypical aging patterns in somatic issues in elder adults with DS; the second section will review the multifaceted relationship between AD and DS.

  12. Evaluation of biochemical parameters and productive performance of japanese quail in response to the replacement of soybean meal with canola meal

    Directory of Open Access Journals (Sweden)

    Ali Asghar Saki


    Full Text Available The present study was conducted to evaluation the different levels canola meal (CM replacement by soybean meal (SBM in Japanese quails diets on productive performance, egg quality trails and blood parameters including blood protein, minerals concentration, liver enzymes and thyroid hormones. In this study, 160 of Japanese quails in second laying phase from 46 to 56 weeks of age were divided in four treatments with four replicates and 10 quail in each per replicate, on a completely randomized design (CRD.Treatments consisted:T1 control (without CM, T2, T3 and T4 replacing 30, 60 and 90% of SBM with CM respectively. The results were shown no significant variation in body weight between treatments in initial and end of the experiment (p > 0.05. There was significant decrease in feed intake by increasing level of CM (p 0.05. However, the egg shell thickness showed significantly decreased with increasing levels of canola meal in the diet  (p < 0.05. According to this study results, 30% replacement of SBM by CM in the diet (contained 10% CM has no adverse effect on quail’s performance.

  13. Neuroleptic malignant syndrome induced by atypical antipsychotics. (United States)

    Farver, Debra K


    A review of the English literature confirms that neuroleptic malignant syndrome (NMS) occurs with both traditional and atypical antipsychotic medications. Published reports of NMS induced by the traditional antipsychotics have given the practitioner valuable information on the prevention and treatment of this adverse effect. Case reports have also been published concerning NMS and clozapine, risperidone, olanzapine and quetiapine. By evaluating the case reports of atypical antipsychotic-induced NMS, valuable information may be obtained concerning similarities or differences from that induced by the traditional antipsychotics. The case reports of NMS with atypical antipsychotics were evaluated for diagnosis, age/sex of patient, risk factors, antipsychotic doses and duration of use, symptoms of NMS, and clinical course.

  14. [Atypical antipsychotic-induced weight gain]. (United States)

    Godlewska, Beata R; Olajossy-Hilkesberger, Luiza; Marmurowska-Michałowska, Halina; Olajossy, Marcin; Landowski, Jerzy


    Introduction of a new group of antipsychotic drugs, called atypical because of the proprieties differing them from classical neuroleptics, gave hope for the beginning of a new era in treatment of psychoses, including schizophrenia. Different mechanisms of action not only resulted in a broader spectrum of action and high efficacy but also in a relative lack of extrapiramidal symptoms. However, atypical neuroleptics are not totally free from adverse effects. Symptoms such as sedation, metabolic changes and weight gain, often very quick and severe - present also in the case of classical drugs, but put to the background by extrapiramidal symptoms--have become prominent. Weight gain is important both from the clinical and subjective point of view--as associated with serious somatic consequences and as a source of enormous mental distress. These problems are addressed in this review, with the focus on weight gain associated with the use of specific atypical neuroleptics.

  15. An Interesting Case of Life-Threatening Hypercalcemia Secondary to Atypical Parathyroid Adenoma versus Parathyroid Carcinoma

    Directory of Open Access Journals (Sweden)

    Ankur Mishra


    Full Text Available Context. Severe hypercalcemia is a life-threatening condition. Atypical parathyroid adenoma and parathyroid carcinomas are uncommon causes which can be difficult to differentiate. Objective. We report a case of a 36-year-old male with very high serum calcium due to a possible atypical parathyroid adenoma versus parathyroid carcinoma. Case Illustration. A serum calcium level of 23.2 mg/dl was noted on admission. He was initially treated with IV hydration, pamidronate, and salmon calcitonin to lower his calcium levels. He also underwent a surgical en bloc resection of parathyroid mass. Pathology showed a mixed picture consistent with possible atypical adenoma versus parathyroid carcinoma. However, due to the possible involvement of the recurrent laryngeal nerve, parathyroid carcinoma was more likely. Also after operation the patient developed hungry bones syndrome and his calcium was replaced vigorously. He continues to be on calcium, vitamin D, and calcitriol supplementation. Results. A review of the literature was conducted to identify previous studies pertaining to parathyroid adenomas and parathyroid cancer. Conclusion. We thereby conclude that hypercalcemia requires very careful monitoring especially after operation. Also it can be very difficult to distinguish between atypical parathyroid adenomas and parathyroid carcinomas as in our case and no clear cut guidelines yet exist to differentiate the two based on histology.

  16. Aeronautical Information System Replacement - (United States)

    Department of Transportation — Aeronautical Information System Replacement is a web-enabled, automation means for the collection and distribution of Service B messages, weather information, flight...

  17. Atypical RNAs in the coelacanth transcriptome. (United States)

    Nitsche, Anne; Doose, Gero; Tafer, Hakim; Robinson, Mark; Saha, Nil Ratan; Gerdol, Marco; Canapa, Adriana; Hoffmann, Steve; Amemiya, Chris T; Stadler, Peter F


    Circular and apparently trans-spliced RNAs have recently been reported as abundant types of transcripts in mammalian transcriptome data. Both types of non-colinear RNAs are also abundant in RNA-seq of different tissue from both the African and the Indonesian coelacanth. We observe more than 8,000 lincRNAs with normal gene structure and several thousands of circularized and trans-spliced products, showing that such atypical RNAs form a substantial contribution to the transcriptome. Surprisingly, the majority of the circularizing and trans-connecting splice junctions are unique to atypical forms, that is, are not used in normal isoforms.

  18. An atypical monomelic presentation of Mazabraud syndrome

    Directory of Open Access Journals (Sweden)

    Jun Wan


    Full Text Available Mazabraud syndrome is a rare condition characterized by a combination of fibrous dysplasia and intramuscular myxomas. In Mazabraud syndrome, the distribution of fibrous dysplasia is mostly polyomelic and frequently located in the femur, with myxomas adjacent to the fibrous dysplasia lesion of bone (mostly in the quadriceps muscle. However, when presented as atypical clinical features, patients of Mazabraud syndrome is either misdiagnosed or difficult to diagnose. We report an atypical monomelic case of Mazabraud syndrome in the right upper arm and discuss the difficulties in making an accurate diagnosis.

  19. Influence of Total Knee Arthroplasty on Gait Mechanics of the Replaced and Non-Replaced Limb During Stair Negotiation. (United States)

    Standifird, Tyler W; Saxton, Arnold M; Coe, Dawn P; Cates, Harold E; Reinbolt, Jeffrey A; Zhang, Songning


    This study compared biomechanics during stair ascent in replaced and non-replaced limbs of total knee arthroplasty (TKA) patients with control limbs of healthy participants. Thirteen TKA patients and fifteen controls performed stair ascent. Replaced and non-replaced knees of TKA patients were less flexed at contact compared to controls. The loading response peak knee extension moment was greater in control and non-replaced knees compared with replaced. The push-off peak knee abduction moment was elevated in replaced limbs compared to controls. Loading and push-off peak hip abduction moments were greater in replaced limbs compared to controls. The push-off peak hip abduction moment was greater in non-replaced limbs compared to controls. Future rehabilitation protocols should consider the replaced knee and also the non-replaced knee and surrounding joints.

  20. Growth response and feed utilization of Clarias gariepinus (Burchell, 1822 juveniles fed graded levels of boiled Senna obtusifolia l. seed meal as a replacement for soybean meal

    Directory of Open Access Journals (Sweden)

    Abdullateef Yusuf


    Materials and methods: Five isocaloric and isonitrogenous diets were formulated. The diest were- control diet (with 0% inclusion level boiled S. obtusifolia seed meal i.e., 100% soybean meal, 25%, 50%, 75% and 100% inclusion level (total replacement of soya bean meal. The feeds were fed to C. gariepinus juveniles at 5% of their body weight for 84 days in an indoor partial flow through system. Weight and standard length of C. gariepinus juveniles were taken every fortnight and water quality parameters were monitored weekly. Results: Experimental fish fed diets with 0 and 25% inclusion level of boiled S. obtusifolia seed meal gave the best results in terms of Mean Weight Gain (MWG (20.22 and 19.79 g, respectively, Specific Growth Rate (1.82 and 1.83 respectively, Protein Efficiency Ratio (2.75 and 2.00, respectively and the Lowest Feed Conversion Ratio (1.29 and 1.39 respectively. The lowest growth and feed utilization were observed in fish fed 100% inclusion level. The weight gain of fish decreased with increase in replacement level above 25%. There was no significant difference between control diet 0 and 25% inclusion level (P and #8805;0.05. Conclusion: Boiled S. obtusifolia seed meal is a nutritive source of plant protein and a good replacement for soybean meal at 25% inclusion level of boiled S. obtusifolia seed meal in formulating catfish feed for C. gariepinus juveniles without any deleterious effect. [J Adv Vet Anim Res 2016; 3(4.000: 345-352

  1. Radiation Source Replacement Workshop

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, Jeffrey W.; Moran, Traci L.; Bond, Leonard J.


    This report summarizes a Radiation Source Replacement Workshop in Houston Texas on October 27-28, 2010, which provided a forum for industry and researchers to exchange information and to discuss the issues relating to replacement of AmBe, and potentially other isotope sources used in well logging.

  2. Effect of replacement of fish meal by meat and bone meal and poultry by-product meal in diets on the growth and immune response of Macrobrachium nipponense. (United States)

    Yang, Yong; Xie, Shouqi; Lei, Wu; Zhu, Xiaoming; Yang, Yunxia


    The potential use of poultry by-product meal (PBM) and meat and bone meal (MBM) as alternative dietary protein sources for juvenile Macrobrachium nipponense was studied by a 70-day growth trial. Triplicate groups of M. nipponense (initial body weight: 0.37 g) were fed at 20.7-22.4 degrees C on each of the five isoenergetic and isonitrogenous diets (protein content about 38%) with different replacement of fish meal by MBM or PBM. The control diet used white fish meal as the sole protein source, the other four diets were prepared with 15% or 50% fish meal protein substituted by either MBM (MBM(15), MBM(50)) or PBM (PBM(15), PBM(50)). The results showed that replacement of fish meal by MBM in diets did not affect growth performance of M. nipponense (P > 0.05), while specific growth rate in PBM(15) was significantly higher than that in other groups (P meal protein in diets for M. nipponense.

  3. Atypical visuomotor performance in children with PDD

    NARCIS (Netherlands)

    Schlooz, W.A.J.M.; Hulstijn, W.


    Children with autism spectrum disorders (ASD) frequently encounter difficulties in visuomotor tasks, which are possibly caused by atypical visuoperceptual processing. This was tested in children (aged 9–12 years) with pervasive developmental disorder (PDD; including PDD-NOS and Asperger syndrome),

  4. Atypical Pyoderma Gangrenosum Mimicking an Infectious Process

    Directory of Open Access Journals (Sweden)

    Derek To


    Full Text Available We present a patient with atypical pyoderma gangrenosum (APG, which involved the patient’s arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

  5. Atypical pyoderma gangrenosum mimicking an infectious process. (United States)

    To, Derek; Wong, Aaron; Montessori, Valentina


    We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

  6. Biochemical and molecular studies of atypical nevi

    NARCIS (Netherlands)

    Nieuwpoort, Arno Frans van


    The results obtained in this thesis suggest that the most explicit differences between normal and atypical melanocytes are subtle changes in pigment biosynthesis and the functioning of the antioxidant system. Impairment of the antioxidant system and increased levels of pheomelanin result in increase

  7. Non-diabetic atypical necrobiosis lipoidica

    Directory of Open Access Journals (Sweden)

    Mittal R


    Full Text Available One 8 year female child had asymptomatic, anaesthetic, hypohidrotic, atrophic, yellowish, waxy plaque on the front of left thigh since 2 months. No nerve thickening was observed clinically or histopathologically. Hyperkeratosis, follicular keratosis, epidermal atrophy, degeneration of collagen, mononuclear granulomas and perivascular mononuclear infiltrate confirmed the clinical diagnosis of atypical necrobiosis lipoidica.

  8. [Atypical depression in Japan--39 case series]. (United States)

    Tada, Koji; Yamayoshi, Kayoko; Matsuzaki, Yamato; Kojima, Takuya


    In Japan, relatively little attention has been paid to atypical depression, which is defined as the presence of mood reactivity and two of four associated features: hyperphagia, hypersomnia, leaden paralysis, rejection sensitivity. The present study was undertaken to obtain detailed clinical information from patients with a diagnosis of atypical depression. We assessed clinical characteristics of each atypical feature, comorbidity of other psychiatric disorders, presence of a stressful life event, and underlying psychological stress in 39 psychiatric outpatients. We also examined the relationship of interpersonal sensitivity to each atypical feature. Mean age of onset was 22 +/- 6, 74% were female, 20 patients (51%) had comorbid social phobia. Thirty (77%) had hyperphagia and 25 of these were women. Twenty (74%) had hypersomnia. Only seven patients reported daytime sleepiness and others (13) reported difficulty in staying awake due to lack of energy. Nineteen (49%) had leaden paralysis. Thirty-two patients (82%) had rejection sensitivity and this symptom correlated with scores of FNE (fears of negative evaluation), LSAS (Liebowits social anxiety scale) and Brief social phobia scale (BSPA). Seven patients reported disappointment in love as a stressful life event preceding the depressive episode. In patients with comorbid social phobia, loss of confidence due to hypersensitivity to rejection or criticism seemed to be the most important factor as a chronic psychologica stress. Seven patients met criteria for bipolar disorder and five out of seven had comorbid generalized social phobia. The clinical and theoretical implications of these findings were discussed.

  9. Atypical Alpha Asymmetry in Adults with ADHD (United States)

    Hale, T. Sigi; Smalley, Susan L.; Hanada, Grant; Macion, James; McCracken, James T.; McGough, James J.; Loo, Sandra K.


    Introduction: A growing body of literature suggests atypical cerebral asymmetry and interhemispheric interaction in ADHD. A common means of assessing lateralized brain function in clinical populations has been to examine the relative proportion of EEG alpha activity (8-12 Hz) in each hemisphere (i.e., alpha asymmetry). Increased rightward alpha…

  10. Atypical Alpha Asymmetry in Adults with ADHD (United States)

    Hale, T. Sigi; Smalley, Susan L.; Hanada, Grant; Macion, James; McCracken, James T.; McGough, James J.; Loo, Sandra K.


    Introduction: A growing body of literature suggests atypical cerebral asymmetry and interhemispheric interaction in ADHD. A common means of assessing lateralized brain function in clinical populations has been to examine the relative proportion of EEG alpha activity (8-12 Hz) in each hemisphere (i.e., alpha asymmetry). Increased rightward alpha…

  11. Atypical visuomotor performance in children with PDD

    NARCIS (Netherlands)

    Schlooz, W.A.J.M.; Hulstijn, W.


    Children with autism spectrum disorders (ASD) frequently encounter difficulties in visuomotor tasks, which are possibly caused by atypical visuoperceptual processing. This was tested in children (aged 9–12 years) with pervasive developmental disorder (PDD; including PDD-NOS and Asperger syndrome), a

  12. Atypical fractures on long term bisphosphonates therapy.

    LENUS (Irish Health Repository)

    Hussein, W


    Bisphosphonates reduce fractures risk in patients with osteoporosis. A new pattern of fractures is now being noted in patients on prolonged bisphosphonate therapy. We report a case of an atypical femoral fracture with preceding pain and highlight the characteristics of these fractures.

  13. Psychiatric syndromes associated with atypical chest pain

    Directory of Open Access Journals (Sweden)

    Nikolić Gordana


    Full Text Available Background/Aim. Chest pain often indicates coronary disease, but in 25% of patients there is no evidence of ischemic heart disease using standard diagnostic tests. Beside that, cardiologic examinations are repeated several times for months. If other medical causes could not be found, there is a possibility that chest pain is a symptom of psychiatric disorder. The aim of this study was to determine the presence of psychiatric syndromes, increased somatization, anxiety, stress life events exposure and characteristic of chest pain expression in persons with atypical chest pain and coronary patients, as well as to define predictive parameters for atypical chest pain. Method. We compared 30 patients with atypical chest pain (E group to 30 coronary patients (K group, after cardiological and psychiatric evaluation. We have applied: Mini International Neuropsychiatric Interview (MINI, The Symptom Checklist 90-R (SCL-90 R, Beck Anxiety Inventory (BAI, Holms-Rahe Scale of stress life events (H-R, Questionnaire for pain expression Pain-O-Meter (POM. Significant differences between groups and predictive value of the parameters for atypical chest pain were determined. Results. The E group participants compared to the group K were younger (33.4 ± 5.4 : 48.3 ± 6,4 years, p < 0.001, had a moderate anxiety level (20.4 ± 11.9 : 9.6 ± 3.8, p < 0.001, panic and somatiform disorders were present in the half of the E group, as well as eleveted somatization score (SOM ≥ 63 -50% : 10%, p < 0.01 and a higher H-R score level (102.0 ± 52.2 : 46.5 ± 55.0, p < 0.001. Pain was mild, accompanied with panic. The half of the E group subjects had somatoform and panic disorders. Conclusion. Somatoform and panic disorders are associated with atypical chest pain. Pain expression is mild, accompained with panic. Predictive factors for atypical chest pain are: age under 40, anxiety level > 20, somatization ≥ 63, presence of panic and somatoform disorders, H-R score > 102

  14. Side effects induced by the acute levodopa challenge in Parkinson’s Disease and atypical parkinsonisms (United States)

    Mostile, Giovanni; Dibilio, Valeria; Sciacca, Giorgia; Contrafatto, Donatella; Cicero, Calogero Edoardo; Raciti, Loredana; Luca, Antonina; Zappia, Mario


    Introduction Acute levodopa challenge may be performed to predict levodopa chronic responsiveness. The aim of the study was to investigate frequency of side effects during the acute levodopa challenge in PD and atypical parkinsonisms. Methods We enrolled 34 de novo PD patients and 29 patients affected by atypical parkinsonisms (Multiple System Atrophy, MSA, n = 10; Progressive Supranuclear Palsy, PSP, n = 12 and Corticobasal Degeneration, CBD, n = 7) who underwent an acute levodopa challenge. Side effects occurring during test were recorded. Results Side effects were more frequent among atypical parkinsonisms as unique group when compared to PD patients (64.3% versus 23.5%; p-value 0.002) with an adjusted OR of 4.36 (95%CI 1.40–13.5). Each atypical parkinsonisms showed almost double occurrence of side effects (MSA 90%, PSP 41.7% and CBD 57%). Conclusions Side effects during acute levodopa challenge may be frequent in atypical parkinsonisms. This information could be useful in order to better prepare the patient for the test. Furthermore, it could represent a useful cue in differential diagnosis with PD. PMID:28207803

  15. Atypical hemolytic-uremic syndrome: a case report and literature review. (United States)

    Rafiq, Arsalan; Tariq, Hassan; Abbas, Naeem; Shenoy, Roopalekha


    Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal dysfunction. It is a disease related to genetic mutations in the alternative complement pathway and has a distinct pathophysiology but is difficult to differentiate from other thrombotic microangiopathies. We present a case of a 59-year-old female patient who presented with accelerated hypertension, acute renal failure, hemolysis, and encephalopathy. She was managed with antihypertensive medication, but her encephalopathy did not improve. Evaluation resulted in our impression of the disease being atypical hemolytic-uremic syndrome. The patient continued to be managed with good blood pressure control and later was started on eculizumab, but evaluation of response to therapy was hindered by the patient's non-compliance with therapy and follow-up appointments. We have a very limited understanding of the genetics and epidemiology of atypical HUS, and the overlapping clinical features sometimes delay diagnosis and initiation of appropriate treatment of this rare disease.

  16. Atypical visual change processing in children with autism: an electrophysiological study. (United States)

    Cléry, Helen; Bonnet-Brilhault, Frederique; Lenoir, Pascal; Barthelemy, Catherine; Bruneau, Nicole; Gomot, Marie


    Children with Autism Spectrum Disorder (ASD) may display atypical behaviors in reaction to unattended changes that occur in all sensory modalities. Atypical automatic auditory change processing has been highlighted in ASD via the analysis of mismatch negativity (MMN). The present study investigated visual deviancy detection in children with ASD in order to determine whether unusual reactions to change operate in other sensory modalities. Twelve children with ASD were presented with a passive visual oddball paradigm using dynamic stimuli. Compared to controls, children with ASD showed an earlier visual mismatch response, suggesting a hypersensitivity to visual deviancy. This study is thus consistent with the hypothesis of the existence of "general" atypical change detection processing in children with ASD that might contribute to their intolerance of change. Copyright © 2013 Society for Psychophysiological Research.

  17. Replacing a Missing Tooth (United States)

    ... vessels in the tooth pulps are rather large. Drilling down these teeth for crowns may expose the ... porcelain replacement tooth is held in place by metal extensions cemented to the backs of the adjacent ...

  18. Hormone Replacement Therapy (United States)

    ... before and during menopause, the levels of female hormones can go up and down. This can cause ... hot flashes and vaginal dryness. Some women take hormone replacement therapy (HRT), also called menopausal hormone therapy, ...

  19. Knee joint replacement (United States)

    ... of your kneecap. Your kneecap is called the patella. The replacement part is usually made from a ... long. Then your surgeon will: Move your kneecap (patella) out of the way, then cut the ends ...

  20. Knee joint replacement - slideshow (United States)

    ... this page: // Knee joint replacement - series—Normal anatomy To use the ... to slide 4 out of 4 Overview The knee is a complex joint. It contains the distal ...

  1. The effect of verbalization strategy on wisconsin card sorting test performance in schizophrenic patients receiving classical or atypical antipsychotics

    Directory of Open Access Journals (Sweden)

    Cavallaro Roberto


    Full Text Available Abstract Background A number of reports showed en encouraging remediation in some patients' executive deficits thanks to the use of 'information processing strategies'. Moreover the impact of antipsychotics on cognitive functions of the schizophrenics is an important issue, especially if an integrated psychosocial treatment is needed. The aim of this paper is to evaluate different executive performance and response to verbalization, a strategy of the Wisconsin Card Sorting Test (WCST remediation, in subjects on classical vs atypical antipsychotic (AP treatment. Methods Sixty-three schizophrenic subjects undertook the WCST under standard and modified (verbalization administration. Subjects were stratified by the kind of WCST response (i.e. good, poor and remediable and AP treatment (i.e. atypical vs. classical. Results Subjects on atypical APs showed a better performance than those on classical ones. More poor performers who did not remediate were seen in the sample with classical Aps while subjects who remediated the performance were seen in the subgroup with atypical APs only. An increase of perseverative and total errors was seen in poor performers subjects on classical APs. Conclusion Subjects on atypicals showed a better cognitive pattern in terms of WCST performance. Since the naturalistic assignment of medication we cannot draw conclusions about its effect on cognitive performance and its interaction with cognitive remediation potential. However the data lead us to hypothesize that subjects with potential room for remediation did so with the atypical APs.

  2. Product Platform Replacements

    DEFF Research Database (Denmark)

    Sköld, Martin; Karlsson, Christer


    Purpose – It is argued in this article that too little is known about product platforms and how to deal with them from a manager's point of view. Specifically, little information exists regarding when old established platforms are replaced by new generations in R&D and production environments...... originality and value is achieved by focusing on product platform replacements believed to represent a growing management challenge....

  3. ACKR2: An Atypical Chemokine Receptor Regulating Lymphatic Biology (United States)

    Bonavita, Ornella; Mollica Poeta, Valeria; Setten, Elisa; Massara, Matteo; Bonecchi, Raffaella


    The lymphatic system plays an important role in the induction of the immune response by transporting antigens, inflammatory mediators, and leukocytes from peripheral tissues to draining lymph nodes. It is emerging that lymphatic endothelial cells (LECs) are playing an active role in this context via the expression of chemokines, inflammatory mediators promoting cell migration, and chemokine receptors. Particularly, LECs express atypical chemokine receptors (ACKRs), which are unable to promote conventional signaling and cell migration while they are involved in the regulation of chemokine availability. Here, we provide a summary of the data on the role of ACKR2 expressed by lymphatics, indicating an essential role for this ACKRs in the regulation of the inflammation and the immune response in different pathological conditions, including infection, allergy, and cancer. PMID:28123388

  4. Benign occipital lobe seizures: Natural progression and atypical evolution

    Directory of Open Access Journals (Sweden)

    Prithika Chary


    Full Text Available Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.

  5. Atypical streptococcal infection of gingiva associated with chronic mouth breathing. (United States)

    Haytac, M Cenk; Oz, I Attila


    Streptococcal infections of oral tissues are mainly seen in young children who experience a variety of upper respiratory tract infections. The disease is characterized by fever, lymphadenopathy, and ulcers on the gingiva, lips, and tonsils. This case report presents an atypical streptococcal infection of the gingiva in an 18-year-old man. The patient was referred to the periodontology department complaining of a 2-month history of gingival enlargement. He had persistent fever (39.5 degrees C) and general malaise for 2 weeks. Intraoral examination revealed extremely inflamed and enlarged gingiva with spontaneous bleeding and suppuration. Based on the otolaryngologic consultation and the hematologic, immunologic, and microbiologic tests, the final diagnosis was an atypical streptococcal gingivitis with chronic adenoid-related mouth breathing and oral hygiene neglect as contributing factors. Treatment consisted of a broad-spectrum antibiotic regimen, supragingival and subgingival debridement, adenoidectomy, and scaling and root planing. A good response to nonsurgical therapy was achieved despite poor patient compliance, and no recurrence of gingival enlargement was observed after 1 year. Streptococcal gingivitis should be included in the differential diagnosis of suppurative gingival enlargements. Furthermore, chronic mouth breathing may initiate and/or contribute to this disease.

  6. Effects of replacing soybean meal with rubber seed meal on growth, antioxidant capacity, non-specific immune response, and resistance to Aeromonas hydrophila in tilapia (Oreochromis niloticus × O. aureus). (United States)

    Deng, Junming; Mai, Kangsen; Chen, Liqiao; Mi, Haifeng; Zhang, Lu


    This study evaluated the effects of replacing soybean meal (SBM) with rubber seed meal (RSM) on growth, antioxidant capacity, non-specific immune response and resistance to Aeromonas hydrophila in tilapia (Oreochromis niloticus × Oreochromis aureus). Five experimental diets were formulated with 0 (control), 10, 20, 30, and 40% RSM replacing graded levels of SBM, respectively. Fish were fed one of the five experimental diets for eight weeks, and then challenged by A. hydrophila via intraperitoneal injection and kept for seven days. Dietary RSM inclusion level up to 30% did not affect the weight gain and daily growth coefficient, whereas these were depressed by a further inclusion. Fish fed diet with 40% RSM showed the lowest serum total antioxidant capacity, lysozyme, alternative complement pathway, respiratory burst and phagocytic activities. Dietary RSM inclusion gradually depressed the post-challenge survival rate, and that was significantly lower in fish fed diet with 40% RSM compared to fish fed the control diet. Conversely, the inclusion of RSM generally increased the serum total cholesterol level, the plasma alanine aminotransferase and aspartate aminotransferase activities, and these were significantly higher in fish fed diet with 40% RSM compared to fish fed the control diet. The results indicated that RSM can be included at level up to 30% in diet for tilapia without obvious adverse effects on the growth, antioxidant capacity, non-specific immune response and resistance to A. hydrophila infection, whereas these were depressed by a further inclusion.

  7. Primary lateral sclerosis mimicking atypical parkinsonism

    DEFF Research Database (Denmark)

    Norlinah, Ibrahim M; Bhatia, Kailash P; Østergaard, Karen


    Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one...... of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa-unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67...... included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis...

  8. Herpes zoster - typical and atypical presentations. (United States)

    Dayan, Roy Rafael; Peleg, Roni


    Varicella- zoster virus infection is an intriguing medical entity that involves many medical specialties including infectious diseases, immunology, dermatology, and neurology. It can affect patients from early childhood to old age. Its treatment requires expertise in pain management and psychological support. While varicella is caused by acute viremia, herpes zoster occurs after the dormant viral infection, involving the cranial nerve or sensory root ganglia, is re-activated and spreads orthodromically from the ganglion, via the sensory nerve root, to the innervated target tissue (skin, cornea, auditory canal, etc.). Typically, a single dermatome is involved, although two or three adjacent dermatomes may be affected. The lesions usually do not cross the midline. Herpes zoster can also present with unique or atypical clinical manifestations, such as glioma, zoster sine herpete and bilateral herpes zoster, which can be a challenging diagnosis even for experienced physicians. We discuss the epidemiology, pathophysiology, diagnosis and management of Herpes Zoster, typical and atypical presentations.

  9. Typical and Atypical Manifestations of Intrathoracic Sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hyun Jin; Jung, Jung Im; Chung, Myung Hee; Song, Sun Wha; Kim, Hyo Lim; Baik, Jun Hyun; Han, Dae Hee [St. Vincent' s Hospital, The Catholic University of Korea, Suwon (Korea, Republic of); Kim, Ki Jun [Incheon St. Mary' s Hospital, The Catholic University of Korea, Incheon (Korea, Republic of); Lee, Kyo Young [Seoul St. Mary' s Hospital, The Catholic University of Korea, Seoul (Korea, Republic of)


    Sarcoidosis is a systemic disorder of unknown cause that is characterized by the presence of noncaseating granulomas. The radiological findings associated with sarcoidosis have been well described. The findings include symmetric, bilateral hilar and paratracheal lymphadenopathy, with or without concomitant parenchymal abnormalities (multiple small nodules in a peribronchovascular distribution along with irregular thickening of the interstitium). However, in 25% to 30% of cases, the radiological findings are atypical and unfamiliar to most radiologists, which cause difficulty for making a correct diagnosis. Many atypical forms of intrathoracic sarcoidosis have been described sporadically. We have collected cases with unusual radiological findings associated with pulmonary sarcoidosis (unilateral or asymmetric lymphadenopathy, necrosis or cavitation, large opacity, ground glass opacity, an airway abnormality and pleural involvement) and describe the typical forms of the disorder as well. The understanding of a wide range of the radiological manifestations of sarcoidosis will be very helpful for making a proper diagnosis.

  10. Recurrent conjunctival atypical fibroxanthoma in Pigmentosum Xeroderma. (United States)

    Cerdà-Ibáñez, M; Barreiro-González, A; Barranco González, H; Aviñó Martínez, J; Évole-Buselli, M; Harto-Castaño, M Á


    A 7 year-old boy with Xeroderma Pigmentosum (XP) and who presents a recurrent conjunctival atypical fibroxanthoma after two surgeries. This is the third procedure and the patient is treated with a surgical excision of the tumour and cryotherapy at the surgical bed. Due to the risk of recurrence, topical Mitomycin C 0,02% was added at post-operative care achieving a good clinical outcome. Surgical exeresis with cryotherapy and topical Mitomycin C is an effective treatment for a case of an atypical fibroxanthoma with a high potential for recurrence and invasion. An ophthalmologic follow-up is required for these patients, as well as general paediatric care and support aids. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. An atypical presentation of antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Deepti D′Souza


    Full Text Available Cutaneous manifestations in antiphospholipid antibody syndrome (APS though common, are extremely diverse and it is important to know which dermatological finding should prompt consideration of antiphospholipid syndrome. The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis, and systemic involvement is invariably an accomplice in APS. Cutaneous ulcers with sharp margins can be seen in APS and they are usually seen on the legs. This case had an atypical presentation, as the initial presentation was painful necrotic ulcers over the legs, which resembled pyoderma gangrenosum and she had no systemic manifestations. There was no history of any arterial or venous thrombosis or any abortions. Antiphospholipid syndrome can be tricky to diagnose when cutaneous lesions are atypical. Nonetheless, it is very important to pin down this syndrome early due to its systemic complications.

  12. An atypical presentation of antiphospholipid antibody syndrome. (United States)

    D'souza, Deepti; Dandakeri, Sukumar; Bhat, M Ramesh; Srinath, M K


    Cutaneous manifestations in antiphospholipid antibody syndrome (APS) though common, are extremely diverse and it is important to know which dermatological finding should prompt consideration of antiphospholipid syndrome. The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis, and systemic involvement is invariably an accomplice in APS. Cutaneous ulcers with sharp margins can be seen in APS and they are usually seen on the legs. This case had an atypical presentation, as the initial presentation was painful necrotic ulcers over the legs, which resembled pyoderma gangrenosum and she had no systemic manifestations. There was no history of any arterial or venous thrombosis or any abortions. Antiphospholipid syndrome can be tricky to diagnose when cutaneous lesions are atypical. Nonetheless, it is very important to pin down this syndrome early due to its systemic complications.

  13. Primary atypical sacral meningioma- not always benign

    Energy Technology Data Exchange (ETDEWEB)

    Bhadra, A.K.; Casey, A.T.H.; Saifuddin, A.; Briggs, T.W. [Royal National Orthopaedic Hospital, Stanmore, London (United Kingdom)


    We present a case of an atypical recurrent meningioma of the sacrum with pulmonary metastasis in a 31-year-old man. He presented with deep-seated buttock pain and urinary hesitancy for 3 months. MRI revealed a lesion occupying the central and left side of the sacral canal at the S1-S2 level. Surgical excision of the lesion via a posterior approach was undertaken, and the patient became symptom-free post-operatively. Histology confirmed atypical meningioma. Eight months later he re-presented with similar symptoms, and MRI confirmed local recurrence. The patient underwent left hemisacrectomy. Six months later he again presented with low back pain and MRI confirmed a second local recurrence. A CT scan of the chest showed multiple lung metastases. The patient died of a severe chest infection 18 months later. (orig.)

  14. Atypical presentations of methemoglobinemia from benzocaine spray. (United States)

    Tantisattamo, Ekamol; Suwantarat, Nuntra; Vierra, Joseph R; Evans, Samuel J


    Widely used for local anesthesia, especially prior to endoscopic procedures, benzocaine spray is one of the most common causes of iatrogenic methemoglobinemia. The authors report an atypical case of methemoglobinemia in a woman presenting with pale skin and severe hypoxemia, after a delayed repeat exposure to benzocaine spray. Early recognition and prompt management of methemoglobinemia is needed in order to lessen morbidity and mortality from this entity.

  15. An atypical mycobacterial infection of the shoulder

    Directory of Open Access Journals (Sweden)

    Christopher L Talbot


    Full Text Available Mycobacterium malmoense is an acid-fast non-tuberculous organism that most commonly causes pulmonary infection. Extrapulmonary infection has also been reported. With an increased emphasis being placed on the clinical importance of this organism, especially within Europe, we report the first case of septic arthritis of the shoulder caused by this organism. We also highlight the importance of considering atypical mycobacterium infection in the differential diagnosis of shoulder infection and issues surrounding the management of this entity.

  16. Atypical reactive histiocytosis. A case report.

    Directory of Open Access Journals (Sweden)

    Jorge E. Barleta del Castillo


    Full Text Available This paper presents the case of a 50 year old chronic alcoholic and heavy smoker female that was assisted at the provincial university hospital ¨Dr. Gustavo Aldereguía Lima¨ in Cienfuegos city due to a severe adenic syndrome and who was diagnosed as a case of atypical reactive histiocytosis , problem which disappeared with the abstinence of toxic habits, improving her health.

  17. Congenital left atrial appendage aneurysm: Atypical presentation

    Directory of Open Access Journals (Sweden)

    Mehdi Bamous


    Full Text Available Congenital left atrial appendage aneurysm is a rare condition caused by dysplasia of the atrial muscles. We report a case of a 14-year-old boy, with a 5-month history of cough and in sinus rhythm. Transthoracic echocardiography and computerized tomographic angiography confirmed the aneurysm of the left atrial appendage which was resected through median sternotomy on cardiopulmonary bypass. This case is presented not only for its rarity but also for its atypical clinical presentation.

  18. Atypical manifestations of multiple myeloma: Radiological appearance

    Energy Technology Data Exchange (ETDEWEB)

    Hess, Thomas [Department of Radiology, St-Vincenz Hospital, Auf dem Schafsberg, D-65549 Limburg (Germany)]. E-mail:; Egerer, Gerlinde [Department of Internal Medicine V, Haematology/Oncology, University of Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg (Germany); Kasper, Bernd [Department of Internal Medicine V, Haematology/Oncology, University of Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg (Germany); Rasul, Kakil Ibrahim [Hamad Medical Center, Moha (Qatar); Goldschmidt, Hartmut [Department of Internal Medicine V, Haematology/Oncology, University of Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg (Germany); Kauffmann, G.W. [Department of Radiology, University of Heidelberg, Im Neuenheimer Feld 110, 69120 Heidelberg (Germany)


    Diagnostic procedures performed on patients with multiple myeloma typically reveal lytic bone lesions, osteopenia or osteoporosis, bone marrow infiltration by plasma cells as well as overproduction of immunoglobulin or light chains in the serum or urine. Skeletal manifestations are extremely variable and the unusual forms have been described extensively. Extramedullary plasma-cell tumours (plasmocytoma) are found in about 5% of newly diagnosed patients with multiple myelomas. In this paper we present eight patients with atypical forms of multiple myeloma.

  19. Bisphosphonate-induced atypical subtrochanteric femoral fracture



    The use of bisphosphonates (BPs) is universally accepted in the management of osteoporosis. However, a small percentage of patients have been recognised to develop atypical subtrochanteric fractures of the femur with the prolonged use of BPs. We report a rare case of bilateral insufficiency lesions in the proximal femora, where a major subtrochanteric fracture developed with a minor fall. This was successfully treated with internal fixation using proximal femoral nail.

  20. An atypical case of segmental spinal dysgenesis

    Energy Technology Data Exchange (ETDEWEB)

    Zana, Elodie; Chalard, Francois; Sebag, Guy [Hopital Robert Debre, Department of Paediatric Imaging, Paris (France); Mazda, Keyvan [Hopital Robert Debre, Department of Paediatric Orthopaedic Surgery, Paris (France)


    Spinal segmental dysgenesis is a complex closed dysraphism. The diagnostic criteria are: lumbar or thoracolumbar vertebral dysgenesis causing kyphosis, focal spinal cord narrowing without exiting roots, deformity of the lower limbs and paraplegia or paraparesis. We present a newborn who showed atypical features of bifocal spinal cord narrowing, without any vertebral abnormality at the proximal level. This seems to be a variant of this rare entity, whose early diagnosis is important, as surgical stabilisation of the spine is required. (orig.)

  1. Prophylactic Nailing of Incomplete Atypical Femoral Fractures

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    Chang-Wug Oh


    Full Text Available Introduction. Recent reports have described the occurrence of low-energy subtrochanteric and femoral shaft fractures associated with long-term bisphosphonate use. Although information regarding the surgical treatment of these atypical femoral fractures is increasing, it is unclear if the preventive operation is useful in incomplete fractures. This study examined the results of preventive intramedullary nailing for incomplete atypical femoral fractures. Material and Methods. A retrospective search was conducted for patients older than 50 years receiving bisphosphonate therapy, with incomplete, nondisplaced fractures in either the subtrochanteric or diaphyseal area of the femur. Seventeen patients with a total of 20 incomplete, non-displaced lesions were included. The mean duration of bisphosphonate use was 50.5 months. Eleven of the 17 (64.7% patients had complete or incomplete fractures on the contralateral femur. All were treated with prophylactic fixation of an intramedullary (IM nail. The minimum followup was 12 months. Results. All cases healed with a mean period of 14.3 weeks. Nineteen of the 20 cases healed with the dissolution of incomplete fractures of the lateral aspect. A complete fracture developed at the time of nailing in one patient, but it healed with callus bridging. Conclusion. IM nailing appears to be a reliable way of preventing the progress of incomplete atypical femoral fractures.

  2. Variations in plasma and urinary lipids in response to enzyme replacement therapy for Fabry disease patients by nanoflow UPLC-ESI-MS/MS. (United States)

    Byeon, Seul Kee; Kim, Jin Yong; Lee, Jin-Sung; Moon, Myeong Hee


    A deficiency of α-galactosidase A causes Fabry disease (FD) by disrupting lipid metabolism, especially trihexosylceramide (THC). Enzyme replacement therapy (ERT) is clinically offered to FD patients in an attempt to lower the accumulated lipids. Studies on specific types of lipids that are directly or indirectly altered by FD are very scarce, even though they are crucial in understanding the biological process linked to the pathogenesis of FD. We performed a comprehensive lipid profiling of plasma and urinary lipids from FD patients with nanoflow liquid chromatography electrospray-ionization tandem mass spectrometry (nLC-ESI-MS/MS) and identified 129 plasma and 111 urinary lipids. Among these, lipids that exhibited alternations (>twofold) in patients were selected as targets for selected reaction monitoring (SRM)-based high-speed quantitation using nanoflow ultra-performance LC-ESI-MS/MS (nUPLC-ESI-MS/MS) and 31 plasma and 26 urinary lipids showed significant elevation among FD patients. Higher percentages of sphingolipids (SLs; 48% for plasma and 42% for urine) were highly elevated in patients; whereas, a smaller percentage of phospholipids (PLs; 15% for plasma and 13% for urine) were significantly affected. Even though α-galactosidase A is reported to affect THC only, the results show that other classes of lipids (especially SLs) are changed as well, indicating that FD not only alters metabolism of THC but various classes of lipids too. Most lipids showing significant increases in relative amounts before ERT decreased after ERT, but overall, ERT influenced plasma lipids more than urinary lipids.

  3. Atypical pityriasis versicolor case report

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    Full Text Available Pityriasis versicolor is a superficial fungal infection caused by mycelial form of Malassezia spp, which is confined to stratum corneum. It usually present in the trunk as either hypo or hyperpigmented, aymptomatic, round to oval macules of varying sizes, which may merged to form geographic shape. Diagnosis is usually done clinically, or KOH examination which shows typical spagetti and meat balls appearances, or even by wood’s lamp which shows orange to yellow fluorescence. The case series had been recording in between 2012 to 2013. Within that period, we had recorded 32 cases. All the patients which we had recorded presented with multiple, asymptomatic macules of small sizes varying from 1-2 cm in diameter to 3-4mm in diameter, usually round to oval, hypopigmented, non scaly lesions. 26 patients had lesions on forearms, 3 patients had lesions on dorsa of hands bilaterally, 3 patients had similar kind of lesions on thigh. Besnier’s test was positive in 14 (43.75% patients. KOH examinations showed fungal hyphae in 14 (33.33% patients with typical spagetti and meat balls appearances in 9 (8.13% patients. All of them were given and all of them got response and healed within 2-4 months.

  4. Diagnosis of Fanconi anemia in children with atypical clinical features: a primary study

    Institute of Scientific and Technical Information of China (English)

    LIU Rong; HU Tao; LI Jun-hui; LIANG Chao; GU Wei-yue; SHI Xiao-dong; WANG Hong-xing


    Background Fanconi anemia is a severe congenital disorder associated with mutations in a cluster of genes responsible for DNA repair.Arriving at an accurate and timely diagnosis can be difficult in cases of Fanconi anemia with atypical clinical features.It is very important to increase the rate of accurate diagnosis for such cases in a clinical setting.The purpose of this study is to explore the clinical diagnosis of Fanconi anemia in children with atypical clinical features.Methods Six cases of Fanconi anemia with atypical clinical features were enrolled in the study,and their clinical features were recorded,their FANCA gene transcription was assessed by RT-PCR,and FANCA mutations and the ubiquitination of FANCD2 protein were analyzed using DNA sequencing and western blotting respectively.Results All six cases showed atypical clinical features including no apparent deformities,lack of response to immune therapy,and progressively increasing bone marrow failure.They also have significantly increased fetal hemoglobin,negative mitomycin-induced fracture test results,and carry a FANCA gene missense mutation.Single protein ubiquitination of FANCD2 was not observed in those patients.Conclusion The combination of clinical features,FANCA pathogenic gene mutation genotype and the absence of FANCD2 protein ubiquitination are helpful in the accurate and timely diagnosis of Fanconi anemia in children.

  5. Transpupillary thermotherapy for atypical central serous chorioretinopathy

    Directory of Open Access Journals (Sweden)

    Kawamura R


    Full Text Available Ryosuke Kawamura1,2, Hidenao Ideta1, Hideyuki Hori1, Kenya Yuki2, Tsuyoshi Uno1, Tatsurou Tanabe1, Kazuo Tsubota2, Tsutomu Kawasaki11Ideta Eye Hospital, Kumamoto, Japan; 2Keio University, School of Medicine, Department of Ophthalmology, Tokyo, JapanBackground: Central serous chorioretinopathy (CSC has been traditionally treated with laser photocoagulation. We thought that transpupillary thermotherapy (TTT utilizing a lower temperature than that of conventional laser photocoagulation might minimize permanent retinal and choroidal damage. Studies suggest that undesirable effects on vision due to TTT are minimal even if it is applied to foveal and/or parafoveal lesions when TTT requires a larger irradiation spot. The aim of this study was to evaluate the efficacy of TTT in the management of atypical CSC.Methods: We defined atypical CSC as bullous retinal detachment with diffuse or several leakages, severe leakage with fibrin formation under serous retinal detachment, or leakage within a pigment epithelium detachment. Eight consecutive patients with atypical CSC underwent visual acuity testing, ophthalmic examination, color photography, fluorescein angiography, and optical coherence tomography to evaluate the results of transpupillary thermotherapy. Retreatment of atypical CSC was based on ophthalmic examination, optical coherence tomography, and fluorescein angiography. TTT was performed on the leaking spots shown in fluorescein angiography, with a power of 50–250 mW, spot size of 500–1200 µm, and exposure time of 13–60 seconds to minimize retinal damage.Results: In five of eight affected eyes, serous detachments completely resolved within 1 month after the initial TTT. One eye had persistent subretinal fluid and required a second TTT treatment. Two eyes showed no resolution of CSC and were treated by conventional photocoagulation. Initial best-corrected visual acuity (BCVA ranged from 20/600 to 20/20 (mean, 20/40; median, 20/30. Final BCVA

  6. Effects of CB1 receptor agonism and antagonism on behavioral fear and physiological stress responses in adult intact, ovariectomized, and estradiol-replaced female rats. (United States)

    Simone, J J; Malivoire, B L; McCormick, C M


    There is growing interest in the development of cannabis-based therapies for the treatment of fear and anxiety disorders. There are a few studies, but none in females, of the effects of the highly selective cannabinoid receptor type 1 (CB1) agonist, arachidonyl 2'-chlorethylamide (ACEA), on behavioral fear. In experiment 1 involving gonadally-intact females, ACEA (either 0.1 or 0.01 mg/kg) was without effect in the elevated plus maze (EPM), and the lower dose decreased anxiety in the open field test (OFT). AM251 increased anxiety in the EPM and decreased locomotor activity in the OFT. Twenty-four hours after fear conditioning, neither ACEA nor AM251 affected generalized fear or conditioned fear recall. AM251 and 0.1 mg/kg ACEA impaired, and 0.01 mg/kg ACEA enhanced, within-session fear extinction. AM251 increased plasma corticosterone concentrations after the fear extinction session, whereas ACEA was without effect. Based on evidence that estradiol may moderate the effects of CB1 receptor signaling in females, experiment 2 involved ovariectomized (OVX) rats provided with 10-μg 17β-Estradiol and compared with OVX rats without hormone replacement (oil vehicle). Irrespective of hormone treatment, AM251 increased anxiety in the EPM, whereas ACEA (0.01 mg/kg) was without effect. Neither hormone nor drug altered anxiety in the OFT, but estradiol increased and AM251 decreased distance traveled. After fear conditioning, AM251 decreased generalized fear. Neither hormone nor drug had any effect on recall or extinction of conditioned fear, however, ACEA and AM251 increased fear-induced plasma corticosterone concentrations. Further, when results with intact rats were compared with those from OVX rats, gonadal status did not moderate the effects of either AM251 or ACEA, although OVX displayed greater anxiety and fear than did intact rats. Thus, the effects of CB1 receptor antagonism and agonism in adult female rats do not depend on ovarian estradiol.

  7. Effects of typical and atypical antipsychotic drugs on gene expression profiles in the liver of schizophrenia subjects

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    Song Jonathan


    Full Text Available Abstract Background Although much progress has been made on antipsychotic drug development, precise mechanisms behind the action of typical and atypical antipsychotics are poorly understood. Methods We performed genome-wide expression profiling to study effects of typical antipsychotics and atypical antipsychotics in the postmortem liver of schizophrenia patients using microarrays (Affymetrix U133 plus2.0. We classified the subjects into typical antipsychotics (n = 24 or atypical antipsychotics (n = 26 based on their medication history, and compared gene expression profiles with unaffected controls (n = 34. We further analyzed individual antipsychotic effects on gene expression by sub-classifying the subjects into four major antipsychotic groups including haloperidol, phenothiazines, olanzapine and risperidone. Results Typical antipsychotics affected genes associated with nuclear protein, stress responses and phosphorylation, whereas atypical antipsychotics affected genes associated with golgi/endoplasmic reticulum and cytoplasm transport. Comparison between typical antipsychotics and atypical antipsychotics further identified genes associated with lipid metabolism and mitochondrial function. Analyses on individual antipsychotics revealed a set of genes (151 transcripts, FDR adjusted p Conclusion Typical antipsychotics and atypical antipsychotics affect different genes and biological function in the liver. Typical antipsychotic phenothiazines exert robust effects on gene expression in the liver that may lead to liver toxicity. The genes found in the current study may benefit antipsychotic drug development with better therapeutic and side effect profiles.

  8. Acute Kidney Injury and Atypical Features during Pediatric Poststreptococcal Glomerulonephritis

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    Rose M. Ayoob


    Full Text Available The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males, mean age of 8 years, were analyzed. Ninety-four percent had elevated serum BUN levels and decreased GFR. Four of the hospitalized patients had complex presentations that included AKI along with positive ANA or ANCAs. Three patients required renal replacement therapy and two were thrombocytopenic. PSGN usually does not occur as a severe nephritis. Over the 12-month study period, 17 cases associated with low serum albumin in 53%, acute kidney injury in 94%, and thrombocytopenia in 18% were treated. The presentation of PSGN may be severe and in a small subset have associations similar to SLE nephritis findings including AKI, positive ANA, and hematological anomalies.

  9. Comparative, validity and responsiveness of the HOOS-PS and KOOS-PS to the WOMAC physical function subscale in total joint replacement for osteoarthritis

    DEFF Research Database (Denmark)

    Davis, A M; Perruccio, A V; Canizares, M


    . Construct validity and responsiveness were compared to the Western Ontario McMaster Universities' Osteoarthritis Index (WOMAC) Likert 3.0 physical function (PF) subscale and the PF excluding the items in the short measures (PF-exclusions). METHODS: Participants completed the full HOOS or KOOS, measures...... and PF-exclusions with fatigue, CPG, anxiety and depression and HOOS/KOOS pain scales would differ by magnitudes of 1. RESULTS: The THR group (n=201) had a mean age of 62...

  10. Osteocyte physiology and response to fluid shear stress are impaired following exposure to cobalt and chromium: Implications for bone health following joint replacement. (United States)

    Shah, Karan M; Orton, Peter; Mani, Nick; Wilkinson, Jeremy Mark; Gartland, Alison


    The effects of metal ion exposure on osteocytes, the most abundant cell type in bone and responsible for coordinating bone remodeling, remain unclear. However, several studies have previously shown that exposure to cobalt (Co(2+) ) and chromium (Cr(3+) ), at concentrations equivalent to those found clinically, affect osteoblast and osteoclast survival and function. In this study, we tested the hypothesis that metal ions would similarly impair the normal physiology of osteocytes. The survival, dendritic morphology, and response to fluid shear stress of the mature osteocyte-like cell-line MLO-Y4 following exposure to clinically relevant concentrations and combinations of Co and Cr ions were measured in 2D-culture. Exposure of MLO-Y4 cells to metal ions reduced cell number, increased dendrites per cell and increased dendrite length. We found that combinations of metal ions had a greater effect than the individual ions alone, and that Co(2+) had a predominate effect on changes to cell numbers and dendrites. Combined metal ion exposure blunted the responses of the MLO-Y4 cells to fluid shear stress, including reducing the intracellular calcium responses and modulation of genes for the osteocyte markers Cx43 and Gp38, and the signaling molecules RANKL and Dkk-1. Finally, we demonstrated that in the late osteoblasts/early osteocytes cell line MLO-A5 that Co(2+) exposure had no effect on mineralization, but Cr(3+) treatment inhibited mineralization in a dose-dependent manner, without affecting cell viability. Taken together, these data indicate that metal exposure can directly affect osteocyte physiology, with potential implications for bone health including osseointegration of cementless components, and periprosthetic bone remodeling. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society. J Orthop Res 35:1716-1723, 2017. © 2016 The Authors. Journal of Orthopaedic Research Published by Wiley

  11. Current practice in primary total hip replacement: results from the National Hip Replacement Outcome Project


    Best, A. J.; Fender, D.; Harper, W. M.; McCaskie, A. W.; Oliver, K; Gregg, P J


    As part of the National Study of Primary Hip Replacement Outcome, 402 consultant orthopaedic surgeons from three regions were contacted by postal questionnaire which covered all aspects of total hip replacement (THR). There was a 70% response rate of which 71 did not perform hip surgery, a further 33 refused to take part, leaving 181 valid responses. Preoperative assessment clinics were used by 89% of surgeons, but anaesthetists and rehabilitation services were rarely involved at this stage. ...

  12. Robotic mitral valve replacement. (United States)

    Senay, Sahin; Gullu, Ahmet Umit; Kocyigit, Muharrem; Degirmencioglu, Aleks; Karabulut, Hasan; Alhan, Cem


    Robotic surgical techniques allow surgeons to perform mitral valve surgery. This procedure has gained acceptance, particularly for mitral valve repair in degenerative mitral disease. However, mitral repair may not always be possible, especially in severely calcified mitral valve of rheumatic origin. This study demonstrates the basic concepts and technique of robotic mitral valve replacement for valve pathologies that are not suitable for repair.

  13. Replacing America's Job Bank (United States)

    Vollman, Jim


    The Job Central National Labor Exchange ( has become the effective replacement for America's Job Bank with state workforce agencies and, increasingly, with community colleges throughout the country. The American Association of Community Colleges (AACC) has formed a partnership with Job Central to promote its use throughout the…

  14. Replacing America's Job Bank (United States)

    Vollman, Jim


    The Job Central National Labor Exchange ( has become the effective replacement for America's Job Bank with state workforce agencies and, increasingly, with community colleges throughout the country. The American Association of Community Colleges (AACC) has formed a partnership with Job Central to promote its use throughout the…

  15. Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

    Energy Technology Data Exchange (ETDEWEB)

    Simsek, Enver [Department of Paediatrics, Duzce Medical Faculty, Abant Izzet Baysal University, Konuralp-Duzce (Turkey); Yavuz, Cevdet [Department of Neurosurgery, Duzce Medical Faculty, Abant Izzet Baysal University, Konuralp-Duzce (Turkey); Ustundag, Nil [Department of Pathology, Abant Izzet Baysal University School of Medicine, Konuralp-Duzce (Turkey)


    A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child. (orig.)

  16. Response surface optimization of low-fat ice cream production by using resistant starch and maltodextrin as a fat replacing agent. (United States)

    Azari-Anpar, Mojtaba; Khomeiri, Morteza; Ghafouri-Oskuei, Hamed; Aghajani, Narjes


    In this research, maltodextrin (0, 1 and 2% w/w) and resistant starch (0, 1 and 2% w/w) were used in the formulation of low-fat ice cream (4% fat) and their effects on the physicochemical and sensory properties were investigated. The optimum levels of maltodextrin and resistant starch were determined by response surface methodology. Increment of maltodextrin and resistant starch increased acidity, viscosity, melting rate, time of dripping and overrun but decreased melting rate of ice cream. Results showed that the incorporation of maltodextrin and resistant starch at 0 and 2% w/w respectively, resulted into ice cream with suitable viscosity, melting rate, first dripping time, overrun and acidity.

  17. Wilson’s disease: Atypical imaging features

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    Venugopalan Y Vishnu


    Full Text Available Wilson’s disease is a genetic movement disorder with characteristic clinical and imaging features. We report a 17- year-old boy who presented with sialorrhea, hypophonic speech, paraparesis with repeated falls and recurrent seizures along with cognitive decline. He had bilateral Kayser Flescher rings. Other than the typical features of Wilson’s disease in cranial MRI, there were extensive white matter signal abnormalities (T2 and FLAIR hyperintensities and gyriform contrast enhancement which are rare imaging features in Wilson's disease. A high index of suspicion is required to diagnose Wilson’s disease when atypical imaging features are present.

  18. Atypical presentation of mucopolysaccharidosis type IVA

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    Eric T. Rush


    While this case describes neither a novel condition nor a new mutation, it does illustrate three important points in the diagnosis of patients with atypical forms of MPS IVA. First, that in many instances urine glycosaminoglycan analysis is not sufficient to rule out MPS IVA as a potential diagnosis. Patients in whom biochemical screening is advised should have measurement of leukocyte enzymatic activity. Second, that in patients with radiographic evidence of spondyloepiphyseal dysplasia with additional features or with normal targeted testing, MPS IVA should remain in the differential diagnosis. Third, that whole exome sequencing represents a viable diagnostic platform for evaluation of patients with unknown skeletal or metabolic disease.

  19. Atypical calcific tendinitis with cortical erosions

    Energy Technology Data Exchange (ETDEWEB)

    Kraemer, E.J. [College of Medicine, Univ. of Iowa, Iowa City, IA (United States); El-Khoury, G.Y. [Dept. of Radiology and Orthopaedics, Univ. of Iowa, Iowa City, IA (United States)


    Objective. To present and discuss six cases of calcific tendinitis in atypical locations (one at the insertion of the pectoralis major and five at the insertion of the gluteus maximus).Patients and results. All cases were associated with cortical erosions, and five had soft tissue calcifications. The initial presentation was confusing and the patients were suspected of having infection or neoplastic disease.Conclusion. Calcific tendinitis is a self-limiting condition. It is important to recognize the imaging features of this condition to avoid unnecessary investigation and surgery. (orig.)

  20. Atypical outcome in attention deficit hyperactivity disorder. (United States)

    Schmidt, K; Freidson, S


    This report describes the course of psychiatric illness in two boys. Both presented with attention deficit hyperactivity disorder (ADHD) in midchildhood; after puberty, one boy developed a schizophrenic illness while the other boy developed a major affective illness. Although the major ADHD outcome studies have found no link between the childhood occurrence of ADHD and psychosis in adulthood, occasionally such a link may exist. The theoretical and practical implications of this finding are discussed. It should be noted, however, that such outcome is highly atypical and very rare.

  1. Gorlin’s syndrome: Atypical case report

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    Sanjay N. Agrawal


    Full Text Available Gorlin syndrome or basal cell nevus syndrome (BCNS is a rare autosomal dominant disorder. The condition appears to have complete penetrance and variable expressivity, which makes clinilcal presentation among families variable. All known BCNS carry mutations in PATCHED gene. A 65 years old male patient presented with complaints of characteristic skin lesions on his face, back, palms since early adulthood. The lesions were pigmented nodules with characteristic border. The histopathology showed characteristic features suggestive of Basal Cell Carcinoma (BCC. This case was atypical due to appearance of lesions quite later in life.

  2. Atypical And Severe Enlargement Of Right Atrium. (United States)

    Siniscalchi, Carmine; Rossetti, Pietro; Rocci, Anna; Rubino, Pasquale; Basaglia, Manuela; Gaibazzi, Nicola; Quintavalla, Roberto


    A 76 year-old woman was admitted to the Emergency Department for recent-onset dyspnea and cough. The electrocardiogram was considered inconclusive. A thoracic X-ray showed global cardiac profile enlargement. Computed tomography, acutely performed in the clinical suspicion of atypical pneumonia/myocarditis or pericardial effusion, showed cardiac enlargement especially of the right chambers. In order to investigate Ebstein's anomaly, pericardial cysts, tumors or other conditions of the right heart a simple trans-thoracic echocardiogram was performed. Four chambers view showed a giant right atrium aneurysm with moderate tricuspid regurgitation without stenosis or typical Ebstein's echocardiographic pattern.

  3. Atypical Log D profile of rifampicin

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    Mariappan T


    Full Text Available The distribution coefficient (log D values of rifampicin, an essential first-line antitubercular drug, at gastrointestinal pH conditions are not reported in literature. Hence determinations were made using n-octanol and buffers ranging between pH 1-7. Also, log D values were predicted using Prolog D. Both the determinations showed opposite behaviour. The atypical experimental log D profile of rifampicin could be attributed to its surface-active properties, which also explained the reported permeability behaviour of the drug in various gastrointestinal tract segments.

  4. Atypical giant chondroblastoma mimicking a chondrosarcoma. (United States)

    Dhanda, Sunita; Menon, Santosh; Gulia, Ashish


    Chondroblastoma is a rare, benign tumor derived from chondroblasts, commonly presenting in the second decade of life. It is usually found in the epiphysis or apophysis of long bones; however, it may rarely affect flat bones like scapula. Occasionally a histologically typical chondroblastoma may exhibit an aggressive behavior that is not normally associated with benign tumors such as a large size, pulmonary metastases, joint and soft-tissue infiltration and local recurrence. We present a case report of a patient with chondroblastoma showing atypical radiological presentation and non-concordance with age.

  5. Bisphosphonates and Atypical Fractures of Femur

    Directory of Open Access Journals (Sweden)

    Tero Yli-Kyyny


    Full Text Available Bisphosphonates are the most widely prescribed medicines for the treatment of osteoporosis and have generally been regarded as well-tolerated and safe drugs. Since 2005, there have been numerous case reports about atypical fractures of the femur linked to long-term treatment of osteoporosis with bisphosphonates. Some attempts to characterize pathophysiology and epidemiology of these fractures have been published as well. However, as the American Society for Bone and Mineral Research (ASBMR concluded in their task force report, the subject warrants further studies.

  6. Atypical presentation of childhood obsessive compulsive disorder

    Directory of Open Access Journals (Sweden)

    Satyakam Mohapatra


    Full Text Available Obsessive-compulsive disorder (OCD is one of the most prevalent psychiatric disorders in children and adolescents. The phenomenology of OCD in children and adolescent is strikingly similar to that of adults. But at times, the presentation of OCD may be so atypical or unusual in children and adolescents that may lead to misdiagnosis or delay in diagnosis. We report a case of 10-year-old child who was initially misdiagnosed with schizophrenia, and treated with antipsychotic for 2 months. But once the core symptoms were recognized as obsessions and compulsions and appropriately treated in the line of OCD, the symptoms resolved significantly.

  7. MBL2 polymorphisms in women with atypical squamous cells of undetermined significance. (United States)

    Zupin, Luisa; Polesello, Vania; Casalicchio, Giorgia; Freato, Nadia; Maestri, Iva; Comar, Manola; Crovella, Sergio; Segat, Ludovica


    Infection with high risk Human papillomavirus (HPV) is the main known cause of cervical cancer. HPV induces different grades of lesions: among them, Atypical squamous cells of undetermined significance are abnormal lesions that could evolve in pre-cancer lesions or spontaneously regress. The mannose binding lectin (MBL) is an innate immunity serum protein also found in cervico-vaginal mucosa, whose expression is known to be affected by polymorphisms in exon 1 and promoter of the MBL2 gene. In the present study the possible association between MBL2 functional polymorphisms and susceptibility to develop atypical squamous cells of undetermined significance was investigated in a group of women from North-East of Italy, stratified for HPV infection status. The MBL2 D and O alleles and the deficient producer combined genotypes, responsible for low MBL production, were more represented among atypical squamous cells of undetermined significance positive women than healthy controls and the results were confirmed when only HPV negative samples were considered. These results suggest a possible involvement of MBL2 functional polymorphisms in atypical squamous cells of undetermined significance susceptibility. © 2015 Wiley Periodicals, Inc.

  8. Exercise improves the effects of testosterone replacement therapy and the durability of response after cessation of treatment: a pilot randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Dae-Yeon Cho


    Full Text Available The effects of the combination of exercise and TRT on symptoms of late-onset hypogonadism (LOH and the durability of response after cessation of TRT were investigated. A total of fifty patients with erectile dysfunction (ED who had a sedentary lifestyle and low serum total testosterone (T levels were enrolled and followed for 20 weeks. Patients were randomly divided into two groups; all of them received T gel for 12 weeks and it was discontinued for 8 weeks. Patients assigned to Group II were offered a supervised exercise program for 20 weeks. Measurement of serological testing was performed and self-assessment questionnaires and Global Assessment Question (GAQ were asked. Baseline characteristics and the initial symptom scores showed no significant difference between the two groups. Serum total T levels and the symptom scores were increased at 12 weeks in both groups, and Group II showed better results with statistical significance. There was a decrease in T levels and worsening of symptom scores at week 20 compared to week 12 in both groups, and Group II showed better results with statistical significance. On the GAQ, Group II showed higher ratio of "yes" at week 12 and the same tendency was sustained at week 20 with significant difference between two groups. The combination of exercise and TRT showed significant improvements in serum T levels and LOH symptoms compared to TRT alone. In addition, these improvements were maintained in the combination group with continuous exercise, even after cessation of TRT.

  9. Atypical Celiac Disease: From Recognizing to Managing

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    B. Admou


    Full Text Available The nonclassic clinical presentation of celiac disease (CD becomes increasingly common in physician’s daily practice, which requires an awareness of its many clinical faces with atypical, silent, and latent forms. Besides the common genetic background (HLA DQ2/DQ8 of the disease, other non-HLA genes are now notably reported with a probable association to atypical forms. The availability of high-sensitive and specific serologic tests such as antitissue transglutuminase, antiendomysium, and more recent antideamidated, gliadin peptide antibodies permits to efficiently uncover a large portion of the submerged CD iceberg, including individuals having conditions associated with a high risk of developing CD (type 1 diabetes, autoimmune diseases, Down syndrome, family history of CD, etc., biologic abnormalities (iron deficiency anemia, abnormal transaminase levels, etc., and extraintestinal symptoms (short stature, neuropsychiatric disorders, alopecia, dental enamel hypoplasia, recurrent aphtous stomatitis, etc.. Despite the therapeutic alternatives currently in developing, the strict adherence to a GFD remains the only effective and safe therapy for CD.

  10. A-type CpG ODN with higher binding affinity to LvToll1 could probably activate downstream IFN system-like antiviral response in shrimp Litopenaeus vannamei

    Directory of Open Access Journals (Sweden)

    J Xu


    Full Text Available CpG oligodeoxynucleotides (CpG ODNs is a widely used immune adjuvant, which could activate various immune responses including antiviral response through interaction with Toll-like receptor 9 (TLR9 in mammals. In the present study, four types of CpG ODN (CpG-A, CpG-B CpG-C, and CpG-P were synthesized and injected to the shrimp Litopenaeus vannamei in order to evaluate their immune enhancement effect in shrimp. The copy numbers of white spot syndrome virus in the shrimps treated with different types of CpG ODNs were of 3.10×105 (CpG-A, 8.32×105 (CpG-B, 9.84×105 (CpG-C, and 8.12×105 (CpG-P copies ng-1 DNA respectively, which were significantly lower (p < 0.01 than that in PBS group (1.70×106 copies ng-1 DNA. Surface plasmon resonance (SPR assay revealed that the four types of CpG ODN displayed different binding affinity to LvToll1, LvToll2 and LvToll3, and the highest binding affinity was observed between CpG-A and LvToll1. Correspondingly, the mRNA transcripts of LvTolls were up-regulated significantly in CpG-A stimulated shrimps,which was significantly higher than that in CpG-B, CpG-C and CpG-P groups (p < 0.01. The phagocytic rate and ROS level of shrimp hemocytes in CpG-A and CpG-B groups increased significantly compared with that in other groups, which were 1.63-fold, 9.98-fold (p < 0.01 in CpG-A and 1.60-fold, 4.92-fold (p < 0.01 in CpG-B higher than those in PBS group, respectively. Moreover, after CpG-A stimulation, the probable IFN level in shrimp plasma increased to 2.60-fold (p < 0.01 of that in PBS group, and the mRNA expressions of IFN system-like antiviral genes (LvIRF, LvVago4 and LvSTAT were also significantly up-regulated in CpG-A group, displaying a stronger response than that in CpG-B, CpG-C and CpG-P groups. The results indicated that CpG-A could promote the cellular and humoral immunity in shrimp, and induce relatively higher antiviral immune response among the four CpG ODNs. It provided useful information to understand

  11. Supplementation based on protein or energy ingredients to beef cattle consuming low-quality cool-season forages: II. Performance, reproductive, and metabolic responses of replacement heifers. (United States)

    Cappellozza, B I; Cooke, R F; Reis, M M; Moriel, P; Keisler, D H; Bohnert, D W


    This experiment evaluated the influence of supplement composition on performance, reproductive, and metabolic responses of Angus × Hereford heifers consuming a low-quality cool-season forage (8.7% CP and 57% TDN). Sixty heifers (initial age = 226 ± 3 d) were allocated into 15 drylot pens (4 heifers/pen and 5 pens/treatment) and assigned to 1) supplementation with soybean meal (PROT), 2) supplementation with a mixture of cracked corn, soybean meal, and urea (68:22:10 ratio, DM basis; ENER), or 3) no supplementation (CON). Heifers were offered meadow foxtail (Alopecurus pratensis L.) hay for ad libitum consumption during the experiment (d -10 to 160). Beginning on d 0, PROT and ENER were provided daily at a rate of 1.30 and 1.40 kg of DM/heifer to ensure that PROT and ENER intakes were isocaloric and isonitrogenous. Hay and total DMI were recorded for 5 consecutive days during each month of the experiment. Blood was collected every 10 d for analysis of plasma progesterone to evaluate puberty attainment. Blood samples collected on d -10, 60, 120, and 150 were also analyzed for plasma concentrations of plasma urea N (PUN), glucose, insulin, IGF-I, NEFA, and leptin. Liver samples were collected on d 100 from 2 heifers/pen and analyzed for mRNA expression of genes associated with nutritional metabolism. No treatment effect was detected (P = 0.33) on forage DMI. Total DMI, ADG, and mean concentrations of glucose, insulin, and IGF-I as well as hepatic mRNA expression of IGF-I and IGFBP-3 were greater (P ≤ 0.02) for PROT and ENER compared with CON and similar between PROT and ENER (P ≥ 0.13). Mean PUN concentrations were also greater (P beef heifers consuming a low-quality cool-season forage had a similar increase in DMI, growth, and overall metabolic status if offered supplements based on soybean meal or corn at 0.5% of BW.

  12. A macroprolactinoma becoming resistant to cabergoline and developing atypical pathology (United States)

    Farah, George; Fathelrahman, Ahmed; Cudlip, Simon; Ansorge, Olaf; Karavitaki, Niki; Grossman, Ashley B


    Summary Pituitary adenomas are a common intracranial neoplasm, usually demonstrating a benign phenotype. They can be classified according to pathological, radiological or clinical behaviour as typical, atypical or carcinomas, invasive or noninvasive, and aggressive or nonaggressive. Prolactinomas account for 40–60% of all pituitary adenomas, with dopamine agonists representing the first-line treatment and surgery/radiotherapy reserved for drug intolerance/resistance or in neuro-ophthalmological emergencies. We present the case of a 62-year-old man with an apparently indolent prolactin-secreting macroadenoma managed with partial resection and initially showing a biochemical response to cabergoline. Five years later, the tumour became resistant to cabergoline, despite a substantial increase in dosage, showing rapid growth and causing worsening of vision. The patient then underwent two further transsphenoidal operations and continued on high-dose cabergoline; despite these interventions, the tumour continued enlarging and prolactin increased to 107 269 U/L. Histology of the third surgical specimen demonstrated features of aggressive behaviour (atypical adenoma with a high cell proliferation index) not present in the tumour removed at the first operation. Subsequently, he was referred for radiotherapy aiming to control tumour growth. Learning points: The development of secondary resistance to dopamine agonists (DAs) is a serious sign as it may be associated with de-differentiation of the prolactinoma and thus of aggressive or malignant transformation. Significant de-differentiation of the adenoma documented on consecutive histologies suggests a possible transition to malignancy. A combination of histological ‘alarm’ features associated with persistent growth and escape from DAs treatment in recurrent adenomas should alert clinicians and demands close follow-up. A multidisciplinary approach by pathologists, endocrinologists and neurosurgeons is essential. PMID

  13. [Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy]. (United States)

    Silva, Helga Cristina Almeida da; Hiray, Marcia; Vainzof, Mariz; Schmidt, Beny; Oliveira, Acary Souza Bulle; Amaral, José Luiz Gomes do


    Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU.L(-1) (normal 174IU.L(-1)). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. Copyright © 2017 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  14. The Seropositivity Rate of Atypical Agents in Patients with Community-Acquired Pneumonia

    Directory of Open Access Journals (Sweden)

    Ruhan Karakoc Gunes


    Full Text Available The aim of this study was to investigate the IgM antibody positivities of atypical pneumonia agents in patients with community-acquired pneumonia (CAP, and to compare the results with controls. The serum samples which were collected from 87 adult patients and 21 healthy controls have been investigated by a commercial ELISA (Pneumobact ELISA IgM, Vircell, Spain in which four different atypical pneumonia agents were fixed onto a slide. In the patients group, IgM positivity rates for the agents were as follows, respectively; 2.3% for Legionella pneumophila, 56.3% Chlamydia pneumoniae, 33.3% for Mycoplasma pneumoniae, 9.2% for Coxiella burnetii. The rates of IgM positivities in the control group varied 7% for all of the agents except M. Pneumoniae and C. Pneumoniae and 2 of these controls were positive for L. Pneumophila IgM, one was positive for C. Burnetii IgM. According to the statistical evaluation, there were significant differences for IgM seropositivities to Mycoplasma pneumoniae and Chlamydia Pneumoniae,between the patient and the control groups (p0.05. We showed that the seropositivity rate of atypical agents in patients with CAP was significantly higher when compared to healthy control group. This result suggests us, atypical agents might be responsible in CAP patients in a great amount. Furthermore, our study also suggests that clinical and radiological findings are not useful for discriminating atypical from typical pneumonia. [TAF Prev Med Bull 2007; 6(4.000: 279-284

  15. Atypical relapse of hemolytic uremic syndrome after transplantation.

    NARCIS (Netherlands)

    Olie, K.H.; Florquin, S.; Groothoff, J.W.; Verlaak, R.; Strain, L.; Goodship, T.H.; Weening, J.J.; Davin, J.C.


    Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of imme

  16. Aortic valve replacement

    DEFF Research Database (Denmark)

    Kapetanakis, Emmanouil I; Athanasiou, Thanos; Mestres, Carlos A


    BACKGROUND AND AIMS OF THE STUDY: Prompted by anecdotal evidence and observations by surgeons, an investigation was undertaken into the potential differences in implanted aortic valve prosthesis sizes, during aortic valve replacement (AVR) procedures, between northern and southern European...... countries. METHODS: A multi-institutional, non-randomized, retrospective analysis was conducted among 2,932 patients who underwent AVR surgery at seven tertiary cardiac surgery centers throughout Europe. Demographic and perioperative variables including valve size and type, body surface area (BSA) and early...

  17. Total ankle joint replacement. (United States)


    Ankle arthritis results in a stiff and painful ankle and can be a major cause of disability. For people with end-stage ankle arthritis, arthrodesis (ankle fusion) is effective at reducing pain in the shorter term, but results in a fixed joint, and over time the loss of mobility places stress on other joints in the foot that may lead to arthritis, pain and dysfunction. Another option is to perform a total ankle joint replacement, with the aim of giving the patient a mobile and pain-free ankle. In this article we review the efficacy of this procedure, including how it compares to ankle arthrodesis, and consider the indications and complications.

  18. Imaging the neurobiological substrate of atypical depression by SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Pagani, Marco [Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy); Karolinska University Hospital, Department of Nuclear Medicine, Stockholm (Sweden); Salmaso, Dario [Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy); Nardo, Davide [University of Rome La Sapienza, Department of Psychology, Rome (Italy); Jonsson, Cathrine; Larsson, Stig A. [Karolinska University Hospital, Department of Nuclear Medicine, Stockholm (Sweden); Jacobsson, Hans [Karolinska University Hospital, Department of Radiology, Stockholm (Sweden); Gardner, Ann [Karolinska University Hospital Huddinge, Karolinska Institutet, Department of Clinical Neuroscience, Section of Psychiatry, Stockholm (Sweden)


    Neurobiological abnormalities underlying atypical depression have previously been suggested. The purpose of this study was to explore differences at functional brain imaging between depressed patients with and without atypical features and healthy controls. Twenty-three out-patients with chronic depressive disorder recruited from a service for patients with audiological symptoms were investigated. Eleven fulfilled the DSM-IV criteria for atypical depression (mood reactivity and at least two of the following: weight gain, hypersomnia, leaden paralysis and interpersonal rejection sensitivity). Twenty-three healthy subjects served as controls. Voxel-based analysis was applied to explore differences in {sup 99m}Tc-HMPAO uptake between groups. Patients in the atypical group had a higher prevalence of bilateral hearing impairment and higher depression and somatic distress ratings at the time of SPECT. Significantly higher tracer uptake was found bilaterally in the atypical group as compared with the non-atypicals in the sensorimotor (Brodmann areas, BA1-3) and premotor cortex in the superior frontal gyri (BA6), in the middle frontal cortex (BA8), in the parietal associative cortex (BA5, BA7) and in the inferior parietal lobule (BA40). Significantly lower tracer distribution was found in the right hemisphere in the non-atypicals compared with the controls in BA6, BA8, BA44, BA45 and BA46 in the frontal cortex, in the orbito-frontal cortex (BA11, BA47), in the postcentral parietal cortex (BA2) and in the multimodal association parietal cortex (BA40). The differences found between atypical and non-atypical depressed patients suggest different neurobiological substrates in these patient groups. The putative links with the clinical features of atypical depression are discussed. These findings encourage the use of functional neuroimaging in psychiatric disorders. (orig.)

  19. The Optimum Replacement of Weapon

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xiao; ZHANG Jin-chun


    The theory of LCC (Life Cycle Cost) is applied in this paper. The relation between the economic life of weapon and the optimum replacement is analyzed. The method to define the optimum replacement time of weapon is discussed.

  20. Atypical parakeratosis: a marker of dysplasia? (United States)

    Voytek, T M; Kannan, V; Kline, T S


    The Bethesda System categorizes atypical parakeratosis (APK) as "ASCUS or SIL depending on the degree of cellular abnormalities." APK, however, is not well-defined. We retrospectively reviewed 68 cervicovaginal specimens with follow-up material to identify specific criteria and clinical significance of APK. APK cells were small cells, 2-3 times the diameter of neutrophil, with dense, orangeophilic cytoplasm, high nuclear cytoplasmic ratio, dense, often uneven chromatin, and irregular nuclear contour. Of 62 cases with APK, 37 had accompanying dysplastic cells. Of 25 cases with APK alone, follow-up revealed 12 with squamous intraepithelial lesion (5 HSIL and 7 LSIL) and 13 with benign changes. A major diagnostic pitfall of APK was inflammation with degeneration. Abundant APK cells, minimal inflammation and degeneration, and previous history of dysplasia frequently were associated with follow-up SIL. The findings of this study identify APK as an important marker for dysplasia that warrants careful evaluation and follow-up.

  1. Refractory Rheumatic Disorder: Atypical Postpregnancy Osteoporosis

    Directory of Open Access Journals (Sweden)

    Cindy Mourgues


    Full Text Available This is a case report on a young patient with severe osteoporosis that was initially revealed when she presented with polyarthralgia during her second pregnancy. Postpartum, the pain increased and her X-ray did not show any abnormalities. A bone scintigraphy was performed. It indicated an inflammatory rheumatic disorder. Six months after partum, an investigation of right coxalgia revealed a spontaneous basicervical fracture. Given the persistent polyarthralgia, the patient underwent a new scintigraphy, which revealed areas of what looked to be old rib and L1 fractures. A subsequent full body magnetic resonance imaging (MRI scan revealed signal abnormalities that could indicate multiple lower limb bone fractures. Despite exhaustive biological, radiological, and histological testing, no secondary cause for the osteoporosis was found. The patient was started on teriparatide. We finally concluded that, despite the atypical presentation, the patient was suffering from postpregnancy osteoporosis. It is possible that the frequency of occurrence of this still poorly understood disease is underestimated.

  2. Indications of atypical antipsychotics in the elderly. (United States)

    McKean, Andrew; Monasterio, Erik


    Atypical antipsychotics (AAP) have become some of the most commonly prescribed medications in primary and specialist care settings. Off-label prescribing accounts for much of the expanded use of AAPs. This has become common in the elderly. Marketing by pharmaceutical companies appears to have contributed to the off-label use of AAPs, in situations where their safety and efficacy is far from established. Although evidence provides varying degrees of support for their use for behavioural and psychological symptoms of dementia, augmentation of antidepressants in depression, anxiety, insomnia and in the management of psychosis in Parkinson's Disease, there are a number of potential problems with their expanded use in the elderly. These include weight gain, type two diabetes mellitus, sudden cardiac death and increased mortality rates in the elderly with dementia. It is recommended that whenever AAPs are used off-label, a review date is identified, informed consent is obtained and treatment and side-effects are closely monitored.

  3. Atypical Radiological Manifestation of Pulmonary Metastatic Calcification

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Hae; Kim, Eun Sun; Kim, Chul Hwan; Ham, Soo Youn; Oh, Yu Whan [Korea University College of Medicine, Seoul (Korea, Republic of)


    Metastatic pulmonary calcification is a condition of calcium deposition in the normal pulmonary parenchyma, and this is secondary to abnormal calcium metabolism without any prior soft tissue damage. The predisposing factors for this condition include chronic renal failure, hypercalcemia and increased tissue alkalinity. The most common radiologic manifestation consists of poorly defined nodular opacities in the upper lung zone. These opacities reflect the deposition of calcium salts in the pulmonary interstitium. We present here a case of metastatic pulmonary calcification in a patient who recovered from pneumonia with sepsis and whose high-resolution CT (HRCT) images demonstrated localized parenchymal airspace calcification that was limited to the bilateral lower lobes. These lower lobes had been involved with pneumonic consolidation without calcification, as seen on the previous CT scan. In summary, we report here on an atypical presentation of metastatic pulmonary calcification that showed dense airspace consolidation localized to the bilateral lower lobes in a patient with primary hyperparathyroidism and pneumonia.

  4. Achilles tendon rupture in atypical patient populations. (United States)

    Kingsley, Peter


    Rupture of the Achilles tendon is a significant injury, and the likelihood of a good recovery is directly associated with early diagnosis and appropriate referral. Such injuries are commonly assessed and identified by practitioners working in 'minors' areas of emergency departments or urgent care settings. The literature frequently describes rupture of the Achilles tendon as 'typically sport-related' affecting 'middle-aged weekend warriors', but this aetiology accounts for only about 70% of such injuries. Factors such as the natural ageing process, obesity and use of some commonly prescribed medications, can increase the risk of developing a tendinopathy and subsequent rupture, often from a seemingly insignificant incident. However, research suggests that injuries in this patient population are more likely be missed on first examination. This article describes risk factors that should alert clinicians to the possibility of Achilles tendon rupture in 'atypical' patient populations.

  5. Atypical Cogan's syndrome associated with coronary disease

    Institute of Scientific and Technical Information of China (English)

    Ivanovic Branislava; Tadic Marijana; Damjanov Nemanja; Simic Dragan; Zlatanovic Maja


    Cogan's syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis.Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS.

  6. Linguistic recycling in typical and atypical interaction. (United States)

    Perkins, Michael R


    I present evidence that linguistic "recycling" - i.e., the redeployment of linguistic material from prior utterances during conversation - is a striking and prevalent feature not only of interaction between typical speakers, but also, and notably, of interaction involving the communication impaired. In the latter case, recycling may sometimes be used as a compensatory communicative resource when linguistic ability is compromised. Despite its prevalence, however, recycling has largely been ignored by clinical linguists. In addition to providing illustrations of linguistic recycling across a range of communication disorders, I also examine how it is subserved by phenomena such as priming, short-term memory and alignment. I subsequently argue for a shift in perspective that puts recycling at the heart of our perception of how typical and atypical interaction works, and suggest a number of potential benefits for clinical linguistics, ranging from the way we understand and analyse communication disorders to how we assess and treat them.

  7. Clinical grand rounds: atypical hemolytic uremic syndrome. (United States)

    Hodgkins, Kavita S; Bobrowski, Amy E; Lane, Jerome C; Langman, Craig B


    Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening, chronic, genetic disease of uncontrolled alternative pathway complement activation. The understanding of the pathophysiology and genetics of this disease has expanded over recent decades and promising new developments in the management of aHUS have emerged. Regardless of the cause of aHUS, with or without a demonstrated mutation or autoantibody, blockade of terminal complement activation through C5 is of high interest as a mechanism to ameliorate the disease. Eculizumab, an existing monoclonal antibody directed against C5 with high affinity, prevents the perpetuation of the downstream activation of the complement cascade and the damage caused by generation of the anaphylotoxin C5a and the membrane attack complex C5b-9, by blocking C5 cleavage. We report the successful use of eculizumab in a patient after kidney transplantation and discuss the disease aHUS.

  8. Pulmonary Atypical Adenomatous Hyperplasia And Bronchioloalveolar Carcinoma

    Institute of Scientific and Technical Information of China (English)

    MeilinXu; XiaYang; ZhiyaoZhang


    OBJECTIVE To study the relationship between atypical adenomatous hyperplasia (AAH) and bronchioloalveolar carcinoma (BAC). METHODS Morphometric, immunohistochemical and ultrastructural analyses were performed in 4 patients with low grade AAH, 5 with high grade AAH and 7 with BAC. RESULTS The mean nuclear areas of high grade AAH and BAC were greater than those of low grade AAH (P<0.05); p53 protein expression was negative in 4 cases of low grade AAH,while the positive rates in high grade AAH and BAC were 40% (2/5) and 57% (4/7), respectively. CONCLUSION The development of BAC is stepwise. AAH appears to be a lesion closely related with BAC, probably as its genuine precursor.

  9. Brugada Syndrome with atypical characteristics: Case report

    Directory of Open Access Journals (Sweden)

    Hatem Ari


    Full Text Available The Brugada Syndrome (BrS is a heterogeneous genetic disease characterized by persistent or transient ST-segment elevation in the right precordial electrocardiography (ECG leads and a high incidence of sudden death and life-threatening ventricular tachyarrhythmias in patients with structurally normal hearts. The syndrome generally manifests in men during adulthood. The ECG manifestations can be overt or concealed. We report a case of BrS whose type 1 ECG pattern during febrile state converted to type 2 ECG after alleviation of fever with atypical characteristics (78-year-old woman with monomorphic ventricular tachycardia on holter monitoring, a history of the sudden infant death of her child, and without inducible ventricular arrhythmia by programed ventricular stimulation [PVS].

  10. Disentangling the Emerging Evidence around Atypical Fractures

    DEFF Research Database (Denmark)

    Abrahamsen, Bo; Clark, Emma M


    Atypical femur fractures are rare but a growing concern, as they are more common in patients who use bisphosphonates. The best radiology-based studies have had access to only short-term exposure data, while the studies using prescription databases with substantial long-term data did not have access...... to radiology reports. The interests of the patients are probably best served by reserving long-term bisphosphonate treatment for patients who are at the highest risk of osteoporotic fractures and considering drug holidays after 5 years in patients at low risk. Recent studies have further strengthened the case...... for active medical or surgical therapy in patients with incomplete fractures, but patient numbers are small, and randomized controlled trials may not be forthcoming in the immediate future. The recommendations made to establish an international database for such fractures have not yet been followed, and more...

  11. Atypical subtrochanteric and diaphyseal femoral fractures

    DEFF Research Database (Denmark)

    Shane, Elizabeth; Burr, David; Abrahamsen, Bo


    Bisphosphonates (BPs) and denosumab reduce the risk of spine and nonspine fractures. Atypical femur fractures (AFFs) located in the subtrochanteric region and diaphysis of the femur have been reported in patients taking BPs and in patients on denosumab, but they also occur in patients...... with no exposure to these drugs. In this report, we review studies on the epidemiology, pathogenesis, and medical management of AFFs, published since 2010. This newer evidence suggests that AFFs are stress or insufficiency fractures. The original case definition was revised to highlight radiographic features...... that distinguish AFFs from ordinary osteoporotic femoral diaphyseal fractures and to provide guidance on the importance of their transverse orientation. The requirement that fractures be noncomminuted was relaxed to include minimal comminution. The periosteal stress reaction at the fracture site was changed from...

  12. Emphysematous Cystitis: Report of an Atypical Case

    Directory of Open Access Journals (Sweden)

    Karen De Baets


    Full Text Available We report the atypical case of a nondiabetic 66-year old male with severe abdominal pain and vomiting who was found to have emphysematous cystitis. Of all gas-forming infections of the urinary tract emphysematous cystitis is the most common and the least severe. The major risk factors are diabetes mellitus and urinary tract obstruction. Most frequent causative pathogens are Escherichia coli and Klebsiella pneumoniae. The clinical presentation is nonspecific and ranges from asymptomatic urinary tract infection to urosepsis and septic shock. The diagnosis is made by abdominal imaging. Treatment consists of broad-spectrum antibiotics, bladder drainage, and management of the risk factors. Surgery is reserved for severe cases. Overall mortality rate of emphysematous cystitis is 7%. Immediate diagnosis and treatment is necessary because of the rapid progression to bladder necrosis, emphysematous pyelonephritis, urosepsis, and possibly fatal evolution.

  13. Power Plant Replacement Study

    Energy Technology Data Exchange (ETDEWEB)

    Reed, Gary


    This report represents the final report for the Eastern Illinois University power plant replacement study. It contains all related documentation from consideration of possible solutions to the final recommended option. Included are the economic justifications associated with the chosen solution along with application for environmental permitting for the selected project for construction. This final report will summarize the results of execution of an EPC (energy performance contract) investment grade audit (IGA) which lead to an energy services agreement (ESA). The project includes scope of work to design and install energy conservation measures which are guaranteed by the contractor to be self‐funding over its twenty year contract duration. The cost recovery is derived from systems performance improvements leading to energy savings. The prime focus of this EPC effort is to provide a replacement solution for Eastern Illinois University’s aging and failing circa 1925 central steam production plant. Twenty‐three ECMs were considered viable whose net impact will provide sufficient savings to successfully support the overall project objectives.

  14. Total disc replacement. (United States)

    Vital, J-M; Boissière, L


    Total disc replacement (TDR) (partial disc replacement will not be described) has been used in the lumbar spine since the 1980s, and more recently in the cervical spine. Although the biomechanical concepts are the same and both are inserted through an anterior approach, lumbar TDR is conventionally indicated for chronic low back pain, whereas cervical TDR is used for soft discal hernia resulting in cervicobrachial neuralgia. The insertion technique must be rigorous, with precise centering in the disc space, taking account of vascular anatomy, which is more complex in the lumbar region, particularly proximally to L5-S1. All of the numerous studies, including prospective randomized comparative trials, have demonstrated non-inferiority to fusion, or even short-term superiority regarding speed of improvement. The main implant-related complication is bridging heterotopic ossification with resulting loss of range of motion and increased rates of adjacent segment degeneration, although with an incidence lower than after arthrodesis. A sufficiently long follow-up, which has not yet been reached, will be necessary to establish definitively an advantage for TDR, particularly in the cervical spine. Copyright © 2014 Elsevier Masson SAS. All rights reserved.


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    The French Ministry of Foreign Affairs has informed the Organization that it is shortly to replace all diplomatic cards, special cards and employment permits ('attestations de fonctions') now held by members of the personnel and their families. Between 2 July and 31 December 2001, these cards are to be replaced by secure, computerized equivalents. The old cards may continue to be used until 31 December 2001. For the purposes of the handover, members of the personnel must go personally to the cards office (33/1-015), in order to fill in a 'fiche individuelle' form, taking the following documents for themselves and members of their families already in possession of a French card : A recent identity photograph in 4.5 cm x 3.5 cm format. The French card in their possession. An A4 photocopy of the same French card, certified by the cards office as being a true copy. Those members of the personnel whose cards (and/or cards belonging to members of their families) are shortly due to expire, or have recently done...


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    Human Resources Division;


    The French Ministry of Foreign Affairs is currently replacing all diplomatic cards, special cards and employment permits («attestations de fonctions») held by members of the personnel and their families. These cards are replaced by secure, computerized equivalents. The old cards may no longer be used after 31 December 2001. For the purposes of the handover, members of the personnel must go personally to the cards office (33/1-015) between 8h30 and 12h30, in order to fill in a «fiche individuelle» form, taking the following documents for themselves and members of their families already in possession of a French card : A recent identity photograph in 4.5 cm x 3.5 cm format, the French card in their possession, an A4 photocopy of the same French card, certified by the cards office as being a true copy. Those members of the personnel whose cards (and/or cards belonging to members of their families) are shortly due to expire, or have recently done so, are also requested...


    CERN Multimedia

    Human Resources Division


    The French Ministry of Foreign Affairs has informed the Organization that it is shortly to replace all diplomatic cards, special cards and employment permits ('attestations de fonctions') now held by members of the personnel and their families. Between 2 July and 31 December 2001, these cards are to be replaced by secure, computerized equivalents. The old cards may continue to be used until 31 December 2001. For the purposes of the handover, members of the personnel are asked to go to the cards office (33/1-015), taking the following documents for themselves and members of their families already in possession of a French card : A recent identity photograph in 4.5 cm x 3.5 cm format, The French card in their possession, an A4 photocopy of the same French card, certified by the cards office as being a true copy. Those members of the personnel whose cards (and/or cards belonging to members of their families) are shortly due to expire, or have recently done so, are also requested to take these items to the c...


    CERN Multimedia

    Human Resources Division


    The French Ministry of Foreign Affairs has informed the Organization that it is shortly to replace all diplomatic cards, special cards and employment permits ('attestations de fonctions') now held by members of the personnel and their families. Between 2 July and 31 December 2001, these cards are to be replaced by secure, computerized equivalents. A 'personnel office' stamped photocopy of the old cards may continue to be used until 31 December 2001. For the purposes of the handover, members of the personnel must go personally to the cards office (33/1-015), between 8:30 and 12:30, in order to fill a 'fiche individuelle' form (in black ink only), which has to be personally signed by themselves and another separately signed by members of their family, taking the following documents for themselves and members of their families already in possession of a French card : A recent identity photograph in 4.5 cm x 3.5 cm format (signed on the back) The French card in their possession an A4 photocopy of the same Fre...

  19. Power Plant Replacement Study

    Energy Technology Data Exchange (ETDEWEB)

    Reed, Gary


    This report represents the final report for the Eastern Illinois University power plant replacement study. It contains all related documentation from consideration of possible solutions to the final recommended option. Included are the economic justifications associated with the chosen solution along with application for environmental permitting for the selected project for construction. This final report will summarize the results of execution of an EPC (energy performance contract) investment grade audit (IGA) which lead to an energy services agreement (ESA). The project includes scope of work to design and install energy conservation measures which are guaranteed by the contractor to be self-funding over its twenty year contract duration. The cost recovery is derived from systems performance improvements leading to energy savings. The prime focus of this EPC effort is to provide a replacement solution for Eastern Illinois University's aging and failing circa 1925 central steam production plant. Twenty-three ECMs were considered viable whose net impact will provide sufficient savings to successfully support the overall project objectives.

  20. Faster Replacement Paths

    CERN Document Server

    Williams, Virginia Vassilevska


    The replacement paths problem for directed graphs is to find for given nodes s and t and every edge e on the shortest path between them, the shortest path between s and t which avoids e. For unweighted directed graphs on n vertices, the best known algorithm runtime was \\tilde{O}(n^{2.5}) by Roditty and Zwick. For graphs with integer weights in {-M,...,M}, Weimann and Yuster recently showed that one can use fast matrix multiplication and solve the problem in O(Mn^{2.584}) time, a runtime which would be O(Mn^{2.33}) if the exponent \\omega of matrix multiplication is 2. We improve both of these algorithms. Our new algorithm also relies on fast matrix multiplication and runs in O(M n^{\\omega} polylog(n)) time if \\omega>2 and O(n^{2+\\eps}) for any \\eps>0 if \\omega=2. Our result shows that, at least for small integer weights, the replacement paths problem in directed graphs may be easier than the related all pairs shortest paths problem in directed graphs, as the current best runtime for the latter is \\Omega(n^{2.5...

  1. Power Plant Replacement Study

    Energy Technology Data Exchange (ETDEWEB)

    Reed, Gary


    This report represents the final report for the Eastern Illinois University power plant replacement study. It contains all related documentation from consideration of possible solutions to the final recommended option. Included are the economic justifications associated with the chosen solution along with application for environmental permitting for the selected project for construction. This final report will summarize the results of execution of an EPC (energy performance contract) investment grade audit (IGA) which lead to an energy services agreement (ESA). The project includes scope of work to design and install energy conservation measures which are guaranteed by the contractor to be self-funding over its twenty year contract duration. The cost recovery is derived from systems performance improvements leading to energy savings. The prime focus of this EPC effort is to provide a replacement solution for Eastern Illinois University’s aging and failing circa 1925 central steam production plant. Twenty-three ECMs were considered viable whose net impact will provide sufficient savings to successfully support the overall project objectives.

  2. Diet × genotype interactions in hepatic cholesterol and lipoprotein metabolism in Atlantic salmon (Salmo salar) in response to replacement of dietary fish oil with vegetable oil. (United States)

    Morais, Sofia; Pratoomyot, Jarunan; Torstensen, Bente E; Taggart, John B; Guy, Derrick R; Bell, J Gordon; Tocher, Douglas R


    The present study investigates the effects of genotype on responses to alternative feeds in Atlantic salmon. Microarray analysis of the liver transcriptome of two family groups, lean or fat, fed a diet containing either a fish oil (FO) or a vegetable oil (VO) blend indicated that pathways of cholesterol and lipoprotein metabolism might be differentially affected by the diet depending on the genetic background of the fish, and this was further investigated by real-time quantitative PCR, plasma and lipoprotein biochemical analysis. Results indicate a reduction in VLDL and LDL levels, with no changes in HDL, when FO is replaced by VO in the lean family group, whereas in fat fish fed FO, levels of apoB-containing lipoproteins were low and comparable with those fed VO in both family groups. Significantly lower levels of plasma TAG and LDL-TAG were measured in the fat group that was independent of diet, whereas plasma cholesterol was significantly higher in fish fed the FO diet in both groups. Hepatic expression of genes involved in cholesterol homeostasis, β-oxidation and lipoprotein metabolism showed relatively subtle changes. A significantly lower expression of genes considered anti-atherogenic in mammals (ATP-binding cassette transporter A1, apoAI, scavenger receptor class B type 1, lipoprotein lipase (LPL)b (TC67836) and LPLc (TC84899)) was found in lean fish, compared with fat fish, when fed VO. Furthermore, the lean family group appeared to show a greater response to diet composition in the cholesterol biosynthesis pathway, mediated by sterol-responsive element-binding protein 2. Finally, the presence of three different transcripts for LPL, with differential patterns of nutritional regulation, was demonstrated.

  3. Principles of Human Joint Replacement Design and Clinical Application

    CERN Document Server

    Buechel, Frederick F


    Drs. Buechel, an orthopaedic surgeon, and Pappas, a professor of Mechanical Engineering, are the designers of several successful joint replacement systems. The most well-known of these is the pioneering LCS knee replacement. They have written this book for the users and designers of joint replacements. It is an attempt to convey to the reader the knowledge accumulated by the authors during their thirty five year effort on the development of replacement devices for the lower limb for the purpose of aiding the reader in their design and evaluation of joint replacement devices. The early chapters describe the engineering, scientific and medical principles needed for replacement joint evaluation. One must understand the nature and performance of the materials involved and their characteristics in vivo, i.e. the response of the body to implant materials. It is also essential to understand the response of the implants to applied loading and motion, particularly in the hostile physiological environment. A chapter de...

  4. Principles of human joint replacement design and clinical application

    CERN Document Server

    Buechel, Frederick F


    This book is written for the users and designers of joint replacements. In its second extended edition it conveys to the reader the knowledge accumulated by the authors during their forty year effort on the development of replacement devices for the lower limb for the purpose of aiding the reader in their design and evaluation of joint replacement devices. The early chapters describe the engineering, scientific and medical principles needed for replacement joint evaluation. One must understand the nature and performance of the materials involved and their characteristics in vivo, i.e. the response of the body to implant materials. It is also essential to understand the response of the implants to applied loading and motion, particularly in the hostile physiological environment. A chapter describes the design methodology now required for joint replacement in the USA and EU countries. The remaining chapters provide a history of joint replacement, an evaluation of earlier and current devices and sample case hist...

  5. Atypical Cutaneous Manifestations in Adult Onset Still’s Disease

    Directory of Open Access Journals (Sweden)

    Champa Nataraja


    Full Text Available Adult Onset Still’s Disease (AOSD, an adult variant of systemic onset juvenile idiopathic arthritis, is a rare systemic inflammatory disorder of unknown aetiology. The rarity of this disease is associated with low index of suspicion and delayed diagnosis in patients suffering from it and in the presence of atypical features the diagnosis can be further challenging. This is a case report on a 24-year-old woman, who was a diagnostic dilemma for 2 years due to the nonspecific symptoms of recurrent fever, generalized maculopapular persistent pruritic and tender rash, and polyarthralgia. She was initially diagnosed as leukocytoclastic vasculitis on a skin biopsy and was managed by a dermatologist with various medications including NSAIDs, hydroxychloroquine, dapsone, colchicine, cyclosporine, and high doses of oral steroids with minimal response. Subsequently, she has had multiple admissions with similar symptoms with raised inflammatory markers and negative septic workup. On one occasion, her iron study revealed hyperferritinaemia which led to the suspicion of AOSD. Once the rheumatic fever and infectious, malignant, autoimmune, and lymphoproliferative disorders were excluded, she was diagnosed as probable AOSD and managed successfully with IL-1 (interleukin-1 receptor antagonist, Anakinra, with remarkable and lasting response both clinically and biochemically.

  6. Atypical antipsychotics in first admission schizophrenia: medication continuation and outcomes. (United States)

    Mojtabai, Ramin; Lavelle, Janet; Gibson, P Joseph; Bromet, Evelyn J


    This study compares the effects of atypical and conventional antipsychotic medications on treatment continuation and outcomes in a first admission sample of patients with schizophrenia treated in usual practice settings. In a sample of 189 participants with a research diagnosis of DSM-IV schizophrenia drawn from the Suffolk County Mental Health Project, we compared the effects of atypical and conventional agents on change of medication, medication gaps, and rehospitalization. For these analyses we used the method of survival analysis for recurrent events, in which the episodes of treatment rather than individual subjects are the units of analysis. In addition, we compared improvement in positive and negative symptoms from intake to 24- or 48-month followups for subjects who stayed on one type of medication or changed to atypicals from conventional antipsychotics. Atypical agents were associated with lower risk of medication change, medication gaps, and rehospitalization. Both conventional and atypical agents were associated with improvement of positive symptoms at followup, but only subjects on atypical agents at followup experienced a significant improvement in negative symptoms. We conclude that in usual practice settings, as in randomized clinical trials, atypical agents are associated with improved treatment continuation and outcomes.

  7. Integrated genomic analyses of de novo pathways underlying atypical meningiomas (United States)

    Harmancı, Akdes Serin; Youngblood, Mark W.; Clark, Victoria E.; Coşkun, Süleyman; Henegariu, Octavian; Duran, Daniel; Erson-Omay, E. Zeynep; Kaulen, Leon D.; Lee, Tong Ihn; Abraham, Brian J.; Simon, Matthias; Krischek, Boris; Timmer, Marco; Goldbrunner, Roland; Omay, S. Bülent; Baranoski, Jacob; Baran, Burçin; Carrión-Grant, Geneive; Bai, Hanwen; Mishra-Gorur, Ketu; Schramm, Johannes; Moliterno, Jennifer; Vortmeyer, Alexander O.; Bilgüvar, Kaya; Yasuno, Katsuhito; Young, Richard A.; Günel, Murat


    Meningiomas are mostly benign brain tumours, with a potential for becoming atypical or malignant. On the basis of comprehensive genomic, transcriptomic and epigenomic analyses, we compared benign meningiomas to atypical ones. Here, we show that the majority of primary (de novo) atypical meningiomas display loss of NF2, which co-occurs either with genomic instability or recurrent SMARCB1 mutations. These tumours harbour increased H3K27me3 signal and a hypermethylated phenotype, mainly occupying the polycomb repressive complex 2 (PRC2) binding sites in human embryonic stem cells, thereby phenocopying a more primitive cellular state. Consistent with this observation, atypical meningiomas exhibit upregulation of EZH2, the catalytic subunit of the PRC2 complex, as well as the E2F2 and FOXM1 transcriptional networks. Importantly, these primary atypical meningiomas do not harbour TERT promoter mutations, which have been reported in atypical tumours that progressed from benign ones. Our results establish the genomic landscape of primary atypical meningiomas and potential therapeutic targets. PMID:28195122

  8. Malignant atypical cell in urine cytology: a diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Kakkar Nandita


    Full Text Available Abstract Aims The aim of this study was to find out the characteristic morphology of malignant atypical cells which were missed on routine cytology of urine. Materials and methods In this retrospective study, we examined detailed cytomorphology of 18 cases of atypical urinary cytology which were missed on routine examination and were further proved on histopathology as transitional cell carcinoma (TCC of bladder. The cytological features of these cases were compared with 10 cases of benign urine samples. Results There were 11 cases of high grade TCC and 7 cases of low grade TCC on histopathology of the atypical urine samples. Necrosis in the background and necrosed papillae were mostly seen in malignant atypical cells. The comet cells and cells with India ink nuclei (single cells with deep black structure-less nuclei were only observed in malignant atypical cells. The most consistent features in malignant atypical cells were: i high nuclear and cytoplasmic (N/C ratio ii nuclear pleomorphism iii nuclear margin irregularity iv hyperchromasia and v chromatin abnormalities Conclusion The present study emphasizes that nuclear features such as high N/C ratio, hyperchromasia and chromatin abnormalities are particularly useful for assessing the malignant atypical cells. Other cytological features such as comet cells and cells with India ink nuclei are also helpful for diagnosis but have limited value because they are less frequently seen.

  9. Toleration, Synthesis or Replacement?

    DEFF Research Database (Denmark)

    Holtermann, Jakob v. H.; Madsen, Mikael Rask


    to have considerable problems keeping a clear focus on the key question: What are the implications of this empirical turn in terms of philosophy of legal science, of the social understanding of IL, and, not least, of the place of doctrinal scholarship after the alleged Wende? What is needed, we argue......, in order to answer is not yet another partisan suggestion, but rather an attempt at making intelligible both the oppositions and the possibilities of synthesis between normative and empirical approaches to law. Based on our assessment and rational reconstruction of current arguments and positions, we...... therefore outline a taxonomy consisting of the following three basic, ideal-types in terms of the epistemological understanding of the interface of law and empirical studies: toleration, synthesis and replacement. This tripartite model proves useful with a view to teasing out and better articulating...

  10. NF- κB Essential Modulator Deficiency Leading to Disseminated Cutaneous Atypical Mycobacteria

    Directory of Open Access Journals (Sweden)

    Jonathan Braue


    Full Text Available NF- κB essential modulator (NEMO is a kinase integral to the macrophage TNF-α pathway, which leads to the intracellular destruction of Mycobacteria species. Defects in the NEMO pathway lead to a spectrum of diseases, including but not limited to ectodermal dysplasia, Mendelian susceptibility to mycobacterial diseases, and incontinentia pigmenti. In addition, paucity of NEMO can lead to the inability to mount a proper immune response against opportunistic pyogenic and mycobacterial infections, leading to dissemination to various organ systems. This manuscript will discuss the numerous clinical manifestations of NEMO deficiency, the differential diagnosis for atypical mycobacterial infections in immunocompetent adults, and feature a case report of rare isolated susceptibility to disseminated atypical mycobacteria due to a mutation in the first exon of the NEMO gene.

  11. Detection of atypical seismic events on a regional scale (United States)

    Solano-Hernandez, E. A.; Hjorleifsdottir, V.; Perez-Campos, X.; Iglesias, A.


    We propose an event-detection algorithm to locate seismic events on a regional scale. Our goal is to identify non-impulsive or 'atypical' events which are not detected by regional or global networks, due to their low P-wave amplitude. Ekstrom (2006) has developed and implemented a method to detect and locate sources of long-period seismic surface waves on a global scale. Atypical events are generated by, for example, rapid glacial movements (Ekstrom, et al., 2003; Ekstrom, et al., 2006), volcanic events (Schuler and Ekstrom, 2009) and landslides (Ekstrom and Stark, 2013). Furthermore, non-impulsive earthquakes have been located on oceanic transform faults (Abercrombie and Ekstrom, 2001). The current method (Ekstrom, 2006), that is applied on the scale of the globe, routinely detects events with magnitudes around Mw 5 and larger. In this work we wish to lower the detection threshold by using shorter period records registered by regional networks. The difficulty lies in that the shorter period records are strongly influenced by the heterogeneous crust and upper mantle, which need to be accounted for in the modeling process. Our proposed method involves first computing full waveforms, Green's functions or moment tensor responses, between a grid of test locations and existing seismic stations in a 3D medium. We then effectively back propagate observed data through cross correlation with the responses, obtaining a function that localizes in time and space at the source. Our method is a variant of the timereversal method presented by, for example, McMechan (1982), Tromp et al. (2005), Larmat et al. (2006), Gajewski and Tessmer (2005) and Kim et al. (2010). To calibrate the various parameters used by the detection method, we use the aftershocks sequence of the March 20, 2012 Ometepec, Guerrero, Mexico earthquake, recorded by the SSN (Mexican National Network). The lively aftershock sequence provided us with many events of different magnitudes, all occurring approximately

  12. Results of surgical treatment of atypical endometrial hyperplasia

    Directory of Open Access Journals (Sweden)

    O. A. Gornykh


    Full Text Available The results of surgical treatment in 132 patients with atypical endometrial hyperplasia have been studied. Post-operative diagnosis was: en- dometrial cancer – in 19 %, atypical hyperplasia – in 35 %, simple and complex hyperplasia – in 33 %, only atrophic endometrial changes – in 13 % of patients. The tumor was within the endometrium in 5 patients, the superficial invasion of the myometrium (1–2 mm were in 8 patients, invasion to half of the myometrium – in 9 patients, invasion of more than half of the myometrium – in 3 patients. The questions of tactics of treatment of atypical endometrial hyperplasia is under discussion.

  13. Atypical real estate objects: legal regime and control system

    Directory of Open Access Journals (Sweden)

    Voskresenskaya Elena


    Full Text Available The legal concept of immovable things raises controversy in legal practice. Determining and understanding the definition of real estate, the complexity and diversity of these objects, a growing appearance of so-called atypical properties (such as sport stadiums, roads, boreholes, analyzing legislation and judicial practice of this field – all these issues call for a deep study of this topic. There is a conflicting arbitration practice, the subject of which is the learning of the legal nature of atypical real estate (for instance, asphalt playgrounds, car parks, fences, wells. The object of the research is the learning of the legal status of atypical real estate.

  14. Intracranial Tuberculoma Presenting as Atypical Eclampsia: A Case Report (United States)

    Murugesan, Sharmila; Pradeep, Sunitha; John, Lopamudra; Kolluru, Vasavi


    Occurrence of eclampsia before 20 weeks of pregnancy and after 48 hours of delivery in the absence of typical signs of hypertension and or proteinuria is termed as atypical eclampsia. Atypical or non-classic eclampsia will have some symptoms of eclampsia but without the usual proteinuria or hypertension. All patients with atypical onset should undergo neurological evaluation to rule out neurologic causes of seizures. Cerebral tuberculosis is a rare and serious form of disease secondary to haematogenous spread of Mycobacterium tuberculosis. Here we present a case of cerebral tuberculoma with seizures in late pregnancy mimicking eclampsia. PMID:27504359

  15. Testosterone replacement therapy and prostate health. (United States)

    Polackwich, A Scott; Ostrowski, Kevin A; Hedges, Jason C


    There is an emerging evolution in the understanding of the relationship between the prostate and testosterone. It has long been generally believed that with testosterone replacement therapy (TRT), increasing serum testosterone levels led to prostatic growth and worsening of voiding dysfunction and associated complications. A new theory, the Saturation Model of Testosterone and its effect on the Prostate has gained attention. This theory suggests that the prostate's response to increasing levels of serum testosterone reaches a limit beyond which there is minimal effect. This model predicts that testosterone replacement therapy occurs above this saturation point, and replacing testosterone to eugonadal levels should not worsen prostate related benign disease. We evaluated the recent published data, with an emphasis on clinical studies done within the last 3 years, for the effects of testosterone supplementation on benign prostatic disease.

  16. Respuesta productiva de conejos alimentados con forraje verde hidropónico de avena, como reemplazo parcial de concentrado comercial Productive response of rabbits fed with green hydroponic oats forage as partial replacement of commercial concentrate

    Directory of Open Access Journals (Sweden)

    Francisco Fabián Fuentes Carmona


    Full Text Available La respuesta productiva de conejos raza californiana alimentados con forraje verde hidropónico (FVH de avena como reemplazo parcial de concentrado comercial (CC, fue evaluada en condiciones de desierto en el norte de Chile. Se establecieron cinco tratamientos de alimentación, equivalentes a 0, 25, 50, 75 y 100% de reemplazo de la dieta diaria con FVH, el cual fue cosechado y utilizado directamente diez días después de la siembra. Cuarenta y cinco conejos destetados a los 31 días fueron evaluados en un diseño completamente al azar hasta alcanzar un peso de sacrificio de 2 kg de peso vivo. La calidad del FVH de avena fue considerada como buena, presentando similar valor nutritivo que el CC. Las variables evaluadas y sus respectivos rangos fueron: consumo de materia seca promedio (59.17 - 104.73 g/día, ganancia de peso vivo promedio (16.35 - 29.10 g/animal por día, conversión alimenticia promedio (3.31 - 3.93 kg MS/kg PV, tiempo de peso vivo a sacrificio (53 - 91 días, peso vivo final (1430 - 2044 g/animal, peso de la canal (1235 - 1385 g/animal y rendimiento de la canal (59.19 - 62.25%. El reemplazo de hasta 50% de la dieta base con FVH de avena no afectó significativamente (P The productive response of Californian rabbits fed with hydroponic green oats forage (HGOF as a partial replacement of commercial concentrate (CC was evaluated under desert conditions in northern Chile. Five treatments were established as follow: 0, 25, 50, 75 and 100% replacement of daily diet with HGOF, which was harvested and used directly at 10 days after sowing. Forty-five rabbits weaned at 31 days were evaluated in a completely randomized design until slaughter weight of 2.0 kg. HGOF quality was considered as good, presenting similar nutritional value to CC. The variables assessed and range values were: average dry matter intake (59.17-104.73 g/d, average weight gain (16.35-29.10 g/ rabbit for day, feed conversion average (3.31-3.93 kg DM/ kg LW, time of

  17. Relapsing hypocupraemic myelopathy requiring high‐dose oral copper replacement


    Prodan, C.I.; Bottomley, S S; Holland, N R; Lind, S. E.


    Adult‐onset copper deficiency with neurological manifestations is a newly recognised syndrome. Long‐term oral copper replacement therapy has been the mainstay of treatment in the literature. A case of relapsing hypocupraemic myelopathy responsive to increased doses of copper replacement is reported. Standard doses of copper may not be sufficient for all patients.

  18. Atypical presentation of leprosy: A report of two cases

    Directory of Open Access Journals (Sweden)

    Das Sudip


    Full Text Available We report two atypical presentation of leprosy-granuloma annulare like in a case of borderline tuberculoid leprosy, another erythema multiforme like lesion in borderline lepromatous leprosy.

  19. Delayed Recurrence of Atypical Pulmonary Carcinoid Cluster: A Rare Occurrence

    Directory of Open Access Journals (Sweden)

    Salim Surani


    Full Text Available Carcinoid is one of the most common tumors of the gastrointestinal tract followed by the tracheobronchial tree. Bronchial carcinoid compromises 20% of total carcinoid and accounts for 1–5% of pulmonary malignancies. Carcinoid can be typical or atypical, with atypical carcinoid compromises 10% of the carcinoid tumors. Carcinoid usually presents as peripheral lung lesion or solitary endobronchial abnormality. Rarely it can present as multiple endobronchial lesion. We hereby present a rare case of an elderly gentleman who had undergone resection of right middle and lower lobe of lung for atypical carcinoid. Seven years later he presented with cough. CT scan of chest revealed right hilar mass. Flexible bronchoscopy revealed numerous endobronchial polypoid lesions in the tracheobronchial tree. Recurrent atypical carcinoid was then confirmed on biopsy.

  20. Teaching strategies for atypical presentation of illness in older adults. (United States)

    Gray-Miceli, Deanna; Aselage, Melissa; Mezey, Mathy


    Atypical presentation of illness is a phenomenon where "seeing is believing." Expert geriatric nurses and clinicians know all too well the early signs and symptoms of this phenomenon, which frequently masquerades bacterial infections, pain, acute myocardial infarction, heart failure, or other serious medical ailments in older adults. Students, however, as novices to clinical practice, require interactive learning approaches to reflect on the patient's illness presentations, help with developing the necessary skills to analyze and synthesize clinically relevant data, and witness resolution of an atypical presentation when found and treated. Use of a case study as an educational tool can facilitate critical thinking about a clinical problem, such as atypical presentation of illness, for students within a problem-based learning format. Furthermore, we highlight strategies for teaching students atypical presentation of illness with consideration of student learning preferences, which include visual, auditory, reading, and kinesthetic modes of learning.

  1. Iron replacement therapy

    DEFF Research Database (Denmark)

    Nielsen, Ole Haagen; Coskun, Mehmet; Weiss, Günter


    PURPOSE OF REVIEW: Approximately, one-third of the world's population suffers from anemia, and at least half of these cases are because of iron deficiency. With the introduction of new intravenous iron preparations over the last decade, uncertainty has arisen when these compounds should be admini...... treatment, when to follow-up for relapse, which dosage and type of therapy should be recommended or not recommended, and if some patients should not be treated....... be administered and under which circumstances oral therapy is still an appropriate and effective treatment. RECENT FINDINGS: Numerous guidelines are available, but none go into detail about therapeutic start and end points or how iron-deficiency anemia should be best treated depending on the underlying cause...... of iron deficiency or in regard to concomitant underlying or additional diseases. SUMMARY: The study points to major issues to be considered in revisions of future guidelines for the true optimal iron replacement therapy, including how to assess the need for treatment, when to start and when to stop...


    Directory of Open Access Journals (Sweden)

    Jayant Mahadeorao


    Full Text Available Dengue fever and dengue haemorrhagic fever are important arboviral diseases. Dengue virus belongs to family Flaviviridae , has four serotypes that spread by the bite of infected Aedes mosquitoes . Dengue epidemics can have a significant economic and health t oll. Worldwide, an estimated 3.6 billion people are at risk of infection with about 50 - 100 million new cases each year Illness produced by any of the four dengue virus serotypes varies from mild asymptomatic illness to severe fatal dengue haemorrhagic fe ver/dengue shock syndrome (DHF/DSS. During the early febrile stage clinicians cannot predict which patients will progress to severe disease. Atypical manifestations were reported are associated with high risk of mortality. The existing WHO dengue classific ation scheme and case definitions have some drawbacks. A global strategy to reduce the disease burden using integrated vector management in conjunction with early and accurate diagnosis has been advocated. Antiviral drugs and vaccines that are currently un der development could also make an important contribution to dengue control in the future

  3. Atypical sinonasal Schwannomas: a difficult diagnostic challenge. (United States)

    Jacopo, Galli; Micaela, Imperiali; Italo, Cantore; Luigi, Corina; Larocca, Luigi M; Gaetano, Paludetti


    Schwannomas are benign tumours arising from Schwann cells of the peripheral nerve sheath. They are relatively frequent in the head and neck region (25-45%) but rarely involve in the sinonasal tract (4%). The authors outline the diagnostic difficulties and the problems in choosing the best surgical approach in two atypical cases of sinonasal Schwannomas. In the first case reported clinical data, sex and age of the patient, nasal endoscopy and angio-MRI led us to suspect an angiofibroma; therefore, we approached the case without a biopsy performing a preoperative selective embolization followed by an endoscopic resection. In the second case, due to initial visual symptoms and to the ethmoid-orbital compartment involvement, we performed a sinonasal endoscopy and collected a biopsy which resulted to be fundamental in the diagnostic assessment. Tumour excision was then obtained throughout an intracranial/endonasal approach. The two presented cases revealed the presence of cystic Schwannomas. In the first case, diagnosis was made only post-operatively after histological examination. Patients underwent complete surgical excision by means of an endoscopic sinonasal approach, in the second case associated to a left frontal craniotomy. The patients showed no signs of recurrence at a 9 months follow-up. Nasal endoscopy was extremely important in making the diagnosis, allowing an accurate assessment of the tumour extension and a biopsy. The diagnosis of sinonasal Schwannomas remains challenging; sometimes, clinical behaviour and modern imaging may be misleading. The diagnostic and therapeutic importance of sinonasal endoscopy is emphasised in the two presented cases.

  4. Nocturnal manifestations of atypical parkinsonian disorders. (United States)

    Bhidayasiri, Roongroj; Jitkritsadakul, Onanong; Colosimo, Carlo


    Although nocturnal disturbances are increasingly recognized as an integral part of the continuum of daytime manifestations of Parkinson's disease (PD), there is still little evidence in the medical literature to support the occurrence of these complex phenomena in patients with atypical parkinsonian disorders (APDs). Based on the anatomical substrates in APDs, which are considered to be more extensive outside the basal ganglia than in PD, we might expect that patients with APDs encounter the whole range of nocturnal disturbances, including motor, sleep disorders, autonomic dysfunctions, and neuropsychiatric manifestations at a similar, or even greater, frequency than in PD. This article is a review of the current literature on the problems at nighttime of patients with progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. MEDLINE, life science journals and online books were searched by querying appropriate key words. Reports were included if the studies were related to nocturnal manifestations in APDs. Forty articles fulfilled the selection criteria. Differences between these symptoms in APDs and PD are highlighted, given the evidence available about each manifestation. This analysis of nocturnal manifestations of APDs suggests the need for future studies to address these issues to improve the quality of life not only of patients with APDs but the caregivers who encounter the challenges of supporting these patients on a daily basis.

  5. Atypical focal nodular hyperplasia of the liver

    Institute of Scientific and Technical Information of China (English)

    Muhammad Rizwan Khan; Taimur Saleem; Tanveer Ul Haq; Kanwal Aftab


    BACKGROUND: Focal nodular hyperplasia, a benign hepatic tumor, is usually asymptomatic. However, rarely the entity can cause symptoms, mandating intervention. METHOD: We present a case of focal nodular hyperplasia of the liver, which caused a considerable diagnostic dilemma due to its atypical presentation. RESULTS: A 29-year-old woman presented with a 15-year history of a progressively increasing mass in the right upper quadrant which was associated with pain and emesis. Examination showed a firm, mobile mass palpable below the right subcostal margin. A computed tomography scan of the abdomen showed an exophytic mass arising from hepatic segments III and IVb. Trucut biopsy of the hepatic mass was equivocal. Angiography showed a vascular tumor that was supplied by a tortuous branch of the proper hepatic artery. Surgical intervention for removal of the mass was undertaken. Intra-operatively, two large discrete tumors were found and completely resected. Histopathological examination showed features consistent with focal nodular hyperplasia. CONCLUSION: This description of an unusual case of focal nodular hyperplasia of the liver highlights the point that the diagnosis of otherwise benign hepatic tumors may be difficult despite extensive work-up in some cases.

  6. Atypical presentation of mucopolysaccharidosis type IVA. (United States)

    Rush, Eric T


    A 14 year old patient with short stature, type I diabetes, and cataracts was referred for evaluation of avascular necrosis of the femoral head. Radiography was suggestive of spondyloepiphyseal dysplasia with decreased bone mineral density for age. Targeted molecular and biochemical testing were normal in this patient. Whole exome sequencing was performed and showed compound heterozygosity for previously reported pathogenic GALNS variants which were diagnostic of mucopolysaccharidosis, type IVA (Morquio A). While this case describes neither a novel condition nor a new mutation, it does illustrate three important points in the diagnosis of patients with atypical forms of MPS IVA. First, that in many instances urine glycosaminoglycan analysis is not sufficient to rule out MPS IVA as a potential diagnosis. Patients in whom biochemical screening is advised should have measurement of leukocyte enzymatic activity. Second, that in patients with radiographic evidence of spondyloepiphyseal dysplasia with additional features or with normal targeted testing, MPS IVA should remain in the differential diagnosis. Third, that whole exome sequencing represents a viable diagnostic platform for evaluation of patients with unknown skeletal or metabolic disease.

  7. Atypical Presentation of a Pediatric Cerebellar Ganglioglioma. (United States)

    Bram, Richard; Seidman, Roberta J; Chesler, David


    Gangliogliomas (GGs) are rare central nervous system tumors occurring primarily in the supratentorial compartment with infratentorial instances most often involving the brain stem. Infratentorial GGs typically present with signs and symptoms of increased intracranial pressure (ICP), cranial nerve deficits, or focal cerebellar findings; rarely, these tumors have been associated with focal seizures. In this report, we describe an atypical presentation of a cerebellar GG in a 20-month-old male who initially presented with syncope and emesis in the absence of electrographic evidence of seizures, radiographic evidence of hydrocephalus, or elevated ICP. The epidemiology, radiographic, and pathological findings as well as the treatment of these tumors are also discussed. After gross total resection, the patient experienced full resolution of all his preoperative symptoms without the development of new neurological deficits. Unlike their supratentorial counterparts, infratentorial GGs do not commonly present with seizures although rare reports exist in the literature of seizures attributed to cerebellar GG. Moreover, cerebellar GGs may produce nonspecific symptoms in the absence of concrete diagnostic findings. Such a presentation should prompt further neurological evaluation. Most cases of isolated cerebellar GG can be successfully treated with surgical resection and carry a favorable prognosis. © 2017 S. Karger AG, Basel.

  8. Atypical presentation of macrophagic myofasciitis 10 years post vaccination.

    LENUS (Irish Health Repository)

    Ryan, Aisling M


    Macrophagic myofasciitis (MMF) is an uncommon inflammatory disorder of muscle believed to be due to persistence of vaccine-derived aluminium hydroxide at the site of injection. The condition is characterised by diffuse myalgias, arthralgia and fatigue. We describe a patient with histologically confirmed MMF whose presentation was atypical with left chest and upper limb pain beginning more than 10 years post vaccination. Treatment with steroids led to symptomatic improvement. Although rare, clinicians should consider MMF in cases of atypical myalgia.

  9. An Atypical Case of Pityriasis Rosea Gigantea after Influenza Vaccination

    Directory of Open Access Journals (Sweden)

    Dimitrios Papakostas


    Full Text Available Pityriasis rosea is a common erythematosquamous eruption, typically presenting along the cleavage lines of the skin. A wide spectrum of atypical manifestations may challenge even the most experienced physician. Here we report a rare case of a suberythrodermic pityriasis rosea with gigantic plaques after an influenza vaccination, and we discuss the possible triggers of atypical manifestations of such a common dermatological disease in the setting of an altered immunity.

  10. Atypical presentations among Medicare beneficiaries with unstable angina pectoris. (United States)

    Canto, John G; Fincher, Contessa; Kiefe, Catarina I; Allison, Jeroan J; Li, Qing; Funkhouser, Ellen; Centor, Robert M; Selker, Harry P; Weissman, Norman W


    Chest pain is a hallmark symptom in patients with unstable angina pectoris (UAP). However, little is known regarding the prevalence of an atypical presentation among these patients and its relation to subsequent care. We examined the medical records of 4,167 randomly sampled Medicare patients hospitalized with unstable angina at 22 Alabama hospitals between 1993 and 1999. We defined typical presentation as (1) chest pain located substernally in the left or right chest, or (2) chest pain characterized as squeezing, tightness, aching, crushing, arm discomfort, dullness, fullness, heaviness, pressure, or pain aggravated by exercise or relieved with rest or nitroglycerin. Atypical presentation was defined as confirmed UAP without typical presentation. Among patients with confirmed UAP, 51.7% had atypical presentations. The most frequent symptoms associated with atypical presentation were dyspnea (69.4%), nausea (37.7%), diaphoresis (25.2%), syncope (10.6%), or pain in the arms (11.5%), epigastrium (8.1%), shoulder (7.4%), or neck (5.9%). Independent predictors of atypical presentation for patients with UAP were older age (odds ratio 1.09, 95% confidence interval 1.01 to 1.17/decade), history of dementia (odds ratio 1.49, 95% confidence interval 1.10 to 2.03), and absence of prior myocardial infarction, hypercholesterolemia, or family history of heart disease. Patients with atypical presentation received aspirin, heparin, and beta-blocker therapy less aggressively, but there was no difference in mortality. Thus, over half of Medicare patients with confirmed UAP had "atypical" presentations. National educational initiatives may need to redefine the classic presentation of UAP to include atypical presentations to ensure appropriate quality of care.

  11. Importance of the swallowing atypical in them malocclusions


    Jiménez Jiménez, Jonatan; Universidad Nacional Mayor de San Marcos, Facultad de Odontología.


    This review aims to determine the atypical swallowing malocclusions. When there are inadequate move-ments of the tongue and / or other structures during oral and pharyngolaryngeal phase of swallowing, talking about atypical swallowing, as a non-physiological habit. Its objectives may be multiple, simul-taneously acting alone or cumulatively. The size of a large tongue has been found as an impediment to correct such alternations, though this is a cause of causing malocclusion. the importance o...

  12. An Atypical Case of Pityriasis Rosea Gigantea after Influenza Vaccination


    Dimitrios Papakostas; Stavropoulos, Panagiotis G; Dafni Papafragkaki; Ekaterini Grigoraki; Georgia Avgerinou; Christina Antoniou


    Pityriasis rosea is a common erythematosquamous eruption, typically presenting along the cleavage lines of the skin. A wide spectrum of atypical manifestations may challenge even the most experienced physician. Here we report a rare case of a suberythrodermic pityriasis rosea with gigantic plaques after an influenza vaccination, and we discuss the possible triggers of atypical manifestations of such a common dermatological disease in the setting of an altered immunity.

  13. Atypical presentation of macrophagic myofasciitis 10 years post vaccination. (United States)

    Ryan, Aisling M; Bermingham, Niamh; Harrington, Hugh J; Keohane, Catherine


    Macrophagic myofasciitis (MMF) is an uncommon inflammatory disorder of muscle believed to be due to persistence of vaccine-derived aluminium hydroxide at the site of injection. The condition is characterised by diffuse myalgias, arthralgia and fatigue. We describe a patient with histologically confirmed MMF whose presentation was atypical with left chest and upper limb pain beginning more than 10 years post vaccination. Treatment with steroids led to symptomatic improvement. Although rare, clinicians should consider MMF in cases of atypical myalgia.

  14. Atypical meningococcal meningitis with rashless presentation:A case report

    Institute of Scientific and Technical Information of China (English)

    Sunita; Singh Manpreet; Kapoor Dheeraj


    Meningococcal disease is the major health problem in developing world. The clinical presentation is varied, ranging from transient fever and bacteraemia to fulminant disease with death ensuing within hours of the onset of clinical symptoms. The classical clinical manifestations of meningococcal disease have been well described, but atypical presentations if unrecognized, may lead to a delay in treatment and fatal outcome. We here report a case presented with atypical presentation of meningococcal meningitis without classical rash, which was diagnosed and managed successfully.

  15. Atypical Time Course of Object Recognition in Autism Spectrum Disorder (United States)

    Caplette, Laurent; Wicker, Bruno; Gosselin, Frédéric


    In neurotypical observers, it is widely believed that the visual system samples the world in a coarse-to-fine fashion. Past studies on Autism Spectrum Disorder (ASD) have identified atypical responses to fine visual information but did not investigate the time course of the sampling of information at different levels of granularity (i.e. Spatial Frequencies, SF). Here, we examined this question during an object recognition task in ASD and neurotypical observers using a novel experimental paradigm. Our results confirm and characterize with unprecedented precision a coarse-to-fine sampling of SF information in neurotypical observers. In ASD observers, we discovered a different pattern of SF sampling across time: in the first 80 ms, high SFs lead ASD observers to a higher accuracy than neurotypical observers, and these SFs are sampled differently across time in the two subject groups. Our results might be related to the absence of a mandatory precedence of global information, and to top-down processing abnormalities in ASD. PMID:27752088

  16. Assesment, treatment and prevention of atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Azar Nickavar


    Full Text Available Hemolytic uremic syndrome (HUS is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1 D+ HUS: Presentation with a preceding diarrhea; (2 typical HUS: D+ HUS with a single and self-limited episode; (3 atypical HUS (aHUS: Indicated those with complement dysregulation; (4 recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA after improvement of hematologic abnormalities; and (5 familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

  17. Rest mutant zebrafish swim erratically and display atypical spatial preferences. (United States)

    Moravec, Cara E; Li, Edward; Maaswinkel, Hans; Kritzer, Mary F; Weng, Wei; Sirotkin, Howard I


    The Rest/Nrsf transcriptional repressor modulates expression of a large set of neural specific genes. Many of these target genes have well characterized roles in nervous system processes including development, plasticity and synaptogenesis. However, the impact of Rest-mediated transcriptional regulation on behavior has been understudied due in part to the embryonic lethality of the mouse knockout. To investigate the requirement for Rest in behavior, we employed the zebrafish rest mutant to explore a range of behaviors in adults and larva. Adult rest mutants of both sexes showed abnormal behaviors in a novel environment including increased vertical swimming, erratic swimming patterns and a proclivity for the tank walls. Adult males also had diminished reproductive success. At 6 days post fertilization (dpf), rest mutant larva were hypoactive, but displayed normal evoked responses to light and sound stimuli. Overall, these results provide evidence that rest dysfunction produces atypical swimming patterns and preferences in adults, and reduced locomotor activity in larvae. This study provides the first behavioral analysis of rest mutants and reveals specific behaviors that are modulated by Rest.

  18. Atypical neural synchronization to speech envelope modulations in dyslexia. (United States)

    De Vos, Astrid; Vanvooren, Sophie; Vanderauwera, Jolijn; Ghesquière, Pol; Wouters, Jan


    A fundamental deficit in the synchronization of neural oscillations to temporal information in speech could underlie phonological processing problems in dyslexia. In this study, the hypothesis of a neural synchronization impairment is investigated more specifically as a function of different neural oscillatory bands and temporal information rates in speech. Auditory steady-state responses to 4, 10, 20 and 40Hz modulations were recorded in normal reading and dyslexic adolescents to measure neural synchronization of theta, alpha, beta and low-gamma oscillations to syllabic and phonemic rate information. In comparison to normal readers, dyslexic readers showed reduced non-synchronized theta activity, reduced synchronized alpha activity and enhanced synchronized beta activity. Positive correlations between alpha synchronization and phonological skills were found in normal readers, but were absent in dyslexic readers. In contrast, dyslexic readers exhibited positive correlations between beta synchronization and phonological skills. Together, these results suggest that auditory neural synchronization of alpha and beta oscillations is atypical in dyslexia, indicating deviant neural processing of both syllabic and phonemic rate information. Impaired synchronization of alpha oscillations in particular demonstrated to be the most prominent neural anomaly possibly hampering speech and phonological processing in dyslexic readers.

  19. RNA-Binding Proteins in Trichomonas vaginalis: Atypical Multifunctional Proteins

    Directory of Open Access Journals (Sweden)

    Elisa E. Figueroa-Angulo


    Full Text Available Iron homeostasis is highly regulated in vertebrates through a regulatory system mediated by RNA-protein interactions between the iron regulatory proteins (IRPs that interact with an iron responsive element (IRE located in certain mRNAs, dubbed the IRE-IRP regulatory system. Trichomonas vaginalis, the causal agent of trichomoniasis, presents high iron dependency to regulate its growth, metabolism, and virulence properties. Although T. vaginalis lacks IRPs or proteins with aconitase activity, possesses gene expression mechanisms of iron regulation at the transcriptional and posttranscriptional levels. However, only one gene with iron regulation at the transcriptional level has been described. Recently, our research group described an iron posttranscriptional regulatory mechanism in the T. vaginalis tvcp4 and tvcp12 cysteine proteinase mRNAs. The tvcp4 and tvcp12 mRNAs have a stem-loop structure in the 5'-coding region or in the 3'-UTR, respectively that interacts with T. vaginalis multifunctional proteins HSP70, α-Actinin, and Actin under iron starvation condition, causing translation inhibition or mRNA stabilization similar to the previously characterized IRE-IRP system in eukaryotes. Herein, we summarize recent progress and shed some light on atypical RNA-binding proteins that may participate in the iron posttranscriptional regulation in T. vaginalis.

  20. RNA-Binding Proteins in Trichomonas vaginalis: Atypical Multifunctional Proteins. (United States)

    Figueroa-Angulo, Elisa E; Calla-Choque, Jaeson S; Mancilla-Olea, Maria Inocente; Arroyo, Rossana


    Iron homeostasis is highly regulated in vertebrates through a regulatory system mediated by RNA-protein interactions between the iron regulatory proteins (IRPs) that interact with an iron responsive element (IRE) located in certain mRNAs, dubbed the IRE-IRP regulatory system. Trichomonas vaginalis, the causal agent of trichomoniasis, presents high iron dependency to regulate its growth, metabolism, and virulence properties. Although T. vaginalis lacks IRPs or proteins with aconitase activity, possesses gene expression mechanisms of iron regulation at the transcriptional and posttranscriptional levels. However, only one gene with iron regulation at the transcriptional level has been described. Recently, our research group described an iron posttranscriptional regulatory mechanism in the T. vaginalis tvcp4 and tvcp12 cysteine proteinase mRNAs. The tvcp4 and tvcp12 mRNAs have a stem-loop structure in the 5'-coding region or in the 3'-UTR, respectively that interacts with T. vaginalis multifunctional proteins HSP70, α-Actinin, and Actin under iron starvation condition, causing translation inhibition or mRNA stabilization similar to the previously characterized IRE-IRP system in eukaryotes. Herein, we summarize recent progress and shed some light on atypical RNA-binding proteins that may participate in the iron posttranscriptional regulation in T. vaginalis.

  1. EPS profiles: the atypical antipsychotics are not all the same. (United States)

    Weiden, Peter J


    Within the first few years after chlorpromazine began to be used to treat psychosis, it was observed that it could cause many kinds of neurologic reactions that resembled those seen in idiopathic Parkinson's disease. These reactions were termed "extrapyramidal side effects" (EPS) because of their resemblance to the signs of Parkinson's disease, which were associated with degeneration of the dopamine nerve tracks located in the extrapyramidal region of the central nervous system. Eventually this association of dopamine loss, antipsychotics, and parkinsonism became a central part of the dopamine hypothesis of schizophrenia. Unfortunately, this association was also used to support the hypothesis that EPS were absolutely necessary for antipsychotic efficacy--hence the term "neuroleptic" rather than "antipsychotic." This theory, now discredited, was used to justify the practice of inducing EPS as a means to gauge whether an antipsychotic would be effective. The demonstration that clozapine, an antipsychotic virtually devoid of EPS, has better efficacy for psychosis than any other "neuroleptic" disproved the theory that EPS were fundamentally linked to efficacy. Because the idea of a relationship between EPS and efficacy was so ingrained in clinical practice, clozapine was called "atypical." Our understanding of the relationship between EPS and antipsychotic response has come full circle. With the introduction of clozapine and other newer antipsychotics, it has become clear that EPS are harmful and serve no beneficial purpose. The availability of newer antipsychotics with a lower EPS burden means that, at least in theory, it is now possible to treat psychosis without EPS in the vast majority of patients. In practice, however, EPS remain a significant problem even in the era of atypical or second generation antipsychotics (SGAs). One limitation is that the concept of "atypicality," when used to denote antipsychotic efficacy without EPS, is a relative not an absolute

  2. Mitochondrial DNA sequence analysis of patients with 'atypical psychosis'. (United States)

    Kazuno, An-A; Munakata, Kae; Mori, Kanako; Tanaka, Masashi; Nanko, Shinichiro; Kunugi, Hiroshi; Umekage, Tadashi; Tochigi, Mamoru; Kohda, Kazuhisa; Sasaki, Tsukasa; Akiyama, Tsuyoshi; Washizuka, Shinsuke; Kato, Nobumasa; Kato, Tadafumi


    Although classical psychopathological studies have shown the presence of an independent diagnostic category, 'atypical psychosis', most psychotic patients are currently classified into two major diagnostic categories, schizophrenia and bipolar disorder, by the Diagnostic and Statistical Manual of Mental Disorders (4th edn; DSM-IV) criteria. 'Atypical psychosis' is characterized by acute confusion without systematic delusion, emotional instability, and psychomotor excitement or stupor. Such clinical features resemble those seen in organic mental syndrome, and differential diagnosis is often difficult. Because patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) sometimes show organic mental disorder, 'atypical psychosis' may be caused by mutations of mitochondrial DNA (mtDNA) in some patients. In the present study whole mtDNA was sequenced for seven patients with various psychotic disorders, who could be categorized as 'atypical psychosis'. None of them had known mtDNA mutations pathogenic for mitochondrial encephalopathy. Two of seven patients belonged to a subhaplogroup F1b1a with low frequency. These results did not support the hypothesis that clinical presentation of some patients with 'atypical psychosis' is a reflection of subclinical mitochondrial encephalopathy. However, the subhaplogroup F1b1a may be a good target for association study of 'atypical psychosis'.

  3. Atypical sonographic patterns of fibroadenoma of the breast : pathologic correlation

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    Kook, Shin Ho; Kim, Myung Sook; Pae, Won Kil [Kangbuk Samsung Hospital, Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)


    To correlate the atypical sonographic patterns of fibroadenoma of the breast with the pathologic findings. Among 203 surgically proven 43 which were sonographically atypical fibroadenomas, were retrospectively reviewed. The diagnostic criteria for atypical variety, as seen on sonography, were an ill-defined margin, microlobulated or irregular shape, heterogeneous internal echo-pattern, posterior shadowing, microcalcification, and clefts. The atypical sonographic patterns of these 43 fibroadenomas were analysed and compared with the pathologic findings. Among 43 lesions, ill-defined margins or irregular shapes were seen in 15 cases, heterogeneous internal echo-patterns in 27, posterior attenuation in nine, and clefts in seven. Thirty-seven (86%) of the 43 were predominantly ductal or had a mixed ductal and stromal component. Eleven (73.3%) of fifteen ill-defined margin or irregular shaped lesions were caused by interdigitation of surrounding normal breast parenchyma and mass. Twenty two (81.5%) of 27 heterogeneous internal echo-pat-terns were related to dilated ducts, phyllodes features, collagen bundles, adenosis, microcalcification, or fat vacuoles. Eight (88.9%) of nine posterior attenuations were caused by collagen bundles, microcalcification, ductal proliferation or dilatation. All seven cases showing clefts revealed phyllodes features and dilated ducts. Most atypical fibroadenomas had a predominantly ductal or mixed component. Ill-defined margin or irregular shape was mainly due to interdigitation of normal surrounding parenchyma. Variable histologic features were related to the heterogeneous internal echo-pattern, posterior shadowing, and the clefts revealed by atypical sonographic findings.

  4. Neuroleptic malignant syndrome associated with atypical antipsychotic drugs. (United States)

    Trollor, Julian N; Chen, Xiaohua; Sachdev, Perminder S


    Neuroleptic malignant syndrome (NMS) is a rare but potentially severe idiosyncratic adverse reaction usually seen in the context of treatment with antipsychotic drugs. Although NMS is historically associated with the classic or 'typical' antipsychotic drugs, it is also a potential adverse effect of atypical antipsychotic drugs. The widespread use of atypical antipsychotic drugs highlights the need to examine the data relating to the symptomatology, diagnosis, classification and management of NMS with these newer agents. We used MEDLINE and EMBASE to identify NMS case reports and systematic reviews published to June 2008 related to the atypical antipsychotic drugs clozapine, olanzapine, risperidone, paliperidone, aripiprazole, ziprasidone, amisulpride and quetiapine. Case reports and reviews were systematically examined. Our review suggests that, in general, NMS associated with atypical antipsychotic drugs manifests in a typical manner. One notable exception is clozapine-induced NMS, which appears less likely to manifest with extrapyramidal features, including rigidity and tremor. The available literature highlights the divergence of opinion relating to the core diagnostic features of NMS and its conceptualization as a categorical versus dimensional disorder. Both these issues have relevance for the identification of atypical or milder forms of NMS, which are sometimes seen with atypical antipsychotic drugs.

  5. Atypical patterns of respiratory sinus arrhythmia index an endophenotype for depression (United States)

    Yaroslavsky, Ilya; Rottenberg, Jonathan; Kovacs, Maria


    Can atypical patterns of parasympathetic nervous system activity serve as endophenotypes for depression? Using respiratory sinus arrhythmia (RSA) as an index of parasympathetic nervous system function, we examined this question in two studies: one involving mothers with and without depression histories and their offspring (at high and low risk for depression, respectively), and a further study of adolescent sibling pairs concordant and discordant for major depression. In both studies, subjects were exposed to sad mood induction; subjects’ RSA was monitored during rest periods and in response to the mood induction. We used Gottesman and Gould’s (2003) criteria for an endophenotype and a priori defined “atypical” and “normative” RSA patterns (combinations of resting RSA and RSA reactivity). We found that atypical RSA patterns (a) predicted current depressive episodes and remission status among women with histories of juvenile onset depression and healthy controls, (b) predicted longitudinal trajectories of depressive symptoms among high- and low-risk young offspring, (c) were concordant across mothers and their juvenile offspring, (d) were more prevalent among never-depressed youth at high risk for depression than their low-risk peers, and (e) were more concordant across adolescent sibling pairs in which both versus only one had a history of major depression. Thus, the results support atypical RSA patterns as an endophenotype for depression. Possible mechanisms by which RSA patterns increase depression risk and their genetic contributors are discussed. PMID:25422965

  6. Sensory atypicalities in dyads of children with autism spectrum disorder (ASD) and their parents. (United States)

    Glod, Magdalena; Riby, Deborah M; Honey, Emma; Rodgers, Jacqui


    Sensory atypicalities are a common feature of autism spectrum disorder (ASD). To date, the relationship between sensory atypicalities in dyads of children with ASD and their parents has not been investigated. Exploring these relationships can contribute to an understanding of how phenotypic profiles may be inherited, and the extent to which familial factors might contribute towards children's sensory profiles and constitute an aspect of the broader autism phenotype (BAP). Parents of 44 children with ASD and 30 typically developing (TD) children, aged between 3 and 14 years, participated. Information about children's sensory experiences was collected through parent report using the Sensory Profile questionnaire. Information about parental sensory experiences was collected via self-report using the Adolescent/Adult Sensory Profile. Parents of children with ASD had significantly higher scores than parents of TD children in relation to low registration, over responsivity, and taste/smell sensory processing. Similar levels of agreement were obtained within ASD and TD parent-child dyads on a number of sensory atypicalities; nevertheless significant correlations were found between parents and children in ASD families but not TD dyads for sensation avoiding and auditory, visual, and vestibular sensory processing. The findings suggest that there are similarities in sensory processing profiles between parents and their children in both ASD and TD dyads. Familial sensory processing factors are likely to contribute towards the BAP. Further work is needed to explore genetic and environmental influences on the developmental pathways of the sensory atypicalities in ASD. Autism Res 2017, 10: 531-538. © 2016 International Society for Autism Research, Wiley Periodicals, Inc. © 2016 International Society for Autism Research, Wiley Periodicals, Inc.

  7. An Innovative Treatment Approach with Atypical Orthodontic Extraction Pattern in Bimaxillary Protrusion Case

    Directory of Open Access Journals (Sweden)

    Anil Miglani


    Full Text Available This case report describes the treatment of a 23-year-old female with severe bimaxillary dentoalveolar protrusion and missing left mandibular first molar. Her chief concern was significant facial convexity. Generally treatment plan of such cases involves extraction of first premolars but absence of first molar complicates situation with problem of space management, anchorage requirement and post-treatment occlusion. This case report introduces thought provoking treatment approach of selecting atypical teeth for orthodontic extraction without compromising the quality of treatment outcomes. However, three first bicuspids were extracted while the mandibular left second premolar was distalized into the missing molar space and used as an abutment for replacement of the missing first molar by prosthesis. Despite the unusual asymmetric extraction of teeth, superimposition of the pretreatment and post-treatment cephalometric tracings shows excellent treatment outcomes and reduction of facial convexity by maximum retraction of the anterior teeth.

  8. Using your shoulder after replacement surgery (United States)

    Joint replacement surgery - using your shoulder; Shoulder replacement surgery - after ... You have had shoulder replacement surgery to replace the bones of your shoulder joint with artificial parts. The parts include a stem made of metal and a ...

  9. First Japanese case of atypical progeroid syndrome/atypical Werner syndrome with heterozygous LMNA mutation. (United States)

    Motegi, Sei-ichiro; Yokoyama, Yoko; Uchiyama, Akihiko; Ogino, Sachiko; Takeuchi, Yuko; Yamada, Kazuya; Hattori, Tomoyasu; Hashizume, Hiroaki; Ishikawa, Yuichi; Goto, Makoto; Ishikawa, Osamu


    Atypical progeroid syndrome (APS), including atypical Werner syndrome (AWS), is a progeroid syndrome involving heterozygous mutations in the LMNA gene encoding the nuclear protein lamin A/C. We report the first Japanese case of APS/AWS with a LMNA mutation (p.D300N). A 53-year-old Japanese man had a history of recurrent severe cardiovascular diseases as well as brain infarction and hemorrhages. Although our APS/AWS patient had overlapping features with Werner syndrome (WS), such as high-pitched voice, scleroderma, lipoatrophy and atherosclerosis, several cardinal features of WS, including short stature, premature graying/alopecia, cataract, bird-like face, flat feet, hyperkeratosis on the soles and diabetes mellitus, were absent. In immunofluorescence staining and electron microscopic analyses of the patient's cultured fibroblasts, abnormal nuclear morphology, an increase in small aggregation of heterochromatin and a decrease in interchromatin granules in nuclei of fibroblasts were observed, suggesting that abnormal nuclear morphology and chromatin disorganization may be associated with the pathogenesis of APS/AWS.

  10. Educating My Replacement (United States)

    Tarter, Jill

    , in partnership with the dedicated teachers out there, I think I can help promote the critical thinking skills and scientific literacy of the next generation of voters. Hopefully, I can also help train my replacement to be a better scientist, capable of seizing all the opportunities generated by advances in technology and our improved understanding of the universe to craft search strategies with greater probability of success than those I have initiated.

  11. Clozapine versus other atypical antipsychotics for schizophrenia (United States)

    Asenjo Lobos, Claudia; Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; Leucht, Stefan


    Background Clozapine is an atypical antipsychotic demonstrated to be superior in the treatment of refractory schizophrenia which causes fewer movement disorders. Clozapine, however, entails a significant risk of serious blood disorders such as agranulocytosis which could be potentially fatal. Currently there are a number of newer antipsychotics which have been developed with the purpose to find both a better tolerability profile and a superior effectiveness. Objectives To compare the clinical effects of clozapine with other atypical antipsychotics (such as amisulpride, aripiprazole, olanzapine, quetiapine, risperidone, sertindole, ziprasidone and zotepine) in the treatment of schizophrenia and schizophrenia-like psychoses. Search methods We searched the Cochrane Schizophrenia Groups Register (June 2007) and reference lists of all included randomised controlled trials. We also manually searched appropriate journals and conference proceedings relating to clozapine combination strategies and contacted relevant pharmaceutical companies. Selection criteria All relevant randomised, at least single-blind trials, comparing clozapine with other atypical antipsychotics, any dose and oral formulations, for people with schizophrenia or related disorders. Data collection and analysis We selected trials and extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated mean differences (MD) again based on a random-effects model. Main results The review currently includes 27 blinded randomised controlled trials, which involved 3099 participants. Twelve randomised control trials compared clozapine with olanzapine, five with quetiapine, nine with risperidone, one with ziprasidone and two with zotepine. Attrition from these studies was high (overall 30.1%), leaving the interpretation

  12. [Ascending aorta replacement late after aortic valve replacement]. (United States)

    Hayashi, Yasunari; Ito, Toshiaki; Maekawa, Atsuo; Sawaki, Sadanari; Fujii, Genyo; Hoshino, Satoshi; Tokoro, Masayoshi; Yanagisawa, Junji


    Replacement of the asceding aorta is indicated in patients undergoing aortic valve replacement( AVR), if the diameter of the ascending aorta is greater than 5.0 cm. If the diameter of the asceding aorta is from 4.0 to 5.0 cm, it was arguable whether replacement of the ascending aorta should be performed. Nine patients who underwent reoperative ascending aorta replacement after AVR were reviewed retrospectively. Reoperation on the asending aorta replacement was performed 11.8±7.2 years (range 1y5m~23y3m) after AVR. Mean patient age was 69.9±6.3 (range 60~81). In 2 cases, reoperations were performed early year after AVR. Although ascending aorta was dilated at the 1st operation, replacement wasn't performed for the age and minimally invasive cardiac surgery (MICS). In 3 cases, reoperations were performed more than 10 years later. On these cases, ascending aorta aneurysm and dissection occurred with no pain and were pointed out by computed tomography(CT) or ultrasonic cardiogram(UCG). We think that patients with dilatation of the ascending aorta should undergo AVR and aorta replacement at the 1st operation regardness of age. It is important that patients who underwent AVR should undergo a regular checkup on the ascending aorta.

  13. Modelling atypical CYP3A4 kinetics: principles and pragmatism. (United States)

    Houston, J Brian; Galetin, Aleksandra


    The Michaelis-Menten model, and the existence of a single active site for the interaction of substrate with drug metabolizing enzyme, adequately describes a substantial number of in vitro metabolite kinetic data sets for both clearance and inhibition determination. However, in an increasing number of cases (involving most notably, but not exclusively, CYP3A4), atypical kinetic features are observed, e.g., auto- and heteroactivation; partial, cooperative, and substrate inhibition; concentration-dependent effector responses (activation/inhibition); limited substrate substitution and inhibitory reciprocity necessitating sub-group classification. The phenomena listed above cannot be readily interpreted using single active site models and the literature indicates that three types of approaches have been adopted. First the 'nai ve' approach of using the Michaelis-Menten model regardless of the kinetic behaviour, second the 'empirical' approach (e.g., employing the Hill or uncompetitive inhibition equations to model homotropic phenomena of sigmoidicity and substrate inhibition, respectively) and finally, the 'mechanistic' approach. The later includes multisite kinetic models derived using the same rapid equilibrium/steady-state assumptions as the single-site model. These models indicate that 2 or 3 binding sites exist for a given CYP3A4 substrate and/or effector. Multisite kinetic models share common features, depending on the substrate kinetics and the nature of the effector response observed in vitro, which allow a generic model to be proposed. Thus although more complex than the other two approaches, they show more utility and can be comprehensively applied in relatively simple versions that can be readily generated from generic model. Multisite kinetic features, observed in isolated hepatocytes as well as in microsomes from hepatic tissue and heterologous expression systems, may be evident in substrate depletion-time profiles as well as in metabolite formation rates

  14. Childhood Learning Disabilities and Atypical Dementia: A Retrospective Chart Review.

    Directory of Open Access Journals (Sweden)

    Alon Seifan

    Full Text Available To further our understanding of the association between self-reported childhood learning disabilities (LDs and atypical dementia phenotypes (Atypical Dementia, including logopenic primary progressive aphasia (L-PPA, Posterior Cortical Atrophy (PCA, and Dysexecutive-type Alzheimer's Disease (AD.This retrospective case series analysis of 678 comprehensive neuropsychological assessments compared rates of self-reported LD between dementia patients diagnosed with Typical AD and those diagnosed with Atypical Dementia. 105 cases with neuroimaging or CSF data available and at least one neurology follow-up were identified as having been diagnosed by the neuropsychologist with any form of neurodegenerative dementia. These cases were subject to a consensus diagnostic process among three dementia experts using validated clinical criteria for AD and PPA. LD was considered Probable if two or more statements consistent with prior LD were documented within the Social & Developmental History of the initial neuropsychological evaluation.85 subjects (Typical AD n=68, Atypical AD n=17 were included in the final analysis. In logistic regression models adjusted for age, gender, handedness, education and symptom duration, patients with Probable LD, compared to patients without Probable LD, were significantly more likely to be diagnosed with Atypical Dementia vs. Typical AD (OR 13.1, 95% CI 1.3-128.4. All three of the L-PPA cases reporting a childhood LD endorsed childhood difficulty with language. By contrast, both PCA cases reporting Probable childhood LD endorsed difficulty with attention and/or math.In people who develop dementia, childhood LD may predispose to atypical phenotypes. Future studies are required to confirm whether atypical neurodevelopment predisposes to regional-specific neuropathology in AD and other dementias.

  15. Atypical/Nor98 scrapie infectivity in sheep peripheral tissues.

    Directory of Open Access Journals (Sweden)

    Olivier Andréoletti

    Full Text Available Atypical/Nor98 scrapie was first identified in 1998 in Norway. It is now considered as a worldwide disease of small ruminants and currently represents a significant part of the detected transmissible spongiform encephalopathies (TSE cases in Europe. Atypical/Nor98 scrapie cases were reported in ARR/ARR sheep, which are highly resistant to BSE and other small ruminants TSE agents. The biology and pathogenesis of the Atypical/Nor98 scrapie agent in its natural host is still poorly understood. However, based on the absence of detectable abnormal PrP in peripheral tissues of affected individuals, human and animal exposure risk to this specific TSE agent has been considered low. In this study we demonstrate that infectivity can accumulate, even if no abnormal PrP is detectable, in lymphoid tissues, nerves, and muscles from natural and/or experimental Atypical/Nor98 scrapie cases. Evidence is provided that, in comparison to other TSE agents, samples containing Atypical/Nor98 scrapie infectivity could remain PrP(Sc negative. This feature will impact detection of Atypical/Nor98 scrapie cases in the field, and highlights the need to review current evaluations of the disease prevalence and potential transmissibility. Finally, an estimate is made of the infectivity loads accumulating in peripheral tissues in both Atypical/Nor98 and classical scrapie cases that currently enter the food chain. The results obtained indicate that dietary exposure risk to small ruminants TSE agents may be higher than commonly believed.

  16. Age-dependent atypicalities in body- and face-sensitive activation of the EBA and FFA in individuals with ASD. (United States)

    Okamoto, Yuko; Kosaka, Hirotaka; Kitada, Ryo; Seki, Ayumi; Tanabe, Hiroki C; Hayashi, Masamichi J; Kochiyama, Takanori; Saito, Daisuke N; Yanaka, Hisakazu T; Munesue, Toshio; Ishitobi, Makoto; Omori, Masao; Wada, Yuji; Okazawa, Hidehiko; Koeda, Tatsuya; Sadato, Norihiro


    Individuals with autism spectrum disorder (ASD) have difficuly in recognizing bodies and faces, which are more pronounced in children than adults. If such difficulties originate from dysfunction of the extrastriate body area (EBA) and the fusiform face area (FFA), activation in these regions might be more atypical in children than in adults. We preformed functional magnetic resonance imaging while children and adults with ASD and age-matched typically developed (TD) individuals observed face, body, car, and scene. To examine various aspects, we performed individual region of interest (ROI) analysis, as well as conventional random effect group analysis. At individual ROI analysis, we examined the ratio of participants showing a category-sensitive response, the size of regions, location and activation patterns among the four object categories. Adults with ASD showed no atypicalities in activation of the EBA and FFA, whereas children with ASD showed atypical activation in these regions. Specifically, a smaller percentage of children with ASD showed face-sensitive activation of the FFA than TD children. Moreover, the size of the EBA was smaller in children with ASD than in TD children. Our results revealed atypicalities in both the FFA and EBA in children with ASD but not in adults with ASD. Copyright © 2017 Elsevier Ireland Ltd and Japan Neuroscience Society. All rights reserved.

  17. Acanthomatous ameloblastoma with atypical foci in five dogs. (United States)

    Malmberg, Jennifer L; Howerth, Elizabeth W; Powers, Barb E; Schaffer, Paula A


    Acanthomatous ameloblastoma is a common, locally invasive, nonmetastasizing tumor of the canine oral cavity. The long-term prognosis for canine acanthomatous ameloblastoma is good if complete excision can be achieved, usually by maxillectomy or mandibulectomy. A variant of acanthomatous ameloblastoma with atypical foci was noted in 5 dogs. There was no age, breed, or sex predisposition. Atypical cells were pleomorphic with a high mitotic rate. They were immunohistochemically negative for cytokeratin, vimentin, melan A, PNL2, CD3, Pax5, CD18, chromogranin A, and synaptophysin. Ultrastructurally, the atypical cells had modest amounts of electron-lucent cytoplasm, abundant rough endoplasmic reticulum, zonula adherens junctions, cleaved or irregular nuclei, and occasional cytoplasmic structures consistent with secretory granules or lysosomes. Complete excision was achieved by maxillectomy or mandibulectomy in 3 dogs; the lesion was incompletely excised in 2 dogs. No ancillary therapy was elected in any patient. No local recurrence or distant metastasis was reported in any case. One patient died of heart failure 20 mo following complete excision; all other patients were alive at last follow-up (average follow-up: 18.8 mo, range: 6-30 mo). The histogenesis of the atypical foci is unclear, but atypical foci within acanthomatous ameloblastoma do not appear to be associated with metastasis or with a poor prognosis relative to acanthomatous ameloblastoma with typical histologic morphology.

  18. Atypical pityriasis rosea: clinical evaluation of 27 patients. (United States)

    Kilinc, Fadime; Akbas, Ayse; Sener, Sertac; Aktaş, Akın


    Pityriasis rosea (PR) is a common, acute, self-limiting inflammatory skin disease. It can easily be recognized with its typical clinical presentation. However, unusual clinic presentations can cause difficulty in diagnosis. Up to now, not many atypical forms are reported. To determine the clinical characteristics of patients with atypical pityriasis rosea. A total of 27 cases, diagnosed as atypical PR by clinical and/or histopathological examination and applied to the outpatient clinic of dermatology department between the years 2007 and 2015 were analyzed retrospectively. The ages of patients ranged from 2 to 59 years. Of these patients, 15 (55.6%) were male and 12 (44.4%) were female. The male-to-female ratio was 5-4. Five patients had papular, four patients had purpuric, three patients had vesicular, two patients had follicular, one patient had erythema multiforme-like and one patient had eczematous drug-induced atypical form of pityriasis rosea. There were 12 cases of localized, two cases of segmental pityriasis rosea. Four of the localized forms also had atypical morphology. Histopathological evaluation was required for diagnosis in 12 (44.4%) patients. PR can appear in many different uncommon forms. Localization and skin rush can be misleading and diagnosis can be compelling.

  19. Atypical periprosthetic acetabular fracture in long-term alendronate therapy. (United States)

    Marongiu, Giuseppe; Capone, Antonio


    Bisphosphonates have been commonly used in the treatment of osteoporosis, demonstrating its efficacy in fracture risk reduction. However, even if are generally safe and well tolerated, concerns have emerged about atypical fractures related to its prolonged use. Although atypical femoral fracture are more common, case reports demonstrated that even other skeletal areas can be involved by unusual pattern of fracture. We report a atypical acetabular periprosthetic fracture in a 83-year-old female patient after prolonged alendronate treatment for osteoporosis and isolated acetabular revision surgery. The patient underwent to clinical, bioumoral and radiological evaluation and all the history cases were fully reported. We believe this periprosthetic fracture, according to the available data, may have similar underlying pathology to atypical femoral fractures. Awareness of symptoms, in addition to a regular radiographic survey may facilitate early diagnosis and possible prevention of spontaneous periprosthetic fractures, in patients receiving bisphosphonate therapy beyond 5 years. The treatment of this atypical periprosthetic fracture should include both surgical than pharmacological therapy to obtained bone healing.

  20. Atypical periprosthetic acetabular fracture in long-term alendronate therapy (United States)

    Marongiu, Giuseppe; Capone, Antonio


    Summary Bisphosphonates have been commonly used in the treatment of osteoporosis, demonstrating its efficacy in fracture risk reduction. However, even if are generally safe and well tolerated, concerns have emerged about atypical fractures related to its prolonged use. Although atypical femoral fracture are more common, case reports demonstrated that even other skeletal areas can be involved by unusual pattern of fracture. We report a atypical acetabular periprosthetic fracture in a 83-year-old female patient after prolonged alendronate treatment for osteoporosis and isolated acetabular revision surgery. The patient underwent to clinical, bioumoral and radiological evaluation and all the history cases were fully reported. We believe this periprosthetic fracture, according to the available data, may have similar underlying pathology to atypical femoral fractures. Awareness of symptoms, in addition to a regular radiographic survey may facilitate early diagnosis and possible prevention of spontaneous periprosthetic fractures, in patients receiving bisphosphonate therapy beyond 5 years. The treatment of this atypical periprosthetic fracture should include both surgical than pharmacological therapy to obtained bone healing. PMID:28228784

  1. Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

    Directory of Open Access Journals (Sweden)

    Qiang Zhou


    Full Text Available We describe here a rare case of variant of mycosis fungoides (MF: ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG, IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

  2. Management of atypical lobular hyperplasia, atypical ductal hyperplasia, and lobular carcinoma in situ. (United States)

    Clauser, Paola; Marino, Maria A; Baltzer, Pascal A T; Bazzocchi, Massimo; Zuiani, Chiara


    Atypical hyperplasia and lobular carcinoma in situ are rare proliferative breast lesions, growing inside ducts and terminal ducto-lobular units. They represent a marker of increased risk for breast cancer and a non-obligate precursor of malignancy. Evidence available on diagnosis and management is scarce. They are frequently found incidentally associated with other lesions, but can be visible through mammography, ultrasound or magnetic resonance. Due to the risk of underestimation, surgical excision is often performed. The analysis of imaging and histopathological characteristics could help identifying low-risk cases, for which surgery is not necessary. Chemopreventive agents can be used for risk reduction. Careful imaging follow up is mandatory; the role of breast MRI as screening modality is under discussion.

  3. Electrocatalysts Prepared by Galvanic Replacement


    Athanasios Papaderakis; Ioanna Mintsouli; Jenia Georgieva; Sotiris Sotiropoulos


    Galvanic replacement is the spontaneous replacement of surface layers of a metal, M, by a more noble metal, Mnoble, when the former is treated with a solution containing the latter in ionic form, according to the general replacement reaction: nM + mMnoblen+ → nMm+ + mMnoble. The reaction is driven by the difference in the equilibrium potential of the two metal/metal ion redox couples and, to avoid parasitic cathodic processes such as oxygen reduction and (in some cases) hydrogen evolution too...

  4. Testosterone replacement attenuates intimal hyperplasia development in an androgen deficient model of vascular injury. (United States)

    Freeman, Brian M; Univers, Junior; Fisher, Richard K; Kirkpatrick, Stacy S; Klein, Frederick A; Freeman, Michael B; Mountain, Deidra J H; Grandas, Oscar H


    Androgen deficiency (AD) is associated with increased risk of vascular disease. Dysfunctional remodeling of the vessel wall and atypical proliferative potential of vascular smooth muscle cells (VSMCs) are fundamental processes in the development of intimal hyperplasia (IH). We have demonstrated an inverse relationship between dihydrotestosterone (DHT) levels, matrix metalloproteinase activity, and VSMC migration and proliferation in vitro. Here, we investigated the role of AD and testosterone (TST) replacement in IH development in an animal model of vascular injury to elucidate mechanisms modulated by AD that could be playing a role in the development of vascular pathogenesis. Aged orchiectomized male rats underwent TST supplementation via controlled release pellet (0.5-35 mg). Young adult and middle-age adult intact (MI) and orchiectomized placebo (Plac) groups served as controls. All groups underwent balloon angioplasty of the left common carotid at a 14-d post-TST. Carotid tissue was collected at a 14-d post-balloon angioplasty and subjected to morphologic and immunohistochemical analyses. Human male VSMCs were treated with DHT (0-3000 nM) for 24 h then subjected to quantitative PCR for gene expression analyses and costained for F-actin and G-actin for visualization of cytoskeletal organization. I:M ratio was increased in Plac, subphysiological, low-physiological, and high pharmacologic level TST animals compared with MI controls but was decreased with high-physiological TST supplementation. Injury-induced expression of previously defined matrix metalloproteinase remodeling enzymes was not significantly affected by TST status. Urotensin (UTS) receptor (UTSR) staining was low in injured vessels of all young adult intact, MI, and Plac controls but was significantly upregulated in all groups receiving exogenous TST supplementation, irrespective of dose. In vitro DHT exposure increased the expression of UTSR in VSMCs in a dose-dependent manner. However, this did

  5. Atypical Vitamin B-6 Deficiency A Rare Cause of Unexplained Neonatal and Infantile Epilepsies

    DEFF Research Database (Denmark)

    Baumgart, A.; von Spiczak, S.; Verhoeven-Duif, N. M.


    . We identified 1 patient with an epilepsy phenotype resembling Dravet syndrome and likely pathogenic mutations in ALDH7A1. Presenting features were highly atypical of pyridoxine-dependent epilepsy, including febrile seizures, response to anticonvulsive drugs, and periods of seizure freedom without......ALDH7A1 and PNPO deficiencies are rare inborn errors of vitamin B-6 metabolism causing perinatal seizure disorders. The phenotypic variability, however, is broad. To assess the frequency of these deficiencies in unexplained infantile epilepsy, we screened 113 patients for mutations in both genes...... pyridoxine treatment. "Hidden" vitamin B-6 deficiencies might be rare but treatable causes of unexplained epilepsy extending beyond the classical phenotypes....

  6. Acute cutaneous graft-versus-host disease resembling type II (atypical adult) pityriasis rubra pilaris. (United States)

    Surjana, Devita; Robertson, Ivan; Kennedy, Glen; James, Daniel; Weedon, David


    We present a case of cutaneous acute graft-versus-host disease (aGVHD) with confluent erythematous perifollicular hyperkeratosis and ichthyosiform scale in the clinical pattern of type II (atypical adult) pityriasis rubra pilaris (PRP), which developed 26 days after allogeneic peripheral blood stem cell transplant. Skin histology confirmed features of both aGVHD and PRP. The skin lesions were refractory to oral prednisolone and cyclosporine and only partially responsive to a combination of i.v. methylprednisolone, oral tacrolimus, oral mycophenolate mofetil, and infusions of anti-thymocyte globulin and the tumour necrosis factor-α inhibitor, etanercept. © 2013 The Australasian College of Dermatologists.


    Directory of Open Access Journals (Sweden)

    Alessandro Abramo


    Full Text Available Necrotizing fasciitis is a fulminant infection that affects the deep and superficial fascia while initially sparing the overlying skin and underlying muscle. The involvement of the cervicalcompartment is associated with a high morbidity and mortality rate. Immunocompromised patients are prone to this infection. We present a case of a 80 year-old diabetic woman suffering from streptococcal cervical NF (probably secondary to a arm injury with an uncommon involvement of the posterior cervical compartment, highlighting the atypical responsible microorganism (Streptococcus Agalactiae, the role of imaging for early diagnosis and the timely surgical and medical treatment for a successful outcome.

  8. Olanzapine versus other atypical antipsychotics for schizophrenia (United States)

    Komossa, Katja; Rummel-Kluge, Christine; Hunger, Heike; Schmid, Franziska; Schwarz, Sandra; Duggan, Lorna; Kissling, Werner; Leucht, Stefan


    Background In many countries of the industrialised world second generation (“atypical”) antipsychotics have become the first line drug treatment for people with schizophrenia. The question as to whether, and if so how much, the effects of the various second generation antipsychotics differ is a matter of debate. In this review we examined how the efficacy and tolerability of olanzapine differs from that of other second generation antipsychotics. Objectives To evaluate the effects of olanzapine compared to other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychosis. Search methods 1. Electronic searching We searched the Cochrane Schizophrenia Group Trials Register (April 2007) which is based on regular searches of BIOSIS, CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. 2. Reference searching We inspected the reference of all identified studies for more trials. 3. Personal contact We contacted the first author of each included study for missing information. 4. Drug companies We contacted the manufacturers of all atypical antipsychotics included for additional data. Selection criteria We included all randomised trials that used at least single-blind (rater-blind) design, comparing oral olanzapine with oral forms of amisulpride, aripiprazole, clozapine, quetiapine, risperidone, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychosis. Data collection and analysis We extracted data independently. For dichotomous data we calculated relative risks (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated weighted mean differences (WMD) again based on a random effects model. Main results The review currently includes 50 studies and 9476 participants which provided data for six comparisons (olanzapine compared to amisulpride, aripiprazole

  9. Risperidone versus other atypical antipsychotics for schizophrenia (United States)

    Komossa, Katja; Rummel-Kluge, Christine; Schwarz, Sandra; Schmid, Franziska; Hunger, Heike; Kissling, Werner; Leucht, Stefan


    Background In many countries of the industrialised world second-generation (“atypical”) antipsychotics (SGAs) have become the first line drug treatment for people with schizophrenia. The question as to whether and if so how much the effects of the various SGAs differ is a matter of debate. In this review we examined how the efficacy and tolerability of risperidone differs from that of other SGAs. Objectives To evaluate the effects of risperidone compared with other atypical antipsychotics for people with schizophrenia and schizophrenia-like psychosis. Search methods 1. Electronic searching We searched the Cochrane Schizophrenia Group Trials Register (April 2007) which is based on regular searches of BIOSIS, CENTRAL, CINAHL, EMBASE, MEDLINE and PsycINFO. 2. Reference searching We inspected the references of all identified studies for more trials. 3. Personal contact We contacted the first author of each included study for missing information. 4. Drug companies We contacted the manufacturers of all atypical antipsychotics included for additional data. Selection criteria We included all randomised, blinded trials comparing oral risperidone with oral forms of amisulpride, aripiprazole, clozapine, olanzapine, quetiapine, sertindole, ziprasidone or zotepine in people with schizophrenia or schizophrenia-like psychosis. Data collection and analysis We extracted data independently. For dichotomous data we calculated risk ratio (RR) and their 95% confidence intervals (CI) on an intention-to-treat basis based on a random-effects model. We calculated numbers needed to treat/harm (NNT/NNH) where appropriate. For continuous data, we calculated mean differences (MD), again based on a random-effects model. Main results The review currently includes 45 blinded RCTs with 7760 participants. The number of RCTs available for each comparison varied: four studies compared risperidone with amisulpride, two with aripiprazole, 11 with clozapine, 23 with olanzapine, eleven with

  10. Rare Posterior Pharyngeal Mass: Atypical Marginal Zone Hyperplasia. (United States)

    Eliçora, Sultan Şevik; Güven, Mehmet; Varli, Ali F; Yilmaz, Mahmut S; Alponat, Selin


    Cases of posterior pharyngeal masses are quite rare, and are typically derived from schwannoma or encephalocele, or are of vascular or infectious origin. They are clinically significant due to their tendency to cause airway obstruction. The aim of this study was to present a rare atypical marginal hyperplasia case of a posterior pharyngeal wall mass. A 10-year-old male was admitted to our clinic with dyspnea. A plane-surfaced 4 × 3 × 3 cm mass was observed on the posterior pharyngeal wall upon physical examination. The patient underwent magnetic resonance imaging and surgical treatment. Following excision of material from the patient's mass, a pathologic diagnosis of atypical marginal zone hyperplasia was made. Atypical marginal zone hyperplasia of the posterior pharyngeal wall has not yet been reported in the literature. Marginal zone hyperplasia associated with a lymphoproliferative disease should be considered when making differential diagnoses of posterior pharyngeal wall masses.

  11. Familial Mediterranean fever variant with repeated atypical skin eruptions. (United States)

    Takahashi, Tomoko; Fujisawa, Tomomi; Kimura, Masaki; Ohnishi, Hidenori; Seishima, Mariko


    Familial Mediterranean fever (FMF) is characterized by self-limited bouts of fever and polyserositis. Skin involvement is not common in FMF, and erysipelas-like erythema is found to be the most frequent skin eruption which is often accompanied by arthritis and fever, and disappears within 12-72 h. We report a 40-year-old Japanese woman who presented with a 2-year history of recurrent fever with general fatigue, polyarthralgia and transient maculopapular eruptions on her lower extremities and trunk. The histological findings of the maculopapular eruption showed lymphocyte infiltration around the capillaries in the entire dermis. Mutation analysis showed a heterozygous E148Q-P369S mutation of MEFV. These findings suggested a diagnosis of late-onset FMF variant with atypical skin eruptions. The patient was successfully treated with colchicine. Thus, we should pay attention to repeated atypical skin eruptions for the early detection of atypical FMF. © 2015 Japanese Dermatological Association.

  12. Atypical femur fractures associated with bisphosphonates: from prodrome to resolution

    Directory of Open Access Journals (Sweden)

    Braulio Sastre-Jala


    Full Text Available Atypical fractures related to the prolonged use of bisphosphonates are caused by low energy mechanisms and are characterized by oblique and transverse lines and frequent bilateralism. We present a clinical case of a patient who we believe illustrates, both in clinical and radiological aspects, the new definition of atypical femur fracture related to treatment using bisphosphonates treated conservatively and successfully with discharge and teriparatide 20 mcg/80 mcl s.c./24h. The appearance of painful symptoms in the upper thigh, especially if bilateral, in patients treated with bisphosphonates for long periods of time, makes it necessary to dismiss bone lesions that might otherwise suggest atypical fracture. In those cases where the fracture is incomplete, restoring bone metabolism through the administration of teriparatide 20 mcg/80 mcl s.c./24h could prevent displaced fractures.

  13. Atypical cellular blue nevus or malignant blue nevus?* (United States)

    Daltro, Luise Ribeiro; Yaegashi, Lygia Bertalha; Freitas, Rodrigo Abdalah; Fantini, Bruno de Carvalho; Souza, Cacilda da Silva


    Blue nevus is a benign melanocytic lesion whose most frequent variants are dendritic (common) blue nevus and cellular blue nevus. Atypical cellular blue nevus presents an intermediate histopathology between the typical and a rare variant of malignant blue nevus/melanoma arising in a cellular blue nevus. An 8-year-old child presented a pigmented lesion in the buttock since birth, but with progressive growth in the last two years. After surgical excision, histopathological examination revealed atypical cellular blue nevus. Presence of mitoses, ulceration, infiltration, cytological atypia or necrosis may occur in atypical cellular blue nevus, making it difficult to differentiate it from melanoma. The growth of blue nevus is unusual and considered of high-risk for malignancy, being an indicator for complete resection and periodic follow-up of these patients. PMID:28225968

  14. Atypical Imaging Findings in Primary Central Nervous System Lymphoma

    Directory of Open Access Journals (Sweden)

    Zahra Afravi


    Full Text Available Background/Objective: The incidence of primary CNS lymphomas (PCNSL is increasing. Timely diagnosis of PCNSL can lead to proper therapeutic management. There are some atypical imaging findings that may easily be misdiagnosed as other pathologic processes such as infectious and demyelinative diseases. As a result, histopathologic diagnosis is necessary for all suspected lesions."nPatients and Methods: In this research we studied 120 cases of PCNSL over the past 16 years. Some of them had atypical imaging findings, suggesting many differential diagnoses. Having said that, stereotactic biopsy was performed for all cases and the diagnosis was proved."nResults: We selected some interesting cases with atypical imaging findings of PCNSL, which were unlikely to be diagnosed without histopathologic evaluation. "nConclusion: PCNSL must be kept in mind as a differential diagnosis for other brain lesions. Histopathologic diagnosis is necessary for prompt management.

  15. Minimally invasive aortic valve replacement

    DEFF Research Database (Denmark)

    Foghsgaard, Signe; Schmidt, Thomas Andersen; Kjaergard, Henrik K


    In this descriptive prospective study, we evaluate the outcomes of surgery in 98 patients who were scheduled to undergo minimally invasive aortic valve replacement. These patients were compared with a group of 50 patients who underwent scheduled aortic valve replacement through a full sternotomy....... The 30-day mortality rate for the 98 patients was zero, although 14 of the 98 mini-sternotomies had to be converted to complete sternotomies intraoperatively due to technical problems. Such conversion doubled the operative time over that of the planned full sternotomies. In the group of patients whose...... is an excellent operation in selected patients, but its true advantages over conventional aortic valve replacement (other than a smaller scar) await evaluation by means of randomized clinical trial. The "extended mini-aortic valve replacement" operation, on the other hand, is a risky procedure that should...

  16. A potential mechanism underlying atypical antipsychotics-induced lipid disturbances. (United States)

    Cai, H L; Tan, Q Y; Jiang, P; Dang, R L; Xue, Y; Tang, M M; Xu, P; Deng, Y; Li, H D; Yao, J K


    Previous findings suggested that a four-protein complex, including sterol-regulatory element-binding protein (SREBP), SREBP-cleavage-activating protein (SCAP), insulin-induced gene (INSIG) and progesterone receptor membrane component 1 (PGRMC1), within the endoplasmic reticulum appears to be an important regulator responsible for atypical antipsychotic drug (AAPD)-induced lipid disturbances. In the present study, effects of typical antipsychotic drug and AAPDs as well as treatment outcome of steroid antagonist mifepristone (MIF) on the PGRMC1/INSIG/SCAP/SREBP pathway were investigated in rat liver using real-time quantitative polymerase chain reaction (qPCR) and western blot analysis. In addition, serum triacylglycerol, total cholesterol, free fatty acids and various hormones including progesterone, corticosterone and insulin were measured simultaneously. Following treatment with clozapine or risperidone, both lipogenesis and cholesterogenesis were enhanced via inhibition of PGRMC1/INSIG-2 and activation of SCAP/SREBP expressions. Such metabolic disturbances, however, were not demonstrated in rats treated with aripiprazole (ARI) or haloperidol (HAL). Moreover, the add-on treatment of MIF was effective in reversing the AAPD-induced lipid disturbances by upregulating the expression of PGRMC1/INSIG-2 and subsequent downregulation of SCAP/SREBP. Taken together, our findings suggest that disturbances in lipid metabolism can occur at an early stage of AAPD treatment before the presence of weight gain. Such metabolic defects can be modified by an add-on treatment of steroid antagonist MIF enhancing the PGRMC1 pathway. Thus, it is likely that PGRMC1/INSIG-2 signaling may be a therapeutic target for AAPD-induced weight gain.

  17. [Atypical preeclampsia and perinatal success: a case report]. (United States)

    Castelazo-Morales, Ernesto; Monzalbo-Núñez, Diana Elena; López-Rioja, Miguel de Jesus; Castelazo-Alatorre, Santiago


    Preeclampsia is a multi systemic syndrome of variable severity, pregnancy specific, consequence of an abnormal vascular response to placentation, with increase in peripheral vascular resistance, stimulation of platelet aggregation, activation of the coagulation cascade, and endothelial dysfunction. In the majority of cases, it will present with gestational hypertension and proteinuria, after 20 weeks, nevertheless, in other cases, it has presented as an atypical form (with absence of hypertension and/or proteinuria) behaving like severe preeclampsia. We report the case of a patient, 32 year old primigravida, with a 26.6 weeks gestation, that comes to the office with postprandial projectile vomiting. At the time of admission, she presents with sudden headache, phosphenes, and tinnitus, associated to epigastric pain, with a blood pressure of 110/70 mmHg; fetal heart rate of 146 bpm, normal lower limbs and reflexes. The Complete Blood Count and liver function tests are within normal values, urinalysis with proteinuria. Normal hepatic ultrasound. Obstetric ultrasound; with fetal measurements of 2.6 weeks below, with flattening of the growth curve, Doppler flowmetry with an increase in placental resistance and oligohydramnios. Given the clinical manifestations compatible with severe preeclampsia, magnesium sulfate, and antenatal corticosteroid therapy are initiated, and abdominal termination of pregnancy is decided. A female newborn is obtained, of 595 g, Apgar 4/7. On immediate puerperium, she presents with an increase in blood pressure of 150/90 mmHg, discharged with normal blood pressure 96 hours later. The newborn girl was discharged when she reached a weight of 2000 g. Today, she is neurologically unimpaired.

  18. Total joint replacement preadmission programs. (United States)

    Messer, B


    Patients begin to formulate their expectations of the postoperative hospitalization during the preadmission program. The challenge is to better understand the factors patients consider when formulating judgments about the quality of preadmission education. For example, it may be that perceptions of the preadmission program are influenced by what patients believe about their postoperative pain and functional abilities. Specific attention needs to be given both preoperatively and postoperatively to instructing patients on realistic expectations for recovery. One other method of measuring patient outcomes is with the Health Status Profile (SF-36) (Response Healthcare Information Management, 1995). The SF-36 approach emphasizes the outcome of medical care as the patient sees it, in addition to a clinical evaluation of successful health care. This form is currently initiated in the physician's office and returned for scanning at the preadmission class. The patient then completes another SF-36 at 6 months and every year thereafter to compare measurable outcomes. Patients intending to have elective total joint replacements experience anxiety and require much support and education. An effective preadmission program is a major investment in a patient's recovery, as well as a unique marketing tool to customers. Preadmission programs can be viewed as an opportunity to enhance customer satisfaction. Preadmission clinics are an excellent means for nurses to improve the quality of patient care through patient education. the overall goal of preadmission testing programs is to ensure patient preparedness while increasing quality health care and overall customer satisfaction. To enhance program effectiveness, health care providers must lead collaborative efforts to improve the efficiency of systems.

  19. [Comprehensive assessment of atypical-extraction orthodontic treatment]. (United States)

    Yijia, Xie; Zhou, Hu; Qing, Zhao; Fuwei, Lin; Yang, Zeng; Xiaomei, Xu; Lin, Zeng; Pu, Yang


    To discuss through retrospective study associated factors influencing orthodontists to develop orthodontic treatment plans and to calculate constituent ratio of a typical extraction in orthodontic treatment. Systematic sampling was performed among 7 344 orthodontic patients, who received orthodontic treatment from April 2012 to March 2014 in the Department of Orthodontics, West China Hospital of Stomatology, Sichuan University. The study included statistical data on factors that may influence development of treatment plans. Samples were used to calculate extraction-ratio and atypical-extraction-ratio. Research focused on associated factors influencing development of treatment plans while evaluating correlation significances of each factor. Finally, treatment outcomes of atypical-extraction were compared with those of typical-extraction. Among studied patients, 55.31% (406/734) received orthodontic extraction treatment. In orthodontic-extraction-treated patients, typical-extraction accounted for 59.11% (240/406), orthodontists-selected atypical-extraction accounted for 23.15% (94/406), and passive atypical-extraction accounted for 17.73% (72/406). With statistical analysis, we inferred associated factors influencing development of treatment plans as follows: sex and age of patients, sex of orthodontists, accurate condition of specific teeth, Angle's classification, and degree of midline deviation. Tooth loss before treatment also directly influences passive atypical-extraction. Statistically significant factors were not obtained. Significant difference of treatment outcome was not observed between atypical-extraction and typical-extraction-orthodontic treatment (P>0.05). Above mentioned factors may influence orthodontists to develop treatment plans. However, orthodontists should also consider expected results of treatment strategies to prepare individual treatment plans on the basis of comprehensive analysis. 

  20. [Diagnosis and treatment of heparin-induced thrombocytopenia (HIT) based on its atypical immunological features]. (United States)

    Miyata, Shigeki; Maeda, Takuma


    Heparin-induced thrombocytopenia (HIT) is a prothrombotic side effect of heparin therapy caused by HIT antibodies, i.e., anti-platelet factor 4 (PF4)/heparin IgG with platelet-activating properties. For serological diagnosis, antigen immunoassays are commonly used worldwide. However, such assays do not indicate their platelet-activating properties, leading to low specificity for the HIT diagnosis. Therefore, over-diagnosis is currently the most serious problem associated with HIT. The detection of platelet-activating antibodies using a washed platelet activation assay is crucial for appropriate HIT diagnosis. Recent advances in our understanding of the pathogenesis of HIT include it having several clinical features atypical for an immune-mediated disease. Heparin-naïve patients can develop IgG antibodies as early as day 4, as in a secondary immune response. Evidence for an anamnestic response on heparin re-exposure is lacking. In addition, HIT antibodies are relatively short-lived, unlike those in a secondary immune response. These lines of evidence suggest that the mechanisms underlying HIT antibody formation may be compatible with a non-T cell-dependent immune reaction. These atypical clinical and serological features should be carefully considered while endeavoring to accurately diagnose HIT, which leads to appropriate therapies such as immediate administration of an alternative anticoagulant to prevent thromboembolic events and re-administration of heparin during surgery involving cardiopulmonary bypass when HIT antibodies are no longer detectable.

  1. Uremic parkinsonism with atypical phenotypes and radiologic features. (United States)

    Yoon, Jee-Eun; Kim, Ji Sun; Park, Jeong-Ho; Lee, Kyung-Bok; Roh, Hakjae; Park, Sung Tae; Cho, Jin Whan; Ahn, Moo-young


    Uremic encephalopathy with bilateral basal ganglia lesions has been reported as an acute neurometabolic disease which shows reversible clinical course and brain imaging features. The exact nature and pathophysiology have not been well established. We encountered two patients who showed a relapsing and aggravating course and an atypical phenotype including parkinsonism with paroxysmal dystonic head tremor and acute onset monoparesis of the lower extremity. They also showed unusual radiological findings which revealed combined lesions in the basal ganglia and cortex, persistent hemorrhagic transformation, and focal ischemic lesion in the internal capsule. Herein, we present the unusual phenomenology with atypical radiologic findings and suggest the possible multifactorial pathogenesis of uremic encephalopathy.

  2. Atypical Pupillary Light Reflex in Individuals with Autism (United States)


    AWARD NUMBER: W81XWH-10-1-0474 TITLE: Atypical Pupillary Light Reflex in Individuals with Autism ...30th/2014 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER W81XWH-10-1-0474 Atypical Pupillary  Light  Reflex in Individuals with  Autism 5b. GRANT NUMBER...20,  p< .00005 in all instances].  15. SUBJECT TERMS Pupillary light reflex, autism , functional MRI 16. SECURITY CLASSIFICATION OF: 17

  3. Atypical Neuroleptic Malignant Syndrome Associated with Use of Clozapine

    Directory of Open Access Journals (Sweden)

    Quevedo-Florez Leonardo


    Full Text Available The Neuroleptic Malignant Syndrome (NMS is a medical emergency of infrequent presentation in the emergency department, which is associated with the use of psychiatric drugs, such as typical and atypical antipsychotics. Our case addresses a 55-year-old patient diagnosed with undifferentiated schizophrenia for 10 years, who had been receiving clozapine and clonazepam as part of their treatment. This patient presents the symptoms of Neuroleptic Malignant Syndrome without fever, which improves with treatment especially with the withdrawal of clozapine. In the absence of fever and clinical improvement, the patient is considered to have an atypical presentation of this disease.

  4. Atypical hemolytic uremic syndrome: A report of two cases

    Directory of Open Access Journals (Sweden)

    Md. Habibur Rahman


    Full Text Available Hemolytic uremic syndrome (HUS is one of the important cause of acute kidney injury in children. There is excellent outcome in patients with typical HUS but atypical HUS is associated with high mortality, risk of recurrence and may lead to end stage renal disease. We report two cases of 5 and 6 year old child having clinical & laboratory characteristics of atypical HUS. These children had a fulminant course of illness with complications involving various systems. The report provides an insight into the etio pathogenesis, diagnoses and treatment of this condition.

  5. Atypicality in presentation of neuroleptic malignant syndrome caused by olanzapine

    Directory of Open Access Journals (Sweden)

    Mishra Biswaranjan


    Full Text Available Neuroleptic malignant syndrome (NMS is the most serious of acute neurological side effects produced by antipsychotic medication, characterized by hyperthermia, rigidity, altered consciousness and autonomic dysfunction, the prevalence of which varies from 0.4-1.4%. NMS is usually seen in treatment with high potency typical antipsychotics and very rarely with atypical antipsychotics. However, NMS cases have been reported with risperidone, clozapine, olanzapine and quetiapine. The presentations of NMS have often varied, and we report another atypicality in presentation of NMS due to olanzapine use.

  6. [Atypical and rare cardiac revelation about Sheehan's syndrome: A report of three cases]. (United States)

    Bouznad, N; Mghari, G El; Hattaoui, M El; Ansari, N El


    Sheehan syndrome is a potentially serious complication in the postpartum period corresponding to ischemic necrosis of the anterior pituitary related to postpartum haemorrhage. We report three original observations showing an unusual mode of revelation of this syndrome. The first observation is that of a 46-year-old patient admitted initially to resuscitation for a recovered cardiorespiratory arrest, severe hypoglycemia and profound hyponatremia. The second is that of a 45-year-old patient, admitted for recurrent cardiac tamponade after pericardial and pleural puncture and pericardial drainage; clinical survey found signs of slight panhypopituitarism. The latest case is that of a patient of 44 years, admitted to pericardial effusion average abundance revealed by dyspnea and tachypnea with hypotension. The interrogation of all patients revealed the concept of an old hemorrhagic syndrome, absence of lactation and secondary amenorrhea thereafter. Laboratory tests showed insufficient thyroid-stimulating, low cortisol, a hypogonadism hypogonadism. The pituitary magnetic resonance imaging showed an empty sella in the three cases. Patients were placed under replacement therapy with L-thyroxine and hydrocortisone with good clinical, biological and echocardiographic evolution. The three cases illustrate a rare heart atypical presentation for Sheehan's syndrome and underline the importance of early diagnosis and suitable replacement therapy of this syndrome to avoid this complication that can be life threatening.

  7. A child with myoclonus-dystonia (DYT11) misdiagnosed as atypical opsoclonus myoclonus syndrome

    DEFF Research Database (Denmark)

    Drivenes, Bergitte; Born, Alfred Peter; Ek, Jakob


    INTRODUCTION: DYT11 is an autosomal dominant inherited movement disorder characterized by myoclonus and dystonia. CLINICAL PRESENTATION: We present a case with atypical symptoms and with episodes of ataxia and myoclonus preceded by infections. Atypical presentation of opsoclonus myoclonus syndrome...

  8. Knee Replacement: What you can Expect (United States)

    ... improves function lessen with each additional surgery. Artificial knees can wear out Another risk of knee replacement ... replacement surgery to last about two hours. After knee replacement surgery After surgery, you're wheeled to ...

  9. Homologous gene replacement in Physarum

    Energy Technology Data Exchange (ETDEWEB)

    Burland, T.G. [Univ. of Wisconsin, Madison, WI (United States); Pallotta, D. [Laval Univ., Quebec (Canada)


    The protist Physarum polycephalum is useful for analysis of several aspects of cellular and developmental biology. To expand the opportunities for experimental analysis of this organism, we have developed a method for gene replacement. We transformed Physarum amoebae with plasmid DNA carrying a mutant allele, ardD{Delta}1, of the ardD actin gene; ardD{Delta}1 mutates the critical carboxy-terminal region of the gene product. Because ardD is not expressed in the amoeba, replacement of ardD{sup +} with ardD{Delta}1 should not be lethal for this cell type. Transformants were obtained only when linear plasmid DNA was used. Most transformants carried one copy of ardD{Delta}1 in addition to ardD{sup +}, but in two (5%), ardD{sup +} was replaced by a single copy of ardD{Delta}1. This is the first example of homologous gene replacement in Physarum. ardD{Delta}1 was stably maintained in the genome through growth, development and meiosis. We found no effect of ardD{Delta}l on viability, growth, or development of any of the various cell types of Physarum. Thus, the carboxy-terminal region of the ardD product appears not to perform a unique essential role in growth or development. Nevertheless, this method for homologous gene replacement can be applied to analyze the function of any cloned gene. 38 refs., 6 figs., 1 tab.

  10. Giant atypical ossifying fibromyxoid tumour of the calf

    Energy Technology Data Exchange (ETDEWEB)

    Harish, Srinivasan [Cambridge University Hospitals NHS Foundation Trust, Department of Radiology, Cambridge (United Kingdom); Addenbrookes Hospital, Department of Radiology, Hills Road, Box 219, Cambridge (United Kingdom); Polson, Alexander; Griffiths, Meryl [Cambridge University Hospitals NHS Foundation Trust, Department of Histopathology, Cambridge (United Kingdom); Morris, Paul; Malata, Charles [Cambridge University Hospitals NHS Foundation Trust, Department of Plastic Surgery, Cambridge (United Kingdom); Bearcroft, Philip W.P. [Cambridge University Hospitals NHS Foundation Trust, Department of Radiology, Cambridge (United Kingdom)


    We present a case of giant atypical ossifying fibromyxoid tumour (OFMT) of soft tissue, occurring in the calf, in a 77-year-old woman. The patient presented with a history of bleeding ulcer over a calf lump that had been present for over 4 years. Clinical presentation, radiological features and histopathologic findings are described, and the relevant literature is reviewed. (orig.)

  11. Exploring Atypical Verb+Noun Combinations in Learner Technical Writing (United States)

    Luzon Marco, Maria Jose


    Professional and academic discourse is characterised by a specific phraseology, which usually poses problems for students. This paper investigates atypical verb+noun collocations in a corpus of English technical writing of Spanish students. I focus on the type of verbs that most frequently occurred in these awkward or questionable combinations and…

  12. Late atypical atrial flutter after ablation of atrial fibrillation. (United States)

    Ferreira, Raquel; Primo, João; Adão, Luís; Gonzaga, Anabela; Gonçalves, Helena; Santos, Rui; Fonseca, Paulo; Santos, José; Gama, Vasco


    Cardiac surgery for structural heart disease (often involving the left atrium) and radiofrequency catheter ablation of atrial fibrillation have led to an increased incidence of regular atrial tachycardias, often presenting as atypical flutters. This type of flutter is particularly common after pulmonary vein isolation, especially after extensive atrial ablation including linear lesions and/or defragmentation. The authors describe the case of a 51-year-old man, with no relevant medical history, referred for a cardiology consultation in 2009 for paroxysmal atrial fibrillation. After failure of antiarrhythmic therapy, he underwent catheter ablation, with criteria of acute success. Three years later he again suffered palpitations and atypical atrial flutter was documented. The electrophysiology study confirmed the diagnosis of atypical left flutter and reappearance of electrical activity in the right inferior pulmonary vein. This vein was again ablated successfully and there has been no arrhythmia recurrence to date. In an era of frequent catheter ablation it is essential to understand the mechanism of this arrhythmia and to recognize such atypical flutters.

  13. The Cost-Effectiveness of Atypicals in the UK

    NARCIS (Netherlands)

    Heeg, Bart; Buskens, Erik; Botteman, Marc; Caleo, Sue; Ingham, Mike; Damen, Joep; de Charro, Frank; van Hout, Ben


    Background: In 2002, the National Institute for Health and Clinical Excellence (NICE), recommended atypical antipsychotics over conventional ones for first-line schizophrenia treatment, based on their lower risk of extrapyramidal symptoms. Objective: To estimate the incremental cost-effectiveness of

  14. Transposition with atypical coronary pattern: the Aubert technique. (United States)

    Pita-Fernández, Ana; González-López, María T; Gil-Jaurena, Juan M


    The arterial switch operation is currently the gold standard technique for repair of transposition of the great arteries. Some atypical coronary patterns such as intramural, interarterial, and a unique posterior button are associated with more complexity and surgical risk. We report a successful Aubert operation for transposition of the great arteries associated with a single and interarterial coronary artery arising from a posterior sinus.

  15. Atypical Presentation of Fibrolipomatous Hamartoma of Superficial Peroneal Nerve. (United States)

    Dhinsa, Baljinder Singh; Lidder, Surjit; Abbasian, Ali


    Fibrolipomatous hamartoma is a rare presentation in the foot. An accurate diagnosis is key, with magnetic resonance imaging findings considered definitive. The management is dependent on the symptoms. We present an atypical presentation of fibrolipomatous hamartoma of the superficial peroneal nerve and discuss the current published data.

  16. Stereological estimation of nuclear volume in benign and atypical meningiomas

    DEFF Research Database (Denmark)

    Madsen, C; Schrøder, H D


    A stereological estimation of nuclear volume in benign and atypical meningiomas was made. The aim was to investigate whether this method could discriminate between these two meningeal neoplasms. The difference was significant and it was moreover seen that there was no overlap between the two grou...

  17. Treatment of atypical trigeminal neuralgia with microvascular decompression

    Directory of Open Access Journals (Sweden)

    Hai Jian


    Full Text Available Aim: To explore the methods for achieving pain relief in patients with atypical trigeminal neuralgia (TN using microvascular decompression (MVD. Study Design and Settings: Retrospective study of 26 patients treated during the years 2000 to 2004. Materials and Methods: Twenty-six patients in whom vascular compression of the trigeminal nerve was identified by high definition magnetic resonance tomographic angiography (MRTA were treated with MVD for atypical TN in our department. Clinical presentations, surgical findings and clinical outcomes were analyzed retrospectively. Results: In this study, single trigeminal division was involved in only 2 patients (8% and two or three divisions in the other 24 patients (92%. Of prime importance is the fact that in 46.2% of the patients, several conflicting vessels were found in association. Location of the conflicts around the circumference of the trigeminal root was supero-medial to the root in 53.5%, supero-lateral in 30.8% and inferior in 15.7%. MVD for atypical TN resulted in complete pain relief in 50% of the patients with complete decompression, partial pain relief in 30.8% and poor pain relief or pain recurrence in 19.2% of the patients without complete decompression postoperatively. Conclusions: Complete decompression of the entire trigeminal root plays an important role in achieving pain relief in patients with atypical TN with MVD.

  18. Atypical pathogens in community acquired pneumonia of Egyptian children

    Institute of Scientific and Technical Information of China (English)

    Deraz TE; El Sahriggy SA; Shaheen MA; Motawea AA; Gomaa HE; Fawzy SH; Mohamed AA


    Objective:Diagnosis of atypical pathogens as an aetiology for community-acquired pneumonia (CAP)in chil-dren is a challenge world wide.The aim of this study was to detect the frequency of atypical pathogens as a cause of community-acquired pneumonia (CAP)in Egyptian children.Methods:From 50 children (with age ranged from 2 months to 1 2 years)hospitalized for community-acquired pneumonia;respiratory sputum samples were collected by induction or spontaneously.All samples were subjected to conventional cultures and Polymer-ase Chain Reaction(PCR)technique DNA extraction for identification of Mycoplasma,Chlamydia pneumoniae and Legionella pneumophila.Results:A definite pathogen was identified in 78% of the studied children;30%typical bacteria,8% candida albicans and atypical bacteria in 40% of the pneumonic children.Chlamydia pneumoniae was isolated from 26% of the children while Mycoplasma pneumoniae was isolated from 1 4%, whereas Legionella pneumophilla was not isolated at all.Conclusion:Atypical pathogens are evident as a po-tential aetiology for community-acquired pneumonia in (1 3.3%)of young and (80%)of older Egyptian chil-dren.

  19. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.

    NARCIS (Netherlands)

    Legendre, C.M.; Licht, C.; Muus, P.; Greenbaum, L.A.; Babu, S.; Bedrosian, C.; Bingham, C.; Cohen, D.J.; Delmas, Y.; Douglas, K.; Eitner, F.; Feldkamp, T.; Fouque, D.; Furman, R.R.; Gaber, O.; Herthelius, M.; Hourmant, M.; Karpman, D.; Lebranchu, Y.; Mariat, C.; Menne, J.; Moulin, B.; Nurnberger, J.; Ogawa, M.; Remuzzi, G.; Richard, T.; Sberro-Soussan, R.; Severino, B.; Sheerin, N.S.; Trivelli, A.; Zimmerhackl, L.B.; Goodship, T.; Loirat, C.


    BACKGROUND: Atypical hemolytic-uremic syndrome is a genetic, life-threatening, chronic disease of complement-mediated thrombotic microangiopathy. Plasma exchange or infusion may transiently maintain normal levels of hematologic measures but does not treat the underlying systemic disease. METHODS: We

  20. Characterization of atypical Aeromonas salmonicida by different methods

    DEFF Research Database (Denmark)

    Austin, B.; Austin, D.A.; Dalsgaard, Inger;


    Fifty two isolates of atypical Aeromonas salmonicida, recovered from a wide range of hosts and geographical locations, were heterogeneous in terms of molecular and phenotypic characteristics, and represented taxa which could not be accommodated by the current classification of four subspecies...

  1. Sonographic findings of typical and atypical scrotal trauma. (United States)

    Sommers, Daniel N; Jensen, Jeff


    This review article illustrates sonographic findings in the setting of accidental and nonaccidental scrotal trauma. Although sonographic findings may be irrespective of the type of trauma, the goals of sonographic evaluation are similar in both atypical and typical mechanisms of scrotal injury. Familiarity with findings such as disruption of testicular integrity or vascularity facilitates prompt diagnosis and plays a critical role in clinical management.

  2. Experimental replacement of an obligate insect symbiont. (United States)

    Moran, Nancy A; Yun, Yueli


    Symbiosis, the close association of unrelated organisms, has been pivotal in biological diversification. In the obligate symbioses found in many insect hosts, organisms that were once independent are permanently and intimately associated, resulting in expanded ecological capabilities. The primary model for this kind of symbiosis is the association between the bacterium Buchnera and the pea aphid (Acyrthosiphon pisum). A longstanding obstacle to efforts to illuminate genetic changes underlying obligate symbioses has been the inability to experimentally disrupt and reconstitute symbiont-host partnerships. Our experiments show that Buchnera can be experimentally transferred between aphid matrilines and, furthermore, that Buchnera replacement has a massive effect on host fitness. Using a recipient pea aphid matriline containing Buchnera that are heat sensitive because of an allele eliminating the heat shock response of a small chaperone, we reduced native Buchnera through heat exposure and introduced a genetically distinct Buchnera from another matriline, achieving complete replacement and stable inheritance. This transfer disrupted 100 million years (∼ 1 billion generations) of continuous maternal transmission of Buchnera in its host aphids. Furthermore, aphids with the Buchnera replacement enjoyed a dramatic increase in heat tolerance, directly demonstrating a strong effect of symbiont genotype on host ecology.

  3. Clinical Profile of Atypical Manifestations of Dengue Fever. (United States)

    Pothapregada, Sriram; Kamalakannan, Banupriya; Thulasingam, Mahalakshmy


    To study the clinical profile and outcome of the atypical manifestations of dengue fever in children. All children (0-12 y of age) diagnosed and confirmed as dengue fever at a tertiary care hospital at Puducherry, between the 1st of August 2012 and January 31st 2015 were reviewed retrospectively from hospital case records as per the revised World Health Organization (WHO) guidelines 2011 for dengue fever. The diagnosis was confirmed by NS1 antigen-based ELISA test or dengue serology for IgM and IgG antibodies and the data was analyzed using SPSS 16.0 statistical software. Out of 254 children admitted with dengue fever, non-severe dengue and severe dengue were seen in 62.6 % and 37.4 % respectively. Atypical manifestations were seen in 106 cases (41.7 %). Mean age of presentation was 6.9(3.3) y. M: F ratio was 1.2:1. The common manifestations of severe dengue infection were shock (37.4 %), bleeding (20.1 %) and multi-organ dysfunction (2.4 %). The most common atypical manifestations of dengue fever were lymphadenopathy (41.7 %), splenomegaly (21.2 %), biphasic fever (18.1 %), hepatitis (11.4 %), febrile diarrhea (6.3 %), refractory shock (2.4 %) and impaired consciousness (1.9 %). The other atypical manifestations present were portal hypertension, acalculous cholecystitis, appendicitis, acute respiratory distress syndrome (ARDS), myocarditis, pericardial effusion, paroxysmal supraventricular tachycardia (PSVT), myositis, acute kidney injury (AKI), hemophagocytic syndrome and disseminated intravascular coagulopathy (DIC). Platelet count did not always correlate well with the severity of bleeding. There were six deaths (2.4 %) and out of them four presented with impaired consciousness (66.6 %). The common causes for poor outcome were multiorgan failure, encephalopathy and refractory shock. The atypical manifestations of dengue fever are no more a rare entity. Clinicians should have a high index of suspicion and vigilance for atypical manifestations of

  4. Cobra Probes Containing Replaceable Thermocouples (United States)

    Jones, John; Redding, Adam


    A modification of the basic design of cobra probes provides for relatively easy replacement of broken thermocouples. Cobra probes are standard tube-type pressure probes that may also contain thermocouples and that are routinely used in wind tunnels and aeronautical hardware. They are so named because in side views, they resemble a cobra poised to attack. Heretofore, there has been no easy way to replace a broken thermocouple in a cobra probe: instead, it has been necessary to break the probe apart and then rebuild it, typically at a cost between $2,000 and $4,000 (2004 prices). The modified design makes it possible to replace the thermocouple, in minimal time and at relatively low cost, by inserting new thermocouple wire in a tube.

  5. Supervised clustering of immunohistochemical markers to distinguish atypical and non-atypical endometrial hyperplasia. (United States)

    Laas, Enora; Ballester, Marcos; Cortez, Annie; Gonin, Julie; Canlorbe, Geoffroy; Daraï, Emile; Graesslin, Olivier


    The risk of endometrial hyperplasia (EH) progressing into endometrioid endometrial cancer ranges from 1% for simple EH without atypia (EHWA) to 46.2% for atypical EH (AEH). Differentiation between both entities is crucial to determine optimal management. As preoperative diagnosis of AEH can be difficult, we aimed to establish clusters of immunohistochemical markers to distinguish EHWA from AEH. We studied 13 immunohistochemical markers (steroid receptors, pro/anti-apoptotic proteins, metalloproteinases (MMP), tissue inhibitor of metalloproteinase (TIMP), CD44 isoforms) known for their role in endometrial pathology. Using supervised clustering, we determined clusters of co-expressed proteins which contributed the most in differentiating EHWA from AEH. From 39 tissue samples (17 EHWA and 22 AEH), we found three clusters of co-expressed proteins: Cluster 1 included two proteins (over-expression of estrogen receptor (ER) and under-expression of progesterone receptor (PR) B in AEH compared to EHWA); Cluster 2: an ER, PR A, MMP-2 and TIMP-1 over-expression and a PR B and TIMP-2 under-expression; Cluster 3: over-expression of ER and MMP-7 and under-expression of PR B and TIMP-2. AEH can be accurately distinguished from EHWA using a supervised clustering of immunohistochemical markers. This promising approach could be useful to improve the preoperative diagnosis of EH.

  6. Understanding A-type supergiants. I. Ultraviolet and visible spectral atlas of A-type supergiants

    CERN Document Server

    Verdugo, E; Gómez de Castro, A I


    This paper is the first of a series whose aim is to perform a systematic study of A-type supergiant atmospheres and winds. Here we present a spectral atlas of 41 A-supergiants observed by us in high and medium resolution in the visible and ultraviolet. The atlas consists of profiles of the H alpha , H beta , H gamma , H delta , H epsilon , Ca II (H and K), Na I (D1 and D2), Mg II/sub 4481/, Mg II uv1 and Fe II uv1, uv2, uv3, uv62, uv63, uv161 lines for 41 stars with spectral types ranging from B9 to A9 and luminosity classes Ia, Iab and Ib, and provides the basic data for a thoughtful study of these stars. The overall characteristics of the sample as well as the data reduction procedures are described. We also present some examples of spectral variability. Figures 1-3 are only available in electronic form at (27 refs).

  7. Pegasus, the 'atypical' Ikaros family member, influences left-right asymmetry and regulates pitx2 expression. (United States)

    John, Liza B; Trengove, Monique C; Fraser, Fiona W; Yoong, Simon H; Ward, Alister C


    Members of the Ikaros family of zinc-finger transcription factors have been shown to be critical for immune and blood cell development. However, the role of the most divergent family member, Pegasus, has remained elusive, although it shows conservation to invertebrate Hunchback proteins that influence embryonic patterning through regulation of homeodomain genes. Zebrafish was employed as a relevant model to investigate the function of Pegasus since it possesses a single pegasus orthologue with high homology to its mammalian counterparts. During zebrafish embryogenesis pegasus transcripts were initially maternally-derived and later replaced by zygotic expression in the diencephalon, tectum, hindbrain, thymus, eye, and ultimately the exocrine pancreas and intestine. Morpholino-mediated knockdown of the zebrafish pegasus gene resulted in disrupted left-right asymmetry of the gut and pancreas. Molecular analysis indicated that zebrafish Pegasus localised to the nucleus in discrete non-nucleolar structures and bound the 'atypical' DNA sequence GN3GN2G, confirming its presumed role as a transcriptional regulator. In vivo transcriptome analysis identified candidate target genes, several of which encoded homeodomain transcription factors. One of these, pitx2, implicated in left-right asymmetry, possessed appropriate 'atypical' Pegasus binding sites in its promoter. Knockdown of Pegasus affected both the level and asymmetry of pitx2 expression, as well as disrupting the asymmetry of the lefty2 and spaw genes, explaining the perturbed left-right patterning in pegasus morphants. Collectively these results provide the first definitive insights into the in vivo role of Pegasus, supporting the notion that it acts as a broader regulator of development, with potential parallels to the related invertebrate Hunchback proteins.

  8. VH replacement in primary immunoglobulin repertoire diversification. (United States)

    Sun, Amy; Novobrantseva, Tatiana I; Coffre, Maryaline; Hewitt, Susannah L; Jensen, Kari; Skok, Jane A; Rajewsky, Klaus; Koralov, Sergei B


    The genes encoding the variable (V) region of the B-cell antigen receptor (BCR) are assembled from V, D (diversity), and J (joining) elements through a RAG-mediated recombination process that relies on the recognition of recombination signal sequences (RSSs) flanking the individual elements. Secondary V(D)J rearrangement modifies the original Ig rearrangement if a nonproductive original joint is formed, as a response to inappropriate signaling from a self-reactive BCR, or as part of a stochastic mechanism to further diversify the Ig repertoire. VH replacement represents a RAG-mediated secondary rearrangement in which an upstream VH element recombines with a rearranged VHDHJH joint to generate a new BCR specificity. The rearrangement occurs between the cryptic RSS of the original VH element and the conventional RSS of the invading VH gene, leaving behind a footprint of up to five base pairs (bps) of the original VH gene that is often further obscured by exonuclease activity and N-nucleotide addition. We have previously demonstrated that VH replacement can efficiently rescue the development of B cells that have acquired two nonproductive heavy chain (IgH) rearrangements. Here we describe a novel knock-in mouse model in which the prerearranged IgH locus resembles an endogenously rearranged productive VHDHJH allele. Using this mouse model, we characterized the role of VH replacement in the diversification of the primary Ig repertoire through the modification of productive VHDHJH rearrangements. Our results indicate that VH replacement occurs before Ig light chain rearrangement and thus is not involved in the editing of self-reactive antibodies.

  9. Cutaneous and Subcutaneous Metastases From Atypical Laryngeal Carcinoids (United States)

    Wang, Kui-Rong; Jia, Yuan-Jing; Zhou, Shui-Hong; Wang, Qin-Ying; Bao, Yang-Yang; Feng, Zhi-Ying; Yao, Hong-Tian; Fan, Jun


    Abstract The incidence of cutaneous and subcutaneous metastases from atypical laryngeal carcinoids is approximately 20%. However, the pathogenesis and natural history of, and prognostic factors for, the condition remain poorly understood. We reported a 54-year-old female presented with cutaneous and subcutaneous metastases from atypical laryngeal carcinoid. Laryngoscopy revealed a 0.5 × 1.5-cm reddish mass on the laryngeal surface of the epiglottis. Under general anesthesia, a biopsy sample was obtained via suspension laryngoscopy. Routine pathology revealed atypical laryngeal carcinoid. Immunohistochemical staining of the sections of primary tumor was positive for cytokeratin, chromogranin A, synaptophysin, hypoxia-inducible factor-1α, P53, and CD56. GLUT-1, p-Akt, and PI3K were negative. The Ki-67 index was 15%. Supraglottic laryngectomy and selective right-neck dissection were performed. After 6 months, the patient complained of pain in the right wall of the chest; multiple cutaneous and subcutaneous nodules were evident at that site and in the abdomen. An abdominal nodule was biopsied and pathology revealed that the atypical metastatic carcinoid had metastasized to both cutaneous and subcutaneous areas of the abdomen. Chemotherapy was then prescribed. Currently, the intrathecal drug delivery system remains in place. No local recurrence has been detected. Furthermore, we systematically reviewed clinical manifestations of the disease, pathogenesis, prognostic factors, and treatment. The metastasis rate (cutaneous and subcutaneous) was approximately 12.2%. Thirty patients (62.5%) with cutaneous and subcutaneous metastases exhibited contemporaneous lymph node invasion. The 3-, 5-, and 10-year survival rates were 44.0%, 22.0%, and 13.0%, respectively. The prognosis of patients with atypical laryngeal carcinoids was poor. Relevant prognostic factors included the level of p53, human papilloma virus status, certain hypoxic markers, and distant metastasis. No

  10. Renal replacement therapy in ICU

    Directory of Open Access Journals (Sweden)

    C Deepa


    Full Text Available Diagnosing and managing critically ill patients with renal dysfunction is a part of the daily routine of an intensivist. Acute kidney insufficiency substantially contributes to the morbidity and mortality of critically ill patients. Renal replacement therapy (RRT not only does play a significant role in the treatment of patients with renal failure, acute as well as chronic, but also has spread its domains to the treatment of many other disease conditions such as myaesthenia gravis, septic shock and acute on chronic liver failure. This article briefly outlines the role of renal replacement therapy in ICU.

  11. Prioritization methodology for chemical replacement (United States)

    Goldberg, Ben; Cruit, Wendy; Schutzenhofer, Scott


    This methodology serves to define a system for effective prioritization of efforts required to develop replacement technologies mandated by imposed and forecast legislation. The methodology used is a semi quantitative approach derived from quality function deployment techniques (QFD Matrix). QFD is a conceptual map that provides a method of transforming customer wants and needs into quantitative engineering terms. This methodology aims to weight the full environmental, cost, safety, reliability, and programmatic implications of replacement technology development to allow appropriate identification of viable candidates and programmatic alternatives.

  12. Wafer Replacement Cluster Tool (Presentation);

    Energy Technology Data Exchange (ETDEWEB)

    Branz, H. M.


    This presentation on wafer replacement cluster tool discusses: (1) Platform for advanced R and D toward SAI 2015 cost goal--crystal silicon PV at area costs closer to amorphous Si PV, it's 15% efficiency, inexpensive substrate, and moderate temperature processing (<800 C); (2) Why silicon?--industrial and knowledge base, abundant and environmentally benign, market acceptance, and good efficiency; and (3) Why replace wafers?--expensive, high embedded energy content, and uses 50-100 times more silicon than needed.

  13. A systematic review of lessons learned from PET molecular imaging research in atypical parkinsonism

    Energy Technology Data Exchange (ETDEWEB)

    Niccolini, Flavia; Politis, Marios [Neurodegeneration Imaging Group, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King' s College London, London (United Kingdom)


    To systematically review the previous studies and current status of positron emission tomography (PET) molecular imaging research in atypical parkinsonism. MEDLINE, ISI Web of Science, Cochrane Library, and Scopus electronic databases were searched for articles published until 29th March 2016 and included brain PET studies in progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal syndrome (CBS). Only articles published in English and in peer-reviewed journals were included in this review. Case-reports, reviews, and non-human studies were excluded. Seventy-seven PET studies investigating the dopaminergic system, glucose metabolism, microglial activation, hyperphosphorilated tau, opioid receptors, the cholinergic system, and GABA{sub A} receptors in PSP, MSA, and CBS patients were included in this review. Disease-specific patterns of reduced glucose metabolism have shown higher accuracy than dopaminergic imaging techniques to distinguish between parkinsonian syndromes. Microglial activation has been found in all forms of atypical parkinsonism and reflects the known distribution of neuropathologic changes in these disorders. Opioid receptors are decreased in the striatum of PSP and MSA patients. Subcortical cholinergic dysfunction was more severe in MSA and PSP than Parkinson's disease patients although no significant changes in cortical cholinergic receptors were seen in PSP with cognitive impairment. GABA{sub A} receptors were decreased in metabolically affected cortical and subcortical regions in PSP patients. PET molecular imaging has provided valuable insight for understanding the mechanisms underlying atypical parkinsonism. Changes at a molecular level occur early in the course of these neurodegenerative diseases and PET imaging provides the means to aid differential diagnosis, monitor disease progression, identify of novel targets for pharmacotherapy, and monitor response to new treatments. (orig.)

  14. Recurrent opisthotonus in catatonia: An atypical presentation

    Directory of Open Access Journals (Sweden)

    Manjunatha Narayana


    Full Text Available Opisthotonus is known to occur in tetanus, rabies, cerebral malaria, neurosyphilis, acute cerebral injury and other medical conditions. Opisthotonus, so far, has not been reported in any major psychiatric disorder. Authors report a case of recurrent opisthotonus presenting concurrently with other catatonic signs which showed dramatic response to combination of lorazepam and electroconvulsive therapy (ECT. Clinicians should consider the possibility of catatonia in the differential diagnosis of opisthotonus since catatonia can be treated easily with benzodiazepines and ECT.

  15. Double emulsions as fat replacers

    NARCIS (Netherlands)

    Oppermann, Anika


    The use of double (w1/o/w2) emulsions, in which part of the oil is replaced by small water droplets, is a promising strategy to reduce oil content in food products. For successful applications, (1) significant levels of fat reduction (i.e. significant amounts of water inside the oil droplets) have

  16. Replacement policies for dairy cows

    DEFF Research Database (Denmark)

    Nielsen, Lars Relund

    In a recent paper a hierarchical Markov decision processes (MDP) with finite state and action space was formulated for the dairy cow replacement problem with stage lengths of 1 d. Bayesian updating was used to predict the performance of each cow in the herd and economic decisions were based...

  17. Electrocatalysts Prepared by Galvanic Replacement

    Directory of Open Access Journals (Sweden)

    Athanasios Papaderakis


    Full Text Available Galvanic replacement is the spontaneous replacement of surface layers of a metal, M, by a more noble metal, Mnoble, when the former is treated with a solution containing the latter in ionic form, according to the general replacement reaction: nM + mMnoblen+ → nMm+ + mMnoble. The reaction is driven by the difference in the equilibrium potential of the two metal/metal ion redox couples and, to avoid parasitic cathodic processes such as oxygen reduction and (in some cases hydrogen evolution too, both oxygen levels and the pH must be optimized. The resulting bimetallic material can in principle have a Mnoble-rich shell and M-rich core (denoted as Mnoble(M leading to a possible decrease in noble metal loading and the modification of its properties by the underlying metal M. This paper reviews a number of bimetallic or ternary electrocatalytic materials prepared by galvanic replacement for fuel cell, electrolysis and electrosynthesis reactions. These include oxygen reduction, methanol, formic acid and ethanol oxidation, hydrogen evolution and oxidation, oxygen evolution, borohydride oxidation, and halide reduction. Methods for depositing the precursor metal M on the support material (electrodeposition, electroless deposition, photodeposition as well as the various options for the support are also reviewed.

  18. Bonding over Dentin Replacement Materials. (United States)

    Meraji, Naghmeh; Camilleri, Josette


    Dentin replacement materials are necessary in large cavities to protect the pulp and reduce the bulk of filling material. These materials are layered with a composite resin restorative material. Microleakage caused by poor bonding of composite resin to underlying dentin replacement material will result in pulp damage. The aim of this study was to characterize the interface between dentin replacement materials and composite resin and to measure the shear bond strength after dynamic aging. Biodentine (Septodont, Saint Maur-des-Fosses, France), Theracal LC (Bisco, Schaumburg, IL), and Fuji IX (GC, Tokyo, Japan) were used as dentin replacement materials. They were then overlaid with a total-etch and bonding agent or a self-etch primer and composite resin or a glass ionomer cement. All combinations were thermocycled for 3000 cycles. The interface was characterized using scanning electron microscopy and elemental mapping. Furthermore, the shear bond strength was assessed. The Biodentine surface was modified by etching. The Theracal LC and Fuji IX microstructure was unchanged upon the application of acid etch. The Biodentine and glass ionomer interface showed an evident wide open space, and glass particles from the glass ionomer adhered to the Biodentine surface. Elemental migration was shown with aluminum, barium, fluorine, and ytterbium present in Biodentine from the overlying composite resin. Calcium was more stable. The bond strength between Theracal LC and composite using a total-etch technique followed by self-etch primer achieved the best bond strength values. Biodentine exhibited the weakest bond with complete failure of bonding shown after demolding and thermocycling. Dynamic aging is necessary to have clinically valid data. Bonding composite resin to water-based dentin replacement materials is still challenging, and further alternatives for restoration of teeth using such materials need to be developed. Copyright © 2017 American Association of Endodontists

  19. Fatal atypical reversible posterior leukoencephalopathy syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Golombeck Stefanie Kristin


    Full Text Available Abstract Introduction Reversible posterior leukoencephalopathy syndrome – a reversible subacute global encephalopathy clinically presenting with headache, altered mental status, visual symptoms such as hemianopsia or cortical blindness, motor symptoms, and focal or generalized seizures – is characterized by a subcortical vasogenic edema symmetrically affecting posterior brain regions. Complete reversibility of both clinical signs and magnetic resonance imaging lesions is regarded as a defining feature of reversible posterior leukoencephalopathy syndrome. Reversible posterior leukoencephalopathy syndrome is almost exclusively seen in the setting of a predisposing clinical condition, such as pre-eclampsia, systemic infections, sepsis and shock, certain autoimmune diseases, various malignancies and cytotoxic chemotherapy, transplantation and concomitant immunosuppression (especially with calcineurin inhibitors as well as episodes of abrupt hypertension. We describe for the first time clinical, radiological and histological findings in a case of reversible posterior leukoencephalopathy syndrome with an irreversible and fatal outcome occurring in the absence of any of the known predisposing clinical conditions except for a hypertensive episode. Case presentation A 58-year-old Caucasian woman presented with a two-week history of subacute and progressive occipital headache, blurred vision and imbalance of gait and with no evidence for raised arterial blood pressure during the two weeks previous to admission. Her past medical history was unremarkable except for controlled arterial hypertension. Cerebral magnetic resonance imaging demonstrated cortical and subcortical lesions with combined vasogenic and cytotoxic edema atypical for both venous congestion and arterial infarction. Routine laboratory and cerebrospinal fluid parameters were normal. The diagnosis of reversible posterior leukoencephalopathy syndrome was established. Within hours after

  20. Adult-onset Still's disease with atypical cutaneous manifestations (United States)

    Narváez Garcia, Francisco Javier; Pascual, María; López de Recalde, Mercè; Juarez, Pablo; Morales-Ivorra, Isabel; Notario, Jaime; Jucglà, Anna; Nolla, Joan M.


    Abstract The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis. The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome. The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy

  1. Atypical depression in the structure of organic mental disorders (literature review

    Directory of Open Access Journals (Sweden)

    Leonov S.F.


    Full Text Available The review of literature presents current data on cli¬nical picture and diagnostics of atypical depression. Rubric “atypical depression” includes a variety of depressive states characterized by reactively caused changes of mood, sensitivity to interpersonal contacts, inverted vegetative and somatic symptoms such as increased appetite and hypersomnia. The article considers the place of atypical depression in the structure of organic mental disorders. Positions of foreign authors that produce atypical depression as a clinical entity in the structure of Bipolar affective disorder II type are represented, the views of other authors on the structure of atypical depression are considered. The analysis of national concept of non-circular depression is carried out. Questions of atypical affective conditions acquire special significance due to preparation of International Classification of Diseases of the 11th revision, because inclusion in it of Bipolar affective disorder II type, a manifestation of which is considered to be atypical depressions, is under discussion.

  2. Characterization of Atypical Off-Flavor Compounds in Natural Cork Stoppers by Multidimensional Gas Chromatographic Techniques. (United States)

    Slabizki, Petra; Fischer, Claus; Legrum, Charlotte; Schmarr, Hans-Georg


    Natural cork stoppers with sensory deviations other than the typical cork taint were subgrouped according to their sensory descriptions and compared with unaffected control cork stoppers. The assessment of purge and trap extracts obtained from corresponding cork soaks was performed by heart-cut multidimensional gas chromatography-olfactometry (MDGC-O). The identification of compounds responsible for atypical cork taint detected in MDGC-O was further supported with additional multidimensional GC analysis in combination with mass spectrometric detection. Geosmin and 2-methylisoborneol were mainly found in cork stoppers described as moldy and cellarlike; 3-isopropyl-2-methoxypyrazine and 3-isobutyl-2-methoxypyrazine were found in cork stoppers described with green attributes. Across all cork subgroups, the impact compound for typical cork taint, 2,4,6-trichloroanisole (TCA), was present and is therefore a good marker for cork taint in general. Another potent aroma compound, 3,5-dimethyl-2-methoxypyrazine (MDMP), was also detected in each subgroup, obviously playing an important role with regard to the atypical cork taint. Sensory deviations possibly affecting the wine could be generated by MDMP and its presence should thus be monitored in routine quality control.

  3. Atypical sensory sensitivity as a shared feature between synaesthesia and autism (United States)

    Ward, Jamie; Hoadley, Claire; Hughes, James E. A.; Smith, Paula; Allison, Carrie; Baron-Cohen, Simon; Simner, Julia


    Several studies have suggested that there is a link between synaesthesia and autism but the nature of that link remains poorly characterised. The present study considers whether atypical sensory sensitivity may be a common link between the conditions. Sensory hypersensitivity (aversion to certain sounds, touch, etc., or increased ability to make sensory discriminations) and/or hyposensitivity (desire to stimulate the senses , or a reduced response to sensory stimuli are a recently introduced diagnostic feature of autism spectrum conditions (ASC). Synaesthesia is defined by unusual sensory experiences and has also been linked to a typical cortical hyper-excitability. The Glasgow Sensory Questionnaire (GSQ) was administered to synaesthetes and people with ASC. Both groups reported increased sensory sensitivity relative to controls with a large effect size. Both groups also reported a similar pattern of both increased hyper- and hypo-sensitivities across multiple senses. The AQ (Autism-Spectrum Quotient) scores were elevated in the synaesthetes, and one subscale of this measure (attention to detail) placed synaesthetes within the autistic range. A standard laboratory test of visual stress (the Pattern Glare Test), administered online, corroborated the findings of increased sensitivity to aversive visual stimuli in synaesthetes. We conclude that atypical sensory sensitivity is an important shared feature between autism and synaesthesia. PMID:28266503

  4. Atypical PKC-iota Controls Stem Cell Expansion via Regulation of the Notch Pathway

    Directory of Open Access Journals (Sweden)

    In Kyoung Mah


    Full Text Available The number of stem/progenitor cells available can profoundly impact tissue homeostasis and the response to injury or disease. Here, we propose that an atypical PKC, Prkci, is a key player in regulating the switch from an expansion to a differentiation/maintenance phase via regulation of Notch, thus linking the polarity pathway with the control of stem cell self-renewal. Prkci is known to influence symmetric cell division in invertebrates; however a definitive role in mammals has not yet emerged. Using a genetic approach, we find that loss of Prkci results in a marked increase in the number of various stem/progenitor cells. The mechanism used likely involves inactivation and symmetric localization of NUMB, leading to the activation of NOTCH1 and its downstream effectors. Inhibition of atypical PKCs may be useful for boosting the production of pluripotent stem cells, multipotent stem cells, or possibly even primordial germ cells by promoting the stem cell/progenitor fate.

  5. Zoonotic atypical pneumonia due to Chlamydophila psittaci: first reported psittacosis case in Taiwan. (United States)

    Cheng, Yu-Jen; Lin, Kun-Yen; Chen, Chun-Chen; Huang, Yen-Lin; Liu, Chun-Eng; Li, Shu-Ying


    Human psittacosis caused by Chlamydophila psittaci is one of the most common zoonotic atypical pneumonias featuring pulmonary as well as extrapulmonary infections. Most of the cases involve avian contact history especially with psittacine birds. Herein we report a 44-year-old male patient displaying atypical pneumonia symptoms of intermittent fever, dry cough, chest pain, dyspnea, headache, hepatitis, and hyponatremia. He had two sick cockatiels, one of which had died a month previously. A microimmunofluorescence test was performed to check the serum antibody levels against Chlamydophila psittaci. The serum IgM titer showed positive titer of 1:256, 1:256, and 1:128 on Days 11, 23, and 43 after disease onset, respectively. His fever subsided soon and clinical symptoms improved after minocycline was administrated on Day 12. The psittacosis case was confirmed by history of psittacine bird contact, clinical symptoms, treatment response, and positive IgM titer. To our knowledge, this is the first report of a psittacosis case in Taiwan.

  6. Atypical expressions of jealousy in infants of intrusive- and withdrawn-depressed mothers. (United States)

    Hart, Sybil; Jones, Nancy Aaron; Field, Tiffany


    To determine whether atypical jealousy responses in infants of depressed mothers are differentiated by maternal nonoptimal interactive profiles, 12-month-old infants of intrusive-depressed and withdrawn-depressed mothers were observed with their mothers and a stranger in two contexts. In the first, infants were fully ignored in a less stressful context in which the adults focused on a picture book. In the second, infants were fully ignored in a more stressful, jealousy-inducement context, in which the adults attended to an infant-like doll. Cross-context comparisons revealed that the jealousy-inducement condition was associated with infants of intrusive-depressed mothers demonstrating greater play, and lesser proximal and distal behaviors toward their mothers. In contrast, the jealousy-inducement condition was associated with infants of withdrawn-depressed mothers directing greater proximal contacts toward the stranger. This investigation identified that as early as infancy, dysregulated expressions of jealousy are differentiated by depressed mothers' nonoptimal interactive patterns. Findings of this preliminary study call for investigative attention to this uncharted area of inquiry into mental health, and specifically, works addressing the importance of maternal characteristics to the unfolding of normative and atypical jealousy in infancy.

  7. Atypical form of cat scratch disease in immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Kojić Miroslav


    Full Text Available Introduction. Cat scratch disease (CSD is an acute infectious disease with benign course caused by the bacteria Bartonella henselae. Clinically, it is usually manifested as regional lymphadenopathy and mild infective syndrome. Rare forms of the disease which usually occur in immunocompromised presons are: encephalitis, transverse myelitis, neuroretinitis, granulomatosus conjunctivitis, arthritis, hepatitis etc. Case report. We presented an atypical form of cat scratch disease in a young immunocompetent female person. The disease was manifested with prolonged fever, rash, purulent lymphadenitis and hepatitis. The diagnosis was based on characteristic patohystological finding and exclusion of the other causes of lymphadenopathy. The patient was treated by antibiotics for a few weeks, with surgical incision and drainage of the purulent lymphadenitis. Conclusion. Atypical forms of CSD could be an important differential-diagnostic problem, especially if there is no opportunity for serological confirmation of the disease.

  8. Multiplicity of A-type and related stars

    CERN Document Server

    North, Pierre L


    The origin of chemically peculiar stars remains enigmatic, especially regarding their frequency among their "normal" peers. In addition to magnetic fields and rotation, multiplicity may shed light on the question. We mention the main surveys of the three kinds performed so far of intermediate mass stars, either normal or chemically peculiar, magnetic or not: imaging, spectroscopic, and photometric. We also consider the mulitiplicity of red giant stars, since many of them are descendants of A-type stars, through Mermilliod's radial velocity monitoring of open cluster members. We briefly review the orbital properties of binary systems hosting chemically peculiar stars. Some specific objects of special interest are mentioned as deserving further study. Finally, we recall that some binary systems composed of A-type stars are progenitors of Type Ia supernovae, and evoke the potentialities of future surveys like Gaia.

  9. Nightmare-Induced Atypical Midventricular Tako-Tsubo Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Veronica Fibbi


    Full Text Available Tako-Tsubo cardiomyopathy (TTC is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis.

  10. Nightmare-induced atypical midventricular tako-tsubo cardiomyopathy. (United States)

    Fibbi, Veronica; Ballo, Piercarlo; Nannini, Marco; Consoli, Lorenzo; Chechi, Tania; Bribani, Andrea; Fiorentino, Francesca; Chiodi, Leandro; Zuppiroli, Alfredo


    Tako-Tsubo cardiomyopathy (TTC) is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare) in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis.

  11. Not only limbs in atypical restless legs syndrome. (United States)

    Turrini, Alessandra; Raggi, Alberto; Calandra-Buonaura, Giovanna; Martinelli, Paolo; Ferri, Raffaele; Provini, Federica


    Restless legs syndrome (RLS) typically affects the limbs, but the involvement of other body parts has also been reported. In this essay, we critically review all literature reports of atypical RLS cases with unusual localizations. Applying the updated diagnostic criteria of the International restless legs syndrome study group (IRLSSG), which also consider symptoms localized outside of the lower limbs, a few of these atypical cases reported in the previous literature resulted in a definitive diagnosis of RLS. We also discuss the relationship between RLS and burning mouth syndrome (BMS) or restless genital syndrome (RGS). We conclude clinical sleep specialists should be aware of unusual RLS localizations because they respond to the usual treatment for RLS. All the IRLSSG diagnostic criteria should be applied in every suspected case, in order to establish a correct diagnosis of this disabling but treatable condition. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. Learning, plasticity, and atypical generalization in children with autism. (United States)

    Church, Barbara A; Rice, Courtney L; Dovgopoly, Alexander; Lopata, Christopher J; Thomeer, Marcus L; Nelson, Andrew; Mercado, Eduardo


    Individuals with autism spectrum disorder (ASD) show accelerated learning in some tasks, degraded learning in others, and distinct deficits when generalizing to novel situations. Recent simulations with connectionist models suggest that deficits in cortical plasticity mechanisms can account for atypical patterns of generalization shown by some children with ASD. We tested the surprising theoretical prediction, from past simulations, that the children with ASD who show atypical generalization in perceptual categorization tasks will benefit more from training with a single prototypical member of the category than from training with multiple examples, but children with ASD who generalize normally will be comparatively harmed. The experimental results confirmed this prediction, suggesting that plasticity deficits may well underlie the difficulties that some children with ASD have generalizing skills, and these deficits are not specific to the acquisition of social skills, but rather reflect a more general perceptual learning deficit that may impact many abilities.

  13. Identification of the first case of atypical scrapie in Japan (United States)

    IMAMURA, Morikazu; MIYAZAWA, Kohtaro; IWAMARU, Yoshifumi; MATSUURA, Yuichi; YOKOYAMA, Takashi; OKADA, Hiroyuki


    A Corriedale ewe was confirmed as the first atypical scrapie case during an active surveillance program for transmissible spongiform encephalopathies in small ruminants in Japan. The animal was homozygous for the AF141RQ haplotype of PRNP. The animal showed clinical neurological signs possibly due to listeriosis before culling. Western blot analysis showed an unusual multiple banded pattern with a low-molecular fragment at ~7 kDa. Histopathology revealed suppurative meningoencephalitis caused by listeriosis in the brainstem. Fine granular to globular immunostaining of disease-associated prion proteins was mainly detected in the neuropil of the spinal tract of the trigeminal nerve and in the white matter of the spinocerebellar tract. Based on these results, this case was conclusively diagnosed as atypical scrapie with encephalitic listeriosis. PMID:27616556

  14. [Treatment of tics in Tourette syndrome with atypical antipsychotic drugs]. (United States)

    Sindø, Ingrid; Jørgensen, Jan Ib


    We reviewed articles in English dealing with research into the effect of atypical antipsychotic drugs on tic reduction in Tourette's syndrome. In Denmark, there are four registered atypical antipsychotic drugs; clozapine, sulpiride, olanzapine, and risperidone. The topic of interest is the effectiveness and side effects of these drugs as compared to the conventional antipsychotic, pimozide, which is today the preferred pharmacological treatment of Tourette's syndrome among the antipsychotics. The conclusion is that risperidone would be a good first-line antipsychotic drug for the treatment of Tourette's syndrome. It is as effective as pimozide, its side effect profile is overall much more favourable, and unlike pimozide it does not contain the risk of causing heart arrhythmia.

  15. Magnetic field in atypical prominence structures: Bubble, tornado and eruption

    CERN Document Server

    Levens, P J; Ariste, A López; Labrosse, N; Dalmasse, K; Gelly, B


    Spectropolarimetric observations of prominences have been obtained with the THEMIS telescope during four years of coordinated campaigns. Our aim is now to understand the conditions of the cool plasma and magnetism in `atypical' prominences, namely when the measured inclination of the magnetic field departs, to some extent, from the predominantly horizontal field found in `typical' prominences. What is the role of the magnetic field in these prominence types? Are plasma dynamics more important in these cases than the magnetic support? We focus our study on three types of `atypical' prominences (tornadoes, bubbles and jet-like prominence eruptions) that have all been observed by THEMIS in the He I D_3 line, from which the Stokes parameters can be derived. The magnetic field strength, inclination and azimuth in each pixel are obtained by using the Principal Component Analysis inversion method on a model of single scattering in the presence of the Hanle effect. The magnetic field in tornadoes is found to be more ...

  16. Diagnosis and management of typical and atypical lung carcinoids. (United States)

    Pusceddu, Sara; Lo Russo, Giuseppe; Macerelli, Marianna; Proto, Claudia; Vitali, Milena; Signorelli, Diego; Ganzinelli, Monica; Scanagatta, Paolo; Duranti, Leonardo; Trama, Annalisa; Buzzoni, Roberto; Pelosi, Giuseppe; Pastorino, Ugo; de Braud, Filippo; Garassino, Marina Chiara


    An estimated 20% to 30% of all neuroendocrine tumours originate in the bronchial tree and lungs. According to the 2015 World Health Organization categorization, these tumours are separated into four subtypes characterized by increasing biological aggressiveness: typical carcinoid, atypical carcinoid, large-cell neuroendocrine carcinoma and small-cell carcinoma. Although typical and atypical lung carcinoids account for less than 1-5% of all pulmonary malignancies, the incidence of these neoplasms has risen significantly in recent decades. Surgery is the treatment of choice for loco-regional disease but for advanced lung carcinoids there is no recognized standard of care and successful management requires a multidisciplinary approach. The aim of this review is to provide a useful guide for the clinical management of lung carcinoids.

  17. Acute Zonal Occult Outer Retinopathy with Atypical Findings

    Directory of Open Access Journals (Sweden)

    Dimitrios Karagiannis


    Full Text Available Background. To report a case of acute zonal occult outer retinopathy (AZOOR with atypical electrophysiology findings. Case Presentation. A 23-year-old-female presented with visual acuity deterioration in her right eye accompanied by photopsia bilaterally. Corrected distance visual acuity at presentation was 20/50 in the right eye and 20/20 in the left eye. Fundus examination was unremarkable. Visual field (VF testing revealed a large scotoma. Pattern and full-field electroretinograms (PERG and ERG revealed macular involvement associated with generalized retinal dysfunction. Electrooculogram (EOG light rise and the Arden ratio were within normal limits bilaterally. The patient was diagnosed with AZOOR due to clinical findings, visual field defect, and ERG findings. Conclusion. This is a case of AZOOR with characteristic VF defects and clinical symptoms presenting with atypical EOG findings.

  18. Atypical epithelial hyperplasia of the breast: state of the art. (United States)

    Dion, Ludivine; Racin, Adelaïde; Brousse, Susie; Beltjens, Françoise; Cauchois, Aurélie; Levêque, Jean; Coutant, Charles; Lavoué, Vincent


    Atypical epithelial hyperplasia (AEH) of the breast is considered benign histological lesions with breast cancer risk. This review focuses on clinical signification and management of AEH that remains controversial. A review of published studies was performed using medline database. In this review, we fully describe the current evidence available. In particular, we describe 1) data from immunohistochemistry and molecular studies that suggest AEH is a precursor of breast cancer; 2) epidemiological studies demonstrate low rate of breast cancer in women with AEH; 3) surgical excision is necessary after diagnosis of AEH, such as lobular carcinoma in situ or atypical ductal hyperplasia, on core needle biopsy; 4) although current recommendations are evolving to fewer (if not no) excisions for flat epithelial with atypia and classic lobular neoplasia found on percutaneous biopsy (without radiologic indications for excision). Expert commentary: HEA management steel need prospective evidences, but recent retrospective data give some clue for less invasive management for some of HEA.

  19. Urbanization, Ikization, and Replacement Dynamics

    CERN Document Server

    Chen, Yanguang


    The phenomenon of Iks was first found by anthropologists and biologists, but it is actually a problem of human geography. However, it has not yet drawn extensive attention of geographers. In this paper, a hypothesis of ikization is presented that sudden and violent change of geographical environments results in dismantling of traditional culture, which then result in collective depravity of a nationality. By quantitative analysis and mathematical modeling, the causality between urbanization and ikization is discussed, and the theory of replacement dynamics is employed to interpret the process of ikization. Urbanization is in essence a nonlinear process of population replacement. Urbanization may result in ikization because that the migration of population from rural regions to urban regions always give rise to abrupt changes of geographical environments and traditional culture. It is necessary to protect the geographical environment against disruption, and to inherit and develop traditional culture in order t...


    Tabata, Minoru


    Minimally invasive aortic valve replacement (MIAVR) is defined as aortic valve replacement avoiding full sternotomy. Common approaches include a partial sternotomy right thoracotomy, and a parasternal approach. MIAVR has been shown to have advantages over conventional AVR such as shorter length of stay and smaller amount of blood transfusion and better cosmesis. However, it is also known to have disadvantages such as longer cardiopulmonary bypass and aortic cross-clamp times and potential complications related to peripheral cannulation. Appropriate patient selection is very important. Since the procedure is more complex than conventional AVR, more intensive teamwork in the operating room is essential. Additionally, a team approach during postoperative management is critical to maximize the benefits of MIAVR.

  1. Atypical Pelvic Crescent Fracture Caused by Vertical Shear Force



    The crescent fracture consists of a posterior iliac wing fracture with extension into the sacroiliac joint and a dislocation of the sacroiliac joint. This fracture represents a subset of lateral compression injury. The strong posterior ligaments of sacroiliac joint remain intact and a fracture fragment (crescent shape) involving the posterior superior iliac spines remains firmly attached to the sacrum. We report a patient with atypical pelvic crescent fracture that is mainly influenced by ver...

  2. Idiopathic Atypical Haemolytic Uraemic Syndrome presenting with acute dystonia

    LENUS (Irish Health Repository)

    Maduemem, Rizwan K E


    Hemolytic Uremic Syndrome (HUS), a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The atypical HUS (aHUS) results from over activation of complement system with formation of micro thrombi and damage to endothelial cells resulting in renal impairment in 50 % and death in 25 %, commonly in untreated patients. We report an intriguing case of aHUS presenting with acute onset of movement disorder and fluctuating delirium.

  3. Epidemiological and genetical differences between classical and atypical scrapie cases


    Lühken, Gesine; Buschmann, Anne; Brandt, Horst; Eiden, Martin; Groschup, Martin; Erhardt, Georg


    International audience; The aim of this study was to analyze the epidemiology and prion protein (PrP) genetics in scrapie-affected sheep flocks in Germany. For this purpose, 224 German scrapie cases in sheep diagnosed between January 2002 and February 2006 were classified as classical or atypical scrapie and the amino acids at codons 136, 141, 154 and 171 were determined. Likewise, representative numbers of flock mates were genotyped. Significant epidemiological differences were observed betw...

  4. Ureterolithiasis: classical and atypical findings on unenhanced helical computed tomography. (United States)

    Vaswani, Kuldeep K; El-Dieb, Adam; Vitellas, Kenneth M; Bennett, William F; Bova, James G


    Evaluation of patients with acute flank pain using helical computed tomography (CT) is a well-accepted, rapid, and safe procedure in the emergency setting. Various primary and secondary signs are described in the literature for evaluation of these patients. Our purpose is to demonstrate both the classical findings associated with ureteral calculi on unenhanced helical CT and atypical findings and potential pitfalls. We also provide readers with a systematic approach to interpreting unenhanced helical CT scans performed for acute flank pain.

  5. Atypical cytochrome p450 kinetics: implications for drug discovery. (United States)

    Tracy, Timothy S


    The Michaelis-Menten model is commonly used to estimate a drug's potential in vivo hepatic clearance based on in vitro data obtained during drug discovery and development. This paradigm assumes that the drug obeys 'typical' enzyme kinetics and thus can be described by this model. However, it is increasingly being recognised that a number of drugs metabolised not only by the cytochrome P450 enzymes but also by other enzymes and transporters can exhibit atypical kinetic profiles, and thus are not accurately modeled with the Michaelis-Menten model. Application of an incorrect model can then lead to mis-estimation of in vitro intrinsic clearance and thus affect the prediction of in vivo clearance. This review discusses several types of atypical kinetic profiles that may be observed, including examples of homotropic cooperativity (i.e. sigmoidal kinetics, biphasic kinetics and substrate inhibition kinetics) as well as heterotropic cooperativity (i.e. activation). Application of the incorrect kinetic model may profoundly affect estimations of intrinsic clearance. For example, incorrectly applying the Michaelis-Menten model to a kinetic profile exhibiting substrate inhibition kinetics will result in an underestimation of Km (Michaelis-Menten constant) and V(max) (maximal velocity), whereas application of the Michaelis-Menten model to sigmoidal kinetic data typically results in an overestimation of Km and V(max) at the lower substrate concentrations that are typically therapeutically relevant. One must also be careful of potential artefactual causes of atypical kinetic profiles, such as enzyme activation by solvents, buffer dependent kinetic profiles, or altered kinetic parameter estimates due to nonspecific binding of the substrate to proteins. Despite a plethora of data on the effects of atypical kinetic profiles in vitro, only modest effects have been noted in vivo (with the exception of substrate dependent inhibition). Thus, the clinical relevance of these phenomena

  6. Childhood atypical meningioma with perineural spread: MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Wei, Feng-Yu.; Wong, Alex Mun-Ching; Wong, Ho-Fai; Ng, Shu-Hang [Chang Gung Memorial Hospital, Department of Diagnostic Radiology, Kwei-Shan, Tao-Yuan (Taiwan); Wu, Chieh-Tsai [Chang Gung Memorial Hospital, Department of Neurosurgery, Kwei-Shan, Tao-Yuan (Taiwan); Lin, Kuang-Lin [Chang Gung Memorial Hospital, Division of Pediatric Neurology, Kwei-Shan, Tao-Yuan (Taiwan)


    Meningiomas are uncommon in children. When they occur, they are frequently associated with neurofibromatosis type 2. Childhood meningiomas are generally large and commonly associated with cyst formation and an unusual location. Perineural tumor spread, occasionally associated with head and neck malignancies, is very rare in meningiomas. We present the MR findings of an atypical meningioma with perineural spread in a 4.5-year-old girl. (orig.)

  7. Atypical Chronic Myeloid Leukaemia with Trisomy 13: a Case Report

    Institute of Scientific and Technical Information of China (English)

    Guo-yu Hu; Chao-hui Yuan; Kui Tan; Zhen-zhen Chen


    ATYPICAL chronic myeloid leukaemia (aCML),which shows both myeloproliferative and myeIodysplastic features,is a type of myeloproliferative/myelodysplastic disease as defined by the World Health Organisation (WHO) classification of the myeloid neoplasms.1 Because of the presence of neutrophilic leukocytosis,aCML may resemble chronic myeIogenous leukemia (CML).However,in contrast with CML,aCML does not have the Philadelphia chromosome or the bcr/abl fusion gene.

  8. Gamma Knife Radiosurgery for Atypical and Anaplastic Meningiomas. (United States)

    Wang, Wei-Hsin; Lee, Cheng-Chia; Yang, Huai-Che; Liu, Kang-Du; Wu, Hsiu-Mei; Shiau, Cheng-Ying; Guo, Wan-You; Pan, David Hung-Chi; Chung, Wen-Yuh; Chen, Ming-Teh


    Atypical and anaplastic meningiomas have much higher recurrence rates after surgical resection compared with benign meningiomas, but the role of adjuvant radiosurgery remains unclear. This study was undertaken to evaluate the outcomes of gamma knife radiosurgery for patients with atypical and anaplastic meningiomas. In this retrospective analysis of a prospectively maintained database, 46 patients with histologically proven atypical or anaplastic meningiomas by current World Health Organization (WHO) criteria underwent postoperative Gamma Knife radiosurgery between 1993 and 2013. The median follow-up period was 32.6 months. The median tumor volume and margin dose were 11.7 mL (range, 2-53 mL) and 13.1 Gy (range, 12.0-16.5 Gy), respectively. Local control at 3 and 5 years was 50.6% and 32.1%, respectively. Gender (P = 0.013) and marginal dose less than or equal to 13 Gy (P = 0.049) were associated with the local control. The 3- and 5-year overall survival for patients with WHO grade II was 97.1% and 88.3%, respectively, compared with 66.7% and 66.7% for patients with WHO grade III meningiomas. Radiation therapy before Gamma Knife radiosurgery (GKRS; P = 0.018) and tumor grade (P = 0.019) were the factors associated with a worse overall survival rate. Fourteen patients (30.4%) developed adverse radiation effects after GKRS treatment, and all were Radiation Therapy Oncology Group grade I. Postoperative GKRS treatment for patients with atypical and anaplastic meningioma is challenging. More aggressive treatment, including of safely maximizing the extent of surgical resection and using a higher margin dose (>13Gy), should be applied to achieve better local control. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Current Evidence on Atypical Odontalgia: Diagnosis and Clinical Management


    Yoshihiro Abiko; Hirofumi Matsuoka; Itsuo Chiba; Akira Toyofuku


    Patients with atypical odontalgia (AO) complain of medically unexplained toothache. No evidence-based diagnostic criteria or treatment guidelines are yet available. The present paper addresses seven clinical questions about AO based on current knowledge in the literature and discusses diagnostic criteria and guidelines for treatment and management. The questions are (i) What is the prevalence of AO in the community? (ii) What psychological problems are experienced by patients with AO? (iii) A...

  10. Monocular nasal hemianopia from atypical sphenoid wing meningioma. (United States)

    Stacy, Rebecca C; Jakobiec, Frederick A; Lessell, Simmons; Cestari, Dean M


    Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.

  11. Three cases of atypical pneumonia caused by Chlamydophila psittaci. (United States)

    Chau, S; Tso, Eugene Y K; Leung, W S; Fung, Kitty S C


    Psittacosis is a zoonotic disease caused by Chlamydophila psittaci. The most common presentation is atypical pneumonia. Three cases of pneumonia of varying severity due to psittacosis are described. All patients had a history of avian contact. The diagnosis was confirmed by molecular detection of Chlamydophila psittaci in respiratory specimens. The cases showed good recovery with doxycycline treatment. Increased awareness of psittacosis can shorten diagnostic delay and improve patient outcomes.

  12. Atypical lymphohistiocytic infiltrate (pseudolymphoma) of the oral cavity. (United States)

    Kabani, S; Cataldo, E; Folkerth, R; Delellis, R A; Bhan, I; Farren, P; Neville, T


    In the oral cavity, differentiation between reactive and neoplastic lymphoproliferative lesions can, at times, be very difficult. We report an unusual case in which immunohistochemical findings were necessary to determine that the lesion was reactive despite the original interpretation of malignant lymphoma. The relationship of this lesion to atypical histiocytic granuloma, angiolymphoid hyperplasia with eosinophilia, and traumatic ulcerative granuloma with stromal eosinophilia is discussed and possible pathogenetic mechanisms are proposed. The value of immunohistochemistry in the diagnosis of extranodal lymphoproliferative lesions is emphasized.


    Singh, Gurmeet; Sachdev, J. S.


    SUMMARY Twenty-two cases who fulfilled the criteria of having atypical manifestation at any stage of illness and had minimum follow up of three years were studied in detail. Their family history and follow up was analysed. The findings of the present study suggest that the cases showing admixture of schizophrenic and affective symptoms are probably a variant of affective disorders although a possibility of their being a third independent psychosis cannot be ruled out. PMID:22065727

  14. Atypical presentation of Boerhaave's syndrome as Enterococcal bacterial pericardial effusion. (United States)

    Saha, Arin; Jarvis, Martin; Thorpe, James A C; O'Regan, David J


    Boerhaave's perforation is a serious condition describing spontaneous transmural perforation of the oesophagus. The classical presentation of this condition is vomiting, lower thoracic pain and subcutaneous emphysema. However, the condition often presents atypically and it is important to reach the correct diagnosis quickly. We present the case of a 54-year-old woman with a Boerhaave's perforation that presented as Enterococcal bacterial pericardial effusion.

  15. Mitochondrial Replacement: Ethics And Identity


    Wrigley, Anthony; Wilkinson, Stephen; Appleby, John B


    Mitochondrial replacement techniques (MRTs) have the potential to allow prospective parents who are at risk of passing on debilitating or even life-threatening mitochondrial disorders to have healthy children to whom they are genetically related. Ethical concerns have however been raised about these techniques. This article focuses on one aspect of the ethical debate, the question of whether there is any moral difference between the two types of MRT proposed: Pronuclear Transfer (PNT) and Mat...

  16. Replacing magnets at the LHC

    CERN Multimedia

    LHC, LSI2, Point 4


    CERN engineers have been working through the night this week to move the final replacement dipole magnets into position on the Large Hadron Collider (LHC). Though there are several still to go, the teams expect to have completed the task by the end of this month. Dipole magnets bend the paths of particles as they travel around the circular accelerator. Of the LHC's 1232 dipoles – each 15 metres long and weighing 35 tonnes – 15 are being replaced as part of the long shutdown of CERN's accelerator complex. These 15 magnets suffered wear and tear during the LHC's first 4-year run. Three quadrupole-magnet assemblies – which help to focus particles into a tight beam – have also been replaced. Moving such heavy magnets requires specially adapted cranes and trailers both above and below ground. There are several access points on the LHC. Some, such as the 100-metre vertical access shaft down to the ALICE experiment, are equipped with lifts to allow technical personnel and visitors down to the caverns. Other ...

  17. Results of Austin Moore replacement.

    Directory of Open Access Journals (Sweden)

    Jadhav A


    Full Text Available Forty cases of Austin Moore Replacement done for transcervical fractures of the femur in patients were reviewed after a period of 12 to 48 months postoperatively (mean 26 mth. 30 cases (75% had mild to severe pain of non-infective origin, starting as early as 6 months postoperatively. This was irrespective of the make, size or position (varus/valgus of the prosthesis. Though the Aufranc and Sweet clinical scoring was satisfactory in 65% cases, radiological evidence of complications like sinking, protrusion, etc. were seen in majority of the cases. Calcar resorption was seen in 34 cases (85% as early as 4 months postoperatively. Results of THR and bipolar replacement done for transcervical fractures in recent literature show 85% pain-free cases at 5 years. We feel that Austin Moore Replacement should be reserved for patients more than 65 years of age and those who are less active or debilitated because of other factors, because of increased acetabular wear with time in the younger individual. This is corroborated by unsatisfactory results in patients less than 65 years of age (p < 0.05.

  18. The caudal septum replacement graft. (United States)

    Foda, Hossam M T


    To describe a technique for reconstructing the lost tip support in cases involving caudal septal and premaxillary deficiencies. The study included 120 patients with aesthetic and functional nasal problems resulting from the loss of caudal septal and premaxillary support. An external rhinoplasty approach was performed to reconstruct the lost support using a cartilaginous caudal septum replacement graft and premaxillary augmentation with Mersilene mesh. The majority of cases (75%) involved revisions in patients who had previously undergone 1 or more nasal surgical procedures. A caudal septum replacement graft was combined with premaxillary augmentation in 93 patients (77.5%). The mean follow-up period was 3 years (range, 1-12 years). The technique succeeded in correcting the external nasal deformities in all patients and resulted in a significant improvement in breathing in 74 patients (86%) with preoperative nasal obstruction. There were no cases of infection, displacement, or extrusion. The caudal septum replacement graft proved to be very effective in restoring the lost tip support in patients with caudal septal deficiency. Combining the graft with premaxillary augmentation using Mersilene mesh helped increase support and stability over long-term follow-up.

  19. An inherited LMNA gene mutation in atypical Progeria syndrome. (United States)

    Doubaj, Yassamine; De Sandre-Giovannoli, Annachiara; Vera, Esteves-Vieira; Navarro, Claire Laure; Elalaoui, Siham Chafai; Tajir, Mariam; Lévy, Nicolas; Sefiani, Abdelaziz


    Hutchinson-Gilford Progeria syndrome (HGPS) is a rare genetic disorder, characterized by several clinical features that begin in early childhood, recalling an accelerated aging process. The diagnosis of HGPS is based on the recognition of common clinical features and detection of the recurrent heterozygous c.1824C>T (p.Gly608Gly) mutation within exon 11 in the Lamin A/C encoding gene (LMNA). Besides "typical HGPS," several "atypical progeria" syndromes (APS) have been described, in a clinical spectrum ranging from mandibuloacral dysplasia to atypical Werner syndrome. These patients's clinical features include progeroid manifestations, such as short stature, prominent nose, premature graying of hair, partial alopecia, skin atrophy, lipodystrophy, skeletal anomalies, such as mandibular hypoplasia and acroosteolyses, and in some cases severe atherosclerosis with metabolic complications. APS are due in several cases to de novo heterozygous LMNA mutations other than the p.Gly608Gly, or due to homozygous BAFN1 mutations in Nestor-Guillermo Progeria syndrome (NGPS). We report here and discuss the observation of a non-consanguineous Moroccan patient presenting with atypical progeria. The molecular studies showed the heterozygous mutation c.412G>A (p.Glu138Lys) of the LMNA gene. This mutation, previously reported as a de novo mutation, was inherited from the apparently healthy father who showed a somatic cell mosaicism.

  20. Face processing in Williams syndrome is already atypical in infancy

    Directory of Open Access Journals (Sweden)

    Dean eD'Souza


    Full Text Available Face processing is a crucial socio-cognitive ability. Is it acquired progressively or does it constitute an innately-specified, face-processing module? The latter would be supported if some individuals with seriously impaired intelligence nonetheless showed intact face-processing abilities. Some theorists claim that Williams syndrome (WS provides such evidence since, despite IQs in the 50s, adolescents/adults with WS score in the normal range on standardised face-processing tests. Others argue that atypical neural and cognitive processes underlie WS face-processing proficiencies. But what about infants with WS? Do they start with typical face-processing abilities, with atypicality developing later, or are atypicalities already evident in infancy? We used an infant familiarisation/novelty design and compared infants with WS to healthy controls as well as to a group of infants with DS matched on both mental and chronological age. Participants were familiarised with a schematic face, after which they saw a novel face in which either the features (eye shape were changed or just the configuration of the original features. Configural changes were processed successfully by controls, but not by infants with WS who were only sensitive to featural changes and who showed syndrome-specific profiles different from infants with the other neurodevelopmental disorder. Our findings indicate that theorists can no longer use the case of Williams syndrome to support claims that evolution has endowed the human brain with an independent face-processing module.

  1. Atypical (symplastic) leiomyoma of the uterus--a case report. (United States)

    Siti-Aishah, M A; Noriah, O; Malini, M N; Zainul-Rashid, M R; Das, S


    A 30-year-old, nulliparous woman presented with a history of subfertility. On examination she was found to have uterine fibroid of 28 weeks size of gravid uterus and subsequently laporatomy myomectomy was performed. Multilobulated masses, with diameters ranging from 22 mm to 160 mm were found. Cut sections of the lobulated masses showed whitish whorled cut surface. One of the multilobulated masses had a cystic cavity, measuring 60x50x35 mm(3). Light microscopic findings of the mass with the cystic cavity showed a well-circumscribed cellular tumour composed of cells exhibiting moderate nuclear atypia which were enlarged, nuclei with prominent chromatin clumping and were distributed in areas. Some tumour cells showed large nuclear pseudoinclusions, multinucleated or multilobated tumour giant cells, smudging and few enlarged nucleoli. Mitotic activity was 4 MFs per 10 HPFs. Occasional cells with intracytoplasmic inclusions resembling rhabdoid - like features were seen. There were no atypical mitoses or tumour necroses were noted. Diagnosis of atypical leiomyoma or symplastic leiomyoma was made. Atypical or symplastic leiomyomas are rare in the region of Malaysia and the present case discusses its incidence in younger age, its morphological features along with diagnosis and clinical outcome.

  2. Extracutaneous atypical syphilis in HIV-infected patients. (United States)

    Prieto, Paula; Imaz, Arkaitz; Calatayud, Laura; García, Olga; Saumoy, María; Podzamczer, Daniel


    We describe a series of cases of syphilis with atypical extracutaneous clinical presentation diagnosed in HIV-infected patients. Retrospective observational study. All cases of syphilis diagnosed in HIV-infected patients during the period between June 2013 and June 2016 in a tertiary hospital of the Barcelona metropolitan area were analysed. A total of 71 cases of syphilis were diagnosed, 32 of them presenting with clinical signs or symptoms. Seven of these cases (9.8% of the total and 21.8% of the symptomatic cases) had atypical presentations with extracutaneous involvement: ocular (4), gastric (1), multiple hepatic abscesses (1) and generalised adenopathies (1). Patients were treated with intramuscular or intravenous penicillin and the clinical and serological evolution was good in all of them. Extracutaneous atypical clinical presentations were observed in 21.8% of symptomatic cases of syphilis in HIV+ patients with ocular involvement being the most freqent. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  3. Atypical centromeres in plants – what they can tell us

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    Maria eCuacos


    Full Text Available The centromere, visible as the primary constriction of condensed metaphase chromosomes, is a defined chromosomal locus essential for genome stability. It mediates transient assembly of a multi-protein complex, the kinetochore, which enables interaction with spindle fibers and thus faithful segregation of the genetic information during nuclear divisions. Centromeric DNA varies in extent and sequence composition among organisms, but a common feature of almost all active eukaryotic centromeres is the presence of the centromeric histone H3 variant cenH3 (a.k.a. CENP-A.These typical centromere features apply to most studied species. However, a number of species display atypical centromeres, such as holocentromeres (centromere extension along almost the entire chromatid length or neocentromeres (ectopic centromere activity.In this review, we provide an overview of different atypical centromere types found in plants including holocentromeres, de novo formed centromeres and terminal neocentromeres as well as di-, tri- and metapolycentromeres (more than one centromere per chromosomes. We discuss their specific and common features and compare them to centromere types found in other eukaryotic species. We also highlight new insights into centromere biology gained in plants with atypical centromeres such as distinct mechanisms to define a holocentromere, specific adaptations in species with holocentromeres during meiosis or various scenarios leading to neocentromere formation.

  4. Stiripentol in atypical absence seizures in children: an open trial. (United States)

    Farwell, J R; Anderson, G D; Kerr, B M; Tor, J A; Levy, R H


    Stiripentol (STP) was added to the antiepileptic drug (AED) regimen of 10 patients with uncontrolled atypical absence seizures (more than one seizure a day). Seven boys and three girls aged 6-16 years participated in the study. Concomitant AEDs included various combinations of phenobarbital (PB), phenytoin (PHT), carbamazepine (CBZ), and valproate (VPA). Parents counted daily seizures over a 4-week baseline period before institution of STP, and in a 20-week period during STP therapy. To compensate for drug interactions, doses of other AEDs were adjusted during STP administration to keep serum levels close to levels of the baseline period. Maintenance doses of STP were 1,000-3,000 mg/day, giving serum levels of 4-22 micrograms/mL. All patients experienced a decrease in atypical absence seizures. Average decrease was 70% (range 5-95%). Side effects experienced by some patients were dose related and included anorexia, nausea, vomiting, and lethargy. In only 1 patient did an adverse effect (vomiting) require discontinuation of STP. We conclude that STP shows promise in treatment of atypical absence seizures in children, and further trials are warranted.

  5. Atypical resource allocation may contribute to many aspects of autism

    Directory of Open Access Journals (Sweden)

    Emily J. Goldknopf


    Full Text Available Based on a review of the literature and on reports by people with autism, this paper suggests that atypical resource allocation is a factor that contributes to many aspects of autism spectrum conditions, including difficulties with language and social cognition, atypical sensory and attentional experiences, executive and motor challenges, and perceptual and conceptual strengths and weaknesses. Drawing upon resource theoretical approaches that suggest that perception, cognition, and action draw upon multiple pools of resources, the approach states that compared with resources in typical cognition, resources in autism are narrowed or reduced, especially in people with strong sensory symptoms. In narrowed attention, resources are restricted to smaller areas and to fewer modalities, stages of processing, and cognitive processes than in typical cognition; resources may be more intense than in typical cognition. In reduced attentional capacity, overall resources are reduced; resources may be restricted to fewer modalities, stages of processing, and cognitive processes than in typical cognition, or the amount of resources allocated to each area or process may be reduced. Possible neural bases of the hypothesized atypical resource allocation, relations to other approaches, limitations, and tests of the hypotheses are discussed.

  6. Atypical Findings of Guillain-Barré Syndrome in Children

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    Parvaneh KARIMZADEH


    Full Text Available ObjectiveGuillain-Barre syndrome (GBS is an immune-mediated polyneuropathy that occurs mostly after prior infection. The diagnosis of this syndrome is dependent heavily on the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually confirm the diagnosis. This is a retrospective study which was performed to investigate the atypical features of GBS.Materials & MethodsThirty three patients (21/63.6% males and 12/36.4% females with GBS were retrospectively studied and prospectively evaluated at the Child Neurology institute of Mofid Children Hospital of Shahid Beheshti University of Medical Sciences between May 2011 and September 2012.ResultsThe mean age was 5.4 years (range, 1.5-10.5.Twenty one patients (87.9 % had previous history of infections. Eight patients (24.2% admitted with atypical symptoms like upper limb weakness (3%, ptosis (3%, neck stiffness (3%, inability to stand (proximal weakness (9.1%, headache (3% and dysphagia (3%.According to disease process, weakness was ascending in 26 (78.8%, descending in 5 (15.2% and static in 2 (6.1% patients. Cranial nerve involvement was found in 8(24.3% children, most commonly as facial palsy in 3 (9.1%.ConclusionIn this study, 24.3% of our patients presented with atypical symptoms of GBS as upper limb weakness, ptosis, neck stiffness, inability to stand (proximal weakness, headache and dysphagia

  7. Tardive dyskinesia in children treated with atypical antipsychotic medications. (United States)

    Wonodi, Ikwunga; Reeves, Gloria; Carmichael, Dana; Verovsky, Ilene; Avila, Matthew T; Elliott, Amie; Hong, L Elliot; Adami, Helene M; Thaker, Gunvant K


    Recent years have witnessed increased antipsychotic treatment of children despite limited long-term safety data in children. In this study, motor side effects associated with the use of antipsychotic drugs in children were examined in a sample of pediatric psychiatric patients. Child and adolescent psychiatric patients receiving antipsychotics (most were on atypicals) for 6 months or longer (n = 118) were compared with antipsychotic-naïve patients (n = 80) with similar age, sex ratio, and diagnoses. Only 19% of patients on antipsychotics had ever experienced psychotic symptoms. Eleven children (9%) on antipsychotics exhibited dyskinesia, when compared with 0 in the naïve group (P = 0.003, Fisher's exact test). Nine of 62 African-American children (15%) on antipsychotics exhibited dyskinesia, when compared with only 4% (2 of 52) of European-American children (P = 0.003, Fisher's exact test). Children treated with antipsychotic drugs might experience a significant risk of dyskinesia even when treated only with atypical antipsychotics. Ethnicity might also be a risk factor for dyskinesia in children. Side-effect profile of the atypical antipsychotic drugs in children may be much different than that in adults.

  8. Increasing use of atypical antipsychotics and anticonvulsants during pregnancy. (United States)

    Epstein, Richard A; Bobo, William V; Shelton, Richard C; Arbogast, Patrick G; Morrow, James A; Wang, Wei; Chandrasekhar, Rameela; Cooper, William O


    To quantify maternal use of atypical antipsychotics, typical antipsychotics, anticonvulsants, and lithium during pregnancy. Tennessee birth and death records were linked to Tennessee Medicaid data to conduct a retrospective cohort study of 296,817 women enrolled in Tennessee Medicaid throughout pregnancy who had a live birth or fetal death from 1985 to 2005. During the study time period, the adjusted rate of use of any study medication during pregnancy increased from nearly 14 to 31 per 1000 pregnancies (β = 0.08, 95% CI = 0.07, 0.09). Significant increases were reported in use of anticonvulsants alone among mothers with pain and other psychiatric disorders, atypical antipsychotics alone among mothers with bipolar disorders, schizophrenia, unipolar depressive disorders, and other psychiatric disorders, and more than one studied medication for mothers with epilepsy, pain disorders, bipolar disorders, unipolar depressive disorders, and other psychiatric disorders. Significant decreases were reported in use of lithium alone and typical antipsychotics alone for all clinically meaningful diagnosis groups. There was a substantial increase in use of atypical antipsychotics alone, anticonvulsants alone, and medications from multiple studied categories among Tennessee Medicaid-insured pregnant women during the study period. Further examination of the maternal and fetal consequences of exposure to these medications during pregnancy is warranted. Copyright © 2012 John Wiley & Sons, Ltd.

  9. Paranoid personality masking an atypical case of frontotemporal dementia. (United States)

    Iroka, Nneka; Jehangir, Waqas; Ii, Jay Littlefield; Pattan, Vishwanath; Yousif, Abdalla; Mishra, Arunesh K


    Frontotemporal dementia (FTD) is a debilitating disease that is well described in the "Diagnostic and statistical manual of mental disorders, fifth edition (DSM-5)", and typically presents with memory impairment, progressive decline in cortical functioning, and behavioral changes. Age of onset is generally in the late fifties, and usually the first presentation involves a change in behavior and emotional blunting. Treatment of FTD involves management of any neurobehavioral symptoms while trials of atypical antipsychotics are ongoing but suggest some efficacy. We present a case of a patient who first presented with severe paranoid personality traits and frank persecutory delusions. This atypical presentation of our patient first led to her incorrect diagnosis of a psychotic disorder and paranoid personality disorder. As a result of this diagnosis, she was treated unsuccessfully. A subsequent magnetic resonance imaging (MRI) then showed atrophy of frontal and temporal lobes bilaterally (left more prominent than right) which confirmed the diagnosis of FTD. The importance of this case involves the atypical presentation of paranoia and delusions, and our patient's incorrect diagnosis based on her clinical presentation led to a trial of unsuccessful treatment. Only after performing an MRI, which showed atrophy, was the patient appropriately treated and deemed medically stable. This case report illustrates the importance of considering a rare presentation of frontotemporal lobe dementia with patients who are in the typical age range and present with paranoia and delusions.

  10. Genetic characterization of atypical Citrobacter freundii. (United States)

    Delgado, Gabriela; Souza, Valeria; Morales, Rosario; Cerritos, René; González-González, Andrea; Méndez, José Luis; Vázquez, Virginia; Cravioto, Alejandro


    The ability of a bacterial population to survive in different niches, as well as in stressful and rapidly changing environmental conditions, depends greatly on its genetic content. To survive such fluctuating conditions, bacteria have evolved different mechanisms to modulate phenotypic variations and related strategies to produce high levels of genetic diversity. Laboratories working in microbiological diagnosis have shown that Citrobacter freundii is very versatile in its colony morphology, as well as in its biochemical, antigenic and pathogenic behaviours. This phenotypic versatility has made C. freundii difficult to identify and it is frequently confused with both Salmonella enterica and Escherichia coli. In order to determine the genomic events and to explain the mechanisms involved in this plasticity, six C. freundii isolates were selected from a phenotypic variation study. An I-CeuI genomic cleavage map was created and eight housekeeping genes, including 16S rRNA, were sequenced. In general, the results showed a range of both phenotypes and genotypes among the isolates with some revealing a greater similarity to C. freundii and some to S. enterica, while others were identified as phenotypic and genotypic intermediary states between the two species. The occurrence of these events in natural populations may have important implications for genomic diversification in bacterial evolution, especially when considering bacterial species boundaries. In addition, such events may have a profound impact on medical science in terms of treatment, course and outcomes of infectious diseases, evading the immune response, and understanding host-pathogen interactions.


    Directory of Open Access Journals (Sweden)

    C. Anichini


    Full Text Available Atypical swallowing is the persistence of childlike deglutition at the end of dental eruption. One of the main causes is macroglossia, that is the abnormal enlargement of the tongue. The treatment is logopedic and/or surgical. Children with macroglossia have an increased incidence of respiratory diseases and infections, as well as malocclusions, articulatory defects and aesthetic damage. In this study we focused on two genetic syndromes with macroglossia: Beckwith-Wiedemann Syndrome (BWS and Down Syndrome (DS: 7 patients were evaluated for logopedic treatment: 3 are affected by BWS, 2 are affected by DS. In addition, 2 patients with isolated atypical swallowing were included in the study to emphasize problems connected with atypical swallowing. All the patients underwent a global examination and a personalized logopedic therapy scheme was planned. With the exception of one of them who was lost to follow up and who did not continue with the exercises the speech therapist had recommended, all the children showed good response and compliance with remarkable improvements, thus proving the importance of an early, constant and intensive logopedic treatment.

  12. Weeding atypical glandular cell look-alikes from the true atypical lesions in liquid-based Pap tests: a review. (United States)

    Wood, Moira D; Horst, Julie A; Bibbo, Marluce


    The purpose of this review is to identify features that separate atypical glandular cells (AGC) associated with glandular neoplasia from its mimickers, both benign and neoplastic. We reviewed cases of AGC diagnosed on liquid-based Pap tests (LBP) for which corresponding histological follow-up was available. A review of the literature for similar studies in LBP tests was also conducted. We find that certain benign mimics can be reliably separated from AGC, but recommend caution in attempting to increase specificity at the risk of losing sensitivity. Although accounting for only a small percentage of diagnoses AGC require a thorough clinical evaluation, including colposcopy. Most cases are ultimately found to be benign. When evaluating smears suspicious for AGC, it is important to examine the subtle features which make truly atypical cells discernible from their numerous benign mimickers.

  13. Evidence for Broadening Criteria for Atypical Depression Which May Define a Reactive Depressive Disorder. (United States)

    Silverstein, Brett; Angst, Jules


    Objective. Arguing that additional symptoms should be added to the criteria for atypical depression. Method. Published research articles on atypical depression are reviewed. Results. (1) The original studies upon which the criteria for atypical depression were based cited fatigue, insomnia, pain, and loss of weight as characteristic symptoms. (2) Several studies of DSM depressive criteria found patients with atypical depression to exhibit high levels of insomnia, fatigue, and loss of appetite/weight. (3) Several studies have found atypical depression to be comorbid with headaches, bulimia, and body image issues. (4) Most probands who report atypical depression meet criteria for "somatic depression," defined as depression associated with several of disordered eating, poor body image, headaches, fatigue, and insomnia. The gender difference in prevalence of atypical depression results from its overlap with somatic depression. Somatic depression is associated with psychosocial measures related to gender, linking it with the descriptions of atypical depression as "reactive" appearing in the studies upon which the original criteria for atypical depression were based. Conclusion. Insomnia, disordered eating, poor body image, and aches/pains should be added as criteria for atypical depression matching criteria for somatic depression defining a reactive depressive disorder possibly distinct from endogenous melancholic depression.

  14. Evidence for Broadening Criteria for Atypical Depression Which May Define a Reactive Depressive Disorder

    Directory of Open Access Journals (Sweden)

    Brett Silverstein


    Full Text Available Objective. Arguing that additional symptoms should be added to the criteria for atypical depression. Method. Published research articles on atypical depression are reviewed. Results. (1 The original studies upon which the criteria for atypical depression were based cited fatigue, insomnia, pain, and loss of weight as characteristic symptoms. (2 Several studies of DSM depressive criteria found patients with atypical depression to exhibit high levels of insomnia, fatigue, and loss of appetite/weight. (3 Several studies have found atypical depression to be comorbid with headaches, bulimia, and body image issues. (4 Most probands who report atypical depression meet criteria for “somatic depression,” defined as depression associated with several of disordered eating, poor body image, headaches, fatigue, and insomnia. The gender difference in prevalence of atypical depression results from its overlap with somatic depression. Somatic depression is associated with psychosocial measures related to gender, linking it with the descriptions of atypical depression as “reactive” appearing in the studies upon which the original criteria for atypical depression were based. Conclusion. Insomnia, disordered eating, poor body image, and aches/pains should be added as criteria for atypical depression matching criteria for somatic depression defining a reactive depressive disorder possibly distinct from endogenous melancholic depression.

  15. Fusion following failed total ankle replacement. (United States)

    Wünschel, Markus; Leichtle, Ulf G; Leichtle, Carmen I; Walter, Christian; Mittag, Falk; Arlt, Eva; Suckel, Andreas


    Although mid- to long-term results after total ankle replacement have improved because of available second- and third-generation devices, failure of total ankle replacement is still more common compared with total hip replacement and total knee replacement. The portfolio of available total ankle replacement revision component options is small. Furthermore, the bone stock of the tibiotalar region is scarce making it difficult and in some situations impossible to perform revision total ankle replacement. In these cases tibiotalar and tibiotalocalcaneal fusions are valuable options. This article describes which surgical procedures should be performed depending on the initial situation and gives detailed advice on surgical technique, postoperative care, and clinical results.

  16. Fine-tuning of defensive behaviors in the dorsal periaqueductal gray by atypical neurotransmitters

    Directory of Open Access Journals (Sweden)

    M.V. Fogaça


    Full Text Available This paper presents an up-to-date review of the evidence indicating that atypical neurotransmitters such as nitric oxide (NO and endocannabinoids (eCBs play an important role in the regulation of aversive responses in the periaqueductal gray (PAG. Among the results supporting this role, several studies have shown that inhibitors of neuronal NO synthase or cannabinoid receptor type 1 (CB1 receptor agonists cause clear anxiolytic responses when injected into this region. The nitrergic and eCB systems can regulate the activity of classical neurotransmitters such as glutamate and γ-aminobutyric acid (GABA that control PAG activity. We propose that they exert a ‘fine-tuning’ regulatory control of defensive responses in this area. This control, however, is probably complex, which may explain the usually bell-shaped dose-response curves observed with drugs that act on NO- or CB1-mediated neurotransmission. Even if the mechanisms responsible for this complex interaction are still poorly understood, they are beginning to be recognized. For example, activation of transient receptor potential vanilloid type-1 channel (TRPV1 receptors by anandamide seems to counteract the anxiolytic effects induced by CB1 receptor activation caused by this compound. Further studies, however, are needed to identify other mechanisms responsible for this fine-tuning effect.

  17. Testosterone replacement therapy in obese males. (United States)

    Drewa, Tomasz; Olszewska-Słonina, Dorota; Chlosta, Piotr


    Controversy surrounds testosterone replacement therapy in obese ageing due to no generally accepted lower limits of normal testosterone level and high prevalence of hypogonadal symptoms in the ageing male population and the non-specific nature of these symptoms. Late onset hypogonadism is a clinical and biochemical syndrome associated with advancing age, often coexisting with obesity and metabolic syndrome. High fat and carbohydrates (fructose) consumption is responsible for development of obesity and metabolic syndrome which is one of risk factors for hypogonadism in older men. High fructose intake has been shown to cause dyslipidemia and to impair hepatic insulin sensitivity. Obesity and lack of physical activity negatively influence testosterone level. Low testosterone level should be regarded as an effect of obesity, but reverse relationship has not been proved yet. The management of late-onset hypogonadism symptoms has to be treated by a change of a life style and prevented with healthy nutrition and physical activity. The question related to rational indications for testosterone replacement therapy in obese males seems to be still actual.

  18. Hypoparathyroidism: Replacement Therapy with Parathyroid Hormone

    Directory of Open Access Journals (Sweden)

    Lars Rejnmark


    Full Text Available Hypoparathyroidism (HypoPT is characterized by low serum calcium levels caused by an insufficient secretion of parathyroid hormone (PTH. Despite normalization of serum calcium levels by treatment with activated vitamin D analogues and calcium supplementation, patients are suffering from impaired quality of life (QoL and are at increased risk of a number of comorbidities. Thus, despite normalization of calcium levels in response to conventional therapy, this should only be considered as an apparent normalization, as patients are suffering from a number of complications and calcium-phosphate homeostasis is not normalized in a physiological manner. In a number of recent studies, replacement therapy with recombinant human PTH (rhPTH(1-84 as well as therapy with the N-terminal PTH fragment (rhPTH(1-34 have been investigated. Both drugs have been shown to normalize serum calcium while reducing needs for activated vitamin D and calcium supplements. However, once a day injections cause large fluctuations in serum calcium. Twice a day injections diminish fluctuations, but don't restore the normal physiology of calcium homeostasis. Recent studies using pump-delivery have shown promising results on maintaining normocalcemia with minimal fluctuations in calcium levels. Further studies are needed to determine whether this may improve QoL and lower risk of complications. Such data are needed before replacement with the missing hormone can be recommended as standard therapy.

  19. The short-chain oxidoreductase Q9HYA2 from Pseudomonas aeruginosa PAO1 contains an atypical catalytic center. (United States)

    Huether, Robert; Mao, Qilong; Duax, William L; Umland, Timothy C


    The characteristic oxidation or reduction reaction mechanisms of short-chain oxidoreductase (SCOR) enzymes involve a highly conserved Asp-Ser-Tyr-Lys catalytic tetrad. The SCOR enzyme Q9HYA2 from the pathogenic bacterium Pseudomonas aeruginosa was recognized to possess an atypical catalytic tetrad composed of Lys118-Ser146-Thr159-Arg163. Orthologs of Q9HYA2 containing the unusual catalytic tetrad along with conserved substrate and cofactor recognition residues were identified in 27 additional species, the majority of which are bacterial pathogens. However, this atypical catalytic tetrad was not represented within the Protein Data Bank. The crystal structures of unligated and NADPH-complexed Q9HYA2 were determined at 2.3 A resolution. Structural alignment to a polyketide ketoreductase (KR), a typical SCOR, demonstrated that Q9HYA2's Lys118, Ser146, and Arg163 superimposed upon the KR's catalytic Asp114, Ser144, and Lys161, respectively. However, only the backbone of Q9HYA2's Thr159 overlapped KR's catalytic Tyr157. The Thr159 hydroxyl in apo Q9HYA2 is poorly positioned for participating in catalysis. In the Q9HYA2-NADPH complex, the Thr159 side chain was modeled in two alternate rotamers, one of which is positioned to interact with other members of the tetrad and the bound cofactor. A chloride ion is bound at the position normally occupied by the catalytic tyrosine hydroxyl. The putative active site of Q9HYA2 contains a chemical moiety at each catalytically important position of a typical SCOR enzyme. This is the first observation of a SCOR protein with this alternate catalytic center that includes threonine replacing the catalytic tyrosine and an ion replacing the hydroxyl moiety of the catalytic tyrosine.

  20. Radiology of total hip replacement

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, H.J.; Lovelock, J.E.; McCollister Evarts, C.; Geyer, D.


    The radiology of total hip replacement (THR) and its complications is reviewed in conjunction with a long-term follow-up study on 402 patients with 501 prostheses. The indications, contraindications, biomechanics, and operative management of these patients is discussed. Clinical complications such as deep vein thrombosis, pulmonary embolism, and hemorrhage are mentioned. Postoperative infections including granulomatous pseudotumors, dislocations and fractures, true loosening of the prosthesis, and heterotopic bone formation (HBF) are discussed and illustrated. The importance of differentiating the lucent line from true loosening is stressed. Mechanical and other clinical complications which are largely ignored by radiologists are also discussed. The uses of arthrography and bone scanning are included.

  1. [Proximal and total femur replacement]. (United States)

    Pennekamp, P H; Wirtz, D C; Dürr, H R


    Reconstruction of segmental bone defects of the proximal femur following wide tumor resection or revision arthroplasty. Aggressive benign or primary malignant bone tumors of the proximal femur; destructive metastases; massive segmental bone defects of the proximal femur; periprosthetic fractures. Local infection; very short life expectancy (acetabular bone stock. Anterolateral approach. Exposure and detachment of the iliopsoas and gluteus medius muscle from the proximal femur with a sufficient safety margin to the bone; distal transsection of the vastus lateralis/intermedius and rectus femoris muscle according to the extraosseous tumor extension; distal femur osteotomy al least 3 cm beyond the farthest point of tumor extension; in case of total femur replacement, additional lateral arthrotomy of the knee with resection of the ligaments and menisci; reaming of the medullary canal after securing the shaft with a Verbrugge clamp; trial assembly and reduction followed by the definitive implantation of the prosthesis with adjustment of the femoral neck anteversion in 5° increments; soft tissue reconstruction and fixation to an attachment tube covering the prosthesis; in case of total femur replacement, the preparation of the tibia is followed by the coupling of the tibial and femoral components. Infection prophylaxis, 20 kg partial weight bearing, continuous passive motion. A total of 20  patients with proximal femur replacement and 2 patients with total femur replacement implanted between June 2007 and December 2011 were retrospectively reviewed. Three patients had primary malignant bone tumors, while 19 patients underwent resection for metastatic disease. The mean age at surgery was 62.0 ± 18.1 years (18-82 years). Fifteen patients with a mean follow-up of 20.3 ± 17.2 months (4-51 months) were studied. Among the 22 cases, periprosthetic infection occurred in 3 patients (13.6%), dislocation in 2 patients (9.1%). Evaluation of the functional

  2. Typical and atypical symptoms of gastro esophageal reflux disease: Does Helicobacter pylori infection matter?

    Institute of Scientific and Technical Information of China (English)

    Laurino; Grossi; Antonio; Francesco; Ciccaglione; Leonardo; Marzio


    AIM: To analyze whether the presence of Helicobacter pylori(H. pylori) infection could affect the quality of symptoms in gastro-esophageal reflux disease(GERD) patients. METHODS: one hundred and forty-four consecutive patients referred to our Unit for suspected GERD were recruited for the study. All patients underwent esophageal p H-metric recording. For those with a positive test, C13 urea breath test was then performed to assess the H. pylori status. GERD patients were stratified according to the quality of their symptoms and classified as typical, if affected by heartburn and regurgitation, and atypical if complaining of chest pain, respiratory and ears, nose, and throat features. H. pylori-negative patients were also asked whether they had a previous diagnosis of H. pylori infection. If a positive response was given, on the basis of the time period after successful eradication, patients were considered as "eradicated"(E) if H. pylori eradication occurred more than six months earlier or "recently eradicated" if the therapy had been administered within the last six months. Patients without history of infection were identified as "negative"(N). χ2 test was performed by combining the clinical aspects with the H. pylori status.RESULTS: one hundred and twenty-nine of the 144 patients, including 44 H. pylori-positive and 85 H. pylori-negative(41 negative, 21 recently eradicated, 23 eradicated more than 6 mo before), were eligible for the analysis. No difference has been found between H. pylori status and either the number of reflux episodes(138 ± 23 vs 146 ± 36, respectively, P = 0.2, not significant) or the percentage of time with pH values < 4(6.8 ± 1.2 vs 7.4 ± 2.1, respectively, P = 0.3, not significant). The distribution of symptoms was as follows: 13 typical(30%) and 31 atypical(70%) among the 44 H. pylori-positive cases; 44 typical(52%) and 41 atypical(48%) among the 85 H. pylori-negative cases,(P = 0.017 vs H. pylori +; OR = 2.55, 95%CI: 1

  3. Three atypical lethal cases associated with acute Zika virus infection in Suriname

    Directory of Open Access Journals (Sweden)

    Rens Zonneveld


    Full Text Available Acute Zika virus infection usually presents with a self-limiting triad of fever, rash and arthritis. There is limited information on severe or lethal cases. We report three cases of lethal acute Zika infection, confirmed with polymerase chain reaction, in adult patients with some co-morbidities. The patients showed rapid clinical deterioration with hemorrhagic and septic shock, and exaggerated acute and innate inflammatory responses with pronounced coagulopathy, and died soon after admission to the hospital. It remains unclear whether the fatal outcomes were due to acute Zika virus infection alone or to the combination with exacerbated underlying prior disease or co-infection. Nonetheless, the severity of these cases implies that increased awareness for atypical presentations of Zika virus infection, and careful clinical assessment of patients with symptoms of Zika, is warranted during current and future outbreaks.

  4. Early manifestation of communicating hydrocephalus after fractionated stereotactic radiotherapy for aggressive giant atypical prolactinoma. (United States)

    Ohtakara, Kazuhiro; Ohe, Naoyuki; Iwama, Toru; Hoshi, Hiroaki


    Aggressive giant invasive pituitary adenomas refractory to standard surgical or medical treatment remain a genuine challenge. In addition, communicating hydrocephalus (CH) attributed to malabsorption of cerebrospinal fluid (CSF) developing after radiotherapy for pituitary adenomas has not been previously reported. Herein, we describe the case of a 48-year-old male presenting with a giant atypical prolactinoma refractory to previous therapies, including pharmacotherapy and repetitive surgery. He underwent image-guided fractionated stereotactic radiotherapy in 28 fractions, resulting in early manifestation of CH associated with undisputed, both radiological and hormonal response. He recovered well after a shunt placement, with otherwise favorable consequences such as sustained tumor regression, decreasing prolactin level, and retained visual function for a 22-month follow-up. Fractionated stereotactic radiotherapy would provide a viable treatment alternative for these refractory cases, while caution should be exercised regarding the possibility of iatrogenic CH.

  5. Legal, ethical, and human rights considerations for physicians treating children with atypical or ambiguous genitalia. (United States)

    Greenberg, Julie A


    Some governments, human rights organizations, intersex organizations, and doctors have called for a moratorium on genital and gonadal surgeries for infants born with atypical or ambiguous genitalia. Moratorium supporters believe that the surgeries carry physical and emotional risks, the psychosocial benefits of these procedures have not been proven, and the surgeries violate the patients׳ fundamental human rights if they are performed before these patients can provide informed consent. Given these calls for a moratorium, treatment teams must determine how to treat their patients and how to counsel their patients׳ parents. This article examines the treatment teams׳ ethical and legal responsibilities and provides advice for treatment teams to follow that will protect their patients and their practices. Copyright © 2017. Published by Elsevier Inc.

  6. Renal replacement lipomatosis and xanthogranulomatous pyelonephritis: differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Romero, Frederico R. [Universidade Federal do Parana (UFPR), Curitiba, PR (Brazil); Pilati, Roberto; Brenny Filho, Thadeu; Caboclo, Maria Fernanda Sales Ferreira [Hospital Sao Vicente de Curitiba, PR (Brazil); Silva, Antonio de Padua Gomes [Centro de Citopatologia Parana Ltda. (Citopar), Curitiba, PR (Brazil); Cravo, Marco Aurelio [Laboratorio de Patologia e Citologia (Consulpat), Curitiba, PR (Brazil)


    Renal replacement lipomatosis (RRL) is a relatively uncommon entity, although misdiagnosis - mainly with xanthogranulomatous pyelonephritis (XGP) - due to lack of awareness by urologists, radiologists, and pathologists may be responsible for underreporting. We illustrate a case of RRL that was initially misdiagnosed as XGP, and compare it with a classic case of XGP, underscoring the similarities and the differences between them. (author)

  7. Effect of bovine colostrum feeding in comparison with milk replacer and natural feeding on the immune responses and colonisation of enterotoxigenic Escherichia coli in the intestinal tissue of piglets

    DEFF Research Database (Denmark)

    Sugiharto, Sugiharto; Poulsen, Ann-Sofie Riis; Canibe, Nuria;


    (23-d-old) were allocated to one of the following four groups: (1) killed at the beginning of the experiment (Base); (2) separated from the sow and fed BC (BC-fed); (3) separated from the sow and fed a MR (MR-fed); (4) kept with the sow (Sow-Milk). Blood was sampled on days 1 and 8, and faecal samples......The present study investigated the effect of feeding bovine colostrum (BC) to piglets in comparison with feeding a milk replacer (MR) and conventional rearing by the sow on the intestinal immune system and number of enterotoxigenic Escherichia coli (ETEC) colonising the intestinal tissue. Piglets...... were collected on days 1, 3, 5 and 8. On day 8, piglets were killed and gastrointestinal digesta and intestinal segments were collected. The frequency of diarrhoea was found to be higher (P≤ 0·019) in MR-fed piglets than in BC-fed and Sow-Milk piglets. Piglets from the MR-fed group had the lowest...

  8. Discrepant epidemiological patterns between classical and atypical scrapie in sheep flocks under French TSE control measures. (United States)

    Fediaevsky, Alexandre; Gasqui, Patrick; Calavas, Didier; Ducrot, Christian


    The occurrence of secondary cases of atypical and classical scrapie was examined in 340 outbreaks of atypical and 296 of classical sheep scrapie detected in France during active surveillance programmes between 2002 and 2007. The prevalence of atypical scrapie in these flocks was 0.05% under selective culling and 0.07% under intensified monitoring i.e. not significantly different from that detected during active surveillance of the general population (P>0.5), whereas these figures were much higher for classical scrapie (3.67% and 0.25%, respectively, P<10(-5)). In addition the number of atypical scrapie cases per outbreak did not indicate clustering. The results suggest that atypical scrapie occurs spontaneously or is not particularly contagious, and that the control measures in force allowed appropriate control of classical scrapie but were not more efficient than active surveillance in detecting cases of atypical scrapie.

  9. The cost of standing strong for replacement. (United States)

    Brown, Katy


    The testimonies of these individuals largely speak for themselves. The responses point to the importance of specific institutions or research groups that focus on the development and use of alternatives, and these should, of course, be better supported. Those who find themselves outside such institutions or teams, are more likely to feel stranded and isolated. Then again, Liz did have the support of a research group dedicated to replacement, but she has still had a significant struggle to find funding. The interviews with some of these particular young researchers indeed pointed toward a tangible ‘cost’ in terms of having to steer their career on the often difficult path toward the use of non-animal based methods.

  10. Replacement of Swiss "Attestations de fonctions"

    CERN Multimedia

    DG Unit


    The Swiss Permanent Mission has informed CERN that the "attestations de fonctions" issued to certain members of the personnel and the "attestations" issued to their family members will gradually be replaced by P-type "cartes de légitimation" (see specimen copies below). This card’s name, together with its more contemporary design compared with old-style "Attestations" issued in the 1970s, will make the holder’s daily life much easier, e.g. when crossing borders, renting accommodation or setting up a telephone line. The Users Office (PH Department) and the Cards Office (HR Department), which are responsible for handling requests for "cartes de légitimation", will provide information in due course on how they intend to organise the exchange of "attestations" (priority will be given to residents of Switzerland, in parti...

  11. Renal Replacement Therapy in Austere Environments

    Directory of Open Access Journals (Sweden)

    Christina M. Yuan


    Full Text Available Myoglobinuric renal failure is the classically described acute renal event occurring in disaster environments—commonly after an earthquake—which most tests the ingenuity and flexibility of local and regional nephrology resources. In recent decades, several nephrology organizations have developed response teams and planning protocols to address disaster events, largely focusing on patients at risk for, or with, acute kidney injury (AKI. In this paper we briefly review the epidemiology and outcomes of patients with dialysis-requiring AKI after such events, while providing greater focus on the management of the end-stage renal disease population after a disaster which incapacitates a pre-existing nephrologic infrastructure (if it existed at all. “Austere” dialysis, as such, is defined as the provision of renal replacement therapy in any setting in which traditional, first-world therapies and resources are limited, incapacitated, or nonexistent.

  12. B Plant process piping replacement feasibility study

    Energy Technology Data Exchange (ETDEWEB)

    Howden, G.F.


    Reports on the feasibility of replacing existing embedded process piping with new more corrosion resistant piping between cells and between cells and a hot pipe trench of a Hanford Site style canyon facility. Provides concepts for replacement piping installation, and use of robotics to replace the use of the canyon crane as the primary means of performing/supporting facility modifications (eg, cell lining, pipe replacement, equipment reinstallation) and operational maintenenace.


    CERN Multimedia

    Human Resources Division;


    The French Ministry of Foreign Affairs is currently replacing all diplomatic cards, special cards and employment permits («attestations de fonctions») held by members of the personnel and their families. These cards are replaced by secure, computerized equivalents. The old cards may no longer be used after 31 December 2001. For the purposes of the handover, members of the personnel must go personally to the cards office (33/1-015) between 8h30 and 12h30, in order to fill in a «fiche individuelle» form, taking the following documents for themselves and members of their families already in possession of a French card : A recent identity photograph in 4.5 cm x 3.5 cm format, the French card in their possession, an A4 photocopy of the same French card, certified by the cards office as being a true copy. Those members of the personnel whose cards (and/or cards belonging to members of their families) are shortly due to expire, or have recently done so, are also requested...

  14. Total hip replacement in dancers. (United States)

    Buyls, Inge R A E; Rietveld, A B M Boni; Ourila, Tiia; Emerton, Mark E; Bird, H A


    A case report of a professional contemporary dancer who successfully returned to the stage after bilateral total hip replacements (THR) for osteoarthritis is presented, together with her own commentary and a retrospective cohort study of total hip replacements in dancers. In the presented cohort, there were no post-operative dislocations or infections, the original pain had been relieved, rehabilitation was objectively normal and all resumed their dance (teaching) activities. Nevertheless, they were disappointed about the prolonged rehabilitation. Due to their high demands as professional dancers, post-operative expectations were too optimistic in view of the usual quick and favourable results of THR in the older and less physically active, general population. In all dancers with unilateral osteoarthritis, the left hip was involved, which may reflect the tendency to use the left leg as standing leg and be suggestive that strenuous physical activity may lead to osteoarthritis. Better rehabilitation guidelines are needed for dancer patients undergoing THR, especially drawing their attention to realistic post-operative expectations.

  15. Atypical Subtrochanteric Femur Fracture in Patient with Metastatic Breast Cancer Treated with Zoledronic Acid



    Several case series have suggested an association exists between atypical femoral subtrochanteric fractures and long-term use of bisphosphonates. It is thought that prolonged use of bisphosphonates may lead to adynamic, fragile bone. The radiologic features of atypical fractures include diffuse cortical thickening, transverse fracture, and beaking at the lateral subtrochanteric area. Atypical subtrochanteric femur fractures have been reported after use of alendronate, but there have been rare...

  16. Solitary Atypical Adenomatous Hyperplasia in a 12-Year-Old Girl. (United States)

    Jin, Moran; Lee, Yang-Haeng; Kim, Bomi; Yoon, Young Chul; Wi, Jin Hong


    Atypical adenomatous hyperplasia is a premalignant lesion reflecting a focal proliferation of atypical cells. These lesions are usually observed as incidental findings in lungs that have been resected due to other conditions, such as lung cancer. We report the youngest case of atypical adenomatous hyperplasia on record in a 12-year-old girl. In this patient, the lesion was found in association with pneumothorax.

  17. Endometrioid carcinoma infiltrating atypical leiomyoma: A mimicker of malignant mixed Mullerian tumor

    Directory of Open Access Journals (Sweden)

    Qury Sabita Mahapatra


    Full Text Available Atypical or symplastic leiomyoma is a rare histological variant of leiomyoma. This is a case report of 63-year-old patient who underwent hysterectomy with bilateral salpingo-oophorectomy. Histopathology of the polypoid growth seen in the endometrial cavity revealed atypical leiomyoma infiltrated by endometrioid cancer. Atypical leiomyoma can be misdiagnosed as leiomyosarcoma. Thus, carcinosarcoma was ruled out as it has an ominous prognosis. A diagnosis of atypical leiomyoma infiltrated by endometrioid cancer was given. We report this case as there are very few case reports of the above two pathology occurring simultaneously in the same patient.

  18. Atypical presentation of a cervical breast-cancer metastasis mimicking a dumbbell-shaped neurinoma

    Directory of Open Access Journals (Sweden)

    Christoph Kolja Boese


    CONCLUSION: The present case report and the reviewed literature point towards a growing clinical relevance of symptomatic LM in cancer patients and their possible atypical presentations and locations.

  19. Atypical lymphocytes in malaria mimicking dengue infection in Thailand

    Directory of Open Access Journals (Sweden)

    Polrat Wilairatana


    Full Text Available Polrat Wilairatana1, Noppadon Tangpukdee1, Sant Muangnoicharoen1, Srivicha Krudsood2, Shigeyuki Kano31Department of Clinical Tropical Medicine, 2Department of Tropical Hygiene, Faculty of Tropical Medicine, Mahidol University, Bangkok, Thailand; 3Department of Tropical Medicine and Malaria, Research Institute, National Center for Global Health and Medicine, Tokyo, JapanAbstract: Patients with uncomplicated falciparum or vivax malaria usually present with acute febrile illness and thrombocytopenia similar to dengue infection. We retrospectively studied atypical lymphocytes (AL and atypical lymphocytosis (ALO, defined as AL > 5% of total white blood cells in 1310 uncomplicated malaria patients. In 718 falciparum malaria patients, AL and ALO on day 0 were found in 53.2% and 5.7% of the patients, respectively, with median AL on admission of 1% (range 0%–10%, whereas in 592 vivax malaria patients, AL and ALO on day 0 were found in 55.4% and 9.5% of the patients, respectively, with median AL on admission of 1% (range 0%–14%. After antimalarial treatment, AL and ALO declined in both falciparum and vivax malaria. However, AL and ALO remained in falciparum malaria on days 7, 14, and 21, whereas AL and ALO remained in vivax malaria on days 7, 14, 21, and 28. In both falciparum and vivax malaria patients, there was a positive correlation between AL and total lymphocytes, but a negative correlation between AL and highest fever on admission, white blood cells, and neutrophils, eosinophils, and platelets (P < 0.05. In conclusion, AL or ALO may be found in uncomplicated falciparum and vivax malaria mimicking dengue infection. In tropical countries where both dengue and malaria are endemic, presence of AL or ALO in any acute febrile patients with thrombocytopenia (similar to the findings in dengue malaria could not be excluded. Particularly if the patients have risk of malaria infection, confirmative microscopic examination for malaria should be carried out

  20. Atypical Clinical Manifestations of Graves' Disease: An Analysis in Depth

    Directory of Open Access Journals (Sweden)

    Mohamed Osama Hegazi


    Full Text Available Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual manifestations of Graves' disease (GD, that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD.

  1. [Haemophilus influenzae type B meningitis: typical and atypical presentation]. (United States)

    Sánchez, J M; Zurro, F J; Ferreiro, D; Llana, R; Uría, D F


    We present 2 cases of Haemophilus influenzae meningitis. The first is a patient with atypical simptomatology: abdominal pain, fever and two days later pain in the back of his legs. Abdominal pathology was not found. The cerebrospinal fluid (CSF) showed polymorphonuclear cells, hyperproteinorachia and lowered glucose. CSF culture revealed Haemophilus influenzae, blood culture was sterile. The second had suffered surgery at maxilar and ethmoid sinuses four years before, and unknown germ meningitis 6 months before. Haemophilus influenzae was isolated from CSF cultures and CSF rhinorrhea was detected by isotopic cisternography.

  2. Unusual imaging presentation of infantile atypical Kawasaki disease. (United States)

    Kumar, Nishith; Mittal, Mahesh Kumar; Sinha, Mukul; Gupta, Arpita; Thukral, Brij Bhushan


    Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  3. Multiphasic acute disseminated encephalomyelitis associated with atypical rubella virus infection. (United States)

    Shinoda, Koji; Asahara, Hideaki; Uehara, Taira; Miyoshi, Katsue; Suzuki, Satoshi O; Iwaki, Toru; Kira, Jun-ichi


    We report the first case of an occurrence of multiphasic acute disseminated encephalomyelitis (ADEM) associated with atypical rubella virus infection with no rash and long-term increased titers of serum anti-rubella IgM in a 17-year-old male who had no history of rubella vaccination. He suffered from at least six clinical exacerbations with disseminated hyperintense lesions on FLAIR MR images during the course of 18 months. Repeated methylprednisolone pulse therapy and intravenous immunoglobulin therapy resolved the exacerbations. In patients with multiphasic ADEM of unknown etiology, clinicians should also consider the possibility of preceding infection with rubella virus.

  4. Unusual imaging presentation of infantile atypical Kawasaki disease

    Directory of Open Access Journals (Sweden)

    Nishith Kumar


    Full Text Available Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  5. Lipomatosis of the sciatic nerve: typical and atypical MRI features

    Energy Technology Data Exchange (ETDEWEB)

    Wong, Bernadette Zhi Ying [Mayo Clinic School of Medicine, Rochester, MN (United States); University College London, Royal Free and University College Medical School, London (United Kingdom); Amrami, Kimberly K.; Wenger, Doris E. [Mayo Clinic, Department of Radiology, Rochester, MN (United States); Dyck, P. James B. [Mayo Clinic, Department of Neurology, Rochester, MN (United States); Scheithauer, Bernd W. [Mayo Clinic, Department of Pathology, Rochester, MN (United States); Spinner, Robert J. [Mayo Clinic, Department of Neurologic Surgery, Rochester, MN (United States); Mayo Clinic, Department of Orthopedics, Rochester, MN (United States)


    Lipomatosis of nerve, also known as fibrolipomatous hamartoma, is a rare condition of nerve, usually affecting the median nerve. The MRI appearance is characteristic. We describe two cases of lipomatosis of nerve involving the sciatic nerve, an extremely unusual location for this lesion, in patients with sciatic neuropathy. These cases share the typical features previously described in the literature for other nerves, but also contain atypical features not previously highlighted, relating to the variability in distribution and extent of the fatty deposition. Recognition of the MRI appearance of this entity is important in order to avoid unnecessary attempts at surgical resection of this lesion. (orig.)

  6. Atypical presentation of sporotrichosis: report of three cases

    Directory of Open Access Journals (Sweden)

    Melissa Orzechowski Xavier


    Full Text Available Sporotrichosis occurs after fungal implantation of Sporothrix spp. in the skin, and is the main subcutaneous mycosis in Latin America. Here we describe three atypical cases of the disease. The first case report an extra-cutaneous occurrence of the disease with joint infection; the second one describes a patient with bilateral lymphocutaneous form of sporotrichosis; and the third shows a zoonotic cutaneous case with the development of an erythema nodosum as a hypersensitivity reaction. These cases show the disease importance on the region and the necessity of fungal culture to the diagnosis confirmation.

  7. Atypical Clavicular Involvement of Nonbacterial Osteitis: An Orthopaedic Enigma

    Directory of Open Access Journals (Sweden)

    Salil Umrani


    Full Text Available Nonbacterial osteitis (NBO is an underdiagnosed and poorly understood condition caused by sterile inflammation. It can mimic the presentation of many other orthopaedic conditions, for example, osteomyelitis, septic arthritis, or malignancy, in particular for those patients who have unifocal presentation. Because NBO is a diagnosis by exclusion, it poses much difficulty and confusion to many orthopaedic surgeons in treating such disease. Clavicular involvement is common but it is typically present at the medial aspect of the clavicle. We report a case of NBO with atypical clavicular involvement who presented to our orthopaedic clinic with painful swelling in the left shoulder. Appropriate investigations and management are discussed together with literature review.

  8. A rare case of late atypical post-partum eclampsia

    Directory of Open Access Journals (Sweden)

    Sanjay Singh


    Full Text Available Atypical post-partum eclampsia though uncommon is a recognized entity. Here we are presenting a case that developed postpartum eclampsia on day four of delivery without any preceding signs and symptoms of preeclampsia. Keeping this entity in mind would not only help early detection and appropriate institution of management but would also save the patients undergoing unnecessary treatment for a long period of time. This is more relevant for developing country like ours where eclampsia is an important cause of maternal mortality and resources for management of such cases are limited. [Int J Reprod Contracept Obstet Gynecol 2015; 4(5.000: 1645-1646

  9. Posterior cortical atrophy: an atypical variant of Alzheimer disease. (United States)

    Suárez-González, Aida; Henley, Susie M; Walton, Jill; Crutch, Sebastian J


    Posterior cortical atrophy (PCA) is a neurodegenerative syndrome characterized by striking progressive visual impairment and a pattern of atrophy mainly involving posterior cortices. PCA is the most frequent atypical presentation of Alzheimer disease. The purpose of this article is to provide a summary of PCA's neuropsychiatric manifestations. Emotional and psychotic symptoms are discussed in the context of signal characteristic features of the PCA syndrome (the early onset, focal loss of visual perception, focal posterior brain atrophy) and the underlying cause of the disease. The authors' experience with psychotherapeutic intervention and PCA support groups is shared in detail.

  10. Atypical case of primary intraosseous adenoid cystic carcinoma of mandible

    Directory of Open Access Journals (Sweden)

    D P Vinuth


    Full Text Available The Primary central salivary gland neoplasms of the mandible are infrequent. Their clinical and radiographic features may be similar to odontogenic tumors, which are otherwise common. Their accurate diagnosis becomes troublesome.Hence, diagnosis should depend on stringent diagnostic criteria. Adenoid cystic carcinoma is well known for its prolonged clinical course and its tendency for delayed onset of distant metastases. The long-term survival of these patients is therefore poor. Treatment modalities include surgery, radiotherapy and chemotherapy. The purpose of this paper is to report a case of primary central adenoid cystic carcinoma of mandible with an atypical presentation.

  11. Atypical presentation of salivary mucocele: diagnosis and management. (United States)

    Nilesh, Kumar; Chandra, Jagadish


    A mucocele is a common pathological lesion involving the minor salivary glands. It usually presents as an asymptomatic small superficial swelling over the lower labial mucosa. However, uncommon variants of oral mucoceles sometimes occur. Such lesions may be difficult to diagnose due to their unusual size and atypical clinical presentation. This article describes the case of a deeply embedded large mucocele over the buccal mucosa. Ultrasonography was used to visualize the size and position of the lesion, and aspiration was used to help in the eventual diagnosis. An intraoral approach was used in the complete removal of the lesion.

  12. Quantitative methods for somatosensory evaluation in atypical odontalgia

    DEFF Research Database (Denmark)

    Porporatti, André Luís; Costa, Yuri Martins; Stuginski-Barbosa, Juliana;


    A systematic review was conducted to identify reliable somatosensory evaluation methods for atypical odontalgia (AO) patients. The computerized search included the main databases (MEDLINE, EMBASE, and Cochrane Library). The studies included used the following quantitative sensory testing (QST......) methods: mechanical detection threshold (MDT), mechanical pain threshold (MPT) (pinprick), pressure pain threshold (PPT), dynamic mechanical allodynia with a cotton swab (DMA1) or a brush (DMA2), warm detection threshold (WDT), cold detection threshold (CDT), heat pain threshold (HPT), cold pain detection...... compared with healthy subjects. In clinical settings, the most reliable evaluation method for AO in patients with persistent idiopathic facial pain would be intraindividual assessments using HPT or mechanical allodynia tests....

  13. Atypical neuroleptic malignant syndrome with long-term clozapine. (United States)

    Corallo, Carmela E; Ernest, David


    Clozapine-induced neuroleptic malignant syndrome (NMS) may present differently from NMS associated with traditional antipsychotic agents, with fewer clinical features, particularly fewer extrapyramidal manifestations. The risk of developing NMS with clozapine does not appear dose-related. In half of cases, it occurs within 2 weeks of beginning clozapine therapy, but it can develop at any stage, especially with long-term use. We describe a patient who presented with atypical NMS after more than 10 years of clozapine treatment, and who was safely re-challenged with the same drug.

  14. An atypical case of necrotizing fasciitis of the breast. (United States)

    Mufty, H; Smeets, A; Christiaens, M R


    Necrotizing fasciitis is a rare and aggressive soft tissue infection involving the fascia and subcutaneous tissues. It carries a high mortality and morbidity rate. In literature, the few case reports on necrotizing fasciitis of the breast, describe the need for a mastectomy in 90% of the cases. We report on a case of a 72-year old Caucasian women with an atypical presentation of necrotizing fasciitis of the breast in combination with an acute abdomen, successfully treated with breast-conserving debridement and secondary wound closure.


    Directory of Open Access Journals (Sweden)



    Full Text Available A 52 year old woman presented with a history of asymptomatic skin lesions over left leg for the past 4 months. On examination she had multiple skin coloured papules and plaques over left leg. Oedema was also seen over left leg. Histopathology and immunohistochemistry proved the diagnosis of malignant melanoma. Radiological investigation showed metastasis to lung, liver and brain. The patient was asymptomatic at the time of admission but she developed rapid metastasis within a very short span of time. This case is reported for the rare atypical presentation of malignant melanoma.

  16. A case of unilateral atypical orofacial pain with Eagle's syndrome

    Directory of Open Access Journals (Sweden)

    G V Sowmya


    Full Text Available Eagle's syndrome is not an uncommon condition, but less known to physicians, where an elongated styloid process or calcified stylohyoid ligament compresses the adjacent anatomical structures leading to orofacial pain. Diagnosis is made with appropriate radiological examination. Nonsurgical treatment options include reassurance, analgesia, and anti.inflammatory medications; and the surgical option includes a transoral or external approach. Here, we present a case report of a male patient, of age38 years, with a chief complaint of unilateral atypical orofacial pain on the right side of his face radiating to the neck region, for the last two months.

  17. Device-related atypical pressure ulcer after cardiac surgery. (United States)

    Glasgow, D; Millen, I S; Nzewi, O C; Varadarajaran, B


    Medical devices must be closely monitored to prevent harm to patients. Pressure ulcers secondary to medical devices present a significant health burden in terms of length of stay in hospital and cost. Intensivists, anaesthetists and other professionals involved in managing critically ill patients following cardiac surgery need to be aware that pressure ulcers may develop in atypical sites and present at a later stage of the hospital stay. This case report highlights the important issue of device-related pressure ulcers in the cardiac surgical intensive care setting, particularly when the clinical status of the patient may preclude routine assessment and prophylaxis. An algorithm for preventing such pressure ulcers is suggested.

  18. Therapy of hypoparathyroidism by replacement with parathyroid hormone

    DEFF Research Database (Denmark)

    Rejnmark, Lars; Underbjerg, Line; Sikjaer, Tanja


    Hypoparathyroidism (HypoPT) is a state of hypocalcemia due to inappropriate low levels of parathyroid hormone (PTH). HypoPT is normally treated by calcium supplements and activated vitamin D analogues. Although plasma calcium is normalized in response to conventional therapy, quality of life (Qo......L) seems impaired and patients are at increased risk of renal complications. A number of studies have suggested subcutaneous injections with PTH as an alternative therapy. By replacement with the missing hormone, urinary calcium may be lowered and QoL may improve. PTH replacement therapy (PTH-RT) possesses...

  19. [Atypical mucocutaneous manifestations in neonates and infants with chikungunya fever in the municipalities of Cúcuta, Los Patios and Villa del Rosario, Norte de Santander, Colombia, 2014]. (United States)

    Muñoz, Claudia Marcela; Castillo, José Orlando; Salas, Daniela; Valderrama, Milena Alexandra; Rangel, Claudia Teresa; Vargas, Heiddy Patricia; Silva, Diana Carolina


    Atypical clinical manifestations have been observed in newborns and infants suffering from fever caused by the chikungunya virus. Objective: To describe the cases of fever caused by the chikungunya virus in newborns and infants with atypical mucocutaneous lesions. Materials and methods: We reviewed the clinical records, as well as lab tests and histopathological results, of newborns and infants diagnosed with Chikungunya virus and atypical mucocutaneous lesions in three regional hospitals. Results: Out of 18 suspected cases of chikungunya virus in newborns and infants, 11 were positive and presented atypical mucocutaneous manifestations. Six of the eleven confirmed cases corresponded to children under five months of age. The most common symptoms were fever, skin rash, irritability, and diarrhea. Three of the patients were infected with both dengue and chikungunya viruses. The ulcers occurred in the scalp, abdomen, genital and perianal region. We report mucocutaneous manifestations in newborns and infants diagnosed with fever caused by the chikungunya virus in Colombia. The rapid development of ulcers is most likely due to the immune response to the virus. Special attention should be given to pregnant women presenting symptoms of chikungunya virus infection prior to delivery, and their offspring should be followed-up in order to monitor possible complications.


    Institute of Scientific and Technical Information of China (English)

    Young T.PARK; Jing SUN


    Most of the spare ordering policies treated up to now have assumed that preventive and corrective replacement costs are equal, which implies in essential that there is no significant need for preventive replacement. This paper presents an ordering policy for preventive age replacement with minimal repair. Introducing the replacement, repair, inventory holding and shortage costs, the expected cost rate is derived. A procedure to determine jointly the ordering time for a spare and the preventive replacement time for the operating unit so as to minimize the expected cost rate is proposed. To explain the ordering policy and the optimization procedure, a numerical example is also included.