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Sample records for adult refractory idiopathic

  1. Treatment with gabapentin of 11 dogs with refractory idiopathic epilepsy.

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    Platt, S R; Adams, V; Garosi, L S; Abramson, C J; Penderis, J; De Stefani, A; Matiasek, L

    Eleven dogs diagnosed with refractory idiopathic epilepsy were treated orally with gabapentin for a minimum of three months at an initial dose of 10 mg/kg every eight hours. They were all experiencing episodes of generalised tonic-clonic seizures and had been treated chronically with a combination of phenobarbital and potassium bromide at doses sufficient to reach acceptable therapeutic serum levels without causing significant side effects. In each dog, the number of seizures per week, the average duration of the seizures and the number of days on which seizures occurred were compared for the three months before and after they were treated with gabapentin. A minimum 50 per cent reduction in the number of seizures per week was interpreted as a positive response to gabapentin, and six of the dogs showed a positive response. After the addition of gabapentin, both the number of seizures per week (P= 0.005) and the number of days with any seizures in a one-week period (P=0.03) were significantly reduced. Mild side effects of ataxia and sedation were observed in five of the dogs, but they were not severe enough to warrant the treatment being discontinued during the trial.

  2. Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.

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    Caramazza, Domenica; Quintini, Gerlando; Abbene, Ignazio; Malato, Alessandra; Saccullo, Giorgia; Lo Coco, Lucio; Di Trapani, Rosa; Palazzolo, Roberto; Barone, Rita; Mazzola, Giuseppina; Rizzo, Sergio; Ragonese, Paolo; Aridon, Paolo; Abbadessa, Vincenzo; Siragusa, Sergio

    2010-12-01

    Idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare disease responsive to treatment with plasma exchange (PE) but with a high percentage of relapse or refractory patients. A severe deficiency of ADAMTS-13 (<5% of normal activity), congenital or caused by an autoantibody, may be specific for TTP and it has been proposed that severe ADAMTS-13 deficiency now defines TTP. B cells play a key role in both the development and the perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with idiopathic TTP-HUS. This review of the literature focuses on the role of rituximab, a chimeric monoclonal antibody directed against CD20 antigen expressed by B lymphocytes, in patients with relapsing or refractory TTP-HUS with or without ADAMTS-13 deficiency, suggesting that rituximab may produce clinical remission in a significant proportion of patients. Rituximab therapy reduces plasma requirement and avoids complications related to salvage-immunosuppressive therapy. In conclusion, rituximab provides an effective, well-tolerated, and safe treatment option for patients with idiopathic TTP-HUS, thus giving an alternative approach to the current treatment based on PE.

  3. Adult idiopathic scoliosis: the tethered spine.

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    Whyte Ferguson, Lucy

    2014-01-01

    This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected.

  4. Long-term outcomes of combined chemotherapy in chronic refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    TAO Jie; HUANG Ying; LI Hong-qiang; WANG Ting-ting; WANG Xiao-yan; JI Lin-xiang; YANG Ren-chi

    2007-01-01

    @@ Adult idiopathic thrombocytopenic purpura (ITP) is a chronic acquired organ-specific autoimmune hemorrhagic disease characterized by the production of auto-antibodies against antigens on the membranes of platelet, resulting in enhanced Fc-mediated destruction of the platelets by macrophages in the reticuloendothelial system.

  5. Treatment of 37 Patients with Refractory Idiopathic Thrombocytopenic Purpura by Shengxueling(升血灵)

    Institute of Scientific and Technical Information of China (English)

    SHAO Ke-ding; ZHOU Yu-hong; SHEN Yi-ping; YE Bao-dong; GAO Rui-lan; ZHANG Yu

    2007-01-01

    Objective:To explore the clinical effect and possible mechanism of Shengxueling(升血灵,SXL),a Chinese medical preparation mainly consisting of ginseng saponins,in treating refractory idiopathic thrombocytopenic purpura(ITP).Methods:The selected 69 patients with ITP were randomly assigned to two groups,the 37 patients in the treated group were treated orally by SXL with the dose for adult as 60 mg twice a day for two weeks.Then when no marked rise of platelet count after that,the dose would be doubled and administered for another two weeks.Then the dose could be gradually reduced to the initiative level in patients who responded to the treatment,and if they did not,the treatment was regarded as ineffective and be terminated.The 32 patients in the control group were treated with ampeptide elemente instead of SXL,0.4 g each time three times a day in the first two weeks,and,if that was ineffective,0.2 g would be added each time and 1.8 g would be administered a day for two more weeks.Four weeks' treatment was regarded as one therapeutic course for both groups and the observation lasted for two successive courses in patients showing positive response.Results:In the 37 patients in the treated group,markedly effective was obtained in 7(19.0%),favorably effective in 15(40.5%),improved in 5(13.5%) and ineffective in 10(27.0%),the total effective rate being 59.5%.The corresponding number in the 32 patients in the control group was 4(12.5%),6(18.8%),3(9.4%),19(59.4%)and 31.3% respectively.Comparison showed the difference in therapeutic efficacy between the two groups was significant(P<0.05).Conclusion:SXL is a safe and effective preparation for treatment of ITP,showing an immediate effect which is obviously superior to that of ampeptide elemente with less adverse effect.

  6. Adult-onset idiopathic chondrolysis of the hip.

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    Yapp, Liam Z; McClymont, Liusaidh; Beggs, Ian; Gaston, Paul; Salter, Donald M

    2017-05-01

    We report the case of a 23-year-old man diagnosed with adult-onset idiopathic chondrolysis of the hip. Chondrolysis of the hip is a disorder most frequently seen in children who have suffered with slipped capital femoral epiphyses. Idiopathic chondrolysis of the hip is extremely rare and to our knowledge, its onset has never been documented in adults aged over 20. With reference to the available medical literature, we summarise the current clinical management of this unusual but important cause of young adult hip pain.

  7. Treatment of refractory juvenile idiopathic arthritis via pulse therapy using methylprednisolone and cyclophosphamide

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    Tania Caroline Monteiro de Castro

    Full Text Available CONTEXT: Patients with refractory juvenile idiopathic arthritis can benefit from aggressive therapy. CASE REPORT: We followed the clinical course of 4 patients (2 male, 2 female aged 9.1-17.8 years (mean of 14.5 years with polyarticular onset of juvenile rheumatoid arthritis and one 16-year-old boy with juvenile spondyloarthropathy associated with inflammatory bowel disease. All the juvenile rheumatoid arthritis patients fulfilled the diagnostic criteria established by the American College of Rheumatology. All patients had unremitting arthritis despite maximum therapy. All patients began receiving treatment using intravenous cyclophosphamide at 500-750 mg/m² and intravenous methylprednisolone at 30 mg/kg, for 3 days monthly (1 g maximum. The patients received between 3 and 11 monthly treatments, and/or 3-5 treatments every two months for 12 months, according to the severity of the disease and/or response to the therapy. All but one patient were evaluated retrospectively at the start (time 0 and 6 months (time 1, and 12 months (time 2 after the beginning of the treatment. A rapid and clinically significant suppression of systemic and articular manifestations was seen in all patients. Our results showed the favorable effect of this treatment on the clinical and some laboratory manifestations of juvenile idiopathic arthritis.

  8. Multiple cycles of recombinant human thrombopoietin therapy in a patient with chronic refractory idiopathic thrombocytopenic purpura.

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    Hua, Baolai; Zou, Nong; Wang, Shujie; Zhu, Tienan; Zhao, Yongqiang

    2005-06-01

    We describe a 41-year-old woman with chronic idiopathic thrombocytopenic purpura who received recombinant human thrombopoietin (rhTPO) therapy. rhTPO was administrated subcutaneously at a dosage of 1.0 mug/kg daily for a maximum of 14 days until the platelet count was more than 50 x 10/l. The patient received three cycles (six, 13, and eight doses each) of rhTPO, each initiated when the platelet counts was less than 10 x 10/l. The platelet count increased to above 50 x 10/l on days 5, 11 and 8, and peaked at 456 x 10/l, 130 x 10/l and 82 x 10/l on days 9, 15 and 13 in the three respective cycles, each followed by a gradual decline. The durations of platelet counts at more than 50 x 10/l in the three cycles were 13, 7 and 10 days, respectively. rhTPO was well tolerated with no adverse event observed. Antibodies to rhTPO by enzyme-linked immunosorbent assay were not detected. Our observations suggested that rhTPO could transiently increase the peripheral platelet count in patients with chronic refractory idiopathic thrombocytopenic purpura. The reasons why the peak platelet counts decreased and the duration of response shortened after successive cycles of treatment were unclear.

  9. Infection-Related Death among Persons with Refractory Juvenile Idiopathic Arthritis

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    Lane, Jonathan P.; Wood, Mark; Friswell, Mark; Flood, Terence J.; Foster, Helen E.

    2016-01-01

    Severe infections are emerging as major risk factors for death among children with juvenile idiopathic arthritis (JIA). In particular, children with refractory JIA treated with long-term, multiple, and often combined immunosuppressive and antiinflammatory agents, including the new biological disease-modifying antirheumatic drugs (DMARDs), are at increased risk for severe infections and death. We investigated 4 persons with JIA who died during 1994–2013, three of overwhelming central venous catheter–related bacterial sepsis caused by coagulase-negative Staphylococus or α-hemolytic Streptococcus infection and 1 of disseminated adenovirus and Epstein-Barr virus infection). All 4 had active JIA refractory to long-term therapy with multiple and combined conventional and biological DMARDs. Two died while receiving high-dose systemic corticosteroids, methotrexate, and after recent exposure to anti–tumor necrosis factor-α biological DMARDs, and 2 during hematopoietic stem cell transplantation procedure. Reporting all cases of severe infections and especially deaths in these children is of paramount importance for accurate surveillance. PMID:27648582

  10. Felbamate in an adult population with severe refractory epilepsy.

    LENUS (Irish Health Repository)

    Kearney, H

    2011-04-05

    Felbamate (FBM) is efficacious in treating patients with refractory epilepsy but was withdrawn due to cases of aplastic anaemia, hepatic failure and five reported deaths. FBM is currently used in specialist centres and is only being used in one Irish centre to date. This papers aim is to review the efficacy and safety experience of FBM in Irish adult patients with refractory epilepsy. A retrospective chart review was done on patients\\' medical records. Patients were subdivided into responders and non responders based on change in seizure frequency and side effects were recorded for all. Of the 13 patients on FBM nine patients responded to FBM, four patients did not. FBM is a safe and efficacious alternative in an Irish adult population with refractory epilepsy. However close monitoring is still required given the potential fatal side effects that are possible with this anticonvulsant.

  11. Surgical Outcome of Adult Idiopathic Chiari Malformation Type 1

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    Yuh, Woon Tak; Kim, Chi Heon; Kim, Hyun-Jib; Jahng, Tae-Ahn; Park, Sung Bae

    2016-01-01

    Objective The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18–63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (≥50% decrease in the diameter of the syrinx) were investigated. The follow-up period was 72.7±55.6 months. Results Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored. PMID:27651871

  12. Chronic idiopathic thrombocytopenic purpura in adult Chinese patients: a retrospective single-centered analysis of 1791 cases

    Institute of Scientific and Technical Information of China (English)

    LI Hong-qiang; ZHANG Lei; ZHAO Hui; JI Lin-xiang; YANG Ren-chi

    2005-01-01

    Background Adult chronic idiopathic thrombocytopenic purpura (ITP) is a common hematologic disease characterized by persistent thrombocytopenia. So far, there were only a few reports on adult Chinese patients with chronic ITP. This study aimed at defining the treatment outcome and prognostic factors for chronic ITP based on a large cohort of Chinese patients followed up for over 25 years at a single center.Methods The medical records of 1791 patients aged 14 years or older who were diagnosed as having chronic ITP at our hospital from 1974 to 1999 were retrospectively analyzed.Conclusions Adult Chinese chronic ITP patients can have long-term remission after steroid therapy and splenectomies. Primary steroid refractoriness is a prognostic factor predicting poor subsequent response to a splenectomy.

  13. New onset of idiopathic bilateral ear tics in an adult.

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    Agrawal, Amit; Shrestha, Rabin

    2009-04-01

    Tic disorders are commonly considered to be childhood syndromes. Newly presenting tic disorders during adulthood are uncommon and mostly described in relation to an acquired brain lesion or as incidental tics, particularly in context with other neurological or psychiatric diseases. Tic disorder involving the ears is extremely uncommon with only few studies in English literature. In the present case, we describe an adult patient with new-onset idiopathic tics disorder involving both ears, causing social embarrassment. In addition, our patient had recent onset of the tics without any childhood or family history of tic disorders. The single most important component of management is an accurate diagnosis. At the same time, tics should be differentiated from other movement disorders such as chorea, stereotypy, and dystonias.

  14. Selective endovascular embolization for refractory idiopathic epistaxis is a safe and effective therapeutic option: technique, complications, and outcomes.

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    Cohen, José E; Moscovici, Samuel; Gomori, John M; Eliashar, Ron; Weinberger, Jeffrey; Itshayek, Eyal

    2012-05-01

    Epistaxis generally responds to conservative management, but a more invasive approach, such as superselective embolization, is sometimes justified. We report our experience with endovascular procedures in 19 patients from 2002 to 2011 for the treatment of refractory idiopatic posterior epistaxis. The sphenopalatine artery and distal internal maxillary arteries were embolized in all patients. Unilateral embolization was performed in 12 patients (63%), bilateral embolization in seven (37%). Additional embolization of the descending palatine artery was performed in eight patients (42%) and embolization of the facial artery and palatine arteries in four (21%). In one patient the distal ophthalmic artery was embolized with n-butyl cyanoacrylate. No minor or major complications occurred in relation to the embolization procedures. The average hospital stay was 11.1±8.6 days, including an average 5.2±3.4 days after embolization. Average follow-up after discharge was 21.3±25.7 months. Superselective endovascular embolization proved safe and effective in controlling idiopathic epistaxis, refractory to other maneuvers.

  15. [Treatment of chronic refractory idiopathic thrombocytopenia purpura. 10 years experience at the Salvador Zubiran National Institute of Nutrition].

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    Pita-Ramírez, L; Hurtado-Monroy, R; Labardini-Méndez, J

    1992-01-01

    A total of 126 patients with chronic idiopathic thrombocytopenic purpura were diagnosed from January 1980 to January 1990 in our institute. In this group of patients, 21 were refractory to prednisone therapy, splenectomy or both, or had had a relapse after a good response with these treatments. They were given other therapies. There was enough information for evaluation in 16 of the 21 patients. The treatment responses were classified according to the post-therapy platelet counts: complete response (CR) = > 150 x 10(9)/L for more than three months; partial response (PR) = 50-150 x 10(9)/L for more than three months; any response (AR) = CR + PR; no response (NR) = < 50 x 10(9)/L. There were 15 women and one male. The median age was 41 years (range 11 to 65). 6-mercaptopurine was given in all patients with CR = 31.2%, PR = 18.8%, AR = 50% and NR = 50%. Seven patients received cyclophosphamide with CR = 28.6%, PR = 14.3%, AR = 42.9% and NR = 57%. Vincristine was given in four patients with only one PR. Interferon alpha 2B was given in four patients with two transitory PR. One patient received colchicine and vitamin C without response. It is concluded that 6-mercaptopurine and cyclophosphamide are useful drugs in refractory thrombocytopenic purpura.

  16. Autoantibodies in adult patients with idiopathic inflammatory myopathies in Buenos Aires.

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    Gómez, Graciela N; Gargiulo, María De Los Ángeles; Pérez, Nicolás; Collado, María Victoria; Suárez, Lorena V; Khoury, Marina; Sarano, Judith F

    2016-01-01

    The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results.

  17. Prevalence of Back Problems in 1069 Adults With Idiopathic Scoliosis and 158 Adults Without Scoliosis.

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    Grauers, A; Topalis, C; Möller, H; Normelli, H; Karlsson, Mk; Danielsson, A; Gerdhem, P

    2014-04-08

    Study Design. Multi-center case-control studyObjective. To investigate the prevalence of back problems in adults with idiopathic scoliosis.Summary of Background Data. Information on the prevalence of back problems in adults with idiopathic scoliosis is scarce, especially in untreated individuals, males and individuals with an age at onset of the scoliosis of less than 10 years.Methods. 1069 individuals with idiopathic scoliosis and 158 individuals without scoliosis, all aged 20-65 years, answered a questionnaire on back problems. Individuals with scoliosis were diagnosed between ages 4 and 20 years and any treatment was terminated before age 20. Logistic regression or ANCOVA was used for group comparisons.Results. Mean (SD) age at the time of investigation in individuals with scoliosis (123 men and 946 women) was 41 (9) years, and in individuals without scoliosis (75 men and 83 women) 45 (13) years. 374 individuals with scoliosis were untreated, 451 had been brace treated and 244 surgically treated. The mean prevalence of back problems was 64% in the individuals with scoliosis and 29% in the individuals without scoliosis (pscoliosis, 69% reported back problems, among the brace treated 61%, and among the surgically treated 64% (p = 0.06). When comparing females and males with scoliosis, and individuals with juvenile and adolescent scoliosis, there were no statistically significant differences in the prevalence of back problems (p = 0.10 and p = 0.23, respectively).Conclusion. Adults with idiopathic scoliosis have a higher prevalence of back problems than individuals without scoliosis. Treatment, gender and juvenile or adolescent onset of diagnosis was not related to the prevalence of back problems in adulthood.

  18. Efficacy of botulinum toxin type A 100 units versus 200 units for treatment of refractory idiopathic overactive bladder

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    Osama Abdelwahab

    2015-12-01

    Full Text Available Objective: To evaluate the efficacy and safety of a single intra detrusor injection of BoNTA comparing two different doses (100 U or 200 U in patients with idiopathic overactive bladder. Materials and Methods: A randomized prospective study evaluated the efficacy of BoNTA in management of refractory idiopathic overactive bladder and included 80 patients. All patients were assessed initially by taking a history, a physical examination, overactive bladder symptom score, urine analysis, routine laboratory investigations, KUB and pelviabdominal. OABSS was adjusted on all patients postoperative at 1,3,6,9 months also Urodynamic was done for all patients preoperative and postoperative at 3, 6, 9 months. Results: The mean age was 30.22±8.37 and 31.35±7.61 in group I and II respectively. There was no statistically difference between both groups in all parameters all over the study except at 9 months after treatment. Hematuria was observed 6 and 9 patients in group I and II respectively. Dysuria was observed in 6 and 15 patients in group I and II respectively. UTI was detected in 3 and 7 patients in group I and II respectively. Conclusion: A single-injection procedure of 100 U or 200 U BoNTA is an effective and safe treatment for patients with IOAB who failed anticholinergic regimens. OABSS and QoL were improved for 6 months; 100 U injections seemed to have comparable results with 200 U. There was a significant difference at month 9 towards 200 U with more incidences of adverse events.

  19. Efficacy of Anakinra in Refractory Adult-Onset Still's Disease

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    Ortiz-Sanjuán, Francisco; Blanco, Ricardo; Riancho-Zarrabeitia, Leyre; Castañeda, Santos; Olivé, Alejandro; Riveros, Anne; Velloso-Feijoo, María.L.; Narváez, Javier; Jiménez-Moleón, Inmaculada; Maiz-Alonso, Olga; Ordóñez, Carmen; Bernal, José A.; Hernández, María V.; Sifuentes-Giraldo, Walter A.; Gómez-Arango, Catalina; Galíndez-Agirregoikoa, Eva; Blanco-Madrigal, Juan; Ortiz-Santamaria, Vera; del Blanco-Barnusell, Jordi; De Dios, Juan R.; Moreno, Mireia; Fiter, Jordi; Riscos, Marina de los; Carreira, Patricia; Rodriguez-Valls, María J.; González-Vela, M. Carmen; Calvo-Río, Vanesa; Loricera, Javier; Palmou-Fontana, Natalia; Pina, Trinitario; Llorca, Javier; González-Gay, Miguel A.

    2015-01-01

    Abstract Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2–6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3–47.5] mg/day at ANK onset to 5 [0–10] at 12 months. After a median [IQR] follow-up of 16 [5–50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations. PMID:26426623

  20. Transanal Irrigation for Refractory Chronic Idiopathic Constipation: Patients Perceive a Safe and Effective Therapy

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    Minty, Ian; Bain, Iain M.; Cundall, Jeremy; Yiannakou, Yan

    2017-01-01

    Background. Transanal irrigation (TAI) can successfully treat neurogenic bowel dysfunction (NBD), but patient perception of its use in chronic idiopathic constipation (CIC) is unknown. Objective. To evaluate patient perceptions of the efficacy and safety of TAI for CIC and whether there are predictive factors of perceived treatment response. Methods. Prospective data collection of baseline physiology and symptom severity; retrospective evaluation of efficacy and safety perceptions using a snapshot survey. All patients fulfilling the Rome III criteria for functional constipation with chronic idiopathic aetiology were included. The main outcome measure was the duration of patients' usage of TAI. Results. 102 patients reported 21,476 irrigations over 119 patient years, with a mean duration of therapy use of 60.5 weeks [SD 73.2 : SE 7.3]. Overall symptom improvement included general well-being (65%), rectal clearance (63%), bloating (49%), abdominal pain (48%), and bowel frequency (42%). 68 patients (67%) were “moderately better” or “very much better” on a satisfaction question. Reported complications were minor. No correlation was demonstrated between duration of therapy use and baseline measures. Conclusion. A significant proportion of CIC sufferers use TAI as a long-term or bridging therapy and perceive it as safe. This therapy demands a prospective investigation of efficacy and safety. PMID:28115930

  1. Radiotherapy With or Without Surgery for Patients With Idiopathic Sclerosing Orbital Inflammation Refractory or Intolerant to Steroid Therapy

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    Lee, Jong Hoon [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Kim, Yeon-Sil, E-mail: yeonkim7@catholic.ac.kr [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Yang, Suk Woo; Cho, Won-Kyung [Department of Ophthalmology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Lee, Sang Nam [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Lee, Kyung Ji [Department of Hospital Pathology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Ryu, Mi-Ryeong; Jang, Hong Seok [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of)

    2012-09-01

    Purpose: To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. Methods and Materials: We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2 Gy per fraction. Presenting signs and treatment outcomes were assessed. Results: Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they had received radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. Conclusion: Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.

  2. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies

    DEFF Research Database (Denmark)

    Schwartz, TThomas W; Diederichsen, L. P.; Lundberg, Ingrid E.

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM ( JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also...

  3. Splenectomy in children with idiopathic thrombocytopenic purpura : A prospective study of 134 children from the Intercontinental Childhood ITP Study Group

    NARCIS (Netherlands)

    Kuehne, Thomas; Blanchette, Victor; Buchanan, George R.; Ramenghi, Ugo; Donato, Hugo; Tamminga, Rienk Y. J.; Rischewski, Johannes; Berchtold, Willi; Imbach, Paul

    2007-01-01

    Background. Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. Procedure. Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in

  4. Lack of efficacy of pyridoxine (vitamin B6) treatment in acquired idiopathic sideroblastic anaemia, including refractory anaemia with ring sideroblasts.

    Science.gov (United States)

    Baumann Kreuziger, Lisa M; Wolanskyj, Alexandra P; Hanson, Curtis A; Steensma, David P

    2011-06-01

    Pyridoxine, or vitamin B6, is commonly used to treat acquired idiopathic sideroblastic anaemia (AISA, including refractory anaemia with ring sideroblasts), but the efficacy of this therapy in an unselected AISA population (i.e. patients without confirmed ALAS2 or other pyridoxine-responsive germline mutations) has not been established. We reviewed clinical data from 231 patients with AISA and found that 42% of 203 evaluable patients had been treated with pyridoxine. Only 6.8% of pyridoxine-treated patients experienced a haemoglobin improvement (≥ 1.5 g/dL) meeting 2006 International Working Group for Myelodysplastic Syndromes standardised response criteria. As some patients received combination therapy with erythropoietin or other agents, improvement could be attributed to pyridoxine monotherapy in only one patient (1.4%). Smaller, less meaningful increments in haemoglobin levels of 0.5 g/dL were observed in 13.5% of patients. Response to therapy did not correlate with International Prognostic Scoring System (IPSS) risk group or multilineage vs unilineage dysplasia. New symptomatic peripheral neuropathy was noted in 2.3% of patients treated with pyridoxine. In this large series of unselected patients with sideroblastic anaemia, pyridoxine therapy was ineffective and was associated with a risk of adverse effects. Pyridoxine therapy should be reserved for patients with known or suspected pyridoxine-responsive mutations.

  5. Sagittal plane analysis of the spine and pelvis in adult idiopathic scoliosis

    Institute of Scientific and Technical Information of China (English)

    LI Wei-shi; LI Gang; CHEN Zhong-qiang; Kirkham B Wood

    2010-01-01

    Background There has been an increasing recognition of the importance of sagittal spinopelvic alignment in patients with scoliosis as it relates to clinical outcomes. However, the changes seen in sagittal spinopelvic alignment in adult idiopathic scoliosis patients is poorly defined. This study was conducted to evaluate the sagittal alignment of pelvis and spine in adult idiopathic scoliosis patients.Methods The sagittal parameters of the spine and pelvis were analyzed in lateral standing radiographs of 124 patients (mean age 47.4 years) with adult idiopathic scoliosis, including thoracic kyphosis (TK), thoracolumbar junction kyphosis (TLJ), lumbar lordosis (LL), pelvic incidence (PI), sacrum slope (SS), pelvic tilt (PT) and C7 plumb line (C7PL). The patients were divided into three groups according to the age: 20-40 years, 41-64 years, and ≥65 years. The parameters were compared with those in normal adults and adolescent idiopathic scoliosis (AIS) patients. The relationship between all parameters as well as age and sagittal parameters were analyzed.Results The PI in patients with adult idiopathic scoliosis was 58.1°±13.0°, which was significantly higher than that in normal adults. The PT (19.9°±10.6°) was also higher than that in both normal adults and AIS patients, while the SS (38.1°±12.0°) was similar or smaller. As age increased, C7PL, PT and TJL increased while LL decreased. There was no relationship between age and both PI and TK. PT had the strongest statistical association with the C7PL.Conclusions PI is higher in adult idiopathic scoliosis than normal subjects. The PT is the most relevant pelvic parameter to the global sagittal alignment of the spine. Age significantly influences sagittal parameters of the spine and pelvis except the PI and TK.

  6. Prognostic analysis of refractory anaemia in adult myelodysplastic syndromes

    Institute of Scientific and Technical Information of China (English)

    WANG Xiao-qin; CHEN Zi-xing; CHEN Shu-chang; LIN Guo-wei; JI Mei-rong; LIANG Jian-ying; LIU Dun-dan; LI De-gao; MA Yan

    2008-01-01

    Background Patients with myelodysplastic syndrome (MDS) display a very diverse pattem. In this study, we investigated prognostic factors and survival rate in adult patients with MDS refractory anaemia (MDS-RA) diagnosed according to French-American-British classification and evaluated the International Prognostic Scoring System (IPSS) for Chinese patients.Methods A multi-center study on diagnosis of MDS-RA was conducted to charactedze the clinical features of Chinese MDS patients. The morphological criteria for the diagnosis of MDS-RA were first standardized. Clinical data of 307 MDS-RA patients collected from Shanghai, Suzhou and Beijing from 1995 to 2006 were analyzed using Kaplan-Meier curve, log rank and Cox regression model.Results The median age of 307 MDS-RA cases was 52 years. The frequency of 2 or 3 lineage cytopenias was 85.6%. Abnormal karyotype occurred in 35.7% of 235 patients. There were 165 cases (70.2%) in the good IPSS cytogenetic subgroup, 44 cases (18.7%) intermediate and 26 cases (11.1%) poor. IPSS showed 20 (8.5%) categodzed as low dsk,195 cases (83.0%) as intermediate-I risk and 20 cases (8.5%) as intermediate-ll dsk. The 1-, 2-, 3-, 4- and 5-year survival rates were 90.8%, 85.7%, 82.9%, 74.9% and 71.2% respectively. Fifteen cases (4.9%) transformed to acute myeloid leukaemia (median time 15.9 months, range 3-102 months). Lower white blood ceil count (<1.5x109/L), platelet count (<30x109/L) and cytogenetic abnormalities were independent prognostic factors by multivariate analysis, but age (≥65 years), IPSS cytogenetic subgroup and IPSS risk subgroup were not independent prognostic factors associated with survival time.Conclusions Chinese patients were younger, and had lower incidence of cytogenetic abnormalities, more severe cytopenias but a more favourable prognosis than Western patients. The major prognostic factors were lower white blood cell count, lower platelet count and fewer abnormal karyotypes. The intemational prognostic scodng

  7. Holistic Acupuncture approach to idiopathic refractory nausea, abdominal pain and bloating

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    AIM: To evaluate the effectiveness of a holistic acupuncture approach on nausea, pain, bloating and electrogastrogram (EGG) parameters in patients with intractable symptoms. METHODS: Twelve patients with no or mild nausea (those without nausea had bloating or pain) and 10 with a history of moderate to severe nausea were referred for acupuncture. All underwent an EGG and were treated at acupuncture points PC6, SP4 and DU20. Visual analog scales (VAS) assessing severity of nausea, pain and bloating were obtained before and after acupuncture treatment. Nineteen patients received three and three patients received two treatments. RESULTS: VAS scores for nausea reflected the clinical assessment and differed significantly between mild and moderate/severe nausea groups. Acupuncture significantly improved severity of nausea in both groups with improved pre-treatment nausea between the first and third treatments in the moderate/severe nausea group. Pain scores improved with acupuncture in the mild nausea group only and bloating improved only with the first treatment in this group. Patients with bloating with VAS scores greater than 35 pre-treatment improved with acupuncture and over all VAS scores for pain improved with treatment. Acupuncture increased the power in the 2.7 to 3.5 cpm range in the EGG. CONCLUSION: In this uncontrolled clinical study, a holistic acupuncture approach significantly improved nausea in patients with refractory symptoms and increased the power in the 2.7-3.5 cpm component of the electrogastrogram. Bloating and pain VAS scores improved acutely with treatment. This study suggests that acupuncture may be effective in this refractory group of patients and further study using appropriate controls is warranted.

  8. Anterior Hypopituitarism is Rare and Autoimmune Disease is Common in Adults with Idiopathic Central Diabetes Insipidus.

    LENUS (Irish Health Repository)

    2012-02-01

    Objective: Central diabetes insipidus is a rare clinical condition with a heterogenous aetiology. Up to 40% of cases are classified as idiopathic, though many of these are thought to have an autoimmune basis. Published data has suggested that anterior hypopituitarism is common in childhood onset idiopathic diabetes insipidus. We aimed to assess the incidence of anterior hypopituitarism in a cohort of adult patients with idiopathic diabetes insipidus. Design and Patients: We performed a retrospective review of the databases of two pituitary investigation units. This identified 39 patients with idiopathic diabetes insipidus. All had undergone MRI scanning and dynamic pituitary testing (either insulin tolerance testing or GHRH\\/arginine and short synacthen testing) to assess anterior pituitary function. Results: One patient had partial growth hormone deficiency; no other anterior pituitary hormonal deficits were found. 33% had at least one autoimmune disease in addition to central diabetes insipidus. Conclusions: Our data suggest that anterior hypopituitarism is rare in adult idiopathic diabetes insipidus. Routine screening of these patients for anterior hypopituitarism may not therefore be indicated. The significant prevalence of autoimmune disease in this cohort supports the hypothesis that idiopathic diabetes insipidus may have an autoimmune aetiology.

  9. Refractory nausea and vomiting in the setting of well-controlled idiopathic intracranial hypertension.

    Science.gov (United States)

    Barnett, Dennis L; Rosenbaum, Rachel A; Diaz, Jonathan R

    2014-06-03

    Summary A 27-year-old woman with a history of recurrent nausea and vomiting in the setting of idiopathic intracranial hypertension (IIH) was admitted for control of unremitting nausea and vomiting. Initial antiemetic therapy included optimisation of IIH therapy by titrating acetazolamide, in addition to using ondansetron and metoclopramide as needed, with minimal relief. She was ultimately treated with palonosetron with complete resolution of her acute nausea. Nausea, often treated with 5-hydroxytryptamine (5-HT3) receptor antagonists, approved for perioperative and chemotherapy-induced nausea, are used off-label to treat nausea and vomiting outside of those settings. The efficacy of different regimens has been compared in the literature and continues to remain controversial. When choosing from different 5-HT3 antagonists there are other considerations, in addition to efficacy to consider: dosing schedule, half-life, time of onset, duration and cost-to-benefit ratio, and although one 5-HT3 antagonist may not have been effective, another one may be. In our case palonosetron, with a significantly longer half-life than other 5-HT3 antagonists, was effective in resolving nausea when compared with the more commonly used ondansetron.

  10. Severe aortic coarctation in a patient with refractory hypertension

    OpenAIRE

    Hiram Tápanes Daumy

    2015-01-01

    Idiopathic hypertension is common in adults, and some patients are considered resistant or refractory to treatment. In such cases it is often associated with a patho-logical process which hinders its control, in spite of changes in lifestyle and the proper use of drugs. This article is about an adult female patient with refractory hypertension due to aortic coarctation. CT scan and angiographic images are shown.Key words: Hypertension, Treatment, Aortic coarctation

  11. High risk of adrenal insufficiency in adults previously treated for idiopathic childhood onset growth hormone deficiency

    DEFF Research Database (Denmark)

    Lange, Martin; Feldt-Rasmussen, Ulla; Svendsen, Ole Lander;

    2003-01-01

    The aim was to reevaluate a group of adults treated for idiopathic childhood onset GH deficiency (GHD) after 18 yr without GH treatment. Twenty-six (11 females) patients participated. All but two had isolated GHD. Childhood diagnosis was established by insulin tolerance test (ITT). The patients w...

  12. Clinical efficiency of cyclosporine in chronic idiopathic urticaria in adults

    Directory of Open Access Journals (Sweden)

    V.I. Petrov

    2010-06-01

    Full Text Available The purpose of the research is to evaluate the clinical effectiveness of cyclosporine and other antihistamines in patients with chronic forms of urticaria resistant to basic first-line therapy. Open randomized controlled study has been performed in parallel groups. 53 patients with chronic idiopathic urticaria ages 18-50 years have been examined. In case of ineffectiveness of previous therapy, patients have been randomized into 2 groups: group I receiving cyclosporine (Sandimmune Neoral ® 2,5 mg/kg/day, group II receiving cetirizine (Zyrtec ® 10 mg/day and ranitidine (Zantac ® 300 mg/day orally. It has been found that the administration of cyclosporine in patients with severe chronic idiopathic urticaria provides a more rapid achievement of clinical effect than the therapy with H1/H2 histamine antagonists. It is confirmed by a significant decrease of total index of severity of illness and major symptoms of skin lesions. This tendency towards normalization of quality of life of patients taking cyclosporine remains during 8 weeks after the medication. Thus administration of cyclosporine can be considered as therapy of choice in patients with chronic idio-pathic urticaria with a severe course and ineffective long-term therapy with antihistamines / systemic corticosteroids

  13. Biological therapies for the treatment of juvenile idiopathic arthritis: Lessons from the adult and pediatric experiences

    Directory of Open Access Journals (Sweden)

    Matthew L Stoll

    2008-06-01

    Full Text Available Matthew L Stoll, Alisa C GotteDepartment of Pediatrics, Division of Rheumatology, UT Southwestern Medical Center, Dallas, TX, USAAbstract: Biologics have advanced the therapy of adult and pediatric arthritis. They have been linked to rare serious adverse outcomes, but the actual risk of these events is controversial in adults, and largely unknown in pediatrics. Because of the paucity of safety and efficacy data in children, pediatric rheumatologists often rely on the adult literature. Herein, we reviewed the adult and pediatric literature on five classes of medicines: Tumor necrosis factor (TNF inhibitors, anakinra, rituximab, abatacept, and tocilizumab. For efficacy, we reviewed randomized controlled studies in adults, but did include lesser qualities of evidence for pediatrics. For safety, we utilized prospective and retrospective studies, rarely including reports from other inflammatory conditions. The review included studies on rheumatoid arthritis and spondyloarthritis, as well as juvenile idiopathic arthritis. Overall, we found that the TNF inhibitors have generally been found safe and effective in adult and pediatric use, although risks of infections and other adverse events are discussed. Anakinra, rituximab, abatacept, and tocilizumab have also shown positive results in adult trials, but there is minimal pediatric data published with the exception of small studies involving the subgroup of children with systemic onset juvenile idiopathic arthritis, in whom anakinra and tocilizumab may be effective therapies.Keywords: juvenile idiopathic arthritis, biologics, rheumatoid arthritis

  14. Idiopathic adult ileoileal and ileocolic intussusception in situs inversus totalis: A rare coincidence

    Directory of Open Access Journals (Sweden)

    Nazish Butt

    2012-01-01

    Full Text Available Situs inversus totalis is a rare autosomal recessive congenital anomaly that is characterized by mirror image anatomy of the abdominal and thoracic organs. We report a case of a 28-year-old male with situs inversus totalis, who developed an idiopathic ileoileal and ileocolic intussusception, which was diagnosed on computed tomography scan. Patient underwent successfully ileal resection and side-to-side functional anastomosis of ileum 12 cms from ileocecal junction. Postoperative course was uneventful. To the best of our knowledge, this is the first case of idiopathic adult intussusception with situs inversus totalis in the literature.

  15. High rates of unsuccessful transfer to adult care among young adults with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Duffy Ciarán M

    2010-01-01

    Full Text Available Abstract Background This study aimed to describe the proportion of patients with juvenile idiopathic arthritis (JIA who had experienced an unsuccessful transfer from a pediatric rheumatology team to an adult rheumatologist and to compare the characteristics of those who achieved successful transfer to those who did not. Methods We conducted a systematic chart review of all patients with JIA who attended their final Montreal Children's Hospital JIA clinic appointment between 1992 and 2005. We tracked these patients for the two years after transfer to an adult rheumatologist. We then compared characteristics of patients with successful and unsuccessful transfers of care. Variables pertaining to disease characteristics, disease severity and psychosocial factors were examined. Univariate analyses were performed to determine if any single factor was associated with the outcome of unsuccessful transfer of care. Results 52% of patients fulfilled our criteria for unsuccessful transfer. Of the variables tested, an active joint count (AJC of zero at last visit was associated with the outcome of unsuccessful transfer (OR = 2.67 (CI 1.16-6.16; p = 0.0199. Conclusions Despite the presence of a coordinated process of transfer from pediatric to adult health care for the majority of the patients in this study, there was a high rate of unsuccessful transfer and/or sustained follow up which is disheartening. We found that patients with less active disease at the time of transfer, as indicated by a lower AJC, were more likely to be lost to follow up. Recent literature suggests that even in the least severe categories of JIA, 50% of patients persist with active disease into adulthood. Thus educating all JIA patients about the possibility of disease flare in adulthood may improve their adherence to recommendations for sustained follow-up in the adult milieu. This may lead to improvement of longitudinal outcomes for all JIA patients.

  16. Biological therapies for the treatment of juvenile idiopathic arthritis: Lessons from the adult and pediatric experiences.

    Science.gov (United States)

    Stoll, Matthew L; Gotte, Alisa C

    2008-06-01

    Biologics have advanced the therapy of adult and pediatric arthritis. They have been linked to rare serious adverse outcomes, but the actual risk of these events is controversial in adults, and largely unknown in pediatrics. Because of the paucity of safety and efficacy data in children, pediatric rheumatologists often rely on the adult literature. Herein, we reviewed the adult and pediatric literature on five classes of medicines: Tumor necrosis factor (TNF) inhibitors, anakinra, rituximab, abatacept, and tocilizumab. For efficacy, we reviewed randomized controlled studies in adults, but did include lesser qualities of evidence for pediatrics. For safety, we utilized prospective and retrospective studies, rarely including reports from other inflammatory conditions. The review included studies on rheumatoid arthritis and spondyloarthritis, as well as juvenile idiopathic arthritis. Overall, we found that the TNF inhibitors have generally been found safe and effective in adult and pediatric use, although risks of infections and other adverse events are discussed. Anakinra, rituximab, abatacept, and tocilizumab have also shown positive results in adult trials, but there is minimal pediatric data published with the exception of small studies involving the subgroup of children with systemic onset juvenile idiopathic arthritis, in whom anakinra and tocilizumab may be effective therapies.

  17. Rufinamide for refractory epilepsy in a pediatric and young adult population.

    Science.gov (United States)

    Joseph, Jacob R; Schultz, Rebecca J; Wilfong, Angus A

    2011-01-01

    This chart review investigated the efficacy and safety of rufinamide in 45 children and young adults who experienced a broad spectrum of partial and generalized seizure/epilepsy types which have been refractory to therapy. Of these patients, 19 (46%) achieved a >50% decrease in seizure frequency on rufinamide, and 7 patients achieved a >75% decrease in seizure frequency. While 17 (37.8%) patients stopped their trial of rufinamide prior to the end of the review period, only 2 (4.4%) were due to adverse effects. Although additional research must be done, this data shows promise that rufinamide is a safe and efficacious adjunct for cases of refractory epilepsy.

  18. Etanercept in the treatment of disease-modifying anti-rheumatic drug (DMARD)-refractory polyarticular course juvenile idiopathic arthritis: experience from Japanese clinical trials.

    Science.gov (United States)

    Mori, Masaaki; Takei, Syuji; Imagawa, Tomoyuki; Imanaka, Hiroyuki; Nerome, Yasuhito; Kurosawa, Rumiko; Kawano, Yoshifumi; Yokota, Shumpei; Sugiyama, Noriko; Yuasa, Hirotoshi; Fletcher, Tracey; Wajdula, Joseph S

    2011-12-01

    Efficacy, safety, and pharmacokinetics results from 4 studies-3 open-label (OL) and 1 randomized double-blind (DB)-have provided data for approval of etanercept for treatment of disease-modifying anti-rheumatic drug (DMARD)-refractory juvenile idiopathic arthritis (JIA) in Japan. Results from the 3 shorter-term (2 OL and 1 DB) studies are reported here. Subjects (4-17 years) enrolled in the OL studies had active JIA, i.e. ≥5 swollen joints and ≥3 joints with limitation of motion and pain or tenderness. Subjects enrolled in the primary OL study received etanercept 0.4 mg/kg subcutaneously twice weekly; in the lower-dose OL study subjects received etanercept 0.2 mg/kg. Subjects in the primary OL study who completed ≥48 weeks could continue into a 12-week DB dose-down extension study in which subjects received etanercept 0.4 or 0.2 mg/kg twice weekly. The primary endpoint in all 3 studies, i.e. 30% improvement in the American College of Rheumatology criteria for JIA (ACR Pedi 30) at 12 weeks, was achieved by ≥80% of subjects by week 2 and sustained to week 12. Common adverse events reported were injection site reactions, nasopharyngitis, and gastroenteritis. These results provide further evidence that etanercept is effective therapy for DMARD-refractory polyarticular JIA patients.

  19. Refractory Adult Primary Autoimmune Neutropenia that Responded to Alemtuzumab.

    Science.gov (United States)

    Neerukonda, Anu R; Lan, Fengshuo; Gabig, Theodore; Saraya, Takeshi

    2016-01-01

    Primary autoimmune neutropenia (P-AIN) is an extremely rare disease. The most effective treatment for primary P-AIN is a granulocyte colony-stimulating factor; however, no curative treatment has been reported. We herein report a case of an adult P-AIN patient with a relatively mild medical history (irrespective of the severe neutropenia) who showed a sustained hematological response over seventeen months after the initiation of treatment with subcutaneous Alemtuzumab.

  20. Premature subclinical atherosclerosis in children and young adults with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Bohr, Anna-Helene; Fuhlbrigge, Robert C; Karup Pedersen, Freddy

    2016-01-01

    -reporting. This is important as studies on PA in children with JIA have shown that most patients are less physically active than their healthy peers, and as physical inactivity in several large studies of normal schoolchildren is found to be associated with increased clustering of risk factors for cardiovascular disease......Many studies show that Juvenile Idiopathic Arthritis (JIA) is associated with early subclinical signs of atherosclerosis. Chronic inflammation per se may be an important driver but other known risk factors, such as dyslipidemia, hypertension, insulin insensitivity, a physically inactive lifestyle......, were considered.We found 13 descriptive cross sectional studies with healthy controls, one intervention study and two studies on adults diagnosed with JIA. Only one study addressed obesity, and physical activity (PA) has only been assessed in one study on adults with JIA and only by self...

  1. High risk of adrenal insufficiency in adults previously treated for idiopathic childhood onset growth hormone deficiency

    DEFF Research Database (Denmark)

    Lange, Martin; Feldt-Rasmussen, Ulla; Svendsen, Ole Lander;

    2003-01-01

    The aim was to reevaluate a group of adults treated for idiopathic childhood onset GH deficiency (GHD) after 18 yr without GH treatment. Twenty-six (11 females) patients participated. All but two had isolated GHD. Childhood diagnosis was established by insulin tolerance test (ITT). The patients...... were retested with an ITT to evaluate adult GH status. In five patients, an arginine and a synacthen test were performed instead of an ITT. Eleven of 25 patients had a subnormal cortisol response to ITT or synacthen. Ten patients had a GH peak less than 3.0 microg/liter (0.5. +/- 0.5 microg....../liter), whereas 16 patients displayed a normal GH response (12.3 +/- 10.6 microg/liter) after ITT. IGF-I values were decreased in the patients with a pathological retest as well as in patients with a normal GH response compared with controls (P

  2. Rituximab chimeric anti-CD20 monoclonal antibody treatment for adult refractory idiopathic thrombocytopenic purpura

    DEFF Research Database (Denmark)

    Braendstrup, Peter; Bjerrum, Ole W; Nielsen, Ove J;

    2005-01-01

    with a dose regimen of 375 mg/m2 i.v. approximately once weekly for 4 consecutive weeks. Six patients received a fixed dose of 500 mg disregarding their weight supplemented by 100 mg of methylprednisone i.v. or 50-100 mg of Pred given as premedication together with an antihistamine just before infusion...

  3. Proposal for a Candidate Core Set of Fitness and Strength Tests for Patients with Childhood or Adult Idiopathic Inflammatory Myopathies

    NARCIS (Netherlands)

    van der Stap, Djamilla K D; Rider, Lisa G; Alexanderson, Helene; Huber, Adam M; Gualano, Bruno; Gordon, Patrick; van der Net, Janjaap; Mathiesen, Pernille; Johnson, Liam G; Ernste, Floranne C; Feldman, Brian M; Houghton, Kristin M; Singh-Grewal, Davinder; Kutzbach, Abraham Garcia; Munters, Li Alemo; Takken, Tim

    2016-01-01

    OBJECTIVE: Currently there are no evidence-based recommendations regarding fitness and strength tests for patients with childhood or adult idiopathic inflammatory myopathies (IIM). This hinders clinicians and researchers in choosing the appropriate fitness- or muscle strength-related outcome measure

  4. [Splenectomy in chronic idiopathic thrombopenic purpura in adults. Apropos of 49 cases].

    Science.gov (United States)

    Melki, J; Dauce, J P; Kunlin, A; Tilly, H; Julien, J P; Monconduit, M; Piguet, H

    1989-01-01

    The authors reviewed the case files of 49 adult patients undergoing splenectomy for chronic idiopathic thrombocytopenic purpura at the Centre Henri Becquerel between 1970 and 1987. Although the postoperative course was straightforward in 83.7% of cases, one reoperation for subphrenic abscess was necessary and there was one postoperative death. Remission from thrombocytopenia was obtained in 87.5% of the patients, but only transiently in 8.5% of them. No preoperative predictive factors could be demonstrated. An early postoperative rise in the platelet count to more than 500 G/litre appears to ensure a good subsequent result. Secondary infectious complications are not exceptional and can be fatal (one death in our series); they require prophylaxis by anti-pneumococcal vaccination. The place of prophylactic antibiotic therapy has yet to be defined.

  5. Effectiveness and safety of TNF inhibitors in adults with juvenile idiopathic arthritis

    Science.gov (United States)

    McErlane, Flora; Foster, Helen E; Lunt, Mark; Watson, Kath D; Hyrich, Kimme L

    2016-01-01

    Introduction Many children with juvenile idiopathic arthritis (JIA) continue to have active disease into adulthood. Adults with JIA are a heterogeneous group, and the effects of tumour necrosis factor inhibitor (TNFi) therapies are not well described. This analysis aims to describe treatment outcomes among patients with JIA starting TNFi for the first time in adulthood. Methods Patients with arthritis onset polyarticular JIA were compared with a cohort (weighted for age and gender) of patients with rheumatoid arthritis (RA). Results In 443 adults with JIA starting a first TNFi, disease activity over 1 year improved across all measures. There were 58 first serious infections (IR 22.3/1000 pyrs); 4 cardiovascular events (IR 1.4/1000 pyrs); 11 uveitis events (IR 4.0/1000 pyrs) and 16 malignancies (IR 3.9/1000 pyrs). Compared with the weighted RA cohort, disease activity improvement was similar; malignancy rates were lower and uveitis rates much higher. While crude IR were similar, JIA patients had a lower risk of serious infection (HR 0.5 (95% CI 0.3 to 0.9)). Conclusions This is the largest study to describe disease activity and safety outcomes in adults with JIA receiving TNFi. Disease activity improved after 1 year in all disease patterns, suggesting TNFi is an effective therapy in this population. PMID:27843575

  6. Curative effect of treatment of refractory juvenile idiopathic arthritis by glucocorticoid%大剂量激素治疗难治性幼年特发性关节炎疗效观察

    Institute of Scientific and Technical Information of China (English)

    张曼; 王璐

    2011-01-01

    Objective To comprehend the clinical characteristic and definition of refractory juvenile idiopathic arthritis and the efficiency of glucocorticoid in therapy on this kind of disease. Methods Thirty children of refractory juvenile idiopathic arthritis to treat with large dose glu-cocorticoid( l0mg ~ 15mg/(kg · D). And the efficient was observed by change of ESR and CRP. Results The above measurements of joints were significantly improved compared with the level before combined therapy. But after 3 month one child use the Recombinant Human Tumor Necrosis Factor -a Receptor Ⅱ : Igg Fc Fusion Protein For injection. Conclusions Large dose glucocorticoid therapy is very effective to control the activity of disease in patients of refractory juvenile idiopathic arthritis.%目的 了解难治性幼年特发性关节炎的临床特点以及大剂量糖皮质激素治疗的疗效及价值.方法 30例难治性类风湿患儿均加用大剂量激素10 ~ 15mg/(kg·d)治疗,定期监测血沉、C反应蛋白的变化及临床症状的改善情况.结果 30例患儿治疗后血沉、C反应蛋白均降至正常,临床症状消失.有1例在3个月后病情反复,加用益赛普后症状缓解.结论 大剂量激素治疗难治性类风湿有明显疗效,并可改善患儿预后.

  7. 重组人血小板生成素治疗难治性特发性血小板减少性紫癜的循证评价%Evidence-based Evaluation of Recombinant Human Thromobopoietin for Refractory Idiopathic Thrombocytopenic Purpura

    Institute of Scientific and Technical Information of China (English)

    黄琳; 任晓蕾; 刘一; 李玉珍

    2011-01-01

    目的:评价重组人血小板生成素(rhTPO)治疗成人慢性难治性特发性血小板减少性紫癜(ITP)的有效性与安全性.方法:采用循证医学的文献分析评价方法,计算机检索CBM、CNKI、MEDLINE、EMbase、Cochrane图书馆临床对照试验资料库等数据库,收集rhTPO治疗成人慢性难治性ITP的临床研究证据.结果:检索到rhTPO治疗成人慢性难治性ITP的文献有:自身前后对照试验5篇,随机对照试验2篇.按Jadad量表评分:1篇随机对照研究质量较高,为4分,其余文献均小于3分.5项自身对照研究结果基本一致,认为rhTPO可升高成人难治性ITP患者的血小板计数,且不良反应轻微.2项随机对照试验认为rhTPO联合达那唑治疗难治性ITP的效果明显优于单用达那唑,且对于单用达那唑治疗无效者,接受rhTPO治疗后血小板计数亦明显升高.结论:现有研究显示rhTPO在治疗成人慢性难治性ITP方面疗效确切、安全性好,但试验设计存在不足,尚需进行设计良好的大样本研究.%OBJECTIVE: To evaluate the efficacy and safety of recombinant human thromobopoietin (rhTPO) for adult chronic refractory idiopathic thrombocytopenic purpura (ITP).METHODS: By literature analysis based on evidence-based medicine, clinical trials about rhTPO in the treatment of adult chronic refractory ITP were retrieved from CMB, CNKI, MEDLINE, EMbase and Cochrane library.RESULTS: Of collected pertinent literature about rhTPO in the treatment of adult chronic refractory ITP, five were self-control studies and two were randomized controlled trials (RCTs).The quality of seven studies was evaluated according to Jadad scale, of which one scored four points, and the others below three points.The five self-control studies all supported that rhTPO could effectively elevate the platelet counts and was safe for patients with adult chronic refractory ITP.Two RCTs indicated that rhTPO combined with danazol was more effective than danazol for

  8. Clinical Observation on the Treatment of Childhood Refractory Idiopathic Thrombocytopenic Purpura with Dihuang Zhixue Capsule(地黄止血胶囊)

    Institute of Scientific and Technical Information of China (English)

    LIU Qing-chi; WU Wei-hai; WU Da-yong; FENG Xin-wang; MA Ya-hui; LI Jian-ying; PANG Yu-hui; SONG Shu-hua

    2008-01-01

    Objective:To observe the clinical effect of Dihuang Zhixue Capsule(地黄止血胶囊,DZC,a Chinese preparation for cooling blood and dispelling toxic substances) in the treatment of childhood refractory idiopathic thrombocytopenic purpura(RITP),with cyclosporin A(CsA)used as the control.Methods:Fody-one children of RITP were randomized into the treated group and the control group.The 21 patients in the treated group were orally given 2 to 3 DZC capsules each time,thrice a day and the 20 in the control group were given 3 mg/kg CsA per day,with 3 months as one therapeutic course.The therapeutic efficacy,platelet count and adverse reaction in the two groups were compared at the end of the course.Results:(1)In the treated group,1 (4.8%)patient was evaluated as cured,3(14.3%)as markedly effective,5(23.8%)as effective,5(23.8%)as improved,7(33.3%)as ineffective,with the total effective rate being 66.7%:while in the control group,the corresponding numbers were 0,2(10.0%),2(10.0%),3(15.0%),13(65.0%)and 35.0%,respectively,showing statistical significance in difference between the total effective rates of the two groups(X2=4.11,P=0.0426).(2)As compared with the baseline,the platelet count increased in both groups after 2 months'treatment(P<0.05).After 3 months'treatment,the platelet count was higher in the treated group than in the control group(P<0.05).(3)The improvement of hemorrhage in the treated group after 8 weeks'treatment was better than that in the control group (P<0.05).(4)No apparent adverse reaction was observed in fhe treated group,while in the control group,hirsutism was shown in 15 cases;gingival hyperplasia in 10;digestive reaction in 5,liver function impairment in 5.hypedension in 2 and renal impairment in 2.Conclusion:The therapeutic efficacy of DZC is better than that of CsA.and DZC shows good compliance but brings no obvious adverse reaction.

  9. Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA

    Directory of Open Access Journals (Sweden)

    van Pelt Philomien A

    2012-08-01

    Full Text Available Abstract Background As patients with juvenile idiopathic arthritis (JIA progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. Methods Sixty-three patients with JIA (aged 10–27 years were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10–13 years; 14–17 years and 18–27 years. Results Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn’t influence outcome. There is no association with current sports participation. Conclusion Reduced aerobic capacity is present in children and adolescents with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.

  10. The clinical and pathological features of idiopathic membranous nephropathy in 246 Chinese adults

    Institute of Scientific and Technical Information of China (English)

    HUAN Hong-di; ZHANG Jing-hong; LIU Zhi-hong; LI Lei-shi; CHEN Hui-ping; ZHENG Feng

    2001-01-01

    Objective: To investigate the clinical and pathological features of idiopathic membranous nephropathy (IMN) in Chinese adults. Methods: From 1986 to 1997, 264 patients with biopsy proven membranous nephropathy were selected in this study. Clinical and pathological features were compared between patients at different ages by t test. Results: (1) Patients from 21- 40 years old were inclined to membranous nephropathy. (2) One hundred and six of the patients had heavy proteinuria at presentation. Hypertension was found in 35 patients. Renal insuffeiency occurred in 7.7% of the patients in renal biopsy. Microscopic hematuria was found in 40.2% of the patients. Seventy-four patients presented nephrotic syndrome. (3) Eight of the 57 patients had deterioration of renal function during an average 49-month follow-up. (4) Patients of stage Ⅰ, Ⅱ, Ⅲ and Ⅳ accounted for 42.3%, 48.7%, 6.0% and 3.0% respectively. Glomeruli IgG, C3 and C1qdeposition was found in 93.2%, 98.8% and 58.3% of the patients. Conclusion: Younger patients are inclined to membranous nephropathy. The incidence of hypertension, microscopic hematuria and renal insuffcieney is similar to that of other countries, while nephrotic syndrome is uncommon.

  11. Clinical significance of magnetic resonance imaging of skeletal muscles in idiopathic inflammatory myopathies of adults

    Energy Technology Data Exchange (ETDEWEB)

    Nishikai, Masahiko; Akiya, Kumiko [National Tokyo Medical Center (Japan)

    2000-12-01

    The purpose of this study was to evaluate the clinical significance of magnetic resonance imaging (MRI) of skeletal muscles in Japanese patients with idiopathic inflammatory myopathies (IIM). MRI was performed in 23 adult patients with IIM, including 10 with polymyositis, 12 with dermatomyositis, and 1 with focal myositis. Seven (73%) of 11 patients with active IIM and 2 (17%) of 12 patients with inactive IIM showed hyperintensity of T2-weighted images and normal intensity of T1-weighted images, indicating 'edema-like abnormalities' (MRI findings for active myositis). Muscle lipomatosis and fibrosis were demonstrated in four patients and 1 patient, respectively. Considerable selectivity of muscles in developing inflammatory disorders was found. In quadriceps muscles, for example, vastus muscles seemed to be more often affected in DM patients, whereas adductors were more often affected in PM patients. Serial examination of muscle MRIs was carried out in 4 patients and the findings paralleled the disease activities. The muscle MRI findings did not necessarily correlate with other findings, such as the presence of muscle weakness, elevated serum creatine kinase levels, myogenic electromyogram, or muscle biopsy findings. The muscle MRI was considered to be an additional useful tool for the diagnosis, evaluation of disease activity, and planning treatment of IIM. (author)

  12. Reversal of childhood idiopathic scoliosis in an adult, without surgery: a case report and literature review

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    Hawes Martha C

    2009-12-01

    Full Text Available Abstract Background Some patients with mild or moderate thoracic scoliosis (Cobb angle Case presentation A diagnosis of thoracic scoliosis (Cobb angle 45 degrees with pectus excavatum and thoracic hypokyphosis in a female patient (DOB 9/17/52 was made in June 1964. Immediate spinal fusion was strongly recommended, but the patient elected a daily home exercise program taught during a 6-week period of training by a physical therapist. This regime was carried out through 1992, with daily aerobic exercise added in 1974. The Cobb angle of the primary thoracic curvature remained unchanged. Ongoing clinical symptoms included dyspnea at rest and recurrent respiratory infections. A period of multimodal treatment with clinical monitoring and treatment by an osteopathic physician was initiated when the patient was 40 years old. This included deep tissue massage (1992-1996; outpatient psychological therapy (1992-1993; a daily home exercise program focused on mobilization of the chest wall (1992-2005; and manipulative medicine (1994-1995, 1999-2000. Progressive improvement in chest wall excursion, increased thoracic kyphosis, and resolution of long-standing respiratory symptoms occurred concomitant with a >10 degree decrease in Cobb angle magnitude of the primary thoracic curvature. Conclusion This report documents improved chest wall function and resolution of respiratory symptoms in response to nonsurgical approaches in an adult female, diagnosed at age eleven years with idiopathic scoliosis.

  13. Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA

    Directory of Open Access Journals (Sweden)

    van Pelt Philomine A

    2012-08-01

    Full Text Available Abstract Background As patients with juvenile idiopathic arthritis (JIA progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. Methods Sixty-three patients with JIA (aged 10–27 years were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10–13 years; 14–17 years and 18–27 years. Results Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn’t influence outcome. There is no association with current sports participation. Conclusion Reduced aerobic capacity is present in adolescents and young adults with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.

  14. Immunosuppressive treatment for nephrotic idiopathic membranous nephropathy: a meta-analysis based on Chinese adults.

    Directory of Open Access Journals (Sweden)

    Guoqiang Xie

    Full Text Available BACKGROUND: Idiopathic membranous nephropathy (IMN is the most common pathological type for nephrotic syndrome in adults in western countries and China. The benefits and harms of immunosuppressive treatment in IMN remain controversial. OBJECTIVES: To assess the efficacy and safety of different immunosuppressive agents in the treatment of nephrotic syndrome caused by IMN. METHODS: PubMed, EMBASE, Cochrane Library and wanfang, weipu, qinghuatongfang, were searched for relevant studies published before December 2011. Reference lists of nephrology textbooks, review articles were checked. A meta-analysis of randomized controlled trials (RCTs meeting the criteria was performed using Review Manager. MAIN RESULTS: 17 studies were included, involving 696 patients. Calcineurin inhibitors had a better effect when compared to alkylating agents, on complete remission (RR 1.61, 95% CI 1.13, to 2.30 P = 0.008, partial or complete remission (effective (CR/PR, RR 1.29, 95% CI 1.09 to 1.52 P = 0.003, and fewer side effects. Among calcineurin inhibitors, tacrolimus (TAC was shown statistical significance in inducing more remissions. When compared to cyclophosphamide (CTX, leflunomide (LET showed no beneficial effect, mycophenolate mofetil (MMF showed significant beneficial on effectiveness (CR/PR, RR: 1.41, 95% CI 1.16 to 1.72 P = 0.0006 but not significant on complete remission (CR, RR: 1.38, 95% CI 0.89 to 2.13 P = 0.15. CONCLUSIONS: This analysis based on Chinese adults and short duration RCTs suggested calcineurin inhibitors, especially TAC, were more effective in proteinuria reduction in IMN with acceptable side effects. Long duration RCTs were needed to confirm the long-term effects of those agents in nephrotic IMN.

  15. Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases.

    Science.gov (United States)

    Sakamoto, Ana Paula; Pinheiro, Marcelo M; Barbosa, Cássia Maria Passarelli Lupoli; Fraga, Melissa Mariti; Len, Claudio Arnaldo; Terreri, Maria Teresa

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA. We describe six cases of JIA patients followed at a referral center for Rheumatology and Pediatric Rheumatology, submitted to treatment with rituximab (RTX) after refractoriness to three anti-TNF agents. Patients received RTX cycles with two infusions every six months. Response to treatment was assessed by DAS28, HAQ/CHAQ, and an overall assessment by the doctor and the patient. Of our six patients, four were girls (mean age at onset of disease: 6.1 years; mean disease evolution time: 15.1 years; mean age upon receiving RTX: 21.6 years). Four patients belonged to polyarticular subtype (1 rheumatoid factor [RF]-negative, 3 FR-positive), a patient with systemic JIA subtype with a polyarticular course and arthritis related to enthesitis. Of our six patients, five responded to treatment; and during the course of 12 months, the clinical response was maintained, although not sustained. However, discontinuation by infusion reactions caused the withdrawal of RTX in two patients. The use of RTX in JIA is restricted to cases refractory to other biological agents and, even considering that this study was held in a small number of advanced patients, RTX proved to be an effective therapeutic option.

  16. The efficacy of topiramate in adult refractory status epilepticus: experience of a tertiary care center.

    Science.gov (United States)

    Synowiec, Andrea S; Yandora, Kristin A; Yenugadhati, Vamsi; Valeriano, James P; Schramke, Carol J; Kelly, Kevin M

    2012-02-01

    Refractory status epilepticus (RSE) occurs in patients with SE when they fail to respond to traditional medical therapy. Because there are very few case reports of topiramate (TPM) treatment of RSE in adult patients, we examined our experience with TPM with regard to its safety and efficacy in seizure termination in RSE in an adult patient population. We report a retrospective review of 35 adult patients with RSE who were treated with TPM in addition to other antiepileptic drugs (AEDs) between 2003 and 2010. After failure of initial treatments of benzodiazepines and weight-based intravenous loading doses of standard AEDs, TPM tablets were crushed and administered via nasogastric tube. Data were collected on age, gender, history of epilepsy, etiology of RSE, daily dose of TPM, co-therapeutic agents, treatment response, and disposition. Following initiation of TPM use and discontinuation of continuous intravenous anesthetics with no additional AEDs administered, cumulative cessation of RSE in patients was 4/35 (11%) at one day, 10/35 (29%) at two days, and 14/35 (40%) at three days. However, when including all patients and comparing the two patient groups in which RSE was or was not terminated within three days of initiating TPM as the last or not last AED given, there was no significant difference. Time to TPM response was not associated with the type of seizures, etiology of SE, or whether there was a history of epilepsy. There were no documented side effects or complications of therapy with TPM. This study provides support for the use of TPM as an adjunctive agent in the treatment of RSE.

  17. Prospective randomised controlled trial comparing trigone-sparing versus trigone-including intradetrusor injection of abobotulinumtoxinA for refractory idiopathic detrusor overactivity.

    LENUS (Irish Health Repository)

    Manecksha, Rustom P

    2012-05-01

    Botulinum toxin A is effective for treatment of idiopathic detrusor overactivity (IDO). The trigone is generally spared because of the theoretical risk of vesicoureteric reflux (VUR), although studies assessing injection sites are lacking.

  18. Drug utilization profile in adult patients with refractory epilepsy at a tertiary referral center

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    Priscila de Freitas-Lima

    2013-11-01

    Full Text Available Objective To evaluate the utilization profile of antiepileptic drugs in a population of adult patients with refractory epilepsy attending a tertiary center. Method Descriptive analyses of data were obtained from the medical records of 112 patients. Other clinical and demographic characteristics were also registered. Results Polytherapies with ≥3 antiepileptic drugs were prescribed to 60.7% of patients. Of the old agents, carbamazepine and clobazam were the most commonly prescribed (72.3% and 58.9% of the patients, respectively. Among the new agents, lamotrigine was the most commonly prescribed (36.6% of the patients. At least one old agent was identified in 103 out of the 104 polytherapies, while at least one new agent was prescribed to 70.5% of the population. The most prevalent combination was carbamazepine + clobazam + lamotrigine. The mean AED load found was 3.3 (range 0.4–7.7. Conclusion The pattern of use of individual drugs, although consistent with current treatment guidelines, is strongly influenced by the public health system.

  19. Management of refractory status epilepticus in adults: still more questions than answers.

    Science.gov (United States)

    Rossetti, Andrea O; Lowenstein, Daniel H

    2011-10-01

    Refractory status epilepticus (RSE) is defined as status epilepticus that continues despite treatment with benzodiazepines and one antiepileptic drug. RSE should be treated promptly to prevent morbidity and mortality; however, scarce evidence is available to support the choice of specific treatments. Major independent outcome predictors are age (not modifiable) and cause (which should be actively targeted). Recent recommendations for adults suggest that the aggressiveness of treatment for RSE should be tailored to the clinical situation. To minimise intensive care unit-related complications, focal RSE without impairment of consciousness might initially be approached conservatively; conversely, early induction of pharmacological coma is advisable in generalised convulsive forms of the disorder. At this stage, midazolam, propofol, or barbiturates are the most commonly used drugs. Several other treatments, such as additional anaesthetics, other antiepileptic or immunomodulatory compounds, or non-pharmacological approaches (eg, electroconvulsive treatment or hypothermia), have been used in protracted RSE. Treatment lasting weeks or months can sometimes result in a good outcome, as in selected patients after encephalitis or autoimmune disorders. Well designed prospective studies of RSE are urgently needed.

  20. Effects of nebulized sodium cromoglycate on adult patients with severe refractory asthma.

    Science.gov (United States)

    Sano, Yasuyuki; Adachi, Mitsuru; Kiuchi, Takahiro; Miyamoto, Terumasa

    2006-03-01

    Many patients with severe refractory asthma, which is insufficiently controlled by additional high-dose of inhaled corticosteroids, require oral corticosteroids and/or immunosuppressant. Clinicians should seek for suitable medications, for its' chronic use may induce high risk of side effects. The purpose of this study was to evaluate the efficacy and safety of nebulized sodium cromoglycate (3-4 times/day) in adult severe asthmatic patients with poorly controlled asthmatic symptoms, despite treatments with high-dose inhaled corticosteroids. Adult patients with severe asthma (n=251) were enrolled in a randomized clinical trial at 30 medical centers in Japan. Isotonic saline was used as placebo. The study was conducted for 10 weeks; with initial 2 weeks of observation followed by 8 weeks of treatments. Efficacy was primarily evaluated based on improvements in morning peak expiratory flow after treatment. All patients who applied inhalation of nebulized sodium cromoglycate (SCG group) or saline (Controls) were treated with high-dose of inhaled corticosteroids (median of beclomethasone dipropionate equivalent dose: 1600 microg/days) and second-line control therapy including oral corticosteroids. There was no significant difference in morning peak expiratory flow between SCG group and controls. However, when patients were stratified into atopic and non-atopic groups, morning peak expiratory flow had significantly improved in the atopic SCG group compared to atopic Controls. Additional inhalation of nebulized sodium cromoglycate with inhaled corticosteroids is effective even in patients with severe atopic asthma. This finding shows that nebulized sodium cromoglycate is expected to be new second-line therapeutic option in severe asthma.

  1. Idiopathic Multilocular thymic Cyst: Presentation of 4 Cases in Adults; Quiste timico multilocular idiopatico: presentacion de cuatro casos en adultos

    Energy Technology Data Exchange (ETDEWEB)

    Yano, C.; Serrano Puche, F.; Padin, M.; Serrano Ramos, F. [Hospital Regional Carlos Haya. Malaga (Spain)

    2004-07-01

    Idiopathic multilocular thymic cysts are relatively rare entities found inside the anterior mediastinal masses. Most patients are asymptomatic. Accordingly, the discovery of such cysts are typically made by chance during the examination of routine chest X-rays performed for other reasons. On other occasions, patients may complain of such unspecific clinical symptoms as chest pain, cough breathing difficulty or fever. There are presented 4 multilocular thymic cyst cases in adult patients (2 women, 2 men) of patho-anatomical diagnosis. The findings described entail simple radiology. CAT, ultrasound scan in one case and MR in another. (Author) 6 refs.

  2. 重组人血小板生成素在难治性血小板减少性紫癜中的应用%Application of recombinant human thrombopoietin on refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    曹慧琴; 任羽; 苏保雄; 郝彩霞; 刘卉; 刘宇宏

    2012-01-01

    Objective To evaluate the efficacy and safety of human recombinant thrombopoietin ( rhTPO ) on refractory idiopathic thrombocytopenic purpura ( ITP ). Methods 35 patients with ITP were divided into three groups: test group 1 ( treated with rhTPO combined danazol,n = 12 ),test group 2( only use rhTPO,n = 8 ),control group ( only use danazol,n = 15 ). Results The median counts of platelet in test group 1 was 6( 1. 0 ~ 15. 0 ) ×109/L before medi- cation;the maximum value increased to 97( 43 ~ 136 )× 109/L after 14 d of treatment(P 0.05).结论 rhTPO治疗难治性ITP安全、有效,rhTPO联合达那唑比单用达那唑起效快,能更好地控制出血症状.

  3. 高压氧联合糖皮质激素治疗难治性突发性聋疗效观察%Therapeutic effects of hyperbaric oxygen combined with glucocorticoid on refractory idiopathic sudden deafness

    Institute of Scientific and Technical Information of China (English)

    杨晨; 张禹; 李航; 孟祥恩; 潘树义

    2014-01-01

    目的 观察高压氧(hyperbaric oxygen,HBO)联合糖皮质激素(glucocorticoid,GC)对难治性突发性聋临床疗效.方法 经患者知情同意,并经医院伦理委员会批准,根据患者是否使用GC治疗,将有完整临床资料的79例突发性聋患者分为HBO联合GC治疗组44例,单纯HBO组35例.另外,在我院同期耳鼻喉科收治的复诊就医的突发聋患者中,选取使用GC治疗但未进行HBO治疗患者32例,作为单纯GC组.通过分析治疗有效率、治愈率和听力改善率,对比联合治疗与单纯HBO治疗及单纯GC药物治疗的疗效差异.结果 HBO联合GC组患者的治疗有效率(59.52%)和听力改善率[(61.3±14.7)%]明显高于单纯HBO组[28.13%,(43.6±5.5)%]和单纯GC组[31.25%,(56.4±4.1)%].结论 对难治性突发性聋HBO联合GC较单纯HBO治疗或GC治疗效果更好.%Objective Observe the therapeutic effects of hyperbaric oxygen (HB0) combined with glucocorticoid (GC) on refractory idiopathic sudden deafness.Methods Forty-two patients with sudden deafness (refractory idiopathic sudden deafness),who failed to recover after over one month of onset,were admitted into our Center from 2010 to 2013 for treatment with HBO combined with GC (referred to as the combined treatment group).Another 2 groups of patients with refractory idiopathic sudden deafness (each consisting of 32 patients),who were admitted into either our Center or the Department of Otorhinolaryngology of the Navy General Hospital and treated with simple HBO or GC,were used as controls.Through analyses of effective rates,cure rates and improvement rates in hearing,differences in therapeutic effects were compared between the patient group treated with HB0 combined with GC and the 2 control groups.Results The effective rate (59.52%) and improvement rate [(61.3 ± 4.7)%] for the combined treatment group were obviously higher than those of the simple HBO group[28.13%,(43.6 ± 5.5)%] and the simple hormone group [31.25

  4. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis: Views and Experiences of Patients

    NARCIS (Netherlands)

    Ammerlaan, Judy J.W.; Scholtus, Lieske W.; Drossaert, Constance H.C.; Os-Medendorp, van Harmieke; Prakken, Berent; Kruize, Aike A.; Bijlsma, Johannes J.W.

    2015-01-01

    Background: To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities wer

  5. Feasibility of a Website and a Hospital-Based Online Portal for Young Adults With Juvenile Idiopathic Arthritis : Views and Experiences of Patients

    NARCIS (Netherlands)

    Ammerlaan, Judy Jw; Scholtus, Lieske W; Drossaert, Constance Hc; van Os-Medendorp, Harmieke; Prakken, Berent; Kruize, Aike A; Bijlsma, Johannes JW

    2015-01-01

    BACKGROUND: To improve knowledge and to encourage active involvement of young adults with juvenile idiopathic arthritis (JIA), an informative website with written and video information and an online portal with access to the personal medical record, self-monitoring, and e-consult functionalities wer

  6. Idiopathic toe-walking in children, adolescents and young adults: a matter of local or generalised stiffness?

    Directory of Open Access Journals (Sweden)

    van de Putte Elise

    2011-03-01

    Full Text Available Abstract Background Idiopathic Toe Walking (ITW is present in children older than 3 years of age still walking on their toes without signs of neurological, orthopaedic or psychiatric diseases. ITW has been estimated to occur in 7% to 24% of the childhood population. To study associations between Idiopathic Toe Walking (ITW and decrease in range of joint motion of the ankle joint. To study associations between ITW (with stiff ankles and stiffness in other joints, muscle strength and bone density. Methods In a cross-sectional study, 362 healthy children, adolescents and young adults (mean age (sd: 14.2 (3.9 years participated. Range of joint motion (ROM, muscle strength, anthropometrics sport activities and bone density were measured. Results A prevalence of 12% of ITW was found. Nine percent had ITW and severely restricted ROM of the ankle joint. Children with ITW had three times higher chance of severe ROM restriction of the ankle joint. Participants with ITW and stiff ankle joints had a decreased ROM in other joints, whereas bone density and muscle strength were comparable. Conclusion ITW and a decrease in ankle joint ROM might be due to local stiffness. Differential etiological diagnosis should be considered.

  7. Idiopathic lymphocytic pleuritis: radiographic and high-resolution CT appearances and changes in response to therapy in two adults.

    LENUS (Irish Health Repository)

    O' Donnell, David H

    2012-02-01

    Inflammatory conditions of the pleura characterized by a predominantly lymphocytic infiltrate are described in several disorders. The commonest underlying aetiologies include tuberculous infection, autoimmune disorders (particularly Sjogren\\'s syndrome), and post coronary artery bypass graft surgery. Idiopathic lymphocytic pleuritis (ILP) is a rare form of diffuse pleural inflammation characterized by extensive lymphocytic infiltration for which no cause is found. Radiological descriptions of ILP are limited. We describe the radiographic and high-resolution computed tomography (HRCT) imaging features and response to corticosteroid therapy of ILP in two adults. Both patients presented with bilateral diffuse pleural thickening of >10 mm thickness extending >10 cm craniocaudally with small focal areas of atelectasis. Both cases demonstrated marked improvement in the degree and extent of pleural thickening and rounded atelectasis following corticosteroid therapy. HRCT provided a useful noninvasive method of assessing disease response to therapy.

  8. Idiopathic Hypertrophic Pyloric Stenosis in an Adult, a Potential Mimic of Gastric Carcinoma

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    Alireza Zarineh

    2010-01-01

    Full Text Available Primary or idiopathic hypertrophy of the pyloric muscle (IHPM is a rare entity with uncertain pathogenesis which both clinically and pathologically mimics gastric cancer. We present a rare late-occurring case of IHPM in a 71-year-old Caucasian man with no apparent predisposing factor. Imaging studies demonstrated gastric distension with air fluid levels and no evidence of extrinsic compression. At upper endoscopy, massive gastric distension and no evidence of any ulcer or other mucosal defects were observed. Microscopically, marked hypertrophy of muscularis mucosa with smooth muscle cells arranged in whorls and fascicles was present which gradually transitioned to normal areas. The muscle fibers stained with smooth muscle actin and trichrome stain highlighted fibrosis between the muscle fibers. Although uncommon, IHPM can clinically and histologically mimic other proliferations in the gastric wall, such as gastrointestinal stromal tumor or a spindle cell neoplasm. The recent advances in understanding the pathogenesis of IHPM are discussed.

  9. Cough in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    M.J.G. Van Manen (Mirjam J.G.); S.S. Birring (Surinder S.); C. Vancheri (Carlo); V. Cottin (Vincent); Renzoni, E.A. (Elisabetta A.); Russell, A.-M. (Anne-Marie); M.S. Wijsenbeek (Marlies)

    2016-01-01

    textabstractMany patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF,

  10. Optimization Correction Strength Using Contra Bending Technique without Anterior Release Procedure to Achieve Maximum Correction on Severe Adult Idiopathic Scoliosis

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    Ahmad Jabir Rahyussalim

    2016-01-01

    Full Text Available Adult scoliosis is defined as a spinal deformity in a skeletally mature patient with a Cobb angle of more than 10 degrees in the coronal plain. Posterior-only approach with rod and screw corrective manipulation to add strength of contra bending manipulation has correction achievement similar to that obtained by conventional combined anterior release and posterior approach. It also avoids the complications related to the thoracic approach. We reported a case of 25-year-old male adult idiopathic scoliosis with double curve. It consists of main thoracic curve of 150 degrees and lumbar curve of 89 degrees. His curve underwent direct contra bending posterior approach using rod and screw corrective manipulation technique to achieve optimal correction. After surgery the main thoracic Cobb angle becomes 83 degrees and lumbar Cobb angle becomes 40 degrees, with 5 days length of stay and less than 800 mL blood loss during surgery. There is no complaint at two months after surgery; he has already come back to normal activity with good functional activity.

  11. Premature subclinical atherosclerosis in children and young adults with juvenile idiopathic arthritis. A review considering preventive measures.

    Science.gov (United States)

    Bohr, Anna-Helene; Fuhlbrigge, Robert C; Pedersen, Freddy Karup; de Ferranti, Sarah D; Müller, Klaus

    2016-01-06

    Many studies show that Juvenile Idiopathic Arthritis (JIA) is associated with early subclinical signs of atherosclerosis. Chronic inflammation per se may be an important driver but other known risk factors, such as dyslipidemia, hypertension, insulin insensitivity, a physically inactive lifestyle, obesity, and tobacco smoking may also contribute substantially. We performed a systematic review of studies through the last 20 years on early signs of subclinical atherosclerosis in children and adolescents with JIA with the purpose of investigating whether possible risk factors, other than inflammation, were considered.We found 13 descriptive cross sectional studies with healthy controls, one intervention study and two studies on adults diagnosed with JIA. Only one study addressed obesity, and physical activity (PA) has only been assessed in one study on adults with JIA and only by self-reporting. This is important as studies on PA in children with JIA have shown that most patients are less physically active than their healthy peers, and as physical inactivity in several large studies of normal schoolchildren is found to be associated with increased clustering of risk factors for cardiovascular disease. It is thus possible that an inactive lifestyle in patients with JIA is an important contributor to development of the subclinical signs of atherosclerosis seen in children with JIA, and that promotion of an active lifestyle in childhood and adolescence may diminish the risk for premature atherosclerotic events in adulthood.

  12. Outcome analysis of high-dose chemotherapy and autologous stem cell transplantation in adolescent and young adults with relapsed or refractory Hodgkin lymphoma.

    Science.gov (United States)

    Akhtar, Saad; Rauf, Shahzad M; Elhassan, Tusneem A M; Maghfoor, Irfan

    2016-09-01

    High-dose chemotherapy (HDC) and autologous stem cell transplantation (auto-SCT) can salvage many patients with relapsed or refractory Hodgkin's lymphoma (HL). We are reporting the outcome of HDC auto-SCT and the impact of 21 prognostic factors in relapsed and refractory adolescent (14-21 years) and young adult (>21-30 years) (AYA) HL patients. We used Fine and Gray's competing risk analysis method and regression model for outcome analysis. From 1996 to 2013, 290 consecutive patients with biopsy-proven HL underwent HDC auto-SCT for relapsed/refractory HL; 216 patients (74.5 %) were AYA at the time of auto-SCT. Male/female were equal, median age at auto-SCT was 22.4 years, and there were 94 adolescent (43.5 %) and 122 young adults (56.5 %). There was refractory disease in 121 (56 %) patients, relapsed in 95 (44 %). Median follow-up was 72.6 months. The Kaplan-Meier method estimated that 5-year overall survival is 62.7 % (adolescents (63.5 %), young adults (62 %)) and event-free survival was 51.3 %. Five-year cumulative incidence of disease-specific death (DS-death) is 33 % and that of DS-event is 45 %. For DS-death, the multivariate analysis identified complete remission (CR) duration of young adults.

  13. O adulto com artrite idiopática juvenil poliarticular The adult patient with polyarticular juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Liz Wallin

    2009-08-01

    Full Text Available Crianças com a forma poliarticular da artrite idiopática juvenil (AIJ, ao entrarem na idade adulta, têm um quadro similar ao de pacientes com artrite reumatoide (AR de início no adulto. No presente estudo comparam-se características clínicas e imunológicas desses dois grupos de pacientes. Para isso, foram estudados vinte adultos com AIJ poliarticular e cinquenta pacientes com AR (pareados para sexo e tempo de duração de doença, para presença de autoanticorpos, nódulos subcutâneos, síndrome de Sjögren secundária, hipotireoidismo e para a determinação de índices funcionais e antropométricos. Encontraram-se, nesses dois grupos, características similares, exceto pela presença de fator reumatoide (menor no grupo de AIJ poliarticular; P = 0,026 e menor IMC nos pacientes com AIJ poliarticular (P When children with polyarticular juvenile idiopathic arthritis (JIA reach adulthood, they have a condition similar to that of patients with adult onset rheumatoid arthritis (RA. In the present study, the clinical and immunological characteristics of these two groups of patients are compared. The presence of autoantibodies, subcutaneous nodules, secondary Sjögren syndrome, and hypothyroidism, was determined in 20 adult patients with polyarticular JIA and in 50 patients with RA (paired for gender and duration of the disease, as well as the determination of functional and anthropometric indexes. Both groups had similar characteristics, except for the presence of rheumatoid factor (lower in the polyarticular JIA group; P = 0.026 and lower BMI in patients with polyarticular JIA (P < 0.001.

  14. A prospective study of the modified Atkins diet for adults with idiopathic generalized epilepsy.

    Science.gov (United States)

    Kverneland, Magnhild; Selmer, Kaja K; Nakken, Karl O; Iversen, Per O; Taubøll, Erik

    2015-12-01

    For children with pharmacoresistant epilepsy, the ketogenic diet is an established treatment option worldwide. However, for adults, this treatment is less frequently offered, and its efficacy less well-documented. The aim of this study was to examine efficacy and tolerability of such a diet as an adjuvant therapy to antiepileptic drugs for adult patients with pharmacoresistant generalized epilepsy. Thirteen patients (12 women) aged 16-57 years were included prospectively. They were treated with a modified Atkins diet for 12 weeks. Nine of the 13 participants had juvenile myoclonic epilepsy (JME), two had childhood absence epilepsy, one had Jeavons syndrome, and one had generalized epilepsy of unknown type. Six participants, all with JME, completed the 12-week study period. Among these six, four had >50% seizure reduction. Their seizure severity, using the revised Liverpool Seizure Severity Scale, was reduced by 1, 5, 57.5, and 70 points, respectively (scale: 1-100 points). In three of these four responders, quality of life, assessed by QOLIE-89, increased more than 20 points (scale: 0-100 points). Mean reduction of body weight after 12 weeks on diet was 6.5 (range: 4.3-8.1) kg. Lack of motivation, poor compliance, and seizure aggravation were the main reasons for premature termination of the diet. Apart from one patient who developed gallstones when ending the treatment after 10 months, no adverse effects were noted. In conclusion, using a modified Atkins diet for 12 weeks led to a clinically relevant reduction of seizure frequency in four of thirteen adult patients with pharmacoresistant generalized epilepsy. All responders were diagnosed with JME. In three of the four, the benefits of diet were so considerable that they chose to continue the treatment.

  15. High-dose, continuous-infusion cyclophosphamide, cytarabine, vincristine, and prednisone for remission induction in refractory adult acute leukemia.

    Science.gov (United States)

    Guthrie, T H

    1987-04-01

    Fifteen consecutive patients with refractory adult acute leukemia (RAAL) were treated with a combination of high-dose, continuous-infusion cyclophosphamide, cytarabine, vincristine, and prednisone (Hi-COAP). The initial nine patients received cyclophosphamide 350 mg/m2 as a 24-hour intravenous (IV) infusion over 5 days; cytarabine, 100 mg/m2 IV bolus every 12 hours for ten doses; vincristine, 2.0 mg IV bolus on day 1; and prednisone, 100 mg orally for 7 days. The last six patients had the cyclophosphamide infusion lengthened to 7 days, and the cytarabine increased to 14 doses. All patients were evaluable for toxicity and response. Seven patients (47%) obtained a complete remission and six patients (40%) a partial remission. Median duration of all remissions has been 7.0 months with a range of 1 to 32 months. Toxicity has been limited to primarily myelosuppression with no hemorrhagic cystitis, central nervous system (CNS), hepatic, or pulmonary toxicity noted. Gastrointestinal toxicity was mild, with no effect on nutritional status noted. Median duration of complete responders was 8.5 months. Thus, Hi-COAP demonstrates promising efficacy with minimal toxicity in RAAL and warrants further exploration in multiinstitutional trials.

  16. A monocentric retrospective study comparing pulse cyclophosphamide therapy versus low dose rituximab in the treatment of refractory autoimmune hemolytic anemia in adults.

    Science.gov (United States)

    Fu, Rong; Yan, Siyang; Wang, Xiaoming; Wang, Guojin; Qu, Wen; Wang, Huaquan; Wu, Yuhong; Liu, Hong; Song, Jia; Guan, Jin; Xing, Limin; Ruan, Erbao; Li, Lijuan; Liu, Hui; Shao, Zonghong

    2016-10-01

    This retrospective study aims at confirming the efficacy and safety of low dose rituximab and pulse cyclophosphamide in the treatment of refractory AIHA in adults and making comparison of the two. Forty-nine adult patients with refractory AIHA have been enrolled. Results showed low dose rituximab combined with steroid therapy (group B) got more CR (78.9 %, 15/19) compared to that in intermittent intravenous cyclophosphamide combined with steroid therapy (group A) (42.1 %, 8/19) (P = 0.04) at 6 months after treatment. The hemoglobin level in group B was higher than group A at the time point of 1 month (P = 0.02) after treatments. The RFS in group A was 87.9 % at 6 months and 82.7 % at 12 months, which were no significant difference with group B (91.1 % at 6 months and 86.0 % at 12 months) (P = 0.81). Both the two therapies were well tolerated with pulmonary infections as the most common side effects. In conclusion, low dose rituximab combined with steroid therapy presents to be a better choice in the treatment of refractory AIHA in adults comparing with pulse cyclophosphamide therapy.

  17. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?

    Science.gov (United States)

    Prendiville, Julie S; Tucker, Lori B; Cabral, David A; Crawford, Richard I

    2004-01-01

    The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic

  18. 成人特发性喉痉挛30例临床诊治分析%Clinical analysis of diagnosis of idiopathic laryngospasm in adult 30 cases

    Institute of Scientific and Technical Information of China (English)

    袁林林; 娄卫华; 桑建中; 孙慧如

    2011-01-01

    目的 探讨成人特发性喉痉挛临床诊断及治疗方法.方法 分析郑州大学第一附属医院鼻咽喉头颈外科自2009年2月至2011年2月病房及门诊收治的30例成人特发性喉痉挛患者的临床资料.全部患者应用地塞米松针静脉点滴,用醋酸泼尼松龙针与利多卡因针混合行双侧喉上神经封闭,选择性应用抗生素及止咳药物、抗胃酸反流药物.结果 治疗2周后,22例未再发作喉痉挛.喉痉挛发作频率明显减少者7例,无明显效果者1例.结论 喉痉挛诊断主要依据临床表现及喉镜检查.采用针对性诱因治疗及双侧喉上神经封闭治疗成人特发性喉痉挛是有效的.%Objective To discuss the diagnosis and treatment of idiopathic laryngospasm in adult. Methods A retrospective analysis was performed on 30 patients of idiopathic laryngospasm in adull from February 2009 to February 2011, based on a careful review on their clinical data. All patients with intravenous dexamethasone needle, prednisolone acetate needle and lidocaine needle mixture of bilateral superior laryngeal nerve closed, the application of selective antibiotic and cough medicines, resistant to acid reflux drugs. Results After 2 weeks treatment, 22 cases did not attack again laryngeal spasm. Laryngeal spasm seizure frequency reduced obviously in 7 cases, no obvious effect in 1 cases. Conclusions Strategic on the diagnosis of idiopathic laryngospasm in adult is rely on clinical manifestations and laryngoscopy. Use targeted treatment of predisposing factors and bilateral superior laryngeal nerve block in the treatment of adult idiopathic laryngeal spasm is effective.

  19. 不同类型成人脊柱侧凸的影像学研究%Imaging diagnosis of degenerative scoliosis and adult idiopathic scoliosis

    Institute of Scientific and Technical Information of China (English)

    吴志宏; 宋海峰; 王以朋; 闫家智; 邱贵兴

    2008-01-01

    目的 探讨成人脊柱侧凸中最常见的退行性脊柱侧凸(DS)的影像学特征,并与成人特发性脊柱侧凸(IS)相比较,以提高诊断准确率.方法 通过对站立位X线、Bending像的相关参数测量及脊髓造影和CTM检查,回顾性分析98例成人脊柱侧凸的临床资料和影像学特点.结果 DS和成人Is的发病年龄、性别、侧凸的解剖位置和顶点分布上有明显区别,冠状面Cobb角分别为(18.67±7.62)°和(56.2±10.10)°,顶椎旋转度(1.36±0.33)°和(2.34±0.61)°,顶椎偏距(27.4±10.7)mm和(44.7±12.1)mm,主弯的节段数(3.7±2.3)节和(7.1±4.4)节,差异有统计学意义(均P<0.05).结论 两种侧凸的影像学表现有明显区别,结合临床表现可做出正确诊断.%Objective To analyze and compare the Imaging findings of adult idiopathic scoliosis and degenerative scoliosis which were the most common adult scoliosis, and evaluate imaging characteristics. Method The radiological and clinical data of 98 case, among them, 41 cases of adult idiopathic scoliosis and 57 cases of degenerative scoliosis,were analyzed retrospectively. Result There were differences at presence age, sex ratio, anatomic area of scoliosis and apex between two types of adult scoliosis. The analyses and comparison between the two groups revealed significant change in the Cobb angle, involved segment and convex side orientation (all P <0. 05). Conclusion Adult idiopathic scoliosis and degenerative scoliosis show distinctive imaging characteristics. These characteristics combining clinical data are decisive in diagnosis.

  20. Idiopathic scoliosis.

    Science.gov (United States)

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit.

  1. 成人难治性破伤风的诊断与治疗分析%Diagnosis and treatment of adult patients with refractory tetanus

    Institute of Scientific and Technical Information of China (English)

    段劲峰; 唐宇凤; 张芸

    2013-01-01

    目的 探讨成人难治性破伤风的临床表现与治疗方法.方法 回顾性分析5例成人难治性破伤风患者的病历资料.结果 5例患者综合治疗效果不佳,再次对患者所有伤口进行反复清创,并加用头孢三代抗生素,同时延长破伤风抗毒素使用疗程,4例痊愈,1例死亡.结论 应积极控制成人难治性破伤风患者的肺部感染,反复搜寻患者的感染灶并进行彻底清创,必要时可多次使用破伤风抗毒素进行治疗.%Objective To explore the clinical manifestations and therapeutic methods of adults with refractory tetanus. Methods We retrospectively analyzed medical records of the 5 adult patients with refractory tetanus. Results At the beginning,no satisfactory curative effects were obtained after we adopted a variety of treatment methods,including intravenous injection TIG or TAT,nutrition supporting,ventilator, sedative and muscle loose agent. Afterwards, we performed repeated debridement and added cephalosporins of three generation antibiotics. Meanwhile, we extended use of treatment of tetanus antitoxin. As a result ,4 patients recovered and one case died. Conclusions The doctor should actively control pulmonary infection for adult patients with refractory tetanus, repeatedly search all possible focus of infection and thoroughly debride. It should carry out the multiple use of TAT if it is necessary.

  2. A new Leukemia Prognostic Scoring System for refractory/relapsed adult acute myelogeneous leukaemia patients: a GOELAMS study.

    Science.gov (United States)

    Chevallier, P; Labopin, M; Turlure, P; Prebet, T; Pigneux, A; Hunault, M; Filanovsky, K; Cornillet-Lefebvre, P; Luquet, I; Lode, L; Richebourg, S; Blanchet, O; Gachard, N; Vey, N; Ifrah, N; Milpied, N; Harousseau, J-L; Bene, M-C; Mohty, M; Delaunay, J

    2011-06-01

    A simplified prognostic score is presented based on the multivariate analysis of 138 refractory/relapsed acute myeloid leukaemia (AML) patients (median age 55 years, range: 19-70) receiving a combination of intensive chemotherapy+Gemtuzumab as salvage regimen. Overall, 2-year event-free survival (EFS) and overall survival (OS) were 29±4% and 36±4%, respectively. Disease status (relapse Leukemia Prognostic Scoring System was then validated on an independent cohort of 111 refractory/relapsed AML patients. This new simplified prognostic score, using three clinical and biological parameters routinely applied, allow to discriminate around two third of the patients who should benefit from a salvage intensive regimen in the setting of refractory/relapsed AML patients. The other one third of the patients should receive investigational therapy.

  3. Modern Therapies for Idiopathic Inflammatory Myopathies (IIMs): Role of Biologics.

    Science.gov (United States)

    Moghadam-Kia, Siamak; Oddis, Chester V; Aggarwal, Rohit

    2017-02-01

    Despite the lack of placebo-controlled trials, glucocorticoids are considered the mainstay of initial treatment for idiopathic inflammatory myopathy (IIMs) and myositis-associated ILD (MA-ILD). Glucocorticoid-sparing agents are often given concomitantly with other immunosuppressive agents, particularly in patients with moderate or severe disease. As treatment of refractory cases of idiopathic inflammatory myopathies has been challenging, there is growing interest in evaluating newer therapies including biologics that target various pathways involved in the pathogenesis of IIMs. In a large clinical trial of rituximab in adult and juvenile myositis, the primary outcome was not met, but the definition of improvement was met by most of this refractory group of myositis patients. Rituximab use was also associated with a significant glucocorticoid-sparing effect. Intravenous immune globulin (IVIg) can be used for refractory IIMs or those with severe dysphagia or concomitant infections. Anti-tumor necrosis factor (anti-TNF) utility in IIMs is generally limited by previous negative studies along with recent reports suggesting their potential for inducing myositis. Further research is required to assess the role of new therapies such as tocilizumab (anti-IL6), ACTH gel, sifalimumab (anti-IFNα), and abatacept (inhibition of T cell co-stimulation) given their biological plausibility and encouraging small case series results. Other potential novel therapies include alemtuzumab (a humanized monoclonal antibody which binds CD52 on B and T lymphocytes), fingolimod (a sphingosine 1-phosphate receptor modulator that traps T lymphocytes in the lymphoid organs), eculizumab, and basiliximab. The future investigations in IIMs will depend on well-designed controlled clinical trials using validated consensus core set measures and improvements in myositis classification schemes based on serologic and histopathologic features.

  4. Refractory vasculitis

    NARCIS (Netherlands)

    Rutgers, Bram; Kallenberg, Cees G. M.

    2011-01-01

    Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side effects

  5. Acute-onset opioid-induced hyperalgesia in a child with juvenile idiopathic arthritis.

    Science.gov (United States)

    Vijayan, Vini; Moran, Ryan; Elder, Melissa E; Sukumaran, Sukesh

    2012-10-01

    We describe a child with polyarticular juvenile idiopathic arthritis (JIA) presenting with severe diffuse pain refractory to nonsteroidal anti-inflammatory agents and high-dose opioids. Her JIA involved her knees and ankles and was mildly active on etanercept and nonsteroidal anti-inflammatory agents. At presentation, she complained of hip pain progressing to severe diffuse pain and allodynia involving her extremities. No abnormalities were seen in her laboratory parameters and imaging of her lower extremities. After appreciating no substantial benefit by increasing her opioids, her opioids were tapered and discontinued, and this was followed by significant alleviation in her pain, and a diagnosis of opioid-induced hyperalgesia (OIH) was made. Despite reports in adults, the phenomenon of OIH has been reported infrequently in children. To our knowledge, OIH has not been described in children with rheumatologic conditions. We recommend investigating the possibility of OIH when treating a child with JIA and severe refractory pain.

  6. [Juvenile idiopathic arthritis].

    Science.gov (United States)

    Herlin, Troels

    2002-08-19

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis. In addition to the clinical characteristics, genetic and biochemical differences suggest that JIA could be regarded as a general term covering various diseases. Complications described are uveitis, temporomandibular joint affection and growth disturbances. The therapeutic strategy should be planned individually according to age, subtype and disease activity and carried out as teamwork with several specialties. Drugs showing significant effectiveness in controlled studies are primarily methotrexate and sulphasalazine. An immunomodulating agent, etanercept, a soluble TNF alpha-receptor fusion protein, has shown a promising effect in severe polyarticular JIA refractory to methotrexate treatment.

  7. Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage

    Science.gov (United States)

    Oliveira-Ramos, Filipa; Eusébio, Mónica; M Martins, Fernando; Mourão, Ana Filipa; Furtado, Carolina; Campanilho-Marques, Raquel; Cordeiro, Inês; Ferreira, Joana; Cerqueira, Marcos; Figueira, Ricardo; Brito, Iva; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

    2016-01-01

    Objectives To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Methods Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index—articular (JADI-A) and Juvenile Arthritis Damage Index—extra-articular (JADI-E) damage index and disease activity were analysed. Results 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Still's disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Conclusions Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive

  8. Administration of alemtuzumab and G-CSF to adults with relapsed or refractory acute lymphoblastic leukemia: results of a phase II study.

    Science.gov (United States)

    Gorin, Norbert-Claude; Isnard, Françoise; Garderet, Laurent; Ikhlef, Souhila; Corm, Selim; Quesnel, Bruno; Legrand, Ollivier; Cachanado, Marine; Rousseau, Alexandra; Laporte, Jean-Philippe

    2013-10-01

    The outlook for adults with refractory and relapsed acute lymphocytic leukemia (ALL) is poor. CD52 is expressed in most patients with ALL. Alemtuzumab is an anti-CD52 humanized monoclonal antibody. This phase II study assessed the efficacy of alemtuzumab combined with granulocyte-colony stimulating factor (G-CSF) to boost antibody-dependent cell cytotoxicity mediated by neutrophils. Twelve patients with relapsed (n = 11) or refractory (n = 1) ALL, including four relapses postallogeneic stem cell transplantation, were treated and monitored between October 2006 and January 2011. Patients received 1 wk of alemtuzumab every other day at increasing doses of 3, 10, and 30 mg to test tolerance and 30 mg three times a week for 12-18 infusions. If in complete remission (CR), patients received maintenance therapy for 1 wk, every 2 months. G-CSF was administered at 5 μg/kg per day during alemtuzumab administration. The primary endpoint was disappearance of blast cells on a marrow aspirate. CD52 was expressed in all patients. Four patients reached CR. In one additional patient, clearance of blast cells was observed in peripheral blood but not in the marrow. The most frequent adverse events during course 1 of treatment were fever and chills (n = 3), skin rash (n = 3), and bronchospasm (n = 2). Tumor lysis syndrome was observed at treatment initiation in one patient who reached CR. All patients progressed within a few months and all but one died. The surviving patient is still alive after relapse and a second allogeneic stem cell transplantation. This study shows that in relapse/refractory ALL, alemtuzumab with G-CSF can produce good responses of short duration.

  9. Idiopathic inflammatory myopathies: diagnosis, treatment and outcome

    NARCIS (Netherlands)

    van de Vlekkert, J.

    2015-01-01

    The idiopathic inflammatory myopathies (IIM) in adults are a heterogenic group of disorders characterized by muscle inflammation and progressive muscle weakness. This group consists of five subacute-onset disorders: polymyositis (PM) which is extremely rare, (clinically amyopathic) dermatomyositis (

  10. Multidrug resistance-1 in T lymphocytes and natural killer cells of adults with idiopathic thrombocytopenic purpura: effect of prednisone treatment.

    Science.gov (United States)

    López-Karpovitch, Xavier; Graue, Gerardo; Crespo-Solís, Erick; Piedras, Josefa

    2008-07-01

    High P-glycoprotein-mediated multidrug resistance-1 (P-gp/MDR1) activity in lymphocytes from idiopathic thrombocytopenic purpura (ITP) patients may affect disease outcome. ITP treatment includes glucocorticoids that are substrates of P-gp; hence, P-gp functional activity and antigenic expression were assessed by flow cytometry in T and natural killer (NK) cells from ITP patients before and after prednisone therapy. Herein, patients' T and NK cells did not show increased MDR1 functional activity, whereas P-gp antigenic expression was significantly enhanced in both therapy-free and prednisone-treated patients. Prednisone treatment did not significantly modify the function and expression of MDR1 in T and NK cells of ITP patients.

  11. Idiopathic Scoliosis

    OpenAIRE

    2014-01-01

    Introduction. Idiopathic scoliosis is a structural and lateral curvature of the spine for which a currently recognizable cause has not been found and there is no basic evidence for physical and radiographic pathology. Complications. Scoliosis could be a cause of the back pain, deformities, respiratory and cardiology problems. There is a higher risk for decreasing of bone mineral density. Diagnosis and Management. Physical examination, radiography and stereophotogrametry are used in diag...

  12. Modern Therapies for Idiopathic Inflammatory Myopathies (IIMs): Role of Biologics

    OpenAIRE

    Moghadam-Kia, Siamak; Oddis, Chester V.; Aggarwal, Rohit

    2017-01-01

    Despite the lack of placebo-controlled trials, glucocorticoids are considered the mainstay of initial treatment for idiopathic inflammatory myopathy (IIMs) and myositis-associated ILD (MA-ILD). Glucocorticoid-sparing agents are often given concomitantly with other immunosuppressive agents, particularly in patients with moderate or severe disease. As treatment of refractory cases of idiopathic inflammatory myopathies has been challenging, there is growing interest in evaluating newer therapies...

  13. Idiopathic short stature.

    Science.gov (United States)

    Pasquino, A M; Albanese, A; Bozzola, M; Butler, G E; Buzi, F; Cherubini, V; Chiarelli, F; Cavallo, L; Drop, S L; Stanhope, R; Kelnar, C J

    2001-07-01

    Idiopathic short stature (ISS) is a term used to describe the status of children with short stature that cannot be attributed to a specific cause. Many children diagnosed as having ISS have partial GH insensitivity, which can result from disturbances at various points of the GH-IGF-I axis. Several clinical studies on spontaneous growth in ISS showed that adult height was almost in the range of target height. GH treatment led to adult height not significantly higher than the pretreatment predicted adult height in most reports. No metabolic side effects have been observed, even when the dose was higher than in GH deficiency. Manipulation of puberty with gonadotrophin releasing hormone analogues reported by a few authors in a small number of children has shown conflicting results. Long-term psychological benefits of GH therapy for short normal children have not been demonstrated to date.

  14. The Comparison of the Average Thresholds of Auditory Steady-State Response in Adult Subjective Idiopathic Tinnitus and Normal Subjects

    Directory of Open Access Journals (Sweden)

    Zahra Ghasem Ahmad

    2009-12-01

    Full Text Available Background and Aim: Tinnitus is a common symptom among lots of people but little is known about its origins. This study was aimed at comparing the Auditory Steady-State Response (ASSR thresholds in normal cases and patients with subjective idiopathic tinnitus (SIT in order to diagnose its real origins.Materials and Methods: This case-control study was conducted on 19 patients with tinnitus and 24 normal cases aged 18-40 yr.The patients underwent broad medical tests to roll out any background reason for their tinnitus. ASSR thresholds were estimated in both groups at 20 and 40 amplitude modulation. The patients were selected from tinnitus patients in Research Center in Hazrat Rasoul Hospital, Tehran, Iran.Results: The mean ASSR thresholds at 40HZ modulation were worse in tinnitus patients compared to normal ones (p<0.05 but no significant statistical differences was detected at 20HZ. These results were found in both situations in which we averaged both ears thresholds and when we estimated the thresholds of the ears separately.Conclusion: It seems that the origin of the responses of the modulation of 40Hz, primary auditory cortex, midbrain regions and subcortical areas, in these patients is involved or the origin of their tinnitus is related to some kind of problems in these areas, although more investigation is needed about 20Hz.

  15. Idiopathic Scoliosis

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    Jens Ivar Brox

    2014-07-01

    Full Text Available Idiopathic scoliosis (IS is a lifetime condition and is defined as a structural, lateral rotated curvature of the spine of >10° on standing coronal plane radiographs. It should be distinguished from other causes of scoliosis. It can be classified as infantile, juvenile, and adolescent according to age. As a rule of thumb, about 80% of all curves are idiopathic, right convex thoracic, and present in otherwise healthy girls at the beginning of puberty. A family member most commonly detects scoliosis. The structural asymmetry of the spine is best observed by asking the patient to bend forward. IS is often seen in more than one member of a family, but the aetiology remains unknown. Multiple genes are likely to be involved with incomplete penetrance and variable expressivity. Early detection by screening allows for monitoring curve progression and timely initiation of bracing, but school screening is controversial and practises vary worldwide. Most patients have minor scoliosis and treatment is generally not recommended for patients with curves 45°. Scoliosis surgery was not successful until the introduction of Harrington’s instrumentation in the 1960s. Modern instrumentation has evolved from the Cotrel-Dubousset system in the 1980s, and a variety of methods are available today. Although scoliosis may be a burden, long-term studies suggest that a good quality of life is maintained in most patients.

  16. Outcome in adults with juvenile idiopathic arthritis. Comparison of the DUTCH-AIMS2 between JIA and RA.

    NARCIS (Netherlands)

    Bruinooge, LN; Moussaoui, R El; Taal, E; Dijkmans, B.A.C.; Soesbergen, RM Van

    2003-01-01

    or = 4.80). The internal consistency and the validity of the DUTCH-AIMS2 were satisfactory. CONLUSIONS: Overall, the health outcome of oligoarticular and polyarticular adult JIA and young RA patients was quite good. The DUTCH-AIMS2 can be used as an instrument in outcome studies in JIA patients.

  17. Nivolumab and Dasatinib in Treating Patients With Relapsed or Refractory Philadelphia Chromosome Positive Acute Lymphoblastic Leukemia

    Science.gov (United States)

    2016-08-25

    B Acute Lymphoblastic Leukemia With t(9;22)(q34;q11.2); BCR-ABL1; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

  18. High Throughput Drug Sensitivity Assay and Genomics- Guided Treatment of Patients With Relapsed or Refractory Acute Leukemia

    Science.gov (United States)

    2016-11-14

    Acute Leukemia of Ambiguous Lineage; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

  19. Repeat microvascular decompression for recurrent idiopathic trigeminal neuralgia

    NARCIS (Netherlands)

    Bakker, Nicolaas A.; van Dijk, J. Marc C.; Immenga, Steven; Wagemakers, Michiel; Metzemaekers, Jan D. M.

    2014-01-01

    Object. Microvascular decompression (MVD) is considered the method of choice to treat idiopathic trigeminal neuralgia (TN) refractory to medical treatment. However, repeat MVD for recurrent TN is not well established. In this paper, the authors describe a large case series in which patients underwen

  20. Mental Health of Adults Treated in Adolescence with Scoliosis-Specific Exercise Program or Observed for Idiopathic Scoliosis

    OpenAIRE

    2014-01-01

    Objective. To examine general mental health in adult males and females, who in adolescence participated in a scoliosis-specific therapeutic exercise program or were under observation due to diagnosis of scoliosis. Design. Registry-based, cross-sectional study with retrospective data collection. Methods. Sixty-eight subjects (43 women) aged 30.10 (25–39) years, with mild or moderate scoliosis (11–36° Cobb angle), and 76 (38 women) nonscoliotic subjects, aged 30.11 (24–38) years, participated. ...

  1. REFRACTORY THROMBOCYTOPENIA AND NEUTROPENIA: A DIAGNOSTIC CHALLENGE

    Directory of Open Access Journals (Sweden)

    Emmanuel Gyan

    2015-02-01

    Full Text Available Background. The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT, and refractory neutropenia (RN, characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors.  Diagnosing RT first requires exclusion of familial thrombocytopenia, chronic auto-immune thrombocytopenia, concomitant medications, viral infections, or hypersplenism. Diagnosis of RN should also be made after ruling out differential diagnoses such as ethnic or familial neutropenia, as well as acquired, drug-induced, infection-related or malignancy-related neutropenia. An accurate quantification of dysplasia should be performed in order to distinguish RT or RN from the provisional entity named idiopathic cytopenia of unknown significance (ICUS. Cytogenetic analysis, and possibly in the future somatic mutation analysis (of genes most frequently mutated in MDS, and flow cytometry analysis aberrant antigen expression on myeloid cells may help in this differential diagnosis. Importantly, we and others found that, while isolated neutropenia and thrombocytopenia are not rare in MDS, those patients can generally be classified (according to WHO 2008 classification as refractory cytopenia with multilineage dysplasia or refractory anemia with excess blasts, while RT and RN (according to WHO 2008 are quite rare.These results suggest in particular that identification of RT and RN as distinct entities could be reconsidered in future WHO classification updates.

  2. Prognostic Factors for Refractory Status Epilepticus

    Directory of Open Access Journals (Sweden)

    J. Gordon Millichap

    2013-03-01

    Full Text Available Researchers at the Mayo Clinic, Rochester, MN studied the outcome and identified prognostic factors for refractory status epilepticus (RSE in 54 adult patients, median age 52 years [range 18-93].

  3. Autologous Stem Cell Transplant Followed by Donor Stem Cell Transplant in Treating Patients With Relapsed or Refractory Lymphoma

    Science.gov (United States)

    2016-02-23

    Prolymphocytic Leukemia; Recurrent Adult Hodgkin Lymphoma; Recurrent Childhood Hodgkin Lymphoma; Recurrent Childhood Non-Hodgkin Lymphoma; Recurrent Chronic Lymphocytic Leukemia; Recurrent Non-Hodgkin Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hodgkin Lymphoma; Refractory Non-Hodgkin Lymphoma; Refractory Small Lymphocytic Lymphoma; T-Cell Chronic Lymphocytic Leukemia; T-Cell Prolymphocytic Leukemia

  4. New-onset refractory status epilepticus in an adult with an atypical presentation of cat-scratch disease: successful treatment with high-dose corticosteroids.

    Science.gov (United States)

    Laswell, Emily M; Chambers, Kasandra D; Whitsel, Danielle R; Poudel, Kiran

    2015-06-01

    New-onset refractory status epilepticus (NORSE) is defined as a sudden onset of refractory status epilepticus in patients who do not have a history of epilepsy. It is a neurologic emergency, and determining the underlying etiology is an important factor for effectively managing and predicting the prognosis of NORSE. We describe the case of a 28-year-old woman who was hospitalized with NORSE secondary to an unknown etiology. She did not respond to traditional anticonvulsant therapy, including benzodiazepines, fosphenytoin, propofol, and levetiracetam. The patient was placed on continuous electroencephalography (EEG) monitoring and was treated further with multiple antiepileptics, which were titrated aggressively based on EEG readings and therapeutic drug levels; despite this treatment, EEG monitoring revealed continued seizures. Thus, high-dose corticosteroids were started for seizure control. Her workup included computed tomography and magnetic resonance imaging of the head, a lumbar puncture, toxicology screening, and extensive testing for multiple infectious and inflammatory etiologies. The patient's history revealed recent exposure to a new cat. Serologic results were positive for Bartonella henselae, and she was diagnosed with cat-scratch disease (CSD). She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD. Doxycycline 100 mg and rifampin 300 mg twice daily were added to the patient's anticonvulsant and corticosteroid therapy. She was hospitalized for a total of 26 days and discharged with only minor neurologic impairment (short-term memory deficits and minor cognitive problems). The patient was discharged receiving antiepileptics, antibiotics, and a corticosteroid taper. To our knowledge, this is the first clinically known case of NORSE secondary to CSD without typical CSD symptoms in the adult population. The patient failed to respond to traditional anticonvulsant therapy alone. With the addition of high

  5. Mental Health of Adults Treated in Adolescence with Scoliosis-Specific Exercise Program or Observed for Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Maciej Płaszewski

    2014-01-01

    Full Text Available Objective. To examine general mental health in adult males and females, who in adolescence participated in a scoliosis-specific therapeutic exercise program or were under observation due to diagnosis of scoliosis. Design. Registry-based, cross-sectional study with retrospective data collection. Methods. Sixty-eight subjects (43 women aged 30.10 (25–39 years, with mild or moderate scoliosis (11–36° Cobb angle, and 76 (38 women nonscoliotic subjects, aged 30.11 (24–38 years, participated. The time period since the end of the exercise or observation regimes was 16.5 (12-26 years. Beck Depression Inventory (BDI and General Health Questionnaire (GHQ-28 scores were analyzed with the χ2 and U tests. Multiple regression analyses for confounders were also performed. Results. Intergroup differences of demographic characteristics were nonsignificant. Scoliosis, gender, participation in the exercise program, employment, and marital status were associated with BDI scores. The presence of scoliosis and participation in the exercise program manifested association with the symptoms. Higher GHQ-28 “somatic symptoms” subscale scores interacted with the education level. Conclusions. Our findings correspond to the reports of a negative impact of the diagnosis of scoliosis and treatment on mental health. The decision to introduce a therapeutic program in children with mild deformities should be made with judgment of potential benefits, risks, and harm.

  6. Cough in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Mirjam J.G. van Manen

    2016-09-01

    Full Text Available Many patients with idiopathic pulmonary fibrosis (IPF complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.

  7. Rituximab in treatment of idiopathic glomerulopathy

    Directory of Open Access Journals (Sweden)

    Kamel El-Reshaid

    2012-01-01

    Full Text Available The aim of our study was to assess the role of rituximab (Mabthera in the treatment of patients with corticosteroid-resistant and calcineurin-inhibitors ± cellcept refractory idiopathic nephrotic syndrome (INS. A total of 83 patients who had required the previous treatment for a minimum of two years were included in the study. Our protocol included the use of rituximab in four-weekly slow infusions. Five patients were excluded as they could not tolerate rituximab infusion for allergic reaction. As expected, none of the patients had a decline in the total circulating lymphocyte counts yet all had achieved decline of their initially normal CD20 to < 0.5% one month after infusion. The decline persisted for eight to ten months later. In the minimal change disease (MCD group, 31 of the 32 patients had complete remission (CR and were off any immunosuppressive therapy and one of the previous non-responders (NR did not respond. Excluding two patients who had required retreatment, the others remained in CR (17 up to 28 months and six up to 36 months. Treatment with rituximab resulted in amelioration of NS in 17 of the 18 patients with focal segmental glomerulosclerosis (FSGS, while only one patient remained NR. Although renal function remained stable, proteinuria reappeared by eight to 12 months. Retreatment with rituximab resulted in a similar response with stable kidney function. In the 28 patients with membranous glomerulopathy (MG, 24 had achieved CR. Two patients failed to respond and two had partial remission. By 12 months, all patients relapsed. The response was within one month following treatment in patient with MCD, but was gradual within three months in FSGS and MG. Relapsers in all groups responded in a similar pattern to repeat dosing with the drug subsequently. Our prospective study represents an adequate number of patients with biopsy-proven subgroups of INS in both children and adults with long-term follow-up of treatment with rituximab

  8. Managing juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Hawkins, Madeleine J; Dick, Andrew D; Lee, Richard J W; Ramanan, Athimalaipet V; Carreño, Ester; Guly, Catherine M; Ross, Adam H

    2016-01-01

    Bilateral chronic anterior uveitis is an extra-articular feature of juvenile idiopathic arthritis. Although figures vary, uveitis occurs in approximately 11%-13% of patients with this disease and is most commonly associated with the female gender, oligoarthritis, and presence of antinuclear antibodies. The disease has an insidious onset and is often asymptomatic. Managing patients with juvenile idiopathic arthritis-associated uveitis remains challenging as the disease may prove to be refractory to traditional treatment regimens. Stepwise immunomodulatory therapy is indicated, with new biologic drugs being used last in cases of refractory uveitis. Small scale studies and practice have provided the evidence to undertake randomized control trials to evaluate the efficacy, safety, and cost-effectiveness of anti-tumor necrosis factor-α therapies, such as infliximab and adalimumab. These have demonstrated promising results, with further data awaited from ongoing trials for adalimumab (as SYCAMORE and ADJUVITE trials). Lower grade evidence is supporting the use of newer biologics such as rituximab, daclizumab, tocilizumab, and abatacept in those cases refractory to anti-tumor necrosis factor-α therapy.

  9. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  10. Polymyositis - adult

    Science.gov (United States)

    ... rash is a sign of a similar condition, dermatomyositis . Common symptoms include: Muscle weakness in the shoulders ... in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. ...

  11. 来氟米特联合阿魏酸钠治疗难治性原发性肾病综合征的临床研究%The Clinical Research on Leflunomide Jointed Sodium Ferulate in The treatment of Refractory Idiopathic Nephritic Syndrome

    Institute of Scientific and Technical Information of China (English)

    缪旭

    2013-01-01

    Objective:To explore the curative effect that leflunomide jointed sodium ferulate in the treatment of refractory idiopathic nephritic syndrome. Method:80 patients were randomly divided into the treatment group and the control group,which have been diagnosed as refractory idiopathic nephritic syndrome in the Nephrology Department of our hospital from February 2012 to January 2013.Each group contained 40 patients.The methods of treatment in the control group consisted of cytotoxic drug (cyclophosphamide) and routine treatments,such as using glucocorticoid,diuresis,reducing urine protein,anti-hyperlipidemia and anticoagulation. Excepting the routine treatments,the treatment group also added that,oral leflunomide piece (25 mg bid,the first three days,then 25 mg qd),and sodium ferulate (0.3 g,iv drip qd). The treatment course was 1 month. One month later,we observed the changes of the clinical symptoms of nephrotic syndrome. In addition,we recorded the changes of 24 hours urinary protein,serum albumin,total cholesterol and quantitative serum creatinine,as well as the adverse drug reaction.Result:After 1 month of therapy,the complete response rates in the treatment group were significantly higher than the control group(P<0.001). In the meantime,24 hours urinary protein,total cholesterol and quantitative serum creatinine in the treatment group were significantly lower compared to the control group (P<0.05). On the contrary,the serum albumin was significantly higher in the treatment group(P<0.05).Conclusion:Leflunomide jointed sodium ferulate in the treatment of refractory idiopathic nephritic syndrome have an obvious positive effect.%目的:探讨来氟米特联合阿魏酸钠治疗难治性原发性肾病综合征的疗效。方法:选择2012年2月-2013年1月广东省农垦中心医院肾内科收治的80例难治性原发性肾病综合征患者,随机分为治疗组和对照组,各40例。对照组予激素、利尿、减少尿蛋白、降血脂、抗凝等

  12. 小剂量利妥昔单抗治疗难治性血小板减少性紫癜序贯维持的临床研究%Clinical research of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王吉刚; 周凡; 刘彦琴; 刘景华; 白颖; 徐宪平; 董援利; 孟广晗; 佟丹江

    2016-01-01

    Objective To investigate the efficacy and safety of low-dose rituximab therapy and sequential maintenance for patients with refractory idiopathic thrombocytopenic purpura. Methods Thirty-three patients with refractory idiopathic thrombocytopenic purpura received intravenous rituximab at the dose of 100 mg once a week for 4 consecutive weeks. Complete blood cell count and serum concentrations of immunoglobulin (IgG,IgM and IgA) were monitored regularly. The numbers of CD3+ and CD19+ CD20+ lymphocyte cells were assayed by flow cytometry before and after therapy. Twenty-five patients with responses(complete response and response) were divided into maintained group (12 patients) and control group (13 patients) by random digits table method. The patients in maintained group were treated with rituximab 100 mg every 6 months. The efficacy of maintenance therapy was evaluated through long-term follow-up. Results The complete response(CR) rate, response (R) rate and no response(NR) rate were 48.48%(16/33), 27.27%(9/33) and 24.24% (8/33), respectively. As a result, total effective rate was 75.76% (25/33). There were no significant changes of peripheral blood white blood cell count,hemoglobin,serum immunoglobulin and CD3+lymphocyte counts before and after treatment (P>0.05). However, CD19+ CD20+ cells were almost depleted in patients treated with rituximab: (3.71±2.64)×106/L vs. (279.33±92.78)×106/L, P<0.01. Five patients suffered from allergic response, and 1 patient developed pneumonia and respiratory failure. The relapse rates of maintained group and control group were 1/12 and 4/13, respectively. Conclusions Treatment with low-dose rituximab may be an effective and safe approach in patients with idiopathic thrombocytopenic purpura. Relapse rates can be decreased through maintenance therapy with refractory low-dose rituximab. However, the optimal therapeutic schedule need further investigation.%目的:探讨小剂量利妥昔单抗治疗难治性特发性血小板

  13. Refractory Celiac Disease

    Directory of Open Access Journals (Sweden)

    K Khatami

    2014-04-01

    Full Text Available Refractory celiac disease (RCD is when malabsorption symptoms and villous atrophy persist despite strict adherence to a gluten free diet (GFD for more than 12 months and other causes of villous atrophy have been ruled out.  RCD is considered a rare disease and almost exclusively occurs in adults. Persistent diarrhea, abdominal pain, weight loss are the most common symptoms in RCD. Also, anemia, fatigue, malaise, thromboembolic events and coexisting autoimmune disorders are frequent. Diagnosis of RCD is based on other causes of unresponsiveness to the GFD, particularly collagenous sprue, ulcerative jejunitis, and enteropathy-associated T-cell lymphoma. Many disorders such as autoimmune enteropathy, tropical sprue, common variable immunodeficiency, and intolerance to non-gluten dietary proteins may have similar histological findings but not necessarily identical with CD and therefore should be excluded. Repeat intestinal biopsy may help to differentiate causes of non-responsive CD associated with ongoing villous atrophy (e.g., gluten contamination, small-bowel bacterial overgrowth, RCD. There are 2 subtypes of RCD according to absence (type I or presence (type II of an abnormal intraepithelial lymphocyte population. RCD type 1 usually becomes better with a combination of aggressive nutritional support, adherence to GFD, and pharmacologic therapies such as prednisone, budesonide and azathioprine. For RCD type 2, more aggressive therapeutic approach is needed since clinical response to therapies is less certain and may evolve into aggressive enteropathy associated T-cell lymphoma and the prognosis is poor.   Key words: Celiac Disease, Refractory.  

  14. High-Dose Y-90-Ibritumomab Tiuxetan Added to Reduced-Intensity Allogeneic Stem Cell Transplant Regimen for Relapsed or Refractory Aggressive B-Cell Lymphoma

    Science.gov (United States)

    2016-07-08

    Post-Transplant Lymphoproliferative Disorder; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent B-Cell Non-Hodgkin Lymphoma; Recurrent Burkitt Lymphoma; Refractory B-Cell Non-Hodgkin Lymphoma; Refractory Burkitt Lymphoma; Refractory Diffuse Large B-Cell Lymphoma

  15. "Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V

    2013-10-15

    The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children.

  16. Idiopathic chondrolysis - diagnostic difficulties

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Scougall, J.

    1984-07-01

    Four cases of idiopathic chondrolysis of the hip in three white girls and one Maori girl are reported. The authors stress the causes why a disease with characteristic clinical and radiographic appearances and normal biochemical findings presents diagnostic difficulties. It is suspected that idiopathic chondrolysis is a metabolic disorder of chondrocytes, triggered by environment circumstances in susceptible individuals. Idiopathic chondrolysis is probably one of the most common causes of coxarthrosis in women.

  17. Treatment Options for Primary Refractory/Recurrent Hodgkin Lymphoma in Children and Adolescents

    Science.gov (United States)

    ... Primary Refractory/Recurrent Hodgkin Lymphoma in Children and Adolescents Primary refractory Hodgkin lymphoma is lymphoma that continues ... treated with an adult treatment regimen . Children and adolescents may have treatment-related side effects that appear ...

  18. Osteoarthritis, osteoarthrosis, and idiopathic condylar resorption.

    Science.gov (United States)

    Mercuri, Louis G

    2008-05-01

    The term "osteoarthritis" has classically been defined as a low-inflammatory arthritic condition. The term "osteoarthrosis," a synonym for osteoarthritis in the medical orthopedic literature, has recently come to be identified in the dental/temporomandibular joint (TMJ) disorders literature with any noninflammatory arthritic condition that results in similar degenerative changes as in osteoarthritis. The term "idiopathic condylar resorption," also known as "progressive condylar resorption," is described as a dysfunctional remodeling of the TMJ manifested by morphologic change, decreased ramal height, progressive mandibular retrusion in the adult, or decreased mandibular growth in the juvenile. This article discusses the diagnosis and management of osteoarthritic TMJ disorders and idiopathic condylar resorption.

  19. Exergaming as a viable therapeutic tool to improve static and dynamic balance among older adults and people with idiopathic Parkinson’s disease: a systematic review and meta-analysis

    Directory of Open Access Journals (Sweden)

    Dale Michael Harris

    2015-09-01

    Full Text Available The use of virtual reality games (known as ‘exergaming’ as a neurorehabilitation tool is gaining interest. Therefore, we aim to collate evidence for the effects of exergaming on the balance and postural control of older adults and people with idiopathic Parkinson’s disease (IPD. Six electronic databases were searched, from inception to April 2015, to identify relevant studies. Standardised mean differences (SMD and 95% confidence intervals (CI were used to calculate effect sizes between experimental and control groups. I2 statistics were used to determine levels of heterogeneity. 309 older adults and 74 people with IPD were assessed across eleven studies. The results showed that exergaming improved static balance (SMD 1.069, 95% CI 0.563 to 1.576, postural control (SMD 0.826, 95% CI 0.481 to 1.170 and dynamic balance (SMD -0.808, 95% CI -1.192 to -0.424 in healthy older adults. Two PD studies showed an improvement in static balance (SMD 0.124, 95% CI -0.581 to 0.828 and postural control (SMD 2.576, 95% CI 1.534 to 3.599. Our findings suggest that exergaming might be an appropriate therapeutic tool for improving balance and postural control in older adults, but more large-scale trials are needed to determine if the same is true for people with IPD.

  20. IDIOPATHIC PYODERMA GANGRENOSUM: REPORT IN A THREE YEAR OLD CHILD

    Directory of Open Access Journals (Sweden)

    Vidyadhar Kinhal

    2015-01-01

    Full Text Available Pyoderma gangrenosum is a rare, non - infectious and destructive skin disease. Its exact etiopathogenesis is not clearly understood and is believed to be due to immune dysfunction. Several theories have been postulated; however none are consistent in all patients. Pyoderma gangrenosum mostly affects adults and rarely in children. An underlying systemic diseas e is often seen in most of the cases. A three - year - old male child presented with a non - healing ulcer over left thigh. Clinical features, histopathology, other investigations and dramatic response to corticosteroids suggested a diagnosis of idiopathic pyo derma gangrenosum. Associated idiopathic thrombocytosis was also seen. Idiopathic pyoderma gangrenosum is very rare in children. Idiopathic pyoderma gangrenosum associated with idiopathic thrombocytosis and keloidal scarring has not been reported earlier

  1. Clinical study on recombinant human thrombopoietin in the treatment of refractory idiopathic thrombocytopenic purpura%重组人血小板生成素治疗难治性特发性血小板减少性紫癜临床观察

    Institute of Scientific and Technical Information of China (English)

    张晓丽; 刘延方; 孙慧; 谢新生; 姜中兴; 孙玲; 万鼎铭; 刘林湘

    2010-01-01

    Objective To observe the efficacy and toxicity of recombinant human thrombopoietin (rhTPO)in the treatment of chronic refractory idiopathic thrombocytopenic purpura(ITP).Methods Twenty- four patients with chronic ITP were administered with rhTPO subcutaneously at 300 IU/(kg · d)for 14 days or until the platelet count > 100 × 109/L.Results Out of 24 patients with ITP, 12 cases showed significant response and 5 cases showed good response to the treatment with rhTPO.The peak platelet count after treatment was(129 ± 97)× 109/L, significantly higher than the mean platelet count [(10 ±5)× 109/L]before treatment(t = 5.822, P <0.01).The agent was well tolerated in most patients.Conclusions rhTPO exerts satisfactory efficacy in the treatment of chronic ITP with mild side effects.%目的 观察重组人血小板生成素(rhTPO)治疗慢性难治性特发性血小板减少性紫癜(ITP)的临床疗效及不良反应.方法 24例慢性难治性ITP患者皮下注射rhTPO 300 IU/(kg·d),疗程14 d或血小板>100×109/L后停药.结果 24例患者rhTPO治疗后,显效12例,有效5例,用药前血小板平均值为(10±5)×109/L,治疗后血小板计数的升高的最高值为(129±97)×109/L,与用药前比较差异有统计学意义(t=5.822,P<0.01).多数患者对药物耐受良好.结论 rhTPO治疗慢性难治性ITP具有良好的疗效,不良反应轻微.

  2. Bortezomib for refractory antibody-mediated cardiac allograft rejection.

    Science.gov (United States)

    Eckman, Peter M; Thorsgard, Marit; Maurer, David; Kim, Youngki; Alloway, Rita R; Woodle, E Steve

    2009-01-01

    This experience demonstrates that a bortezomib-based regimen provided effective therapy for late, refractory AMR in an adult heart transplant recipient and was well tolerated. This remarkably positive experience despite the refractory nature of the AMR episode argues strongly for continued evaluation of bortezomib use in this patient population.

  3. Idiopathic membranous nephropathy complicated with malignant hypertension: a case report

    Institute of Scientific and Technical Information of China (English)

    TAO Jian-ling; LI Hang; WEN Yu-bing; LI Xue-wang

    2007-01-01

    @@ Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults. Its insidious onset and progression often hinder timely renal biopsy and early diagnosis delaying treatment while worsening prognosis. The complication of malignant hypertension(MHT) is rarely seen in idiopathic MN. To provide a better understanding of the disease we report a case of idiopathic MN diagnosed by biopsy six years after onset.

  4. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

    Full Text Available Scoliosis is called idiopathic when no other underlying disease can be identified. The etiology of adolescent idiopathic scoliosis (AIS is still unknown despite many years of research effort. Theories on AIS's etiology have included mechanical, hormonal, metabolic, neuromuscular, growth, and genetic abnormalities. Skeletally immature patients with adolescent idiopathic scoliosis are at risk of curve progression. The adolescent onset of severe idiopathic scoliosis has traditionally been evaluated using standing posteroanterior radiographs of the full spine to assess lateral curvature with the Cobb method. Scoliosis in children of school age and above primarily occurs in girls. The therapeutic goal in children is to prevent progression. In children, scoliosis of 20 and deg; or more should be treated with a brace, and scoliosis of 45 and deg; or more with surgery. [Arch Clin Exp Surg 2014; 3(3.000: 174-182

  5. Review of idiopathic pancreatitis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins,autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted.

  6. Clinical Therapentic Efficacy of Rituximab for Relapsed and Refractory Idiopathic Thromocytopenic Purpura%利妥昔单克隆抗体治疗难治复发性特发性血小板减少性紫癜临床疗效初步评估

    Institute of Scientific and Technical Information of China (English)

    周立宇; 张征; 宋陆茜; 张曦; 苏基滢; 李晓; 常春康

    2012-01-01

    The aim of this study was to evaluate the effect of rituximab treatments for refractory and relapsed idiopathic thrombocytopenic purpura (ITP). 18 patients with refractory and relapsed ITP who received 22 courses of rituximab treatments from January 2007 to December 2010 were analyzed retrospectively. Rituximab was given at a dose of 375 mg/m2 intravenously weekly for a continuous 4 weeks. The results indicated that responses were achieved in 15 of 22 (68% ) courses, out of which complete responses were achieved in 10 of 22 (45% ) courses, partial and minimal responses were achieved in 5 of 22 (23% ) courses, and no responses were achieved in 7 of 22 (32% ) courses. The median time of response was 3 weeks (1-17 weeks) from the start of treatment and median duration of response was 13 weeks (1 week -42 months). The responses were mostly short -sustained and follow-up median time was 20 months (1-52 months). The responses of 8 patients (36% ) sustained for over 6 months, 6 patients (27% ) sustained for over 1 year, and 4 patients also showed sustained response at last visit of follow-up. Previous splenectomy resulted in a poor response (P = 0. 037). Patients who failed in rituximab treatment and prior received multiple treatments including splenectomy, had a poor response to further therapies. It is concluded that rituximab is well tolerated by patients and is useful in some patients with relapsed and refractory ITP, however, only about 20% patients can achieve sustained remissions. The patients who failed in rituximab treatment has a poor response to further treatment.%本研究评价利妥昔单克隆抗体治疗难治复发性特发性血小板减少性紫癜(ITP)的临床疗效.选择我院2007年1月-2010年12月期间确诊为难治、复发性ITP患者18例,给予利妥昔单克隆抗体375mg/m2静脉滴注,每周1次,连用4周为1个疗程.结果表明,18例患者接受22个疗程的利妥昔单克隆抗体治疗,治疗有效率为68%,完全反应率为45

  7. Adult patients with relapsed and/or refractory B-precursor Acute lymphoblastic leukaemia | EU Clinical Trials Register [EU Clinical Trials Register

    Lifescience Database Archive (English)

    Full Text Available alutare l’efficacia dell’anticorpo BiTE® blinatumomab rispetto alla chemioterapia standard in soggetti adulti affetti da leucemia...rispetto alla chemioterapia standard, in soggetti adulti con leucemia linfoblastica acuta che non hanno risp... with relapsed and/or refractory B-precursor Acute lymphoblastic leukaemia Soggetti adulti affetti da leucemia...esponding to therapy Pazienti adulti con leucemia linfoblastica acuta - un tumore del sangue e midollo - che...alattia- Neoplasia prostatica intraepiteliale senza evidenza di tumore alla prostata .2 . diagnosi di leucemia

  8. XK469R in Treating Patients With Refractory Hematologic Cancer

    Science.gov (United States)

    2013-02-07

    Adult Acute Basophilic Leukemia; Adult Acute Eosinophilic Leukemia; Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Blastic Phase Chronic Myelogenous Leukemia; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Chronic Lymphocytic Leukemia; Relapsing Chronic Myelogenous Leukemia; Secondary Myelodysplastic Syndromes; Stage III Chronic Lymphocytic Leukemia; Stage IV Chronic Lymphocytic Leukemia; Untreated Adult Acute Myeloid Leukemia

  9. Silica Refractory Bricks

    Institute of Scientific and Technical Information of China (English)

    Yu Lingyan; Peng Xigao

    2011-01-01

    @@ 1.Scope This standard specifies the classification,technical requirements,test methods,quality appraisal procedures,packing,marking,transportation,storage,and quality certificate of silica refractory bricks.This standard is applicable to silica refractory bricks with single weight≤40 kg.

  10. Developing Refractories for Exporting

    Institute of Scientific and Technical Information of China (English)

    PANShang-xin

    1996-01-01

    The total output of China refractory products and export volume of China refractory raw materials rank the first in the world,In future the task is to improve the quality of the products to enlarge the variety and to expand export.

  11. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

    Full Text Available Atherosclerosis is a chronic inflammatory disease of the arteries. Clinical consequences of the atherosclerotic process occur in the adult population, however atherosclerotic process begins in childhood. The classic risk factors for atherosclerosis include obesity, dyslipidaemia, age, gender or family history. In recent years, attention has been drawn to the similarity between atherosclerotic inflammatory processes and inflammatory changes in the course of systemic connective tissue disease, in particular systemic lupus etythematosus (SLE or rheumatoid arthritis (RA. There is also observed the similarity of the pathogenetic background of development of atherosclerosis and juvenile idiopathic arthritis (JIA. Elevated levels of pro-inflammatory cytokines are observed in the course of juvenile idiopathic arthritis. Also homocysteine concentrations, which may play a significant role in the development of atherosclerotic lesions, are observed higher in patients with JIA. Some studies revealed higher carotid intima-media thickness (IMT index values in children with JIA. In view of the fact that atherosclerotic process begins as early as in childhood, the introduction of appropriate preventive measures in children is a matter of utmost importance.

  12. Development of improved refractories

    Energy Technology Data Exchange (ETDEWEB)

    Wereszczak, A.A.; Ferber, M.K.; Liu, K.C. [Oak Ridge National Lab., TN (United States); Moore, R.E. [Univ. of Missouri, Rolla, MO (United States)

    1997-04-01

    The goal of the proposed project is to provide expertise and facilities for the high temperature mechanical properties characterization of refractory materials which are of interest to the US DOE`s Office of Industrial Technologies Advanced Industrial Materials Project. Initially the project would establish dedicated refractory testing facilities which would be capable of generating representative engineering creep and high temperature modulus of elasticity (MOE) data to a temperature of 3300{degrees}F (1815{degrees}C) in ambient air. The generated engineering creep and MOE data would serve R&D requirements of refractories-manufacturers and its glass-manufacturer end-users and designers. The relevance of this effort to the refractory and glass-making industries would be ensured by coordinating the research activities through a membership with Alfred University`s Center for Glass Research (CGR) Satellite Center at the University of Missouri-Rolla (UMR), an NSF Center. Valid engineering creep and high temperature MOE data currently do not exist for almost all commercial refractories. Refractory end-users such as glass-manufacturers require such data for efficient and economical design of their various glass-melting furnace superstructures (e.g., furnace crowns). Refractories in glass production furnaces may be subjected to extreme temperatures as high as 3200{degrees}F (1760{degrees}C). With the simultaneous imposition of mechanical and thermal stresses, creep deformation of the refractory material will assuredly occur as a consequence. Designers must ensure that the structural integrity is maintained, so these high temperature deformations must be considered for successful glass furnace superstructure design. These criteria can only be satisfied with the utilization of representative engineering creep and high temperature MOE data for the refractory materials that are chosen for the design of the refractory superstructures.

  13. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

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

    2013-01-01

    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...

  14. Propiverine: a review of its use in the treatment of adults and children with overactive bladder associated with idiopathic or neurogenic detrusor overactivity, and in men with lower urinary tract symptoms.

    Science.gov (United States)

    McKeage, Kate

    2013-01-01

    Propiverine is a well established antimuscarinic agent with a mixed mode of action in the treatment of symptoms associated with overactive bladder (OAB). As well as blocking muscarinic receptors in the detrusor muscle, the drug also inhibits cellular calcium influx, thereby diminishing muscle spasm. In patients with symptoms of OAB resulting from idiopathic detrusor overactivity (IDO) or neurogenic detrusor overactivity (NDO), propiverine demonstrated dose-dependent efficacy and tolerability, with adverse events consistent with those associated with all antimuscarinic agents. In adults with IDO, propiverine demonstrated similar efficacy to that of other antimuscarinic agents (including solifenacin, tolterodine, oxybutynin and imidafenacin) and, in adults with NDO, propiverine and oxybutynin demonstrated similar efficacy. Propiverine was generally well tolerated in these patient populations, with a lower incidence of dry mouth than that associated with oxybutynin. In men with lower urinary tract symptoms (LUTS), and in whom the presence of benign prostatic enlargement (BPE) was implicated, propiverine administered as add-on therapy to an α(1)-adrenoceptor antagonist demonstrated similar or superior efficacy to that achieved with an α(1)-adrenoceptor antagonist alone, and combination therapy was particularly effective in patients with urinary storage symptoms. Combination therapy was generally well tolerated, but was associated with a higher incidence of adverse events than an α(1)-adrenoceptor antagonist alone. In children and adolescents with IDO/OAB or NDO, propiverine was generally more effective and better tolerated than oxybutynin. In conclusion, propiverine provides a valuable option for the treatment of adults and children with OAB associated with IDO or NDO, and in men with storage LUTS.

  15. [Adult-onset case of idiopathic neurodegeneration with brain iron accumulation without mutations in the PANK2 and PLA2G6 genes].

    Science.gov (United States)

    Saiki, Shinji; Sekine, Takeshi; Ueno, Yuji; Yoshino, Hiroyo; Takahashi, Junko; Tani, Yoshihiko; Kambe, Yasunori; Motoi, Yumiko; Hattori, Nobutaka

    2009-08-01

    A 47-year-old man with a 15-year history of bipolar disorder treated with anti-depressants, lithium carbonate or neuroleptics was admitted because of marked difficulty in gait and speech. At the age 45, he was unable to walk without bilateral assists and became a wheel-chair state. There was no family history and his mother, father and younger sister were neurologically free. General physical examinations revealed no abnormalities. Neurologically, he was moderately demented (mini mental state examination: 18/30) and showed bilateral horizontal gaze nystagmus, parkinsonism, cerebellar ataxia, dysarthria and moderate spastic paraparesis. No involuntary movements were noted. Wet blood smear showed acanthocytes, while blood chemistries revealed no abnormalities including levels of serum creatine kinase, hepatic enzymes and blood beta-lipoprotein. Kell antigen expressions of the red blood cells were within normal limit. Western blot analysis with anti-chorein antibody detected normal chorein expression levels of the red blood cells. Cranial MRI showed severe symmetric atrophy of the frontotemporal lobes, caudate nuclei, putamen, and brainstem. Also, MRI-gradient echo showed symmetric iron accumulation in the medial portion of the globus pallidus without surrounding high intensity areas, so called "eye-of-the-tiger sign". Genetic analyses revealed no mutations in the PANK2 and PLA2G6 genes. Therefore, he was diagnosed as idiopathic neurodegeneration with brain iron accumulation (NBIA). These findings suggest that NBIA is heterogeneous and other additional genes remain to be found.

  16. Risk HLA-DQA1 and PLA(2)R1 alleles in idiopathic membranous nephropathy

    NARCIS (Netherlands)

    Stanescu, H.C.; Arcos-Burgos, M.; Medlar, A.; Bockenhauer, D.; Kottgen, A.; Dragomirescu, L.; Voinescu, C.; Patel, N.; Pearce, K.; Hubank, M.; Stephens, H.A.; Laundy, V.; Padmanabhan, S.; Zawadzka, A.; Hofstra, J.M.; Coenen, M.J.H.; Heijer, M. den; Kiemeney, L.A.L.M.; Bacq-Daian, D.; Stengel, B.; Powis, S.H.; Brenchley, P.; Feehally, J.; Rees, A.J.; Debiec, H.; Wetzels, J.F.M.; Ronco, P.; Mathieson, P.W.; Kleta, R.

    2011-01-01

    BACKGROUND: Idiopathic membranous nephropathy is a major cause of the nephrotic syndrome in adults, but its etiologic basis is not fully understood. We investigated the genetic basis of biopsy-proven cases of idiopathic membranous nephropathy in a white population. METHODS: We performed independent

  17. Idiopathic pulmonary artery aneurysm.

    Science.gov (United States)

    Kotwica, Tomasz; Szumarska, Joanna; Staniszewska-Marszalek, Edyta; Mazurek, Walentyna; Kosmala, Wojciech

    2009-05-01

    Pulmonary artery aneurysm (PAA) is an uncommon lesion, which may be associated with different etiologies including congenital cardiovascular diseases, systemic vasculitis, connective tissue diseases, infections, and trauma. Idiopathic PAA is sporadically diagnosed by exclusion of concomitant major pathology. We report a case of a 56-year-old female with an idiopathic pulmonary artery dilatation identified fortuitously by echocardiography and confirmed by contrast-enhanced computed tomography. Neither significant pulmonary valve dysfunction nor pulmonary hypertension and other cardiac abnormalities which might contribute to the PAA development were found. Here, we describe echocardiographic and computed tomography findings and review the literature on PAA management.

  18. Tavistock Adult Depression Study (TADS: a randomised controlled trial of psychoanalytic psychotherapy for treatment-resistant/treatment-refractory forms of depression

    Directory of Open Access Journals (Sweden)

    Taylor David

    2012-07-01

    Full Text Available Abstract Background Long-term forms of depression represent a significant mental health problem for which there is a lack of effective evidence-based treatment. This study aims to produce findings about the effectiveness of psychoanalytic psychotherapy in patients with treatment-resistant/treatment-refractory depression and to deepen the understanding of this complex form of depression. Methods/Design INDEX GROUP: Patients with treatment resistant/treatment refractory depression. DEFINITION & INCLUSION CRITERIA: Current major depressive disorder, 2 years history of depression, a minimum of two failed treatment attempts, ≥14 on the HRSD or ≥21 on the BDI-II, plus complex personality and/or psycho-social difficulties. EXCLUSION CRITERIA: Moderate or severe learning disability, psychotic illness, bipolar disorder, substance dependency or receipt of test intervention in the previous two years. DESIGN: Pragmatic, randomised controlled trial with qualitative and clinical components. TEST INTERVENTION: 18 months of weekly psychoanalytic psychotherapy, manualised and fidelity-assessed using the Psychotherapy Process Q-Sort. CONTROL CONDITION: Treatment as usual, managed by the referring practitioner. RECRUITMENT: GP referrals from primary care. RCT MAIN OUTCOME: HRSD (with ≤14 as remission. SECONDARY OUTCOMES: depression severity (BDI-II, degree of co-morbid disorders Axis-I and Axis-II (SCID-I and SCID-II-PQ, quality of life and functioning (GAF, CORE, Q-les-Q, object relations (PROQ2a, Cost-effectiveness analysis (CSRI and GP medical records. FOLLOW-UP: 2 years. Plus: a. Qualitative study of participants’ and therapists’ problem formulation, experience of treatment and of participation in trial. (b Narrative data from semi-structured pre/post psychodynamic interviews to produce prototypes of responders and non-responders. (c Clinical case-studies of sub-types of TRD and of change. Discussion TRD needs complex, long-term intervention and

  19. Fireclay Refractory Bricks

    Institute of Scientific and Technical Information of China (English)

    Zhang Xiaohui

    2010-01-01

    @@ 1 Scope This standard specifies the classification,brand,shape,dimension,technical requirements,test methods,quality appraisal procedures,packing,marking,transportation,storage,and quality certificate of fireclay refractory bricks.

  20. Refractory duodenal ulcer

    Directory of Open Access Journals (Sweden)

    Al Freihi Hussein

    1995-01-01

    Full Text Available Refractory or intractable ulcer is defined as an ulcer that fails to heal completely after eight to twelve weeks, despite appropriate treatment with a modern antiulcer therapy in a compliant patient. Refractory ulcer should be suspected in individuals diagnosed to have peptic ulcer if their symptoms persist longer than usual: occurrence of complications or simply their ulcers fail to heal, since up to 25% of such patients remain asymptomatic. Conditions associated with refractory ulcer include noncompliance, continuous consumption of nonsteroidal anti-inflam-matory drugs, acid hypersecretion, smoking. male gender and other factors with questionable role like advanced age, large ulcer size, prolonged duration of symptoms and the presence of complication like bleeding. Nonpeptic ulcers like tuberculosis, malignancy, Crohn′s disease and primary intestinal lymphoma should always be considered in the differential diagnosis. Colonization with H. pylori which is well-known as a cause of frequent recurrences, has not been linked with refractoriness. Patients with refractory ulcers must undergo thorough re-evaluation including repeated endoscopies, obtaining biopsies for microbiology and histology and determination of serum-gastrin level. Once diseases with identifiable etiologies have been ruled out, aggressive medical management with single or multiple antiulcer drugs should be instituted. Such treatments will virtually heal all refractory ulcers. Surgery should be reserved for patients whose ulcers fail to respond to optimal medical therapy or those who develop com-plications necessitating surgical intervention.

  1. Juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Prakken, Berent; Albani, Salvatore; Martini, Alberto

    2011-01-01

    Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years. Pivotal studies in the past 5 years have led to substantial progress in various areas, ranging from disease classification to new treatments. Gene expres

  2. Talazoparib and Temozolomide in Treating Younger Patients With Refractory or Recurrent Malignancies

    Science.gov (United States)

    2016-10-10

    Adult Solid Neoplasm; Childhood Solid Neoplasm; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Refractory Central Nervous System Neoplasm

  3. Chronic idiopathic intestinal pseudo-obstruction in an English bulldog.

    Science.gov (United States)

    Dvir, E; Leisewitz, A L; Van der Lugt, J J

    2001-05-01

    A case of chronic idiopathic intestinal pseudo-obstruction in an English bulldog is described. The dog was presented with chronic weight loss and vomiting. An intestinal obstruction was suspected based on clinical and radiological findings. A diagnosis of chronic idiopathic intestinal pseudo-obstruction was made on the basis of full thickness intestinal biopsies. The dog was refractory to any antiemetic therapy. Necropsy revealed marked atrophy and fibrosis of the tunica muscularis, together with a mononuclear cell infiltrate extending from the duodenum to the colon. This case was presented with clinical findings consistent with visceral myopathy in humans--namely, atony and dilatation of the whole gut--but the histological findings resembled sclerosis limited to the gastrointestinal tract.

  4. RHI Refractories Opens Dalian Plant

    Institute of Scientific and Technical Information of China (English)

    Stefan Pirker

    2004-01-01

    @@ End of November the opening ceremony of the RHI Refractories Dalian plant took place in the presence of 250 guests from China and Europe. RHI Refractories welcomed highly official guests from the Dalian City and the Dalian Development Area, the Supervisory Board of RHI, the company's biggest shareholder, the Board of Management, representatives of customers in China as well as Professors of China's refractory academic field. The ceremony' s highlight was the speech of Professor Zhong Xiangchong from Zhengzhou University, member of the China Academy of Science, who pointed out RHI Refractories' long and successful history of refractory innovations during the last decades and the future possibilities of collaboration with RHI Refractories.

  5. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  6. Imaging in juvenile idiopathic arthritis with a focus on ultrasonography

    DEFF Research Database (Denmark)

    Laurell, Louise; Court-Payen, Michel; Boesen, Mikael;

    2013-01-01

    Early therapeutic intervention and use of new highly efficacious treatments have improved the outcome in many patients with juvenile idiopathic arthritis (JIA), but have also led to the need for more precise methods to evaluate disease activity. In adult rheumatology, numerous studies have establ...

  7. Idiopathic Normal Pressure Hydrocephalus

    OpenAIRE

    2016-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” ...

  8. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

    Full Text Available We report a case of acute idiopathic scrotal edema (AISE in a 4-year-old boy who presented with acute scrotal pain and erythema. The clinical features, ultrasound appearance, and natural history of this rare diagnosis are reviewed. In this report, we highlight the importance of good ultrasound technique in differentiating the etiology of the acute scrotum and demonstrate the color Doppler “Fountain Sign” that is highly suggestive of AISE.

  9. High-Dose [131I]Tositumomab (anti-CD20) Radioimmunotherapy and Autologous Hematopoietic Stem Cell Transplantation for Adults ≥ 60 Years Old with Relapsed or Refractory B-Cell Lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Gopal, Ajay K.; Rajendran, Joseph G.; Gooley, Ted; Pagel, John M.; Fisher, Darrell R.; Petersdorf, Stephen; Maloney, David G.; Eary, Janet F.; Appelbaum, Frederick R.; Press, Oliver W.

    2007-04-10

    Purpose: The majority of patients with relapsed or refractory B-cell, non-Hodgkin’s lymphoma (NHL) are over 60 years of age, yet they are often denied potentially curative high-dose therapy and autologous stem cell transplants (ASCT) due to the risk of excessive treatment-related morbidity and mortality. Myeloablative anti-CD20 radioimmunotherapy (RIT) can deliver curative radiation doses to tumor sites while limiting exposure to normal organs and may be particularly suited for older adults requiring high-dose therapy. Methods: Patients over age 60 with relapsed B-NHL received infusions of tositumomab anti-CD20 antibody labeled with 5-10mCi I-131 tracer for dosimetry purposes followed 10 days later by individualized therapeutic infusions of I-131-tositumomab (median 525 mCi, range 328-1154 mCi) to deliver 25-27Gy to the critical normal organ receiving the highest radiation dose. ASCT was performed approximately 2 weeks after therapy. Results: Twenty-four patients with a median age of 64 (range 60-76) who had received a median of four prior regimens (range 2-14) were treated. Thirteen (54%) had chemotherapy-resistant disease. The estimated 3-year overall and progression-free survivals were 59% and 51%, respectively with a median follow-up of 2.9 years (range 1-6 years). All patients experienced expected myeloablation with engraftment of platelets (≥20K/µL) and neutrophils (≥500/µL) occurring a median of 9 and 15 days, respectively following ASCT. There were no treatment-related deaths, and only two patients experienced grade 4 non-hematologic toxicity. Conclusions: Myeloablative RIT and ASCT is a safe and effective therapeutic option for older adults with relapsed B-NHL.

  10. Refractory peptic ulcer disease.

    Science.gov (United States)

    Napolitano, Lena

    2009-06-01

    Refractory PUD is a diagnostic and therapeutic challenge. Optimal management of severe or refractory PUD requires a multidisciplinary team approach, using primary care providers, gastroenterologists, and general surgeons. Medical management has become the cornerstone of therapy. Identification and eradication of H pylori infection combined with acid reduction regimens can heal ulceration and also prevent recurrence. Severe, intractable or recurrent PUD and associated complications mandates a careful and methodical evaluation and management strategy to determine the potential etiologies and necessary treatment (medical or surgical) required.

  11. Unusual cause of respiratory distress misdiagnosed as refractory asthma

    Directory of Open Access Journals (Sweden)

    Al-Otair Hadil

    2006-01-01

    Full Text Available We report a young lady, who was labeled as a case of refractory asthma for a few years, based on history of shortness of breath on minimal exertion, noisy breathing and normal chest radiograph. Repeated upper airway exam by an otolaryngologist and computerized tomography scan, were normal. On presentation to our hospital, she was diagnosed to have fixed upper airway obstruction, based on classical flow-volume loop findings. Fibroptic bronchoscopy revealed a web-shaped subglottic stenosis. The histopathology of a biopsy taken from that area, showed non-specific inflammation. No cause for this stenosis could be identified. The patient was managed with rigid bronchoscopy dilatation, without recurrence. We report this case as idiopathic subglottic stenosis, that was misdiagnosed as refractory bronchial asthma, stressing the importance of performing spirometry in the clinic.

  12. Clinical application of extracorporeal membrane oxygenation for treatment of adult refractory cardiogenic shock%体外膜肺氧合在成人心原性休克救治中的临床应用

    Institute of Scientific and Technical Information of China (English)

    侯六生; 谢钢; 李建伟; 蒋崇慧; 袁勇; 李斌飞; 姜海明; 宁晔; 吴桂深

    2013-01-01

    Objective To summarize the clinical experience of extracorporeal membrane oxygenation (ECMO) treatment for adult refractory cardiogenic shock.Methods From January 2003 to January 2011,patients with refractory cardiogenic shock required veno-arterial ECMO by failure of conventional therapy and intra-aortic balloon pump counterpulsation therapy were retrospectively studied.Patients with severe traumatic brain injury,advanced malignancies and multiple organ failure were excluded.Patients were divided into weaned group(n =31) and not weaned group(n =23) according to the ECMO weaning.Results The duration of ECMO was 24.16(14.12,56.75) hours.Twenty-two out of 31 patients in the weaned group survived and were discharged,9 patients died after successfully weaned from ECMO (5 due to multisystem organ failure,2 due to reoccurred cardiogenic shock,1 due to infectious shock and 1 due to disseminated or diffuse intravascular coagulation).Pre-ECMO mean arterial pressure,ejection fraction,the duration of ECMO were significantly higher while pre-ECMO blood lactate [(8.64 ± 3.17) vs.(14.44 ± 2.52),P <0.01],the duration of ROSC [(16.70 ± 5.29) vs.(35.64 ± 5.89),P <0.01] and multisystem organ failure [0 vs.17.4% (4/23),P <0.05] were lower in weaned group than in not wean group.Conclusions ECMO is an effective mechanical assistant therapy strategy for adult refractory cardiogenic shock patients.Timely applying this strategy on suitable patients is crucial for the success of ECMO.Cardiac function and reversibility of heart failure are key factors determine the fate of weaned or not weaned ECMO in adult refractory cardiogenic shock patients.%目的 总结体外膜肺氧合(ECMO)在成人心原性休克(CS)中的临床救治经验,探讨ECMO的临床应用价值及影响脱机的可能危险因素.方法 回顾性分析中山大学附属中山医院2003年1月至2011年1月因药物和(或)主动脉内球囊反搏(IABP)无效而行ECMO救治的成人CS患者的临床资料,

  13. Refractory Minerals in Henan Province

    Institute of Scientific and Technical Information of China (English)

    JIN Qinguo; LI Jing; LIU Jiehua; LIU Yanjun

    2004-01-01

    Henan province is very rich in refractory minerals of many varieties including silica, dolomite, graphite,pearlite, sepiolite, olivine, and sillimanite group minerals, besides the abundant reserves of fireclay and bauxite,which lay a good foundation for the development of the refractories industry of the province. The paper introduces the reserves, distribution and character of the refractory minerals in Henan province.

  14. Dynamic Testing of Gasifier Refractory

    Energy Technology Data Exchange (ETDEWEB)

    Michael D. Mann; Wayne S. Seames; Devdutt Shukla; Xi Hong; John P. Hurley

    2005-12-01

    The University of North Dakota (UND) Chemical Engineering Department in conjunction with the UND Energy & Environmental Research Center (EERC) have initiated a program to examine the combined chemical (reaction and phase change) and physical (erosion) effects experienced by refractory materials under slagging coal gasification conditions. The goal of this work is to devise a mechanism of refractory loss under these conditions. The controlled-atmospheric dynamic corrodent application furnace (CADCAF) was utilized to simulate refractory/slag interactions under dynamic conditions that more realistically simulate the environment in a slagging coal gasifier than any of the static tests used previously by refractory manufacturers and researchers. High-alumina and high-chromia refractory bricks were tested using slags obtained from two solid fuel gasifiers. Testing was performed at 1475 C in a reducing atmosphere (2% H{sub 2} in N{sub 2}) The CADCAF tests show that high-chrome refractories have greater corrosion resistance than high-aluminum refractories; coal slag readily diffuses into the refractory through its grain boundaries; the refractory grains are more stable than the matrix in the tests, and the grains are the first line of defense against corrosion; calcium and alkali in the slag are more corrosive than iron; and silicon and calcium penetrate the deepest into the refractory. The results obtained from this study are preliminary and should be combined with result from other research programs. In particular, the refractory corrosion results from this study should be compared with refractories removed from commercial gasifiers.

  15. 成年难治性癫痫患者立体定向术后生活质量的调查分析%Analysis of stereotactic surgery on quality of life in adults with refractory epilepsy

    Institute of Scientific and Technical Information of China (English)

    王一芳; 谭启富; 许爱刚; 王正伟; 陈昌

    2011-01-01

    目的 调查分析立体定向手术治疗对成年难治性癫痫患者生活质量的影响.方法 对92例无明确致痫灶的成年难治性癫痫患者进行立体定向手术治疗,采用症状自评量表(SCL-90),癫痫患者生活质量量表--31(QOLIE- 31)在手术前及手术后半年、1年、2年、3年、4年、5年对患者进行连续评估.结果 尽管部分患者术后癫痫症状不能完全控制,但患者总的生活质量在手术后各阶段均较术前明显提高.手术效果Engel I级患者术后各时间段的生活质量优于Ⅱ~Ⅳ级患者的生活质量.术前具有明显精神症状患者术后各时间段的生活质量与术前无精神症状患者的术后生活质量比较无明显差异.结论 对于经术前综合评估不能确定致痛灶,特别是伴有明显精神症状的难治性癫痫患者,立体定向手术治疗是一种有效的治疗手段,可显著改善这部分患者的生活质量.%Objective To investigate the affection of stereotactic surgery on quality of life (QOL) in adults with refractory epilepsy. Methods 92 patients of refractory epilepsy with no definitive epileptic focus were treated by stereotactic techniques. Psychiatric evaluation of these patients was performed before surgery, 6 months after surgery, and annually for 5 years, using the Symptom Checklist-90 (SCL-90) and Quality of Life in Epilepsy-31 (QOLIE-31) questionnaires. Results Patients as a whole had a significant improvement in their quality of life after surgery, even if they did not achieve total control of seizures. Nevertheless, those who became seizure free showed even more positive differences in quality of life in comparison with patients in Engel classes Ⅱ-Ⅳ. The quality of life after surgery had no difference between patients without presurgical psychopathology and patients who had a psychiatric diagnosis before suegery. Conclusion For the patients of refractory epilepsy with no definitive epileptic focus,especially for

  16. Refractory chronic migraine

    DEFF Research Database (Denmark)

    Martelletti, Paolo; Katsarava, Zaza; Lampl, Christian

    2014-01-01

    The debate on the clinical definition of refractory Chronic Migraine (rCM) is still far to be concluded. The importance to create a clinical framing of these rCM patients resides in the complete disability they show, in the high risk of serious adverse events from acute and preventative drugs...

  17. A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

    Directory of Open Access Journals (Sweden)

    Poongavanam Paranthaman

    2016-08-01

    Full Text Available Primary Pulmonary Hypertension is a rare disease occurring in 1-2 per million population. It is 2-4 times more common in female. Idiopathic or primary pulmonary hypertension is defined as a disorder with no identifiable cause in which resting mean pulmonary artery pressure in adults is above 25 mmHg and 30 mmHg with exercise. Idiopathic or primary pulmonary hypertension is diagnosed after ruling out all the possible secondary causes of pulmonary hypertension. We are presenting a case of middle-aged male who presented with dyspnoea and on further evaluation found to have primary pulmonary hypertension, which is uncommon in male

  18. Surgical Treatment of Adult Degenerative Scoliosis

    OpenAIRE

    Cho, Kyu-Jung; Kim, Young-tae; Shin, Sang-hyun; Suk, Se-Il

    2014-01-01

    The rapid increase of elderly population has resulted in increased prevalence of adult scoliosis. Adult scoliosis is divided into adult idiopathic scoliosis and adult degenerative scoliosis. These two types of scoliosis vary in patient age, curve pattern and clinical symptoms, which necessitate different surgical indications and options. Back pain and deformity are major indications for surgery in adult idiopathic scoliosis, whereas radiating pain to the legs due to foraminal stenosis is what...

  19. Treatment of early and refractory dermatomyositis with infliximab: a report of two cases.

    Science.gov (United States)

    Dold, Sylvia; Justiniano, Maria E; Marquez, Javier; Espinoza, Luis R

    2007-07-01

    The idiopathic inflammatory myopathies embody the largest group of acquired and potentially treatable causes of skeletal muscle weakness. The three major groups of this disorder are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. Corticosteroids continue to be the mainstay of initial treatment in the majority of cases of PM/DM. The treatment of refractory disease can be challenging despite the utilization of the medications currently available. We report two patients with refractory DM who were treated with infliximab. We describe their presentation, clinical course, treatment, and outcomes.

  20. Status epilepticus: Refractory and super-refractory.

    Science.gov (United States)

    Dubey, Deepanshu; Kalita, Jayantee; Misra, Usha K

    2017-01-01

    Status epilepticus (SE) is an important neurological emergency. It is defined as seizures lasting for 5 minutes or more or recurrent seizures without recovery of consciousness to baseline between the attacks. Refractory SE (RSE) is defined as SE persisting despite sufficient dose of benzodiazepines and at least one antiepileptic drug (AED), irrespective of time. Super refractory SE (SRSE) is defined as SE that continues for 24 hours or more after the use of anesthetic therapy, including cases that recur on weaning of the anesthestic agent. RSE occurs in 23%-48% of the patients and SRSE in approximately 22% of the patients with SE. In general, RSE occurs in patients with new-onset seizures rather than in patients with chronic epilepsy. The etiology of RSE in developing countries is dominated by central nervous system (CNS) infections and head injury compared to stroke and drug withdrawal in the developed countries. The treatment of RSE and SRSE is not evidence based. Following benzodiazepines, the second line antiepileptic drugs include sodium valproate, phenytoin, levetiracetam, and anesthetic drugs such as midazolam, phenobarbital, and propofol. Most intravenous anesthetic drugs produce hypotension and respiratory suppression; therefore, patients with RSE are managed in intensive care units (ICUs). In RSE patients, electroencephalogram (EEG) burst suppression with interburst interval of 2-20 s or even flat EEG has been tried. Recently, concerns have been raised on the safety of burst suppression in RSE and SRSE. The paucity of ICUs in developing countries limits the use of these management protocols. There is a need to explore intravenous AEDs with safer cardiovascular and respiratory profile for the management of SE.

  1. Idiopathic venous thromboembolism and thrombophilia

    OpenAIRE

    Sinescu, C; Hostiuc, M; Bartos, D.

    2011-01-01

    During the past decade idiopathic venous thromboembolism has become a separate entity, a chronic illness which has required prolonged anticoagulation and other prevention strategies to avoid recurrences. This article reviews recent developments regarding unprovoked venous thromboembolism and its relation with thrombophilia. In the beginning, the latest definition of idiopathic venous thromboembolism is presented. The article continues with statistics about thrombophilia, related venous thromb...

  2. Refractory headache: classification and nomenclature.

    Science.gov (United States)

    Levin, Morris

    2008-06-01

    There are a number of reasons to attempt to define and classify refractory headache disorders. Particularly important are the potential benefits in the areas of research, treatment, and medical cost reimbursement. There are challenges in attempting to classify refractory forms of headaches, including the lack of biological or other objective markers and a lack of consensus among practitioners as to what qualifies as refractoriness, or even if a separate category for refractory migraine and other refractory headaches needs to be established. A definition of refractory migraine has been proposed by Schulman et al in this issue ("Defining Refractory Migraine [RM] and Refractory Chronic Migraine [RCM]: Proposed Criteria for the Refractory Headache Special Interests Section of the American Headache Society"), which should be tested for validity and usefulness. It seems reasonable to consider adding this defined syndrome to the International Classification of Headache Disorders, second edition (ICHD-II). In this article, options for adding refractory headache syndromes to the ICHD are discussed with pros and cons for each. Two "best" options for adding the disorder "refractory migraine" to the ICHD are presented along with an illustrative case example.

  3. Analysis of spinopelvic sagittal alignment in adult patients with idiopathic scoliosis%成人特发性脊柱侧凸患者脊柱-骨盆矢状位平衡分析

    Institute of Scientific and Technical Information of China (English)

    李危石; 陈仲强; Kirkham B Wood

    2011-01-01

    Objective:To analyze the spinopelvic sagittal alignment in adult patients with idiopathic scoliosis according to curve types.Method:The sagittal spinopelvie parameters and scoliosis curves types were analyzed retrospectively in standing radiographs of 83 adult patients(mean age 43.0 years) with adult idiopathic scoliosis,these parameters included cobb angle of primary scoliosis,thoracic kyphosis (TK),thoracolumbar junction kyphosis(TLJ),lumbar lordosis(LL),pelvic incidence(PI),sacrum slope(SS),pelvic tilt(PT) and sagittal vertical axis (SVA).According to SRS classification,the patients were divided into two groups,thoracic curve and lumbar curve. All spinopelvic parameters between two groups were compared. The correlations between parameters as well as these parameters with age were analyzed. Result:The average age of 39 patients with thoracic curve was 40.9,while that for 44 patients with lumbar curve was 45.8.No age-related difference was noted between two groups. Also no difference for all spinopelvic parameters except SVA was noted.The SVA in patients with lumbar curve was significantly greater than that with thoracic curve (P<0.05).For patients less than 40 years old,all spinopelvic parameters between two groups showed no difference,while for patients older than 40 years old,SVA and PT in lumbar curve group were significant greater than those in thoracic curve group (P<0.05). In thoracic curve group,9 patients had SVA more than±50mm. Among them 7 patients were negative imbalance. In lumbar curve group, 12 patients had SVA more than±50mm,and 8 patients of them were positive im balance. In both two groups,there were significant correlations between LL and TK,LL and SS,PI and SS,PI and PT,SVA and LL,SVA and SS,SVA and PT,age and SVA,and age and PT.PI showed significant correlaion with LL in patients with thoracic curve while no correlation with LL in patients with lumbar curve. Unlike thoracic curve group,no relationship between PI and LL in lumbar curve

  4. Idiopathic short stature

    Directory of Open Access Journals (Sweden)

    Vlaški Jovan

    2013-01-01

    Full Text Available Growth is a complex process and the basic characteristic of child- hood growth monitoring provides insight into the physiological and pathological events in the body. Statistically, the short stature means departure from the values of height for age and sex (in a particular environment, which is below -2 standard deviation score, or less than -2 standard deviation, i.e. below the third percentile. Advances in molecular genetics have contributed to the improvement of diagnostics in endocrinology. Analysis of patients’ genotypes should not be performed before taking a classical history, detailed clinical examination and appropriate tests. In patients with idiopathic short stature specific causes are excluded, such as growth hormone deficiency, Turner syndrome, short stature due to low birth weight, intrauterine growth retardation, small for gestational age, dysmorphology syndromes and chronic childhood diseases. The exclusion of abovementioned conditions leaves a large number of children with short stature whose etiology includes patients with genetic short stature or familial short stature and those who are low in relation to genetic potential, and who could also have some unrecognized endocrine defect. Idiopathic short stature represents a short stature of unknown cause of heterogeneous etiology, and is characterized by a normal response of growth hormone during stimulation tests (>10 ng/ml or 20 mJ/l, without other disorders, of normal body mass and length at birth. In idiopathic short stature standard deviation score rates <-2.25 (-2 to -3 or <1.2 percentile. These are also criteria for the initiation of growth hormone therapy. In children with short stature there is also the presence of psychological and social suffering. Goals of treatment with growth hormone involve achieving normal height and normal growth rate during childhood.

  5. Intratympanic Steroid Treatment for Idiopathic Sudden Sensorineural Hearing Loss after Failure of Intravenous Therapy

    OpenAIRE

    Ferri, Emanuele; Frisina, Antonio; Fasson, Anna Chiara; Armato, Enrico; Spinato, Giacomo; Amadori, Maurizio

    2012-01-01

    Purpose. The aim of this study is the investigation of the effectiveness of intratympanic steroids therapy (IST) in patients with idiopathic sudden sensorineural hearing loss (ISSHL) who had not responded to intravenous treatment, evaluating the overall hearing recovery and comparing the results with different variables. Materials and Methods. Our study consisted of 55 patients with refractory ISSHL who, at the end of 10 days of therapy with intravenous steroids, had puretone 4-frequency aver...

  6. Hypercalcemia in idiopathic myelofibrosis

    DEFF Research Database (Denmark)

    Voss, A; Schmidt, K; Hasselbalch, H;

    1992-01-01

    A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low level...... of circulating type I procollagen propeptide (PICP) and lack of progression of the bone marrow fibrosis during almost 6 months of follow-up point to an in vivo inhibition of type I collagen synthesis by 1,25-dihydroxyvitamin D3....

  7. [Idiopathic progressive subglottic stenosis].

    Science.gov (United States)

    Sittel, C

    2014-07-01

    Idiopathic subglottic stenosis is causing a narrowing of the central airway at the laryngotracheal junction. Etiology is remaining unclear at large. There is a marked preponderance for women in the fertile age, an association to estrogene or progesterone metabolism remains doubtful. Suggested treatment varies from repeated endoscopic interventions to primary open resection. Therapy selection in this heterogeneous condition should be based on the individual patient situation as well as surgeon's expertise. This complex entity is prone to complications and should preferably be managed in a referral center.

  8. Idiopathic granulomatous orchitis.

    Science.gov (United States)

    Roy, Somak; Hooda, Shveta; Parwani, Anil V

    2011-05-15

    Idiopathic granulomatous orchitis is a rare inflammatory process of the testis of unknown etiology. It is characterized by presence of non-specific granulomatous inflammation and admixed multinucleated giant cells. It usually presents as a testicular mass which is highly suspicious of malignancy. Histologically, there is extensive destruction of seminiferous tubules with tubular or interstitial pattern of granulomatous inflammation and prominent collagen fibrosis. Trauma and possible auto-antibodies against sperms have been postulated to be the underlying mechanism. Differential diagnoses include intratubular germ-cell neoplasia, malignant lymphomas, and malakoplakia. Orchiectomy is currently the most appropriate therapy for this condition.

  9. Idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Nitin Hemchandra Dani

    2015-01-01

    Full Text Available Idiopathic gingival fibromatosis (IGF is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth.

  10. Idiopathic gingival fibromatosis

    Science.gov (United States)

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  11. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

    AIMS: The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH. MATERIALS AND METHODS: We included 44 patients with new-onset IIH. Thirty-four patients with suspected but u...... tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity....

  12. Congenital idiopathic chylothorax in neonates: chemical pleurodesis with povidone-iodine (Betadine).

    Science.gov (United States)

    Brissaud, O; Desfrere, L; Mohsen, R; Fayon, M; Demarquez, J L

    2003-11-01

    Chylothorax is defined as an accumulation of chyle in the pleural space. This condition usually occurs after an operation, the congenital idiopathic form being rare (1/15000 births). Recovery is observed within four to six weeks of diagnosis in most cases. Treatment is either conservative or surgical. Four cases are reported of congenital chylothorax (three idiopathic, one accompanied by diffuse lymphangectasia) managed by chemical pleurodesis (intrapleural injection of povidone-iodine). Tolerance was satisfactory: unaltered thyroid function in the three cases explored; one case of transient generalised oedema. Treatment was deemed successful in three of the four cases. One child died from renal failure (unrelated to the chemical pleurodesis). Pleurodesis by povidone-iodine appears to be well tolerated and may represent a good alternative to mechanical abrasion or surgery for congenital idiopathic chylothorax. Its use for refractory chylothorax may also decrease the morbidity related to prolonged hospital stay.

  13. Therapeutic Efficacy of Fresh, Autologous Mesenchymal Stem Cells for Severe Refractory Gingivostomatitis in Cats

    OpenAIRE

    Arzi, Boaz; Mills-Ko, Emily; Frank J.M. Verstraete; Kol, Amir; Walker, Naomi J.; Badgley, Megan R.; Fazel, Nasim; William J. Murphy; Vapniarsky, Natalia; Borjesson, Dori L.

    2015-01-01

    Mesenchymal stem cells (MSCs) are a promising therapy for immune-mediated and inflammatory disorders, because of their potent immunomodulatory properties. In this study, we investigated the use of fresh, autologous, adipose-derived MSCs (ASCs) for feline chronic gingivostomatitis (FCGS), a chronic, debilitating, idiopathic, oral mucosal inflammatory disease. Nine cats with refractory FCGS were enrolled in this pilot study. Each cat received 2 intravenous injections of 20 million autologous AS...

  14. Observation of curative effect of modified atkins diet in treatment of adults with refractory epilepsy%改良的阿特金斯饮食治疗成人难治性癫痫的疗效观察

    Institute of Scientific and Technical Information of China (English)

    尚丽; 王曼; 龚德山; 张翠荣; 王开颜

    2015-01-01

    目的:评估改良的阿特金斯饮食( MAD)治疗成人难治性癫痫的疗效。方法19例成人难治性癫痫患者接受MAD治疗,随访1~15个月。记录发作频率及不良反应。结果1个月有效率为63%,保留率100%;3个月有效率57%,保留率89%;6个月有效率为47%,保留率为47%。患者治疗前发作频率为4~152次/月,平均25次/月;治疗后1个月发作频率为0~100次/月,平均8次/月。住院期间患者β-羟丁酸(1.5±0.6)mmol/L,血糖(4.6±0.4)mmol/L;随访期间乙酰乙酸波动于触~揣。与治疗前比较,治疗后第1个月和第3个月三酰甘油水平显著升高(均P<0.05),但均值仍在正常范围;总胆固醇及低密度脂蛋白水平差异无统计学意义。不良反应表现为体质量减轻7例,高脂血症6例,乏力4例,便秘3例,腹泻2例,呕吐及肾结石1例。结论 MAD治疗成人难治性癫痫安全有效,没有手术指征的成人难治性癫痫患者排除禁忌后可选择MAD。%Objective To evaluate the efficacy of modified atkins diet ( MAD ) in treatment of adults with refractory epilepsy.Methods Nineteen adult patients with refractory epilepsy were initiated on the MAD and followed 1-15 months.Seizure frequencies and adverse effects were recorded.Results The effective rate was 63%, and retention rate was 100%at 1 month after MAD treatment.The effective rate was 57%, and retention rate was 89%at 3 month after MAD treatment.The effective rate was 47%, and retention rate was 47% at 6 month after MAD treatment.The seizure frequencies was 4-152 times per month before treatment, and the average was 25 times per month;the seizure frequencies was 0-100 times per month at 1 month after treatment, and average was 8 per month. The serumβ-hydroxybutyric acid level was (1.5 ±0.6)mmol/L, blood glucose level was (4.6 ±0.4)mmol/L in the hospitalization period;the level of acetoacetic acid

  15. The structural equation analysis of childhood abuse, adult stressful life events, and temperaments in major depressive disorders and their influence on refractoriness

    Directory of Open Access Journals (Sweden)

    Toda H

    2015-08-01

    Full Text Available Hiroyuki Toda,1 Takeshi Inoue,2,3 Tomoya Tsunoda,1 Yukiei Nakai,2 Masaaki Tanichi,1 Teppei Tanaka,1 Naoki Hashimoto,2 Yasuya Nakato,2 Shin Nakagawa,2 Yuji Kitaichi,2 Nobuyuki Mitsui,2 Shuken Boku,4 Hajime Tanabe,5 Masashi Nibuya,1 Aihide Yoshino,1 Ichiro Kusumi2 1Department of Psychiatry, National Defense Medical College, Tokorozawa, Saitama, Japan; 2Department of Psychiatry, Hokkaido University Graduate School of Medicine, Sapporo, Japan; 3Department of Psychiatry, Tokyo Medical University, Tokyo, Japan; 4Department of Psychiatry, Kobe University Graduate School of Medicine, Kobe, Japan; 5Department of Clinical Human Sciences, Graduate School of Humanities and Social Sciences, Shizuoka University, Shizuoka, Japan Background: Previous studies have shown the interaction between heredity and childhood stress or life events on the pathogenesis of a major depressive disorder (MDD. In this study, we tested our hypothesis that childhood abuse, affective temperaments, and adult stressful life events interact and influence the diagnosis of MDD. Patients and methods: A total of 170 healthy controls and 98 MDD patients were studied using the following self-administered questionnaire surveys: the Patient Health Questionnaire-9 (PHQ-9, the Life Experiences Survey, the Temperament Evaluation of the Memphis, Pisa, Paris, and San Diego Autoquestionnaire, and the Child Abuse and Trauma Scale (CATS. The data were analyzed with univariate analysis, multivariable analysis, and structural equation modeling. Results: The neglect scores of the CATS indirectly predicted the diagnosis of MDD through cyclothymic and anxious temperament scores of the Temperament Evaluation of the Memphis, Pisa, Paris, and San Diego Autoquestionnaire in the structural equation modeling. Two temperaments – cyclothymic and anxious – directly predicted the diagnosis of MDD. The validity of this result was supported by the results of the stepwise multivariate logistic regression analysis

  16. Refractory gastroesophageal reflux disease

    OpenAIRE

    Joaquim Prado P. Moraes-Filho

    2012-01-01

    CONTEXT: Gastroesophageal reflux disease (GERD) is a condition which develops when the reflux of stomach contents causes troublesome symptoms and/or complications. Its pathophysiology, diagnosis and treatment have frequently been analyzed but it is interesting to review some aspects of the GERD refractory patients to the proton pump inhibitors treatment. The treatment encompasses behavioral measures and pharmacological therapy. The majority of the patients respond well to proton pump inhibito...

  17. Colectomy for refractory constipation

    DEFF Research Database (Denmark)

    Raahave, Dennis; Loud, Franck Bjørn; Christensen, Elsebeth;

    2010-01-01

    OBJECTIVE: This study evaluated the type of colectomy, postoperative complications, functional results, and satisfaction in patients with constipation refractory to conservative therapy. Further, colonic transit time (CTT), faecal load (coprostasis), and colon length (redundancies) were compared...... had at hemicolectomy, 11 patients a subtotal colectomy and 3 patients an ileostomy. Two patients had an anastomotic leak and one died. In 11 patients, further surgery was necessary, because of recurrent constipation. Abdominal pain disappeared and defecation patterns improved significantly to 1-4 per...

  18. Refractory status epilepticus

    Directory of Open Access Journals (Sweden)

    Sanjay P Singh

    2014-01-01

    Full Text Available Refractory status epilepticus is a potentially life-threatening medical emergency. It requires early diagnosis and treatment. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication (AED, i.e., Lorazepam + phenytoin. Others regard refractory status epilepticus as failure of benzodiazepine and 2 antiepileptic medications, i.e., Lorazepam + phenytoin + phenobarb. Up to 30% patients in SE fail to respond to two antiepileptic drugs (AEDs and 15% continue to have seizure activity despite use of three drugs. Mechanisms that have made the treatment even more challenging are GABA-R that is internalized during status epilepticus and upregulation of multidrug transporter proteins. All patients of refractory status epilepticus require continuous EEG monitoring. There are three main agents used in the treatment of RSE. These include pentobarbital or thiopental, midazolam and propofol. RSE was shown to result in mortality in 35% cases, 39.13% of patients were left with severe neurological deficits, while another 13% had mild neurological deficits.

  19. Pediatric Idiopathic Intracranial Hypertension: A Case Report

    Directory of Open Access Journals (Sweden)

    Divya Nandwani, OD

    2015-08-01

    Full Text Available Background: Idiopathic intracranial hypertension (IIH presents differently in prepubertal children as compared to postpubertal children and adults. In adults, IIH is most common in obese women of child-bearing age. However, when occurring in prepubertal children, IIH has been shown to have no predilection for gender and does not correlate with obesity. This case report exemplifies the rare occurrence of IIH in a pediatric patient. Case Report: A five-year-old Hispanic female patient was seen at the University Eye Center for a longstanding hypotropia secondary to a likely congenital fourth nerve palsy with an exotropia component. After a strabismus surgery and a series of follow-up visits, bilateral swollen optic nerves were observed, and a prompt referral to the emergency room was made. Consequently, she was diagnosed with IIH. A treatment regimen of acetazolamide was initiated with subsequent improvement of the clinical presentation of increased intracranial pressure, confirmed by the reduction of swelling of her optic nerves. Conclusions: Bilateral swelling of the optic nerves can be an emergency, especially in children. It is important urgently to rule out causes for increased intracranial pressure. If treatment is significantly delayed, or if no treatment is given to a patient with this condition, loss of visual function may occur. In addition to reporting a rare circumstance of pediatric idiopathic intracranial hypertension, this case report serves to remind eye care practitioners of the importance of monitoring the ocular health of patients closely, even in young, healthy children.

  20. 成人特发性孤立性ACTH缺乏症三例临床分析并文献复习%Analysis of three patients with adult idiopathic isolated ACTH deficiency and literature review

    Institute of Scientific and Technical Information of China (English)

    郭清华; 陈康; 陆菊明; 母义明; 窦京涛; 吕朝晖; 巴建明; 李江源; 潘长玉

    2014-01-01

    Objective Adult idiopathic isolated ACTH deficiencv(AIIAD) is an underestimated disorder which is frequently misdiagnosed.In this paper,we presented 3 new cases of AIIAD and summarized the clinical characteristics by analyzing these cases and available literature for further understanding this disease.Methods Three cases of AIIAD in our hospital from 1998 to 2003 were retrospectively identified.Data of clinical features,laboratory findings,and response to treatment were collected.The clinical characteristics and pertinent literature were reviewed and analysed.Results These 3 cases of AIIAD aged 52-68 years old were misdiagnosed and incorrectly treated for 7 months to 4 years.All three cases presented the symptoms of fatigue and loss of weight at the onset,and were misdiagnosed as climacteric syndrome,thyroiditis,and gastrointestinal disease.The prevalence of AIIAD was very low,and it was thought to be an underestimated disorder.Through analyzing the 3 cases and literature review,the clinical features of this disease were summarized as follows:(1) Most patients were aged over 40 years and had no history of glucocorticoid medication.(2) Clinical presentations were insidious,with symptoms such as fatigue and weight loss.(3) Patients usually had no excessive pigmentation.(4) Hypogonadism in men and amenorrhea in women could be present.(5) Blood chemistry usually revealed hyponatremia but rarely hyperkalemia.(6) Blood lymphocytosis and eosinophilia were common.(7) Plasma ACTH and cortisol levels markedly decreased with normal secretion of other pituitary hormones.(8) High prevalence of thvroid disorder or positive anti-thyroid autoantibodv.(9) Usually the other accompanied autoimmune diseases might be ameliorated with glucocorticoid replacement.(10) MRI revealed no structural pituitary defects except for empty sella.(11) No evidence of infiltration,trauma,surgery,infection,and radiotherapy of the pituitary gland was found.Conclusions AIIAD is an unspecified and

  1. Refractories Utilizability for Slagging Gasifiers

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Slagging coal gasification process became a highlight of coal chemical industry in China during the last decade. Refractory lining's life of slagging gasifiers is one of the most critical factors for a cost -effective operation. The paper introduces current status of coal gasification in China, lining structure of slagging gasifiers and performance of refractory lining. It also summarizes the major factors impacting on refractory wear in slagging coal gasifiers in four Chinese chemical plants, based on ten years of industrial experience. The utilizability is discussed in terms of cost -effectiveness of high chromia refractories and possibility of the alternatives.

  2. ADHD in idiopathic epilepsy

    Directory of Open Access Journals (Sweden)

    Marcos H. C. Duran

    2014-01-01

    Full Text Available Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners’ Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%, seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

  3. Juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Krupa H Bhatt

    2014-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  4. Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Basant R. Nassar BS

    2016-04-01

    Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

  5. Idiopathic Bilateral Bloody Tearing

    Directory of Open Access Journals (Sweden)

    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  6. Idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    L Kayal

    2014-01-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

  7. PS-341 in Treating Patients With Refractory or Relapsed Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, Chronic Myeloid Leukemia in Blast Phase, or Myelodysplastic Syndrome

    Science.gov (United States)

    2013-01-22

    Adult Acute Promyelocytic Leukemia (M3); Blastic Phase Chronic Myelogenous Leukemia; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia

  8. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

    Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

  9. Refractoriness in human atria

    DEFF Research Database (Denmark)

    Skibsbye, Lasse; Jespersen, Thomas; Christ, Torsten

    2016-01-01

    drugs. Cardiomyocyte excitability depends on availability of sodium channels, which involves both time- and voltage-dependent recovery from inactivation. This study therefore aims to characterise how sodium channel inactivation affects refractoriness in human atria. METHODS AND RESULTS: Steady......-state activation and inactivation parameters of sodium channels measured in vitro in isolated human atrial cardiomyocytes were used to parameterise a mathematical human atrial cell model. Action potential data were acquired from human atrial trabeculae of patients in either sinus rhythm or chronic atrial...... in pharmacological management of chronic atrial fibrillation....

  10. Orind Refractories Limited

    Institute of Scientific and Technical Information of China (English)

    Mr R. Mishra; Group Manging Director

    2005-01-01

    @@ "Sight can be acquired, Vision cannot". Orind Refractories Limited (ORIND), China was formed with this rare vision. At a time when the world was testing the tepid waters of China; Mr. Ravin Jhunjhunwala, Chairman of ORIND and the management of ORIND India had looked over the Great Wall to begin a journey of success. Incorported on 18th August 1994 with an initial investment of USD 5 million, ORL caters to the ever-demanding needs of the steel industry and beyond. Incidentally ORIND was the first wholly owned India company to set up base in China. Pesently, ORIND China has a 616 strong work force including 23 expatriates.

  11. Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Niaz Faraz

    2010-01-01

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

  12. Development of Refractories for Continuous Casting

    Institute of Scientific and Technical Information of China (English)

    TIAN Shouxin; JIN Congjin; YAO Jinfu; LI Zeya

    2004-01-01

    The paper introduces refractories for continuous casting, especially, refractories for continuous casting for clean steel in baosteel. Developing direction of refractories for continuous casting has been pointed out to satisfy the new metallurgical operating practice.

  13. Juvenile idiopathic arthritis

    Science.gov (United States)

    ... arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... wrists, or knees. It also affects the eyes. Spondyloarthritis of children resembles the disorder in adults and ...

  14. Distinct synovial immunopathologic characteristics of juvenile-onset spondylarthritis and other forms of juvenile idiopathic arthritis

    NARCIS (Netherlands)

    E. Kruithof; V. van den Bossche; L. de Rycke; B. Vandooren; R. Joos; J.D. Canete; P.P. Tak; A.M.H. Boots; E.M. Veys; D. Baeten

    2006-01-01

    Objective. To characterize the synovial immunopathologic features of juvenile-onset spondylarthritis (SpA) in relation to adult SpA and other forms of juvenile idiopathic arthritis (JIA). Methods. Synovial biopsy samples were obtained from 10 patients with juvenile-onset SpA, 23 with adult SpA, 19 w

  15. Australian Paediatric Rheumatology Group standards of care for the management of juvenile idiopathic arthritis.

    Science.gov (United States)

    Munro, Jane; Murray, Kevin; Boros, Christina; Chaitow, Jeffrey; Allen, Roger C; Akikusa, Jonathan; Adib, Navid; Piper, Susan E; Singh-Grewal, Davinder

    2014-09-01

    This standards document outlines accepted standards of management for children, adolescents and young adults with juvenile idiopathic arthritis (JIA) in Australia. This document acknowledges that the chronic inflammatory arthritis conditions (JIA) in childhood are different diseases from inflammatory arthritis in adults and that specific expertise is required in the care of children with arthritis.

  16. Approaches to refractory epilepsy

    Directory of Open Access Journals (Sweden)

    Jerome Engel

    2014-01-01

    Full Text Available Epilepsy is one of the most common serious neurological conditions, and 30 to 40% of people with epilepsy have seizures that are not controlled by medication. Patients are considered to have refractory epilepsy if disabling seizures continue despite appropriate trials of two antiseizure drugs, either alone or in combination. At this point, patients should be referred to multidisciplinary epilepsy centers that perform specialized diagnostic testing to first determine whether they are, in fact, pharmacoresistant, and then, if so, offer alternative treatments. Apparent pharmacoresistance can result from a variety of situations, including noncompliance, seizures that are not epileptic, misdiagnosis of the seizure type or epilepsy syndrome, inappropriate use of medication, and lifestyle issues. For patients who are pharmacoresistant, surgical treatment offers the best opportunity for complete freedom from seizures. Surgically remediable epilepsy syndromes have been identified, but patients with more complicated epilepsy can also benefit from surgical treatment and require more specialized evaluation, including intracranial EEG monitoring. For patients who are not surgical candidates, or who are unwilling to consider surgery, a variety of other alternative treatments can be considered, including peripheral or central neurostimulation, ketogenic diet, and complementary and alternative approaches. When such alternative treatments are not appropriate or effective, quality of life can still be greatly improved by the psychological and social support services offered by multidisciplinary epilepsy centers. A major obstacle remains the fact that only a small proportion of patients with refractory epilepsy are referred for expert evaluation and treatment.

  17. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

    Directory of Open Access Journals (Sweden)

    Legakis Nikolaos

    2006-02-01

    Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  18. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median

  19. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  20. Idiopathic Retroperitoneal Hematoma

    Directory of Open Access Journals (Sweden)

    Tomoyuki Abe

    2010-09-01

    Full Text Available A 34-year-old female presented with sudden onset of severe abdominal pain in a flank distribution. A large mass was palpable in the right upper quadrant on physical examination. Abdominal contrast-enhanced computed tomography showed a well-defined, right-sided, retroperitoneal cystic lesion located between the abdominal aorta and the inferior vena cava (IVC. The tumor size was 55 × 58 mm, and it compressed the gallbladder and the duodenum. Upper gastrointestinal radiography revealed a stricture of the second portion of the duodenum by the tumor. T2-weighted magnetic resonance imaging showed that the whole part was hyperintense with hypointense rims, but the inner was partially hypointense. Based on the radiological findings, the preoperative differential diagnosis included retroperitoneal teratoma, Schwannoma, abscess, and primary retroperitoneal tumor. On laparotomy, the tumor was located in the right retroperitoneal cavity. Kocher maneuver and medial visceral rotation, which consists of medial reflection of the upper part of right colon and duodenum by incising their lateral peritoneal attachments, were performed. Although a slight adhesion to the IVC was detected, the tumor was removed safely. Thin-section histopathology examination detected neither tumor tissues nor any tissues such as adrenal gland, ovarian tissue, or endometrial implants. The final pathological diagnosis was idiopathic retroperitoneal hematoma; the origin of the bleeding was unclear. The patient was discharged without any complication 5 days after the operation.

  1. Refractory chronic cluster headache

    DEFF Research Database (Denmark)

    Mitsikostas, Dimos D; Edvinsson, Lars; Jensen, Rigmor H

    2014-01-01

    Chronic cluster headache (CCH) often resists to prophylactic pharmaceutical treatments resulting in patients' life damage. In this rare but pragmatic situation escalation to invasive management is needed but framing criteria are lacking. We aimed to reach a consensus for refractory CCH definition...... for clinical and research use. The preparation of the final consensus followed three stages. Internal between authors, a larger between all European Headache Federation members and finally an international one among all investigators that have published clinical studies on cluster headache the last five years....... Eighty-five investigators reached by email. Proposed criteria were in the format of the International Classification of Headache Disorders III-beta (description, criteria, notes, comments and references). Following this evaluation eight drafts were prepared before the final. Twenty-four (28...

  2. Sample Preparation for Monolithic Refractories Part 1: Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    Zhang Xiaohui; Peng Xigao

    2010-01-01

    @@ 1 Scope This standard specifies the definition and test methods of flowability of dense and insulating refractory castables,and moulding equipment,moulding methods,curing and drying conditions of castables samples.

  3. Present Situation of Refractories for Baosteel

    Institute of Scientific and Technical Information of China (English)

    TAI Li; SHEN Keyin

    2004-01-01

    The paper reviews the present situation of refractories for Baosteel, including refractories for iron-making system, converters, ladles and tundishes. Descriptions have been also conducted on the environment protection and reutilization of refractories in Baosteel, together with somesuggestions for refractories suppliers to meet the advancement in iron and steel making technology of Baosteel.

  4. Male idiopathic oligoasthenoteratozoospermia.

    Science.gov (United States)

    Cavallini, Giorgio

    2006-03-01

    Idiopathic oligoasthenoteratozoospermia (iOAT) affects approximately 30% of all infertile men. This mini-review discussed recent data in this field. Age, non-inflammatory functional alterations in post-testicular organs, infective agents (Chlamydia trachomatis, herpes virus and adeno-associated viruses), alterations in gamete genome, mitochondrial alterations, environmental pollutants and "subtle" hormonal alterations are all considered possible causes of iOAT. Increase of reactive oxygen species in tubules and in seminal plasma and of apoptosis are reputed to affect sperm concentration, motility and morphology. iOAT is commonly diagnosed by exclusion, nevertheless spectral traces of the main testicular artery may be used as a diagnostic tool for iOAT. The following can be considered therapies for iOAT: 1) tamoxifen citrate (20 mg/d) + testosterone undecanoate (120 mg/d) (pregnancy rate per couple/month [prcm]: 3.8%); 2) folic acid (66 mg/d) + zinc sulfate (5 mg/d); 3) L-carnitine (2 g/d) alone or in combination with acetyl-L-carnitine (1 g/d) (prcm: 2.3%); and 4) both carnitines = one 30 mg cinnoxicam suppository every 4 days (prcm: 8.5%). Alpha-blocking drugs improved sperm concentration but not morphology, motility or pregnancy rate. Tranilast (300 mg/d) increased sperm parameters and pregnancy rates in an initial uncontrolled study. Its efficacy on sperm concentration (but not on sperm motility, morphology or prcm) was confirmed in subsequent published reports. The efficacy of tamoxifen + testosterone undecanoate, tamoxifen alone, and recombinant follicle-stimulating hormone is still a matter for discussion.

  5. Male idiopathic oligoasthenoteratozoospermia

    Institute of Scientific and Technical Information of China (English)

    Giorgio Cavallini

    2006-01-01

    Idiopathic oligoasthenoteratozoospermia (iOAT) affects approximately 30% of all infertile men. This mini-review discussed recent data in this field. Age, non-inflammatory functional alterations in post-testicular organs, infective agents (Chlamydia trachomatis, herpes virus and adeno-associated viruses), alterations in gamete genome, mitochondrial alterations, environmental pollutants and "subtle" hormonal alterations are all considered possible causes of iOAT.Increase of reactive oxygen species in tubules and in seminal plasma and of apoptosis are reputed to affect sperm concentration, motility and morphology. iOAT is commonly diagnosed by exclusion, nevertheless spectral traces of the main testicular artery may be used as a diagnostic tool for iOAT. The following can be considered therapies for iOAT: 1) tamoxifen citrate (20 mg/d) + testosterone undecanoate (120 mg/d) (pregnancy rate per couple/month [prcm]:3.8%); 2) folic acid (66 mg/d) + zinc sulfate (5 mg/d); 3) L-carnitine (2 g/d) alone or in combination with acetyl-L-carnitine (1 g/d) (prcm: 2.3%); and 4) both carnitines + one 30 mg cinnoxicam suppository every 4 days (prcm: 8.5%).Alpha-blocking drugs improved sperm concentration but not morphology, motility or pregnancy rate. Tranilast (300 mg/d) increased sperm parameters and pregnancy rates in an initial uncontrolled study. Its efficacy on sperm concentration (but not on sperm motility, morphology or prcm) was confirmed in subsequent published reports. The efficacy of tamoxifen + testosterone undecanoate, tamoxifen alone, and recombinant follicle-stimulating hormone is still a matter for discussion.

  6. Adolescent idiopathic scoliosis: current perspectives

    Directory of Open Access Journals (Sweden)

    Miyanji F

    2014-01-01

    Full Text Available Firoz MiyanjiBritish Columbia Children's Hospital, Vancouver, BC, CanadaAbstract: Adolescent idiopathic scoliosis (AIS remains a common and potentially severe musculoskeletal disorder. Although its etiology is largely unknown, ongoing research endeavors continue to improve our understanding of its potential origin and its natural history. Advances in understanding its true three-dimensional nature have sought to improve classification and management strategies. This review will attempt to highlight and give an overview of the current concepts in adolescent idiopathic scoliosis.Keywords: AIS, current, natural history, management strategies

  7. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...

  8. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant......Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...

  9. Self-flow Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    Zhang Xiaohui; Peng Xigao

    2010-01-01

    @@ 1 Scope This standard specifies the term and definition,classification, technical requirements, test methods,quality appraisal procedures, packing, marking, transportation, storage, and quality certificate of self-flow refractory castables.

  10. Alumina-magnesia Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    Wang Jing

    2010-01-01

    @@ 1 Scope This standard specifies the classification,technical requirements,test methods,quality appraisal procedures,packing,marking,transportation,storage,and quality certificate of alumina-magnesia refractory castables.

  11. Treatment for Relapsed/Refractory AML Based on a High Throughput Drug Sensitivity Assay

    Science.gov (United States)

    2016-05-10

    Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Chronic Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts

  12. Idiopathic atrophie blanche.

    Science.gov (United States)

    Amato, Lauretta; Chiarini, Caterina; Berti, Samantha; Massi, Daniela; Fabbri, Paolo

    2006-01-01

    clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.

  13. Genetics in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Albers, Heleen Marion

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is a non-common disease in children that can persist into adulthood. JIA is considered to be an auto-immune disease. Genetic factors play a role in the pathogenesis. In a new cohort of JIA patients from North-West European descent genetic candidate gene associatio

  14. Extracorporeal Membrane Oxygenation in a Patient With Refractory Acute Respiratory Distress Syndrome Secondary to Toxic Epidermal Necrolysis.

    Science.gov (United States)

    2014-12-01

    life support (ECLS) in adults with acute respiratory distress syndrome (ARDS) has increased markedly during the past few years after suc- cessful...Extracorporeal Membrane Oxygenation in a Patient With Refractory Acute Respiratory Distress Syndrome Secondary to Toxic Epidermal Necrolysis Christy...COVERED - 4. TITLE AND SUBTITLE Extracorporeal Membrane Oxygenation in a Patient With Refractory Acute Respiratory Distress Syndrome Secondary to

  15. 成人特发性膜性肾病激素敏感性与中医证型的相关性研究%Relationship of corticosteroid sensitivity with TCM Syndromes in Adult Patients with Idiopathic Membranous Nephropathy

    Institute of Scientific and Technical Information of China (English)

    王超; 罗月中

    2012-01-01

    Objective To assess the correlation between corticosteroid sensitivity and TCM syndromes in adult patients with Idiopathic Membranous Nephropathy (IMN). Methods A total of 200 patients with primary nephrotic syndrome (PNS) was enrolled in this study, including 38 patients of IMN in experimental group and 162 patients of other types of PNS in control group. According to the reaction of corticosteroid, experimental group was divided into experimental group A who was sensitive to corticosteroid and experimental group B who was insensitive to corticosteroid, and control group was also divided into control group A who was sensitive to corticosteroid and control group B who was insensitive to corticosteroid. All patients were differentiated according to differentiation of TCM syndromes. Results 1) the age of experimental group was higher than control group (P < 0.05); the age of control group B was higher than control group A(P < 0.01), and woman in control group B had a higher incidence than control group A(P < 0.05 ); 2) the experimental group had a lower corticosteroid sensitivity than control group(P < 0.05) ; 3) qi(yang) deficiency syndrome and blood stasis syndrome in experimental group had a higher incidence than control group (P < 0.05) , but ying deficiency syndrome and damp-heat syndrome had a lower incidence than control group(P < 0.01) ; qi(yang) deficiency syndrome and rheumatism syndrome in control group A had a higher incidence than control group B(P < 0.01-0.05) , but ying deficiency syndrome had a lower incidence than control group B(P < 0.05). Conclusions 1) Our study finds that the age of IMN patients was order than other types of PNS, but the age didn't lead to occurrence of steroid-resistant patients of IMN; on the contrary, among other types of PNS patients, the age of steroid-resistant patients was higher than steroid-sensitive patients; 2) Our study confirms that corticosteroid sensitivity and pathologic types in PNS patients is connected, and IMN

  16. Juvenile idiopathic arthritis in the new world of biologics.

    Science.gov (United States)

    Ostring, Genevieve Tyra; Singh-Grewal, Davinder

    2013-09-01

    Juvenile idiopathic arthritis results in significant pain and disability in both children and adults. Advances in treatment resulting in improved long-term outcomes have occurred; however, an emphasis on early and aggressive diagnosis and management hopes to improve outcomes further. Juvenile idiopathic arthritis remains a clinical diagnosis of exclusion, but further research may delineate biological markers associated with the disease and its subtypes. Therapy for patients includes intra-articular steroid injections, disease modifying agents such as methotrexate and biological agents. Biological agents have provided exciting new therapeutic options in the last decade; however, long-term side effects of modulating the immune system are not yet fully understood. Systemic steroids may also be required but their long-term use is avoided. Uveitis needs to be screened for in all of those with the diagnosis. Multidisciplinary team care is required in managing these young people.

  17. Impact of alternate fuels on industrial refractories and refractory insulation applications. An Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Wei, G.C.; Tennery, V.J.

    1976-09-01

    The effects of use of alternate fuels such as distillate oils, residual oils, coal, producer gas, and electricity on refractory insulation are evaluated. Sections are included on alternate fuels for 1976 to 1980, assessment by industry of fuel conversion impact on industrial refractories in the period 1976 to 1980, interactions of alternate fuel combustion products with refractories and refractory insulation, and analysis of degradation mechanisms in refractories and refractory materials. (JRD)

  18. Radiation Effects in Refractory Alloys

    Science.gov (United States)

    Zinkle, Steven J.; Wiffen, F. W.

    2004-02-01

    In order to achieve the required low reactor mass per unit electrical power for space reactors, refractory alloys are essential due to their high operating temperature capability that in turn enables high thermal conversion efficiencies. One of the key issues associated with refractory alloys is their performance in a neutron irradiation environment. The available radiation effects data are reviewed for alloys based on Mo, W, Re, Nb and Ta. The largest database is associated with Mo alloys, whereas Re, W and Ta alloys have the least available information. Particular attention is focused on Nb-1Zr, which is a proposed cladding and structural material for the reactor in the Jupiter Icy Moons Orbiter (JIMO) project. All of the refractory alloys exhibit qualitatively similar temperature-dependent behavior. At low temperatures up to ~0.3TM, where TM is the melting temperature, the dominant effect of radiation is to produce pronounced radiation hardening and concomitant loss of ductility. The radiation hardening also causes a dramatic decrease in the fracture toughness of the refractory alloys. These low temperature radiation effects occur at relatively low damage levels of ~0.1 displacement per atom, dpa (~2×1024 n/m2, E>0.1 MeV). As a consequence, operation at low temperatures in the presence of neutron irradiation must be avoided for all refractory alloys. At intermediate temperatures (0.3 to 0.6 TM), void swelling and irradiation creep are the dominant effects of irradiation. The amount of volumetric swelling associated with void formation in refractory alloys is generally within engineering design limits (>10 dpa). Very little experimental data exist on irradiation creep of refractory alloys, but data for other body centered cubic alloys suggest that the irradiation creep will produce negligible deformation for near-term space reactor applications.

  19. Refractory generalised convulsive status epilepticus : a guide to treatment.

    Science.gov (United States)

    Kälviäinen, Reetta; Eriksson, Kai; Parviainen, Ilkka

    2005-01-01

    The patient with status epilepticus has continuous or rapidly repeating seizures. Generalised convulsive status epilepticus (GCSE) is the most common form of the disorder and is a life-threatening condition that requires prompt medical management. Status epilepticus that does not respond to first-line benzodiazepines (lorazepam or diazepam) or to second-line antiepileptic drugs (phenytoin/fosphenytoin, phenobarbital or valproate) is usually considered refractory and requires more aggressive treatment. The optimal treatment of refractory GCSE has not been defined, but patients should be treated in an intensive care unit, as artificial ventilation and haemodynamic support are required. Invasive haemodynamic monitoring is often necessary and EEG monitoring is essential. The drug treatment of refractory GCSE involves general anaesthesia with continuous intravenous anaesthetics given in doses that abolish all clinical and electrographic epileptic activity, often requiring sedation to the point of burst suppression on the EEG. Barbiturate anaesthetics, pentobarbital in the US and thiopental sodium in Europe and Australia, are the most frequently used agents and are highly effective for refractory GCSE both in children and adults. Indeed, they remain the only way to stop seizure activity with certainty in severely refractory cases. Other options are midazolam for adults and children and propofol for adults only.Regardless of the drug selected, intravenous fluids and vasopressors are usually required to treat hypotension. Once seizures have been controlled for 12-24 hours, continuous intravenous therapy should be gradually tapered off if the drug being administered is midazolam or propofol. Gradual tapering is probably not necessary with pentobarbital or thiopental sodium. Continuous EEG monitoring is required during high-dose treatment and while therapy is gradually withdrawn. During withdrawal of anaesthetic therapy, intravenous phenytoin/fosphenytoin or valproate should

  20. Neostigmine in the treatment of refractory constipation in critically ill children.

    Science.gov (United States)

    González, Rafael; López-Herce, Jesús; García, Ana; Botrán, Marta; Solana, Maria Jose; Urbano, Javier

    2011-08-01

    Constipation is a common complication in critically ill children and it is occasionally resistant to the drugs typically used in treatment. Neostigmine has been used in some cases of refractory constipation in critically ill adults. There is no reference to its use in critically ill children. We describe 3 cases of refractory constipation in critically ill children treated with intravenous neostigmine by continuous infusion. Two patients responded well. There were no adverse effects. We conclude that continuous intravenous neostigmine can be effective in critically ill children with refractory constipation. Further studies are necessary to determine the dose and safety of the treatment.

  1. Inheritance and genetics in idiopathic scoliosis

    OpenAIRE

    Grauers, Anna

    2015-01-01

    Idiopathic scoliosis is the most common spine deformity, affecting approximately 3% of children and adolescents. Its aetiology is still unknown. However, relatives of individuals with idiopathic scoliosis have a higher risk of developing scoliosis compared to the general population. The aim of this thesis was to improve our understanding of the hereditary and genetic background of idiopathic scoliosis. Self - reported data on scoliosis in twins (n=64,578) i...

  2. [Idiopathic interstitial pneumonias in 2016].

    Science.gov (United States)

    Debray, M-P; Borie, R; Danel, C; Khalil, A; Majlath, M; Crestani, B

    2017-02-01

    Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.

  3. Adolescent idiopathic scoliosis: current perspectives

    OpenAIRE

    2014-01-01

    Firoz MiyanjiBritish Columbia Children's Hospital, Vancouver, BC, CanadaAbstract: Adolescent idiopathic scoliosis (AIS) remains a common and potentially severe musculoskeletal disorder. Although its etiology is largely unknown, ongoing research endeavors continue to improve our understanding of its potential origin and its natural history. Advances in understanding its true three-dimensional nature have sought to improve classification and management strategies. This review will attem...

  4. Dietary therapy is the best option for refractory nonsurgical epilepsy.

    Science.gov (United States)

    Felton, Elizabeth A; Cervenka, Mackenzie C

    2015-09-01

    Ketogenic diet therapies for epilepsy have been described since the fifth century and published in scientific literature since the early 1900s. Since that time, the diet's popularity has waxed and waned as newer drugs and other treatments have been introduced. However, in recent years, dietary therapy for epilepsy has been increasingly accepted by physicians and desired by patients as an alternative to new drugs and neurostimulation. The introduction of less restrictive versions of the classic ketogenic diet, such as the modified Atkins diet (MAD), have led to increased numbers of adult patients with refractory epilepsy who are initiating dietary treatment. Approximately half of adults and children who start a ketogenic diet have a >50% seizure reduction, which is impressive given that these patients typically have medically refractory epilepsy. We believe that ketogenic dietary treatment is the best option for children and adults with refractory nonsurgical epilepsy due to its efficacy, rapid seizure reduction, synergistic effects with other antiseizure treatments, known and treatable side effects, potential to treat comorbid medical conditions, and worldwide availability.

  5. SINTERED REFRACTORY TUNGSTEN ALLOYS. Gesinterte hochschmelzende wolframlegierungen

    Energy Technology Data Exchange (ETDEWEB)

    Kieffer, R.; Sedlatschek, K.; Braun, H.

    1971-12-15

    Dependence of the melting point of the refractory metals on their positions in the periodic system - alloys of tungsten with other refractory metals - sintering of the alloys - processing of the alloys - technological properties.

  6. Company Profile Dengfeng Chunsheng Refractories Co., Ltd.

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Dengfeng Chunsheng Refractories Co., Ltd. is an industrial enterprise integrating refractories research, design, manufacturing and engineering service into one and was entitled as one of China's high-quality silica bricks production and export bases.

  7. Gastaut type idiopathic occipital epilepsy

    Directory of Open Access Journals (Sweden)

    I. V. Volkov

    2015-01-01

    Full Text Available Idiopathic occipital epilepsy is a rare epileptic syndrome. Its incidence in a Novosibirsk cohort of patients with idiopathic focal epilepsy is 0.9%. Objective: to present a clinical description of new cases of Gastaut syndrome, the types of its course, and treatment options in these patients. Patients and methods. The study covers 17 cases of Gastaut type idiopathic occipital epilepsy in 13 women and 4 men aged 11–53 years. Results. Among 17 cases we present 4 family cases with the disease. Three generations in 2 families were observed to have epilepsy, including Gastaut syndrome concurrent with childhood absence epilepsy. The adolescent onset of the disease was seen in most cases. Its main symptoms were focal visual seizures (100%, focal sensory seizures (58.9%, cephalalgia (47.1%, speech disorders (41.2%, and secondarily generalized convulsive seizures (35.3%. According to the frequency of seizures, the investigators identified 5 types of the course: single focal seizures, rare focal seizures with or without convulsions, frequent focal seizures with or without convulsions. The identity of the course of epilepsy was found in familial cases. 76.5% of the patients had a good quality of life: 41.2% of them were untreated while 35.3% were treated; no seizures were noted. 

  8. Magnesia-Chrome Refractories for Flash Furnace

    Institute of Scientific and Technical Information of China (English)

    LI Yong; CHEN Kaixian; LU Xinghua; LIU Jianlong; SUN Jialin; HONG Yanruo

    2002-01-01

    The rapid development of our country's heavy nonferrous metallurgical technology and the revolution in new type of heavynon-ferrous metallurgical furnace have imposed more critical demand on the refractory materials ,i. e. high quality and long service life. This paper presents the domestic status of the refractories for flash furnace , briefly describes the wear of the refractory used , and it is considered that the domestic in refractories for flashfurnace can be surely realized.

  9. Reuse and Reproduction of Used Refractories

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    The paper analyzed the recycle condition and developing trend of used refractories China and other countries, including research achievements of recycles of used refractories such as MgO-C bricks, Al2O3-MgO-C bricks, Al2O3-SiC-C castable and MgO-Cr2O3 bricks. Recycled refractories exhibit the same or even better properties as compared with the original. In addition, prospects for recycle of used refractories are also discusseded.

  10. Refractory disease in autoimmune diseases

    NARCIS (Netherlands)

    Vasconcelos, Carlos; Kallenberg, Cees; Shoenfeld, Yehuda

    2011-01-01

    Refractory disease (RD) definition has different meanings but it is dynamic, according to knowledge and the availability of new drugs. It should be differentiated from severe disease and damage definitions and it must take into account duration of adequate therapy and compliance of the patient. It c

  11. Management of chronic refractory cough.

    Science.gov (United States)

    Gibson, Peter G; Vertigan, Anne E

    2015-12-14

    Chronic refractory cough (CRC) is defined as a cough that persists despite guideline based treatment. It is seen in 20-46% of patients presenting to specialist cough clinics and it has a substantial impact on quality of life and healthcare utilization. Several terms have been used to describe this condition, including the recently introduced term cough hypersensitivity syndrome. Key symptoms include a dry irritated cough localized around the laryngeal region. Symptoms are not restricted to cough and can include globus, dyspnea, and dysphonia. Chronic refractory cough has factors in common with laryngeal hypersensitivity syndromes and chronic pain syndromes, and these similarities help to shed light on the pathophysiology of the condition. Its pathophysiology is complex and includes cough reflex sensitivity, central sensitization, peripheral sensitization, and paradoxical vocal fold movement. Chronic refractory cough often occurs after a viral infection. The diagnosis is made once the main diseases that cause chronic cough have been excluded (or treated) and cough remains refractory to medical treatment. Several treatments have been developed over the past decade. These include speech pathology interventions using techniques adapted from the treatment of hyperfunctional voice disorders, as well as the use of centrally acting neuromodulators such as gabapentin and pregabalin. Potential new treatments in development also show promise.

  12. Chronic Idiopathic Urticaria (CIU) is no longer idiopathic

    DEFF Research Database (Denmark)

    Maurer, M; Bindslev-Jensen, C; Gimenez-Arnau, A;

    2013-01-01

    for the same type of urticaria, which has implications for comparing study outcomes and drug licensing. Consequently, there is an urgent need for a harmonized and universally accepted nomenclature and classification of urticaria and to avoid the routine use of the outdated aetiological term, chronic idiopathic......During recent years our knowledge of the aetiology and pathogenesis of urticaria has advanced considerably allowing us to better characterize urticaria subtypes. However, although the classification of urticaria has undergone some revisions during this time (1), authors still use different names...... urticaria (CIU)....

  13. Adult onset tic disorders

    OpenAIRE

    Chouinard, S; Ford, B.

    2000-01-01

    BACKGROUND—Tic disorders presenting during adulthood have infrequently been described in the medical literature. Most reports depict adult onset secondary tic disorders caused by trauma, encephalitis, and other acquired conditions. Only rare reports describe idiopathic adult onset tic disorders, and most of these cases represent recurrent childhood tic disorders.
OBJECTIVE—To describe a large series of patients with tic disorders presenting during adulthood, to compare cl...

  14. Ultrastructural differences between diabetic and idiopathic gastroparesis

    NARCIS (Netherlands)

    Faussone-Pellegrini, Maria Simonetta; Grover, Madhusudan; Pasricha, Pankaj J.; Bernard, Cheryl E.; Lurken, Matthew S.; Smyrk, Thomas C.; Parkman, Henry P.; Abell, Thomas L.; Snape, William J.; Hasler, William L.; Uenalp-Arida, Aynur; Nguyen, Linda; Koch, Kenneth L.; Calles, Jorges; Lee, Linda; Tonascia, James; Hamilton, Frank A.; Farrugia, Gianrico

    2012-01-01

    The ultrastructural changes in diabetic and idiopathic gastroparesis are not well studied and it is not known whether there are different defects in the two disorders. As part of the Gastroparesis Clinical Research Consortium, full thickness gastric body biopsies from 20 diabetic and 20 idiopathic g

  15. Chromia refractory brick with carbon treatment

    Energy Technology Data Exchange (ETDEWEB)

    Bennett, James P.; Kwong, Kyei-Sing

    2017-03-21

    The disclosure provides a refractory brick system comprising a chromia refractory brick for operation in the slagging environment of an air-cooled gasifier. The chromia refractory brick comprises a ceramically-bonded porous chromia refractory having a porosity greater than 9% and having carbon deposits residing within the pores. The brick may be further comprised of Al.sub.2O.sub.3. The air-cooled gasifier generates a liquefied slag in contact with the refractory brick and generally operates at temperatures between 1250.degree. C. and 1575.degree. C. and pressures between 300 psi to 1000 psi, with oxygen partial pressures generally between 10.sup.-4 and 10.sup.-10 atm. The refractory brick performs without substantial chromium carbide or chromium metal formation in the low oxygen partial pressure environment. The inclusion of carbon without chromium carbide formation provides for significant mitigation of slag penetration and significantly reduced refractory wear.

  16. Idiopathic inflammatory myopathies and the lung

    Directory of Open Access Journals (Sweden)

    Jean-Christophe Lega

    2015-06-01

    Full Text Available Idiopathic inflammatory myositis (IIM is a group of rare connective tissue diseases (CTDs characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD.

  17. [Juvenile idiopathic arthritis and oral health].

    Science.gov (United States)

    Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

    2016-05-04

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

  18. Glucocorticoids in juvenile idiopathic arthritis.

    Science.gov (United States)

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  19. Imaging of juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, Karl [Birmingham Children' s Hospital, Radiology Department, Birmingham (United Kingdom)

    2006-08-15

    Over the past decade there have been considerable changes in the classification and imaging of juvenile idiopathic arthritis (JIA). Radiology now has a considerable role in the management of JIA, the differential diagnosis, monitoring disease progression and detecting complications. The different imaging modalities available, their role and limitations are discussed in this article and the various disease features that the radiologist should be aware of are described. An approach to the imaging of the child with joint disease and in the monitoring of disease complications are also discussed. (orig.)

  20. Epidemiology of adolescent idiopathic scoliosis

    OpenAIRE

    Konieczny, Markus Rafael; Senyurt, Hüsseyin; Krauspe, Rüdiger

    2012-01-01

    Adolescent idiopathic scoliosis is a common disease with an overall prevalence of 0.47–5.2 % in the current literature. The female to male ratio ranges from 1.5:1 to 3:1 and increases substantially with increasing age. In particular, the prevalence of curves with higher Cobb angles is substantially higher in girls than in boys: The female to male ratio rises from 1.4:1 in curves from 10° to 20° up to 7.2:1 in curves >40°. Curve pattern and prevalence of scoliosis is not only influenced by gen...

  1. Addison's disease presenting with idiopathic intracranial hypertension in 24-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Moore Peter

    2010-02-01

    Full Text Available Abstract Introduction Idiopathic intracranial hypertension can rarely be associated with an underlying endocrine disorder such as Cushing's syndrome, hyperthyroidism, or with administration of thyroxine or growth hormone. Though cases of idiopathic intracranial hypertension associated with Addison's disease in children have been reported, there is only one documented case report of this association in adults. We describe a case of an acute adrenal insufficiency precipitated by idiopathic intracranial hypertension in a Caucasian female. Case presentation A 24-year-old Caucasian woman was acutely unwell with a background of several months of generalised fatigue and intermittent headaches. She had unremarkable neurological and systemic examination with a normal computerised tomography and magnetic resonance imaging of the brain. Normal cerebrospinal fluid but increased opening pressure at lumbar puncture suggested intracranial hypertension. A flat short synacthen test and raised level of adrenocorticotrophic hormone were consistent with primary adrenal failure. Conclusion Addison's disease can remain unrecognised until precipitated by acute stress. This case suggests that idiopathic intracranial hypertension can rarely be associated with Addison's disease and present as an acute illness. Idiopathic intracranial hypertension is possibly related to an increase in the levels of arginine vasopressin peptide in serum and cerebrospinal fluid secondary to a glucocorticoid deficient state.

  2. Alloimmune refractoriness to platelet transfusions.

    Science.gov (United States)

    Sandler, S G

    1997-11-01

    Patients who are transfused on multiple occasions with red cells or platelets may develop platelet-reactive alloantibodies and experience decreased clinical responsiveness to platelet transfusion. This situation, conventionally described as "refractoriness to platelet transfusions," is defined by an unsatisfactory low post-transfusion platelet count increment. If antibodies to HLAs are detected, improved clinical outcomes may result from transfusions of HLA-matched or donor-recipient cross-matched platelets. Because refractoriness is an expected, frequently occurring phenomenon, prevention of HLA alloimmunization is an important management strategy. Prevention strategies include efforts to decrease the number of transfusions, filtration of cellular components to reduce the number of HLA-bearing leukocytes, or pretransfusion ultraviolet B irradiation of cellular components to decrease their immunogenicity. Other investigational approaches include reducing the expression of HLAs on transfused platelets, inducing a transient reticuloendothelial system blockade by infusions of specialized immunoglobulin products, or transfusing semisynthetic platelet substitutes (thromboerythrocytes, thrombospheres) or modified platelets (infusible platelet membranes, lyophilized platelets).

  3. "Immune Complexes in Juvenile Idiopathic Arthritis"

    Directory of Open Access Journals (Sweden)

    Terry Lynn Moore

    2016-05-01

    Full Text Available Abstract for invited review in Molecular Mechanisms of Immune Complex Pathophysiology thematic issue to be published in Frontiers in Immunology. Immune Complexes(ICin Juvenile Idiopathic Arthritis (JIA Terry L. Moore, MD, FAAP, FACR, MACR Professor of Internal Medicine,Pediatrics, and Molecular Biology and Immunology Director of Adult and Pediatric Rheumatology Saint Louis University School of Medicine Saint Louis, Missouri 631`04,USA Juvenile idiopathic arthritis (JIA reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs and associated complement activation by-products in their sera. ICs have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column,C1q solid phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these IC s have shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF, isotypes of anti-cyclic citrullinated (CCP peptide antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We

  4. Iron refractory iron deficiency anemia

    OpenAIRE

    De Falco, Luigia; Sanchez, Mayka; Silvestri, Laura; Kannengiesser, Caroline; Muckenthaler, Martina U; Iolascon, Achille; Gouya, Laurent; Camaschella, Clara; Beaumont, Carole

    2013-01-01

    Iron refractory iron deficiency anemia is a hereditary recessive anemia due to a defect in the TMPRSS6 gene encoding Matriptase-2. This protein is a transmembrane serine protease that plays an essential role in down-regulating hepcidin, the key regulator of iron homeostasis. Hallmarks of this disease are microcytic hypochromic anemia, low transferrin saturation and normal/high serum hepcidin values. The anemia appears in the post-natal period, although in some cases it is only diagnosed in ad...

  5. KDIGO指南解读:成人微小病变肾病和特发性局灶节段性肾小球硬化治疗%Management of minimal-change disease and idiopathic focal segmental glomerulosclerosis in adults:interpretation of KDIGO guideline

    Institute of Scientific and Technical Information of China (English)

    陈楠; 任红

    2012-01-01

    Minimal-change disease( MCD) and idiopathic focal segmental glomerulosclerosis (KSGS) are common reasons for nephrotic syndrome in adults. The KDIGO clinical practice guideline for glomerulonephritis published in June,2012 provided the newest recommendations on the treatment of primary and secondary glomerulonephritis. The doses and courses of glucocorticosteroid treatment in the first episode of MOD and FSGS,in relapsed patients,in corticosteroid dependent or corti-costeroid resistant patients were suggested. The recommendations for the choice of first-line immunosuppressive agents were given. Here we unscrambled the KDIGO guideline on the treatment of MCD and FSGS in adults regarding the characteristics of Chinese patients.%微小病变肾病(MCD)和特发性局灶节段性肾小球硬化(FSGS)是成人肾病综合征的常见病理类型.2012年6月发表的改善全球肾脏病预后组织(KDIGO)指南对多种原发及继发性肾小球肾炎的治疗给出了最新意见,对初发、复发、激素依赖和激素抵抗型MCD和FSGS患者的激素使用剂量、疗程及一线免疫抑制剂的选择等给予了建议.本文结合中国患者的特点对该指南关于MCD和FSGS治疗的建议做一解读.

  6. Qualitative Dermatoglyphics In Idiopathic Epilepsy

    Directory of Open Access Journals (Sweden)

    Ranganath Priya

    2004-01-01

    Full Text Available Genetic aetiology has been proposed for both idiopathic epilepsy and dermatoglyphics. Hence, the present study has been undertaken to find out the existence of any correlation between dermatoglyphics and idiopathic epilepsy. Material consisted of 100 patients (58 males and 42 females and 100 controls (52 males and 48 females. Patient′s age ranged from 5 to 40 years and controls were between 18 and 25 years. Dermatoglyphics were obtained by painting method. Qualitative parameters observed were percentage frequency of fingerprint patterns (loops, whorls and arches,, patterns in hypothenar area/ interdigital are and flexion creases (Simian crease, sydney line. On comparison with controls, in males, with hands combined, loops (52.24% and arches (7.93% were increased and whorls (39.83% were decreased (p<0.05. In females, with hands combined, arches (13.1% and whorls (36.43% were increased and loops (50.48% were decreased (p< 0.03. Significant differences have not been observed for the patterns in hypothenar area /interdigital area and flexion creases. These dermatoglyphics features could be used as additional markers to evaluate patients of epilepsy.

  7. Posterior asymmetry and idiopathic scoliosis

    CERN Document Server

    Rousie, D L; Berthoz, A

    2009-01-01

    Study design Are there neuro-anatomical abnormalities associated with idiopathic scoliosis (IS)? Posterior Basicranium (PBA) reflects cerebellum growth and contains vestibular organs, two structures suspected to be involved in scoliosis. Objective The aim of this study was to compare posterior basicranium asymmetry (PBA) in Idiopathic scoliosis (IS) and normal subjects. Method: To measure the shape of PBA in 3D, we defined an intra-cranial frame of reference based on CNS and guided by embryology of the neural tube. Measurements concerned three directions of space referred to a specific intra cranial referential. Data acquisition was performed with T2 MRI (G.E. Excite 1.5T, mode Fiesta). We explored a scoliosis group of 76 women and 20 men with a mean age of 17, 2 and a control group of 26 women and 16 men, with a mean age of 27, 7. Results: IS revealed a significant asymmetry of PBA (Pr>|t|<.0001) in 3 directions of space compared to the control group. This asymmetry was more pronounced in antero-posterior...

  8. Understanding and treating refractory constipation

    Institute of Scientific and Technical Information of China (English)

    Gabrio; Bassotti; Corrado; Blandizzi

    2014-01-01

    Chronic constipation is a frequently encountered disorder in clinical practice. Most constipated patients benefit from standard medical approaches. However, current therapies may fail in a proportion of patients. These patients deserve better evaluation and thorough investigations before their labeling as refractory to treatment. Indeed, several cases of apparent refractoriness are actually due to misconceptions about constipation, poor basal evaluation (inability to recognize secondary causes of constipation, use of constipating drugs) or inadequate therapeutic regimens. After a careful reevaluation that takes into account the above factors, a certain percentage of patients can be defined as being actually resistant to first-line medical treatments. These subjects should firstly undergo specific diagnostic examination to ascertain the subtype of constipation. The subsequent therapeutic approach should be then tailored according to their underlying dysfunction. Slow transit patients could benefit from a more robust medical treatment, based on stimulant laxatives (or their combination with osmotic laxatives, particularly over the short-term), enterokinetics (such as prucalopride) or secretagogues (such as lubiprostone or linaclotide). Patients complaining of obstructed defecation are less likely to show a response to medical treatment and might benefit from biofeedback, when available. When all medical treatments prove to be unsatisfactory, other approaches may be attempted in selected patients (sacral neuromodulation, local injection of botulinum toxin, anterograde continence enemas), although with largely unpredictable outcomes. A further although irreversible step is surgery (subtotal colectomy with ileorectal anastomosis or stapled transanal rectal resection), which may confer some benefit to a few patients with refractoriness to medical treatments.

  9. Idiopathic dystonia clinical, profile of 76 brazilian patients

    Directory of Open Access Journals (Sweden)

    Luiz A. F. Andrade

    1992-12-01

    Full Text Available Dystonia may be classified by age of onset (childhood, adolescence, adult onset, body distribution of the abnormal movements (focal, segmental, unilateral, multifocal and generalized and etiology (idiopathic and symptomatic. We studied 76 patients with idiopathic dystonia among 122; cases of dystonic syndrome (62.3% of the total. There were 48 female and 28 male patients. Adult-onset focal dystonia was the most frequent feature (37 patients. The onset of generalized dystonia was more frequently seen under the age of 20, whereas focal and segmental dystonia usually started over this age. Postural tremor of the hands was observed in 19.7% of the patients. Spasmodic torticollis was the most prevalent form of dystonia overall. Except for writer's cramp, which occurred more frequently in males, and generalized dystonia, which was equally divided between sexes, all other forms were more frequent in females. Our data suggest that differences in racial origin, social and economical status and environmental factors do not account for a different manifestation in dystonia pattern.

  10. Vitamin D deficiency in chronic idiopathic urticaria.

    Science.gov (United States)

    Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

    2015-04-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

  11. Flavopiridol in Treating Patients With Relapsed or Refractory Lymphoma or Multiple Myeloma

    Science.gov (United States)

    2016-06-27

    Adult Lymphocyte Depletion Hodgkin Lymphoma; Adult Lymphocyte Predominant Hodgkin Lymphoma; Adult Mixed Cellularity Hodgkin Lymphoma; Adult Nodular Sclerosis Hodgkin Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Splenic Marginal Zone Lymphoma; Stage I Multiple Myeloma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Waldenström Macroglobulinemia

  12. Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort.

    Science.gov (United States)

    Bodoki, Levente; Nagy-Vincze, Melinda; Griger, Zoltán; Betteridge, Zoe; Szöllősi, Lászlóné; Dankó, Katalin

    2014-12-01

    Idiopathic inflammatory myopathies (IIMs) are chronic systemic autoimmune diseases characterised by symmetrical, proximal muscle weakness. Dermatomyositis represents one subset of IIMs, in which skin rashes are present in addition to muscle weakness. Myositis-specific antibodies can only be detected in myositis, and they are directed against specific proteins found in the cytoplasm or in the nucleus of cells. With this case-based article, we introduce the recently detected anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5 antibodies that form various clinical groups. These antibodies could be detected in patients with dermatomyositis. The myositis-specific autoantibodies of three hundred and thirty-seven Hungarian patients with IIM were detected. Retrospective analysis of the clinical findings has also been introduced by revision of the medical history. We had twelve patients with anti-TIF1γ positivity, four patients with anti-NXP2 positivity and four patients with anti-SAE positivity. We did not have any positive anti-MDA5 patients. The most relevant clinical findings were similar to those seen in previously published reports. Eleven of the twelve patients with anti-TIF1γ positivity had classical dermatomyositis. Three of the twelve anti-TIF1γ patients had cancer during the disease progression. This was two out of four for the anti-NXP2 subgroup and one in four for the anti-SAE subgroup. In two juvenile dermatomyositis cases, typical ulceration was seen in patients with anti-TIF1γ positivity. The frequency of pulmonary fibrosis during the disease progression was 2/12, 1/4 and 1/4 in anti-TIF1γ, anti-NXP2 and anti-SAE, respectively. Other extra-muscular manifestations, such as arthralgia, dysphagia, dysphonia and dyspnoea, were also detectable. The myositis subgroups determined by these myositis-specific autoantibodies differ from each other in their symptoms, prognosis and therapy responsiveness. Their detection is helpful for the preparation of an adequate

  13. Idiopathic subglottic stenosis: a familial predisposition.

    Science.gov (United States)

    Dumoulin, Elaine; Stather, David R; Gelfand, Gary; Maranda, Bruno; Maceachern, Paul; Tremblay, Alain

    2013-03-01

    Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis. Although there is a female preponderance, no familial cases have been reported in the literature. We describe two pairs of sisters as well as a mother and daughter presenting with idiopathic subglottic stenosis. All known causes of tracheal stenosis were excluded, including prolonged intubation, surgery, autoimmune and inflammatory disorders, infection and gastroesophageal reflux disease. These are the first cases reported in the literature that suggest a genetic predisposition for idiopathic subglottic stenosis.

  14. Refractories Quality Improvement for Glass Industry Upgrading

    Institute of Scientific and Technical Information of China (English)

    ZENG Dafan

    2009-01-01

    @@ 1 Glass Industry and Refractories Industry Closely Connect 1.1 Glass Development Drives Refractories Progress Refractories are indispensable to glass industry; the rapid development of glass industry drives the growth of refractories industry. China's glass industry developed slowly before the mid 1980s. The kilns and furnaces were backward and small-scale with furnace life of only 2-3 years; glass was produced with extremely low efficiency and poor quality. During that period, refractories for glass melting furnaces had very limited varieties and inferior quality. The fused cast refractories for advanced glass melting furnaces were imported, for the materials made in China could not meet the requirements, which seriously restrained the technical progress of China's glass industry.

  15. Phase 1 trial of gemtuzumab ozogamicin in combination with enocitabine and daunorubicin for elderly patients with relapsed or refractory acute myeloid leukemia: Japan Adult Leukemia Study Group (JALSG)-GML208 study.

    Science.gov (United States)

    Ito, Yoshikazu; Wakita, Atsushi; Takada, Satoru; Mihara, Masahiro; Gotoh, Moritaka; Ohyashiki, Kazuma; Ohtake, Shigeki; Miyawaki, Shuichi; Ohnishi, Kazunori; Naoe, Tomoki

    2012-10-01

    We conducted a phase 1 study of a combination of gemtuzumab ozogamicin (GO) plus conventional chemotherapy in elderly patients (≥ 65 years old) with relapsed or refractory CD33-positive acute myeloid leukemia (AML). Patients received a standard dose of enocitabine (200 mg/m² × 8 days) and daunorubicin (30 mg/m² × days 1-3) plus an escalating dose of GO (1.5-5 mg/m² on day 4). The dose escalation of GO was done according to a standard 3 + 3 design following a modified Fibonacci sequence. No dose-limiting toxicities were observed in three patients (median age, 71) at level 1 (1.5 mg/m²) or in three patients (median age, 73) at level 2 (3 mg/m²). Neither veno-occlusive diseases nor sinusoidal obstructive syndromes were noted at either level. However, as GO was withdrawn from the US market in June 2010, based on a randomized study in newly diagnosed AML, we decided not to proceed to the level 3 (5 mg/m²) in order to avoid possibly more severe adverse effects, and also because all six patients experienced grade 4 myelosuppression, with complete remission in three. This study showed that 3 mg/m² of GO in combination with enocitabine and daunorubicin may be a recommendable dose for a phase 2 study in Japanese elderly patients with CD33-positive AML. The study was registered at the University Hospital Medical Information Network (UMIN) Clinical Trials Registry ( http://www.umin.ac.jp/ctr/ ) as UMIN000002603.

  16. Decitabine Followed by Idarubicin and Cytarabine in Treating Patients With Relapsed or Refractory Acute Myeloid Leukemia or Myelodysplastic Syndromes

    Science.gov (United States)

    2013-10-09

    Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts

  17. Idiopathic arterial calcification in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Maya [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Red Cross Children' s Hospital, School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch, Cape Town (South Africa); Andronikou, Savvas; Solomon, Rustum; Sinclair, Paul; McCulloch, Mignon [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2004-08-01

    Idiopathic arterial calcification in infancy is usually fatal with death in early life and diagnosis at post mortem. This report describes a unique, late presentation with hypertension and cardiac failure in a child aged 33 months, found to have widespread arterial calcification at radiological imaging. The calcium-phosphate axis was normal and there was no other demonstrable cause for calcification. Additionally, the histological features of arterial calcification at renal biopsy paralleled the findings in infants with this disorder. The late presentation in this case is unusual and has not been previously reported. Ultrasound and CT are sensitive for calcification, and the disease should be suspected in children presenting with cardiac or respiratory manifestations and features of arterial calcification, where no metabolic cause is established. (orig.)

  18. Idiopathic Renal Infarction Mimicking Appendicitis

    Science.gov (United States)

    Lisanti, Francesco; Scarano, Enrico

    2017-01-01

    Renal infarction is a rare cause of referral to the emergency department, with very low estimated incidence (0.004%–0.007%). Usually, it manifests in patients aged 60–70 with risk factors for thromboembolism, mostly related to heart disease, atrial fibrillation in particular. We report a case of idiopathic segmental renal infarction in a 38-year-old patient, presenting with acute abdominal pain with no previous known history or risk factors for thromboembolic diseases. Because of its aspecific clinical presentation, this condition can mimic more frequent pathologies including pyelonephritis, nephrolithiasis, or as in our case appendicitis. Here we highlight the extremely ambiguous presentation of renal infarct and the importance for clinicians to be aware of this condition, particularly in patients without clear risk factors, as it usually has a good prognosis after appropriate anticoagulant therapy. PMID:28203466

  19. Idiopathic interstitial pneumonias: Classification revision

    Directory of Open Access Journals (Sweden)

    Demosthenes Bouros MD, PhD, FCCP

    2010-01-01

    Full Text Available The American Thoracic Society (ATS, the European Respiratory Society (ERS and the Japan Respiratory Society (JRS are planning a revision of the 2002 ATS/ERS International Multidisciplinary Classification of Idiopathic Interstitial Pneumonias (IIPs1. In two years’ time it will be 10 years since its publication and with a view to publishing the revision after 10 years (i.e., in 2012, a steering committee has been established, which met in New Orleans during ATS congress in May 2010 and more recently in Barcelona during the ERS congress (Photo. The committee will meet again during the ATS and the ERS congresses that will be held in the next two years, with an additional meeting in Modena, Italy, in Αpril 2011.

  20. Idiopathic Inflammatory Myopathies: An update

    Directory of Open Access Journals (Sweden)

    Bulent KURT

    2016-06-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of disease with complex clinical features. It has been sub-classified as: (1 Dermatomyositis, (2 Polymyositis, and (3 Inclusion body myositis (IBM. Nowadays, there are some studies in literature suggest necrotizing autoimmune myopathy and immune-mediated necrotizing myopathy should also be added to this group of disease. There is a debate in the diagnosis of IIMs and up until now, about 12 criteria systems have been proposed. Some of the criteria systems have been used widely such as Griggs et al.'s proposal for IBM. Clinical findings, autoantibodies, enzymes, electrophysiological, and muscle biopsy findings are diagnostic tools. Because of diseases' complexity, none of the findings are diagnostic alone. In this study, we discussed the diagnostic criteria of IMMs and described detailed morphological features. [J Interdiscipl Histopathol 2016; 4(2.000: 41-45

  1. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  2. Nonchylous idiopathic pleural effusion in the newborn

    Directory of Open Access Journals (Sweden)

    Geeta Gathwala

    2011-01-01

    Full Text Available Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

  3. Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies

    Directory of Open Access Journals (Sweden)

    Aarat Patel

    2016-01-01

    Full Text Available Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required. There are second-line therapies available, but many patients are also refractory to those options. H.P. Acthar® Gel (repository corticotropin injection [RCI] is a melanocortin peptide that can induce steroid-dependent effects and steroid-independent effects. Herein, we present a series of cases that involved the use of RCI in the management of dermatomyositis and polymyositis. RCI treatments resulted in improvement in three of four patients, despite failure with previous therapies. The use of RCI did not exacerbate any comorbidity and no significant changes in blood pressure, weight, or glycemic control were observed. Overall, these results are encouraging and suggest that randomized, controlled clinical trials applying RCI to dermatomyositis and polymyositis are warranted.

  4. Polyarticular juvenile idiopathic arthritis - epidemiology and management approaches.

    Science.gov (United States)

    Oberle, Edward J; Harris, Julia G; Verbsky, James W

    2014-01-01

    Juvenile idiopathic arthritis (JIA) is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months) is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.

  5. Idiopathic scoliosis: etiological concepts and hypotheses

    OpenAIRE

    2013-01-01

    Scoliosis is diagnosed as idiopathic in 70 % of structural deformities affecting the spine in children and adolescents, probably reflecting our current misunderstanding of this disease. By definition, a structural scoliosis should be the result of some primary disorder. The goal of this article is to give a comprehensive overview of the currently proposed etiological concepts in idiopathic scoliosis regarding genetics, molecular biology, biomechanics, and neurology, with particular emphasis o...

  6. Unilateral Idiopathic Calcinosis Cutis: A Case Report

    Science.gov (United States)

    Alsaif, Fahad; Abduljabbar, Amr M.

    2017-01-01

    Calcinosis cutis is a rare disorder characterized by the deposition of calcium in the skin and subcutaneous tissue. Unilateral idiopathic calcinosis cutis has only rarely been reported in the literature. Here, we report the case of a 7-year-old healthy girl who presented with multiple asymptomatic hard nodules on the right side of her body. Histopathological, radiological, and extensive blood investigations confirmed the diagnosis of unilateral idiopathic calcinosis cutis. PMID:28203159

  7. Cognitive function in idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne Maria; Fagerlund, Birgitte; Forchhammer, Hysse Birgitte;

    2014-01-01

    OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department and a terti......OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department...

  8. Family history of idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Dauvilliers, Yves; Postuma, Ronald B; Ferini-Strambi, Luigi;

    2013-01-01

    To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort.......To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort....

  9. TNF and LT binding capacities in the plasma of arthritis patients: effect of etanercept treatment in juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Gudbrandsdottir, S; Larsen, R; Sørensen, L K;

    2004-01-01

    Etanercept (Enbrel) induces a rapid and sustained decline in disease activity in the majority of patients with refractory juvenile idiopathic arthritis (JIA). For unknown reasons, however, a number of JIA patients fail to respond to this therapy. During this treatment neutralisation of tumour...... necrosis factor (TNF, previously termed TNF alpha) and lymphotoxin (LT, previously termed TNF beta) may be mediated by etanercept itself as well as by naturally occurring soluble TNF receptors. In light of this, it was of interest to study the total TNF neutralizing capacity in plasma before and during...... treatment with etanercept....

  10. Genetics Home Reference: iron-refractory iron deficiency anemia

    Science.gov (United States)

    ... refractory iron deficiency anemia iron-refractory iron deficiency anemia Enable Javascript to view the expand/collapse boxes. ... All Close All Description Iron-refractory iron deficiency anemia is one of many types of anemia , which ...

  11. Arsenic Trioxide in Treating Patients With Relapsed or Refractory Lymphoma or Leukemia

    Science.gov (United States)

    2013-01-31

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  12. Refractory ceramic fibers (RCFs) induce germline aneuploidy in Drosophila oocytes.

    Science.gov (United States)

    Osgood, C J

    1994-06-01

    Mineral fibers are ubiquitous in the natural environment and are widely used in industry in such applications as insulators. We have previously shown that asbestos fibers induce aneuploidy in oocytes of Drosophila melanogaster; here we extend those studies by testing refractory ceramic fibers (RCFs) for their mutagenicity to germ cells. The results establish that the tested RCFs are inducers of aneuploidy following feeding of adult females. A subset of the RCFs were also effective following larval feeding. Our results suggest that RCFs, like certain asbestos fibers, may pose a hazard to germ cells.

  13. Idiopathic thrombocytopenic purpura: laboratory diagnosis and management

    Directory of Open Access Journals (Sweden)

    Alvina Alvina

    2016-02-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP or immune thrombocytopenic purpura is a disease characterized by low platelet count (<150,000/ìL caused by autoantibody-mediated platelet destruction and the absence of other causes of thrombocytopenia. Acute primary ITP is more common in children 2-6 years of age, with similar incidence between males and females, while the chronic form is usually encountered in adults with median age of 40-45 years. The clinical signs of ITP are purpura, ecchymosis, petechiae and gastrointestinal tract bleeding, gingival bleeding, epistaxis, and urinary tract bleeding. Spontaneous mucosal, intracranial, and gastrointestinal hemorrhage may occur at platelet counts of <10000/ìL. To date, the diagnosis of ITP is still arrived at by exclusion, i.e. by elimination of other causes of thrombocytopenia. The diagnosis of ITP also requires a medical history (anamnesis, physical examination, platelet count, and examination of a peripheral blood smear. The latter examination in ITP shows low numbers of normal-sized platelets, occasionally also giant platelets, while erythrocytes and leukocytes have a normal morphology. The bone marrow is usually normal or shows increased megakaryocytes. Assessment of antithrombocyte antibody may assist in establishing the diagnosis of ITP. Management of ITP is based on platelet count and severity of bleeding. Treatment is aimed at interfering with antibodies that damage the platelets, by inhibiting the functions of macrophage Fcã receptors and decreasing the production of antiplatelet antibodies. Thrombopoietin (TPO receptor agonists including eltrombopag and romiplostim have offered an important new option in treating ITP.

  14. Management of refractory cardiogenic shock.

    Science.gov (United States)

    Reyentovich, Alex; Barghash, Maya H; Hochman, Judith S

    2016-08-01

    Cardiogenic shock is a life-threatening condition that occurs in response to reduced cardiac output in the presence of adequate intravascular volume and results in tissue hypoxia. Cardiogenic shock has several underlying aetiologies, with the most common being acute myocardial infarction (AMI). Refractory cardiogenic shock presents as persistent tissue hypoperfusion despite administration of adequate doses of two vasoactive medications and treatment of the underlying aetiology. Investigators of the SHOCK trial reported a long-term mortality benefit of emergency revascularization for shock complicating AMI. Since the publication of the SHOCK trial and subsequent guideline recommendations, the increase in community-based use of percutaneous coronary intervention for this condition has resulted in a significant decline in mortality. Despite these successes in the past 15 years, mortality still remains exceptionally high, particularly in patients with refractory cardiogenic shock. In this Review, we discuss the aetiology and pathophysiology of cardiogenic shock and summarize the data on the available therapeutics and their limitations. Although new mechanical circulatory support devices have been shown to improve haemodynamic variables in patients with shock complicating AMI, they did not improve clinical outcomes and are associated with high costs and complications.

  15. Case 40. Misdiagnosis of refractory macrocytic anemia.

    Science.gov (United States)

    Stringaris, Kate; Bain, Barbara

    2008-11-01

    A diagnosis of myelodysplastic syndrome, refractory anemia subtype, was made in an elderly Indian woman on the basis of a refractory macrocytic anemia with normal vitamin B(12) and folate assays, normal thyroid function, essentially normal liver function and normal cytogenetic analysis. Disease evolution revealed that the diagnosis was erroneous.

  16. Research on High Performance New Refractories

    Institute of Scientific and Technical Information of China (English)

    Zhong Xiangchong

    2007-01-01

    @@ At the age of 86,Prof.Zhong Xiangchong has his new book-"Research on High Performance New Refractories" published by Henan Science and Technology Publishing House to commemorate his 65 years service in the refractories science and technology including the recent 6 years at High Temperature Ceramics Institute which he founded in Zhengzhou University in 2001.

  17. Spectral reflecting characteristics of dinas refractory

    Energy Technology Data Exchange (ETDEWEB)

    Zapechnikov, V.N.; Pushkin, V.T.; Zen' kovskii, A.G.

    1987-09-01

    This article outlines a nondestructive testing format which uses spectral reflectance to assess the remaining service life, the thermal degradation, and the insulating effectiveness of refractories used as insulating liners in glass melters. Thermal as well as mechanical influences on the reflectance behavior of several refractories are discussed.

  18. Modern technologies of refractory glaucoma treatment. Review

    Directory of Open Access Journals (Sweden)

    A. Yu. Rascheskov

    2014-07-01

    Full Text Available this article presents a review of available literature concerning basic directions of refractory glaucoma treatment. the surgical management of refractory glaucoma is presented by two main groups of surgical techniques: filtering surgery (including surgery with the use of antimetabolites and implants and cyclodestructive procederes. types of glaucoma drainages are described, modern valve drainage system is presented.

  19. Refractory Pellet for Hot Blast Stove

    Institute of Scientific and Technical Information of China (English)

    Wang Jing; Peng Xigao

    2011-01-01

    1 Scope This standard specifies the term,definition,classification,specification,technical requirements,test methods,quality appraisal procedures,packing,marking,transportation,storage,and quality certificate of refractory pellet for hot blast stove.This standard is applicable to refractory pellet for hot blast stove.

  20. Plasma Exchange for Renal Vasculitis and Idiopathic Rapidly Progressive Glomerulonephritis: A Meta-analysis

    DEFF Research Database (Denmark)

    Walsh, Michael; Catapano, Fausta; Szpirt, Wladimir;

    2010-01-01

    BACKGROUND:: Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. STUDY DESIGN:: Systematic review and meta-analysis of articles identified from...... electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. SETTING & POPULATION:: Adults with idiopathic renal vasculitis or rapidly progressive glomerulonephritis. SELECTION CRITERIA FOR STUDIES:: Randomized controlled trials that compared...... standard care with standard care plus adjuvant plasma exchange in adult patients with either renal vasculitis or idiopathic rapidly progressive glomerulonephritis. INTERVENTION:: Adjuvant plasma exchange. OUTCOME:: Composite of end-stage renal disease or death. RESULTS:: We identified 9 trials including...

  1. Increased mean platelet volume in patients with idiopathic subjective tinnitus.

    Science.gov (United States)

    Sarıkaya, Yasin; Bayraktar, Cem; Karataş, Mehmet; Doğan, Sedat; Olt, Serdar; Kaskalan, Emin; Türkbeyler, İbrahim Halil

    2016-11-01

    Tinnitus is the perception of sound with no external stimulus and idiopathic subjective tinnitus is the most common type in adults. Mean platelet volume (MPV) alterations were shown in some inflammatory diseases and were evaluated as a clinically useful marker. Our aim was to investigate MPV alterations in idiopathic subjective tinnitus patients. A total of 101 patients and 54 age- and sex-matched healthy control subjects were enrolled in the study. Patients included in the study had complaints of tinnitus for at least 3 months. All patients underwent detailed otolaryngologic examination, blood sampling, pure tone audiometry, magnetic resonance imaging of ear, and vertebrobasilar artery Doppler ultrasonography to make the differential diagnosis of tinnitus. Blood sampling consisted of renal-liver-thyroid function tests, lipid profile, and complete blood count. All tests and examinations except the imaging modalities were also performed for the control group. There were no differences in age and sex distribution of groups. Mean platelet volume values were significantly increased in tinnitus patients when compared with controls (p = 0.001). We think that MPV can be qualified as a useful marker in tinnitus patients.

  2. TOTAL JOINT REPLACEMENT OF THE LOWER EXTREMITY IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

    OpenAIRE

    Стюарт Б. Гудмэн

    2014-01-01

    Joint replacement of the lower extremity in Juvenile Idiopathic Arthritis (JIA) is becoming more commonly performed worldwide. These young adults experience severe pain and disability from end-stage arthritis, and require joint replacement of the hip or knee to alleviate pain, and restore ambulation and function. These procedures are very challenging from the anesthesia and surgical point of view, due to small overall proportions, numerous bony and other deformities and soft tissue contractur...

  3. Juvenile idiopathic arthritis overview and involvement of the temporomandibular joint: prevalence, systemic therapy.

    Science.gov (United States)

    Carrasco, Ruy

    2015-02-01

    The temporomandibular joint (TMJ) is one of the many joints involved in the inflammatory arthritides. As imaging of joints has developed, so have the data regarding extent and prevalence of TMJ involvement in these diseases. TMJ disease is especially prevalent in juvenile arthritis. The adult and pediatric inflammatory arthritides share common pathophysiology but are still markedly different. The preponderance of TMJ arthritis research exists in juvenile arthritis. This article discusses classification, treatment, and TMJ involvement in juvenile idiopathic arthritis.

  4. Diagnosis and treatment of adolescent idiopathic scoliosis.

    Science.gov (United States)

    Burton, Monique S

    2013-11-01

    Scoliosis is defined as a lateral curvature of the spine greater than 10 degrees on radiography that is typically associated with trunk rotation. The three major types of scoliosis are congenital, idiopathic, and neuromuscular. Idiopathic scoliosis is divided into three subcategories based on the age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between 3 and 10 years, and adolescent idiopathic scoliosis (AIS) occurs in skeletally immature patients older than 10 years. AIS is the most common form of idiopathic scoliosis. Approximately 2% to 4% of children aged 10 to 16 years have some degree of spinal curvature. Although some researchers view routine screening for AIS as controversial, well-child examinations and sports physicals are an optimal time to evaluate for AIS in the clinical setting. In 2008, the American Academy of Orthopaedic Surgeons, the Scoliosis Research Society, the Pediatric Orthopaedic Society of North America, and the American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening and issued an information statement concluding that although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment can be substantial. Recommendations are now that females are screened twice, at age 10 and 12 years, and males once at age 13 or 14 years. Screening during routine well-child examinations and/or school-based evaluations will help identify patients who need ongoing monitoring. The evaluation of curvatures in conjunction with the level of skeletal maturity will help to guide the management of the curvature.

  5. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Jun Wei

    2005-03-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  6. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Laing

    2005-10-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  7. Genetics Home Reference: familial idiopathic basal ganglia calcification

    Science.gov (United States)

    ... idiopathic basal ganglia calcification ( FIBGC , formerly known as Fahr disease) is a condition characterized by abnormal deposits of ... on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). Am J Hum Genet. 1999 Sep;65(3): ...

  8. Measures of Autonomic Dysfunction in Diabetic and Idiopathic Gastroparesis

    Science.gov (United States)

    Mohammad, Mohammad Khalid; Pepper, Dominique J.; Kedar, Archana; Bhaijee, Feriyl; Familoni, Babajide; Rashed, Hani; Cutts, Teresa; Abell, Thomas L.

    2016-01-01

    Background Gastroparesis is a condition classically characterized by delayed gastric emptying and is associated with considerable morbidity. While the etiology of gastroparesis remains elusive, autonomic dysfunction may play an important role, especially as many patients with gastroparesis also have diabetes. The aim of this study was to determine whether measures of autonomic function differ between adults with diabetic gastroparesis (DG) and adults with idiopathic gastroparesis (IG). Methods Tests of systemic autonomic function were performed among 20 adults with GD (six men and 14 women, mean age: 42 years) and 21 adults with IG (seven men and 14 women, mean age: 37 years). Measures included vagal cholinergics by R-R interval percentage variation (RRI-PV) and sympathetic adrenergics by vasoconstriction to cold (VC) and postural adjustment ratio (PAR). The two groups were compared using Wilcoxon rank sum tests and linear regression analysis (STATA 10.0). Results In univariate analysis, the following autonomic measures differed significantly between DG and IG: VC (P = 0.004), PAR (P = 0.045), VC + PAR (P = 0.002) and RRI-PV (P < 0.001). In multivariate analysis (P = 0.002, R2 = 0.55), only RRI-PV (adjusted odds ratio (aOR): 1.02, 95% confidence interval (CI): 1.01 - 1.03) differed significantly between DG and IG patients. Conclusions Vagal cholinergics are affected to a greater degree in DG compared to IG, suggesting that impaired vagal tone is not a universal mechanism for gastroparesis. PMID:27785328

  9. [Managing of platelet transfusion refractoriness of haematological malignancies. Experience IPC-EFSAM].

    Science.gov (United States)

    Dettori, I; Ladaique, P

    2014-11-01

    The platelet refractoriness is a complication of transfusion treatments potentially dramatic in onco-haematology. Chemo-treatment of haematological malignancies or packs of allogeneic bone marrow transplants require iterative platelet transfusion requirements. The discovery of a platelet refractoriness along with its support should be the most reactive as possible but also adapted to the cause. In the case of allo-immunization, it may be expected. The purpose of this presentation is to recall the different etiologies and perform a feedback on the support transfusion platelet of onco-haematology adult patients at Institut Paoli-Calmettes (IPC) in partnership with the EFSAM.

  10. Telltale teeth: Idiopathic Hypergonadotropic Hypogonadism

    Directory of Open Access Journals (Sweden)

    G S Lele

    2014-01-01

    Full Text Available The detection of any atypical extraoral or intraoral features warrants a thorough investigation, even if the patient is asymptomatic or unaware of these. At times, dental findings help in the diagnosis of an underlying systemic problem. These findings may or may not be associated with any syndrome. Thus, thorough examination and exhaustive investigations should be carried out for every atypical finding to ensure optimal oral and general health for the patient. Case Description: This is a case report of seventeen year old male who presented with peculiar/atypical dentition which ′told the tale′ and led to the diagnosis of underlying endocrinological problem about which the parents were totally unaware. The patient was short with central obesity and microcephaly. Intraorally, there was presence of thirty six microdonts. Consultation with pediatrician and endocrinologist, and thorough investigations confirmed the condition to be of ′Idiopathic Hypergonadotropic Hypogonadism′. The patient underwent not only oral rehabilitation, but also timely consultation and treatment from a pediatrician and an endocrinologist.

  11. Idiopathic fistula-in-ano

    Institute of Scientific and Technical Information of China (English)

    Sherief Shawki; Steven D Wexner

    2011-01-01

    Fistula-in-ano is the most common form of perineal sep- sis. Typically, a fistula includes an internal opening, a track, and an external opening. The external opening might acutely appear following infection and/or an abs-cess, or more insiduously in a chronic manner. Mana-gement includes control of infection, assessment of the fistulous track in relation to the anal sphincter muscle, and finally, definitive treatment of the fistula. Fistulo-tomy was the most commonly used mode of manage-ment, but concerns about post-fistulotomy incontinence prompted the use of sphincter preserving techniques such as advancement flaps, fibrin glue, collagen fistula plug, ligation of the intersphincteric fistula track, and stem cells. Many descriptive and comparative studies have evaluated these different techniques with variable outcomes. The lack of consistent results, level I eviden-ce, or long-term follow-up, as well as the heterogeneity of fistula pathology has prevented a definitive treatment algorithm. This article will review the most commonly available modalities and techniques for managing idio-pathic fistula-in-ano.

  12. Idiopathic aneurysm of pulmonary artery

    Energy Technology Data Exchange (ETDEWEB)

    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  13. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Alireza Rezaie

    2004-10-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  14. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Jun Wei

    2005-04-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  15. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Jun Wei

    2005-01-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  16. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang

    2005-10-01

    The University of Missouri-Rolla identified materials that permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project was to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study attempted to define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials were selected/developed that either react with the gasifier environment to form protective surfaces in

  17. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang

    2005-07-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  18. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Alireza Rezaie; Xiaoting Liang; Musa Karakus; Jun Wei

    2005-12-01

    The University of Missouri-Rolla identified materials that permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project was to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study attempted to define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials were selected or developed that reacted with the gasifier environment to form protective surfaces in

  19. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick; Musa Karakus; Alireza Rezaie

    2004-03-30

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  20. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Alireza Rezaie

    2003-08-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the materials problems encountered during the operation of low-pressure high-temperature (LFHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  1. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Alireza Rezaie

    2003-12-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the materials problems encountered during the operation of low-pressure high-temperature (LFHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  2. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Alireza Rezaie

    2004-07-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the material problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  3. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr.; Alireza Rezaie

    2004-04-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the materials problems encountered during the operation of low-pressure high-temperature (LPHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  4. Refractory for Black Liquor Gasifiers

    Energy Technology Data Exchange (ETDEWEB)

    Robert E. Moore; William L. Headrick; Alireza Rezaie

    2003-03-31

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the materials problems encountered during the operation of low-pressure high-temperature (LFHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  5. REFRACTORY FOR BLACK LIQUOR GASIFIERS

    Energy Technology Data Exchange (ETDEWEB)

    William L. Headrick Jr.; Alireza Rezaie

    2003-12-01

    The University of Missouri-Rolla will identify materials that will permit the safe, reliable and economical operation of combined cycle gasifiers by the pulp and paper industry. The primary emphasis of this project will be to resolve the materials problems encountered during the operation of low-pressure high-temperature (LFHT) and low-pressure low-temperature (LPLT) gasifiers while simultaneously understanding the materials barriers to the successful demonstration of high-pressure high-temperature (HPHT) black liquor gasifiers. This study will define the chemical, thermal and physical conditions in current and proposed gasifier designs and then modify existing materials and develop new materials to successfully meet the formidable material challenges. Resolving the material challenges of black liquor gasification combined cycle technology will provide energy, environmental, and economic benefits that include higher thermal efficiencies, up to three times greater electrical output per unit of fuel, and lower emissions. In the near term, adoption of this technology will allow the pulp and paper industry greater capital effectiveness and flexibility, as gasifiers are added to increase mill capacity. In the long term, combined-cycle gasification will lessen the industry's environmental impact while increasing its potential for energy production, allowing the production of all the mill's heat and power needs along with surplus electricity being returned to the grid. An added benefit will be the potential elimination of the possibility of smelt-water explosions, which constitute an important safety concern wherever conventional Tomlinson recovery boilers are operated. Developing cost-effective materials with improved performance in gasifier environments may be the best answer to the material challenges presented by black liquor gasification. Refractory materials may be selected/developed that either react with the gasifier environment to form protective

  6. Primordial Compositions of Refractory Inclusions

    Energy Technology Data Exchange (ETDEWEB)

    Grossman, L; Simon, S B; Rai, V K; Thiemens, M H; Hutcheon, I D; Williams, R W; Galy, A; Ding, T; Fedkin, A V; Clayton, R N; Mayeda, T K

    2008-02-20

    Bulk chemical and oxygen, magnesium and silicon isotopic compositions were measured for each of 17 Types A and B refractory inclusions from CV3 chondrites. After bulk chemical compositions were corrected for non-representative sampling in the laboratory, the Mg and Si isotopic compositions of each inclusion were used to calculate its original chemical composition assuming that the heavy-isotope enrichments of these elements are due to Rayleigh fractionation that accompanied their evaporation from CMAS liquids. The resulting pre-evaporation chemical compositions are consistent with those predicted by equilibrium thermodynamic calculations for high-temperature nebular condensates but only if different inclusions condensed from nebular regions that ranged in total pressure from 10{sup -6} to 10{sup -1} bar, regardless of whether they formed in a system of solar composition or in one enriched in OC dust relative to gas by a factor of ten relative to solar composition. This is similar to the range of total pressures predicted by dynamic models of the solar nebula for regions whose temperatures are in the range of silicate condensation temperatures. Alternatively, if departure from equilibrium condensation and/or non-representative sampling of condensates in the nebula occurred, the inferred range of total pressure could be smaller. Simple kinetic modeling of evaporation successfully reproduces observed chemical compositions of most inclusions from their inferred pre-evaporation compositions, suggesting that closed-system isotopic exchange processes did not have a significant effect on their isotopic compositions. Comparison of pre-evaporation compositions with observed ones indicates that 80% of the enrichment in refractory CaO + Al{sub 2}O{sub 3} relative to more volatile MgO + SiO{sub 2} is due to initial condensation and 20% due to subsequent evaporation for both Type A and Type B inclusions.

  7. Idiopathic Circumscripta Calcinosis Cutis of the Fingers

    Directory of Open Access Journals (Sweden)

    Ömer Çalka

    2013-05-01

    Full Text Available Calcinosis cutis is an uncommon disorder characterized by deposition of insoluble calcium salts in the skin. Based on the etiology of the deposition calcinosis cutis may be devided into four major groups namely metastatic, dystrophic, idiopathic, and iatrogenic. The pathophysiology of calcinosis cutis remains unclear. The dystrophic form is the most common whereas the idiopathic one is the rarest. Idiopathic calcinosis cutis occurs in the absence of any identifiable causes and is not associated with drug use. Idiopathic calcinosis cutis has two major types called localized (circumscript and generalized (universalis. Localized type generally involves the vulva, scrotum, penis and breast. A definitive diagnosis requires the histologic demonstration of the accumulation of calcium in the skin and exclusion of other clinic types.There is not an effective treatment for the disease. A 47-year-old woman referred to our outpatient clinic for painless, yellowish-white nodules on her hand enduring for 30 years. A diagnosis of idiopathic calcinosis cutis was made by clinical and histological findings. We present this case because localization of lesions in our case was previously unreported in the literature to our knowledge.

  8. Rituximab in Treating Patients Undergoing Donor Peripheral Blood Stem Cell Transplant for Relapsed or Refractory B-cell Lymphoma

    Science.gov (United States)

    2015-11-23

    B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  9. Vagus nerve stimulation for the treatment of refractory epilepsy

    Directory of Open Access Journals (Sweden)

    Gorgan M.R.

    2015-06-01

    Full Text Available Vagus nerve stimulation (VNS represents one of the main surgical options for the treatment of the refractory epilepsy in pediatric and adult patients. There are several mechanism involved in vagal nerve stimulation which could influence the pathophysiology of seizures like neuromodulation of the thalamic and subthalamic nuclei involved in seizure initiation and the modulation of the neurotransmitters pattern norepinefrin, GABA, and serotonin. The VNS system is composed of the implanted components (the generator, the lead with the electrodes attached and the programming system components (programming wand and handheld computer. The authors present their experience with 81 patients diagnosed with refractory epilepsy, investigated, selected and implanted with vagal neurostimulators between December 2012 and January 2015 in Neurosurgery Clinic, "Bagdasar-Arseni" Emergency Hospital. The surgical technique and the potential pitfalls are described in detail. There were 20 children (24,7% and 61 (75,3% adults in this series. There was no death in this series and no intraoperative incidence. One patient presented dysphagia postoperatively which completely remitted after two months of follow-up. The outcome in term of seizure frequency and severity was better for patients under 30 years compared with patients older than 30 years. VNS represents now a safe, quick and efficient surgical procedure with a minimum period of hospitalization and a short recovery period. The good results on long term improve the quality of life of the patients and facilitate the social and professional reinsertion

  10. Providence nighttime bracing, in treatment of adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Simony, A.; Beuschau, Inge; Quisth, Lena

    2015-01-01

    Introduction: Since 2008 the non-surgical treatment of adolescent idiopathic scoliosis (AIS) in the southern part of Denmark, went from full-time bracing with Boston brace, to Providence night-time bracing. Methods: Since 2008, skeletally immature patients diagnosed with AIS and a primary curve...... with apex at T7 or below, were treated with the Providence night-time brace. The patients were evaluated every 6 months, with standing x-rays. Compliance with the treatment was recorded during the study. Bracing was continued up to two years post menarche or, for males upon reaching the expected adult......, and the follow up was 12 months (8-37m). The SRS22r Function score was 4, 32, Mental Health 3, 81, SRS22r total 3, 9 and satisfaction with management 3, 90. The Pain domain was 3, 8. Conclusion: This study shows a good curve control with the Providence Night time brace and an acceptable 20 % curve progression...

  11. Refractories for Industrial Processing. Opportunities for Improved Energy Efficiency

    Energy Technology Data Exchange (ETDEWEB)

    Hemrick, James G. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Hayden, H. Wayne [Metals Manufacture Process and Controls Technology, Inc., Oak Ridge, TN (United States); Angelini, Peter [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Moore, Robert E. [R.E. Moore Associates, Maricopa, AZ (United States); Headrick, William L. [R.E. Moore Associates, Maricopa, AZ (United States)

    2005-01-01

    Refractories are a class of materials of critical importance to manufacturing industries with high-temperature unit processes. This study describes industrial refractory applications and identifies refractory performance barriers to energy efficiency for processing. The report provides recommendations for R&D pathways leading to improved refractories for energy-efficient manufacturing and processing.

  12. Refractory Septic Shock Treated with Nephrectomy under the Support of Extracorporeal Membrane Oxygenation

    Directory of Open Access Journals (Sweden)

    Young Kun Lee

    2015-08-01

    Full Text Available Conventional medical therapies have not been very successful in treating adults with refractory septic shock. The effects of direct hemoperfusion using polymyxin B and veno-arterial extracorporeal membrane oxygenation (ECMO for refractory septic shock remain uncertain. A 66-year-old man was admitted to the emergency department and suffered from sepsis-induced hemodynamic collapse. For hemodynamic improvement, we performed direct hemoperfusion using polymyxin B. Computed tomography scan of this patient revealed emphysematous pyelonephritis (EPN, for which he underwent emergent nephrectomy with veno-arterial ECMO support. To the best of our knowledge, this is the first report of successful treatment of EPN with refractory septic shock using polymyxin B hemoperfusion and nephrectomy under the support of ECMO.

  13. Idiopathic non-specific interstitial pneumonia.

    Science.gov (United States)

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.

  14. Clinical variants of idiopathic torsion dystonia.

    Science.gov (United States)

    Fahn, S

    1989-06-01

    Some patients with dystonic movements and postures not known to be caused by environmental or degenerative disorders can be segregated from classical-appearing idiopathic torsion dystonia on the basis of distinctive clinical and pharmacologic features. Many of them should be considered within the family of dystonia, as clinical variants of idiopathic torsion dystonia, while others are better classified as being part of other families of dyskinesias. In the former group are paradoxical dystonia, myoclonic dystonia, diurnal dystonia, and dopa-responsive dystonia. The latter group consists of dystonic tics and the various entities comprising paroxysmal dystonia, namely kinesigenic, nonkinesigenic and hypnogenic dystonia.

  15. An unusual case of idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Vikender S Yadav

    2013-01-01

    Full Text Available Idiopathic gingival fibromatosisis, a condition of undetermined cause can develop as an isolated disorder, but mostly it is associated with some syndrome. It usually begins at the time of eruption of permanent teeth but can develop with the eruption of deciduous dentition and rarely present at birth. This case report describes an unusual case of non-syndromic generalized idiopathic gingival fibromatosis in a 15-year-old male present since birth. Surgical treatment in the form of ledge and wedge procedure with internal bevel gingivectomy was performed. No recurrence of enlargement was seen after 2 years of follow-up.

  16. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    Science.gov (United States)

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

  17. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia and Neuroendocrine Hyperplasia of Infancy.

    Science.gov (United States)

    Carr, Laurie L; Kern, Jeffrey A; Deutsch, Gail H

    2016-09-01

    Although incidental reactive pulmonary neuroendocrine cell hyperplasia (PNECH) is seen on biopsy specimens in adults with chronic lung disease, disorders characterized by marked PNECH are rare. Primary hyperplasia of neuroendocrine cells in the lung and obstructive lung disease related to remodeling or physiologic constriction of small airways define diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) in the adult and neuroendocrine cell hyperplasia of infancy (NEHI) in children. DIPENCH and NEHI share a similar physiology, typical imaging appearance, and increased neuroendocrine cells on biopsy. However, there are important differences related to the underlying disease mechanisms leading to disparate outcomes.

  18. Alumina Carbon Refractory Products for Continuous Casting

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ 1 Scope This standard specifies the classification.techni-cal requirements,test methods,inspection rules,packing,marking,transportation,storage and quality certificate of alumina carbon refractory products for continuous casting.

  19. Ultra High Temperature Refractory Materials Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Legacy refractory materials that have origins dating to the original Saturn program are commonly used in current launch facilities. Although they failure to meet the...

  20. Ultra High Temperature Refractory Materials Project

    Data.gov (United States)

    National Aeronautics and Space Administration — Legacy refractory materials that have origins dating to the original Saturn program are commonly used in current launch facilities. Although they fail to meet the...

  1. Fireclay Refractory Bricks for Hot Blast Stove

    Institute of Scientific and Technical Information of China (English)

    Wang Jing; Zhang Yongfang; Chai Junlan

    2008-01-01

    @@ 1 Scope This standard specifies the classification, shape, dimension, technical requirements, test method,inspection rules, packing, marking,transportation,storage and quality certification of fireclay refractory bricks for hot blast stove.

  2. Spalling Resistance Fast-drying Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    Wang Jing; Peng Xigao

    2010-01-01

    @@ 1 Scope This standard specifies the term and definition,classification, technical requirements, test methods,quality appraisal procedures, packing, marking, transportation, storage, and quality certificate of spalling resistance fast-drying refractory castables.

  3. Situation of China's Refractories Industry in Last Sixty Years

    Institute of Scientific and Technical Information of China (English)

    HUO Jiangping; PAN Shangxin; LI Chaowen

    2009-01-01

    The annual output of China's refractories in 1949 was 74 thousand tons, and was 29.11 million tons in 2007, which was 65% of the world total refractories output. The export of China's refractory products and raw materials in 2008 was 6.18 million tons, which were exported to hundreds of countries and regions in the world. China is a big country in the field of refractories production and refractories export, which has devoted a lot to relative high temperature industry.

  4. The modified atkins diet in refractory epilepsy.

    Science.gov (United States)

    Sharma, Suvasini; Jain, Puneet

    2014-01-01

    The modified Atkins diet is a less restrictive variation of the ketogenic diet. This diet is started on an outpatient basis without a fast, allows unlimited protein and fat, and does not restrict calories or fluids. Recent studies have shown good efficacy and tolerability of this diet in refractory epilepsy. In this review, we discuss the use of the modified Atkins diet in refractory epilepsy.

  5. Neuromodulation in refractory epilepsy: Brazilian specialists consensus

    Directory of Open Access Journals (Sweden)

    Vera Cristina Terra

    Full Text Available ABSTRACT Epilepsy is a potentially devastating brain disorder characterized by a predisposition to spontaneous epileptic seizures. In patients with medically refractory epilepsy, new non-pharmacological therapeutic approaches may be considered. In this scenario, palliative surgery such as vagus nerve stimulation (VNS or deep brain stimulation (DBS may be indicated in a subset of patients. In this paper we make recommendations for the use of VNS and DBS in patients in Brazil with refractory epilepsy.

  6. The Modified Atkins Diet in Refractory Epilepsy

    Directory of Open Access Journals (Sweden)

    Suvasini Sharma

    2014-01-01

    Full Text Available The modified Atkins diet is a less restrictive variation of the ketogenic diet. This diet is started on an outpatient basis without a fast, allows unlimited protein and fat, and does not restrict calories or fluids. Recent studies have shown good efficacy and tolerability of this diet in refractory epilepsy. In this review, we discuss the use of the modified Atkins diet in refractory epilepsy.

  7. Alcohol percutaneous neurolysis of the sphenopalatine ganglion in the management of refractory cranio-facial pain

    Energy Technology Data Exchange (ETDEWEB)

    Kastler, Adrian [Grenoble University Hospital, Neuroradiology Department, Grenoble (France); Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); Cadel, Gilles; Gory, Guillaume [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); Comte, Alexandre [University Hospital Besancon, Functional Imaging Research Department, Besancon (France); Piccand, Veronique [University Hospital Jean Minjoz, Pain Evaluation and Treatment Unit, Besancon (France); Tavernier, Laurent [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); University Hospital Jean Minjoz, Head and Neck Surgery-Otolaryngology Unit, Besancon (France); Kastler, Bruno [Franche Comte University, I4S Laboratory, EA4268, IFR133, Besancon (France); University Hospital Jean Minjoz, Interventional Pain Management Unit, Besancon (France)

    2014-07-15

    The sphenopalatine ganglion (SPN) has been proven to be involved in various types of facial pain syndromes. Management of these cranio-facial pain syndromes can be challenging, and existing specific treatments are sometimes inefficient and may fail. The purpose of this study is to describe and evaluate alcohol SPN in the management of cranio-facial pain. Forty-two patients suffering from refractory facial pain who underwent 58 consecutive SPN were included in this study between 2000 and 2013. Patients were divided into three groups: group ''cluster headache'' (CH), group ''persistent idiopathic facial pain'' (PFIP), and group ''Other''. Pain was assessed using Visual Analogue Scale scores (measured immediately before and after procedure and at regular intervals following the procedure). Alcohol SPN was considered to be effective when pain relief was equal to or greater than 50 % and lasting for at least 1 month. All procedures were realized ambulatory under CT guidance and consisted of an injection of 1 ml of absolute alcohol. Overall efficacy rate of alcohol SPN was 67.2 %, with mean pain relief duration of 10.3 months. Procedure was graded either not painful or tolerable by patients in 64.2 %. Analysis showed a higher efficacy rate in the groups CH (76.5 %) and PFIP (85.7 %) compared to the group Other (40 %). No difference was found between groups regarding the recurrence rate. Alcohol SPN under CT guidance appears as a safe and effective treatment of refractory facial pain, especially in cases of cluster headache and persistent idiopathic facial pain. (orig.)

  8. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    Science.gov (United States)

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.

  9. Refractory migraine in a headache clinic population

    Directory of Open Access Journals (Sweden)

    Fernandez-Torron Roberto

    2011-08-01

    Full Text Available Abstract Background Many migraineurs who seek care in headache clinics are refractory to treatment, despite advances in headache therapies. Epidemiology is poorly characterized, because diagnostic criteria for refractory migraine were not available until recently. We aimed to determine the frequency of refractory migraine in patients attended in the Headache Unit in a tertiary care center, according to recently proposed criteria. Methods The study population consisted of a consecutive sample of 370 patients (60.8% females with a mean age of 43 years (range 14-86 evaluated for the first time in our headache unit over a one-year period (between October 2008 and October 2009. We recorded information on clinical features, previous treatments, Migraine Disability Assessment Score (MIDAS, and final diagnosis. Results Overall migraine and tension-type headache were found in 46.4% and 20.5% of patients, respectively. Refractory migraine was found in 5.1% of patients. In refractory migraineurs, the mean MIDAS score was 96, and 36.8% were medication-overusers. Conclusions Refractory migraine is a relatively common and very disabling condition between the patients attended in a headache unit. The proposed operational criteria may be useful in identifying those patients who require care in headache units, the selection of candidates for combinations of prophylactic drugs or invasive treatments such as neurostimulation, but also to facilitate clinical studies in this patient group.

  10. Advances in Modern Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    ZHOU Ningsheng; HU Shuhe; ZHANG Sanhua

    2004-01-01

    Recent developments in terms of binding system, new material system, installation method and pre-cast shapes of refractory castables are introduced and discussed. In authors view, binding system is advancing on the one hand towards "purification" to minimize impurity components from binder materials, and on the other hand "stabilization" to minimize the structure destroy by volatility and decomposition of binder materials. Challenges of higher service temperature, slag resistance, clean steel production and better thermal shock resistance have promoted the booming of new varieties of castables including MgObased, natural flake graphite containing and non-oxides like Si3 N4, AlON and SiAlON containing castables. Installation technique is developing towards convenience,simplification and high efficiency. Advancement in installation and dry-out is of an incentive to the boost of selfflow castables, shotcreting castables, infiltration casting,quick dry-out castables and pre-cast shapes. Along with the increased adoption of pre-cast shapes of castables, the boundary between "shaped" and "unshaped" is becoming vague.

  11. Advances in Modern Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    ZHOUNingsheng; HUShuhe; ZHANGSanhua

    2004-01-01

    Recent developments in terms of binding system, new material system, installation method and pre-cast shapes of refractory castables are introduced and discussed. In authors' view, binding system is advancing on the one hand towards "purification" to minimize impurity components from binder materials, and on the other hand "stabilization" to minimize the structure destroy by volatility and decomposition of binder materials. Challenges of higher service temperature, slag resistance, clean steel production and better thermal shock resistance have promoted the booming of new varieties of castables including MgO based, natural flake graphite containing and non-oxides like Sis N4, AION and SIMON containing castables. Installation technique is developing towards convenience, simplification and high efficiency. Advancement in installation and dry-out is of an incentive to the boost of selfflow castables, shotcreting castables, infiltration casting, quick dry-out castables and pre-cast shapes. Along wit hthe increased adoption of pre-cast shapes of castables, the boundary between "shaped" and " unshaped " is becoming vague.

  12. Minimally invasive scoliosis surgery: an innovative technique in patients with adolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Gambassi Melanie

    2011-08-01

    Full Text Available Abstract Minimally invasive spine surgery is becoming more common in the treatment of adult lumbar degenerative disorders. Minimally invasive techniques have been utilized for multilevel pathology, including adult lumbar degenerative scoliosis. The next logical step is to apply minimally invasive surgical techniques to the treatment of adolescent idiopathic scoliosis (AIS. However, there are significant technical challenges of performing minimally invasive surgery on this patient population. For more than two years, we have been utilizing minimally invasive spine surgery techniques in patients with adolescent idiopathic scoliosis. We have developed the present technique to allow for utilization of all standard reduction maneuvers through three small midline skin incisions. Our technique allows easy passage of contoured rods, placement of pedicle screws without image guidance, and allows adequate facet osteotomy to enable fusion. There are multiple potential advantages of this technique, including: less blood loss, shorter hospital stay, earlier mobilization, and relatively less pain and need for pain medication. The operative time needed to complete this surgery is longer. We feel that a minimally invasive approach, although technically challenging, is a feasible option in patients with adolescent idiopathic scoliosis. Although there are multiple perceived benefits, long term data is needed before it can be recommended for routine use.

  13. Idiopathic Low-Flow Priapism in Prepuberty: A Case Report and a Review of Literature

    Directory of Open Access Journals (Sweden)

    Ihab A. Hekal

    2008-01-01

    Full Text Available Introduction. The incidence of priapism in adults is higher than in children. Although approximately 50% of all episodes of priapism are thought to be idiopathic, there are a number of known specific causes of this disorder. In adults intracavernous therapy with papaverine, phentolamine, alprostadil or combinations of these agents is the most common cause of ischemic priapism. In children the most common etiology is sickle cell anemia for low-flow priapism or post-traumatic high-flow priapism. We present a 13-year-old boy, not sexually active presented to our outpatient clinic suffering from long standing (3.5 hours sustained painful erection. To the best of our knowledge the idiopathic low-flow priapism in pre-pubertal boy was not reported before in literature. Our case is the first case to be reported in pre-pubertal age. Conclusion. In pre-pubertal boys idiopathic recurrent priapism is a rare condition. In the literature, several empirical therapies are described. Recently, it is postulated that a low dose of a PDE5 inhibitor. The early conservative management is the best treatment option to safe the corporeal smooth muscles from irreversible damage.

  14. Decitabine and Valproic Acid in Treating Patients With Refractory or Relapsed Acute Myeloid Leukemia or Previously Treated Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma

    Science.gov (United States)

    2013-09-27

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Recurrent Adult Acute Myeloid Leukemia; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Untreated Adult Acute Myeloid Leukemia

  15. Anti CD20 (Rituximab therapy in refractory pediatric rheumatic diseases

    Directory of Open Access Journals (Sweden)

    Joel Reis

    2016-01-01

    Full Text Available Objectives: We aim to report the efficacy and safety of rituximab (RTX in patients diagnosed with juvenile systemic lupus erythematosus (JSLE or juvenile idiopathic arthritis (JIA refractory to conventional treatment. Methods: A retrospective review was made of all medical records of patients with JSLE or JIA treated with RTX between January 2009 and January 2015 in the Pediatric Rheumatology Unit of a central hospital. Results: Five patients, 4 with JSLE and 1 with extended oligoarticular JIA, received 10 cycles of RTX (23 infusions. The scheme of RTX frequently used was 750 mg/m2 two weeks apart. The median follow-up time after receiving the first cycle of RTX was 24 months (12 – 70. The four patients with JSLE were female (three caucasian and one black. The patient with JIA was a caucasian male. The median age at diagnosis was 10 years (16 months – 17years. The median evolution time until receiving RTX was 6 years (5 months – 15 years. Refractory class IV lupus nephritis was the most common indication for receiving RTX. Previous treatment to RTX included nonsteroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, immunosuppressive drugs and corticosteroids in all patients and anti-TNFα (etanercept in the patient with JIA. It was possible to reduce the mean oral corticosteroid dose after RTX, ranging from 23 mg/day (20-25mg/day before RTX to 11 mg/day (0–20 mg/day at the last evaluation. Disease activity before RTX and at last evaluation also improved. The SLEDAI score, for JSLE, decreased from a median of 15, 5 (11 – 18 to 3 (0 – 6, and the JADAS-27 score, for JIA, also diminished from 40.4 to 3.5. Adverse events occurred in 2 patients, including delayed second dose after the diagnosis of cryptococcosis and respiratory tract infection with concomitant hypogammaglobulinemia needing of immunoglobulin replacement and antibiotic therapy. Conclusions: Rituximab might have a role in the treatment of JSLE and JIA

  16. Incidence of herpes zoster infections in juvenile idiopathic arthritis patients.

    Science.gov (United States)

    Nimmrich, S; Horneff, G

    2015-03-01

    The risk of herpes zoster among patients with juvenile idiopathic arthritis (JIA) exposed to biologics has not been evaluated. We determined incidence rates of herpes zoster among children with JIA in correlation with medication at time of occurrence and total drug exposure. The German biologics register database was used to identify patients with herpes zoster. Crude infection rates and incidence ratios (IRR) were compared to published rates. Demographics and overall exposure and particular exposure time to corticosteroids, immunosuppressive drugs and biologics were analyzed. The JIA cohort included 3,042 patients with 5,557.9 person-years of follow-up; 1,628 have used corticosteroids, 2,930 methotrexate and 1,685 etanercept. In total, 17 herpes zoster events have been documented [6/1,000 patients (3.5-9.0); 3.1/1,000 patient-years (1.9-4.9)]. Thus, the incidence rate in JIA patients was higher than expected [IRR 2.9 (1.8-4.5), p herpes zoster. Compared to the healthy population, a significant higher IRR is observed in JIA patients who received a monotherapy with etanercept or in combination with steroids and methotrexate, but not in JIA patients exposed to methotrexate without biologics. In comparison with our control group of patients treated with methotrexate, the IRR was higher for exposure to etanercept monotherapy and combination of etanercept and corticosteroids irrespective of methotrexate use. A generally higher incidence rate in JIA patients treated with etanercept was observed. No serious or refractory manifestations occurred.

  17. Polyarticular juvenile idiopathic arthritis – epidemiology and management approaches

    Directory of Open Access Journals (Sweden)

    Oberle EJ

    2014-10-01

    Full Text Available Edward J Oberle, Julia G Harris, James W VerbskyDepartment of Pediatrics, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI, USAAbstract: Juvenile idiopathic arthritis (JIA is a group of disorders characterized by arthritis persisting for at least 6 weeks with onset before the age of 16 years. Within this cluster of conditions, the polyarticular form (involving more than four joints within the first 6 months is further divided based on the presence of rheumatoid factor. Children with polyarticular JIA pose unique diagnostic and therapeutic challenges compared to children with involvement of fewer joints. Polyarticular JIA patients tend to have a more refractory course and therefore are at increased risk for joint damage, resulting in poorer functional outcomes and decreased quality of life. Although the ability to treat this disorder continues to improve, especially with the advent of biologic agents, there is still much about the epidemiology and pathogenesis of polyarticular JIA that is unknown. The epidemiology of polyarticular JIA varies worldwide with a vast difference in reported cases between different global regions as well as within individual countries. Several genetic risk loci have been identified conferring increased susceptibility to JIA, many within the human leukocyte antigen region. Beyond the genome, environmental factors also seem to contribute to the etiology of polyarticular JIA. This review article will focus on the epidemiology and current treatments of polyarticular JIA and briefly discuss genetic and environmental influences on the pathogenesis of JIA as well as new and emerging therapies.Keywords: juvenile arthritis, polyarticular, epidemiology, treatment, rheumatology

  18. Rituximab Therapy for Severe Cutaneous Leukocytoclastic Angiitis Refractory to Corticosteroids, Cellcept and Cyclophosphamide

    Directory of Open Access Journals (Sweden)

    Kamel El-Reshaid

    2013-04-01

    Full Text Available We report our clinical experience with rituximab in the treatment of 2 patients with idiopathic cutaneous angiitis who relapsed after treatment with high-dose corticosteroids and cyclophosphamide. A 39-year-old woman and a 51-year-old man presented with ulcerating maculopapular rash in both lower limbs which relapsed 6 months after treatment with a combination of high-dose corticosteroids and cyclophosphamide. After treatment with 2 g of rituximab, the first patient has still been in clinical remission for 32 months while the second has finished 28 months. Interestingly, CD19 which had dropped to 0.5% 8 months later in both patients. Despite that, our patients are still in clinical remission. No significant side effects were noted during infusions and up to the period of follow-up. In conclusion, rituximab is a useful and safe agent in the treatment of idiopathic cutaneous angiitis refractory to conventional therapy. Clinical remission persists years after improvement of B-cell suppression.

  19. Idiopathic Spinal Epidural Abscess: A Case Report

    Directory of Open Access Journals (Sweden)

    Chaitali Biswas

    2011-11-01

    Full Text Available Epidural abscess is a potentially life-threatening disease which can lead to medical-surgical emergency. Idiopathic spinal epidural abscess (SEA with atypical manifestations is extremely rare. We describe such a case which led to severe neurological compromise and was not associated with any known risk factors.

  20. Idiopathic Spinal Epidural Abscess: A Case Report

    OpenAIRE

    Chaitali Biswas; Anirban Pal; Saswata Bharati; Nitesh Sinha

    2011-01-01

    Epidural abscess is a potentially life-threatening disease which can lead to medical-surgical emergency. Idiopathic spinal epidural abscess (SEA) with atypical manifestations is extremely rare. We describe such a case which led to severe neurological compromise and was not associated with any known risk factors.

  1. Vitamin A and Idiopathic Intracranial Hypertension

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-11-01

    Full Text Available Vitamin A levels were measured in the cerebrospinal fluid of a total of 78 patients having idiopathic intracranial hypertension (IIH;n=20, elevated pressure of other causes (E-ICP;n=19, and normal pressure (N-ICP;n=39, in a study at the University of Utah, Salt Lake City, UT.

  2. Sialosis, Gout Induced or Idiopathic? Case Report.

    Science.gov (United States)

    Naik, Keyur; Mandel, Louis

    2017-02-01

    Sialosis is observed in relation to diabetes, alcoholism, and malnutrition. An assumed relation between gout and sialosis is probably based on confusion that originated from the therapeutic use of phenylbutazone for gout and the sialadenitis that the medication caused. This report describes a case of sialosis in a patient with a longstanding history of gout that was idiopathic in origin.

  3. GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

    Directory of Open Access Journals (Sweden)

    A. A. Chernova

    2012-01-01

    Full Text Available Published data demonstrating genetic determination of sick sinus syndrome is presented. The definition of this pathology is presented; the main symptoms are described, as well as genes that influence the development of idiopathic sick sinus syndrome, their polymorphisms and role in disorders of the cardiovascular system.

  4. The human microbiome and juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Verwoerd, Anouk; ter Haar, Nienke M.; de Roock, Sytze; Vastert, Sebastiaan J.; Bogaert, Debby

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The

  5. Pirfenidone treatment in idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Salih, Goran Nadir; Shaker, Saher Burhan; Madsen, Helle Dall;

    2016-01-01

    BACKGROUND: Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect...

  6. Idiopathic hepatic arterial malformation: a case report

    Institute of Scientific and Technical Information of China (English)

    郑蔚巍; 周康荣; 王佩芬; 陈祖望

    2003-01-01

    @@ Hepatic arterial malformation is a rare disorder which either origi nates idiopathically or may be associated with hereditary hemorrhagic telangiect asia (also known as Osler-Weber-Rendu disease). Although previous reports presented only descriptions of sonographic and angiographic findings,1-6 we present a case of splenic infarct caused by this disorder with CT and CTA findi ngs.

  7. Neocortical gamma oscillations in idiopathic generalized epilepsy

    DEFF Research Database (Denmark)

    Benedek, Krisztina; Berenyi, Antal; Gombkoto, Peter

    2016-01-01

    Objective: Absence seizures in patients with idiopathic generalized epilepsy (IGE) may in part be explained by a decrease in phasic GABAA (type-A c-aminobutyric acid) receptor function, but the mechanisms are only partly understood. Here we studied the relation between ictal and interictal spike-...

  8. Idiopathic erythrocytosis in IgA nephropathy

    Science.gov (United States)

    Mahesh, E.; Madhyastha, P. R.; Kalashetty, M.; Gurudev, K. C.; Bande, S.; John, M. M.

    2017-01-01

    We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

  9. [Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification].

    Science.gov (United States)

    Barrera, Ana Madeleine; Vargas, Leslie

    2016-12-01

    Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged.

  10. Acute surgical management in idiopathic intracranial hypertension.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Idiopathic intracranial hypertension is a headache syndrome with progressive symptoms of raised intracranial pressure. Most commonly, it is a slow process where surveillance and medical management are the main treatment modalities. We describe herein an acute presentation with bilateral sixth nerve palsies, papilloedema and visual deterioration, where acute surgical intervention was a vision-saving operation.

  11. Etiology and pathogenesis of adolescent idiopathic scoliosis

    NARCIS (Netherlands)

    Schlösser, Tom P C; Colo, Dino; Castelein, RM

    2015-01-01

    Despite many years of dedicated research into the etio-pathogenesis, not one single cause for adolescent idiopathic scoliosis has been identified. The purpose of this review is to give a comprehensive overview of the current evidence and main etiological theories. Intrinsic causal mechanisms are fou

  12. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currentl...

  13. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    OpenAIRE

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis.

  14. Selective fusion in adolescent idiopathic scoliosis

    Institute of Scientific and Technical Information of China (English)

    WANG Ting; XU Jian-guang; ZENG Bing-fang

    2008-01-01

    @@ Despite the continual evolution in the surgical treatment of adolescent idiopathic scoliosis (AIS), the goals of surgery remain to correct and stabilize the deformity in three dimensions, to maintain equilibrium of the shoulders and trunk, and to leave as many mobile spinal segments as possible.

  15. Idiopathic trigeminal neuropathy in a poodle

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Aparicio

    2010-12-01

    Full Text Available A seven years old, male poodle is examined presenting acute mandible paralysis (dropped jaw, drooling and difficulty for the apprehension and chewing; not evidence of an other alteration of cranial nerves. The muscular biopsy rules out a myositisof masticatory muscles. The disorder is resolved completely in 3 weeks confirming diagnosis of idiopathic trigeminal neuropathy.

  16. Capsaicin in Idiopathic Rhinitis A Hot Topic

    NARCIS (Netherlands)

    J.B. van Rijswijk

    2005-01-01

    textabstractIn chapter I the currently known causes for nonallergic noninfectious rhinitis and possible treatments are summarised. Also possible pathophysiological mechanisms underlying idiopathic rhinitis (IR) are discussed. In chapter II the aims of the studies are presented. This thesis comprises

  17. The lived experience with idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer

    2016-01-01

    The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family...

  18. Optimal management of idiopathic scoliosis in adolescence

    Directory of Open Access Journals (Sweden)

    Kotwicki T

    2013-07-01

    Full Text Available Tomasz Kotwicki,1 Joanna Chowanska,1,2 Edyta Kinel,3 Dariusz Czaprowski,4,5 Marek Tomaszewski,1 Piotr Janusz1 1Department of Pediatric Orthopedics, University of Medical Sciences, Poznan, Poland; 2National Scoliosis Foundation, Stoughton, MA, USA; 3Department of Rehabilitation, University of Medical Sciences, Poznan Poland; 4Department of Physiotherapy, Józef Rusiecki University College, Olsztyn, 5Rehasport Clinic, Poznan, Poland Abstract: Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid

  19. Management of idiopathic gingival fibromatosis: report of a case and literature review.

    Science.gov (United States)

    Ramnarayan, B K; Sowmya, Krishna; Rema, J

    2011-01-01

    Gingival hyperplasia is a rare condition and is of importance for cosmetic and mechanical reasons. Idiopathic gingival fibromatosis, a benign, slow-growing proliferation of the gingival tissues, is genetically heterogeneous. The enlargement is most intense during the eruption of the primary and permanent teeth, and minimal or nondetectable growth is observed in adults. The genetic aspect, clinical feature, histopathology, immunohistochemistry, and treatment aspects are reviewed. The purpose of this paper was to report a case of idiopathic gingival fibromatosis in a 13-year-old female who had a negative family history for a similar type of gingival enlargement. The diagnosis was established through history, clinical examination, and histopathology using both hematoxylin and eosin and Van Giesen stain (a special stain for collagen). Surgical treatment, which included both gingivectomy and gingivoplasty, was carried out. The case showed remarkable esthetic and functional improvement. The patient returned after a year and showed no recurrence.

  20. Minoxidil-associated anorexia in an infant with refractory hypertension.

    Science.gov (United States)

    Vesoulis, Zachary A; Attarian, Stephanie J; Zeller, Brandy; Cole, Francis Sessions

    2014-12-01

    Minoxidil is a potent antihypertensive used as an adjunctive agent in refractory hypertension. It exerts an antihypertensive effect through two mechanisms: selective arterial vasodilation by activation of potassium channels in the vascular smooth muscle and stimulation of carotid and aortic baroreceptors, leading to downstream release of renin and norepinephrine. Although frequently cited in reviews of antihypertensive agents, limited data about the use of minoxidil in neonates are available. We describe an infant girl, born at 35 weeks of gestation, who was diagnosed with idiopathic hypertension after extensive diagnostic evaluation. Adequate blood pressure control was not achieved with captopril, amlodipine, and clonidine. Oliguria secondary to captopril and rapid-onset congestive heart failure due to persistent hypertension led to the introduction of intravenous agents labetalol and nitroprusside. Although adequate blood pressure control was achieved, attempts to transition back to oral agents were unsuccessful, prompting the use of minoxidil as an alternative agent. Although good blood pressure control was achieved, the infant's oral intake plummeted from 210 to 63 ml/kg/day. The anorexia quickly resolved after stopping minoxidil, and she was discharged home at 5 months of age receiving propranolol, amlodipine, and doxazosin. Use of the Naranjo adverse drug reaction probability scale indicated a definite relationship (score of 10) between the patient's development of anorexia and minoxidil therapy. To our knowledge, there have been no previous reports of minoxidil-associated anorexia in preterm or term infants. Clinicians should be aware that anorexia is a possible adverse effect of minoxidil in this patient population when initiating the drug in similar patients.

  1. Effectiveness of intratympanic dexamethasone for refractory sudden sensorineural hearing loss.

    Science.gov (United States)

    Erdur, Omer; Kayhan, Fatma Tulin; Cirik, Ahmet Adnan

    2014-06-01

    The purpose of this study was to investigate the effectiveness of intratympanic steroids in patients with idiopathic sudden sensorineural hearing loss who did not respond to initial systemic steroid therapy. This retrospective study involved 51 patients, who did not respond to systemic steroids as a first-line treatment. Initial systemic steroid therapy consisted of administration of methylprednisolon intravenously (250 mg) at the first day and followed by orally (1 mg/kg) tapering for 14 days. Twenty-one patients accepted intratympanic treatment, and the remaining 30 patients who refused intratympanic treatment were evaluated as the control group. Steroids (dexamethasone drops, 1 mg/mL) were administered through a ventilation tube. Hearing was assessed immediately before treatment and 2 months after treatment. Recovery of hearing was defined as an improvement of >20 dB in the pure tone average. We tested 250, 500, 1,000, 2,000, 4,000, and 8,000 Hz frequencies for the pure tone audiometric evaluation. Statistically Student's t test, Mann-Whitney U test, Chi-squared and Fisher's exact tests were used. The pure tone average improved in 47.6% of the intratympanic group and in 10% of the control group (p = 0.002), with pure tone average improvements of 19.9 ± 16.5 and 4.76 ± 9.6 dB in the intratympanic and control groups, respectively. When the hearing threshold at each frequency was analyzed, improvements at all frequencies were significantly greater in the intratympanic steroid group when compared with the control group (p sudden sensorineural hearing loss in patients, who are refractory to primary systemic steroid therapy.

  2. Intratympanic steroid treatment for idiopathic sudden sensorineural hearing loss after failure of intravenous therapy.

    Science.gov (United States)

    Ferri, Emanuele; Frisina, Antonio; Fasson, Anna Chiara; Armato, Enrico; Spinato, Giacomo; Amadori, Maurizio

    2012-01-01

    Purpose. The aim of this study is the investigation of the effectiveness of intratympanic steroids therapy (IST) in patients with idiopathic sudden sensorineural hearing loss (ISSHL) who had not responded to intravenous treatment, evaluating the overall hearing recovery and comparing the results with different variables. Materials and Methods. Our study consisted of 55 patients with refractory ISSHL who, at the end of 10 days of therapy with intravenous steroids, had puretone 4-frequency average (PTA) of worse than 30 dB. The patients received 0.5 mL of methylprednisolone by direct intratympanic injection. The procedure was carried out up to 7 times within a 20-days period. Statistical analysis was carried out. Results. Overall 29 patients (52.7%) showed improvement in PTA, 24 (43.8%) had no change in hearing, and 2 (3.5%) worsened. There was a significant statistical correlation between hearing recovery and time to onset of symptoms, severity of hearing loss and frequency of hearing loss. Conclusions. IST is an effective and safe therapy in sudden sensorineural hearing loss cases that are refractory to standard treatment. The earlier IST, the hearing losses less than 90 dB and the involvement of the low frequencies seem to influence positively the hearing recovery.

  3. 两种矫形方法治疗成人特发性脊柱侧凸的疗效比较%Comparison of the clinical outcome for adult idiopathic scoliosis treated by posterior spinal instrumentation with or without Halo-femoral traction after anterior release

    Institute of Scientific and Technical Information of China (English)

    张兴; 邱勇; 朱锋; 朱泽章; 钱邦平; 刘臻; 郭倞; 吕峰

    2012-01-01

    目的:比较单纯后路矫形术和一期前路松解、Halo-股骨髁上牵引加二期后路矫形术治疗成人特发性脊柱侧凸的疗效.方法:选取我院脊柱外科2003年1月~2007年12月收治的有完整影像学资料、Cobb角65°~90°的成人特发性脊柱侧凸患者30例,年龄20~30岁,平均23.4岁.均为初次手术,术前无神经损害.根据不同手术方法分为两组,行单纯后路矫形术的14例患者为A组,行一期前路松解、Halo-股骨髁上牵引及二期后路矫形术的16例患者为B组.两组患者术前侧凸Cobb角、胸椎后凸角、年龄、性别比、侧凸类型相匹配.随访时间为12~72个月,平均40个月.比较两组患者手术时间、出血量、住院时间、并发症情况、侧凸矫正率和冠状面平衡情况.结果:平均手术时间和平均住院时间A组分别为6.7±1.2h和24±18d,B组分别为9.9±1.4h和41±10d,B组均显著长于A组(P<0.05).所有病例术后均无瘫痪、呼吸衰竭、死亡等并发症发生.术后侧凸矫正率A组为(51.3±11.8)%,B组为(64.5±11.6)%,B组显著大于A组(P<0.05);胸椎后凸角、C7中垂线与骶骨中线的距离A组为20.6°±8.4°、1.32±0.65cm,B组为20.4°±6.7°、1.30±0.70cm,两组比较均无显著性差异(P>0.05).末次随访时A组侧凸矫正丢失率为(3.5±2.4)%,B组为(2.8±1.5)%,两组无显著性差异(P>0.05).结论:两种治疗方案治疗中度成人特发性脊柱侧凸均可获得较好的畸形矫正,一期前路松解、Halo-双侧股骨髁上牵引可以增加侧凸Cobb角矫正率,但是存在显著增加手术时间和住院时间等不足.%Objectives: To compare the clinical outcome of posterior spinal instrumentation with or without Halo-femoral traction after anterior spinal release in the treatment of adult idiopathic scoliosis. Methods: 30 adult idiopathic scoliosis patients with Cobb angle range from 65° -90° treated from January 2003 to December 2007 were recruited into

  4. Clobazam therapy of refractory epilepsy in tuberous sclerosis complex.

    Science.gov (United States)

    Jennesson, Melanie; van Eeghen, Agnies M; Caruso, Paul A; Paolini, Jan L; Thiele, Elizabeth A

    2013-05-01

    Clobazam (CLB) was recently approved by the FDA, but has not been evaluated in tuberous sclerosis complex (TSC). We retrospectively reviewed a cohort of patients with TSC and refractory epilepsy who started CLB over a 5-year period. Clinical characteristics and number of tubers on MRI were assessed. Duration of therapy, therapeutic response and adverse events were recorded. CLB was prescribed in 29 adults and children of whom 72% were cognitively impaired, with a median age at seizure onset of 5 months. Mean duration of CLB therapy was 17.3 months with a 12 and 24-month estimated retention rate of 82% and 68%, respectively. Twenty patients (69%) reported a good response (>50% seizure reduction) at the end of the titration, and six patients (21%) remained good responders after 12 months of CLB therapy. Adverse events occurred in 13 patients, predominantly somnolence and behavioral disorders. One quarter of the responders reported improvement in behavior. No predictive factor for a good response could be identified. CLB appears to be a well-tolerated and valuable option for treatment of refractory epilepsy in TSC.

  5. Analysis on the Causes for Refractory GERD

    Institute of Scientific and Technical Information of China (English)

    陈婕; 许军英; 徐勇; 谢小平; 易粹琼; 侯晓华

    2002-01-01

    To analyze the causes of failure in conventional treatment to refractory gastroesophageal reflux diseases (GERD) patients, 16 refractory GERD patients (group R) and 16 eases of GERD primarily diagnosed (group P) were studied. Endoscopy, pathologic examination and 14C urea breath test were conducted in every patient. 24 h ambulatory pH and bilirubin monitoring were performed with Digitrapper MI Ⅲ and Synetics Bilitec 2000. It was found that esophagitis in group R was more severe than in group P. The rate of Helicobacter pylori infection in group R was significantly lower than in group P. Fraction time pH below 4. 00 was not longer while the bile reflux represented by fraction time abs above 0. 14 was greater for patients in the group R as compared with those in the group P. The mixed refluxes and pure bile refluxes between the two groups had significant difference. The reflux episodes in the group R mainly occurred during nights. These results indicated that severe esophagitis, especially Barrett's esophagus with complications makes it difficult to control GERD. Severe duodenogastroesophageal refluxes (DGER) are often accompanied by refractory GERD. Mixed refluxes aggravate the esophageal injuries. Pure bile refluxes and nocturnal refluxes may cause failure of administration of proton pump inhibitors (PPI) in the morning. Helicobacter pylori infection and acid refluxes may not be the direct cause of refractoriness. Individual refractory GERD patient without abnormal results on pH or bile reflux recently should be diagnosed again.

  6. Identification of platelet refractoriness in oncohematologic patients

    Directory of Open Access Journals (Sweden)

    Aline Aparecida Ferreira

    2011-01-01

    Full Text Available OBJECTIVES: To identify the occurrence and the causes of platelet refractoriness in oncohematologic patients. INTRODUCTION: Platelet refractoriness (unsatisfactory post-transfusion platelet increment is a severe problem that impairs the treatment of oncohematologic patients and is not routinely investigated in most Brazilian services. METHODS: Forty-four episodes of platelet concentrate transfusion were evaluated in 16 patients according to the following parameters: corrected count increment, clinical conditions and detection of anti-platelet antibodies by the platelet immunofluorescence test (PIFT and panel reactive antibodies against human leukocyte antigen class I (PRA-HLA. RESULTS: Of the 16 patients evaluated (median age: 53 years, nine (56% were women, seven of them with a history of pregnancy. An unsatisfactory increment was observed in 43% of the transfusion events, being more frequent in transfusions of random platelet concentrates (54%. Platelet refractoriness was confirmed in three patients (19%, who presented immunologic and non-immunologic causes. Alloantibodies were identified in eight patients (50% by the PIFT and in three (19% by the PRA-HLA. Among alloimmunized patients, nine (64% had a history of transfusion, and three as a result of pregnancy (43%. Of the former, two were refractory (29%. No significant differences were observed, probably as a result of the small sample size. CONCLUSION: The high rate of unsatisfactory platelet increment, refractoriness and alloimmunization observed support the need to set up protocols for the investigation of this complication in all chronically transfused patients, a fundamental requirement for the guarantee of adequate management.

  7. An operational definition of primary refractory acute myeloid leukemia allowing early identification of patients who may benefit from allogeneic stem cell transplantation

    DEFF Research Database (Denmark)

    Ferguson, Paul; Hills, Robert K; Grech, Angela

    2016-01-01

    Up to 30% of adults with acute myeloid leukemia fail to achieve a complete remission after induction chemotherapy - termed primary refractory acute myeloid leukemia. There is no universally agreed definition of primary refractory disease, nor have the optimal treatment modalities been defined. We.......0001) cohorts. The utilization of REF1 criteria permits the early identification of patients whose outcome after one course of induction chemotherapy is very poor, and informs a novel definition of primary refractory acute myeloid leukemia. Furthermore, these data demonstrate that allogeneic stem cell...

  8. Optimal management of idiopathic scoliosis in adolescence.

    Science.gov (United States)

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%-3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

  9. Idiopathic Short Stature: Conundrums of Definition and Treatment

    Directory of Open Access Journals (Sweden)

    Arlan L. Rosenbloom

    2009-01-01

    Full Text Available Children with idiopathic short stature (ISS are statistically defined by height SDS <−2 for their bone age and should be distinguished from children with familial short stature for whom height SDS corresponds to mean parental SDS and from the most common explanation for short stature referred to pediatric endocrinologists, constitutional delay in growth and maturation (CDGM, in which there is normal height for bone age and predicted normal adult stature. Low IGF-I levels reported in ISS may be the result of subtle undernutrition or reference to standards appropriate for chronologic age but not osseous maturation in CDGM inappropriately labeled as ISS. While growth hormone (GH treatment of ISS may add 4-5 cm to adult height, meta-analysis indicates that there is no documented evidence that such treatment improves health related quality of life or psychological adaptation. Thus, the estimated cost of US$52 000/inch gained is difficult to justify. Absence of data regarding efficacy of the use of IGF-I for treatment of ISS has been noted in a recent consensus statement from the North American and European pediatric endocrinology societies. This report further emphasizes the importance of discouraging the expectation that taller stature from GH treatment will improve quality of life.

  10. Anatomical Consideration in Catheter Ablation of Idiopathic Ventricular Arrhythmias.

    Science.gov (United States)

    Yamada, Takumi; Kay, G Neal

    2016-01-01

    Idiopathic ventricular arrhythmias (VAs) are ventricular tachycardias (VTs) or premature ventricular contractions (PVCs) with a mechanism that is not related to myocardial scar. The sites of successful catheter ablation of idiopathic VA origins have been progressively elucidated and include both the endocardium and, less commonly, the epicardium. Idiopathic VAs usually originate from specific anatomical structures such as the ventricular outflow tracts, aortic root, atrioventricular (AV) annuli, papillary muscles, Purkinje network and so on, and exhibit characteristic electrocardiograms based on their anatomical background. Catheter ablation of idiopathic VAs is usually safe and highly successful, but can sometimes be challenging because of the anatomical obstacles such as the coronary arteries, epicardial fat pads, intramural and epicardial origins, AV conduction system and so on. Therefore, understanding the relevant anatomy is important to achieve a safe and successful catheter ablation of idiopathic VAs. This review describes the anatomical consideration in the catheter ablation of idiopathic VAs.

  11. Topical cidofovir for refractory verrucae in children.

    Science.gov (United States)

    Gupta, Monique; Bayliss, Susan J; Berk, David R

    2013-01-01

    Warts are common and are a challenge to treat in some children, especially immunocompromised children and those who fail or cannot tolerate salicylic acid preparations and cryotherapy. Cidofovir, a nucleotide analogue with antiviral activity, has demonstrated promising results when compounded into a topical form to treat refractory warts. We present a retrospective institutional review of 12 children with refractory verrucae treated with 1% to 3% topical cidofovir compounded in an unscented moisturizing cream, applied every other day to daily. In our institutional series, only three patients (25%) demonstrated complete clearance of their verrucae. An additional four patients (33%) demonstrated partial clearance. Our experience using topical cidofovir has been less successful than previous institutional reviews, possibly because we used a lower concentration and less-frequent dosing. More studies are needed to better characterize the efficacy, safety, and dosing of topical cidofovir for the treatment of refractory warts.

  12. Refractory Behaviors of Magnetite-Kaolin Bricks

    Science.gov (United States)

    Adeosun, S. O.; Akpan, E. I.; Gbenebor, O. P.; Taiwo, O. O.; Eke, I. J.

    2016-11-01

    In this work, the suitability of using kaolin-magnetite-plastic clay to produce refractory bricks has been experimentally explored. Thirty bricks of different compositions were produced and fired at 1200°C. The density, shrinkage moisture content, loss on ignition, porosity and permeability of the bricks were examined. Results show that the bricks remained stable during firing and thus possess good insulating characteristics. The highest (2.23 g/cm3) and lowest (2.00 g/cm3) bulk densities obtained in this study are higher than the highest bulk density reported for Al dross-filled refractories (1.23 g/cm3). The bricks also possessed very low effective moisture content (10-23%) and very high compression modulus (16-100 MPa) desirable in insulating refractory bricks with high resistance to abrasion.

  13. Dramatic response to infliximab in refractory neurosarcoidosis

    Directory of Open Access Journals (Sweden)

    Chintamaneni Sreekanth

    2010-01-01

    Full Text Available Sarcoidosis is a systemic disease characterized by noncaseating granulomas in the involved organs. Neurologic manifestations involving the central and/or peripheral nervous system occur in about 5% of patients. Neurosarcoidosis is often refractory to conventional treatment and therefore more effective treatment options are needed. While the etiology of the disease is still unknown, there is now a better understanding of its pathogenesis on a molecular level. It is clear that tumor necrosis factor-α (TNFα plays a pivotal role in the development of the granulomas and it is believed to be a key cytokine involved in the pathogenesis of the disease. Taking advantage of this better understanding of disease pathogenesis, anti-TNFα agents are being increasingly used to treat refractory sarcoidosis. We report a patient with refractory neurosarcoidosis who showed dramatic improvement in the clinical and radiological manifestations following treatment with infliximab; he suffered a relapse upon discontinuation of the medication.

  14. Clinical characteristics of adult epilepsy patients in the 1997 Hong Kong epilepsy registry

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To study the clinical characteristics of 2952 patients with epilepsy who had received drug treatment from the neurology outpatient clinics of eight major hospitals in Hong Kong. Methods Retrospective review of outpatient records. Results 1601 (54.3%) males and 1351 (45.7%) females with a median age of 35.8 years (range, 10-94.8) were studied. Seizure types included generalized tonic-clonic in 80.7% of patients, complex partial in 28.3%, simple partial in 14.4%, atypical absence in 2.6% and myoclonic in 1.4%, and 30.4% of patients had more than one seizure type. EEG, CT brain, MRI brain and neuropsychological evaluation were obtained in 81.2%, 61.7%, 17.0% and 2.2% of patients, respectively. The etiology of epilepsy was cryptogenic in 59.9%, symptomatic in 35.1% and idiopathic in 3.9%; the commonest were intracranial infection, cerebral vascular disease, cranial trauma and perinatal insult. Phenytoin, carbamazepine and valproate were the most frequently used drugs and 25.9% of patients were taking more than two drugs. 48.3% of patients had active seizures in the past six months and 26.4% were considered to have unsatisfactory control of their epilepsy. Medical refractoriness of epilepsy was associated with a history of perinatal insult, intracranial infection, congenital brain malformation, intracranial neoplasm, cerebral vascular disease, hippocampal sclerosis, mental retardation and a history of status epilepticus (P<0.05). Conclusion In this local cohort of adult patients with epilepsy under specialist care, there were a considerable number of patients falling into the category of cryptogenic epilepsy. Risk factors associated with medical refractoriness are similar to previous studies.

  15. Propylene Glycol Toxicity in Adolescent with Refractory Myoclonic Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Kara A. Bjur

    2017-01-01

    Full Text Available Propylene glycol (PG is a solvent commonly used in medications that, while benign at low doses, may cause toxicity in adults and children at high doses. We describe a case and the physiologic sequelae of propylene glycol toxicity manifested in a critically ill adolescent male with refractory myoclonic status epilepticus aggressively treated with multiple PG-containing medications (lorazepam, phenobarbital, and pentobarbital—all within accepted dosing guidelines and a total daily PG exposure previously recognized to be safe. Hemodynamic measurements by bedside echocardiography during clinical toxicity are also reported. Clinicians should have a high index of suspicion for propylene glycol toxicity in patients treated with PG-containing medications even when the total PG exposure is lower than currently accepted limits.

  16. Refractory pediatric cardiogenic shock: A case for mechanical support

    Directory of Open Access Journals (Sweden)

    Anil Sachdev

    2016-01-01

    Full Text Available Acute left ventricular dysfunction in children justifies aggressive treatment because of the high potential for complete recovery. The options for providing mechanical support to the failing heart in a child include extracorporeal membrane oxygenation, left ventricular assist devices, and the use of the intra-aortic balloon pump (IABP. The IABP is a commonly used method of temporary circulatory support in adults. However, despite the availability of pediatric size balloons, the usage of IABP for temporary circulatory support in children has not been widespread. Current case report, first from India in pediatric age group, aims to aware the pediatric intensivist about the role of IABP in providing temporary mechanical cardiovascular support in managing patients with refractory low cardiac output state.

  17. AUTOLOGOUS SERUM SKIN TESTING (ASST) IN CHRONIC IDIOPATHIC URTICARIA

    OpenAIRE

    Arun; Suresh; Raghavendra; Vijay; Ramesh,; Asha; Manali; Jitendra

    2014-01-01

    Chronic idiopathic urticaria (CIU) is a form of urticaria , in which there appears to be persistent activation of mast cells , but the mechanism of mast cell triggering is unknown. The Autologous serum skin test (ASST) is an in vivo test which assesses auto reactivity. ASST could be good screening test for Autoreactive urticaria a subset of chronic idiopathic urticaria. AIMS : To study the clinical profile of chronic idiopathic urticaria and pattern of A...

  18. Idiopathic intracranial hypertension and transverse sinus stenoses

    DEFF Research Database (Denmark)

    Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

    2013-01-01

    An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed to be asso......An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed...... to be associated with the IIH. Initial treatment consisted of symptom relief by a temporary lumbar drain for cerebrospinal fluid (CSF) diversion, while the pros and cons of a more permanent solution by insertion of a ventriculoperitoneal shunt (VPS) or bilateral transverse sinus stent was discussed. A VPS...

  19. Clinical characteristics of idiopathic portal hypertension

    Institute of Scientific and Technical Information of China (English)

    Ozgur Harmanci; Yusuf Bayraktar

    2007-01-01

    Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

  20. Idiopathic gingival enlargement and its management

    Directory of Open Access Journals (Sweden)

    Shetty Arvind

    2010-01-01

    Full Text Available Idiopathic gingival enlargement is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival enlargement exist. This case report addresses the diagnosis and treatment of a case of idiopathic gingival enlargement in a 13-year-old female. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal / occlusal third of the tooth resulting in difficulty in speech and mastication since last three years. Patient also gave a history of surgical treatment being carried out four years back in upper anterior region suggesting of recurrence. Biopsy report confirmed the diagnosis of gingival hyperplasia. Gingivectomy was carried out in all four quadrants by using four different methods.

  1. Idiopathic gingival enlargement and its management

    Directory of Open Access Journals (Sweden)

    Pawan Kumar

    2015-01-01

    Full Text Available Increase in the size of gingiva referred to as "gingival enlargement," usually overfills the interproximal spaces, ballooning out over the teeth and sometimes even protruding into the oral cavity. It may occur as localized enlargement in relation to a single tooth, a group of teeth, or may be generalized involving the entire dentition. It is multifactorial in origin having the influence of bacterial plaque, systemic factors or conditions, genetic predisposition (hereditary or familial, and various medications. Sometimes, it may appear as diffuse enlargement of gingiva without any specific etiologic factor. It is then considered as idiopathic enlargement. In this case report, an idiopathic gingival enlargement was successfully treated using gingivectomy by ledge and wedge procedure. The surgical technique performed yielded functionally as well as esthetically satisfying results. Gingivectomy provides a viable option to manage diffuse enlargement cases. This procedure provides the functional as well as esthetic demands and at the same time, it maintains the integrity of periodontium.

  2. [Pathogenesis of the idiopathic inflammatory myopathies].

    Science.gov (United States)

    Riebeling-Navarro, Carlos; Nava, Arnulfo

    2009-11-01

    The inflammatory myopathies, commonly described as idiopathic, are a group of acquired diseases characterized by an inflammatory infiltrate of the skeletal muscle. On the basis of clinical and immuno-pathological features, three major diseases can be identified: dermatomiositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Immunopathogenesis mechanisms are crucial for discriminating between the three different subsets of inflammatory myopathies. DM is a complement-mediated microangiopathy affecting skin and muscle. PM and IBM are T cell-mediated disorders, where CD8-positive cytotoxic T cells invade muscle fibres expressing MHC class I antigens. This article summarizes the main immunopathological markers. The impact of this new knowledge must be defined in relation to potential therapeutic targets for idiopathic inflammatory myopathies.

  3. Office Procedures in Refractory Chronic Rhinosinusitis.

    Science.gov (United States)

    Thamboo, Andrew; Patel, Zara M

    2017-02-01

    Office procedures in chronic rhinosinusitis (CRS) can be considered before and after medical management, as well as before and after surgical management. This article focuses specifically on refractory CRS, meaning those patients who have failed medical and surgical management already. The options available in the management of refractory CRS depend on the personnel, equipment, and instrumentation available in the office setting; surgeon experience; and patient suitability and tolerability. This article provides readers with possible procedural options that can be done in their clinics with indications, patient selection, potential complications, and postoperative considerations.

  4. Refractory Materials for Flame Deflector Protection System Corrosion Control: Refractory Ceramics Literature Survey

    Science.gov (United States)

    Calle, Luz Marina; Hintze, Paul E.; Parlier, Christopher R.; Curran, Jerome P.; Kolody, Mark; Perusich, Stephen; Whitten, Mary C.; Trejo, David; Zidek, Jason; Sampson, Jeffrey W.; Coffman, Brekke E.

    2009-01-01

    Ceramics can be defmed as a material consisting of hard brittle properties produced from inorganic and nonmetallic minerals made by firing at high temperatures. These materials are compounds between metallic and nonmetallic elements and are either totally ionic, or predominately ionic but having some covalent character. This definition allows for a large range of materials, not all applicable to refractory applications. As this report is focused on potential ceramic materials for high temperature, aggressive exposure applications, the ceramics reviewed as part of this report will focus on refractory ceramics specifically designed and used for these applications. Ceramic materials consist of a wide variety of products. Callister (2000) 1 characterized ceramic materials into six classifications: glasses, clay products, refractories, cements, abrasives, and advanced ceramics. Figure 1 shows this classification system. This review will focus mainly on refractory ceramics and cements as in general, the other classifications are neither applicable nor economical for use in large structures such as the flame trench. Although much work has been done in advanced ceramics over the past decade or so, these materials are likely cost prohibitive and would have to be fabricated off-site, transported to the NASA facilities, and installed, which make these even less feasible. Although the authors reviewed the literature on advanced ceramic refractories 2 center dot 3 center dot 4 center dot 5 center dot 6 center dot 7 center dot 8 center dot 9 center dot 10 center dot 11 center dot 12 after the review it was concluded that these materials should not be ' the focus of this report. A review is in progress on materials and systems for prefabricated refractory ceramic panels, but this review is focusing more on typical refractory materials for prefabricated systems, which could make the system more economically feasible. Refractory ceramics are used for a wide variety of applications

  5. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summariz...

  6. Idiopathic intracranial hypertension in female homozygous twins.

    OpenAIRE

    Fujiwara, S; Sawamura, Y; Kato, T.; Abe, H.; Katusima, H

    1997-01-01

    The authors report on female homozygous twins with idiopathic intracranial hypertension. At the age of 12 years, both twins simultaneously developed visual disturbances with photophobia. At the age of 19 years, an ophthalmological examination disclosed papilloedema in both their eyes. At the age of 22 years, a lumbar puncture showed raised CSF pressure over (200 mm H2O) in both twins. Their neurological and radiological examinations were extremely similar; both of them had severely impaired v...

  7. Optimal management of idiopathic scoliosis in adolescence

    OpenAIRE

    Kotwicki T; Chowanska J; Kinel E; Czaprowski D; Tomaszewski M; Janusz P

    2013-01-01

    Tomasz Kotwicki,1 Joanna Chowanska,1,2 Edyta Kinel,3 Dariusz Czaprowski,4,5 Marek Tomaszewski,1 Piotr Janusz1 1Department of Pediatric Orthopedics, University of Medical Sciences, Poznan, Poland; 2National Scoliosis Foundation, Stoughton, MA, USA; 3Department of Rehabilitation, University of Medical Sciences, Poznan Poland; 4Department of Physiotherapy, Józef Rusiecki University College, Olsztyn, 5Rehasport Clinic, Poznan, Poland Abstract: Idiopathic scoliosis is a three-dimension...

  8. Chronic Idiopathic Intestinal Pseudo-obstruction.

    Science.gov (United States)

    Malagelada

    2000-08-01

    The definition of chronic idiopathic intestinal pseudo-obstruction (CIIP) is somewhat vague because it was based on clinical observations that preceded modern advances in the measurement of gut motility and neuromuscular integrity. Appropriate management of patients with CIIP requires an initial consideration, supported by pertinent diagnostic tests, of the tissue affected (muscle, nerves, both), extent and magnitude of gut propulsive failure, and extraintestinal disease.

  9. The genetic epidemiology of idiopathic scoliosis

    OpenAIRE

    Gorman, Kristen Fay; Julien, Cédric; Moreau, Alain

    2012-01-01

    Purpose Idiopathic scoliosis is a complex developmental syndrome defined by an abnormal structural curvature of the spine. High treatment costs, chronic pain/discomfort, and the need for monitoring at-risk individuals contribute to the global healthcare burden of this musculoskeletal disease. Although many studies have endeavored to identify underlying genes, little progress has been made in understanding the etiopathogenesis. The objective of this comprehensive review was to summarize geneti...

  10. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    2012-01-01

    Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received. Cyclophosphamid

  11. Idiopathic intracranial hypertension in pediatric patients

    Directory of Open Access Journals (Sweden)

    Nada Jirásková

    2008-11-01

    Full Text Available Nada Jirásková, Pavel RozsívalDepartment of Ophthalmology, University Hospital, Hradec Králové, Czech RepublicPurpose: To evaluate retrospectively the features, treatment, and outcome of idiopathic intracranial hypertension (IIH in children.Methods: Nine patients, 15 years and younger, diagnosed with IIH. Inclusion criteria were papilledema, normal brain computer tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 250 mm H2O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy.Results: Of the nine patients, eight were girls. Five girls were overweight and one boy was obese. The most common presenting symptom was headache (5 patients. Diplopia or strabismus did not occur in our group. Visual field abnormalities were present in all eyes, and severe visual loss resulting in light perception vision occurred in both eyes of one patient. Eight patients were treated medically with acetazolamide alone, and one girl needed a combination of acetazolamide and corticosteroids. This girl also required optic nerve sheath decompression surgery. Resolution of papilledema and recovery of visual function occurred in all patients.Conclusions: Idiopathic intracranial hypertension in prepubertal children is rather uncommon. Prompt diagnosis and management are important to prevent permanent visual loss.Keywords: idiopathic intracranial hypertension, pediatric, treatment

  12. JUVENILE IDIOPATHIC ARTHRITIS – A CASE REPORT

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    Paresh H

    2012-11-01

    Full Text Available ABSTRACT: The prevalence of Juvenile idiopathic arthritis (JIA is 0.86 per 1000 children. Subcutaneous nodules have been reported in 5% to 10% of children with JIA. Approximately 90% of patients with RA and subc utaneous nodules test positive for rheumatoid factor (RF, and approximately 40% o f all RF-seropositive patients with RA have subcutaneous nodules, whereas only 6% in volvement is seen in seronegative cases. We hereby report a case of atypical Juvenile idiopathic arthritis (JIA in a 6 year old, female child with joint pain & myalgia along with subcutaneous nodules over the dorsum of feet, hands and elbows. Joint pain initial ly involving the left ankle, slowly progressed to involve the knee, shoulder, wrist, metacar pophalangeal and interphalangeal joints over a period of one year. Joint involvement was not symmetric. RF was Negative. Fundoscopy examination was normal. Histopathological examinat ion revealed a central zone of Fibrinoid necrosis surrounded by epithelioid h istiocytes and occasional lymphocytes. Differential diagnosis of Rheumatoid Nodule (R N or Subcutaneous Granuloma Annulare (SGA or Necrobiosis Lipoidica Diabeticorum was made. In light of clinicopathological findings, both SGA and NLD were ruled out a nd the diagnosis of Juvenile idiopathic arthritis presenting as RF-negative polyarthritis was made.

  13. Idiopathic noncirrhotic portal hypertension: current perspectives

    Directory of Open Access Journals (Sweden)

    Riggio O

    2016-07-01

    Full Text Available Oliviero Riggio,1 Stefania Gioia,1 Ilaria Pentassuglio,1 Valeria Nicoletti,1 Michele Valente,2 Giulia d’Amati2 1Department of Clinical Medicine, Center for the Diagnosis and Treatment of Portal Hypertension, 2Department of Radiological, Oncological, and Pathological Sciences, Sapienza University of Rome, Rome, Italy Abstract: The term idiopathic noncirrhotic portal hypertension (INCPH has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals, splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. Keywords: idiopathic portal hypertension, obliterative portal venopathy, esophageal varices, splenomegaly

  14. Brace Treatment in Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Ali Sehirlioglu

    2012-01-01

    Full Text Available Adolescent idiopathic scoliosis (AIS, the most common pediatric spine problem, is a structural lateral and rotatory curvature of the spine arising in otherwise normal children during puberty. It occurs before skeletal maturity. Although there is still no cause for AIS, the natural history of AIS has been established in the literature very well. The aim of nonoperative treatment is mainly an attempt to prevent progression of the curve. Bracing and surgery have been used for large and or progressive curves currently. Many conservative treatments are available for adolescents with idiopathic scoliosis (AIS. Although there are numerous studies in literature that have tried to summarize the results of treatment, the evidence for their accepted use is still unclear. Many clinicians skeptical about the efficacy of conservative treatments. Because there is no consistency of both the inclusion criteria and the definitions of brace effectiveness. The definition of success or who should be included in the analysis have never been universally agreed upon. The Scoliosis Research Society established parameters for all future AIS bracing studies to be able to make comparison among studies more valid and reliable. These guidelines may standardize orthotic studies by recommending inclusion and assessment criteria and allow the promotion of the effectiveness of different braces and decrease the suspicion about their usefulness. Orthotic treatment in adolescent idiopathic scoliosis is used to control spinal curvatures while waiting for skeletal maturation.

  15. Pulmonary function in children with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Tsiligiannis Theofanis

    2012-03-01

    Full Text Available Abstract Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased work of breathing at rest, during exercise and sleep. Pulmonary hypertension and respiratory failure may develop in severe disease. In this review the epidemiological and anatomical aspects of idiopathic scoliosis are noted, the pathophysiology and effects of idiopathic scoliosis on respiratory function are described, the pulmonary function testing including lung volumes, respiratory flow rates and airway resistance, chest wall movements, regional ventilation and perfusion, blood gases, response to exercise and sleep studies are presented. Preoperative pulmonary function testing required, as well as the effects of various surgical approaches on respiratory function are also discussed.

  16. Treatment of Relapsed and/or Chemotherapy Refractory B-cell Malignancy by CART19

    Science.gov (United States)

    2016-01-26

    Hematopoietic/Lymphoid Cancer; Adult Acute Lymphoblastic Leukemia in Remission; B-cell Adult Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Prolymphocytic Leukemia; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Refractory Chronic Lymphocytic Leukemia; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Chronic Lymphocytic Leukemia; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Chronic Lymphocytic Leukemia; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma

  17. Lubiprostone for chronic constipation in adults.

    Science.gov (United States)

    2014-04-01

    Lubiprostone (Amitiza-Sucampo Pharma Europe), a chloride-channel activator is licensed for the treatment of chronic idiopathic constipation in adults. It received a marketing authorisation in the UK in September 2012. In this article, we consider the evidence for lubiprostone in the management of constipation and how the treatment fits with current management strategies for constipation.

  18. Progress on the M-type phospholipase A2 receptor in idiopathic membranous nephropathy

    Institute of Scientific and Technical Information of China (English)

    Wang Chao; Lu Huan; Yang Cui; Luo Yuezhong

    2014-01-01

    Objective To highlight current knowledge about M-type phospholipase A2 receptor (PLA2R) which is the first human autoantigen discovered in adult idiopathic membranous nephropathy.Data sources Relevant articles published in English from 2000 to present were selected from PubMed.Searches were made using the terms "idiopathic membranous nephropathy,M-type PLA2R and podocyte." Study selection Articles studying the role of M-type PLA2R in idiopathic membranous nephropathy were reviewed.Articles focusing on the discovery,detection and clinical observation of anti-PLA2R antibodies were selected.Results M-type PLA2R is a member of the mannose receptor family of proteins,locating on normal human glomeruli as a transmembrane receptor.The anti-PLA2R in serum samples from MN were primarily IgG4 subclass.Technologies applied to detect anti-PLA2R autoantibody are mainly WB,lIFT,ELISA and so on.Studies from domestic and overseas have identified a strongly relationship between circulating anti-PLA2R levels and disease activity.Conclusion Recent discoveries corresponding to PLA2R facilitate a better understanding on IMN pathogenesis and may provide a new tool to its diagnosis,differential diagnosis,risk evaluation,response monitoring and patient-specific treatment.

  19. Idiopathic systemic granulomatous pathology causing sudden death due to myocarditis: A rare case report

    Directory of Open Access Journals (Sweden)

    Harpal Singh

    2015-01-01

    Full Text Available Idiopathic granulomatous myocarditis is extremely rare, particularly since the introduction of drugs effective against tuberculosis (TB, viruses, fungi and the effective treatment of sarcoidosis. Here is a case of a 65-year-old female prisoner having history of sudden collapse and ultimately death. Autopsy findings of various viscera on histopathological examination show granulomatous pathology, that is, in spleen, liver and in the left ventricular wall of heart. Ziehl-Neelsen staining of the sections show the absence of acid fast bacilli, negative for fungal staining as most of the granulomas are noncaseating type with presence of giant cells having no asteroid body and Schuamann body, real-time polymerase chain reaction for TB is negative. Idiopathic giant cell myocarditis is a disease of relatively young adults, that is, between 3 rd and 4 th decade of life. So, this case is strongly considered to be a case of sudden death due to myocarditis as a result of idiopathic systemic granulomatous pathology, a rare case in in literature.

  20. Pedicle morphology of the thoracic spine in preadolescent idiopathic scoliosis: magnetic resonance supported analysis.

    Science.gov (United States)

    Catan, Hüseyin; Buluç, Levent; Anik, Yonca; Ayyildiz, Erhan; Sarlak, Ahmet Yilmaz

    2007-08-01

    Although several studies have been reported on the adult vertebral pedicle morphology, little is known about immature thoracic pedicles in patients with idiopathic scoliosis. A total of 310 pedicles (155 vertebrae) from T1 to T12 in 10-14 years age group were analyzed with the use of magnetic resonance imaging and digital measurement program in 13 patients with right-sided thoracic idiopathic scoliosis. Each pedicle was measured in the axial and sagittal planes including transverse and sagittal pedicle width and angles, chord length, interpedicular distance and epidural space width on convex and concave sides of the curve. The smallest transverse pedicle widths were in the periapical region and the largest were in the caudal region. No statistically significant difference in transverse pedicle widths was detected between the convex and concave sides. The transverse pedicle angle measured 15.56 degrees at T1 and decreased to 6.32 degrees at T12. Chord length increased gradually from the cephalad part of the thoracic spine to the caudad part as the shortest length was seen at T1 convex level with a mean of 30.45 mm and the largest length was seen at T12 concave level with a mean of 41.73 mm. The width of epidural space on the concave side was significantly smaller than that on the convex side in most levels of the curve. Based on the anatomic measurements, it may be reasonable to consider thoracic pedicle screws in preadolescent idiopathic scoliosis.

  1. Reduced articular cartilage thickness in joints without a history of active arthritis in children with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Pradsgaard, Dan Østergaard; Spannow, Anne Helene; Heuck, Carsten;

    Background: The functional disability experienced in juvenile idiopathic arthritis is primarily caused by degeneration of the osteocartilaginous structures due to the inflammatory process in the synovium. It is therefore essential for evaluating the therapeutic efficacy to closely monitor...... the structural damage during the disease course. During the past decade musculoskeletal ultrasound (US) has become an established diagnostic method in adult rheumatology and within recent years an increased attention for utilizing US in pediatric rheumatology has emerged. Previously we have found differences...

  2. Health-related quality of life of adolescents conservatively treated for idiopathic scoliosis in Korea: a cross-sectional study

    OpenAIRE

    Lee, Hyejung; Choi, Jihea; Hwang, Jin-Ho; Park, Jung Hyun

    2016-01-01

    Background Young adolescents with scoliosis are more likely than adults to experience psychological distress affecting health-related quality of life (HRQoL). Adolescence is a sensitive period of psychological development, and thus physical deformity from scoliosis can negatively affect body image and appearance of adolescents. The present study evaluated HRQoL in young Korean adolescents with idiopathic scoliosis and identified related factors. Methods One hundred and ten adolescents with id...

  3. An Overview on China's Refractories-Market and New Demands

    Institute of Scientific and Technical Information of China (English)

    LIU Jiehua; ZHOU Ningsheng

    2004-01-01

    Based on the authors' information sources and personal viewpoints, this paper provides updated information on China s refractories situation in terms of output, consumption in main refractory using sectors, exportation and importation of refractory products and raw materials,structural change, future prosperity and new demands .From authors understanding, some analyses have also been given.

  4. Export and Import Situation of Refractories in China

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    China has been the biggest country in production and exporting of refractories. The article describes the problems in the export and import and suggestions on development of refractories. In the future, China will expand its cooperation with world refractories community in science, technology, economy and trade to play a more important role in the international and domestic markets.

  5. Tungsten and other refractory metals for VLSI applications II

    Energy Technology Data Exchange (ETDEWEB)

    Broadbent, E.K.

    1987-01-01

    This book presents papers on tungsten and other refractory metals for VLSI applications. Topics include the following: Selectivity loss and nucleation on insulators, fundamental reaction and growth studies, chemical vapor deposition of tungsten, chemical vapor deposition of molybdenum, reactive ion etching of refractory metal films; and properties of refractory metals deposited by sputtering.

  6. Challenges in the management of juvenile idiopathic arthritis with etanercept

    Directory of Open Access Journals (Sweden)

    Clare E Pain

    2009-03-01

    Full Text Available Clare E Pain, Liza J McCannAlder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, UKAbstract: Biologic agents have been designed with the help of immunological studies to target particular areas of the immune system which are thought to play a role in the pathogenesis of disease. Etanercept is a soluble anti-tumor necrosis factor alpha (TNF-α agent licensed for the treatment of active poly-articular juvenile idiopathic arthritis (JIA in children aged 4 to 17 years who have failed to respond to methotrexate alone, or who have been intolerant of methotrexate. The safety and efficacy of etanercept in this patient group has been established by one randomized controlled trial and several longitudinal studies. This, together with the fact that until recently etanercept was the only anti-TNF licensed in JIA, has made it the most common first choice biologic for many clinicians. However, there are still many unanswered questions about etanercept, including its efficacy and safety in different subtypes of JIA, in children under 4 years of age and in those with uveitis. There are still concerns about the long term safety of TNF antagonists in the pediatric age group and unanswered questions about increased risks of malignancy and infection. Although adult studies are useful to improve understanding of these risks, they are not a substitute for good quality pediatric research and follow-up studies. Adult trials often include greater numbers of patients. However, they evaluate a different population and drug behavior may vary in children due to differences in metabolism, growth and impact on a developing immune system. In addition, rheumatoid arthritis is a different disease than JIA. Clinicians need to carefully weigh up the risk benefit ratio of anti-TNF use in children with JIA and push for robust clinical trials to address the questions that remain unanswered. This article summarizes the evidence available for use of etanercept in children

  7. Synthetic Raw Materials for Refractories in China

    Institute of Scientific and Technical Information of China (English)

    SHIGan; YUEWeidong

    2001-01-01

    The development and status of synthetic raw materials for refractories in China are reviewed,including magne-site-based materials,alumina ,ullite,calcium alumi-nate cement ,ultrafine powder,spinel,silicon carbide and cordierite.The research work on calcium zirconate and sialon materials are also introduced.

  8. Clinical management and outcome of refractory asthma in the UK from the British Thoracic Society Difficult Asthma Registry.

    Science.gov (United States)

    Sweeney, Joan; Brightling, Chris E; Menzies-Gow, Andrew; Niven, Robert; Patterson, Chris C; Heaney, Liam G

    2012-08-01

    Refractory asthma represents a significant unmet clinical need. Data from a national online registry audited clinical outcome in 349 adults with refractory asthma from four UK specialist centres in the British Thoracic Society Difficult Asthma Network. At follow-up, lung function improved, with a reduction in important healthcare outcomes, specifically hospital admission, unscheduled healthcare visits and rescue courses of oral steroids. The most frequent therapeutic intervention was maintenance oral corticosteroids and most steroid sparing agents (apart from omalizumab) demonstrated minimal steroid sparing benefit. A significant unmet clinical need remains in this group, specifically a requirement for therapies which reduce systemic steroid exposure.

  9. Antibodies to m-type phospholipase A2 receptor in children with idiopathic membranous nephropathy.

    Science.gov (United States)

    Kumar, Vinod; Ramachandran, Raja; Kumar, Ashwani; Nada, Ritambhra; Suri, Deepti; Gupta, Anju; Kohli, Harbir Singh; Gupta, Krishan Lal; Jha, Vivekanand

    2015-08-01

    Idiopathic membranous nephropathy (IMN), the commonest cause of adult nephrotic syndrome (NS), accounts for only a minority of paediatric NS. Antibodies to m-type phospholipase A2 receptor (PLA2R) are seen in two-thirds of adult IMN cases. PLA2R staining in glomerular deposits is observed in 74% and 45% of adult and paediatric IMN cases, respectively. However, there are no reports of anti-PLA2R in paediatric IMN. We evaluated anti-PLA2R levels and PLA2R in gloemrular deposits in paediatric IMN seen at our center. Five cases were enrolled, all the cases stained for PLA2R in glomeruli and three (60%) had antibodies to PLA2R antigen. There was a parellel reduction in proteinuria and anti-PLA2R titer. The present report suggests that PLA2R has a contributory role in the pathogenesis of paediatric IMN.

  10. Monoclonal Antibody Therapy Before Stem Cell Transplant in Treating Patients With Relapsed or Refractory Lymphoid Malignancies

    Science.gov (United States)

    2015-12-07

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  11. Idiopathic scoliosis; a biomechanical and functional anatomical study.

    NARCIS (Netherlands)

    Veldhuizen, Albert Gerrit

    1985-01-01

    Although many types of causes of scoliosis are known, the idiopathic variety comprises the largest group and as its name indicates, its a etiology is unknown. Idiopathics coliosis develops in a previously normal vertebral column and has been recognized as an entity for more than a century.

  12. Abnormal glomerular basement membrane in idiopathic multicentric osteolysis

    NARCIS (Netherlands)

    Bakker, SJL; Vos, GD; Verschure, PDMM; Mulder, AH; Tiebosch, TMG

    1996-01-01

    The primary cause of nephropathy in idiopathic multicentric osteolysis is as yet unknown. We report a young girl with idiopathic multicentric osteolysis and nephropathy. An abnormal glomerular basement membrane was the only abnormality found in a renal biopsy taken 2 years before the development of

  13. Perceived health status in self-reported adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Andersen, Mikkel Ø; Thomsen, Karsten; Kyvik, Kirsten O

    2010-01-01

    A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort.......A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort....

  14. 2011 China's Refractories Raw Materials Symposium Held in Zhengzhou

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    @@ On April 20-22,2011,China's Refractories Raw Materials Symposium,co-sponsored by Refractories Branch of The Chinese Society for Metals and China Henan Refractory Industry Association,was held successfully in Zhengzhou,Henan Province.Over 150 delegates from 100 refractory raw materials and products enterprises,testing equipment manufacturers,raw materials trade companies,institutes,universities,testing institutions,and industrial organizations attended the symposium.Wang Shouye,secretary-general of Refractories Branch of The Chinese Society for Metals and vice president of Sinosteel Luoyang Institute of Refractories Research Co.,Ltd.,made the address of welcome.Zhang Yazhong,vice chairman of Henan Committee of the Chinese People's Political Consultative Conference and honorary chairman of China Henan Refractory Industry Association,gave a speech.

  15. Stiripentol in refractory status epilepticus.

    Science.gov (United States)

    Grosenbaugh, Denise K; Mott, David D

    2013-09-01

    Benzodiazepines (BZDs), which enhance γ-aminobutyric acid (GABAA ) receptor-mediated inhibition, are the first-line therapy for treatment of status epilepticus (SE). However, pharmacoresistance to BZDs develops rapidly after SE initiation. This is due to an activity-dependent internalization of BZD-sensitive GABAA receptors during SE. Stiripentol (STP) is a positive allosteric modulator of GABAA receptors with a unique subunit selectivity profile. We report that in a rodent model of SE, STP terminates behavioral seizures and remains effective in established SE when seizures have become BZD resistant. The anticonvulsant effects of STP are age dependent, with greater potency in juvenile animals. Whole cell recordings from dentate granule cells in hippocampal slices reveal that STP potentiates GABAergic inhibitory postsynaptic currents (IPSCs) and tonic GABAergic currents by acting at a site on the GABAA receptor that is separate from the benzodiazepine binding site. This potentiation persists in established SE, whereas potentiation of GABAergic inhibition by BZDs is lost. STP potentiates IPSCs in juvenile animals with greater potency than in adult animals. We suggest that STP, either alone or as add-on therapy, may prove useful in treating established and BZD-resistant status epilepticus. Furthermore, STP may be particularly effective in terminating SE in children when SE is most prevalent.

  16. 成人膜性肾病患者血清抗PLA2R抗体与病情的相关性%Correlation of anti-M-type phospholipase A2 receptor antibody with disease severity in adult patients with idiopathic membranous nephropathy

    Institute of Scientific and Technical Information of China (English)

    周广宇; 金玲; 于晶; 张芝平

    2012-01-01

    目的 分析成人膜性肾病患者血清抗M型磷脂酶A2受体(PLA2R)抗体与特发性膜性肾病( IMN)实验室指标的相关性,探讨抗PLA2R抗体在IMN发病中的作用.方法 选取经肾活检证实的46例肾小球疾病患者,包括20例IMN、7例IgA肾病、6例乙型肝炎病毒相关性膜性肾病( HBV-MN)、6例微小病变性肾病、4例局灶性节段性肾小球硬化、3例Ⅴ型狼疮肾炎.应用Western印迹法检测血清抗PLA2R抗体,并对其与IMN患者血清白蛋白、24 h尿蛋白量、血清总胆固醇和Scr作相关性分析.结果 20例IMN患者中15例血清抗PLA2R抗体阳性,阳性比例为75%;7例IgA肾病患者中1例抗PLA2R抗体阳性,阳性比例为14.29%;6例HBV-MN患者中1例阳性,阳性比例为16.67%;其余患者均为阴性.IMN患者血清抗PLA2R抗体阳性比例显著高于继发性膜性肾病和其他肾小球肾炎(均P<0.01),且抗PLA2R抗体水平与IMN患者尿蛋白量呈正相关(r=0.803,P<0.01);与血清白蛋白呈负相关(r=-0.816,P<0.01).结论 IMN患者血清抗PLA2R抗体阳性比例高,提示抗PLA2R抗体可能是IMN的特异性抗体.该抗体与尿蛋白量呈正相关,提示抗PLA2R抗体可能是IMN的致病性抗体.%Objective To investigate the correlation of serum anti-M-type phospholipase A2 receptor (PLA2R) antibody with laboratory parameters of idiopathic membranous nephropathy (IMN) in adult patients with membranous nephropathy (MN),and to explore the role of anti-PLA2R antibody in the pathogenesis of IMN. Methods Forty-six adult patients with biopsy-proved glomerular diseases were involved in this study,including 20 cases with IMN,7 cases with IgA nephropathy (IgAN),6 cases with hepatitis B-associated membranous nephropathy (HBV-MN),6 cases with minimal change nephropathy (MCN),4 cases with focal segmental glomerulosclerosis (FSGS) and 3 cases with class Ⅴ lupus nephritis.Total RNA was extracted from human glomeruli and was reversely transcribed to the

  17. Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis.

    Science.gov (United States)

    Milman, N; Pedersen, F M

    1998-07-01

    Idiopathic pulmonary haemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar haemorrhage, often presenting with haemoptysis. Many patients have iron deficiency anaemia due to deposition of haemosiderin iron in the alveoli, and eventually develop moderate pulmonary fibrosis. Typically, intensive search for an aetiology ends up negative. There is no evidence of pulmonary vasculitis or capillaritis. The aetiology is obscure, but may be an immunological or toxic mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH affects both children and adults. During an acute episode, a chest X-ray demonstrates bilateral, alveolar infiltrates. Sputum examination discloses haemosiderin-laden alveolar macrophages. Diagnosis is established by lung biopsy (fiber-optic or thoracoscopic), showing large numbers of haemosiderin-laden macrophages in the alveoli and without evidence of capillaritis or deposition of immunoglobulins. Corticosteroids and/or immunosuppressive drugs may be effective during an acute bleeding episode, and may in some patients improve symptoms and prognosis on the long-term, but the response to treatment displays great interindividual variation.

  18. Idiopathic thoracic aortic aneurysm at pediatric age.

    Science.gov (United States)

    Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J

    2009-03-01

    A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

  19. The Electrophysiology in Idiopathic Senile Macular Hole

    Institute of Scientific and Technical Information of China (English)

    1992-01-01

    Of 12 patients with idiopathic senile full- thickness macular hole, 3 had bilateral involvement, 9 had monocular macular hole. Flash ERG and pattern VEP were performed in the bilateral eyes of all patients. The abnormal rate of the pattern VEP was 93.3% when we used 15' checkboard stimulus, the changes of the VEP appeared as delayed latencies, reduced amplitudes or malformation of P100. The abnormal rate of the flash ERG was 53.3%, showing primary characteristics of reduced amplitudes of cone response b...

  20. Genetics of idiopathic generalized epilepsy: An overview

    Directory of Open Access Journals (Sweden)

    D. K. V. Prasad

    2013-01-01

    Full Text Available Idiopathic generalized epilepsy (IGE is a common type of epilepsy. Strong support for a genetic role in IGE comes from twin and family studies. Several subtypes of IGE have been reported but families often have members affected with different subtypes. Major advances have been made in the understanding of genetic basis of monogenic inherited epilepsies. However, most IGEs are complex genetic diseases and some susceptible IGE genes are shared across subtypes that determine subtypes in specific combinations. The high throughput technologies like deoxyribonucleic acid microarrays and sequencing technologies have the potential to identify causative genes or loci in non-familial cases.

  1. Juvenile idiopathic arthritis: a clinical overview

    Energy Technology Data Exchange (ETDEWEB)

    Davidson, J

    2000-02-01

    The chronic arthritides in childhood remain a poorly understood group of conditions. Their classification has been a source of much confusion over the years with differences in terminology between Europe and North America. A significant step forward in paediatric rheumatology has been the recent development of an internationally agreed classification system which uses the overall term juvenile idiopathic arthritis (JIA). The various subtypes of JIA and their clinical features are described, together with an overview of their differential diagnosis, complications and outcomes. An outline of current management strategies is given and potential future developments highlighted.

  2. Idiopathic calcinosis cutis of nasal dorsum

    Directory of Open Access Journals (Sweden)

    Shrinath D.P Kamath

    2012-01-01

    Full Text Available A 23-year-old lady presented with a slowly progressing firm mass on the nasal dorsum since 8 months. Her biochemical, haematological and collagen vascular disease screening tests were normal. Radiographs of the nasal bones showed a subcutaneous calcifying lesion with no evidence of nasal bone erosion. A diagnosis of idiopathic calcinosis cutis (ICC was made. The mass was excised and soft tissue defect was augmented with silicone prosthesis. The histopathology with the haematoxylin and eosin staining and von Kossa stain confirmed the diagnosis of calcinosis cutis. This is an unusual presentation of ICC involving the nasal dorsum requiring surgery and nasal dorsal augmentation with silicone prosthesis.

  3. Juvenile idiopathic arthritis: the paediatric perspective

    Energy Technology Data Exchange (ETDEWEB)

    Jordan, Alison [Birmingham Children' s Hospital, Department of Adolescent Rheumatology, Birmingham (United Kingdom); McDonagh, Janet E. [Birmingham Children' s Hospital, Institute of Child Health, Birmingham (United Kingdom)

    2006-08-15

    Paediatric rheumatology is a relatively new specialty that has developed rapidly over the last 30 years. There have been major advances, which have included improvements in the classification and management of juvenile idiopathic arthritis (JIA). The former has led to enhanced international collaboration with disease registries, multicentre research and the development of new therapeutic agents. This has resulted in improved disease control and remission induction in many. There is, however, still significant morbidity associated with JIA during childhood, adolescence and adulthood, and challenges for the future include early identification of those with a poorer prognosis, appropriate administration of safe therapies and optimizing outcomes as young people move through adolescence into adulthood. (orig.)

  4. Update on idiopathic hirsutism: diagnosis and treatment.

    Science.gov (United States)

    Erem, C

    2013-01-01

    Idiopathic hirsutism (IH) is defined as hirsutism in conjunction with normal ovulatory function and normal serum androgen levels. The pathogenesis of IH is still not clear. Increased peripheral 5alpha-reductase enzyme activity and abnormalities of androgen receptor gene polymorphisms have been postulated to explain the pathogenesis of this disorder. It is diagnosed in women who have hirsutism, normal ovulatory function, and normal levels of serum total or free testosterone. Combination treatment of IH, including androgen suppression, peripheral androgen blockade and cosmetic methods is most effective.

  5. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does...... however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...... of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting....

  6. Idiopathic gingival enlargement: A case report

    Directory of Open Access Journals (Sweden)

    Rajesh Shah

    2015-09-01

    Full Text Available Idiopathic gingival fibromatosis is a relatively rare condition characterized by the proliferation of the gingival tissues resulting in masticatory, esthetics, phonetics and psychological disturbances. We present a case with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal/occlusal third of the tooth in the left maxillary region. Gingivectomy was carried out in all four quadrants. Periodic recalls showed maintenance of good oral hygiene and one year follow-up revealed no recurrence.JCMS Nepal. 2015;11(1: 26-28

  7. Idiopathic esophagopleural fistula in the newborn

    Energy Technology Data Exchange (ETDEWEB)

    Iannaccone, G.; Cozzi, F.; Roggini, M.; Capocaccia, P.

    1982-07-01

    Idiopathic rupture of the esophagus in the neonate is a rare event, probably related to the same mechanism of ischemic necrosis responsible for other 'spontaneous' g.i. tract perforations in the newborn. The laceration is usually located on the right aspect of the distal esophagus and is complicated by esophagopleural fistula and hydropneumothorax. Plain chest film and esophagography are diagnostic. The condition is an emergency one and usually carries a bad prognosis without prompt surgical repair. A typical case is reported in a baby who survived without early surgery; a residual tiny blind pouch and a small hiatal hernia required surgery at 1 year of age.

  8. Behavioral conditioning and idiopathic environmental intolerance.

    Science.gov (United States)

    Giardino, N D; Lehrer, P M

    2000-01-01

    Idiopathic environmental intolerance (IEI) is a poorly understood condition that may involve disturbances in immunologic, neurologic, endocrine, behavioral, emotional, and cognitive processes. This chapter reviews theories and evidence that behavioral conditioning processes, including pharmacologic sensitization, conditioned immunomodulation, and conditioned odor and taste aversions, may play a role in the development and maintenance of IEI. It also reviews the psychophysiologic concepts of individual response specificity and situational response stereotypy as potential explanations for the individual differences observed in specific responses to environmental stimuli in patients with IEI. Finally, the treatment implications of a conditioning account of IEI are discussed as part of a more comprehensive treatment approach that incorporates other behavioral and nonbehavioral strategies.

  9. Idiopathic environmental intolerance: case definition issues.

    Science.gov (United States)

    Kreutzer, R

    2000-01-01

    Case definitions of the same phenomenon may be different for different purposes. Case definitions usually become more specific over time as more information about the condition becomes available. Idiopathic environmental intolerance is one of many labels for a heterogeneous group of conditions in which subjects describe multiple symptoms that are attributed to exposure to extremely low doses of common chemicals. Dr. Kreutzer presents issues in case definition for clinical purposes and for population-based studies, and makes recommendations for the clinician and for the public health investigator.

  10. Autonomic symptoms in idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Ferini-Strambi, Luigi; Oertel, Wolfgang; Dauvilliers, Yves

    2014-01-01

    , and sexual dysfunction. Our results show that compared to control subjects with a similar overall age and sex distribution, patients with iRBD experience significantly more problems with gastrointestinal, urinary, and cardiovascular functioning. The most prominent differences in severity of autonomic......Patients with idiopathic REM sleep behavior disorder (iRBD) are at very high risk of developing neurodegenerative synucleinopathies, which are disorders with prominent autonomic dysfunction. Several studies have documented autonomic dysfunction in iRBD, but large-scale assessment of autonomic...

  11. Phototesting In The Idiopathic Photodermatoses Among Indians

    Directory of Open Access Journals (Sweden)

    Bejoy P

    1998-01-01

    Full Text Available 50 patients with idiopathic photodermatoses were phototested using the solar simulator, with broad spectrum wavebands of the whole spectrum (WS and the whole spectrum â€" ultraviolet B (WS- UVB to determine the minimal erythema dose. Cases diagnosed as solar urticaria were further tested with visible light. Among the photodermatoses seen in this part of the country polymorphic light eruption (PLE was the commonest followed by chronic actinic dermatitis (CAD and solar urticaria. The UVB wavelengths appeared to be more responsible than UVA in PLE and CAD while the UVA and visible wavelengths were responsible for solar urticaria.

  12. The application septonasal bidirectional mucoperiosteal flap in treatment of refractory choanal atresia in adults%鼻中隔双向黏骨膜瓣应用于成人难治性后鼻孔闭锁的手术治疗

    Institute of Scientific and Technical Information of China (English)

    王传喜; 刘少峰; 范国康; 杨蓓蓓

    2012-01-01

    目的:探讨鼻中隔双向黏骨膜瓣治疗成人难治性后鼻孔闭锁的效果.方法:9例后鼻孔闭锁患者,其中先天性闭锁2例,鼻咽癌放疗后闭锁7例.所有患者均接受了鼻内镜下后鼻孔成形术,术中使用鼻中隔双向黏骨膜瓣,术后未放置硅胶扩张管.观察患者术后的症状改善程度及是否发生后鼻孔再狭窄或闭锁.结果:9例患者术后鼻塞、张口呼吸等症状明显改善.在平均19.3个月的随访时间内,新成形后鼻孔通畅,黏膜瓣无移位及坏死,亦无后鼻孔再狭窄或闭锁发生.其中2例患者出现鼻腔粘连,l例出现后鼻孔边缘肉芽组织增生,经鼻内镜处理后无复发.结论:鼻内镜下后鼻孔成形术中,鼻中隔双向黏骨膜瓣的应用可降低再狭窄或闭锁的发生率,术后可不用放置硅胶扩张管.%Objective:To explore the effects of septonasal bidirectional mucoperiosteal flap in the treatment of refractory choanal atresia in adults. Method: Analysis of 9 cases for choanal atresia was conducted. Two cases were of congenital origin and 7 cases of acquired origin, which was a complication of radiotherapy for nasopharyn-geal carcinoma. The patients received transnasal endoscopic surgery using the septonasal bidirectional mucoperiosteal flap, without positioning the postoperative expansion tube. After the operation, the changes of symptoms were observed and the recurrent rate of restenosis or atresia was investigated in the follow-up time. Result; The symptoms of nasal obstruction and mouth breathing improved significantly in the 9 patients. At an average follow-up time of 19. 3 months, the new forming posterior nare remained patent. The mucoperiosteal flap had no shift or necrosis. No restenosis or atresia happened. Nasal adhesion occurred in two patients. Granulation tissue hyperpla-sia was found in 1 patient,who underwent endoscopic revision without recurrence. Conclusion:The application of mucoperiosteal flap in transnasal

  13. EXTRASOLAR REFRACTORY-DOMINATED PLANETESIMALS: AN ASSESSMENT

    Energy Technology Data Exchange (ETDEWEB)

    Jura, M.; Xu, S., E-mail: jura@astro.ucla.edu, E-mail: sxu@astro.ucla.edu [Department of Physics and Astronomy, University of California, Los Angeles, CA 90095-1562 (United States)

    2013-02-01

    Previously published observations of 60 externally polluted white dwarfs show that none of the stars have accreted from intact refractory-dominated parent bodies composed mainly of Al, Ca, and O, although planetesimals with such a distinctive composition have been predicted to form. We propose that such remarkable objects are not detected by themselves because, unless they are scattered outward from their initial orbit, they are engulfed and destroyed during the star's asymptotic giant branch evolution. As yet, there is at most only weak evidence supporting a scenario where the composition of any extrasolar minor planet can be explained by blending of an outwardly scattered refractory-dominated planetesimal with an ambient asteroid.

  14. [Liposteroid therapy for refractory epileptic spasms].

    Science.gov (United States)

    Shimono, Kuriko Kagitani; Imai, Katsumi; Idoguchi, Rie; Kamio, Noriko; Okinaga, Takeshi; Ozono, Keiichi

    2003-11-01

    Liposteroid was administered intravenously to 6 patients with refractory epileptic spasms. In one case, the spasms initially disappeared but then reappeared after three months. Another case had a transient and slight decrease of epileptic spasms. In the only patient in whom spasms disappeared, EEG abnormalities were greatly improved with diffuse spikes and waves changing into focal spikes. Two cases displayed hyperexcitability, insomnia and acting out behavior, and the therapy was discontinued in one of them. One case had appetite loss and another showed an increase in tonic seizures. No patient had serious adverse effects such as infection, edema, subdural hematoma and brain shrinkage. Although liposteroid therapy has been recommended as an easy, useful and safe alternative for ACTH, we found considerable adverse effects and only a small effect on refractory spasms, and conclude that the regimen should be modified.

  15. Midazolam and pentobarbital for refractory status epilepticus.

    Science.gov (United States)

    Holmes, G L; Riviello, J J

    1999-04-01

    Status epilepticus, a serious, life-threatening emergency characterized by prolonged seizure activity, occurs most commonly in pediatric patients. Although initial therapies with agents such as diazepam, phenytoin, or phenobarbital generally terminate seizure activity within 30-60 minutes, patients with refractory status epilepticus (RSE) lasting longer require additional intervention. High-dose pentobarbital has been the most commonly prescribed agent for the management of RSE in children; however, midazolam has emerged as a new treatment option. This review compares the use of midazolam with pentobarbital in published reports of pediatric RSE. Both drugs effectively terminated refractory seizure activity, although pentobarbital use was complicated by hypotension, delayed recovery, pneumonia, and other adverse effects. Midazolam use was effective and well tolerated, affirming its value in pediatric RSE management.

  16. Current Approaches in the Treatment of Relapsed and Refractory Acute Myeloid Leukemia

    Directory of Open Access Journals (Sweden)

    Nestor R. Ramos

    2015-04-01

    Full Text Available The limited sensitivity of the historical treatment response criteria for acute myeloid leukemia (AML has resulted in a different paradigm for treatment compared with most other cancers presenting with widely disseminated disease. Initial cytotoxic induction chemotherapy is often able to reduce tumor burden to a level sufficient to meet the current criteria for “complete” remission. Nevertheless, most AML patients ultimately die from their disease, most commonly as clinically evident relapsed AML. Despite a variety of available salvage therapy options, prognosis in patients with relapsed or refractory AML is generally poor. In this review, we outline the commonly utilized salvage cytotoxic therapy interventions and then highlight novel investigational efforts currently in clinical trials using both pathway-targeted agents and immunotherapy based approaches. We conclude that there is no current standard of care for adult relapsed or refractory AML other than offering referral to an appropriate clinical trial.

  17. Early Use of the NMDA Receptor Antagonist Ketamine in Refractory and Superrefractory Status Epilepticus

    Directory of Open Access Journals (Sweden)

    F. A. Zeiler

    2015-01-01

    Full Text Available Refractory status epilepticus (RSE and superrefractory status epilepticus (SRSE pose a difficult clinical challenge. Multiple cerebral receptor and transporter changes occur with prolonged status epilepticus leading to pharmacoresistance patterns unfavorable for conventional antiepileptics. In particular, n-methyl-d-aspartate (NMDA receptor upregulation leads to glutamate mediated excitotoxicity. Targeting these NMDA receptors may provide a novel approach to otherwise refractory seizures. Ketamine has been utilized in RSE. Recent systematic review indicates 56.5% and 63.5% cessation in seizures in adults and pediatrics, respectively. No complications were described. We should consider earlier implementation of ketamine or other NMDA receptor antagonists, for RSE. Prospective study of early implementation of ketamine should shed light on the role of such medications in RSE.

  18. Molecular sidebands of refractory elements for ISOL

    Energy Technology Data Exchange (ETDEWEB)

    Kronenberg, A. [Oak Ridge Associated Universities, Oak Ridge (United States)], E-mail: kronenberga@ornl.gov; Spejewski, E.H.; Carter, H.K.; Mervin, B.; Jost, C. [Oak Ridge Associated Universities, Oak Ridge (United States); Stracener, D.W. [Oak Ridge National Laboratory, TN 37831 (United States); Lapi, S. [TRIUMF (Canada); Bray, T. [Auburn University (United States)

    2008-10-15

    The formation of molecular sidebands of refractory elements, such as V, Re, Zr, Mo, Tc, is discussed. The focus is on in situ sideband formation and its advantage for the release process. An atomic {sup 48}V beam has been produced in a two step process, forming the oxide in situ, transporting it through the target-ion source as a chloride and destroying the chlorine sideband in the ion source. The sideband formation of Re, Zr, Mo, Tc is discussed.

  19. Molecular Sidebands of Refractory Elements for ISOL

    Energy Technology Data Exchange (ETDEWEB)

    Kronenberg, Andreas [Oak Ridge Associated Universities (ORAU); Spejewski, Eugene H. [Oak Ridge Associated Universities (ORAU); Carter, H Kennon [Oak Ridge Associated Universities (ORAU); Mervin, Brenden T. [Oak Ridge Associated Universities (ORAU); Jost, Cara [Oak Ridge Associated Universities (ORAU); Stracener, Daniel W [ORNL; Lapi, Suzanne [TRIUMF, Canada; Bray, T. H. [Auburn University, Auburn, Alabama

    2008-01-01

    The formation of molecular sidebands of refractory elements, such as V, Re, Zr, Mo, Tc, is discussed. The focus is on in situ sideband formation and its advantage for the release process. An atomic 48V beam has been produced in a two step process, forming the oxide in situ, transporting it through the target-ion source as a chloride and destroying the chlorine sideband in the ion source. The sideband formation of Re, Zr, Mo, Tc is discussed.

  20. Extracorporeal life support for refractory ventricular tachycardia

    Science.gov (United States)

    Joseph, Nicholas; Hofmann, James P.; Saranteas, Theodosios; Papadimos, Thomas J.

    2017-01-01

    Extracorporeal life support (ECLS) is a very effective bridging therapy in patients with refractory ventricular tachycardia (VT) associated with cardiogenic shock. A moribund patient in extremis, is not amenable to optimization by standard ACC/AHA guidelines. New approaches and novel salvage techniques are necessary to improve outcomes in patients with refractory clinical settings such as malignant ventricular arrhythmias, cardiac arrest, cardiogenic shock and/or pulmonary failure until further management options are explored. Data base searches were done using key words such as ECLS, VT, cardiac arrest, VT ablation, venoarterial extra-corporeal membrane oxygenation (VA-ECMO). The use of ECLS has been described in a few case reports to facilitate VT ablation for incessant VT refractory to medical therapy. For patients with, out-of- hospital ventricular fibrillation (VF) and VT, Minnesota Resuscitation Consortium has implemented emergent advanced perfusion and reperfusion strategy, followed by coronary angiography and primary coronary intervention to improve outcome. The major indications for ECLS are cardiogenic shock related to acute myocardial infarction, myocarditis, post embolic acute cor pulmonale, drug intoxication and post cardiac arrest syndrome with the threat of multi-organ failure. ECLS permits the use of negative inotropic antiarrhythmic drug therapy, facilitates the weaning of catecholamine administration, thereby ending the vicious cycle of catecholamine driven electric storm. ECLS provides hemodynamic support during ablation procedure, while mapping and induction of VT is undertaken. ECLS provides early access to cardiac catheterization laboratory in patients with cardiac arrest due to shockable rhythm. The current evidence from literature, supports the use of ECLS to ensure adequate vital organ perfusion in patients with refractory VT. ECLS is a safe, feasible and effective therapeutic option when conventional therapies are insufficient to support

  1. Correlation of Secreted Phospholipase A2 - Ⅰ B, Anti - phospholipase A2 Receptor Antibody with Idiopathic Membranous Nephropathy among Adult Patients%成人特发性膜性肾病与分泌型磷脂酶A2-ⅠB及抗磷脂酶A2受体抗体的相关性研究

    Institute of Scientific and Technical Information of China (English)

    杨雪芬; 潘阳彬; 丁国华; 万建新; 马屹茕; 杨红霞

    2015-01-01

    -phospholipase A2 receptor antibody ( Anti - PLA2R AB ) with idiopathic membranous nephropathy ( IMN ) in adult patients. Methods 20 adult patients with IMN,and 10 adult patients with secondary membranous nephropathy( SMN)who were admitted to the People′s Hospital of Wuhan University and the First Affiliated Hospital of Fujian Medical University from July to December in 2013,were selected as study subjects,the diseases of cases were confirmed by renal biopsy,5 healthy volunteers were included in control group at the same time. The enrolled cases were divided into low proteinuria group(7 cases),middle proteinuria group(11 cases) and high proteinuria group(17 cases)according to 24-hour proteinuria. Serum sPLA2-ⅠB level was assessed by ELISA, the expression of sPLA2-ⅠB in kidney was detected by immunohistochemistry. Serum Anti-PLA2R AB level was detected by the indirect immunofluorescence test. Results Serum sPLA2-ⅠB level in IMN group and in SMN group was significantly higher than that in control group, respectively( P 0. 05). For IMN patients and SMN patients,the concentration of serum sPLA2-ⅠB was positively correlated with age and 24-hour proteinuria,respectively( r=0. 475,P=0. 008;r=0. 581,P=0. 002),and was negatively correlated with serum total protein and serum albumin,respectively(r= -0. 487,P=0. 009;r= -0. 473,P=0. 011). According to the results of multivariate linear regression analysis,age and 24-hour proteinuria was independently correlated with serum sPLA2-ⅠB level,respectively(P <0. 05). The concentration of serum sPLA2-ⅠB and the proportion of sPLA2-ⅠB positive area to total glomerulus area in high proteinuria group were significantly higher than those in middle proteinuria group and in low proteinuria group,respectively(P<0. 05),and the above two indicators in middle proteinuria group were significantly higher than those in low proteinuria group,respectively(P<0. 05). The expression rate of Anti-PLA2R AB in IMN group was 60%(12/20),which was

  2. Posaconazole treatment of refractory eumycetoma and chromoblastomycosis.

    Science.gov (United States)

    Negroni, Ricardo; Tobón, Angela; Bustamante, Beatriz; Shikanai-Yasuda, Maria Aparecida; Patino, Hernando; Restrepo, Angela

    2005-01-01

    Eumycetoma and chromoblastomycosis are chronic, disfiguring fungal infections of the subcutaneous tissue that rarely resolve spontaneously. Most patients do not achieve sustained long-term benefits from available treatments; therefore, new therapeutic options are needed. We evaluated the efficacy of posaconazole, a new extended-spectrum triazole antifungal agent, in 12 patients with eumycetoma or chromoblastomycosis refractory to existing antifungal therapies. Posaconazole 800 mg/d was given in divided doses for a maximum of 34 months. Complete or partial clinical response was considered a success; stable disease or failure was considered a nonsuccess. All 12 patients had proven infections refractory to standard therapy. Clinical success was reported for five of six patients with eumycetoma and five of six patients with chromoblastomycosis. Two patients were reported to have stable disease. As part of a treatment-use extension protocol, two patients with eumycetoma who initially had successful outcome were successfully retreated with posaconazole after a treatment hiatus of > 10 months. Posaconazole was well tolerated during long-term administration (up to 1015 d). Posaconazole therapy resulted in successful outcome in most patients with eumycetoma or chromoblastomycosis refractory to standard therapies, suggesting that posaconazole may be an important treatment option for these diseases.

  3. Barrier Coatings for Refractory Metals and Superalloys

    Energy Technology Data Exchange (ETDEWEB)

    SM Sabol; BT Randall; JD Edington; CJ Larkin; BJ Close

    2006-02-23

    In the closed working fluid loop of the proposed Prometheus space nuclear power plant (SNPP), there is the potential for reaction of core and plant structural materials with gas phase impurities and gas phase transport of interstitial elements between superalloy and refractory metal alloy components during service. Primary concerns are surface oxidation, interstitial embrittlement of refractory metals and decarburization of superalloys. In parallel with kinetic investigations, this letter evaluates the ability of potential coatings to prevent or impede communication between reactor and plant components. Key coating requirements are identified and current technology coating materials are reviewed relative to these requirements. Candidate coatings are identified for future evaluation based on current knowledge of design parameters and anticipated environment. Coatings were identified for superalloys and refractory metals to provide diffusion barriers to interstitial transport and act as reactive barriers to potential oxidation. Due to their high stability at low oxygen potential, alumina formers are most promising for oxidation protection given the anticipated coolant gas chemistry. A sublayer of iridium is recommended to provide inherent diffusion resistance to interstitials. Based on specific base metal selection, a thin film substrate--coating interdiffusion barrier layer may be necessary to meet mission life.

  4. IMPROVED CORROSION RESISTANCE OF ALUMINA REFRACTORIES

    Energy Technology Data Exchange (ETDEWEB)

    John P. Hurley; Patty L. Kleven

    2001-09-30

    The initial objective of this project was to do a literature search to define the problems of refractory selection in the metals and glass industries. The problems fall into three categories: Economic--What do the major problems cost the industries financially? Operational--How do the major problems affect production efficiency and impact the environment? and Scientific--What are the chemical and physical mechanisms that cause the problems to occur? This report presents a summary of these problems. It was used to determine the areas in which the EERC can provide the most assistance through bench-scale and laboratory testing. The final objective of this project was to design and build a bench-scale high-temperature controlled atmosphere dynamic corrosion application furnace (CADCAF). The furnace will be used to evaluate refractory test samples in the presence of flowing corrodents for extended periods, to temperatures of 1600 C under controlled atmospheres. Corrodents will include molten slag, steel, and glass. This test should prove useful for the glass and steel industries when faced with the decision of choosing the best refractory for flowing corrodent conditions.

  5. [Renal replacement therapy for refractory heart failure].

    Science.gov (United States)

    Schwenger, V; Remppis, A B

    2012-07-01

    After broad cardiological and nephrological evaluation and consideration of optimal conservative options according to national and international guidelines, renal replacement therapy might be helpful in patients with refractory heart failure even if they are not dialysis-dependent. This is even more important as renal failure is a strong predictor for mortality in patients with severe congestive heart failure (CHF) and CHF is one of the fastest growing morbidities in western countries. Although peritoneal dialysis (PD) is frequently used in patients with CHF its role remains unclear. Acute chronic volume overload in refractory CHF is still an unresolved clinical problem. In patients with acute heart and renal failure with need of management in an intensive care unit, extracorporeal ultrafiltration or a dialysis modality should be preferred. In patients with chronic refractory CHF, volume overload and renal failure, peritoneal dialysis should be the therapy of choice. Due to the limited data available, treatment and outcome parameters should be recorded in the registry of the German Society of Nephrology (http://www.herz-niere.de).

  6. Idiopathic juxtafoveolar retinal telangiectasis: A current review

    Directory of Open Access Journals (Sweden)

    Nowilaty Sawsan

    2010-01-01

    Full Text Available Idiopathic juxtafoveolar retinal telangiectasis (IJFT, also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

  7. Idiopathic Adrenal Hematoma Masquerading as Neoplasm

    Directory of Open Access Journals (Sweden)

    Kazuki Sasaki

    2012-04-01

    Full Text Available We report herein a case of idiopathic adrenal hematoma. A 59-year-old Japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity. The tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland. His chief complaint was weight loss of 8 kg over the previous 6 months. He had no past medical history and took no medications, including no anticoagulants. Laboratory data were almost normal except for a slight elevation of PIVKA-II. The origin of the tumor was found to be the adrenal gland, as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries. A fine needle biopsy of the lesion was unable to confirm the diagnosis. Open right adrenalectomy was performed. The histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue. In the absence of any obvious etiology, the diagnosis was idiopathic adrenal hematoma.

  8. Systemic-onset juvenile idiopathic arthritis.

    Science.gov (United States)

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition.

  9. Idiopathic pulmonary fibrosis: current and future directions.

    Science.gov (United States)

    Soo, E; Adamali, H; Edey, A J

    2017-01-21

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and is increasingly recognised. Prior to the advent of effective therapies, achieving an early diagnosis was arguably of little prognostic consequence given IPF was considered an untreatable and uniformly fatal disease. The advent of new drug treatments has given hope for the future and raised the profile of IPF. International management guidelines highlight the critical role of radiology as part of an interstitial lung disease multidisciplinary team approach in reaching an accurate and early diagnosis of IPF. The diagnostic criteria and levels of diagnostic confidence for the radio-pathological pattern associated with the clinical syndrome of IPF, usual interstitial pneumonia (UIP), appear seemingly straightforward; however, with increasing research and recognition of radiopathological interobserver variability, limitations of this classification model are becoming increasingly apparent. This review describes ancillary radiological features, comorbidities, and emerging new entities that potentially co-exist with IPF. Beyond diagnosis radiology is developing as a key prognostic tool to inform longitudinal patient evaluation. These diagnostic and prognostic clinical challenges and the future role of radiology in IPF are discussed.

  10. RADIOFREQUENCY ABLATION OF IDIOPATHIC RIGHT VENTRICULAR TACHYCARDIA

    Institute of Scientific and Technical Information of China (English)

    华伟; JituVohra

    1998-01-01

    This paper presents our experieaee with radioreqencey ablation (RFA) for idiopathic ventricular tschycardia (VT) arising from right ventricle in 12 patients(pts). The age range d patients was 21~50, with a mean of 38. 5 years. Ten out of 12 were females, 1 patient had eandia failure due to almost incessant VT while the rest had normal left ventricular function.Twelve pts had VT arising from the fight ventricle; of those, 9 were from the outflow truct, 2 from the RV apex, and l from the RV inflow. In all tats the diagnostic study and therapeutic RFA were combined in a single procedure, pacemapping and local aetlvition time were used to guide the site of RFA in Ors with VT arising froth the tight ventricle.RFA was successful in 11 of the 12 pts ( 91%). Ntmaher of RF applications were 1~27, mean 9. 6; fluoroscopy time were 4~75, mean 26. 9 minutes. RFA for idiopathic RV has a high success rate. This mode of treament should be considered as a nonphartaaeologieal curative treatment for symptomatic pts.

  11. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    Science.gov (United States)

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  12. Idiopathic membranous nephropathy: an autoimmune disease.

    Science.gov (United States)

    Makker, Sudesh P; Tramontano, Alfonso

    2011-07-01

    For more than 50 years researchers have debated the evidence for an autoimmune basis of human idiopathic membranous nephritis (MN). Work published in the past 2 years has substantially strengthened the belief that MN is indeed an autoimmune disease of the kidney. Autoantibodies of the IgG4 subclass to at least three podocyte membrane proteins including phospholipase A(2)-receptor, aldose reductase, and manganese superoxide dismutase have been detected by immunoblotting in sera as well as in acid eluates prepared from renal biopsy tissue of patients with this disease, using either whole tissue or microdissected glomeruli from frozen sections. In each case the podocyte antigen has been shown to co-localize with the subepithelial glomerular immune deposits in renal tissue of the same patients. It is not certain if any of these podocyte proteins is an inciting/primary autoantigen or whether they are secondary antigens recruited by intermolecular epitope-spreading, initiating from a yet-to-be-discovered autoantigen. Although it is clear that autoantibodies to podocyte membrane proteins are elicited in idiopathic MN and contribute to the formation of the subepithelial deposits, many questions remain concerning the triggers for their development and their contribution toward proteinuria and progression of the disease.

  13. Idiopathic spontaneous pneumomediastinum: radiologic and clinical features

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Young; Kim, Su Young; Kim, Yong Hoon; Hwang, Yoon Joon; Seo, Jung Wook; Han, Yoon Hee; Cha, Soon Joo; Hur, Gham [College of Medicine, Inje Univ., Goyang (Korea, Republic of)

    2004-07-01

    To evaluate the clinical presentations, radiological characteristics, and natural history of healthy adolescents presenting with idiopathic spontaneous pneumomediastinum. We retrospectively reviewed the simple radiographs of 14 consecutive patients (11 males) with spontaneous pneumomediastinum, who were examined over a period of 8 years, and analyzed their clinical history, radiographic findings including distribution, combined subcutaneous emphysema, mediastinal widening, pneumothorax, pleural effusion, and resolving period on follow up chest radiographs. We also obtained CT images of 7 patients for the assessment of additional information. The most common complaint at the time of presentation was chest pain and chest discomfort (8/14), followed by neck discomfort (6/14). The chest radiograph was of diagnostic value in alI cases. The main distribution of the pneumomediastinum was cervical (14/14), upper lung (13/14) and lower lung (6/14). Combined subcutaneous emphysema was observed in 6 patients. However, there were no cases of mediastinal widening, pneumothorax or pleural effusion. Complete resolution of the pneumomediastinum on the radiograph was observed after 10 days (mean 5.6), following purely conservative treatment. There was no additional information on the CT images, as compared with that on the radiographs. Idiopathic spontaneous pneumomediastinum is a benign entity that usually goes undiagnosed, but which responds very weIl to conservative treatment.

  14. Acute and subacute idiopathic interstitial pneumonias.

    Science.gov (United States)

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.

  15. Managing idiopathic short stature: role of somatropin (rDNA origin for injection

    Directory of Open Access Journals (Sweden)

    J Paul Frindik

    2010-06-01

    Full Text Available J Paul Frindik1, Stephen F Kemp11University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, AR, USAAbstract: Idiopathic short stature (ISS is a term that describes short stature in children who do not have growth hormone (GH deficiency and in whom the etiology of the short stature is not identified. Between 1985 and 2000, more than 40 studies were published regarding GH therapy for ISS. Only 12 of these had data to adult height, of which only 4 were controlled studies. A subsequent placebo-controlled study that followed subjects to adult height indicated that there was a gain of 3.7–7.5 cm in height with GH treatment. In 2003, the US Federal Drug Administration (FDA approved GH for treatment of short stature. Even before FDA approval, patients with ISS made up about 20% of patients in GH databases, which is largely unchanged since FDA approval. There remains some controversy as to whether GH should be used to treat ISS. This controversy centers on the fact that there has been no definitive demonstration that short stature results in a disadvantage or problems with psychological adjustment, and thus, no demonstration that GH therapy results in improvement in quality of life.Keywords: idiopathic short stature, ISS, growth hormone therapy, somatotropin, somatropin, insulin-like growth factor I, IGF-1

  16. Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

    Directory of Open Access Journals (Sweden)

    Mercier Pierre

    2009-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis is characterized by a three-dimensional deviation of the vertebral column and its etiopathogenesis is unknown. Various factors cause idiopathic scoliosis, and among these a prominent role has been attributed to the vestibular system. While the deficits in sensorimotor transformations have been documented in idiopathic scoliosis patients, little attention has been devoted to their capacity to integrate vestibular information for cognitive processing for space perception. Seated idiopathic scoliosis patients and control subjects experienced rotations of different directions and amplitudes in the dark and produced saccades that would reproduce their perceived spatial characteristics of the rotations (vestibular condition. We also controlled for possible alteration of the oculomotor and vestibular systems by measuring the subject's accuracy in producing saccades towards memorized peripheral targets in absence of body rotation and the gain of their vestibulo-ocular reflex. Results Compared to healthy controls, the idiopathic scoliosis patients underestimated the amplitude of their rotations. Moreover, the results revealed that idiopathic scoliosis patients produced accurate saccades to memorized peripheral targets in absence of body rotation and that their vestibulo-ocular reflex gain did not differ from that of control participants. Conclusion Overall, results of the present study demonstrate that idiopathic scoliosis patients have an alteration in cognitive integration of vestibular signals. It is possible that severe spine deformity developed partly due to impaired vestibular information travelling from the cerebellum to the vestibular cortical network or alteration in the cortical mechanisms processing the vestibular signals.

  17. Splenectomy in patients with idiopathic thrombocytopenic purpura: Analysis of 109 cases

    Directory of Open Access Journals (Sweden)

    Enver Ay

    2012-03-01

    Full Text Available Objectives: Splenectomy is performed in order to provide the treatment in the patients with severe idiopathic thrombocytopenic purpura, refractory to medical treatment. In this study, we aimed to investigate the postoperatif and longterm outcomes in the patients who underwent splenectomy with the diagnosis of idiopathic thrombocytopenic purpura.Materials and Methods: Between 2001-2010 at Dicle University Medical Faculty, General Surgery Department, a retrospective review of the 109 patients who had undergone splenectomy for ITP was reviewed. Age, gender, presence of accessory spleens and location, duration of the operation, number of preoperative platelet tranfusion, number of preoperative and postoperative blood transfusion, length of hospital stay, long-term outcomes, morbidity and mortality were recorded.Results: The mean age was 37.10 ± 16.62 (16-72, and there were 88 (80.7% female and 21 (19.3% male patients. The mean operation time was 44.87 ± 10:32 (30-120 minutes. The average postoperative blood and preoperative platelet transfusion were 1.63 ± 0.85 (0-3 and 2.01 ± 0.71 (1-3 units, respectively. The accessory spleens were encountered in 20 (18.3% patients at the ultrasonographic examination. And also the accessory spleens were encountered in 23 (21.1% patients during operation and confirmed with histopathologic examination. The most common localization of accessory spleens were splenic hilus. The postoperative complications were occurred in 16 patients (14.7% and the most complication was atelectasia. The mean length of hospital stay was 4:56 ± 2:45 (2-12 days. Patients were followed for an average of 28 (9-48 months. At the follow-up period, 1 (0.9 % patient had died.Conclusion: Splenectomy can be performed safely in the treatment of the patients with idiopathic thrombocytopenic purpura unresponsive to medical treatment. Long-term good results can be obtained with splenectomy in these patients. The accessory spleens should not be

  18. Comprehensive Creep and Thermophysical Performance of Refractory Materials

    Energy Technology Data Exchange (ETDEWEB)

    Ferber, M.K.; Wereszczak, A.; Hemrick, J.A.

    2006-06-29

    Furnace designers and refractory engineers recognize that optimized furnace superstructure design and refractory selection are needed as glass production furnaces are continually striving toward greater output and efficiencies. Harsher operating conditions test refractories to the limit, while changing production technology (such as the conversion to oxy-fuel from traditional air-fuel firing) can alter the way the materials perform [1-3]. Refractories for both oxy- and air-fuel fired furnace superstructures (see Fig. 1) are subjected to high temperatures that may cause them to creep excessively or subside during service if the refractory material is not creep resistant, or if it is subjected to high stress, or both. Furnace designers can ensure that superstructure structural integrity is maintained if the creep behavior of the refractory material is well understood and well represented by appropriate engineering creep models. Several issues limit the abilities of furnace designers to (1) choose the optimum refractory for their applications, (2) optimize the engineering design, or (3) predict the service mechanical integrity of their furnace superstructures. Published engineering creep data are essentially nonexistent for almost all commercially available refractories used for glass furnace superstructures. The limited data that do exist are supplied by the various refractory suppliers. Unfortunately, the suppliers generally have different ways of conducting their mechanical testing, and they interpret and report their data differently. This inconsistency makes it hard for furnace designers to draw fair comparisons between competing grades of candidate refractories. Furthermore, the refractory suppliers' data are often not available in a form that can be readily used for furnace design or for the prediction and design of long-term structural integrity of furnace superstructures. As a consequence, the U.S. Department of Energy (DOE) Industrial Technology Program

  19. 利妥昔单抗治疗糖皮质激素无效的特发性血小板减少性紫癜疗效观察%Rituximab treatment for adults with steroid-resistant idiopathic thrombocytopenic purpura

    Institute of Scientific and Technical Information of China (English)

    王文; 俞庆宏; 张海燕; 初晓霞; 陈峰; 张春青; 周郁鸿; 侯明

    2008-01-01

    Objective To investigate the efficacy and safety as well as the effects of rituximab on B-lymphocytes and anti-platelet glycoprotein-specific antibodies,in patients with steroid-resistant idiopathic thrombocytopenic purpura(ITP).Methods Twelve steroid-resistant ITP patients,16 to 54 years old,received intravenous rituximab at the dose of 375 mg/m2 once-weekly for 4 weeks.Lab studies included CBC,serum concentrations of IgG,IgM and IgA.CD+3,CD+4,CD+8,CD+19,CD+20 cell numbers were assayed by flow cytometry and anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)were assayed by monoclonal antibody-specific immobilisation of platelet antigens prior to and following rituximab therapy.Results A complete response(platelet counts ≥100×109/L)was observed in 4 cases,a partial response (platelet counts between 50 and 100×109/L)in 3 cases,a minor response(platelet counts between 30 and 50×109/L)in 2 cases,and non response(platelet counts<30×109/L)in 3 cases.Responses were sustained 0.5 to 12 months(median 5 months).After 4 weeks of rituximab therapy,anti-platelet glycoprotein-specific antibodies(GP Ⅱ b/Ⅲ a,GP Ⅰ b/Ⅸ)disappeared except one NR patient and CD+19/CD+20 cells were almost depleted in all patients(295.0±86.4)×106/L vs(4.1±2.2)×106/L(P<0.01).As expected,the T cell counts,and the serum concentrations of IgG,IgM and IgA were not changed after therapy.No severe side effects were observed.Conclusion Rituximab may be an effective and safe treatment for adults with steroid-resistant ITP.%目的 探讨利妥昔单抗治疗特发性血小板减少性紫癜(ITP)的疗效、安全性及治疗前后B细胞、血小板膜糖蛋白(GP)特异性自身抗体的变化.方法 利妥昔单抗(375 mg/m2,每周1次,连用4周)治疗12例糖皮质激素治疗无效的ITP患者,监测治疗前后的血常规、血清免疫球蛋白定量(IgG、IgM、IgA)、血小板GPⅡb/Ⅲa和(或)GP Ⅰ b/Ⅸ特异性自身抗体、CD+3、CD+4、CD+8、CD+19

  20. 血清抗M型磷脂酶A2受体抗体对成年人特发性膜性肾病诊治的指导作用%Role of M _ type Phospholipase A2 Receptor Antibody in the Diagnosis and Treatment of Idiopathic Membranous Nephropathy in Adults

    Institute of Scientific and Technical Information of China (English)

    鲁欢; 汤水福; 陈刚毅; 苏保林; 吴兴波; 王超; 涂海涛; 罗月中

    2016-01-01

    Objective To evaluate the role of M _ type phospholipase A2 receptor antibody( anti-PLA2R) in the diagnosis and treatment of idiopathic membranous nephropathy(IMN)in adults. Methods From January 2014 to May 2015, we enrolled 58 patients who were definitely diagnosed with glomerular disease by renal biopsy in the First Affiliated Hospital of Guangzhou University of Chinese Medicine. Among them,there were 31 IMN patients(IMN group)and 27 non _ IMN patients (non _ IMN group). ELISA method was used to detect serum anti-PLA2R,in order to determine anti-PLA2R was positive or negative. Comparison was made between IMN group and non _ IMN group in the general data,laboratory examination indexes〔24 h UTP,Alb,Scr,urine RBC( HPF),UA,PT,INR,APTT and FIB〕,pathological indexes( mesangial proliferation, crescent formation,glomerulosclerosis,tubular atrophy/ fibrosis,renal interstitial inflammatory cell infiltration,renal interstitial vascular disease and immunofluorescence deposition) and clinical outcomes and the same comparison was made between anti-PLA2R positive and anti-PLA2R negative. Results In IMN group,there were 21 patients with anti-PLA2R positive with a positive rate of 67. 7%. In non _ IMN group,there was one patient with anti-PLA2R positive with a positive rate of 3. 7% . IMN group was higher in age,the optical density of serum anti-PLA2R and lower in urine RBC(HPF),PT,INR and APTT than non _ IMN group(P 0. 05). Patients with serum anti-PLA2R positive were higher than patients with anti-PLA2R negative in the optical density of serum anti-PLA2R(P 0. 05). Spearman correlation analysis showed that the optical density of serum anti-PLA2R had no correlation with MAP,UTP,Alb and Scr(rs = 0. 097, _ 0. 208,0. 266, _ 0. 358;P > 0. 05). Patients with anti-PLA2R positive and patients with anti-PLA2R negative were not significantly different in mesangial proliferation,crescent formation, glomerulosclerosis,tubular atrophy / fibrosis,renal interstitial inflammatory cell

  1. Zonisamide Efficacy as Adjunctive Therapy in Children With Refractory Epilepsy

    Directory of Open Access Journals (Sweden)

    Parvaneh KARIMZADEH

    2013-06-01

    . Healthcare, Philadelphia (2002, pp. 873-879.16. Baulac M. Introduction to zonisamide. Epilepsy Res2006 Feb;68 (Suppl 2:S3-9. 17. Baulac M, Ilo E. Leppik. Efficacy and safety of adjunctive zonisamide therapy for refractory partial seizures. Epilepsy Research 2007;75:75-83. 18. Coppola G, Grosso S, Verrotti A, Parisi P, Luchetti A,Franzoni E, et al. Zonisamide in children and young adults with refractory epilepsy: An open label, multicenter Italian study. Epilepsy Research 2009;83:112-116.19. Tan HJ, Martland TR, Appleton RE, Kneen R. Effectiveness and tolerability of zonisamide in children with epilepsy: A retrospective review. Seizure 2010;19:31-35.20. Stephen LJ, Kelly K, Wilson EA, Parker P, Brodie MJ. A prospective audit of adjunctive zonisamide in an everyday clinical setting. Epilepsy Behav 2010 Apr;17(4:455-60.21. Catarino CB, Bartolini E, Bell GS, Yuen AWC, Duncan JS, Sander JW.The long-term retention of zonisamide in a large cohort of people with epilepsy at a tertiary referral centre. Epilepsy Research 2011;96:39-44.22. Loscher W, Schmidt D. Experimental and clinical evidence for loss of effect(tolerance during prolonged treatment with antiepileptic drugs. Epilepsia 2006;47(8:1253-84.23. Eun SH, Kim HD, Eun BL, Lee IK, Chung HJ, Kim JS, et al. Comparative trial of low- and high-dose zonisamide as monotherapy for childhood epilepsy.Seizure 2011;20(7:558-63.

  2. Ipsilateral idiopathic gingival enlargement and it's management using conventional gingivectomy and diode laser: A recurrent case after 15 years.

    Science.gov (United States)

    Devi, Potharaju Kamala; Kumar, Gudi Pavan; Bai, Yendluri Durga; Ammaji, Annamdevula Durga

    2013-05-01

    Idiopathic gingival fibromatosis is a relatively rare condition characterized by the proliferation of the gingival tissues resulting in masticatory, esthetics, phonetics and psychological disturbances. The severity of the overgrowth can range from a solitary isolated mass to a more generalized and diffused enlargement. The etiopathogenesis of this bizarre condition is poorly understood and has been attributed to various factors. It can present as a single disorder or may manifest as part of a syndrome. This case reports an ipsilateral diffused idiopathic gingival enlargement in a middle aged adult recurring after a gap of 15 years. External bevel gingivectomy on the buccal aspects of maxillary and mandibular gingiva and diode laser for excision of the enlarged tissue on the lingual/palatal aspect was carried out to eliminate the excessive tissue. Periodic recalls showed maintenance of good oral hygiene and 1 year follow-up revealed no recurrence.

  3. Idiopathic Calcium Nephrolithiasis And Hypercalciuria: The Role Of Genes

    Science.gov (United States)

    Gambaro, Giovanni; Abaterusso, Cataldo

    2007-04-01

    Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.

  4. Export and Import Situation of Refractories and Refractory Raw Materials in China

    Institute of Scientific and Technical Information of China (English)

    PANShangxin; ZHANGZhongqi; 等

    2000-01-01

    The article states China is a great nation in producing and exporting refractories and refractory raw materials,which, has earned much foreign exchange for our country ,only a few advanced refactory material, zircon and chrome ore are needed to be imported.The ar-ticle points out the problems in the export and import,and raises suggestions on development.After the entry of WTO,China will expand its co-operations with other countries in science,technology,economy and trade to play a more important role in the world market.

  5. Development and Application of Refractory Materials for Molten Aluminum Applications

    Energy Technology Data Exchange (ETDEWEB)

    Hemrick, James Gordon [ORNL; Headrick, William [University of Missouri, Rolla; Peters, Klaus-Markus [ORNL

    2008-01-01

    Two new refractory materials have been developed for use in molten aluminum contact applications which exhibit improved corrosion and wear resistance, along with improved thermal management through reduced heat losses. The development of these materials was based on understanding of the corrosion and wear mechanisms associated with currently used aluminum contact refractories through physical, chemical, and mechanical characterization and analysis performed by Oak Ridge National Laboratory (ORNL) and the University of Missouri, Rolla (UMR) along with their industrial partners, under the ITP Materials project "Multifunctional Metallic and Refractory Materials for Energy Efficient Handling of Molten Metals". Spent castable refractories obtained from a natural gas fired reverberatory aluminum alloy melting furnace were analyzed leading to identification of several refractory degradation mechanisms and strategies to produce improved materials. The newly developed materials have been validated through both R&D industrial trials and independent commercial trials by the refractory manufacturers.

  6. Technology Progress of Refractories for Iron Making in China

    Institute of Scientific and Technical Information of China (English)

    CHAI Junlan; CHENG Qingxian; SHEN Keyin

    2005-01-01

    This paper reviews the present development situation of refractories for iron making system in China, which includes blast furnace, hot blast stove and coke dry quenching, etc. Varieties and performances of refractories used by the top steel and iron groups such as Baosteel, Wuhan I&S Group and Anshan I&S Group for iron making were exemplified and concerned problems were analyzed,together with solutions and suggestions for future refractories R&D work.

  7. Handbook of industrial refractories technology principles, types, properties and applications

    CERN Document Server

    Caniglia, Stephen

    1989-01-01

    Encompasses the entire range of industrial refractory materials and forms: properties and their measurement, applications, manufacturing, installation and maintenance techniques, quality assurance, and statistical process control.

  8. Refractory Coated/Lined Low Density Structures Project

    Data.gov (United States)

    National Aeronautics and Space Administration — This project addresses the development of refractory coated or lined low density structures applicable for advanced future propulsion system technologies. The...

  9. Properties of Refractory Concrete in Tension and Compression

    Science.gov (United States)

    Sampson, Jeffrey

    2009-01-01

    Refractory concrete on the LC-39A Flame Deflector has been damaged during multiple Space Shuttle launches (e.g. STS-124, STS-126, STS-119, and STS-125, STS-127). These events have prompted a better understanding of the system via an analytical model of the Flame Deflector assembly to include the Fondu Fyre refractory concrete. This model requires test data inputs of the refractory concrete's mechanical properties, which include stress versus strain curves in tension and compression, modulus of elasticity, and Poisson's ratio. Sections of Fondu Fyre refractory concrete removed from the LC-39A Flame Deflector were provided for this testing.

  10. Bendamustine Plus Alemtuzumab for Refractory Chronic Lymphocytic Leukemia (CLL)

    Science.gov (United States)

    2013-08-20

    Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Stage III Chronic Lymphocytic Leukemia; Stage III Small Lymphocytic Lymphoma; Stage IV Chronic Lymphocytic Leukemia; Stage IV Small Lymphocytic Lymphoma

  11. Report of a family with idiopathic knuckle pads and review of idiopathic and disease-associated knuckle pads.

    Science.gov (United States)

    Hyman, Charles H; Cohen, Philip R

    2013-05-15

    Knuckle pads are a rare, frequently overlooked, thickening of the skin usually overlying the extensor surface of the proximal interphalangeal joints. They are well- circumscribed, benign lesions that generally do not require treatment. Idiopathic knuckle pads must be differentiated from similar appearing lesions or trauma-induced pseudo-knuckle pads. Knuckle pads have been observed in association with autosomal dominant conditions such as Bart-Pumphrey syndrome, Dupuytren's contracture, Ledderhose disease, and Peyronie's disease. To the best of our knowledge, idiopathic familial knuckle pads have not previously been described in the English language literature. We describe a sister and brother with familial idiopathic knuckle pads with no associated conditions.

  12. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

  13. Bulk Vitrification Castable Refractory Block Protection Study

    Energy Technology Data Exchange (ETDEWEB)

    Hrma, Pavel R.; Bagaasen, Larry M.; Beck, Andrew E.; Brouns, Thomas M.; Caldwell, Dustin D.; Elliott, Michael L.; Matyas, Josef; Minister, Kevin BC; Schweiger, Michael J.; Strachan, Denis M.; Tinsley, Bronnie P.; Hollenberg, Glenn W.

    2005-05-01

    Bulk vitrification (BV) was selected for a pilot-scale test and demonstration facility for supplemental treatment to accelerate the cleanup of low-activity waste (LAW) at the Hanford U.S. DOE Site. During engineering-scale (ES) tests, a small fraction of radioactive Tc (and Re, its nonradioactive surrogate) were transferred out of the LAW glass feed and molten LAW glass, and deposited on the surface and within the pores of the castable refractory block (CRB). Laboratory experiments were undertaken to understand the mechanisms of the transport Tc/Re into the CRB during vitrification and to evaluate various means of CRB protection against the deposition of leachable Tc/Re. The tests used Re as a chemical surrogate for Tc. The tests with the baseline CRB showed that the molten LAW penetrates into CRB pores before it converts to glass, leaving deposits of sulfates and chlorides when the nitrate components decompose. Na2O from the LAW reacts with the CRB to create a durable glass phase that may contain Tc/Re. Limited data from a single CRB sample taken from an ES experiment indicate that, while a fraction of Tc/Re is present in the CRB in a readily leachable form, most of the Tc/Re deposited in the refractory is retained in the form of a durable glass phase. In addition, the molten salts from the LAW, mainly sulfates, chlorides, and nitrates, begin to evaporate from BV feeds at temperatures below 800 C and condense on solid surfaces at temperatures below 530 C. Three approaches aimed at reducing or preventing the deposition of soluble Tc/Re within the CRB were proposed: metal lining, sealing the CRB surface with a glaze, and lining the CRB with ceramic tiles. Metal liners were deemed unsuitable because evaluations showed that they can cause unacceptable distortions of the electric field in the BV system. Sodium silicate and a low-alkali borosilicate glaze were selected for testing. The glazes slowed down molten salt condensate penetration, but did little to reduce the

  14. Launch Pad Flame Trench Refractory Materials

    Science.gov (United States)

    Calle, Luz M.; Hintze, Paul E.; Parlier, Christopher R.; Bucherl, Cori; Sampson, Jeffrey W.; Curran, Jerome P.; Kolody, Mark; Perusich, Steve; Whitten, Mary

    2010-01-01

    The launch complexes at NASA's John F. Kennedy Space Center (KSC) are critical support facilities for the successful launch of space-based vehicles. These facilities include a flame trench that bisects the pad at ground level. This trench includes a flame deflector system that consists of an inverted, V-shaped steel structure covered with a high temperature concrete material five inches thick that extends across the center of the flame trench. One side of the "V11 receives and deflects the flames from the orbiter main engines; the opposite side deflects the flames from the solid rocket boosters. There are also two movable deflectors at the top of the trench to provide additional protection to shuttle hardware from the solid rocket booster flames. These facilities are over 40 years old and are experiencing constant deterioration from launch heat/blast effects and environmental exposure. The refractory material currently used in launch pad flame deflectors has become susceptible to failure, resulting in large sections of the material breaking away from the steel base structure and creating high-speed projectiles during launch. These projectiles jeopardize the safety of the launch complex, crew, and vehicle. Post launch inspections have revealed that the number and frequency of repairs, as well as the area and size of the damage, is increasing with the number of launches. The Space Shuttle Program has accepted the extensive ground processing costs for post launch repair of damaged areas and investigations of future launch related failures for the remainder of the program. There currently are no long term solutions available for Constellation Program ground operations to address the poor performance and subsequent failures of the refractory materials. Over the last three years, significant liberation of refractory material in the flame trench and fire bricks along the adjacent trench walls following Space Shuttle launches have resulted in extensive investigations of

  15. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.; Colby, Thomas V.; Egan, Jim J.; Hansell, David M.; Johkoh, Takeshi; Kaminski, Naftali; Kim, Dong Soon; Kondoh, Yasuhiro; Lynch, David A.; Müller-Quernheim, Joachim; Myers, Jeffrey L.; Nicholson, Andrew G.; Selman, Moisés; Toews, Galen B.; Wells, Athol U.; Martinez, Fernando J.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed. PMID:17585107

  16. Proton MR spectroscopy in idiopathic spasmodic torticollis

    Energy Technology Data Exchange (ETDEWEB)

    Federico, F.; Lucivero, V.; Simone, I.L.; Defazio, G.; De Salvia, R.; Mezzapesa, D.M.; Petruzzellis, M.; Tortorella, C.; Livrea, P. [Dept. of Neurology and Psychiatry, Bari (Italy)

    2001-07-01

    Single-voxel proton magnetic resonance spectroscopy ({sup 1}H-MRS), localised to the basal ganglia, was used to determine changes in metabolite levels in idiopathic spasmodic torticollis (IST). We examined nine patients and 13 healthy subjects. The mean values ({+-} SD) of peak area ratios were: IST: N-acetyl-aspartate (NAA)/choline-containing compounds (Cho) 1.79 {+-} 0.39, NAA/creatine and phosphocreatine compounds (Cr) 1.61 {+-} 0.38, Cho/Cr 0.91 {+-} 0.19; controls: NAA/Cho 2.07 {+-} 0.35, NAA/Cr 1.82 {+-} 0.31, Cho/Cr 0.89 {+-} 0.12. Statistical analysis showed that NAA/Cho and NAA/Cr were significantly lower in patients than in controls (P = 0.0304 and 0.0431, respectively). These results indicate a reduction in NAA, and suggest striatal involvement in the pathogenesis IST. (orig.)

  17. The prognosis of idiopathic generalized epilepsy.

    Science.gov (United States)

    Seneviratne, Udaya; Cook, Mark; D'Souza, Wendyl

    2012-12-01

    Prognosis describes the trajectory and long-term outcome of a condition. Most studies indicate a better prognosis in idiopathic generalized epilepsy (IGE) in comparison with other epilepsy syndromes. Studies looking at the long-term outcome of different IGE syndromes are relatively scant. Childhood absence epilepsy appears to have a higher rate of remission compared to juvenile absence epilepsy. In absence epilepsies, development of myoclonus and generalized tonic-clonic seizures predicts lower likelihood of remission. Although most patients with juvenile myoclonic epilepsy (JME) achieve remission on antiepileptic drug therapy, remission without treatment. Data on the prognosis of other IGE syndromes are scarce. There are contradictory findings reported on the value of electroencephalography as a predictor of prognosis. Comparisons are made difficult by study heterogeneity, particularly in methodology and diagnostic criteria.

  18. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    Directory of Open Access Journals (Sweden)

    Michelle Fog Andersen

    2015-01-01

    Full Text Available Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting.

  19. Diffuse Idiopathic Skeletal Hyperosteosis: A Review

    Directory of Open Access Journals (Sweden)

    Sevgi İkbali Afşar

    2015-12-01

    Full Text Available Diffuse idiopathic skeletal hyperosteosis (DISH is also known as Forestier disease and is a systemic non-inflammatory disorder seen more commonly in males and elderly. It is characterized by calcification of the anterior longitudinal ligament of the vertebral column and various extraspinal ligaments. It is usually asymptomatic while the most common symptoms are spinal pain, limited range of spinal motion and dysphagia due to esophagus compression. The etiopathogenesis is not clear. It is commonly seen together with diabetes mellitus, obesity, hyperinsulinemia, hypertension and lipid and purine metabolism disorders, indicating an association with metabolic disorders. Recent studies have emphasized that the pathological calcification of the anterior longitudinal ligament plays a role in the pathophysiology. The aim of this study was to summarize new pathogenetic, clinical and therapeutic insights of this disease, based on published literature.

  20. Idiopathic gynaecomastia in twins: a case report

    Directory of Open Access Journals (Sweden)

    S.V. Kumar

    2014-08-01

    Full Text Available Gynaecomastia is a common pathological condition of breast seen in men. This is defined as benign enlargement of breast tissue in males. It was first described by Palus Aegineta (AD 635-690. Herewith we are presenting cases of 14 year old twins attending the surgical O.P.D with bilateral enlarged breasts and pain. A diagnosis of pubertal gynaecomastia was made with no evidence of malignancy by histopathological examinations and mammography. One boy with 5 x 6 cm of right breast and 2 x3 cm of left breast was treated by subcutaneous mastectomy. Other boy had only enlargement of nipple and areola. He was reassured and sent home without surgical intervention. A rare case of idiopathic gynaecomastia in pubertal twins is described here. [Int J Res Med Sci 2014; 2(4.000: 1755-1757

  1. Psychological treatment of psychogenic idiopathic environmental intolerance.

    Science.gov (United States)

    Staudenmayer, H

    2000-01-01

    This chapter focuses on the psychotherapy of individuals who suffer distress from functional somatic syndromes; specifically, idiopathic environmental intolerance (IEI). While patients believe environmental intolerances cause their distress, its origin is treated as psychological, mediated through psychophysiological systems and mechanisms associated with the stress response. Factors considered include stress and trauma premorbid to the alleged onset of IEI; somatization and its expression through affective, anxiety, and somatoform disorders; personality disorders and associated psychological defenses; motivation for the sick role; and iatrogenic suggestion and reinforcement of unsubstantiated toxicogenic theories and treatments. Psychotherapies include behavioral desensitization, cognitive-behavioral therapy, cognitive therapy, and psychotropic medications. The greatest challenge in treatment is to overcome the patient's disabling belief in a toxicogenic explanation for his or her symptoms.

  2. Clinical Orofacial Examination in Juvenile Idiopathic Arthritis

    DEFF Research Database (Denmark)

    Stoustrup, Peter; Twilt, Marinka; Spiegel, Lynn

    2017-01-01

    review. The level of evidence for the 5 recommendations was derived primarily from descriptive studies, such as cross-sectional and case-control studies. CONCLUSION: Five recommendations are proposed for the orofacial examination of patients with JIA to improve the clinical practice and aid standardized......OBJECTIVE: To develop international consensus-based recommendations for the orofacial examination of patients with juvenile idiopathic arthritis (JIA), for use in clinical practice and research. METHODS: Using a sequential phased approach, a multidisciplinary task force developed and evaluated...... a set of recommendations for the orofacial examination of patients with JIA. Phase 1: A Delphi survey was conducted among 40 expert physicians and dentists with the aim of identifying and ranking the importance of items for inclusion. Phase 2: The task force developed consensus about the domains...

  3. Idiopathic sclerosing encapsulating peritonitis: Abdominal cocoon

    Institute of Scientific and Technical Information of China (English)

    Jenny N Tannoury; Bassam N Abboud

    2012-01-01

    Abdominal cocoon,the idiopathic form of sclerosing encapsulating peritonitis,is a rare condition of unknown etiology that results in an intestinal obstruction due to total or partial encapsulation of the small bowel by a fibrocollagenous membrane.Preoperative diagnosis re quires a high index of clinical suspicion.The early clinical features are nonspecific,are often not recognized and it is difficult to make a definite pre-operative diagnosis.Clinical suspicion may be generated by the recurrent episodes of small intestinal obstruction combined with relevant imaging findings and lack of other plausible etiologies.The radiological diagnosis of abdominal cocoon may now be confidently made on computed tomography scan.Surgery is important in the management of this disease.Careful dissection and excision of the thick sac with the release of the small intestine leads to complete recovery in the vast majority of cases.

  4. Idiopathic intracranial hypertension is not benign

    DEFF Research Database (Denmark)

    Yri, Hanne M; Wegener, Marianne; Sander, Birgit

    2012-01-01

    Idiopathic intracranial hypertension (IIH) primarily affects young obese females, and potentially causes visual loss and severe headache. The aim of this experiment is to examine relapse rate and long-term outcome in IIH patients. The methods involved in this experiment include a prospective...... controlled study of 18 newly diagnosed IIH patients followed for a mean observation period of 21.1 (±8.0) months. Treatment regime included diuretics, dietary recommendations and check-up visits at a dietician. Baseline and follow-up included neurological examination, detailed headache history...... atrophy was clinically detectable in only one patient. Headache was still present in 67% of the patients at follow-up. Headache was heterogenic and unrelated to relapse. After an initial reduction, weight increased again in the relapse group compared to reduced weight in the non-relapse group (p = 0...

  5. Malignant Hyperthermia and Idiopathic HyperCKemia

    Directory of Open Access Journals (Sweden)

    Pashtoon Murtaza Kasi

    2011-01-01

    Full Text Available Malignant hyperthermia (MH is a rare but life-threatening condition that is more frequently encountered and discussed within the anesthesia literature. Here we through a case specifically discuss the susceptibility of individuals and/or families with asymptomatic unexplained elevations of creatine kinase (CK, also frequently referred to as hyperCKemia or idiopathic hyperCKemia (IHCK in recent reports. The clinical implications would be to underscore the importance of this as a susceptibility to developing MH and highlight the importance of genetic susceptibility testing in such cases. Anesthesiologists and critical care intensivists as well as primary care physicians should keep this in mind when seeing patients with asymptomatic hyperCKemia and potentially inform them about the possibility of developing MH if exposed to triggering agents. Genetic susceptibility testing should be considered if available and family members should also receive nontriggering agents when undergoing anesthesia and wear Medic Alert tags.

  6. Imaging findings in idiopathic pelvic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Wiesner, W.; Bongartz, G. [Inst. of Diagnostic Radiology University Hospital Basel (Switzerland); Stoffel, F. [Inst. of Urology, University Hospital Basel (Switzerland)

    2001-04-01

    Two patients presented with ureteric obstruction, and voiding symptoms and constipation, respectively, and were examined by means of intravenous urography and computed tomography. One patient was additionally examined by means of MR tomography. After CT (performed in both patients) and MRT (performed in one patient) had shown a diffuse, contrast-enhancing, infiltrating process in the small pelvis with infiltration of adjacent organs and vessels, surgical biopsy proved the diagnosis of idopathic pelvic fibrosis. Extension of retroperitoneal fibrosis below the pelvic rim is very rare. Clinical symptoms of pelvic fibrosis are variable and imaging findings may lead to a broad list of differential diagnoses. We present two patients with idiopathic pelvic fibrosis and discuss radiological findings and differential diagnoses of this rare disease. (orig.)

  7. Idiopathic Paroxysmal Ventricular Tachycardia in Infants and Children

    Science.gov (United States)

    Hernandez, Antonio; And Others

    1975-01-01

    Laboratory tests including blood count serum electrolyte measures, and electroencephalograms were performed on seven children ages 1 day to 18 years with recurrent attacks of rapid heart action known as idiopathic paroxysmal ventricular tachycardia. (CL)

  8. CLINICAL HISTORY AND OUTCOME OF 59 PATIENTS WITH IDIOPATHIC HYPERPROLACTINEMIA

    NARCIS (Netherlands)

    SLUIJMER, AV; LAPPOHN, RE

    1992-01-01

    Objective: To investigate the clinical course of hyperprolactinemia without demonstrable cause. Design: Prospective study of all patients with idiopathic hyperprolactinemia first seen between 1974 and 1985. Setting: Outpatient Department of University Hospital. Patients: Fifty-nine patients followed

  9. [Idiopathic epiretinal membrane: definition, classification, current understanding of pathogenesis].

    Science.gov (United States)

    Ponomareva, E N; Kazarian, A A

    2014-01-01

    Idiopathic epiretinal membrane is a result of a complex biomechanical interaction of the retina and vitreous. This paper discusses classification problems, epidemiological data of multicenter studies, and current hypotheses of epiretinal membrane pathogenesis.

  10. Diagnostic criteria and possible treatment of idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Prior, Thomas; Hilberg, Ole; Bendstrup, Elisabeth

    2014-01-01

    Pleuroparenchymal fibroelastosis is an idiopathic interstitial pneumonia, recently recognized as a new entity by American Thoracic Society/European Respiratory Society. We present the first Danish cases of pleuroparenchymal fibroelastosis in two young sisters, who had complained of dyspnoea since...

  11. [Idiopathic ventricular tachycardia, an arrhythmia with good prognosis].

    NARCIS (Netherlands)

    Camaro, C.; Bos, H.S.; Smeets, J.L.R.M.

    2010-01-01

    Three patients, one experiencing palpitations and two complaining of chest pain in stressful situations, appeared to have monomorphic wide complex tachycardia. After excluding channelopathy, structural abnormalities and ischaemia of the heart, this arrhythmia was classified as idiopathic. Symptoms d

  12. The idiopathic cavus foot-not so subtle after all.

    Science.gov (United States)

    Abbasian, Ali; Pomeroy, Gregory

    2013-12-01

    Idiopathic cavus deformity even in its mild form can result in several associated symptoms. Management of these symptoms without addressing the underlying biomechanical abnormality may result in failure of treatment. A careful clinical assessment is paramount.

  13. Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mar Carrion Martin, Maria del [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Ruiz Santiago, Fernando, E-mail: ferruizsan@terra.e [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Pozuelo Calvo, Rocio [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Guzman Alvarez, Luis [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain)

    2010-07-15

    Purpose: To compare clinical and computed tomography (CT) measures in extension, 20{sup o} and 30{sup o} of flexion of symptomatic knees of patient with idiopathic patellofemoral pain syndrome with the contra lateral asymptomatic knee. Materials and methods: Knees of 52 consecutive patients with idiopathic patellofemoral pain were studied with CT. In 28 patients this condition was unilateral and asymptomatic knee was used as control; 76 knees were symptomatic. Results: In patients with idiopathic patellofemoral pain we found a greater Q angle and internal condylar facet width in symptomatic knees with regard to asymptomatic knees. Conclusion: Greater Q angle and medial condylar facet can lead to overpressure on the medial knee compartment during maneuvers that increase contact between patella and medial condylar facet, such as knee flexion and squatting, contributing to development of idiopathic patellofemoral pain.

  14. Idiopathic internal resorption: Report of a case with unusual features

    Directory of Open Access Journals (Sweden)

    Santosh Hunasgi

    2012-01-01

    Full Text Available Tooth resorption can occur from the internal surface of a tooth or from the external surface of a tooth. Internal resorption is commonly termed to be "idiopathic" because of unknown cause. The aim is to report a case of idiopathic internal resorption showing unusual features. A 25-year-old female patient complains of mobility of tooth in right lower back tooth region since 2 months. Clinically, there was slight mobility in 48. Radiographically a resorptive area was seen in crown region of 48. The crown part was removed with gentle pressure using probe. A hollow crown with resorbed dentin and intact thin enamel was seen in gross specimen. A final diagnosis of idiopathic internal resorption was given. Early detection is essential for successful management of idiopathic internal resorption. This prevents further weakening of remaining tooth structure leading to crown or root perforations.

  15. PATTERN OF INHERITANCE OF IDIOPATHIC HYPERCALCIURIA IN TWO FAMILIES

    Directory of Open Access Journals (Sweden)

    A. Nickavar

    2006-09-01

    Full Text Available Idiopathic hypercalciuria is a leading cause of frequency-dysuria syndrome in childhood. Different modes of inheritance have been suggested in this disease. This article presents the occurrence of idiopathic hypercalciuria in all children of two families. In the first family, a 5.5 year old girl with a history of renal stones and dysuria due to hypercalciuria, had two involved brothers and one sister. In the second family, hypercalciuria and medullary nephrocalcinosis were detected in two siblings who were admitted for polyuria and dysuria. Idiopathic type of hypercalciuria was diagnosed in these two families by normal laboratory exams and exclusion of other causes of normocalcemic hypercalciuria. According to the involvement of all offsprings (both sexes in these two families, it is suggested that idiopathic hypercalciuria is an autosomal dominant disease with complete penetration.

  16. Gastroesophageal Reflux in Children with Refractory Asthma

    Directory of Open Access Journals (Sweden)

    Alaa S. Deeb

    2010-07-01

    Full Text Available Objectives: To investigate the association between clinical and macroscopic findings of GER and severity of refractory asthma (statistical study.Methods: A prospective study in a university-based practice of 75 children who were diagnosed with refractory asthma because they exhibited no satisfactory response for at least three months of treatment. Medical history, physical examination, spirometrical measurements and prick skin test were conducted on all patients. Endoscopic and macroscopic evaluations for esophagitis were performed on all patients regardless of the presence of GER symptoms.Results: Endoscopy was done for 75 children with refractory asthma. GER was symptomatic in 65% of all patients with no statistical significance (p>0.05 and the most frequent symptom was abdominal pain (67%. The frequency of these symptoms was 50% in mild asthma, 58% in moderate asthma and 72% in severe asthma with no statistical significance (p>0.05. The frequency of macroscopic esophagitis was 71%, distributed in three asthmatic groups as in order of 75% in mild asthma, 58.3% in moderate asthma and 76.6% in severe asthma (p>0.05. Regarding the relationship between GER and nocturnal attacks or spasmodic cough, the frequency of the latest was 66.7%. The differences have no statistical significance regarding GER symptoms (p>0.05, but they are statistically significant regarding the reflux esophagitis (p<0.05. Also endoscopic reflux was as frequent as76.3% in non allergic patients, and this result is statistically significant (p<0.05.Conclusion: In spite of the fact that there was no relationship between the severity of asthma and the symptoms of GER, or its endoscopic findings, the frequency of GER in asthmatic children was higher than its frequency in other children.

  17. Refractory thrombotic thrombocytopenic purpura following acute pancreatitis

    Institute of Scientific and Technical Information of China (English)

    Ebisa Bekele; Bethel Shiferaw; Alexandra Sokolova; Arpan Shah; Phillip Saunders; Alida Podrumar; Javed Iqbal

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with an estimated incidence of 4–5 cases per million people per year. It is characterized by small-vessel platelet-rich thrombi that cause thrombocytopenia, microangiopathic hemolytic anemia and organ damage. There are reports in literature that TTP and acute pancreatitis are associated, indicating each can be the cause of the other. However, acute pancreatitis triggering TTP is very rare. A 71 years old female presented with abdominal pain of 3 days, followed by dark urine. She had icteric sclera, petechial rash and mild epigastric tenderness. Lab findings were significant for hemolytic anemia, thrombocytopenia and elevated lipase. CT of abdomen showed evidence of pancreatitis and cholelithiasis. After admission, patient developed symptoms of stroke. Further investigation showed elevated lactate dehydrogenase and normal coagulation studied with peripheral blood smear showed 5–6 schistocytes/high power field. Disintegrin and metalloproteinase with thrombospondin motifs-13 (ADAMTS13) activity showed less than 3% with high ADAMTS13 inhibitor 2.2. Patient required 6–7 weeks of daily plasmapheresis until she showed complete response. Our patient presented with clinical features of pancreatitis prior to having dark urine and petechial rash. Therefore, we strongly believe that our patient had pancreatitis which was followed by TTP. Patient's ADMTS13 activity was 6%after 10 plasma exchanges, signifying refractory TTP and higher risk for morbidity and mortality. There are limited data and consensus on the management of refractory TTP. TTP and acute pancreatitis are associated. However, refractory TTP following acute pancreatitis is rarely mentioned in the literature. We would like to emphasize the importance of having higher clinical suspicion of the association of both disease entities.

  18. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Flossmann, O; Baslund, B; Bruchfeld, A

    2008-01-01

    at entry and prednisolone doses adjusted according to clinical status. Deoxyspergualin, 0.5 mg/kg per day, was self-administered by subcutaneous injection in six cycles of 21 days with a 7-day washout between cycles. Cycles were stopped early for white blood count less than 4000 cells/mm(3). The primary......-threatening (> or = grade 3) treatment-related adverse events occurred in 24 (53%) patients mostly due to leucopaenias. CONCLUSIONS: Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. Adverse events were common but rarely led...

  19. Flow and Flow Decay of Refractory Castables

    Institute of Scientific and Technical Information of China (English)

    CHEN Zhiqiang; Bjom Myhre; Bjorn Sandberg

    2003-01-01

    Installation of refractoty castables depend not only on flow, but also on how soon the flow is lost because of setting. The loss of flow (flow decay) has always been one of the main problems of refractory castable manufacturers,a problem that has not been too well described in literature. The flow decay has been studied for a castable system based on alumina, pointing out some general trends. The flow decay was found very temperature sensitive, being strongly accelerated by termperature increases. To compensate for excessive flow loss, a retarder like citric acid may be used. Thus flow decay was measured as a function of citric acid (retarder) addition at 35 ℃ .

  20. Refractory leg ulcers associated with Klinefelter syndrome.

    Science.gov (United States)

    Yabuno, Yuto; Tosa, Mamiko; Iwakiri, Itaru; Nomoto, Shunichi; Kaneko, Mayuko; Kuwahara, Kousuke; Hyakusoku, Hiko; Murakami, Masahiro

    2015-01-01

    We present a man with refractory leg ulcers, bilateral varicosis of the lower extremities, and Buerger disease. Autoimmune work-up was negative. However, chromosome analysis showed Klinefelter syndrome (48 XXY). Ulcerative lesions of the lower extremities are a complication of Klinefelter syndrome. To date, the pathogenesis of ulcers in Klinefelter syndrome has not been clarified, but several factors, such as abnormalities of fibrinolysis and prothrombotic states, might be involved. Our present case emphasizes the importance of considering Klinefelter syndrome in the differential diagnosis of a male patient with nonhealing ulcers of the lower extremities.